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Sample records for carbohydrate metabolism inborn errors

  1. Inborn errors of metabolism

    Science.gov (United States)

    ... metabolism. A few of them are: Fructose intolerance Galactosemia Maple sugar urine disease (MSUD) Phenylketonuria (PKU) Newborn ... disorder. Alternative Names Metabolism - inborn errors of Images Galactosemia References Bodamer OA. Approach to inborn errors of ...

  2. Inborn errors of cytoplasmic triglyceride metabolism.

    Science.gov (United States)

    Wu, Jiang Wei; Yang, Hao; Wang, Shu Pei; Soni, Krishnakant G; Brunel-Guitton, Catherine; Mitchell, Grant A

    2015-01-01

    Triglyceride (TG) synthesis, storage, and degradation together constitute cytoplasmic TG metabolism (CTGM). CTGM is mostly studied in adipocytes, where starting from glycerol-3-phosphate and fatty acyl (FA)-coenzyme A (CoA), TGs are synthesized then stored in cytoplasmic lipid droplets. TG hydrolysis proceeds sequentially, producing FAs and glycerol. Several reactions of CTGM can be catalyzed by more than one enzyme, creating great potential for complex tissue-specific physiology. In adipose tissue, CTGM provides FA as a systemic energy source during fasting and is related to obesity. Inborn errors and mouse models have demonstrated the importance of CTGM for non-adipose tissues, including skeletal muscle, myocardium and liver, because steatosis and dysfunction can occur. We discuss known inborn errors of CTGM, including deficiencies of: AGPAT2 (a form of generalized lipodystrophy), LPIN1 (childhood rhabdomyolysis), LPIN2 (an inflammatory condition, Majeed syndrome, described elsewhere in this issue), DGAT1 (protein loosing enteropathy), perilipin 1 (partial lipodystrophy), CGI-58 (gene ABHD5, neutral lipid storage disease (NLSD) with ichthyosis and "Jordan's anomaly" of vacuolated polymorphonuclear leukocytes), adipose triglyceride lipase (ATGL, gene PNPLA2, NLSD with myopathy, cardiomyopathy and Jordan's anomaly), hormone-sensitive lipase (HSL, gene LIPE, hypertriglyceridemia, and insulin resistance). Two inborn errors of glycerol metabolism are known: glycerol kinase (GK, causing pseudohypertriglyceridemia) and glycerol-3-phosphate dehydrogenase (GPD1, childhood hepatic steatosis). Mouse models often resemble human phenotypes but may diverge markedly. Inborn errors have been described for less than one-third of CTGM enzymes, and new phenotypes may yet be identified. PMID:25300978

  3. Screening for Inborn Errors of Metabolism

    Directory of Open Access Journals (Sweden)

    F.A. Elshaari

    2013-09-01

    Full Text Available Inborn errors of metabolism (IEM are a heterogeneous group of monogenic diseases that affect the metabolic pathways. The detection of IEM relies on a high index of clinical suspicion and co-ordinated access to specialized laboratory services. Biochemical analysis forms the basis of the final confirmed diagnosis in several of these disorders. The investigations fall into four main categories1.General metabolic screening tests2.Specific metabolite assays3.Enzyme studies4.DNA analysis The first approach to the diagnosis is by a multi-component analysis of body fluids in clinically selected patients, referred to as metabolic screening tests. These include simple chemical tests in the urine, blood glucose, acid-base profile, lactate, ammonia and liver function tests. The results of these tests can help to suggest known groups of metabolic disorders so that specific metabolites such as amino acids, organic acids, etc. can be estimated. However, not all IEM needs the approach of general screening. Lysosomal, peroxisomal, thyroid and adrenal disorders are suspected mainly on clinical grounds and pertinent diagnostic tests can be performed. The final diagnosis relies on the demonstration of the specific enzyme defect, which can be further confirmed by DNA studies.

  4. Infant with cardiomyopathy: When to suspect inborn errors of metabolism?

    Institute of Scientific and Technical Information of China (English)

    Stephanie; L; Byers; Can; Ficicioglu

    2014-01-01

    Inborn errors of metabolism are identified in 5%-26% of infants and children with cardiomyopathy. Although fatty acid oxidation disorders, lysosomal and glycogen storage disorders and organic acidurias are well-known to be associated with cardiomyopathies, emerging reports suggest that mitochondrial dysfunction and congenital disorders of glycosylation may also account for a proportion of cardiomyopathies. This review article clarifies when primary care physicians and cardiologists should suspect inborn errors of metabolism in a patient with cardiomyopathy, and refer the patient to a metabolic specialist for a further metabolic work up, with specific discussions of “red flags” which should prompt additional evaluation.

  5. Inborn Errors of Energy Metabolism Associated with Myopathies

    OpenAIRE

    Das, Anibh M.; Ulrike Steuerwald; Sabine Illsinger

    2010-01-01

    Inherited neuromuscular disorders affect approximately one in 3,500 children. Structural muscular defects are most common; however functional impairment of skeletal and cardiac muscle in both children and adults may be caused by inborn errors of energy metabolism as well. Patients suffering from metabolic myopathies due to compromised energy metabolism may present with exercise intolerance, muscle pain, reversible or progressive muscle weakness, and myoglobinuria. In this review, the physiolo...

  6. Inborn errors of metabolism: Clinical approach and management

    OpenAIRE

    Low, LCK

    1996-01-01

    Although individual metabolic disorders are rare, collectively, inborn errors of metabolism are not uncommon and paediatricians should be alert to the possibility of such disorders. The presenting symptoms are frequently non-specific and may include lethargy, poor feeding, vomiting, coma, and seizures. After investigations, appropriate therapeutic options including exchange transfusion, peritoneal- and haemo-dialysis, forced diuresis, mega-dosing of vitamin cofactors, and special dietary ther...

  7. Patterns of Brain Injury in Inborn Errors of Metabolism

    OpenAIRE

    Gropman, Andrea L.

    2012-01-01

    Many inborn errors of metabolism (IEMs) are associated with irreversible brain injury. For many, it is unclear how metabolite intoxication or substrate depletion accounts for the specific neurologic findings observed. IEM-associated brain injury patterns are characterized by whether the process involves gray matter, white matter, or both, and beyond that, whether subcortical or cortical gray matter nuclei are involved. Despite global insults, IEMs may result in selective injury to deep gray m...

  8. Inborn errors in the metabolism of glutathione

    Directory of Open Access Journals (Sweden)

    Larsson Agne

    2007-03-01

    Full Text Available Abstract Glutathione is a tripeptide composed of glutamate, cysteine and glycine. Glutathione is present in millimolar concentrations in most mammalian cells and it is involved in several fundamental biological functions, including free radical scavenging, detoxification of xenobiotics and carcinogens, redox reactions, biosynthesis of DNA, proteins and leukotrienes, as well as neurotransmission/neuromodulation. Glutathione is metabolised via the gamma-glutamyl cycle, which is catalyzed by six enzymes. In man, hereditary deficiencies have been found in five of the six enzymes. Glutathione synthetase deficiency is the most frequently recognized disorder and, in its severe form, it is associated with hemolytic anemia, metabolic acidosis, 5-oxoprolinuria, central nervous system (CNS damage and recurrent bacterial infections. Gamma-glutamylcysteine synthetase deficiency is also associated with hemolytic anemia, and some patients with this disorder show defects of neuromuscular function and generalized aminoaciduria. Gamma-glutamyl transpeptidase deficiency has been found in patients with CNS involvement and glutathionuria. 5-Oxoprolinase deficiency is associated with 5-oxoprolinuria but without a clear association with other symptoms. Dipeptidase deficiency has been described in one patient. All disorders are very rare and inherited in an autosomal recessive manner. Most of the mutations are leaky so that many patients have residual enzyme activity. Diagnosis is made by measuring the concentration of different metabolites in the gamma-glutamyl cycle, enzyme activity and in glutathione synthetase and gamma-glutamylcysteine synthetase deficiency, also by mutation analysis. Prenatal diagnosis has been preformed in glutathione synthetase deficiency. The prognosis is difficult to predict, as few patients are known, but seems to vary significantly between different patients. The aims of the treatment of glutathione synthesis defects are to avoid hemolytic

  9. The individual (single patient) IND for inborn errors of metabolism.

    Science.gov (United States)

    Dickson, Patricia I; Tolar, Jakub

    2015-01-01

    Under the United States Food and Drug Administration's Expanded Access program, a physician may treat a single patient with an experimental medication under an individual investigational new drug application (iIND). Metabolic geneticists may be among the specialists most likely to be asked to obtain an iIND, because there are many experimental treatments for inborn errors of metabolism which work in animals but suffer delays in translation to the bedside. The iIND has the potential to help bridge that gap, by gathering initial evidence in support of the use of an experimental drug in humans. If done with experimental rigor, the data will be useful, despite being limited to a single patient. However, iINDs may pose risks to drug development if patients are not carefully selected. Whatever their advantages and disadvantages, iIND studies are here to stay. Metabolic specialists should cautiously consider the iIND as a tremendous opportunity for therapeutic experimentation. PMID:25456747

  10. In vivo enzyme activity in inborn errors of metabolism

    International Nuclear Information System (INIS)

    Low-dose continuous infusions of [2H5]phenylalanine, [1-13C]propionate, and [1-13C]leucine were used to quantitate phenylalanine hydroxylation in phenylketonuria (PKU, four subjects), propionate oxidation in methylmalonic acidaemia (MMA, four subjects), and propionic acidaemia (PA, four subjects) and leucine oxidation in maple syrup urine disease (MSUD, four subjects). In vivo enzyme activity in PKU, MMA, and PA subjects was similar to or in excess of that in adult controls (range of phenylalanine hydroxylation in PKU, 3.7 to 6.5 mumol/kg/h, control 3.2 to 7.9, n = 7; propionate oxidation in MMA, 15.2 to 64.8 mumol/kg/h, and in PA, 11.1 to 36.0, control 5.1 to 19.0, n = 5). By contrast, in vivo leucine oxidation was undetectable in three of the four MSUD subjects (less than 0.5 mumol/kg/h) and negligible in the remaining subject (2 mumol/kg/h, control 10.4 to 15.7, n = 6). These results suggest that significant substrate removal can be achieved in some inborn metabolic errors either through stimulation of residual enzyme activity in defective enzyme systems or by activation of alternate metabolic pathways. Both possibilities almost certainly depend on gross elevation of substrate concentrations. By contrast, only minimal in vivo oxidation of leucine appears possible in MSUD

  11. Inborn errors of metabolism in Latin America: challenges and opportunities.

    Science.gov (United States)

    Giugliani, Roberto

    2010-10-01

    Latin America includes more than 40 countries and possessions, and its population of 570 million has an important representation of the three main human races. The area is experiencing an economic improvement, progressively bringing the inborn errors of metabolism (IEM) to a higher level among health priorities. Challenges to the progress of the IEM field include the huge disparities, the high prevalence of malnutrition and infections, the co-existence of very different models of public health services, the unstable socio-economic and political conditions, and the difficulties in integrating the countries. However, a rapidly changing social and economic environment is presenting many opportunities to the IEM field, like the improvements in infrastructure, the concentration of the population in urban areas, the continuous growth of neonatal screening, the use of filter paper samples, the availability of internet communication, and the interest in IEM by the new population medical genetics discipline. Analyzing this picture, several proposals are presented, such as the development of activities of provision of health services, education and research as an integrated package, the increase in training of human resources, the expansion of access to diagnostic tests, and the use the neonatal screening framework to expand the provision of services. In a continent with few IEM centers, there is a major need for such groups to work in collaboration, complementing each other's capabilities, providing training of human resources, and developing joint projects. The integration of these groups into a large transnational network of reference centers would be a major task for the coming years. PMID:20454860

  12. Identification and characterization of an inborn error of metabolism caused by dihydrofolate reductase deficiency

    NARCIS (Netherlands)

    Banka, S.; Blom, H.J.; Walter, J.; Aziz, M.; Urquhart, J.; Clouthier, C.M.; Rice, G.I.; Brouwer, A.P.M. de; Hilton, E.; Vassallo, G.; Will, A.; Smith, D.E.; Smulders, Y.M.; Wevers, R.A.; Steinfeld, R.; Heales, S.; Crow, Y.J.; Pelletier, J.N.; Jones, S.; Newman, W.G.

    2011-01-01

    Dihydrofolate reductase (DHFR) is a critical enzyme in folate metabolism and an important target of antineoplastic, antimicrobial, and antiinflammatory drugs. We describe three individuals from two families with a recessive inborn error of metabolism, characterized by megaloblastic anemia and/or pan

  13. Treatable newborn and infant seizures due to inborn errors of metabolism.

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    Campistol, Jaume; Plecko, Barbara

    2015-09-01

    About 25% of seizures in the neonatal period have causes other than asphyxia, ischaemia or intracranial bleeding. Among these are primary genetic epileptic encephalopathies with sometimes poor prognosis and high mortality. In addition, some forms of neonatal infant seizures are due to inborn errors of metabolism that do not respond to common AEDs, but are amenable to specific treatment. In this situation, early recognition can allow seizure control and will prevent neurological deterioration and long-term sequelae. We review the group of inborn errors of metabolism that lead to newborn/infant seizures and epilepsy, of which the treatment with cofactors is very different to that used in typical epilepsy management. PMID:26234933

  14. Neurocognitive clinical outcome assessments for inborn errors of metabolism and other rare conditions.

    Science.gov (United States)

    Shapiro, Elsa; Bernstein, Jessica; Adams, Heather R; Barbier, Ann J; Buracchio, Teresa; Como, Peter; Delaney, Kathleen A; Eichler, Florian; Goldsmith, Jonathan C; Hogan, Melissa; Kovacs, Sarrit; Mink, Jonathan W; Odenkirchen, Joanne; Parisi, Melissa A; Skrinar, Alison; Waisbren, Susan E; Mulberg, Andrew E

    2016-06-01

    Well-defined and reliable clinical outcome assessments are essential for determining whether a drug provides clinically meaningful treatment benefit for patients. In 2015, FDA convened a workshop, "Assessing Neurocognitive Outcomes in Inborn Errors of Metabolism." Topics covered included special challenges of clinical studies of inborn errors of metabolism (IEMs) and other rare diseases; complexities of identifying treatment effects in the context of the dynamic processes of child development and disease progression; and the importance of natural history studies. Clinicians, parents/caregivers, and participants from industry, academia, and government discussed factors to consider when developing measures to assess treatment outcomes, as well as tools and methods that may contribute to standardizing measures. Many issues examined are relevant to the broader field of rare diseases in addition to specifics of IEMs. PMID:27132782

  15. Report: Human biochemical genetics: an insight into inborn errors of metabolism

    Institute of Scientific and Technical Information of China (English)

    YU Chunli; SCOTT C. Ronald

    2006-01-01

    Inborn errors of metabolism (IEM) include a broad spectrum of defects of various gene products that affect intermediary metabolism in the body. Studying the molecular and biochemical mechanisms of those inherited disorder, systematically summarizing the disease phenotype and natural history, providing diagnostic rationale and methodology and treatment strategy comprise the context of human biochemical genetics. This session focused on: (1) manifestations of representative metabolic disorders; (2) the emergent technology and application of newborn screening of metabolic disorders using tandem mass spectrometry; (3) principles of managing IEM; (4) the concept of carrier testing aiming prevention. Early detection of patients with IEM allows early intervention and more options for treatment.

  16. Treatable inborn errors of metabolism presenting as cerebral palsy mimics: systematic literature review

    OpenAIRE

    Leach, Emma L; Shevell, Michael; Bowden, Kristin; Stockler-Ipsiroglu, Sylvia; van Karnebeek, Clara DM

    2014-01-01

    Background Inborn errors of metabolism (IEMs) have been anecdotally reported in the literature as presenting with features of cerebral palsy (CP) or misdiagnosed as ‘atypical CP’. A significant proportion is amenable to treatment either directly targeting the underlying pathophysiology (often with improvement of symptoms) or with the potential to halt disease progression and prevent/minimize further damage. Methods We performed a systematic literature review to identify all reports of IEMs pr...

  17. Metabolic Modeling of Inborn Errors of Metabolism: Carnitine Palmitoyltransferase II Deficiency and Respiratory Chain Complex I Deficiency

    OpenAIRE

    Brewer, Judy

    2015-01-01

    The research goal was to assess the current capabilities of a metabolic modeling environment to support exploration of inborn errors of metabolism (IEMs); and to assess whether, drawing on evidence from published studies of EMs, the current capabilities of this modeling environment correlate with clinical measures of energy production, fatty acid oxidation, accumulation of toxic by-products of defective metabolism, and mitigation via therapeutic agents. IEMs comprise several hundred disor...

  18. Risk factors and birth prevalence of birth defects and inborn errors of metabolism in Al Ahsa, Saudi Arabia

    OpenAIRE

    Waleed Hamad Al Bu Ali; Magdy Hassan Balaha; Mohammed Saleh Al Moghannum; Ibrahim Hashim

    2011-01-01

    Background Birth defects and inborn errors of metabolism are related to variable poor perinatal and neonatal outcomes. Our aim was to explore the pattern and prevalence of birth defects and metabolic birth errors in Al-Ahsa Governorate in the Eastern Province of Saudi Arabia. Methods This retrospective case control study was done from April 2006 to 2009. Children with any birth defect or metabolic errors of metabolism at birth or in the neonatology section were our sample for study. Control g...

  19. An introduction to nutritional treatment in inborn errors of metabolism--different disorders, different approaches.

    Science.gov (United States)

    Wilcken, Bridget

    2003-01-01

    catabolism. Glycogen storage disorders require strict attention to providing carbohydrate, at all times including throughout the night. Many patients with inborn errors do not need any specific dietary therapy, (eg those with storage or neurodegenerative disorders), although all children benefit from an optimal diet, and sick children need this especially. PMID:15906735

  20. Perspectives on dietary adherence among women with inborn errors of metabolism.

    Science.gov (United States)

    Kemper, Alex R; Brewer, Cheryl A; Singh, Rani H

    2010-02-01

    Adherence to highly restrictive diets is critical for women of childbearing age who have inborn errors of metabolism such as phenylketonuria. The purpose of this study was to explore attitudes about diet, barriers to and facilitators of dietary adherence, and experiences with the health care system in promoting dietary adherence among adolescent and adult women with inborn errors of metabolism to identify policy-level interventions to improve adherence. We analyzed the results of four focus groups including a total of 19 women between the ages of 12 and 52 years with phenylketonuria, methylmalonic acidemia, or maple syrup urine disease attending an educational summer camp in 2008. Themes were identified after independent analysis of transcripts. Most participants were highly knowledgeable about their dietary requirements and some could describe their own specific negative experiences of nonadherence. Many reported specific challenges, such as feelings of being different, that they experienced in elementary and middle school. Friends and family play an important role in maintaining dietary adherence. Participants identified one registered dietitian in particular who has played an important supportive role. Insurance coverage for medical foods was a common concern. Most participants identified concerns about transitioning from pediatric to adult medical services. We identified four specific strategies for future evaluation that may improve dietary adherence and health outcomes for women and their potential offspring: symptom-based dietary monitoring for some, educating school officials about medical diets, expanding the role of registered dietitians; and assisting with the transition from pediatric to adult health care providers. PMID:20102852

  1. Tratamento de erros inatos do metabolismo Treatment of inborn errors of metabolism

    Directory of Open Access Journals (Sweden)

    Ida Vanessa Schwartz

    2008-08-01

    metabolism, priority was given to the most important methods for managing intoxication, in view of the importance for pediatricians to treat acute and life-threatening cases. The article also provides a general overview of the treatment for lysosomal and peroxisomal diseases, with emphasis on enzyme replacement therapy, which is a treatment modality that is growing in use and with which pediatricians should make themselves familiar. SUMMARY OF THE FINDINGS: The most important measures used to manage the intoxication present in many inborn errors of intermediate metabolism were presented (restriction of substrate build-up by means of diet or enzymatic inhibition, removal of toxic products, stimulation of residual enzyme activity, replacement of the deficient product. The section on treatment for lysosomal and peroxisomal diseases includes tables providing information on the treatments available. CONCLUSIONS: Treating inborn errors of metabolism is a complex task that should be performed by a multidisciplinary team of which the pediatrician is the key member. This article provides practical information relating to the management of some inborn errors of metabolism and provides pediatricians with a general overview of recent developments in this area of medicine.

  2. Management and emergency treatments of neonates with a suspicion of inborn errors of metabolism.

    Science.gov (United States)

    Ogier de Baulny, Hélène

    2002-02-01

    During the neonatal period, inborn errors of metabolism mostly present with an overwhelming illness that requires prompt diagnosis and both supportive and specific treatments. The most frequent situations are due to branched-chain organic acidurias that present with ketoacidosis and urea cycle defects that are characterized by hyperammonaemia. During both situations, toxin removal procedures and nutritional support with a free-protein and high-energy diet are pivotal treatments. In patients presenting with hypoglycaemia blood glucose levels must be corrected. Progress following glucose provision is useful in recognizing the disorders that are mainly implicated. Hyperinsulinism requires high-glucose infusion. Glycogen storage diseases and gluconeogenesis defects are easily treated with a permanent glucose provision while hypoglycaemias quickly recur. In patients with galactosaemia, hereditary fructose intolerance or tyrosinaemia type I, the presentation is dominated by a liver failure requiring galactose and fructose exclusion associated with a low-protein diet. Many patients with beta-oxidation defects may present with hypoglycaemia that is usually easily corrected. The precise diagnosis can be easily missed in those patients that do well in the following weeks but may develop cardiac failure, arrhythmia and/or liver failure. Patients presenting with intractable convulsions, vitamin responsiveness to biotin, pyridoxine and folate must be considered. PMID:12069535

  3. Targeted Next Generation Sequencing in Patients with Inborn Errors of Metabolism

    Science.gov (United States)

    Yubero, Dèlia; Brandi, Núria; Ormazabal, Aida; Garcia-Cazorla, Àngels; Pérez-Dueñas, Belén; Campistol, Jaime; Ribes, Antonia; Palau, Francesc

    2016-01-01

    Background Next-generation sequencing (NGS) technology has allowed the promotion of genetic diagnosis and are becoming increasingly inexpensive and faster. To evaluate the utility of NGS in the clinical field, a targeted genetic panel approach was designed for the diagnosis of a set of inborn errors of metabolism (IEM). The final aim of the study was to compare the findings for the diagnostic yield of NGS in patients who presented with consistent clinical and biochemical suspicion of IEM with those obtained for patients who did not have specific biomarkers. Methods The subjects studied (n = 146) were classified into two categories: Group 1 (n = 81), which consisted of patients with clinical and biochemical suspicion of IEM, and Group 2 (n = 65), which consisted of IEM cases with clinical suspicion and unspecific biomarkers. A total of 171 genes were analyzed using a custom targeted panel of genes followed by Sanger validation. Results Genetic diagnosis was achieved in 50% of patients (73/146). In addition, the diagnostic yield obtained for Group 1 was 78% (63/81), and this rate decreased to 15.4% (10/65) in Group 2 (X2 = 76.171; p < 0.0001). Conclusions A rapid and effective genetic diagnosis was achieved in our cohort, particularly the group that had both clinical and biochemical indications for the diagnosis. PMID:27243974

  4. Long-Chain Polyunsaturated Fatty Acids in Inborn Errors of Metabolism

    Directory of Open Access Journals (Sweden)

    Katalin Fekete

    2010-09-01

    Full Text Available The treatment of children with inborn errors of metabolism (IEM is mainly based on restricted dietary intake of protein-containing foods. However, dietary protein restriction may not only reduce amino acid intake, but may be associated with low intake of polyunsaturated fatty acids as well. This review focuses on the consequences of dietary restriction in IEM on the bioavailability of long-chain polyunsaturated fatty acids (LCPUFAs and on the attempts to ameliorate these consequences. We were able to identify during a literature search 10 observational studies investigating LCPUFA status in patients with IEM and six randomized controlled trials (RCTs reporting effect of LCPUFA supplementation to the diet of children with IEM. Decreased LCPUFA status, in particular decreased docosahexaenoic acid (DHA status, has been found in patients suffering from IEM based on the evidence of observational studies. LCPUFA supplementation effectively improves DHA status without detectable adverse reactions. Further research should focus on functional outcomes of LCPUFA supplementation in children with IEM.

  5. Inborn errors of metabolism in the biosynthesis and remodelling of phospholipids.

    Science.gov (United States)

    Wortmann, Saskia B; Espeel, Marc; Almeida, Ligia; Reimer, Annette; Bosboom, Dennis; Roels, Frank; de Brouwer, Arjan P M; Wevers, Ron A

    2015-01-01

    Since the proposal to define a separate subgroup of inborn errors of metabolism involved in the biosynthesis and remodelling of phospholipids, sphingolipids and long chain fatty acids in 2013, this group is rapidly expanding. This review focuses on the disorders involved in the biosynthesis of phospholipids. Phospholipids are involved in uncountable cellular processes, e.g. as structural components of membranes, by taking part in vesicle and mitochondrial fusion and fission or signal transduction. Here we provide an overview on both pathophysiology and the extremely heterogeneous clinical presentations of the disorders reported so far (Sengers syndrome (due to mutations in AGK), MEGDEL syndrome (or SERAC defect, SERAC1), Barth syndrome (or TAZ defect, TAZ), congenital muscular dystrophy due to CHKB deficiency (CHKB). Boucher-Neuhäuser/Gordon Holmes syndrome (PNPLA6), PHARC syndrome (ABHD12), hereditary spastic paraplegia type 28, 54 and 56 (HSP28, DDHD1; HSP54, DDHD2; HSP56, CYP2U1), Lenz Majewski syndrome (PTDSS1), spondylometaphyseal dysplasia with cone-rod dystrophy (PCYT1A), atypical haemolytic-uremic syndrome due to DGKE deficiency (DGKE). PMID:25178427

  6. Inborn errors of metabolism with 3-methylglutaconic aciduria as discriminative feature: proper classification and nomenclature.

    Science.gov (United States)

    Wortmann, Saskia B; Duran, Marinus; Anikster, Yair; Barth, Peter G; Sperl, Wolfgang; Zschocke, Johannes; Morava, Eva; Wevers, Ron A

    2013-11-01

    Increased urinary 3-methylglutaconic acid excretion is a relatively common finding in metabolic disorders, especially in mitochondrial disorders. In most cases 3-methylglutaconic acid is only slightly elevated and accompanied by other (disease specific) metabolites. There is, however, a group of disorders with significantly and consistently increased 3-methylglutaconic acid excretion, where the 3-methylglutaconic aciduria is a hallmark of the phenotype and the key to diagnosis. Until now these disorders were labelled by roman numbers (I-V) in the order of discovery regardless of pathomechanism. Especially, the so called "unspecified" 3-methylglutaconic aciduria type IV has been ever growing, leading to biochemical and clinical diagnostic confusion. Therefore, we propose the following pathomechanism based classification and a simplified diagnostic flow chart for these "inborn errors of metabolism with 3-methylglutaconic aciduria as discriminative feature". One should distinguish between "primary 3-methylglutaconic aciduria" formerly known as type I (3-methylglutaconyl-CoA hydratase deficiency, AUH defect) due to defective leucine catabolism and the--currently known--three groups of "secondary 3-methylglutaconic aciduria". The latter should be further classified and named by their defective protein or the historical name as follows: i) defective phospholipid remodelling (TAZ defect or Barth syndrome, SERAC1 defect or MEGDEL syndrome) and ii) mitochondrial membrane associated disorders (OPA3 defect or Costeff syndrome, DNAJC19 defect or DCMA syndrome, TMEM70 defect). The remaining patients with significant and consistent 3-methylglutaconic aciduria in whom the above mentioned syndromes have been excluded, should be referred to as "not otherwise specified (NOS) 3-MGA-uria" until elucidation of the underlying pathomechanism enables proper (possibly extended) classification. PMID:23296368

  7. Living donor liver transplantation for inborn errors of metabolism - An underutilized resource in the United States.

    Science.gov (United States)

    Pham, Thomas A; Enns, Gregory M; Esquivel, Carlos O

    2016-09-01

    Inborn metabolic diseases of the liver can be life-threatening disorders that cause debilitating and permanent neurological damage. Symptoms may manifest as early as the neonatal period. Liver transplant replaces the enzymatically deficient liver, allowing for metabolism of toxic metabolites. LDLT for metabolic disorders is rarely performed in the United States as compared to countries such as Japan, where they report >2000 cases performed within the past two decades. Patient and graft survival is comparable to that of the United States, where most of the studies are based on deceased donors. No living donor complications were observed, suggesting that LDLT is as safe and effective as deceased donor transplants performed in the USA. Increased utilization of living donors in the USA will allow for early transplantation to prevent permanent neurological damage in those with severe disease. Pediatric transplant centers should consider utilizing living donors when feasible for children with metabolic disorders of the liver. PMID:27392539

  8. Inborn errors of metabolism and expanded newborn screening: review and update.

    Science.gov (United States)

    Mak, Chloe Miu; Lee, Han-Chih Hencher; Chan, Albert Yan-Wo; Lam, Ching-Wan

    2013-11-01

    Inborn errors of metabolism (IEM) are a phenotypically and genetically heterogeneous group of disorders caused by a defect in a metabolic pathway, leading to malfunctioning metabolism and/or the accumulation of toxic intermediate metabolites. To date, more than 1000 different IEM have been identified. While individually rare, the cumulative incidence has been shown to be upwards of 1 in 800. Clinical presentations are protean, complicating diagnostic pathways. IEM are present in all ethnic groups and across every age. Some IEM are amenable to treatment, with promising outcomes. However, high clinical suspicion alone is not sufficient to reduce morbidities and mortalities. In the last decade, due to the advent of tandem mass spectrometry, expanded newborn screening (NBS) has become a mandatory public health strategy in most developed and developing countries. The technology allows inexpensive simultaneous detection of more than 30 different metabolic disorders in one single blood spot specimen at a cost of about USD 10 per baby, with commendable analytical accuracy and precision. The sensitivity and specificity of this method can be up to 99% and 99.995%, respectively, for most amino acid disorders, organic acidemias, and fatty acid oxidation defects. Cost-effectiveness studies have confirmed that the savings achieved through the use of expanded NBS programs are significantly greater than the costs of implementation. The adverse effects of false positive results are negligible in view of the economic health benefits generated by expanded NBS and these could be minimized through increased education, better communication, and improved technologies. Local screening agencies should be given the autonomy to develop their screening programs in order to keep pace with international advancements. The development of biochemical genetics is closely linked with expanded NBS. With ongoing advancements in nanotechnology and molecular genomics, the field of biochemical genetics

  9. Principal component analysis of urine metabolites detected by NMR and DESI-MS in patients with inborn errors of metabolism.

    Science.gov (United States)

    Pan, Zhengzheng; Gu, Haiwei; Talaty, Nari; Chen, Huanwen; Shanaiah, Narasimhamurthy; Hainline, Bryan E; Cooks, R Graham; Raftery, Daniel

    2007-01-01

    Urine metabolic profiles of patients with inborn errors of metabolism were examined with nuclear magnetic resonance (NMR) and desorption electrospray ionization mass spectrometry (DESI-MS) methods. Spectra obtained from the study of urine samples from individual patients with argininosuccinic aciduria (ASA), classic homocystinuria (HCY), classic methylmalonic acidemia (MMA), maple syrup urine disease (MSUD), phenylketonuria (PKU) and type II tyrosinemia (TYRO) were compared with six control patient urine samples using principal component analysis (PCA). Target molecule spectra were identified from the loading plots of PCA output and compared with known metabolic profiles from the literature and metabolite databases. Results obtained from the two techniques were then correlated to obtain a common list of molecules associated with the different diseases and metabolic pathways. The combined approach discussed here may prove useful in the rapid screening of biological fluids from sick patients and may help to improve the understanding of these rare diseases. PMID:16821030

  10. Clinical biological and genetic heterogeneity of the inborn errors of pulmonary surfactant metabolism.

    Science.gov (United States)

    Tredano, M; De Blic, J; Griese, M; Fournet, J C; Elion, J; Bahuau, M

    2001-02-01

    Pulmonary surfactant is a multimolecular complex located at the air-water interface within the alveolus to which a range of physical (surface-active properties) and immune functions has been assigned. This complex consists of a surface-active lipid layer (consisting mainly of phospholipids), and of an aqueous subphase. From discrete surfactant sub-fractions one can isolate strongly hydrophobic surfactant proteins B (SP-B) and C (SP-C) as well as collectins SP-A and SP-D, which were shown to have specific structural, metabolic, or immune properties. Inborn or acquired abnormalities of the surfactant, qualitative or quantitative in nature, account for a number of human diseases. Beside hyaline membrane disease of the preterm neonate, a cluster of hereditary or acquired lung diseases has been characterized by periodic acid-Schiff-positive material filling the alveoli. From this heterogeneous nosologic group, at least two discrete entities presently emerge. The first is the SP-B deficiency, in which an essentially proteinaceous material is stored within the alveoli, and which represents an autosomal recessive Mendelian entity linked to the SFTPB gene (MIM 1786640). The disease usually generally entails neonatal respiratory distress with rapid fatal outcome, although partial or transient deficiencies have also been observed. The second is alveolar proteinosis, characterized by the storage of a mixed protein and lipid material, which constitutes a relatively heterogeneous clinical and biological syndrome, especially with regard to age at onset (from the neonate through to adulthood) as well as the severity of associated signs. Murine models, with a targeted mutation of the gene encoding granulocyte macrophage colony-stimulating factor (GM-CSF) (Csfgm) or the beta subunit of its receptor (II3rb1) support the hypothesis of an abnormality of surfactant turnover in which the alveolar macrophage is a key player. Apart from SP-B deficiency, in which a near-consensus diagnostic

  11. Achieving the "triple aim" for inborn errors of metabolism: a review of challenges to outcomes research and presentation of a new practice-based evidence framework.

    Science.gov (United States)

    Potter, Beth K; Chakraborty, Pranesh; Kronick, Jonathan B; Wilson, Kumanan; Coyle, Doug; Feigenbaum, Annette; Geraghty, Michael T; Karaceper, Maria D; Little, Julian; Mhanni, Aizeddin; Mitchell, John J; Siriwardena, Komudi; Wilson, Brenda J; Syrowatka, Ania

    2013-06-01

    Across all areas of health care, decision makers are in pursuit of what Berwick and colleagues have called the "triple aim": improving patient experiences with care, improving health outcomes, and managing health system impacts. This is challenging in a rare disease context, as exemplified by inborn errors of metabolism. There is a need for evaluative outcomes research to support effective and appropriate care for inborn errors of metabolism. We suggest that such research should consider interventions at both the level of the health system (e.g., early detection through newborn screening, programs to provide access to treatments) and the level of individual patient care (e.g., orphan drugs, medical foods). We have developed a practice-based evidence framework to guide outcomes research for inborn errors of metabolism. Focusing on outcomes across the triple aim, this framework integrates three priority themes: tailoring care in the context of clinical heterogeneity; a shift from "urgent care" to "opportunity for improvement"; and the need to evaluate the comparative effectiveness of emerging and established therapies. Guided by the framework, a new Canadian research network has been established to generate knowledge that will inform the design and delivery of health services for patients with inborn errors of metabolism and other rare diseases. PMID:23222662

  12. Glycosidases: inborn errors of glycosphingolipid catabolism.

    Science.gov (United States)

    Ashida, Hisashi; Li, Yu-Teh

    2014-01-01

    Glycosphingolipids (GSLs) are information-rich glycoconjugates that occur in nature mainly as constituents of biomembranes. Each GSL contains a complex carbohydrate chain linked to a ceramide moiety that anchors the molecule to biomembranes. In higher animals, catabolism of GSLs takes place in lysosomes where sugar chains in GSLs are hydrolyzed by exo-glycosidases to cleave a sugar residue from the non-reducing end of a sugar chain. Inborn errors of GSL-catabolism, collectively called sphingolipidoses or GSL-storage diseases, are caused by the deficiency of exo-glycosidases responsible for the degradation of the specific sugar residues at the non-reducing termini in GSLs. This chapter briefly discusses glycone, anomeric, linkage, and aglycone specificities of exo-glycosidases and some of the historical landmarks on their associations with the chemical pathology of the five best known sphingolipidoses: GM1 gangliosidosis, GM2 gangliosidosis (Tay-Sachs disease), Fabry disease, Gaucher disease, and Krabbe disease. PMID:25151392

  13. 遗传代谢病与小儿癫(癎)%Inborn errors of metabolism and epilepsy in children

    Institute of Scientific and Technical Information of China (English)

    张月华; 田小娟

    2015-01-01

    Epilepsy is a common disorder of neurological system in children.Inborn errors of metabolism (IEMs) is one of an important etiology of epilepsy.Seizures may be the first and the major presenting feature of IEMs.IEMs are a relatively rare cause of epilepsy,but their recognition and diagnosis is very important because several disorders are treatable.This review will concentrate on diseases where epilepsy is the predominant clinical manifestation and especially where the disease course can be positively influenced by specific metabolic therapies.%癫(癎)是小儿神经系统常见疾病,遗传代谢病(IEMs)是导致癫(癎)的重要病因之一.IEMs以神经系统受累最常见,癫(癎)发作可作为首发症状或主要症状.虽然IEMs导致癫(癎)相对少见,但早期识别和明确诊断非常重要,因为部分IEMs有可以治疗的方法.患儿的预后与IEMs的类型及诊断和治疗是否及时密切相关.现将结合发病年龄重点介绍与癫(癎)密切相关的IEMs,尤其是可治疗的IEMs.

  14. Inborn errors of metabolism identified via newborn screening: Ten-year incidence data and costs of nutritional interventions for research agenda planning.

    Science.gov (United States)

    Therrell, Bradford L; Lloyd-Puryear, Michele A; Camp, Kathryn M; Mann, Marie Y

    2014-01-01

    Inborn errors of metabolism (IEM) are genetic disorders in which specific enzyme defects interfere with the normal metabolism of exogenous (dietary) or endogenous protein, carbohydrate, or fat. In the U.S., many IEM are detected through state newborn screening (NBS) programs. To inform research on IEM and provide necessary resources for researchers, we are providing: tabulation of ten-year state NBS data for selected IEM detected through NBS; costs of medical foods used in the management of IEM; and an assessment of corporate policies regarding provision of nutritional interventions at no or reduced cost to individuals with IEM. The calculated IEM incidences are based on analyses of ten-year data (2001-2011) from the National Newborn Screening Information System (NNSIS). Costs to feed an average person with an IEM were approximated by determining costs to feed an individual with an IEM, minus the annual expenditure for food for an individual without an IEM. Both the incidence and costs of nutritional intervention data will be useful in future research concerning the impact of IEM disorders on families, individuals and society. PMID:25085281

  15. Inborn errors of metabolism identified via newborn screening: Ten-year incidence data and costs of nutritional interventions for research agenda planning✰

    Science.gov (United States)

    Therrell, Bradford L.; Lloyd-Puryear, Michele A.; Camp, Kathryn M.; Mann, Marie Y.

    2014-01-01

    Inborn errors of metabolism (IEM) are genetic disorders in which specific enzyme defects interfere with the normal metabolism of exogenous (dietary) or endogenous protein, carbohydrate, or fat. In the U.S., many IEM are detected through state newborn screening (NBS) programs. To inform research on IEM and provide necessary resources for researchers, we are providing: tabulation of ten-year state NBS data for selected IEM detected through NBS; costs of medical foods used in the management of IEM; and an assessment of corporate policies regarding provision of nutritional interventions at no or reduced cost to individuals with IEM. The calculated IEM incidences are based on analyses of ten-year data (2001–2011) from the National Newborn Screening Information System (NNSIS). Costs to feed an average person with an IEM were approximated by determining costs to feed an individual with an IEM, minus the annual expenditure for food for an individual without an IEM. Both the incidence and costs of nutritional intervention data will be useful in future research concerning the impact of IEM disorders on families, individuals and society. PMID:25085281

  16. Neonatal Screening for Inborn Errors of Metabolism in Shanghai%上海地区遗传代谢病的新生儿筛查

    Institute of Scientific and Technical Information of China (English)

    顾学范; 叶军; 韩连书

    2009-01-01

    Objectives Inborn errors of metabolism (IEM) has a diverse spectrum and different incidence in different countries, the early diagnosis at presymptomatic stage is imperative to benefic patient from sequelae. Phenylke-tonuria (PKU) / hyperphenylalaninemia (HPA) is the most common metabolism disorder in Shanghai as well as in other regions. The study is to further clarify the incidence of inborn errors of metabolism among newborn in Shanghai. Methods The dried blood spot specimens were collected from near 90 local maternity and children's hospitals or general hospitals in Shanghai. PKU/HPA screening was carried out by fluorometric method. Neonatal screening using tandem mass spectrometry was performed in one of the study centers, Xinhua neonatal screening center. Results A total of 815 160 cases were screened from 2001 - 2007 in Shanghai, the incidence of PKU/HPA was 1 : 12 351. The tetrahydrobiopterin deficiency was 12.9% among hyperphenylalaninemia patients. According to the 116 000 neonatal samples data detected by tandem mass spectrometry, 20 cases were confirmed diagnosis, including 6 kinds diseases, it was PKU/HPA, maple syrup urine disease, methylmalonicacidemia, propionic acidemia, 3-methylcrotonyl-CoA carboxylase defection, and short chain aeyl-CoA dehydrogenase deficiency. Conclusions The pilot study shown that inborn errors of metabolism neonatal screen-ing using tandem mass was 1 : 5 800 in Shanghai, PKU/HPA was the most common disease. It is expected that the expansion of newborn screening using tandem mass spectrometry could be further considered and further improving inborn errors of metabolism preventive services in Shanghai.

  17. Evidence for Treatable Inborn Errors of Metabolism in a Cohort of 187 Greek Patients with Autism Spectrum Disorder (ASD

    Directory of Open Access Journals (Sweden)

    Martha eSpilioti

    2013-12-01

    Full Text Available We screened for the presence of inborn errors of metabolism (IEM in 187 children (105 males; 82 females, ages 4 -14 years old who presented with confirmed features of ASD. Twelve patients (7% manifested increased 3-hydroxyisovaleric acid (3-OH-IVA excretion in urine, and minor to significant improvement in autistic features was observed in seven patients following supplementation with biotin. Five diagnoses included: Lesch Nyhan syndrome (2, succinic semialdehyde dehydrogenase (SSADH deficiency (2 and phenylketonuria (1 (2.7%. Additional metabolic disturbances suggestive of IEMs included two patients whose increased urine 3-OH-IVA was accompanied by elevated methylcitrate and lactate in sera, and 30 patients that showed abnormal glucose-loading tests. In the latter group, 16/30 patients manifested increased sera beta hydroxybutyrate (b-OH-b production and 18/30 had a paradoxical increase of sera lactate. Six patients with elevated b-OH-b in sera showed improved autistic features following implementation of a ketogenic diet. Five patients showed decreased serum ketone body production with glucose loading. Twelve of 187 patients demonstrated nonspecific MRI pathology, while 25/187 had abnormal EEG findings. Finally, family history was positive for 22/187 patients (1st or 2nd degree relative with comparable symptomatology and consanguinity was documented for 12/187 patients. Our data provide evidence for a new biomarker (3-OH-IVA and novel treatment approaches in ASD patients.Concise 1 sentence take-home message: Detailed metabolic screening in a Greek cohort of autismspectrum disorder (ASD patients revealed biomarkers (urine 3-hydroxyisovaleric acid and serum b-OH-b in 7% (13/187 of patients for whom biotin supplementation or institution of a ketogenic diet resulted in mild to significant clinical improvement in autistic features.

  18. Evidence for treatable inborn errors of metabolism in a cohort of 187 Greek patients with autism spectrum disorder (ASD).

    Science.gov (United States)

    Spilioti, Martha; Evangeliou, Athanasios E; Tramma, Despoina; Theodoridou, Zoe; Metaxas, Spyridon; Michailidi, Eleni; Bonti, Eleni; Frysira, Helen; Haidopoulou, A; Asprangathou, Despoina; Tsalkidis, Aggelos J; Kardaras, Panagiotis; Wevers, Ron A; Jakobs, Cornelis; Gibson, K Michael

    2013-01-01

    We screened for the presence of inborn errors of metabolism (IEM) in 187 children (105 males; 82 females, ages 4-14 years old) who presented with confirmed features of autism spectrum disorder (ASD). Twelve patients (7%) manifested increased 3-hydroxyisovaleric acid (3-OH-IVA) excretion in urine, and minor to significant improvement in autistic features was observed in seven patients following supplementation with biotin. Five diagnoses included: Lesch Nyhan syndrome (2), succinic semialdehyde dehydrogenase (SSADH) deficiency (2), and phenylketonuria (1) (2.7%). Additional metabolic disturbances suggestive of IEMs included two patients whose increased urine 3-OH-IVA was accompanied by elevated methylcitrate and lactate in sera, and 30 patients that showed abnormal glucose-loading tests. In the latter group, 16/30 patients manifested increased sera beta hydroxybutyrate (b-OH-b) production and 18/30 had a paradoxical increase of sera lactate. Six patients with elevated b-OH-b in sera showed improved autistic features following implementation of a ketogenic diet (KD). Five patients showed decreased serum ketone body production with glucose loading. Twelve of 187 patients demonstrated non-specific MRI pathology, while 25/187 had abnormal electroencephalogram (EEG) findings. Finally, family history was positive for 22/187 patients (1st or 2nd degree relative with comparable symptomatology) and consanguinity was documented for 12/187 patients. Our data provide evidence for a new biomarker (3-OH-IVA) and novel treatment approaches in ASD patients. Concise 1 sentence take-home message: Detailed metabolic screening in a Greek cohort of ASD patients revealed biomarkers (urine 3-hydroxyisovaleric acid and serum b-OH-b) in 7% (13/187) of patients for whom biotin supplementation or institution of a KD resulted in mild to significant clinical improvement in autistic features. PMID:24399946

  19. Nutritional Treatment for Inborn Errors of Metabolism: Indications, Regulations, and Availability of Medical Foods and Dietary Supplements Using Phenylketonuria as an Example

    OpenAIRE

    Camp, Kathryn M.; Lloyd-Puryear, Michele A.; Huntington, Kathleen L.

    2012-01-01

    Medical foods and dietary supplements are used to treat rare inborn errors of metabolism (IEM) identified through state-based universal newborn screening. These products are regulated under Food and Drug Administration (FDA) food and dietary supplement statutes. The lack of harmony in terminology used to refer to medical foods and dietary supplements and the misuse of words that imply that FDA regulates these products as drugs have led to confusion. These products are expensive and, although ...

  20. Three new cases of late-onset cblC defect and review of the literature illustrating when to consider inborn errors of metabolism beyond infancy

    OpenAIRE

    Huemer, Martina; Scholl-Bürgi, Sabine; Hadaya, Karine; Kern, Ilse; Beer, Ronny; Seppi, Klaus; Fowler, Brian; Baumgartner, Matthias R; Karall, Daniela

    2014-01-01

    Background The cblC defect is a rare inborn error of intracellular cobalamin metabolism. Biochemical hallmarks are elevated homocysteine and low methionine in plasma accompanied by methylmalonic aciduria. Due to the heterogeneous clinical picture, patients with the late-onset form of the disease (onset >12 months) come to the attention of diverse medical specialists, e.g. paediatricians, neurologists, nephrologists, psychiatrists or haematologists. The report reviews the published clinical da...

  1. Selective screening of 650 high risk Iranian patients for detection of inborn error of metabolism

    Directory of Open Access Journals (Sweden)

    Narges Pishva

    2015-02-01

    Full Text Available Objective: Although metabolic diseases individually are rare ,but overall have an incidence of 1/2000 and can cause devastating and irreversible effect if not diagnosed early and treated promptly. selective screening is an acceptable method for detection of these multi presentation diseases.Method: using panel neonatal screening for detection of metabolic diseases in 650 high risk Iranian patients in Fars province. The following clinical features were used as inclusion criteria for investigation of the patients.Lethargy, poor feeding ,persistent vomiting, cholestasis, intractable seizure ,decreased level of consciousness ,persistent hypoglycemia, unexplained acid base disturbance and unexplained neonatal death.Result: Organic acidemia with 40 cases (42% was the most frequent disorder diagnosed in our high risk populations, followed by disorder of galactose metabolism(30%, 15 patient had classic galactosemia(GALT

  2. Selective screening of 650 high risk Iranian patients for detection of inborn error of metabolism

    Directory of Open Access Journals (Sweden)

    Narges Pishva

    2015-02-01

    Full Text Available Objective: Although metabolic diseases individually are rare ,but overall have an incidence of 1/2000 and can cause devastating and irreversible effect if not diagnosed early and treated promptly. selective screening is an acceptable method for detection of these multi presentation diseases. Method: using panel neonatal screening for detection of metabolic diseases in 650 high risk Iranian patients in Fars province. The following clinical features were used as inclusion criteria for investigation of the patients. Lethargy, poor feeding ,persistent vomiting, cholestasis, intractable seizure ,decreased level of consciousness ,persistent hypoglycemia, unexplained acid base disturbance and unexplained neonatal death. Result: Organic acidemia with 40 cases (42% was the most frequent disorder diagnosed in our high risk populations, followed by disorder of galactose metabolism(30%, 15 patient had classic galactosemia(GALT

  3. 儿科重症医学与遗传代谢病%Pediatric intensive care unit and inborn errors of metabolism

    Institute of Scientific and Technical Information of China (English)

    黄敬孚

    2014-01-01

    Patients with inborn errors of metabolism and metabolic crisis sometimes are admitted in PICU.The symptoms include severe acidosis,hyperammonemia,cerebral edema,coma,lethargy,convulsion,vomit,muscles weakness,jaundice,respiratory disorders and so on.We should improve the understanding of inherited metabolism diseases.This paper reviewed the manifestation,diagnosis,management and therapeutic approach of inborn errors of metabolism,especially mentioned the importance of purification in treatment of metabolic crisis.%遗传代谢病患儿经常以严重酸中毒、高氨血症、脑水肿、昏迷、嗜睡、抽搐、呕吐、肌无力、黄疸、呼吸困难等症状在PICU住院,小儿重症医学科医生应重视对遗传代谢病诊断和治疗的认识,提高对遗传代谢病的诊治水平.本文介绍了小儿重症医学科医生应基本掌握的遗传代谢病的概念、临床表现、诊断方法和基本治疗方法,并提出了对遗传代谢病重症危象患儿血液净化治疗的重要性.

  4. [Clinical, biological and genetic heterogeneity of the inborn errors of pulmonary surfactant metabolism: SP-B deficiency and alveolar proteinosis].

    Science.gov (United States)

    Tredano, M; Blic, J D; Griese, M; Fournet, J C; Elion, J; Bahuau, M

    2001-01-01

    Pulmonary surfactant is a multimolecular complex located at the air-water interface within the alveolus and to which a bulk of functions has been assigned, physical (surface-active properties) as well as immune or depurant. This complex consists of a surface active lipid layer (mainly phospholipids), and of an aqueous subphase. From discrete surfactant sub-fractions, one can isolate very hydrophobic proteins SP-B and SP-C as well as the collectins SP-A and SP-D, which were shown to have structural, metabolic, or defensive properties. Inborn or acquired abnormalities of surfactant, qualitative or quantitative in nature, account for a number human diseases. Beside hyaline membrane disease of the preterm neonate, a cluster of hereditary or acquired lung diseases have been characterized by the storage of periodic acid Schiff-positive material filling the alveoli. From this heterogeneous nosologic bulk, at least two discrete entities presently seem to emerge: 1) SP-B deficiency, in which an essentially proteinaceous material is stored within the alveoli, and which is a bona fide autosomal recessive Mendelian entity linked to the SFTPB gene (MIM 1786640), generally entailing neonatal respiratory distress with rapid fatal outcome, although partial or transient deficiencies have also been observed; 2) alveolar proteinosis, characterized by the storage of a mixed, protein and lipid material, and which constitutes a relatively heterogeneous clinical biological syndrome, with regards to age at onset (from the neonate through to adulthood) as well as the severity of associated signs. Murine models with a targeted mutation of the gene encoding GM-CSF (Csfgm) or the beta subunit of its receptor (Il3rbl) support the hypothesis of an abnormality of surfactant turnover in which the alveolar macrophage would be a key player. Beside SP-B deficiency, in which a near-consensus diagnostic chart can be designed, the ascertainment of other abnormalities of surfactant metabolism is not

  5. Pilot study of gas chromatographic-mass spectrometric screening of newborn urine for inborn errors of metabolism after treatment with urease.

    Science.gov (United States)

    Kuhara, T; Shinka, T; Inoue, Y; Ohse, M; Zhen-wei, X; Yoshida, I; Inokuchi, T; Yamaguchi, S; Takayanagi, M; Matsumoto, I

    1999-08-01

    Gas chromatographic-mass spectrometric (GC-MS) techniques for urinary organic acid profiling have been applied to high-risk screening for a wide range of diseases, mainly for inborn errors of metabolism (IEM), rather than to low-risk screening or mass screening. Using a simplified procedure with urease-pretreatment and the GC-MS technique, which allows simultaneous determination of organic acids, amino acids, sugars and sugar acids, we performed a pilot study of the application of this procedure to neonatal urine screening for 22 IEM. Out of 16,246 newborns screened, 11 cases of metabolic disorders were chemically diagnosed: two each of methylmalonic aciduria and glyceroluria, four of cystinuria, and one each of Hartnup disease, citrullinemia and alpha-aminoadipic aciduria/alpha-ketoadipic aciduria. The incidence of IEM was thus one per 1477, which was higher than the one per 3000 obtained in the USA in a study targeting amino acids and acylcarnitines in newborn blood spots by tandem mass spectrometry. Also, 227 cases were found to have transient metabolic abnormalities: 108 cases with neonatal tyrosinuria, 99 cases with neonatal galactosuria, and 20 cases with other transient metabolic disorders. Two hundred and thirty-eight cases out of 16,246 neonates (approximately 1/68) were thus diagnosed using this procedure as having either persistent or transient metabolic abnormalities. PMID:10492000

  6. Automated Screening for Three Inborn Metabolic Disorders: A Pilot Study

    Directory of Open Access Journals (Sweden)

    Kavitha S

    2006-12-01

    Full Text Available Background: Inborn metabolic disorders (IMDs form a large group of rare, but often serious, metabolic disorders. Aims: Our objective was to construct a decision tree, based on classification algorithm for the data on three metabolic disorders, enabling us to take decisions on the screening and clinical diagnosis of a patient. Settings and Design: A non-incremental concept learning classification algorithm was applied to a set of patient data and the procedure followed to obtain a decision on a patient’s disorder. Materials and Methods: Initially a training set containing 13 cases was investigated for three inborn errors of metabolism. Results: A total of thirty test cases were investigated for the three inborn errors of metabolism. The program identified 10 cases with galactosemia, another 10 cases with fructosemia and the remaining 10 with propionic acidemia. The program successfully identified all the 30 cases. Conclusions: This kind of decision support systems can help the healthcare delivery personnel immensely for early screening of IMDs.

  7. Nutritional treatment for inborn errors of metabolism: indications, regulations, and availability of medical foods and dietary supplements using phenylketonuria as an example.

    Science.gov (United States)

    Camp, Kathryn M; Lloyd-Puryear, Michele A; Huntington, Kathleen L

    2012-09-01

    Medical foods and dietary supplements are used to treat rare inborn errors of metabolism (IEM) identified through state-based universal newborn screening. These products are regulated under Food and Drug Administration (FDA) food and dietary supplement statutes. The lack of harmony in terminology used to refer to medical foods and dietary supplements and the misuse of words that imply that FDA regulates these products as drugs have led to confusion. These products are expensive and, although they are used for medical treatment of IEM, third-party payer coverage of these products is inconsistent across the United States. Clinicians and families report termination of coverage in late adolescence, failure to cover treatment during pregnancy, coverage for select conditions only, or no coverage. We describe the indications for specific nutritional treatment products for IEM and their regulation, availability, and categorization. We conclude with a discussion of the problems that have contributed to the paradox of identifying individuals with IEM through newborn screening but not guaranteeing that they receive optimal treatment. Throughout the paper, we use the nutritional treatment of phenylketonuria as an example of IEM treatment. PMID:22854513

  8. Carbohydrate Metabolism Disorders

    Science.gov (United States)

    ... in your body tissues. If you have a metabolic disorder, something goes wrong with this process. Carbohydrate metabolism disorders are a group of metabolic disorders. Normally your enzymes break carbohydrates down into glucose ( ...

  9. Los errores congénitos del metabolismo como enfermedades raras con un planteamiento global específico Inborn errors of metabolism as rare diseases with a specific global situation

    Directory of Open Access Journals (Sweden)

    P. Sanjurjo

    2008-01-01

    result in the alteration of a protein. Depending on this protein’s function - whether as an enzyme, a hormone, a receiver-transporter of a cellular membrane or forming part of a cellular organelle (lysosome, peroxysome - different groups of diseases emerge, which cause the most outstanding characteristic of inborn errors of metabolism (IEM: their clinical heterogeneity. The majority of these diseases are autosomal recessive, with a limited number of asymptomatic carriers, but there are also those ruled by an autonomous, dominant character inheritance or linked to the X chromosome. Taken individually, CMDs are highly infrequent, but taken as a whole CMDs (of which over 500 have been described to date can affect 1/500 of the newborn. A common characteristic of many CMDs is the possibility of dietary treatment and treatment with enzymatic replacement. For essentially didactic purposes the following groups should be considered: CMDs of the intermediary metabolism (whose types are intoxication and energy deficit, CMDs of cellular organelles, complex CMDs due to cycle alterations and others. A summary is presented of the clinical, diagnostic and therapeutic aspects of one disease of each type of those previously described: hyperphenylalaninemias, deficiencies of the mitochondrial oxidative phosphorilation (OXPHOS and lysosomal storage diseases.

  10. Long-chain polyunsaturated fatty acid concentration in patients with inborn errors of metabolism Concentración de ácidos grasos poliinsaturados de cadena larga en pacientes con errores innatos del metabolismo

    Directory of Open Access Journals (Sweden)

    M.ª A. Vilaseca

    2011-02-01

    Full Text Available Introduction: Long-chain polyunsaturated fatty acid (LCPUFA can be provided by diet (fatty fish, eggs, viscera and human milk or synthetised from essential fatty acids linoleic and α-linolenic acids through the microsomal pathway. However, endogenous LCPUFA synthesis is rather low, especially for docosahexaenoic (DHA, and seems insufficient to achieve normal DHA values in individuals devoid of preformed dietary supply. Inborn errors of metabolism (IEMs are therefore diseases with a special risk for LCPUFA deficient status. Aim: Our aim was to evaluate LCPUFA status in 132 patients with different IEMs. Methods: We performed a cross-sectional study of plasma and erythrocyte LCPUFA composition of 63 patients with IEMs treated with protein-restricted diets compared with data from 69 patients with IEMs on protein-unrestricted diets, and 43 own reference values. Results: Erythrocyte and plasma DHA and arachidonic acid concentrations were significantly decreased in patients treated with protein-restriction compared with those on protein-unrestricted diets and with our reference values (p Introducción: Los ácidos grasos poliinsaturados de cadena larga (LCPUFA pueden ser suministrados por la dieta o sintetizados a partir de los ácidos grasos esenciales, linoleico y α-linolénico. La síntesis endógena de LCPUFA es escasa, especialmente la de ácido docosahe-xaenoico (DHA, e insuficiente para alcanzar los valores normales de DHA en individuos que carecen de un suministro dietético de dichos ácidos preformados. Por ello, los errores innatos del metabolismo (IEM son enfermedades con riesgo especial de deficiencia de LCPUFAs. Objetivos: Evaluar el estado de LCPUFA en 132 pacientes con diferentes IEMs. Métodos: Estudio transversal de LCPUFA en plasma y eritrocitos de 63 pacientes con IEMs tratados con dieta restringida en proteínas comparados con 69 pacientes con IEMs con una dieta libre y 43 valores de referencia. Resultados: Las concentraciones de

  11. Tay-Sachs disease as a model for screening inborn errors.

    Science.gov (United States)

    Blitzer, M G; McDowell, G A

    1992-09-01

    In the absence of treatments for most inborn errors of metabolism, the goal of both geneticists and health care providers has been the prevention of disease through identification of at-risk couples. When the enzyme deficiency responsible for a disorder is known, heterozygotes can frequently be identified by enzyme assay. The presence or absence of specific mutations in the genes coding for these enzymes may be determined directly if the gene of interest has been identified and characterized. Because the inherited metabolic disorders are rare, these approaches are useful only for individuals with a family history of a specific disease or for populations in which the gene frequency for a specific disease is increased. Tay-Sachs disease is a fatal, autosomal recessive, metabolic disease caused by deficient activity of the lysosomal enzyme Hex A. Although it is rare in the general population, in which the heterozygote frequency is approximately 1/167, it is elevated in a few populations, including the Ashkenazi Jewish community, in which the heterozygote frequency is 1/30. The ability to detect TSD heterozygotes reliably and to diagnose TSD prenatally using a simple and rapid enzyme assay has made prevention of this disorder possible through education and carrier screening. The identification of specific TSD mutations at the DNA level enables laboratories to provide more accurate screening and diagnosis in some families. The success of TSD screening in the Ashkenazi Jewish population has made it the prototype for screening among the inborn errors of metabolism. The TSD example becomes increasingly relevant as heterozygote detection becomes possible for other genetic disorders that are increased in well-defined populations. Cystic fibrosis is such a disease in the caucasian population. PMID:1355703

  12. Transaldolase Deficiency: Liver Cirrhosis Associated with a New Inborn Error in the Pentose Phosphate Pathway

    Science.gov (United States)

    Verhoeven, Nanda M.; Huck, Jojanneke H. J.; Roos, Birthe; Struys, Eduard A.; Salomons, Gajja S.; Douwes, Adriaan C.; van der Knaap, Marjo S.; Jakobs, Cornelis

    2001-01-01

    This article describes the first patient with a deficiency of transaldolase (TALDO1 [E.C.2.2.1.2]). Clinically, the patient presented with liver cirrhosis and hepatosplenomegaly during early infancy. In urine and plasma, elevated concentrations of ribitol, d-arabitol, and erythritol were found. By incubating the patient's lymphoblasts and erythrocytes with ribose-5-phosphate and subsequently analyzing phosphate sugar metabolites, we discovered a deficiency of transaldolase. Sequence analysis of the transaldolase gene from this patient showed a homozygous deletion of 3 bp. This deletion results in absence of serine at position 171 of the transaldolase protein. This amino acid is invariable between species and is located in a conserved region, indicating its importance for enzyme activity. The detection of this new inborn error of pentose metabolism has implications for the diagnostic workup of liver problems of unknown etiology. PMID:11283793

  13. Ribose-5-Phosphate Isomerase Deficiency: New Inborn Error in the Pentose Phosphate Pathway Associated with a Slowly Progressive Leukoencephalopathy

    Science.gov (United States)

    Huck, Jojanneke H. J.; Verhoeven, Nanda M.; Struys, Eduard A.; Salomons, Gajja S.; Jakobs, Cornelis; van der Knaap, Marjo S.

    2004-01-01

    The present article describes the first patient with a deficiency of ribose-5-phosphate isomerase (RPI) (Enzyme Commission number 5.3.1.6) who presented with leukoencephalopathy and peripheral neuropathy. Proton magnetic resonance spectroscopy of the brain revealed highly elevated levels of the polyols ribitol and D-arabitol, which were subsequently also found in high concentrations in body fluids. Deficient activity of RPI, one of the pentose-phosphate-pathway (PPP) enzymes, was demonstrated in fibroblasts. RPI gene–sequence analysis revealed a frameshift and a missense mutation. Recently, we described a patient with liver cirrhosis and abnormal polyol levels in body fluids, related to a deficiency of transaldolase, another enzyme in the PPP. RPI is the second known inborn error in the reversible phase of the PPP, confirming that defects in pentose and polyol metabolism constitute a new area of inborn metabolic disorders. PMID:14988808

  14. Fat and Sugar Metabolism During Exercise in Patients With Metabolic Myopathy

    Science.gov (United States)

    2016-04-05

    Metabolism, Inborn Errors; Lipid Metabolism, Inborn Errors; Carbohydrate Metabolism, Inborn Errors; Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency; Glycogenin-1 Deficiency (Glycogen Storage Disease Type XV); Carnitine Palmitoyl Transferase 2 Deficiency; VLCAD Deficiency; Medium-chain Acyl-CoA Dehydrogenase Deficiency; Multiple Acyl-CoA Dehydrogenase Deficiency; Carnitine Transporter Deficiency; Neutral Lipid Storage Disease; Glycogen Storage Disease Type II; Glycogen Storage Disease Type III; Glycogen Storage Disease Type IV; Glycogen Storage Disease Type V; Muscle Phosphofructokinase Deficiency; Phosphoglucomutase 1 Deficiency; Phosphoglycerate Mutase Deficiency; Phosphoglycerate Kinase Deficiency; Phosphorylase Kinase Deficiency; Beta Enolase Deficiency; Lactate Dehydrogenase Deficiency; Glycogen Synthase Deficiency

  15. Study of clinic etiologies about newborn infants with high risk of inborn error of metabolism%高危新生儿遗传代谢病临床病因学分析

    Institute of Scientific and Technical Information of China (English)

    庄太凤; 马建荣; 温春玲; 邢继伟; 张巍; 杨艳玲

    2011-01-01

    Objective To investigate the clinic etiologies about newborn infants with high risk of inborn error of metabolism ( IEM) in NICU. Methods We did a urine organic acid analysis about 100 newhom infants with high risk of IEM by GC/MS. At the same time . blood routine , liver and renal function , blood lactic acid, blood pyruvic acid , β-hydroxyhutyric acid ,blood ammonia and serum homocysteine were determined. There were 24 patients diagnosed IEM by analysis results among the 100 cases. After 1 or 2 courses of treatment to the 24 patients , we did follow-up examination. Results There were 12 cases confirmed with IEM in the 24 patients ,including 2 patients with propionic acidemia ( PA) ,2 with tyrosinemia,2 with homocystinemia , 1 with methylmalonic aciduria ( MMA) ,1 with glutaric acidosis type Ⅱ ( GAⅡ) ,1 with congenital lactose intolerance,1 with hypermethioninemia ,1 with β-ketothiolase deficiency and 1 with ornithine carbamoyltransferase deficiency ( OCTD). Those diseases were autosomal recessive inheritance . There were different clinical features in 12 IEM cases ,including 3 patients with blood vessel pathological changes ( microthrombus engendered and encephalon parenchyma haemo -rrhage),2 with eclampsia ,2 with recurred metabolic acidosis ,1 with sudden death,1 with recurred hypoglycemia,1 with obstinated diarrhoea ,1 with jaundice correlated with inheritance and 1 with severe pneumonia. In the crises of the 12 IEM patients , 100% patients showed hyperammonemia, 83% metabolic acidosis and pyruvemia , 67% nephridium impaired , 50% with liver impaired ,42% with blood impaired. Conclusions The newbom infants with high risk of IEM had complicated etiologies . The neonates' IEM spectrum were amplification by new technique ( eg. GC/MS ). The amplification of IEM spectrum would show more etiologies of newborn and help diagnosis and treatment .%目的 初步研究新生儿重症监护室(NICU)先天性遗传代谢病(IEM)高危新

  16. Inborn errors of mucocutaneous immunity to Candida albicans in humans: a role for IL-17 cytokines?

    OpenAIRE

    Puel, Anne; Picard, Capucine; Cypowyj, Sophie; Lilic, Desa; Abel, Laurent; Casanova, Jean-Laurent

    2010-01-01

    The various clinical manifestations of chronic mucocutaneous candidiasis (CMC) often result from acquired T-cell immunodeficiencies. More rarely, CMC results from inborn errors of immunity, the recent dissection of which has shed light on the molecular mechanisms of mucocutaneous immunity to Candida albicans. CMC may accompany various other infectious diseases in patients with almost any broad and profound T-cell primary immunodeficiency. By contrast, CMC is one of the few key infections in p...

  17. Los errores congénitos del metabolismo como enfermedades raras con un planteamiento global específico Inborn errors of metabolism as rare diseases with a specific global situation

    OpenAIRE

    Sanjurjo, P. (Pablo); A. Baldellou; K. Aldámiz-Echevarría; Montejo, M; M.C. García Jiménez

    2008-01-01

    Las llamadas enfermedades congénitas del metabolismo (ECM) son consecuencia de alteraciones bioquímicas de origen génico que tienen como consecuencia la alteración de una proteína. Dependiendo de la función de esta proteína, ya sea como un enzima; como una hormona; como un receptor-transportador de membrana celular; o formando parte de una organela celular (lisosoma, peroxisoma) surgen diferentes grupos de enfermedades, lo cual origina la característica más destacada de los errores innatos de...

  18. Impact of Dietary Carbohydrate and Protein Levels on Carbohydrate Metabolism

    Science.gov (United States)

    Lasker, Denise Ann

    2009-01-01

    The goal of this dissertation was to investigate the impact of changing dietary carbohydrate (CARB) intakes within recommended dietary guidelines on metabolic outcomes specifically associated with glycemic regulations and carbohydrate metabolism. This research utilized both human and animal studies to examine changes in metabolism across a wide…

  19. Severe infectious diseases of childhood as monogenic inborn errors of immunity.

    Science.gov (United States)

    Casanova, Jean-Laurent

    2015-12-22

    This paper reviews the developments that have occurred in the field of human genetics of infectious diseases from the second half of the 20th century onward. In particular, it stresses and explains the importance of the recently described monogenic inborn errors of immunity underlying resistance or susceptibility to specific infections. The monogenic component of the genetic theory provides a plausible explanation for the occurrence of severe infectious diseases during primary infection. Over the last 20 y, increasing numbers of life-threatening infectious diseases striking otherwise healthy children, adolescents, and even young adults have been attributed to single-gene inborn errors of immunity. These studies were inspired by seminal but neglected findings in plant and animal infections. Infectious diseases typically manifest as sporadic traits because human genotypes often display incomplete penetrance (most genetically predisposed individuals remain healthy) and variable expressivity (different infections can be allelic at the same locus). Infectious diseases of childhood, once thought to be archetypal environmental diseases, actually may be among the most genetically determined conditions of mankind. This nascent and testable notion has interesting medical and biological implications. PMID:26621750

  20. Carbohydrate metabolism in catfish

    International Nuclear Information System (INIS)

    Radiolabeled (U- 14C)-glucose was incorporated in diets and forced-fed to channel catfish and was observed for a 24 hour period. About 95% of fed labeled (U-14C)-glucose was absorbed by catfish, showing a high digestibility of glucose. The amounts of 14C excreted over 24 h as carbon dioxide were 49% and amounts excreted in urine were 3.5%. The amount retained as protein, fat glycogen and other organic compounds were 8.2, 1.2, 6.5 and 32.1 % respectively, for the 24 hour period. The blood concentration of 14 C reached a maximum 2.5 hour after feeding (U-14C)-glucose, then gradually decreased. Based on tissue concentrations of 14C, glycogen was an immediate storage site for absorbed glucose, but 14C- glycogen in liver decreased rapidly. Glucose was quickly and heavily converted into triglyceride, indicating that fat is an important intermediate in the metabolism of glucose in channel catfish. 14C-fat in the serum and liver were transferred to the adipose tissue in the muscle and mesentery about 10 hours after feeding. (Author)

  1. Carbohydrate metabolism in Spirochaeta stenostrepta.

    Science.gov (United States)

    Hespell, R B; Canale-Parola, E

    1970-07-01

    The pathways of carbohydrate metabolism in Spirochaeta stenostrepta, a free-living, strictly anaerobic spirochete, were studied. The organism fermented glucose to ethyl alcohol, acetate, lactate, CO(2), and H(2). Assays of enzymatic activities in cell extracts, and determinations of radioactivity distribution in products formed from (14)C-labeled glucose indicated that S. stenostrepta degraded glucose via the Embden-Meyerhof pathway. The spirochete utilized a clostridial-type clastic reaction to metabolize pyruvate to acetyl-coenzyme A, CO(2), and H(2), without production of formate. Acetyl-coenzyme A was converted to ethyl alcohol by nicotinamide adenine dinucleotide-dependent acetaldehyde and alcohol dehydrogenase activities. Phosphotransacetylase and acetate kinase catalyzed the formation of acetate from acetyl-coenzyme A. Hydrogenase and lactate dehydrogenase activities were detected in cell extracts. A rubredoxin was isolated from cell extracts of S. stenostrepta. Preparations of this rubredoxin stimulated acetyl phosphate formation from pyruvate by diethylaminoethyl cellulose-treated extracts of S. stenostrepta, an indication that rubredoxin may participate in pyruvate cleavage by this spirochete. Nutritional studies showed that S. stenostrepta fermented a variety of carbohydrates, but did not ferment amino acids or other organic acids. An unidentified growth factor present in yeast extract was required by the organism. Exogenous supplements of biotin, riboflavin, and vitamin B(12) were either stimulatory or required for growth. PMID:5423371

  2. Inborn errors of human STAT1: allelic heterogeneity governs the diversity of immunological and infectious phenotypes.

    Science.gov (United States)

    Boisson-Dupuis, Stephanie; Kong, Xiao-Fei; Okada, Satoshi; Cypowyj, Sophie; Puel, Anne; Abel, Laurent; Casanova, Jean-Laurent

    2012-08-01

    The genetic dissection of various human infectious diseases has led to the definition of inborn errors of human STAT1 immunity of four types, including (i) autosomal recessive (AR) complete STAT1 deficiency, (ii) AR partial STAT1 deficiency, (iii) autosomal dominant (AD) STAT1 deficiency, and (iv) AD gain of STAT1 activity. The two types of AR STAT1 defect give rise to a broad infectious phenotype with susceptibility to intramacrophagic bacteria (mostly mycobacteria) and viruses (herpes viruses at least), due principally to the impairment of IFN-γ-mediated and IFN-α/β-mediated immunity, respectively. Clinical outcome depends on the extent to which the STAT1 defect decreases responsiveness to these cytokines. AD STAT1 deficiency selectively predisposes individuals to mycobacterial disease, owing to the impairment of IFN-γ-mediated immunity, as IFN-α/β-mediated immunity is maintained. Finally, AD gain of STAT1 activity is associated with autoimmunity, probably owing to an enhancement of IFN-α/β-mediated immunity. More surprisingly, it is also associated with chronic mucocutaneous candidiasis, through as yet undetermined mechanisms involving an inhibition of the development of IL-17-producing T cells. Thus, germline mutations in human STAT1 define four distinct clinical disorders. Various combinations of viral, mycobacterial and fungal infections are therefore allelic at the human STAT1 locus. These experiments of Nature neatly highlight the clinical and immunological impact of the human genetic dissection of infectious phenotypes. PMID:22651901

  3. Hematopoietic Stem Cell Transplantation (HCT) for Inborn Errors of Metabolism

    Science.gov (United States)

    2012-11-06

    Hurler's Syndrome; Maroteaux-Lamy Syndrome; Sly Syndrome; Alpha Mannosidosis; Fucosidosis; Aspartylglucosaminuria; Sphingolipidoses; Krabbe Disease; Wolman's Disease; Niemann-Pick Disease Type B; Niemann-Pick Disease, Type C

  4. Stem Cell Transplant for Inborn Errors of Metabolism

    Science.gov (United States)

    2012-11-06

    Adrenoleukodystrophy; Metachromatic Leukodystrophy; Globoid Cell Leukodystrophy; Gaucher's Disease; Fucosidosis; Wolman Disease; Niemann-Pick Disease; Batten Disease; GM1 Gangliosidosis; Tay Sachs Disease; Sandhoff Disease

  5. Inborn errors of cobalamin absorption, transport and metabolism

    OpenAIRE

    Sass, Jörn Oliver; Baumgartner, M.

    2013-01-01

    Cobalamin (Cbl, vitamin B12) is a cobalt-containing vitamin which is synthesized by bacteria and archaea. It can be taken up from food of animal origin, but not from higher plants. Various cobalamins differ in the residue R in the upper axial position of the molecule. In adenosylcobalamin (AdoCbl) R is a 5’-deoxyadenosyl moiety, in methylcobalamin (MeCbl) a methyl group. Common vitamin B12 supplements contain hydroxocobalamin (OHCbl, labelled “the natural form of the vitamin”, with R = OH) or...

  6. Role of carbohydrate metabolism in grass tetany

    Energy Technology Data Exchange (ETDEWEB)

    Miller, J.K.; Madsen, F.C.; Lentz, D.E.; Hansard, S.L.

    1977-01-01

    Clinical hypomagnesemia is confined primarily to beef cattle in the United States but also occurs in dairy cattle in other countries, probably due to different management practices. During periods when grass tetany is likely, early vegetative temperate zone grasses are usually low in total readily available carbohydrates and magnesium but high in potassium and nitrogen. The tetany syndrome may include hypoglycemia and ketosis, suggesting an imbalance in intermediary energy metabolism. Many enzyme systems critical to cellular metabolism, including those which hydrolyze and transfer phosphate groups, are activated by Mg. Thus, by inference, Mg is required for normal glucose utilization, fat, protein, nucleic acid and coenzyme synthesis, muscle contraction, methyl group transfer, and sulfate, acetate, and formate activation. Numerous clinical and experimental studies suggest an intimate relationship between metabolism of Mg and that of carbohydrate, glucagon, and insulin. The objective is to review this literature and suggest ways in which these relationships might contribute to a chain of events leading to grass tetany.

  7. Metabolic aspects of low carbohydrate diets and exercise

    Directory of Open Access Journals (Sweden)

    Peters Sandra

    2004-01-01

    Full Text Available Abstract Following a low carbohydrate diet, there is a shift towards more fat and less carbohydrate oxidation to provide energy to skeletal muscle, both at rest and during exercise. This review summarizes recent work on human skeletal muscle carbohydrate and fat metabolic adaptations to a low carbohydrate diet, focusing mainly on pyruvate dehydrogenase and pyruvate dehydrogenase kinase, and how these changes relate to the capacity for carbohydrate oxidation during exercise.

  8. Cause of impaired carbohydrate metabolism in hyperthyroidism

    International Nuclear Information System (INIS)

    Hyperthyroidism (HT) affects glucose metabolism in various ways. The role of insulin, glucagon and growth-hormone (GH) was determined. After glucose loading the insulin response is weaker in HT than in euthyroid subjects. Enhanced degradation of insulin has been reported. It is suggested that in HT the serum insulin concentration declines at a slightly accelerated rate. In HT the deranged carbohydrate metabolism might be a consequence of altered tissue sensitivity to insulin. To elucidate this problem insulin receptors on erythrocytes obtained from hyperthyroid women were investigated. The maximal specific binding of 125I-insulin to RBC of hyperthyroid patients was decreased and the analysis refers to a decreased receptor concentration in RBC. The nature of glucagon secretion and its influence on glucose metabolism in HT was investigated. The basal plasma glucagon is elevated in hyperthyroid patients. The suppression of glucagon secretion induced by an oral glucose loading was of significantly lesser degree in hyperthyroid patients than in controls. Applying the erythrocyte receptor assay a decreased specific binding of 125I-glucagon to RBC of hyperthyroid patients has been found and data indicate a significantly less glucagon receptor concentration in thyrotoxicosis. Physiological elevations of serum GH levels led to a significant impairment of glucose metabolism. Beside the GH-RH and somatostatin, the dopaminergic neurotransmitter system participates in the regulation of GH secretion too. It has been demonstrated that after administration of the dopamine agonist l-dopa the GH response was weaker in HT than in controls. This indicates that in thyrotoxicosis the GH secretion can not be stimulated in such a degree as in euthyroidism. (author)

  9. In vivo 13C MRS studies of carbohydrate metabolism

    International Nuclear Information System (INIS)

    The work described in this thesis was performed by the author, except where indicated, within the Magnetic Resonance Centre at the University of Nottingham during the period between October 1999 and October 2002. Although much is known about the major pathways of carbohydrate metabolism, there is still much to be learnt about the exact mechanisms of many of these pathways. Of particular interest is how these pathways are modified under different physiological conditions and in diseased states. 13C NMR spectroscopy provides a non-invasive means for studying carbohydrate metabolism in vivo, and the work presented within this thesis gives two such examples of this in human subjects. Natural abundance 13C NMR spectroscopy was used to measure glycogen levels in gastrocnemius muscle. The diurnal changes in response to mixed meals were measured in both type 2 diabetic subjects and age and weight matched controls. Metabolic studies were performed to complement the NMR measurements. The data obtained in these studies show the effect of the failure of muscle glucose storage upon post-prandial hyperglycaemia despite a supra-normal increase in plasma insulin in type 2 diabetes. 13C NMR spectroscopy was also used to study cerebral metabolism. Accumulation of 13C label into glutamate and glutamine following infusion of [113C] glucose allows the determination of the rates of the TCA cycle (FTCA) and neurotransmitter cycling (Fcyc). These rates were measured in the visual cortex under control and activated conditions. The increases seen in FTCA upon activation, together with the lack of label accumulation in lactate, suggest that cerebral glucose metabolism is oxidative, even during strong activation. No conclusion can be made as to whether or not a similar increase is seen in Fcyc due to the large associated errors in these values. (author)

  10. CARBOHYDRATE INGESTION AND EXERCISE: EFFECTS ON METABOLISM AND PERFORMANCE

    Institute of Scientific and Technical Information of China (English)

    2000-01-01

    @@KEY POINTS ■ Carbohydrate is the preferred fuel for most competitive sports;an inadequate supply of carbohydrate in the body often leads to poor performance. ■ Carbohydrate ingestion during exercise increases blood glucose availability and maintains the ability of the body to use carbohydrate as fuel during exercise.When carbohydrate is consumed during exercise,glucose uptake by muscles is increased,and the breakdown of glycogen in the liver into blood glucose is reduced,thus saving liver glycogen until late in exercise.The use of muscle glycogen for energy is generally unaffected by carbohydrate feeding.However,during prolonged running,the breakdown of muscle glycogen may be slowed because the supply of blood glucose is improved when carbohydrate is consumed.These metabolic responses underlie the performance benefit that accompanies carbohydrate ingestion during exercise. ■ There are some minor differences among glucose,sucrose,and maltodextrins in their effects on metabolism,but each of them can enhance performance when ingested in the appropriate quantity during exercise.Fructose alone is not an effective carbohydrate supplement because of its slow absorption and slow conversion by the body to glucose,but when small amounts of fructose are combined with other carbohydrates,fructose can be beneficial. ■ Ingesting carbohydrate at a rate of 30-60 grams per hour can improve exercise erformance.A good way to achieve this carbohydrate intake is to consume 600-to-1200 ml(20-to-40 oz)of a sports drink during each hour of exercise.Consuming carbohydrate in a beverage provides an added benefit of preventing potentially harmful effects of dehydration on performance.

  11. Postexercise recovery period: carbohydrate and protein metabolism.

    Science.gov (United States)

    Viru, A

    1996-02-01

    The essence of the postexercise recovery period is normalization of function and homeostatic equilibrium, and replenishment of energy resources and accomplishment of the reconstructive function. The repletion of energy stores is actualized in a certain sequence and followed by a transitory supercompensation. The main substrate for repletion of the muscle glycogen store is blood glucose derived from hepatic glucose output as well as from consumption of carbohydrates during the postexercise period. The repletion of liver glycogen is realized less rapidly. It depends to a certain extent on hepatic gluconeogenesis but mainly on supply with exogenous carbohydrates. The constructive function is founded on elevated protein turnover and adaptive protein synthesis. Whereas during and shortly after endurance exercise intensive protein breakdown was found in less active fast-twitch glycolytic fibers, during the later course of the recovery period the protein degradation rate increased together with intensification of protein synthesis rate in more active fast-twitch glycolytic oxidative and slow-twitch oxidative fibers. PMID:8680938

  12. The Effects of Breakfast Consumption and Composition on Metabolic Wellness with a Focus on Carbohydrate Metabolism.

    Science.gov (United States)

    Maki, Kevin C; Phillips-Eakley, Alyssa K; Smith, Kristen N

    2016-05-01

    Findings from epidemiologic studies indicate that there are associations between breakfast consumption and a lower risk of type 2 diabetes mellitus (T2DM) and metabolic syndrome, prompting interest in the influence of breakfast on carbohydrate metabolism and indicators of T2DM risk. The objective of this review was to summarize the available evidence from randomized controlled trials assessing the impact of breakfast on variables related to carbohydrate metabolism and metabolic wellness. Consuming compared with skipping breakfast appeared to improve glucose and insulin responses throughout the day. Breakfast composition may also be important. Dietary patterns high in rapidly available carbohydrate were associated with elevated T2DM risk. Therefore, partial replacement of rapidly available carbohydrate with other dietary components, such as whole grains and cereal fibers, proteins, and unsaturated fatty acids (UFAs), at breakfast may be a useful strategy for producing favorable metabolic outcomes. Consumption of fermentable and viscous dietary fibers at breakfast lowers glycemia and insulinemia. Fermentable fibers likely act through enhancing insulin sensitivity later in the day, and viscous fibers have an acute effect to slow the rate of carbohydrate absorption. Partially substituting protein for rapidly available carbohydrate enhances satiety and diet-induced thermogenesis, and also favorably affects lipoprotein lipids and blood pressure. Partially substituting UFA for carbohydrate has been associated with improved insulin sensitivity, lipoprotein lipids, and blood pressure. Overall, the available evidence suggests that consuming breakfast foods high in whole grains and cereal fiber, while limiting rapidly available carbohydrate, is a promising strategy for metabolic health promotion. PMID:27184288

  13. Normal Roles for Dietary Fructose in Carbohydrate Metabolism

    Directory of Open Access Journals (Sweden)

    Maren R. Laughlin

    2014-08-01

    Full Text Available Although there are many well-documented metabolic effects linked to the fructose component of a very high sugar diet, a healthy diet is also likely to contain appreciable fructose, even if confined to that found in fruits and vegetables. These normal levels of fructose are metabolized in specialized pathways that synergize with glucose at several metabolic steps. Glucose potentiates fructose absorption from the gut, while fructose catalyzes glucose uptake and storage in the liver. Fructose accelerates carbohydrate oxidation after a meal. In addition, emerging evidence suggests that fructose may also play a role in the secretion of insulin and GLP-1, and in the maturation of preadipocytes to increase fat storage capacity. Therefore, fructose undergoing its normal metabolism has the interesting property of potentiating the disposal of a dietary carbohydrate load through several routes.

  14. Protein,carbohydrate and lipid metabolism

    Institute of Scientific and Technical Information of China (English)

    1995-01-01

    950255 Effects of TPN and indomethacin on stressresponse and protein metabolism after surgery.QUANZhufu(全竹富),et al.General Hosp,Nanjing Com-mand,Nanjing,210002.Med J Chin PLA 1995;20(1):24-26.The study was planned to evaluate effects of TPNand indomethacin on stress response after trauma,andprotein metabolism in patients who had received totalgastrectomy for cardiac cancer of stomach.19 caseswere divided into control,TPN,and indomethacin

  15. Dysfunction in macrophage toll-like receptor signaling caused by an inborn error of cationic amino acid transport.

    Science.gov (United States)

    Kurko, Johanna; Vähä-Mäkilä, Mari; Tringham, Maaria; Tanner, Laura; Paavanen-Huhtala, Sari; Saarinen, Maiju; Näntö-Salonen, Kirsti; Simell, Olli; Niinikoski, Harri; Mykkänen, Juha

    2015-10-01

    Amino acids, especially arginine, are vital for the well-being and activity of immune cells, and disruption of amino acid balance may weaken immunity and predispose to infectious and autoimmune diseases. We present here a model of an inborn aminoaciduria, lysinuric protein intolerance (LPI), in which a single mutation in y(+)LAT1 cationic amino acid transporter gene SLC7A7 leads to a multisystem disease characterized by immunological complications, life-threatening pulmonary alveolar proteinosis and nephropathy. Macrophages are suggested to play a central role in LPI in the development of these severe secondary symptoms. We thus studied the effect of the Finnish y(+)LAT1 mutation on monocyte-derived macrophages where toll-like receptors (TLRs) act as the key molecules in innate immune response against external pathogens. The function of LPI patient and control macrophage TLR signaling was examined by stimulating the TLR2/1, TLR4 and TLR9 pathways with their associated pathogen-associated molecular patterns. Downregulation in expression of TLR9, IRF7, IRF3 and IFNB1 and in secretion of IFN-α was detected, suggesting an impaired response to TLR9 stimulation. In addition, secretion of TNF-α, IL-12 and IL-1RA by TLR2/1 stimulation and IL-12 and IL-1RA by TLR4 stimulation was increased in the LPI patients. LPI macrophages secreted significantly less nitric oxide than control macrophages, whereas plasma concentrations of inflammatory chemokines CXCL8, CXCL9 and CXCL10 were elevated in the LPI patients. In conclusion, our results strengthen the relevance of macrophages in the pathogenesis of LPI and, furthermore, suggest that cationic amino acid transport plays an important role in the regulation of innate immune responses. PMID:26210182

  16. Impeded Carbohydrate Metabolism in Rice Plants under Submergence Stress

    Institute of Scientific and Technical Information of China (English)

    Malay Kumar ADAK; Nirmalya GHOSH; Dilip Kumar DASGUPTA; Sudha GUPTA

    2011-01-01

    The detrimental effects of submergence on physiological performances of some rice varieties with special references to carbohydrate metabolisms and their allied enzymes during post-flowering stages have been documented and clarified in the present investigation.It was found that photosynthetic rate and concomitant translocation of sugars into the panicles were both related to the yield.The detrimental effects of the complete submergence were recorded in generation of sucrose,starch,sucrose phosphate synthase and phosphorylase activity in the developing panicles of the plants as compared to those under normal or control (i.e.non-submerged) condition.The accumulation of starch was significantly lower in plants under submergence and that was correlated with ADP-glucose pyrophosphorylase activity.Photosynthetic rate was most affected under submergence in varying days of post-flowering and was also related to the down regulation of Ribulose bisphosphate carboxylase activity.However,under normal or control condition,there recorded a steady maintenance of photosynthetic rate at the post-flowering stages and significantly higher values of Ribulose bisphosphate carboxylase activity.Still,photosynthetic rate of the plants under both control and submerged conditions had hardly any significant correlation with sugar accumulation and other enzymes of carbohydrate metabolism like invertase with grain yield.Finally,plants under submergence suffered significant loss of yield by poor grain filling which was related to impeded carbohydrate metabolism in the tissues.It is evident that loss of yield under submergence is attributed both by lower sink size or sink capacity (number of panicles,in this case) as well as subdued carbohydrate metabolism in plants and its subsequent partitioning into the grains.

  17. INFLUENCE OF CHITOSAN ON CARBOHYDRATE METABOLISM IN EXERCISING MICE

    Institute of Scientific and Technical Information of China (English)

    石磊; 黄伟

    2004-01-01

    Objective To study the mechanism of chitosan on carbohydrate metabolism disorder in exercising mice. Methods The animal model of carbohydrate metabolism disorder was established through swimming trainings and the content of blood glucose, muscle glycogen and liver glycogen in mice were all surveyed. Results When quiet, liver glycogen, muscle glycogen and blood glucose of drug-taking group were much higher than those of control group(P<0.05). Compared with control group, the liver glycogen and muscle glycogen of instant drug-taking group after exercises level to a higher degree (P<0.05). The renewing level of liver glycogen, muscle glycogen and blood glucose in drug-taking group after spending 24 hours on recovery was evidently higher than that of control-group (P<0.05). The exhaustive swimming time of drug-taking group was longer than that of exercise-control group by 33.99%. Conclusion Chitosan takes good effect on improving carbohydrate metabolism disorder resulting from exercises.

  18. Selective screening in neonates suspected to have inborn errors of metabolism

    Directory of Open Access Journals (Sweden)

    Rabah M. Shawky

    2015-04-01

    Conclusion: IEM represent a high percent (32.5% of neonates who had sepsis like symptoms, and when diagnosed, patients showed marked improvement after therapy. IEM should be considered in differential diagnosis of the sick neonates, and investigations, and management should be started rapidly to decrease morbidity, and mortality till nationwide screen for IEM is applied in Egypt.

  19. Clinical biological and genetic heterogeneity of the inborn errors of pulmonary surfactant metabolism

    OpenAIRE

    Tredano, Mohammed; de Blic, Jacques; Griese, Matthias; Fournet, Jean-Christophe; Elion, Jacques; Bahuau, Michael

    2001-01-01

    Pulmonary surfactant is a multimolecular complex located at the air-water interface within the alveolus to which a range of physical (surface-active properties) and immune functions has been assigned. This complex consists of a surface-active lipid layer (consisting mainly of phospholipids), and of an aqueous subphase. From discrete surfactant sub-fractions one can isolate strongly hydrophobic surf acta nt proteins B (SP-B) and C (SP-C) as well as collectins SP-A and SP-D, which were shown to...

  20. Carbohydrate metabolism of cactus in a desert environment.

    Science.gov (United States)

    Sutton, B G

    1981-09-01

    The concentration of glucan, mucilage, soluble carbohydrates, and malic acid were determined in Opuntia bigelovii Engelm. during a 23-week period. The experiment began during the dry summer by irrigation to stimulate Crassulacean acid metabolism and was followed by 13 weeks of drought. After the 13-week drought period, the plants were irrigated throughout a 10-week period until late December. The maximum level of malic acid determined each day at dawn decreased throughout the drought period and increased after irrigation. High levels of malic acid occurring at dawn are indicative of active Crassulacean acid metabolism. Soluble carbohydrates also decreased during drought and increased after irrigation. Both glucan and mucilage increased slightly for about 9 weeks during the drought period and then began to decrease. Irrigation was accompanied by a further decrease in concentration of glucan and mucilage. Since both glucan and mucilage changed in a similar manner and since their concentrations in the tissue are correlated, it is hypothesized that both function as storage carbohydrates. Whereas glucan is the nocturnal substrate for malic acid synthesis, there are no data to support or refute a similar hypothesis for mucilage. PMID:16661999

  1. Regulation of hepatic carbohydrate metabolism by Selenium during diabetes.

    Science.gov (United States)

    Chen, Hongping; Qiu, Qinqin; Zou, Caiyan; Dou, Lianjun; Liang, Jun

    2015-05-01

    In the present study, we have tried to unravel the role of Selenium supplementation in containing hyperglycemia by regulating enzymes activities involved in carbohydrate metabolism in liver of diabetic animals. Male wistar rats were divided into four groups: normal control, diabetic, Selenium treated control and Selenium treated diabetic group. Diabetes was induced in the animals by injecting alloxan intraperitoneally at a dose level of 150 mg/kg body weight. Selenium in the form of sodium selenite was supplemented to rats at a dose level of 1 PPM in drinking water, ad libitum for two time durations of 2 and 4 weeks. Animals were sacrificed and livers were excised for the analyses of enzymes involved in carbohydrate metabolism as well as the levels of glycogen. In-vitro (14)C-d glucose uptake and its turnover were also assessed in liver slices of all the treatment groups using radiorespirometry. Selenium supplementation to the diabetic rats normalized the enzyme activities of glucose-6-phosphatase, lactate dehydrogenase and glycogen phosphorylase as well as restored the glycogen levels to within the normal limits which were altered during diabetes. Interestingly, when Selenium was supplemented to diabetic rats, (14)C-d glucose uptake and its turnover showed a statistically significant increase in their values which however, were decreased in diabetic rats. In conclusion, Selenium mediates insulin-like role during diabetes by tending to normalize the altered activities of glucose metabolizing enzymes and also improves the glucose uptake and its metabolism by the liver. PMID:25779343

  2. Carbohydrates

    Science.gov (United States)

    ... girls Eating healthy at restaurants Special food issues Vegetarian eating Eating for strong bones Quiz: Food Facts Links to more information girlshealth glossary girlshealth.gov home http://www.girlshealth.gov/ Home Nutrition Nutrition basics Carbohydrates Carbohydrates Carbohydrates (say: kar-boh- ...

  3. Does caffeine alter muscle carbohydrate and fat metabolism during exercise?

    DEFF Research Database (Denmark)

    Graham, Terry E; Battram, Danielle S; Dela, Flemming;

    2008-01-01

    Caffeine, an adenosine receptor antagonist, has been studied for decades as a putative ergogenic aid. In the past 2 decades, the information has overwhelmingly demonstrated that it indeed is a powerful ergogenic aid, and frequently theories have been proposed that this is due to alterations in fat...... and carbohydrate metabolism. While caffeine certainly mobilizes fatty acids from adipose tissue, rarely have measures of the respiratory exchange ratio indicated an increase in fat oxidation. However, this is a difficult measure to perform accurately during exercise, and small changes could be.......05) glycogen and increased (p < 0.05) citrate, acetyl-CoA, and glucose-6-phosphate. The only effects of caffeine were to increase (p < 0.05) citrate in resting muscle and cAMP in exercise. There is very little evidence to support the hypothesis that caffeine has ergogenic effects as a result of enhanced fat...

  4. Metabolic Effects of the Very-Low-Carbohydrate Diets: Misunderstood "Villains" of Human Metabolism

    Directory of Open Access Journals (Sweden)

    Manninen Anssi H

    2004-12-01

    Full Text Available Abstract During very low carbohydrate intake, the regulated and controlled production of ketone bodies causes a harmless physiological state known as dietary ketosis. Ketone bodies flow from the liver to extra-hepatic tissues (e.g., brain for use as a fuel; this spares glucose metabolism via a mechanism similar to the sparing of glucose by oxidation of fatty acids as an alternative fuel. In comparison with glucose, the ketone bodies are actually a very good respiratory fuel. Indeed, there is no clear requirement for dietary carbohydrates for human adults. Interestingly, the effects of ketone body metabolism suggest that mild ketosis may offer therapeutic potential in a variety of different common and rare disease states. Also, the recent landmark study showed that a very-low-carbohydrate diet resulted in a significant reduction in fat mass and a concomitant increase in lean body mass in normal-weight men. Contrary to popular belief, insulin is not needed for glucose uptake and utilization in man. Finally, both muscle fat and carbohydrate burn in an amino acid flame.

  5. Endocrine regulation of carbohydrate metabolism in hypometabolic animals

    Science.gov (United States)

    Musacchia, X. J.

    1988-01-01

    Experimental hypothermia and natural hibernation are two forms of hypometabolism with recognized physiological changes, including depression of endocrine and metabolic functions. To better understand functional changes, helox (i.e., helium and oxygen (80:20) mixtures) and low ambient temperatures have been used to induce hypothermia in hamsters and rats. Both clinical and biological survival, i.e., survival without recovery and survival with recovery from hypothermia, respectively, are related to depth and length of hypothermia. In the rat, body temperatures of 15 degrees C for periods greater than 6-10 h greatly restrict biological survival. The role of glucocorticoids in enhancing thermogenic capacity of rats was assessed using triamcinolone [correction of triamcinalone] acetonide. In the hamster, treatment with cortisone acetate prolonged both clinical and biological survival. Hypothermic hamsters continue utilizing circulating glucose until they become hypoglycemic and die. Hypothermic rats do not utilize glucose and respond with a significant hypoinsulinema. The role of endocrines in the regulation of carbohydrate homeostasis and metabolism differs in hibernation and hypothermia. Glucocorticoids influence the hypothermic response in both species, specifically by prolonging induction of hypothermia in rats and by prolonging survival in hypothermic hamsters.

  6. Carbohydrates

    Science.gov (United States)

    Abumrad NA, Nassi F, Marcus A. Digestion and absorption of dietary fat, carbohydrate, and protein. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger & Fordtran's Gastrointestinal and Liver Disease. 10th ed. ...

  7. REPEATED ACUTE STRESS INDUCED ALTERATIONS IN CARBOHYDRATE METABOLISM IN RAT

    Directory of Open Access Journals (Sweden)

    Nirupama R.

    2010-09-01

    Full Text Available Acute stress induced alterations in the activity levels of rate limiting enzymes and concentration of intermediates of different pathways of carbohydrate metabolism have been studied. Adult male Wistar rats were restrained (RS for 1 h and after an interval of 4 h they were subjected to forced swimming (FS exercise and appropriate controls were maintained. Five rats were killed before the commencement of the experiment (initial controls, 5 control and equal number of stressed rats were killed 2 h after RS and remaining 5 rats in each group were killed 4 h after FS. There was a significant increase in the adrenal 3β- hydroxy steroid dehydrogenase activity following RS, which showed further increase after FS compared to controls and thereby indicated stress response of rats. There was a significant increase in the blood glucose levels following RS which showed further increase and reached hyperglycemic condition after FS. The hyperglycemic condition due to stress was accompanied by significant increases in the activities of glutamate- pyruvate transaminase, glutamate- oxaloacetate transaminase, glucose -6- phosphatase and lactate dehydrogenase and significant decrease in the glucose -6- phosphate dehydrogenase and pyruvate dehydrogenase activities, whereas pyruvate kinase activity did not show any alteration compared to controls. Further, the glycogen and total protein contents of the liver were decreased whereas those of pyruvate and lactate showed significant increase compared to controls after RS as well as FS.The results put together indicate that acute stress induced hyperglycemia results due to increased gluconeogenesis and glycogenolysis without alteration in glycolysis. The study first time reveals that after first acute stress exposure, the subsequent stressful experience augments metabolic stress response leading to hyperglycemia. The results have relevance to human health as human beings are exposed to several stressors in a day and

  8. Carbohydrate restriction improves the features of Metabolic Syndrome. Metabolic Syndrome may be defined by the response to carbohydrate restriction

    Directory of Open Access Journals (Sweden)

    Feinman Richard D

    2005-11-01

    Full Text Available Abstract Metabolic Syndrome (MetS represents a constellation of markers that indicates a predisposition to diabetes, cardiovascular disease and other pathologic states. The definition and treatment are a matter of current debate and there is not general agreement on a precise definition or, to some extent, whether the designation provides more information than the individual components. We consider here five indicators that are central to most definitions and we provide evidence from the literature that these are precisely the symptoms that respond to reduction in dietary carbohydrate (CHO. Carbohydrate restriction is one of several strategies for reducing body mass but even in the absence of weight loss or in comparison with low fat alternatives, CHO restriction is effective at ameliorating high fasting glucose and insulin, high plasma triglycerides (TAG, low HDL and high blood pressure. In addition, low fat, high CHO diets have long been known to raise TAG, lower HDL and, in the absence of weight loss, may worsen glycemic control. Thus, whereas there are numerous strategies for weight loss, a patient with high BMI and high TAG is likely to benefit most from a regimen that reduces CHO intake. Reviewing the literature, benefits of CHO restriction are seen in normal or overweight individuals, in normal patients who meet the criteria for MetS or in patients with frank diabetes. Moreover, in low fat studies that ameliorate LDL and total cholesterol, controls may do better on the symptoms of MetS. On this basis, we feel that MetS is a meaningful, useful phenomenon and may, in fact, be operationally defined as the set of markers that responds to CHO restriction. Insofar as this is an accurate characterization it is likely the result of the effect of dietary CHO on insulin metabolism. Glucose is the major insulin secretagogue and insulin resistance has been tied to the hyperinsulinemic state or the effect of such a state on lipid metabolism. The

  9. Carbohydrate metabolism after one year of using a gestodene containing monophasic oral contraceptive

    Directory of Open Access Journals (Sweden)

    Ayşegül Yıldırım

    2006-11-01

    Full Text Available Aim: To prospectively evaluate the effects of an oral contraceptive containing the progestin gestodene on carbohydrate metabolism in ordinary Turkish women Material / Method: Carbohydrate metabolism was prospectively evaluated in 53 normal women prior to and during their use of monophasic oral contraceptive containing the progestin gestodene plus ethinyl estradiol for one year. The women had a two hour oral glucose tolerance test using 75 gram glucose load, measuring serum glucose and insulin level, performed at the beginning of the contraceptive therapy and after one year. Results: The results demonstrate no significant changes in either of carbohydrate metabolic indices between the two tests.  Conclusion: The progestin containing contraceptive pill can be safely used in consideration of the carbohydrate metabolism.

  10. Carbon catabolite repression and global control of the carbohydrate metabolism in Lactococcus lactis.

    OpenAIRE

    Luesink, E.J.

    1998-01-01

    In view of the economic importance of fermented dairy products considerable scientific attention has been given to various steps of fermentation processes, including the L-lactate formation of lactic acid bacteria (de Vos, 1996). In particular, the carbohydrate metabolism of L. lactis has been the subject of extensive research and several genes encoding proteins involved in the central carbohydrate metabolism have been described (Llanos et al., 1992; Llanos et al., 1993; Cancilla et al., 1995...

  11. Carbohydrates

    Science.gov (United States)

    ... is fine because they contain important vitamins and minerals. But your body rapidly digests the starch in white potatoes. This can raise your blood glucose level. Healthy carbohydrates include: Natural sugars in fruits, vegetables, milk, and milk products Dietary fiber Starches in whole- ...

  12. Normal Roles for Dietary Fructose in Carbohydrate Metabolism

    OpenAIRE

    Laughlin, Maren R.

    2014-01-01

    Although there are many well-documented metabolic effects linked to the fructose component of a very high sugar diet, a healthy diet is also likely to contain appreciable fructose, even if confined to that found in fruits and vegetables. These normal levels of fructose are metabolized in specialized pathways that synergize with glucose at several metabolic steps. Glucose potentiates fructose absorption from the gut, while fructose catalyzes glucose uptake and storage in the liver. Fructose ac...

  13. A holistic view of dietary carbohydrate utilization in lobster: digestion, postprandial nutrient flux, and metabolism.

    Science.gov (United States)

    Rodríguez-Viera, Leandro; Perera, Erick; Casuso, Antonio; Perdomo-Morales, Rolando; Gutierrez, Odilia; Scull, Idania; Carrillo, Olimpia; Martos-Sitcha, Juan A; García-Galano, Tsai; Mancera, Juan Miguel

    2014-01-01

    Crustaceans exhibit a remarkable variation in their feeding habits and food type, but most knowledge on carbohydrate digestion and utilization in this group has come from research on few species. The aim of this study was to make an integrative analysis of dietary carbohydrate utilization in the spiny lobster Panulirus argus. We used complementary methodologies such as different assessments of digestibility, activity measurements of digestive and metabolic enzymes, and post-feeding flux of nutrients and metabolites. Several carbohydrates were well digested by the lobster, but maize starch was less digestible than all other starches studied, and its inclusion in diet affected protein digestibility. Most intense hydrolysis of carbohydrates in the gastric chamber of lobster occurred between 2-6 h after ingestion and afterwards free glucose increased in hemolymph. The inclusion of wheat in diet produced a slow clearance of glucose from the gastric fluid and a gradual increase in hemolymph glucose. More intense hydrolysis of protein in the gastric chamber occurred 6-12 h after ingestion and then amino acids tended to increase in hemolymph. Triglyceride concentration in hemolymph rose earlier in wheat-fed lobsters than in lobsters fed other carbohydrates, but it decreased the most 24 h later. Analyses of metabolite levels and activities of different metabolic enzymes revealed that intermolt lobsters had a low capacity to store and use glycogen, although it was slightly higher in wheat-fed lobsters. Lobsters fed maize and rice diets increased amino acid catabolism, while wheat-fed lobsters exhibited higher utilization of fatty acids. Multivariate analysis confirmed that the type of carbohydrate ingested had a profound effect on overall metabolism. Although we found no evidence of a protein-sparing effect of dietary carbohydrate, differences in the kinetics of their digestion and absorption impacted lobster metabolism determining the fate of other nutrients. PMID:25268641

  14. A holistic view of dietary carbohydrate utilization in lobster: digestion, postprandial nutrient flux, and metabolism.

    Directory of Open Access Journals (Sweden)

    Leandro Rodríguez-Viera

    Full Text Available Crustaceans exhibit a remarkable variation in their feeding habits and food type, but most knowledge on carbohydrate digestion and utilization in this group has come from research on few species. The aim of this study was to make an integrative analysis of dietary carbohydrate utilization in the spiny lobster Panulirus argus. We used complementary methodologies such as different assessments of digestibility, activity measurements of digestive and metabolic enzymes, and post-feeding flux of nutrients and metabolites. Several carbohydrates were well digested by the lobster, but maize starch was less digestible than all other starches studied, and its inclusion in diet affected protein digestibility. Most intense hydrolysis of carbohydrates in the gastric chamber of lobster occurred between 2-6 h after ingestion and afterwards free glucose increased in hemolymph. The inclusion of wheat in diet produced a slow clearance of glucose from the gastric fluid and a gradual increase in hemolymph glucose. More intense hydrolysis of protein in the gastric chamber occurred 6-12 h after ingestion and then amino acids tended to increase in hemolymph. Triglyceride concentration in hemolymph rose earlier in wheat-fed lobsters than in lobsters fed other carbohydrates, but it decreased the most 24 h later. Analyses of metabolite levels and activities of different metabolic enzymes revealed that intermolt lobsters had a low capacity to store and use glycogen, although it was slightly higher in wheat-fed lobsters. Lobsters fed maize and rice diets increased amino acid catabolism, while wheat-fed lobsters exhibited higher utilization of fatty acids. Multivariate analysis confirmed that the type of carbohydrate ingested had a profound effect on overall metabolism. Although we found no evidence of a protein-sparing effect of dietary carbohydrate, differences in the kinetics of their digestion and absorption impacted lobster metabolism determining the fate of other

  15. Neonates with inborn errors of metabolism: spectrum and short-term outcomes at a tertiary care hospital.

    Science.gov (United States)

    Gündüz, Mehmet; Ünal, Sevim; Okur, İlyas; Ayrancı Sucaklı, İclal; Güzel, Fatma; Koç, Nevra

    2015-01-01

    We aimed to evaluate the neonates diagnosed as IEM in our neonatal intensive care unit and their outcomes. Among 2994 neonates hospitalized, 51 were diagnosed as IEM (1.7%). Admission complaints were poor feeding, decreased activity, jaundice, seizures, abnormal screening and respiratory problems. Phenylketonuria (11), organic acidemias (8), maple syrup urine disease (5), citrullinemia (5), galactosemia (4), nonketotic hyperglycinemia (4) and tyrosinemia (2) were the most commonly diagnosed IEMs. The follow-up period was 2.5-43 months. Among the 33 neonates followed, 19 had normal development, 9 had developmental delays and 5 had cerebral palsy according to the Guide for Monitoring Child Development. Postnatal age on admission, Apgar score at 5 minutes, being transferred, peritoneal dialysis, cranial ultrasonographic findings, consanguinity and sibling history had significant effects on outcome. Early diagnosis through expanded neonatal screening in countries with high rates of consanguinity, enabling the initiation of early treatment, is essential for achieving low mortality rates and good prognoses. PMID:26613220

  16. Seasonal influences on carbohydrate metabolism in the CAM bromeliad Aechmea ‘Maya’: consequences for carbohydrate partitioning and growth

    Science.gov (United States)

    Ceusters, Johan; Borland, Anne M.; Ceusters, Nathalie; Verdoodt, Veerle; Godts, Christof; De Proft, Maurice P.

    2010-01-01

    Background and Aims Photosynthetic plasticity in response to a range of environmental factors that include [CO2], water availability, light intensity and temperature, is ubiquitous among plants with crassulacean acid metabolism (CAM). The present study examined how seasonal changes in light availability, as experienced by greenhouse CAM crops in northern latitude regions, influence diel carboxylation patterns and impact on carbon gain and seasonal accumulation of biomass. Methods In the CAM bromeliad Aechmea ‘Maya’ integrated measurements of leaf gas exchange, diel metabolite dynamics (e.g. malate, soluble sugars and starch) and biomass accumulation were made four times a year, i.e. in winter, spring, summer and autumn. Key Results During the brighter seasons (spring and summer) daytime Phases II and IV were dominated by C4 carboxylation, whilst the higher diurnal uptake in the autumn and winter was characterized by equal contributions of both Rubisco and PEPC. As a consequence, net CO2 uptake showed a significant depression at the end of the day in the darker months when supplementary illumination was turned off. Remarkable seasonal consistency was found in the amount of storage reserves available for nocturnal carboxylation, a consequence of predominantly daytime export of carbohydrate in spring and summer whilst nocturnal export was the major sink for carbohydrate in autumn and winter. Conclusions Throughout the different seasons Aechmea ‘Maya’ showed considerable plasticity in the timing and magnitude of C3 and C4 carboxylation processes over the diel cycle. Under low PPFD (i.e. winter and autumn) it appears that there was a constraint on the amount of carbohydrate exported during the day in order to maintain a consistent pool of transient carbohydrate reserves. This gave remarkable seasonal consistency in the amount of storage reserves available at night, thereby optimizing biomass gain throughout the year. The data have important practical

  17. The role of compensation of carbohydrate metabolism in pregnant women with type 1 diabetes mellitus in the development of perinatalcomplications

    OpenAIRE

    Roza Mikhaylovna Esayan; Ol'ga Rafael'evna Grigoryan; Elena Vladimirovna Pekareva

    2009-01-01

    With DM1 morbidity reaching epidemic growth in young people including women of childbearing age, planning and managing pregnancy in patients with disturbances of carbohydrate metabolism acquires increasingly greater significance. Of primary importance are evening mutual influence of DM1 and pregnancy (a high-risk condition) and prevention of perinatal complications resulting from poor compensation of carbohydrate metabolism.

  18. Carbon catabolite repression and global control of the carbohydrate metabolism in Lactococcus lactis.

    NARCIS (Netherlands)

    Luesink, E.J.

    1998-01-01

    In view of the economic importance of fermented dairy products considerable scientific attention has been given to various steps of fermentation processes, including the L-lactate formation of lactic acid bacteria (de Vos, 1996). In particular, the carbohydrate metabolism of L. lactis has been the s

  19. Genomic profiling of carbohydrate metabolism in the ectomycorrhizal fungus Tuber melanosporum.

    Science.gov (United States)

    Ceccaroli, P; Buffalini, M; Saltarelli, R; Barbieri, E; Polidori, E; Ottonello, S; Kohler, A; Tisserant, E; Martin, F; Stocchi, V

    2011-02-01

    • Primary carbohydrate metabolism plays a special role related to carbon/nitrogen exchange, as well as metabolic support of fruiting body development, in ectomycorrhizal macrofungi. In this study, we used information retrieved from the recently sequenced Tuber melanosporum genome, together with transcriptome analysis data and targeted validation experiments, to construct the first genome-wide catalogue of the proteins supporting carbohydrate metabolism in a plant-symbiotic ascomycete. • More than 100 genes coding for enzymes of the glycolysis, pentose phosphate, tricarboxylic acid, glyoxylate and methylcitrate pathways, glycogen, trehalose and mannitol metabolism and cell wall precursor were annotated. Transcriptional regulation of these pathways in different stages of the T. melanosporum lifecycle was investigated using whole-genome oligoarray expression data together with real-time reverse transcription-polymerase chain reaction analysis of selected genes. • The most significant results were the identification of methylcitrate cycle genes and of an acid invertase, the first enzyme of this kind to be described in a plant-symbiotic filamentous fungus. • A subset of transcripts coding for trehalose, glyoxylate and methylcitrate enzymes was up-regulated in fruiting bodies, whereas genes involved in mannitol and glycogen metabolism were preferentially expressed in mycelia and ectomycorrhizas, respectively. These data indicate a high degree of lifecycle stage specialization for particular branches of carbohydrate metabolism in T. melanosporum. PMID:21039570

  20. Metabolic profiling reveals coordinated switches in primary carbohydrate metabolism in grape berry (Vitis vinifera L.), a non-climacteric fleshy fruit

    OpenAIRE

    Dai, Z.; Leon, C.; Feil, R.; Lunn, J.; Delrot, S.; Gomes, E.

    2013-01-01

    Changes in carbohydrate metabolism during grape berry development play a central role in shaping the final composition of the fruit. The present work aimed to identify metabolic switches during grape development and to provide insights into the timing of developmental regulation of carbohydrate metabolism. Metabolites from central carbon metabolism were measured using high-pressure anion-exchange chromatography coupled to tandem mass spectrometry and enzymatic assays during the development of...

  1. A central role of abscisic acid in stress-regulated carbohydrate metabolism.

    Directory of Open Access Journals (Sweden)

    Stefan Kempa

    Full Text Available BACKGROUND: Abiotic stresses adversely affect plant growth and development. The hormone abscisic acid (ABA plays a central role in the response and adaptation to environmental constraints. However, apart from the well established role of ABA in regulating gene expression programmes, little is known about its function in plant stress metabolism. PRINCIPAL FINDINGS: Using an integrative multiparallel approach of metabolome and transcriptome analyses, we studied the dynamic response of the model glyophyte Arabidopsis thaliana to ABA and high salt conditions. Our work shows that salt stress induces complex re-adjustment of carbohydrate metabolism and that ABA triggers the initial steps of carbon mobilisation. SIGNIFICANCE: These findings open new perspectives on how high salinity and ABA impact on central carbohydrate metabolism and highlight the power of iterative combinatorial approaches of non-targeted and hypothesis-driven experiments in stress biology.

  2. Effect of salicylic acid on the growth photosynthesis and carbohydrate metabolism in salt stressed maize plants

    International Nuclear Information System (INIS)

    Aqueous solutions of salicylic acid as a spray to Na CI-treated corn (Zea mays L,) significantly increased the growth of shoots and roots as measured after seven days of treatment. Spraying of salicylic acid caused significant increases in the activity of both ribulose 1,5 bisphosphate carboxylase (rubisco) enzyme and photosynthetic pigments. Moreover, salicylic acid treatment induced high values of soluble carbohydrate fractions in salt stressed plants as compared with salicylic acid treated samples. These data suggest that salicylic acid might improve the growth pattern of NaCl-treated maize plants via increasing the rate of photosynthesis and carbohydrate metabolism

  3. Smith-Lemli-Opitz Syndrome and Inborn Errors of Cholesterol Synthesis: Summary of the 2007 SLOS/RSH Foundation Scientific Conference Sponsored by the National Institutes of Health

    OpenAIRE

    Merkens, Louise S.; Wassif, Christopher; RN, Kristy Healy; Pappu, Anuradha S.; DeBarber, Andrea E.; Penfield, Jennifer A.; Lindsay, Rebecca A.; Roullet, Jean-Baptiste; Porter, Forbes D.; Steiner, Robert D.

    2009-01-01

    In June 2007, the Smith-Lemli-Opitz/RSH Foundation held a scientific conference hosted jointly by Dr. Robert Steiner from Oregon Health & Science University (OHSU) and Dr. Forbes D. Porter from The Eunice Kennedy Shriver National Institute for Child Health and Human Development (NICHD), National Institutes of Health. The main goal of this meeting was to promote interaction between scientists with expertise in cholesterol homeostasis, brain cholesterol metabolism, developmental biology, and ox...

  4. Carbohydrate utilization and metabolism is highly differentiated in Agaricus bisporus

    OpenAIRE

    Patyshakuliyeva, A.; Jurak, E.; Kohler, A; Baker, A.; Battaglia, E; Bruijn, de, M.E.; Burton, K.S.; Challen, M.P.; Cuotinho, P.M.; Eastwood, D.C.; Gruben, B.S.; Makela, M.R.; Martin, F.; Nadal, M; Brink, van den, A Arno

    2013-01-01

    Background - Agaricus bisporus is commercially grown on compost, in which the available carbon sources consist mainly of plant-derived polysaccharides that are built out of various different constituent monosaccharides. The major constituent monosaccharides of these polysaccharides are glucose, xylose, and arabinose, while smaller amounts of galactose, glucuronic acid, rhamnose and mannose are also present. Results - In this study, genes encoding putative enzymes from carbon metabolism were i...

  5. Mcy protein, a potential antidiabetic agent: evaluation of carbohydrate metabolic enzymes and antioxidant status.

    Science.gov (United States)

    Marella, Saritha; Maddirela, Dilip Rajasekhar; Kumar, E G T V; Tilak, Thandaiah Krishna; Badri, Kameswara Rao; Chippada, Apparao

    2016-05-01

    The objective of the present study is to elucidate the long-term effects of anti-hyperglycemic active principle, Mcy protein (MCP), isolated from the fruits of Momordica cymbalaria on carbohydrate metabolism and oxidative stress in experimental diabetic rats. We used streptozotocin induced diabetic rats for the current studies. Our studies showed that MCP (2.5mg/kg.b.w) treatment significantly normalized the deranged activities of critical carbohydrate metabolizing enzymes, hexokinase, glucose-6-phosphate dehydrogenase, glucose-6-phosphatase and fructose-1,6-bis phosphatase. In addition MCP showed inhibitory activity on α-glucosidase and aldose reductase enzymes in in vitro assays. Further MCP treatment improved the antioxidant defensive mechanism by preventing deleterious oxidative products of cellular metabolism, which initiates the lipid peroxidation and by normalizing the antioxidant enzyme (catalase, superoxide dismutase, glutathione peroxidase) activities. Additional structural studies using circular dichroism spectroscopy indicate that MCP contains majorly α-helix. Our findings suggest MCP regulates blood glucose and better manage diabetes mellitus associated complications by regulating carbohydrate metabolism and by protecting from the deleterious effects of oxidative stress. PMID:26826289

  6. Green tea attenuates cardiovascular remodelling and metabolic symptoms in high carbohydrate-fed rats.

    Science.gov (United States)

    Rickman, Celestine; Iyer, Abishek; Chan, Vincent; Brown, Lindsay

    2010-12-01

    Excess carbohydrate in the diet may initiate a chronic state of oxidative stress exacerbating the clinical and biochemical symptoms of diet-induced type 2 diabetes, especially glucose intolerance, lipid abnormalities and cardiovascular complications. This study has tested whether green tea, rich in antioxidants, improves both cardiovascular symptoms and glucose intolerance and also reduces oxidative stress in rats fed a high carbohydrate diet. Male 8 week old Wistar rats were fed a diet including fructose and condensed milk (each 40%) for 16 weeks (112 days); control rats were fed corn starch. Green tea-containing food was started from day 1 for the prevention protocol and from day 56 for the reversal protocol. High carbohydrate diet-fed rats showed glucose intolerance, hypertension, mild left ventricular hypertrophy, approximate doubling of cardiac interstitial and perivascular collagen deposition, increased passive diastolic stiffness and increased plasma malondialdehyde concentrations. Administration of green tea to high carbohydrate diet-fed rats prevented and reversed glucose intolerance and the increased systolic blood pressure, left ventricular wet weight, interstitial collagen and passive diastolic stiffness. Plasma malondialdehyde concentrations were also normalized. In summary, treatment with green tea both prevented and reversed the cardiovascular remodelling and metabolic changes seen in high carbohydrate-fed rats suggesting a chronic state of oxidative stress plays a key role in the symptom initiation and progression. Further, green tea may be a useful complementary therapy in diet-induced type 2 diabetes. PMID:20874683

  7. Effects of Salt Stress on Carbohydrate Metabolism in Desert Soil Alga Microcoleus vaginatus Gom.

    Institute of Scientific and Technical Information of China (English)

    Lan-Zhou Chen; Dun-Hai Li; Li-Rong Song; Chun-Xiang Hu; Gao-Hong Wang; Yong-Ding Liu

    2006-01-01

    The effects of salt stress on carbohydrate metabolism in Microcoleus vaginatus Gom., a cyanobacterium isolated from desert algal crusts, were investigated in the present study. Extracellular total carbohydrates and exopolysaccharides (EPS) in the culture medium produced by M. vaginatus increased significantly during the growth phase and reached a maximum during the stationary phase. The production of extracellular carbohydrates also significantly increased under higher salt concentrations, which was attributed to an increase in low molecular weight carbohydrates. In the presence of NaCl, the production of cellular total carbohydrates decreased and photosynthetic activity was impaired, whereas cellular reducing sugars,water-soluble sugars and sucrose content and sucrose phosphate synthase activity increased, reaching a maximum in the presence of 200 mmol/L NaCl. These parameters were restored to original levels when the algae were transferred to a non-saline medium. Sodium and K+ concentrations of stressed cells decreased significantly and H+-ATPase activity increased after the addition of exogenous sucrose or EPS. The results suggest that EPS and sucrose are synthesized to maintain the cellular osmotic equilibrium between the intra- and extracellular environment, thus protecting algal cells from osmotic damage, which was attributed to the selective exclusion of cellular Na+ and K+ by H+-ATPase.

  8. Flight metabolism in Panstrongylus megistus (Hemiptera: Reduviidae: the role of carbohydrates and lipids

    Directory of Open Access Journals (Sweden)

    Lilián E Canavoso

    2003-10-01

    Full Text Available The metabolism of lipids and carbohydrates related to flight activity in Panstrongylus megistus was investigated. Insects were subjected to different times of flight under laboratory conditions and changes in total lipids, lipophorin density and carbohydrates were followed in the hemolymph. Lipids and glycogen were also assayed in fat body and flight muscle. In resting insects, hemolymph lipids averaged 3.4 mg/ml and significantly increased after 45 min of flight (8.8 mg/ml, P < 0.001. High-density lipophorin was the sole lipoprotein observed in resting animals. A second fraction with lower density corresponding to low-density lipophorin appeared in insects subjected to flight. Particles from both fractions showed significant differences in diacylglycerol content and size. In resting insects, carbohydrate levels averaged 0.52 mg/ml. They sharply declined more than twofold after 15 min of flight, being undetectable in hemolymph of insects flown for 45 min. Lipid and glycogen from fat body and flight muscle decreased significantly after 45 min of flight. Taken together, the results indicate that P. megistus uses carbohydrates during the initiation of the flight after which, switching fuel for flight from carbohydrates to lipids.

  9. Carbohydrate metabolism during prolonged exercise and recovery: interactions between pyruvate dehydrogenase, fatty acids, and amino acids

    DEFF Research Database (Denmark)

    Mourtzakis, Marina; Saltin, B.; Graham, T.;

    2006-01-01

    During prolonged exercise, carbohydrate oxidation may result from decreased pyruvate production and increased fatty acid supply and ultimately lead to reduced pyruvate dehydrogenase (PDH) activity. Pyruvate also interacts with the amino acids alanine, glutamine, and glutamate, whereby the decline...... activity (P < 0.05) and increased PDH kinase 4 mRNA (P < 0.05) during exercise and recovery. At 1 h of exercise, pyruvate production was greatest and was closely linked to glutamate, which was the predominant amino acid taken up during exercise and recovery. Alanine and glutamine were also associated with...... pyruvate metabolism, and they comprised 68% of total amino-acid release during exercise and recovery. Thus reduced pyruvate production was primarily associated with reduced carbohydrate oxidation, whereas the greatest production of pyruvate was related to glutamate, glutamine, and alanine metabolism in...

  10. A Model for Carbohydrate Metabolism in the Diatom Phaeodactylum tricornutum Deduced from Comparative Whole Genome Analysis

    OpenAIRE

    Kroth, Peter G.; Chiovitti, Anthony; Gruber, Ansgar; Martin-jezequel, Veronique; Mock, Thomas; Schnitzler Parker, Micaela; Michele S. Stanley; Kaplan, Aaron; Caron, Lise; Weber, Till; Maheswari, Uma; Armbrust, Elisabeth Virginia; Bowler, Chris

    2008-01-01

    Background:Diatoms are unicellular algae responsible for approximately 20% of global carbon fixation. Their evolution by secondary endocytobiosis resulted in a complex cellular structure and metabolism compared to algae with primary plastids.Methodology/Principal Findings:The whole genome sequence of the diatom Phaeodactylum tricornutum has recently been completed. We identified and annotated genes for enzymes involved in carbohydrate pathways based on extensive EST support and comparison to ...

  11. Effects of Different Carbohydrate Sources on Fructan Metabolism in Plants of Chrysolaena obovata Grown in vitro

    OpenAIRE

    Flavio eTrevisan; Vanessa Fatima eOliveira; Maria Angela Machado Carvalho; Marília Gaspar Gaspar

    2015-01-01

    Chrysolaena obovata (Less.) Dematt., previously named Vernonia herbacea, is an Asteraceae native to the Cerrado which accumulates about 80% of the rhizophore dry mass as inulin-type fructans. Considering its high inulin production and the wide application of fructans, a protocol for C. obovata in vitro culture was recently established. Carbohydrates are essential for in vitro growth and development of plants and can also act as signaling molecules involved in cellular adjustments and metabol...

  12. Cardiovascular Risks Factors and their Relationship with Disorders of Carbohydrate and Lipid Metabolism

    OpenAIRE

    Lilian Leguen Gulgar; Maricel Castellanos; María de Jesús Sánchez Bouza; Mikhail Benet Rodríguez

    2014-01-01

    Background: cardiovascular disease has been the leading cause of death in Cuba, where studies on emerging cardiovascular risk factors as predictors of cardiovascular risk are scarce. Objective: to determine the association between cardiovascular risk factors and disorders of carbohydrate and lipid metabolism. Methods: a correlational study was conducted with a sample of 105 men and women selected from a total of 346 workers of the University of Medical Sciences of Cienfuegos from June 2011 th...

  13. Changes in the carbohydrate-energy metabolism with radiation-induced intestine syndrome

    International Nuclear Information System (INIS)

    A local exposure of the rat abdomen in a dose of 3.6 cC/kg decreases the oxygen uptake, oxidation of glucose and fatty acids, glucose tolerance and insulin resistance, and also causes a trend toward lactic acidosis. These changes in the carbohydrate-energy metabolism are normalized with the administration of insulin and dichloracetate, and they may be interpreted as consequences of a shock provoked by a massive predominant injury to the intestine

  14. INFLUENCE OF LIFETIME EXPOSURE OF SUBLETHAL DOSES OF CADMIUM TO SELECTED PARAMETERS OF CARBOHYDRATE METABOLISM

    OpenAIRE

    František Ništiar; Iveta Cimboláková; Eva Lovásová; Jaroslava Nováková; Agnesa Lukačínová

    2012-01-01

    The aim of the study was to assess the effects of exposure to low doses of cadmium dissolved in drinking water (at a concentration 200 times higher than the maximum permissible dose) on selected parameters of carbohydrate metabolism in 20 Wistar rats. Animals were divided into two groups – control and experimental groups exposed to low doses of cadmium chloride in concentration 20 μM of drinking water. We studied the biochemical parameters, as glucose, hemoglobin, glycated hemoglobin...

  15. Expression profiles of genes related to carbohydrate metabolism provide new insights into carbohydrate accumulation in seeds and seedlings of Ricinus communis in response to temperature

    NARCIS (Netherlands)

    Ribeiro de Jesus, P.R.; Ligterink, W.; Hilhorst, H.W.M.

    2015-01-01

    Ricinus communis possesses a specific metabolic signature to adjust growth and developmental processes in response to temperature: carbohydrates are accumulated at low temperatures, whereas amino acids are accumulated at elevated temperatures. Our objective was to assess tissue-specific changes in t

  16. Changes in carbohydrate metabolism in Plasmopara viticola-infected grapevine leaves.

    Science.gov (United States)

    Gamm, Magdalena; Héloir, Marie-Claire; Bligny, Richard; Vaillant-Gaveau, Nathalie; Trouvelot, Sophie; Alcaraz, Gérard; Frettinger, Patrick; Clément, Christophe; Pugin, Alain; Wendehenne, David; Adrian, Marielle

    2011-09-01

    The oomycete Plasmopara viticola is responsible for downy mildew, a severe grapevine disease. In infected grapevine leaves, we have observed an abnormal starch accumulation at the end of the dark period, suggesting modifications in starch metabolism. Therefore, several complementary approaches, including transcriptomic analyses, measurements of enzyme activities, and sugar quantification, were performed in order to investigate and to understand the effects of P. viticola infection on leaf starch and-to a larger extent-carbohydrate metabolism. Our results indicate that starch accumulation is associated with an increase in ADP-glucose pyrophosphorylase (AGPase) activity and modifications in the starch degradation pathway, especially an increased α-amylase activity. Together with these alterations in starch metabolism, we have observed an accumulation of hexoses, an increase in invertase activity, and a reduction of photosynthesis, indicating a source-to-sink transition in infected leaf tissue. Additionally, we have measured an accumulation of the disaccharide trehalose correlated to an increased trehalase gene expression and enzyme activity. Altogether, these results highlight a dramatic alteration of carbohydrate metabolism correlated with later stages of P. viticola development in leaves. PMID:21649510

  17. Carbohydrate metabolism disorders in patients with rheumatoid arthritis and ankylosing spondylitis – impact of treatment

    Directory of Open Access Journals (Sweden)

    Piotr Dąbrowski

    2014-06-01

    Full Text Available Chronic inflammation – the crucial pathogenic mechanism of rheumatoid arthritis and ankylosing spondylitis – is the main cause of accelerated atherosclerosis, insulin resistance and well-known consequences related to it. The conservative treatment of rheumatoid arthritis and ankylosing spondylitis may provide a significant influence on glucose metabolism. The paper is a literature overview concerning insulin resistance and impaired glucose metabolism during treatment with disease-modifying drugs including biologic DMARDs (disease-modifying antirheumatic drugs, corticosteroids and commonly used non-steroidal anti-inflammatory drugs (NSAID. It has been found that the risk of carbohydrate disorders among those patients is much lower after therapy with hydroxychloroquine, methotrexate and TNF blockers – particularly with infliximab. The NSAID may play an important protective role in reducing risk of diabetes. The recent data show, contrary to general opinion, the advantageous outcome for glucose metabolism after treatment with corticosteroids, especially in the early active stage of rheumatoid arthritis.

  18. The regulation of carbohydrate and fat metabolism during and after exercise.

    Science.gov (United States)

    Holloszy, J O; Kohrt, W M; Hansen, P A

    1998-09-15

    The rate of carbohydrate utilization during prolonged, strenuous exercise is closely geared to the energy needs of the working muscles. In contrast, fat utilization during exercise is not tightly regulated, as there are no mechanisms for closely matching availability and metabolism of fatty acids to the rate of energy expenditure. As a result, the rate of fat oxidation during exercise is determined by the availability of fatty acids and the rate of carbohydrate utilization. Blood glucose and muscle glycogen are essential for prolonged strenuous exercise, and exhaustion can result either from development of hypoglycemia or depletion of muscle glycogen. Both absolute and relative (i.e. % of maximal O2 uptake) exercise intensities play important roles in the regulation of substrate metabolism. The absolute work rate determines the total quantity of fuel required, while relative exercise intensity plays a major role in determining the proportions of carbohydrate and fat oxidized by the working muscles. As relative exercise intensity is increased, there is a decrease in the proportion of the energy requirement derived from fat oxidation and an increase in that provided by carbohydrate oxidation. During moderately strenuous exercise of an intensity that can be maintained for 90 minutes or longer ( approximately 55-75% of VO2max), there is a progressive decline in the proportion of energy derived from muscle glycogen and a progressive increase in plasma fatty acid oxidation. The adaptations induced by endurance exercise training result in a marked sparing of carbohydrate during exercise, with an increased proportion of the energy being provided by fat oxidation. The mechanisms by which training decreases utilization of blood glucose are not well understood. However, the slower rate of glycogenolysis can be explained on the basis of lower concentrations of inorganic phosphate (Pi) in trained, as compared to untrained, muscles during exercise of the same intensity. The

  19. In vivo sup 1 sup 3 C MRS studies of carbohydrate metabolism

    CERN Document Server

    Halliday, J

    2003-01-01

    The work described in this thesis was performed by the except where indicated, within the Magnetic Resonance Centre at the University of Nottingham during the period between October 1999 and October 2002. Although much is known about the major pathways of carbohydrate metabolism, there is still much to be learnt about the exact mechanisms of many of these pathways. Of particular interest is how these pathways are modified under different physiological conditions and in diseased states. sup 1 sup 3 C NMR spectroscopy provides a non-invasive means for studying carbohydrate metabolism in vivo, and the work presented within this thesis gives two such examples of this in human subjects. Natural abundance sup 1 sup 3 C NMR spectroscopy was used to measure glycogen levels in gastrocnemius muscle. The diurnal changes in response to mixed meals were measured in both type 2 diabetic subjects and age and weight matched controls. Metabolic studies were performed to complement the NMR measurements. The data obtained in th...

  20. Effect of alcohol consumption on hormones involved in carbohydrate and lipid metabolism in premenopausal women

    Energy Technology Data Exchange (ETDEWEB)

    Law, J.S.; Bhathena, S.J.; Kim, Y.C.; Berlin, E.; Judd, J.T.; Reichman, M.E.; Taylor, P.R.; Schatzkin, A. (Dept. of Agriculture, Beltsville, MD (United States) NCI, Bethesda, MD (United States))

    1991-03-15

    Alcohol consumption alters carbohydrate and lipid metabolism which are in part regulated by pancreatic and adrenal hormones. The menstrual cycle per se produces changes in several peptide and steroid hormones besides the sex hormones. The authors investigated the effect of moderate alcohol consumption on plasma hormone levels in 40 premenopausal women. The subjects were fed controlled diets containing 35% of calories from fat. In a random crossover design women were given either alcohol or a soft-drink of equal caloric value for 3 menstrual cycles. Fasting blood samples were collected in the third cycle during follicular, ovulatory and luteal phases. Plasma dehydroepiandrosterone-sulphate (DHEA-S), insulin, glucagon and cortisol levels were measured by radioimmunoassay. Moderate alcohol consumption had no effect on plasma insulin and DHEA-S levels but significantly increased glucagon and cortisol levels. Menstrual cycle per se affected plasma glucagon level in that the levels were higher during follicular phase than luteal phase. Thus, changes in carbohydrate and lipid metabolism following alcohol consumption are mediated in part by alterations in hormones involved in their metabolism.

  1. Chitosan-Mediated shRNA Knockdown of Cytosolic Alanine Aminotransferase Improves Hepatic Carbohydrate Metabolism.

    Science.gov (United States)

    González, Juan D; Silva-Marrero, Jonás I; Metón, Isidoro; Caballero-Solares, Albert; Viegas, Ivan; Fernández, Felipe; Miñarro, Montserrat; Fàbregas, Anna; Ticó, Josep R; Jones, John G; Baanante, Isabel V

    2016-02-01

    Alanine aminotransferase (ALT) catalyses a transamination reaction that links carbohydrate and amino acid metabolism. In this study, we examined the effect of silencing cytosolic ALT (cALT) expression on the hepatic metabolism in Sparus aurata. A number of siRNA and shRNA designed to down-regulate cALT expression were validated in HEK-293 cells transfected with plasmids expressing S. aurata cALT or mitochondrial ALT (mALT) isoforms: ALT silencing significantly decreased the expression levels of S. aurata mRNA cALT1 to 62% (siRNA) and 48% (shRNA) of the values observed in control cells. The effect of cALT silencing was analysed in the liver of S. aurata 72 h after intraperitoneal injection of chitosan-tripolyphosphate (TPP) nanoparticles complexed with a plasmid encoding a shRNA to down-regulate cALT expression (pCpG-si1sh1). In fish fed diets with different ratio of protein to carbohydrate and treated with chitosan-TPP-pCpG-si1sh1, cALT1 and cALT2 mRNA levels significantly decreased irrespective of the diet. Consistently, ALT activity decreased in liver of treated animals. In the liver of S. aurata treated with chitosan-TPP-pCpG-si1sh1 nanoparticles, down-regulation of cALT expression increased the activity of key enzymes in glycolysis (6-phosphofructo-1-kinase and pyruvate kinase) and protein metabolism (glutamate dehydrogenase). Besides showing for the first time that administration of chitosan-TPP-pCpG-si1sh1 nanoparticles silences hepatic cALT expression in vivo, our data support that down-regulation of cALT could improve the use of dietary carbohydrates to obtain energy and spare protein catabolism. PMID:26475146

  2. Influence of gamma radiation on carbohydrates metabolism of ripening papaya (Carica papaya L. cv. Solo)

    International Nuclear Information System (INIS)

    Food irradiation is one of the most promising treatments that can be utilized for fruits disinfestation and extension of shelf life. The authors studied the influence of 0,5 kGy of Gamma irradiation on the soluble carbohydrates composition of papaya (Carica papaya L. cv. Solo) fruit, and on sucrose metabolizing enzymes: sucrose synthase (SS), sucrose-phosphate synthase, acid and neutral invertases activities, during ripening. The results demonstrated that ethylene production, total soluble sugars, sucrose content, and sucrose-phosphate synthase and invertases activities were affected by irradiation, but not respiration, glucose and fructose content, and SS activity. (author)

  3. Carbohydrate metabolism and quality of life in patients after surgical treatment of insulinoma

    Directory of Open Access Journals (Sweden)

    Ivan Ivanovich Dedov

    2014-08-01

    Full Text Available Objectives. T study the quality of life and status of carbohydrate metabolism in patients after surgical treatment insulinoma. Methods: The study involved 20 patients divided in two groups: the first group with a catamnesis duration of up to five years; the second group with a catamnesis duration of more than five years. We studied anthropometric parameters and carbohydrate metabolism as well as psychological questioning of patients using SF-36 questionnaire, the data was considered statistically significant at p<0.05. Results. severe combined postoperative complications were more frequent in the first group (63.6% vs. 22.2%, p=0.07, due to extend of the performed surgery. Adrenergic symptoms prior to the surgery were detected in 90.9% of cases in the first group and in 77.7% of cases in the second group. After treatment these numbers decreased to 36.4% and 11.1% respectively (p=0.039 and 0.026. Neuroglycopeniс symptoms before treatment were detected in 90.9% of cases in the first group and for all patients in the second, while after treatment persisted only in 45.5% and 33.3% of cases respectively (p=0.045 and 0.036. Carbohydrate metabolism have normalized for the majority of patients. Two patients (18.2% of the first group showed impaired glucose tolerance. Improved carbohydrate metabolism was associated with a decrease in body weight in both groups. Results of psychological questionnaires were comparable with the survey data obtained in general population in the Russian Federation. Conclusion. Surgical treatment of insulinomas is highly effective. Physical and psychological status of patients in most cases corresponds with those typical for this age-sex group of the population of the Russian Federation. Long-term treatment results do not depend on duration of the catamnesis. Complications that developed from surgical treatment have the main influence on the health of patients.

  4. Chemical radiation protection of certain aspects of carbohydrate metabolism in irradiated rats

    International Nuclear Information System (INIS)

    Because of the practical importance of certain compounds that exhibit protective character against lethal effects of ionizing radiation, repeated investigations have been carried out to evaluate the radioprotective efficiency of such compounds and to study the mechanism through which they manifest their radioprotective effects. The present study is an attempt to investigate the possible role which the heterocyclic nitrogenous compounds (glyoxaline and benzoglyoxaline) can play as radioprotectors against radiation-induced changes in carbohydrate metabolism. The parameters studied were, survival rate, bloodglucose level, gamma insulin level and liver glycogen concentration

  5. beta-adrenergic effects on carbohydrate metabolism in the unweighted rat soleus muscle

    Science.gov (United States)

    Kirby, Christopher R.; Tischler, Marc E.

    1990-01-01

    The effect of unweighting on the response of the soleus-muscle carbohydrate metabolism to a beta-adrenergic agonist (isoproterenol) was investigated in rats that were subjected to three days of tail-cast suspension. It was found that isoproterenol promoted glycogen degradation in soleus from suspended rats to a higher degree than in weighted soleus from control rats, and had no effect in unweighted digitorum longus. However, isoproterenol did not have a greater inhibitory effect on the net uptake of tritium-labeled 2-deoxy-glucose by the unweighted soleus and that isoproterenol inhibited hexose phosphorylation less in the unweighted than in the control muscle.

  6. Serum neutral amino acid concentrations in cirrhotic patients with impaired carbohydrate metabolism.

    Directory of Open Access Journals (Sweden)

    Watanabe,Akiharu

    1983-08-01

    Full Text Available Serum neutral amino acid levels in cirrhotic patients with abnormal oral glucose tolerance test patterns were not different from those of subjects without impaired carbohydrate metabolism. However, the characteristic features of serum aminograms in the patients, that is, increased levels of tyrosine, decreased levels of valine and leucine and the diminished ratio of branched chain amino acids to phenylalanine and tyrosine levels, were less pronounced in those treated with insulin. This finding is clinically important for evaluating the serum aminogram of cirrhotic patients under insulin therapy.

  7. Exogenous Classic Phytohormones Have Limited Regulatory Effects on Fructan and Primary Carbohydrate Metabolism in Perennial Ryegrass (Lolium perenne L.)

    Czech Academy of Sciences Publication Activity Database

    Gasperl, A.; Morvan-Bertrand, A.; Prud'homme, M. P.; van der Graaff, E.; Roitsch, Thomas

    2016-01-01

    Roč. 6, jan (2016), s. 1251. ISSN 1664-462X R&D Projects: GA MŠk(CZ) LO1415 Institutional support: RVO:67179843 Keywords : enzymatic activity * fructan exohydrolase * fructan metabolism * fructosyltransferase * perennial ryegrass * phytohormones * primary carbohydrate metabolism Subject RIV: ED - Physiology Impact factor: 3.948, year: 2014

  8. Inborn anemias in mice

    International Nuclear Information System (INIS)

    hereditary anemias of mice have been the chief objects of investigation. At present under study are four macrocytic anemias, five hemolytic anemias, nonhemolytic microcytic anemia, transitory siderocytic anemia, sex-linked iron-transport anemia, an α-thalassemia, and a new target-cell anemia. Each of these blood dyscrasias is caused by the action of a unique mutant gene, which determines the structure of different intracellular molecules, and thus controls a different metabolic process. Thus our wide range of different hereditary anemias has considerable potential for uncovering many different aspects of hemopoietic homeostatic mechanisms in the mouse. Each anemia is studied through: (a) characterization of peripheral blood values, (b) determinations of radiosensitivity under a variety of conditions, (c) measurements of iron metabolism and heme synthesis, (d) histological and biochemical study of blood-forming tissue, (e) functional tests of the stem cell component, (f) examination of responses to erythroid stimuli, and (g) transplantation of tissue between individuals of differently affected genotypes

  9. Role and metabolism of free leucine in skeletal muscle in protein sparing action of dietary carbohydrate and fat

    International Nuclear Information System (INIS)

    Feeding rats with either a carbohydrate meal or a fat meal to the previously fasted rats caused significant decrease in urinary output of urea and total nitrogen. The content of free leucine in skeletal muscle decreased in the rats fed either a carbohydrate meal or a fat meal. Feeding of either a carbohydrate meal or a fat meal stimulated incorporation of L-leucine-1-14C into protein fraction of skeletal muscle and reduced its oxidation to 14CO2. These results suggest that the metabolism of leucine is under nutritional regulation and that the decrease in content of free leucine in skeletal muscle might be caused by enhanced reutilization of leucine into protein by the feeding of a carbohydrate meal or a fat meal. The role of free leucine in skeletal muscle as a regulator of protein turnover in the tissue are discussed in relation to the metabolism of this branched chain amino acid. (auth.)

  10. Effects of Mixed Isoenergetic Meals on Fat and Carbohydrate Metabolism during Exercise in Older Men

    Directory of Open Access Journals (Sweden)

    Minoo Bassami

    2011-01-01

    Full Text Available The present study was designed to investigate the effects of four different meals on fat and CHO metabolism during subsequent exercise in elderly males. Eight healthy males (age: 63.3 ± 5.2 years reported to the physiology laboratory on four separate occasions, each of which was allocated for the performance of a 30-minute exercise on a cycle ergometer at 60% ̇VO2max after having normal (N, high fat (HF, high carbohydrate high glycaemic index (HGI and high carbohydrate low glycaemic index (LGI meals. Fat oxidation during exercise after the meals (HF=0.26±0.04 g/min; N=0.21±0.04 g/min; HGI=0.22±0.03 g/min; LGI=0.19±0.03 g/min was not significant (>.05, and neither were the rates of carbohydrate oxidation (N=1.79±0.28, HF=1.58±0.22, HGI=1.68±0.22, and LGI=1.77±0.21 g/m. NEFA concentration increased after HF (<.05 but decreased after HGI and LGI (<.05. Glucose concentration decreased as a result of exercise after HF, and LGI (<.05 whereas insulin concentration decreased significantly during exercise after N, HF, and HGI (<.05. It can be concluded that, in elderly males, feeding isoenergetic meals containing different proportions of carbohydrate and fat do not significantly alter oxidation of fat and CHO during exercise in spite of changes in some circulating metabolites.

  11. Determination of the spectrum of low molecular mass organic acids in urine by capillary electrophoresis with contactless conductivity and ultraviolet photometric detection-An efficient tool for monitoring of inborn metabolic disorders

    International Nuclear Information System (INIS)

    A mixture of 29 organic acids (OAs) occurring in urine was analyzed by capillary electrophoresis (CE) with capacitively coupled contactless conductivity detection (C4D) and UV photometric detection. The optimized analytical system involved a 100 cm long polyacrylamide-coated capillary (50 μm i.d.) and the background electrolyte of 20 mM 2-morpholinoethanesulfonic acid (MES)/NaOH + 10% (v/v) methanol, pH 6.0 (pH is related to the 20 mM MES/NaOH buffer in water). The LOD values obtained by C4D for the OAs which do not absorb UV radiation range from 0.6 μM (oxalic acid) to 6.8 μM (pyruvic acid); those obtained by UV photometry for the remaining OAs range from 2.9 μM (5-hydroxy-3-indoleacetic acid) to 10.2 μM (uric acid). The repeatability of the procedure developed is characterized by the coefficients of variation, which vary between 0.3% (tartaric acid) and 0.6% (5-hydroxy-3-indoleacetic acid) for the migration time and between 1.3% (tartaric acid) and 3.5% (lactic acid) for the peak area. The procedure permitted quantitation of 20 OAs in a real urine sample and was applied to monitoring of the occurrence of the inborn metabolic fault of methylmalonic aciduria.

  12. The Roles of Vitamin A in the Regulation of Carbohydrate, Lipid, and Protein Metabolism

    Directory of Open Access Journals (Sweden)

    Wei Chen

    2014-05-01

    Full Text Available Currently, two-thirds of American adults are overweight or obese. This high prevalence of overweight/obesity negatively affects the health of the population, as obese individuals tend to develop several chronic diseases, such as type 2 diabetes and cardiovascular diseases. Due to obesity’s impact on health, medical costs, and longevity, the rise in the number of obese people has become a public health concern. Both genetic and environmental/dietary factors play a role in the development of metabolic diseases. Intuitively, it seems to be obvious to link over-nutrition to the development of obesity and other metabolic diseases. However, the underlying mechanisms are still unclear. Dietary nutrients not only provide energy derived from macronutrients, but also factors such as micronutrients with regulatory roles. How micronutrients, such as vitamin A (VA; retinol, regulate macronutrient homeostasis is still an ongoing research topic. As an essential micronutrient, VA plays a key role in the general health of an individual. This review summarizes recent research progress regarding VA’s role in carbohydrate, lipid, and protein metabolism. Due to the large amount of information regarding VA functions, this review focusses on metabolism in metabolic active organs and tissues. Additionally, some perspectives for future studies will be provided.

  13. Fat and carbohydrate metabolism during exercise in late-onset Pompe disease

    DEFF Research Database (Denmark)

    Preisler, Nicolai; Laforet, Pascal; Madsen, Karen Lindhardt;

    2012-01-01

    Pompe disease is caused by absence of the lysosomal enzyme acid alpha-glucosidase. It is generally assumed that intra-lysosomal hydrolysis of glycogen does not contribute to skeletal muscle energy production during exercise. However, this hypothesis has never been tested in vivo during exercise. We...... examined the metabolic response to exercise in patients with late-onset Pompe disease, in order to determine if a defect in energy metabolism may play a role in the pathogenesis of Pompe disease. We studied six adult patients with Pompe disease and 10 healthy subjects. The participants underwent ischemic...... oxidation, between the patients and the healthy subjects. None of the subjects improved exercise tolerance by IV glucose infusion. In conclusion, peak oxidative capacity is reduced in Pompe disease. However, skeletal muscle fat and carbohydrate use during exercise was normal. The results indicate that a...

  14. [Carbohydrate and nitrogenous metabolism condition in the rat tissue under experimental rhabdomyolysis].

    Science.gov (United States)

    Kaliman, P A; Okhrimenko, S M

    2012-01-01

    Some effects of glycerol injection on indices of the condition of the thiol-disulfide system as well as carbohydrate and nitrogen metabolism in rats in vivo were studied. A decrease was revealed in levels of non-protein SH-groups in the liver, kidney and heart, as well as of protein SH-groups in the kidney and heart of rats following glycerol injection. That might be connected with SH-group oxidation under the excessive arrival of free haem into tissues under rhabdomyolysis. A decrease in glycogen and increase in tyrosine aminotransferase activity in the liver were observed. Activation of nitrogenous metabolism following glycerol injection is indicated by the increase of aminotransferase activity in organs, and concentration of blood urea. High concentration of creatinine in the rat serum can reflect malfiltration in kidneys. PMID:22679761

  15. Human hepatic carbohydrate metabolism. Dynamic observation using 13C MRS without proton decoupling

    International Nuclear Information System (INIS)

    Purpose: Dynamic natural-abundance 13C MR spectroscopy (MRS) studies without proton decoupling were performed in the human liver using commercial 1.5 T MR equipment. Material and methods: A single tuned custom-made circular surface coil with an OD of 20 cm operating at 16.04 MHz was used for the 13C study. Seventy-five grams of glucose dissolved in water was administered for the natural-abundance 13C-MRS dynamic study which lasted for approximately 40 to 60 min. Data acquisition was broken into 20-min and 1.7-min blocks. Localized proton shimming with a whole-body coil was performed with sufficient volume to include the observing area of the surface coil; the line width of the water signal was less than 20 Hz. Results and Conclusion: The glucose and glycogen spectra were clearly visible at 80 to 120 ppm after oral administration of the glucose solution. These data demonstrate that dynamic hepatic carbohydrate metabolism can be observed with commercially available MR equipment. Given that the human hepatic glycogen pool reaches maximum level within less than 10 min, this technique should provide a direct diagnosis of hepatic carbohydrate metabolic disorders. (orig.)

  16. Fifteen years experience: Egyptian metabolic lab

    Directory of Open Access Journals (Sweden)

    Ekram M. Fateen

    2014-10-01

    Conclusion: This study illustrates the experience of the reference metabolic lab in Egypt over 15 years. The lab began metabolic disorder screening by using simple diagnostic techniques like thin layer chromatography and colored tests in urine which by time updated and upgraded the methods to diagnose a wide range of disorders. This study shows the most common diagnosed inherited inborn errors of metabolism among the Egyptian population.

  17. Low-carbohydrate diets cause obesity, low-carbohydrate diets reverse obesity: a metabolic mechanism resolving the paradox

    OpenAIRE

    Mobbs, Charles V.; Mastaitis, Jason; Yen, Kelvin; Schwartz, Joseph; Mohan, Vinuta; Poplawski, Michal; Isoda, Fumiko

    2006-01-01

    High-fat diets produce obesity in part because, per calorie, glucose produces greater post-prandial thermogenesis than lipids, an effect probably mediated by glucose-sensing neurons. A very low carbohydrate/high-fat/high-protein Atkins-type diet produces obesity but is marginally ketogenic in mice. In contrast, high-sucrose/low-fat diets, and very low carbohydrate/high-fat/low-protein (anti-epileptic) ketogenic diets reverse diet-induced obesity independent of caloric intake. We propose that ...

  18. High-carbohydrate, high-fat diet-induced metabolic syndrome and cardiovascular remodeling in rats.

    Science.gov (United States)

    Panchal, Sunil K; Poudyal, Hemant; Iyer, Abishek; Nazer, Reeza; Alam, Md Ashraful; Diwan, Vishal; Kauter, Kathleen; Sernia, Conrad; Campbell, Fiona; Ward, Leigh; Gobe, Glenda; Fenning, Andrew; Brown, Lindsay

    2011-05-01

    The prevalence of metabolic syndrome including central obesity, insulin resistance, impaired glucose tolerance, hypertension, and dyslipidemia is increasing. Development of adequate therapy for metabolic syndrome requires an animal model that mimics the human disease state. Therefore, we have characterized the metabolic, cardiovascular, hepatic, renal, and pancreatic changes in male Wistar rats (8-9 weeks old) fed on a high-carbohydrate, high-fat diet including condensed milk (39.5%), beef tallow (20%), and fructose (17.5%) together with 25% fructose in drinking water; control rats were fed a cornstarch diet. During 16 weeks on this diet, rats showed progressive increases in body weight, energy intake, abdominal fat deposition, and abdominal circumference along with impaired glucose tolerance, dyslipidemia, hyperinsulinemia, and increased plasma leptin and malondialdehyde concentrations. Cardiovascular signs included increased systolic blood pressure and endothelial dysfunction together with inflammation, fibrosis, hypertrophy, increased stiffness, and delayed repolarization in the left ventricle of the heart. The liver showed increased wet weight, fat deposition, inflammation, and fibrosis with increased plasma activity of liver enzymes. The kidneys showed inflammation and fibrosis, whereas the pancreas showed increased islet size. In comparison with other models of diabetes and obesity, this diet-induced model more closely mimics the changes observed in human metabolic syndrome. PMID:21572266

  19. High-carbohydrate high-fat diet–induced metabolic syndrome and cardiovascular remodeling in rats.

    Science.gov (United States)

    Panchal, Sunil K; Poudyal, Hemant; Iyer, Abishek; Nazer, Reeza; Alam, Ashraful; Diwan, Vishal; Kauter, Kathleen; Sernia, Conrad; Campbell, Fiona; Ward, Leigh; Gobe, Glenda; Fenning, Andrew; Brown, Lindsay

    2011-01-01

    The prevalence of metabolic syndrome including central obesity, insulin resistance, impaired glucose tolerance, hypertension, and dyslipidemia is increasing. Development of adequate therapy for metabolic syndrome requires an animal model that mimics the human disease state. Therefore, we have characterized the metabolic, cardiovascular, hepatic, renal, and pancreatic changes in male Wistar rats (8-9 weeks old) fed on a high-carbohydrate, high-fat diet including condensed milk (39.5%), beef tallow (20%), and fructose (17.5%) together with 25% fructose in drinking water; control rats were fed a cornstarch diet. During 16 weeks on this diet, rats showed progressive increases in body weight, energy intake, abdominal fat deposition, and abdominal circumference along with impaired glucose tolerance, dyslipidemia, hyperinsulinemia, and increased plasma leptin and malondialdehyde concentrations. Cardiovascular signs included increased systolic blood pressure and endothelial dysfunction together with inflammation, fibrosis, hypertrophy, increased stiffness, and delayed repolarization in the left ventricle of the heart. The liver showed increased wet weight, fat deposition, inflammation, and fibrosis with increased plasma activity of liver enzymes. The kidneys showed inflammation and fibrosis, whereas the pancreas showed increased islet size. In comparison with other models of diabetes and obesity, this diet-induced model more closely mimics the changes observed in human metabolic syndrome. PMID:20966763

  20. A model for carbohydrate metabolism in the diatom Phaeodactylum tricornutum deduced from comparative whole genome analysis.

    Directory of Open Access Journals (Sweden)

    Peter G Kroth

    Full Text Available BACKGROUND: Diatoms are unicellular algae responsible for approximately 20% of global carbon fixation. Their evolution by secondary endocytobiosis resulted in a complex cellular structure and metabolism compared to algae with primary plastids. METHODOLOGY/PRINCIPAL FINDINGS: The whole genome sequence of the diatom Phaeodactylum tricornutum has recently been completed. We identified and annotated genes for enzymes involved in carbohydrate pathways based on extensive EST support and comparison to the whole genome sequence of a second diatom, Thalassiosira pseudonana. Protein localization to mitochondria was predicted based on identified similarities to mitochondrial localization motifs in other eukaryotes, whereas protein localization to plastids was based on the presence of signal peptide motifs in combination with plastid localization motifs previously shown to be required in diatoms. We identified genes potentially involved in a C4-like photosynthesis in P. tricornutum and, on the basis of sequence-based putative localization of relevant proteins, discuss possible differences in carbon concentrating mechanisms and CO(2 fixation between the two diatoms. We also identified genes encoding enzymes involved in photorespiration with one interesting exception: glycerate kinase was not found in either P. tricornutum or T. pseudonana. Various Calvin cycle enzymes were found in up to five different isoforms, distributed between plastids, mitochondria and the cytosol. Diatoms store energy either as lipids or as chrysolaminaran (a beta-1,3-glucan outside of the plastids. We identified various beta-glucanases and large membrane-bound glucan synthases. Interestingly most of the glucanases appear to contain C-terminal anchor domains that may attach the enzymes to membranes. CONCLUSIONS/SIGNIFICANCE: Here we present a detailed synthesis of carbohydrate metabolism in diatoms based on the genome sequences of Thalassiosira pseudonana and Phaeodactylum tricornutum

  1. TPhP exposure disturbs carbohydrate metabolism, lipid metabolism, and the DNA damage repair system in zebrafish liver

    Science.gov (United States)

    Du, Zhongkun; Zhang, Yan; Wang, Guowei; Peng, Jianbiao; Wang, Zunyao; Gao, Shixiang

    2016-02-01

    Triphenyl phosphate is a high production volume organophosphate flame retardant that has been detected in multiple environmental media at increasing concentrations. The environmental and health risks of triphenyl phosphate have drawn attention because of the multiplex toxicity of this chemical compound. However, few studies have paid close attention to the impacts of triphenyl phosphate on liver metabolism. We investigated hepatic histopathological, metabolomic and transcriptomic responses of zebrafish after exposure to 0.050 mg/L and 0.300 mg/L triphenyl phosphate for 7 days. Metabolomic analysis revealed significant changes in the contents of glucose, UDP-glucose, lactate, succinate, fumarate, choline, acetylcarnitine, and several fatty acids. Transcriptomic analysis revealed that related pathways, such as the glycosphingolipid biosynthesis, PPAR signaling pathway and fatty acid elongation, were significantly affected. These results suggest that triphenyl phosphate exposure markedly disturbs hepatic carbohydrate and lipid metabolism in zebrafish. Moreover, DNA replication, the cell cycle, and non-homologous end-joining and base excision repair were strongly affected, thus indicating that triphenyl phosphate hinders the DNA damage repair system in zebrafish liver cells. The present study provides a systematic analysis of the triphenyl phosphate-induced toxic effects in zebrafish liver and demonstrates that low concentrations of triphenyl phosphate affect normal metabolism and cell cycle.

  2. A mini review of dolphin carbohydrate metabolism and suggestions for future research using exhaled air

    Directory of Open Access Journals (Sweden)

    Sam eRidgway

    2013-12-01

    Full Text Available In the 1960s, I explored some aspects of carbohydrate metabolism in healthy bottlenose dolphins (Tursiops truncatus. Their physiological picture resembled what had been described for hyperthyroid diabetics. Dolphins have elevated thyroid hormone turnover, and fasting dolphins maintain a relatively high level of plasma glucose. After dolphins ingest glucose, plasma levels remain high for many hours. Interestingly, plasma glucose must exceed 300 mg/dL (about twice as high as the human threshold before glucose appears in urine. Due to their diabetes-like states, trainability, and unique natural respiratory anatomy and physiology, dolphins may offer useful clues to metabolites in the breath that may be used to non-invasively monitor diabetes in humans. Dolphins take very rapid and deep breaths that are four or five times as deep as humans and other terrestrial mammals, making them ideal for physiological assessment using non-invasive exhaled air. Avenues for successfully identifying breath-based markers for metabolic disease and physiology in dolphins can be done with both modern technology and the evolutionarily advantageous canine nose. This review summarizes aspects of dolphin metabolism previously learned and offers new directions for diabetes research that may benefit both dolphin and human health.

  3. Regulation of glucose and lipid metabolism by dietary carbohydrate levels and lipid sources in gilthead sea bream juveniles.

    Science.gov (United States)

    Castro, Carolina; Corraze, Geneviève; Firmino-Diógenes, Alexandre; Larroquet, Laurence; Panserat, Stéphane; Oliva-Teles, Aires

    2016-07-01

    The long-term effects on growth performance, body composition, plasma metabolites, liver and intestine glucose and lipid metabolism were assessed in gilthead sea bream juveniles fed diets without carbohydrates (CH-) or carbohydrate-enriched (20 % gelatinised starch, CH+) combined with two lipid sources (fish oil; or vegetable oil (VO)). No differences in growth performance among treatments were observed. Carbohydrate intake was associated with increased hepatic transcripts of glucokinase but not of 6-phosphofructokinase. Expression of phosphoenolpyruvate carboxykinase was down-regulated by carbohydrate intake, whereas, unexpectedly, glucose 6-phosphatase was up-regulated. Lipogenic enzyme activities (glucose-6-phosphate dehydrogenase, malic enzyme, fatty acid synthase) and ∆6 fatty acyl desaturase (FADS2) transcripts were increased in liver of fish fed CH+ diets, supporting an enhanced potential for lipogenesis and long-chain PUFA (LC-PUFA) biosynthesis. Despite the lower hepatic cholesterol content in CH+ groups, no influence on the expression of genes related to cholesterol efflux (ATP-binding cassette G5) and biosynthesis (lanosterol 14 α-demethylase, cytochrome P450 51 cytochrome P450 51 (CYP51A1); 7-dehydrocholesterol reductase) was recorded at the hepatic level. At the intestinal level, however, induction of CYP51A1 transcripts by carbohydrate intake was recorded. Dietary VO led to decreased plasma phospholipid and cholesterol concentrations but not on the transcripts of proteins involved in phospholipid biosynthesis (glycerol-3-phosphate acyltransferase) and cholesterol metabolism at intestinal and hepatic levels. Hepatic and muscular fatty acid profiles reflected that of diets, despite the up-regulation of FADS2 transcripts. Overall, this study demonstrated that dietary carbohydrates mainly affected carbohydrate metabolism, lipogenesis and LC-PUFA biosynthesis, whereas effects of dietary lipid source were mostly related with tissue fatty acid composition

  4. Cystinuria: an inborn cause of urolithiasis

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    Eggermann Thomas

    2012-04-01

    Full Text Available Abstract Cystinuria (OMIM 220100 is an inborn congenital disorder characterised by a defective cystine metabolism resulting in the formation of cystine stones. Among the heterogeneous group of kidney stone diseases, cystinuria is the only disorder which is exclusively caused by gene mutations. So far, two genes responsible for cystinuria have been identified: SLC3A1 (chromosome 2p21 encodes the heavy subunit rBAT of a renal b0,+ transporter while SLC7A9 (chromosome 19q12 encodes its interacting light subunit b0,+AT. Mutations in SLC3A1 are generally associated with an autosomal-recessive mode of inheritance whereas SLC7A9 variants result in a broad clinical variability even within the same family. The detection rate for mutations in these genes is larger than 85%, but it is influenced by the ethnic origin of a patient and the pathophysiological significance of the mutations. In addition to isolated cystinuria, patients suffering from the hypotonia-cystinuria syndrome have been reported carrying deletions including at least the SLC3A1 and the PREPL genes in 2p21. By extensive molecular screening studies in large cohort of patients a broad spectrum of mutations could be identified, several of these variants were functionally analysed and thereby allowed insights in the pathology of the disease as well as in the renal trafficking of cystine and the dibasic amino acids. In our review we will summarize the current knowledge on the physiological and the genetic basis of cystinuria as an inborn cause of kidney stones, and the application of this knowledge in genetic testing strategies.

  5. Exogenous Classic Phytohormones Have Limited Regulatory Effects on Fructan and Primary Carbohydrate Metabolism in Perennial Ryegrass (Lolium perenne L.)

    OpenAIRE

    Gasperl, Anna; Morvan-Bertrand, Annette; Prud'homme, Marie-Pascale; van der Graaff, Eric; Roitsch, Thomas

    2016-01-01

    Fructans are polymers of fructose and one of the main constituents of water-soluble carbohydrates in forage grasses and cereal crops of temperate climates. Fructans are involved in cold and drought resistance, regrowth following defoliation and early spring growth, seed filling, have beneficial effects on human health and are used for industrial processes. Perennial ryegrass (Lolium perenne L.) serves as model species to study fructan metabolism. Fructan metabolism is under the control of bot...

  6. Status of carbohydrate and lipid metabolism in obese patients with type 2 diabetes mellitus after biliopancreatic diversion surgery

    Directory of Open Access Journals (Sweden)

    E V Ershova

    2013-10-01

    Full Text Available We examined 70 patients with obesity and diabetes mellitus type 2 before and within 5 years after BPD: these patients showed a significant improvement in the status of carbohydrate and lipid metabolism within 3 months after surgery. This improvement has remained stable along with the reduced body weight during the whole observation period of up to 5 years.

  7. Partial restoration of dietary fat induced metabolic adaptations to training by 7 days of carbohydrate diet

    DEFF Research Database (Denmark)

    Helge, Jørn Wulff; Watt, Peter W; Richter, Erik A;

    2002-01-01

    -fat (Fat-CHO; 62% fat, 21% carbohydrate) and 6 a high-carbohydrate diet (CHO; 20% fat, 65% carbohydrate) for 7 wk, and thereafter both groups consumed the carbohydrate diet for an eighth week. Training was performed throughout. After 8 wk, during 60 min of exercise (71 +/- 1% pretraining maximal oxygen...... +/- 59 vs. 688 +/- 43 mmol/kg dry wt) in Fat-CHO than in CHO. In conclusion, shift to carbohydrate diet after prolonged adaptation to fat diet and training causes increased resting muscle glycogen levels but impaired leg glucose uptake and similar muscle glycogen breakdown, despite higher resting levels......, compared with when the carbohydrate diet is consumed throughout training....

  8. Carbohydrate Analysis

    Science.gov (United States)

    Bemiller, James N.

    Carbohydrates are important in foods as a major source of energy, to impart crucial textural properties, and as dietary fiber which influences physiological processes. Digestible carbohydrates, which are converted into monosaccharides, which are absorbed, provide metabolic energy. Worldwide, carbohydrates account for more than 70% of the caloric value of the human diet. It is recommended that all persons should limit calories from fat (the other significant source) to not more than 30% and that most of the carbohydrate calories should come from starch. Nondigestible polysaccharides (all those other than starch) comprise the major portion of dietary fiber (Sect. 10.5). Carbohydrates also contribute other attributes, including bulk, body, viscosity, stability to emulsions and foams, water-holding capacity, freeze-thaw stability, browning, flavors, aromas, and a range of desirable textures (from crispness to smooth, soft gels). They also provide satiety. Basic carbohydrate structures, chemistry, and terminology can be found in references (1, 2).

  9. Ruminal protein metabolism and intestinal amino acid utilization as affected by dietary protein and carbohydrate sources in sheep.

    Science.gov (United States)

    Hussein, H S; Jordan, R M; Stern, M D

    1991-05-01

    Eight wether lambs fitted with ruminal, duodenal, and ileal cannulas were used in a replicated 4 x 4 Latin square design to study the effects of carbohydrate and protein sources on ruminal protein metabolism and carbohydrate fermentation and intestinal amino acid (AA) absorption. Treatments were arranged as a 2 x 2 factorial. Carbohydrate sources were corn and barley; protein sources were soybean meal (SBM) and fish meal (FM). Diets contained 15.5% CP, of which 40% was supplied by SBM or FM. Corn or barley provided 39% of dietary DM that contained equal amounts of grass hay and wheat straw. Fish meal diets produced a lower (P less than .05) ruminal NH3 concentration and resulted in less CP degradation and bacterial protein flow to the duodenum than did SBM diets. Replacing SBM with FM increased (P less than .05) ruminal digestion of all fiber fractions. In addition, cellulose and hemicellulose digestibilities in the rumen tended to increase (P greater than .05) when barley replaced corn in the FM diets. Carbohydrate x protein interactions (P less than .05) were observed for OM digestion in the rumen and AA absorption in the small intestine (percentage of AA entering); these interactions were highest for the barley-FM diet. These results suggest that feeding FM with barley, which is high in both degradable carbohydrate and protein, might benefit ruminants more than feeding FM with corn, which is high in degradable carbohydrate but relatively low in degradable protein. PMID:1648551

  10. The effects of space flight on some rat liver enzymes regulating carbohydrate and lipid metabolism

    Science.gov (United States)

    Abraham, S.; Lin, C. Y.; Klein, H. P.; Volkmann, C.

    1981-01-01

    The effects of space flight conditions on the activities of certain enzymes regulating carbohydrate and lipid metabolism in rat liver are investigated in an attempt to account for the losses in body weight observed during space flight despite preflight caloric consumption. Liver samples were analyzed for the activities of 32 cytosolic and microsomal enzymes as well as hepatic glycogen and individual fatty acid levels for ground control rats and rats flown on board the Cosmos 936 biosatellite under normal space flight conditions and in centrifuges which were sacrificed upon recovery or 25 days after recovery. Significant decreases in the activities of glycogen phosphorylase, alpha-glycerol phosphate acyl transferase, diglyceride acyl transferase, aconitase and 6-phosphogluconate dehydrogenase and an increase in palmitoyl CoA desaturase are found in the flight stationary relative to the flight contrifuged rats upon recovery, with all enzymes showing alterations returning to normal values 25 days postflight. The flight stationary group is also observed to be characterized by more than twice the amount of liver glycogen of the flight centrifuged group as well as a significant increase in the ratio of palmitic to palmitoleic acid. Results thus indicate metabolic changes which may be involved in the mechanism of weight loss during weightlessness, and demonstrate the equivalence of centrifugation during space flight to terrestrial gravity.

  11. Disturbances in carbohydrate metabolism in radiation sickness and its repair under the effect of therapeutic preparations

    International Nuclear Information System (INIS)

    The effect of taurin (200mg/kg) in combination with insulin (0.2 IU/kg) on the repair of hormonal activity and of carbohydrate metabolism in an experimentally released radiation sickness was examined. White rats of both sexes weighting 180-200 g were irradiated with a gamma-unit GUM-Co-50 with 700 rad, that corresponds to LDsub(70/30). The preparations were simultaneously administered intraperitoneally every other day altogether 8 times from the 5th day after irradiation. Survival rate in the groups of treated animals was by about 27% higher than in the control. With the administration of therapeutic preparations a repair of the insulin-like plasma activity to the normal levels and a considerable inhibition of liver phosphorylase activity could be observed. Different from insulin action alone a combined use of insulin and taurin led to decrease in blood level of 11-oxycorticosteroids the metabolism of which being essentially impaired by irradiation to the normal value. The restoration of correlation between hormonal activity of adrenal cortex and of the insular apparatus favoured glycogen reproduction in the liver and the decrease in blood-sugar level. Experiments with intact animals as well as in vitro experiments reveal that taurin acts insulin-like

  12. Iminosugar inhibitors of carbohydrate-active enzymes that underpin cereal grain germination and endosperm metabolism.

    Science.gov (United States)

    Andriotis, Vasilios M E; Rejzek, Martin; Rugen, Michael D; Svensson, Birte; Smith, Alison M; Field, Robert A

    2016-02-15

    Starch is a major energy store in plants. It provides most of the calories in the human diet and, as a bulk commodity, it is used across broad industry sectors. Starch synthesis and degradation are not fully understood, owing to challenging biochemistry at the liquid/solid interface and relatively limited knowledge about the nature and control of starch degradation in plants. Increased societal and commercial demand for enhanced yield and quality in starch crops requires a better understanding of starch metabolism as a whole. Here we review recent advances in understanding the roles of carbohydrate-active enzymes in starch degradation in cereal grains through complementary chemical and molecular genetics. These approaches have allowed us to start dissecting aspects of starch degradation and the interplay with cell-wall polysaccharide hydrolysis during germination. With a view to improving and diversifying the properties and uses of cereal grains, it is possible that starch degradation may be amenable to manipulation through genetic or chemical intervention at the level of cell wall metabolism, rather than simply in the starch degradation pathway per se. PMID:26862201

  13. The nutritional and metabolic effects of a carbohydrate-rich diet in a glycogen supercompensation training regimen.

    Science.gov (United States)

    Jetté, M; Pelletier, O; Parker, L; Thoden, J

    1978-12-01

    It has been suggested that glycogen depletion followed by a protein-fat diet and a carbohydrate-rich diet improves performance. This study was designed to determine the nutritional and metabolic effects of a carbohydrate-rich diet in a glycogen supercompensation training regimen. Four male subjects participated in a 5-week protocol of which the first 3 weeks were devoted to a control period and the last 2 weeks to the experimental phase of the study. The variables measured before, during, and following the experimental phase included anthropometric and basal metabolic rate measurements, urinary and serum analysis for vitamins, SMA 12/60 blood profile and aerobic performance (VO2max). Results indicated an appreciable modification of the metabolic and nutritional profile of the subjects as a result of the diets. During the protein-fat diet there was a decrease in serum glucose and resting respiratory quotient and an increase in cholesterol, blood urea nitrogen, riboflavin, and N1-methylnicotinamide excretion. Subsequent to the carbohydrate-rich diet there was an increase in triglycerides and vitamin C, riboflavin, and thiamin excretion while there was a decrease in serum blood urea nitrogen, and N1-methylnicotinamide excretion. Aerobic performance was slightly decreased and the mean postexercise lactate levels were slightly higher after the carbohydrate-rich diet. It was hypothesized that the reduced niacin intake during the carohydrate-rich diet may hamper the aerobic oxidative pathways. PMID:727161

  14. Carbohydrate metabolism in germinating caryopses of Oryza sativa L. exposed to prolonged anoxia.

    Science.gov (United States)

    Pompeiano, Antonio; Guglielminetti, Lorenzo

    2016-09-01

    Anoxia tolerance can be evaluated not only in terms of growth or survival of plant organs during oxygen deprivation, but also in relation to carbohydrate utilization in the context of a well-modulated fermentative metabolism. Rice (Oryza spp.) is unique among cereals, in that it has the distinctive ability to germinate under complete anaerobiosis by using the starchy reserves in its seeds to fuel the anaerobic metabolism. The aim of the present study was to evaluate the ability of germinating rice seedlings to survive a long-term oxygen deficiency [40 days after sowing (DAS)] and the effects on sugar metabolism, focusing on starch degradation as well as soluble sugars transport and storage under anoxia. No significant decline in vitality occurred until 30 DAS though no recovery was detected following longer anoxic treatments. Growth arrest was observed following anoxic treatments longer that 20 DAS, in concomitance with considerably lower ethanol production. Amylolytic activity in embryos and endosperms had similar responses to anoxia, reaching maximum content 30 days after the onset of stress, following which the levels declined for the remainder of the experiment. Under anoxia, average amylolytic activity was twofold higher in embryos than endosperms. Efficient starch degradation was observed in rice under anoxia at the onset of the treatment but it decreased over time and did not lead to a complete depletion. Our analysis of α-amylase activity did not support the hypothesis that starch degradation plays a critical role in explaining differences in vitality and coleoptile growth under prolonged oxygen deprivation. PMID:27289587

  15. Abnormalities in carbohydrate and lipid metabolisms in high-fructose dietfed insulin-resistant rats: amelioration by Catharanthus roseus treatments.

    Science.gov (United States)

    Rasineni, Karuna; Bellamkonda, Ramesh; Singareddy, Sreenivasa Reddy; Desireddy, Saralakumari

    2013-09-01

    High intake of dietary fructose has been shown to exert a number of adverse metabolic effects in humans and experimental animals. The present study was proposed to elucidate the effect of Catharanthus roseus (C. roseus) leaf powder treatment on alterations in carbohydrate and lipid metabolisms in rats fed with high-fructose diet. Male Wistar rats of body weight around 180 g were divided into four groups, two of these groups (groups C and C+CR) were fed with standard pellet diet and the other two groups (groups F and F+CR) were fed with high-fructose (66 %) diet. C. roseus leaf powder suspension in water (100 mg/kg body weight/day) was administered orally to group C+CR and group F+CR. At the end of a 60-day experimental period, biochemical parameters related to carbohydrate and lipid metabolisms were assayed. C. roseus treatment completely prevented the fructose-induced increased body weight, hyperglycemia, and hypertriglyceridemia. Hyperinsulinemia and insulin resistance observed in group F was significantly decreased with C. roseus treatment in group F+CR. The alterations observed in the activities of enzymes of carbohydrate and lipid metabolisms and contents of hepatic tissue lipids in group F rats were significantly restored to near normal values by C. roseus treatment in group F+CR. In conclusion, our study demonstrates that C. roseus treatment is effective in preventing fructose-induced insulin resistance and hypertriglyceridemia while attenuating the fructose-induced alterations in carbohydrate and lipid metabolisms. This study suggests that the plant can be used as an adjuvant for the prevention and/or management of insulin resistance and disorders related to it. PMID:23334857

  16. Influence of dietary carbohydrate on the metabolism of juvenile Litopenaeus stylirostris.

    Science.gov (United States)

    Rosas; Cuzon; Gaxiola; Arena; Lemaire; Soyez; Van Wormhoudt A

    2000-06-28

    The effect of dietary carbohydrates (CBH) on glucose and glycogen, digestive enzymes, ammonia excretion and osmotic pressure and osmotic capacity of Litopenaeus stylirostris juveniles was studied. The increase of CBH, ranging between 1 and 33%, stimulates activities of alpha-amylase and alpha-glucosidase in the hepatopancreas. High levels of glucose in hemolymph and of glycogen in the hepatopancreas were reached at the highest level of dietary CBH; however, the kinetics of accumulation is different. Shrimps fed with low level of CBH needed 3 h to reached glucose peak, whereas only 1 h is necessary for high CBH levels. A saturation curve was observed in glycogen level and alpha-amylase activity with maximum values in shrimp-fed diets containing 21% CBH. This level could be used to be included as a maximum shrimp dietary CBH level. Pre-prandial glycogen levels were observed in shrimp fed a diet containing 1% CBH, indicating an important gluconeogenesis, which affected the protein metabolism. The present results show that a diet containing 10% CBH may not be enough to cover the CBH requirement, which could be satisfied by dietary protein content. The low osmotic capacity observed in shrimp fed on a diet containing 10% CBH coincided with a relatively low post-prandial nitrogen excretion which reflects a low concentration of amino acids circulating in hemolymph, which affected the osmotic pressure and the osmotic capacity. These results reflect the high plasticity of shrimp species to use protein to obtain metabolic energy from food and its limited capacity for processing dietary CBH. PMID:10841934

  17. Linked gene networks involved in nitrogen and carbon metabolism and levels of water-soluble carbohydrate accumulation in wheat stems.

    Science.gov (United States)

    McIntyre, C Lynne; Casu, Rosanne E; Rattey, Allan; Dreccer, M Fernanda; Kam, Jason W; van Herwaarden, Anthony F; Shorter, Ray; Xue, Gang Ping

    2011-12-01

    High levels of water-soluble carbohydrates (WSC) provide an important source of stored assimilate for grain filling in wheat. To better understand the interaction between carbohydrate metabolism and other metabolic processes associated with the WSC trait, a genome-wide expression analysis was performed using eight field-grown lines from the high and low phenotypic tails of a wheat population segregating for WSC and the Affymetrix wheat genome array. The 259 differentially expressed probe sets could be assigned to 26 functional category bins, as defined using MapMan software. There were major differences in the categories to which the differentially expressed probe sets were assigned; for example, probe sets upregulated in high relative to low WSC lines were assigned to category bins such as amino acid metabolism, protein degradation and transport and to be involved in starch synthesis-related processes (carbohydrate metabolism bin), whereas downregulated probe sets were assigned to cell wall-related bins, amino acid synthesis and stress and were involved in sucrose breakdown. Using the set of differentially expressed genes as input, chemical-protein network analyses demonstrated a linkage between starch and N metabolism via pyridoxal phosphate. Twelve C and N metabolism-related genes were selected for analysis of their expression response to varying N and water treatments in the field in the four high and four low WSC progeny lines; the two nitrogen/amino acid metabolism genes demonstrated a consistent negative association between their level of expression and level of WSC. Our results suggest that the assimilation of nitrogen into amino acids is an important factor that influences the levels of WSC in the stems of field-grown wheat. PMID:21789636

  18. Efficacy of dietary supplementation with botanicals on carbohydrate metabolism in humans.

    Science.gov (United States)

    Cefalu, William T; Ye, Jianping; Wang, Zhong Q

    2008-06-01

    Botanical products are widely used in nutritional supplementation for promotion of health or prevention of diseases. With the high prevalence of obesity and type 2 diabetes, abnormalities in carbohydrate metabolism are common in the general population and obtaining glycemic control is important in reducing the complications of diabetes. If shown to be effective, botanical products have a unique position in potentially aiding the general public in regard to obesity and diabetes. They can be obtained "over-the-counter" and may have less side effects compared to many synthetic drugs. Although most of the popular botanicals have a long history in folk medicine, there is paucity of data regarding their efficacy and safety, particularly as it relates to human studies. In this review, we discuss the data that was available in the literature for nine botanicals that are frequently promoted to help manage blood glucose. They are Bitter Melon (Momordica charantia), Fenugreek (trigonella foenum graecum), Gymnema Sylvestre, Ivy Gourd (Coccinia indica), Nopal or Prickly Pear Cactus (Opuntia streptacantha), Ginseng, Aloe Vera, Russian Tarragon (Artemisia dracunculus), and Garlic (Allium sativum). The discussion is emphasized on the clinical aspect of these botanicals. Due to the lack of sufficient evidence from clinical studies for any of the botanicals reviewed, it is premature to actively recommend use of any particular herb to treat either glucose or other risk factors. Thus, well defined randomized clinical trials are warranted in this area. PMID:18537692

  19. Cardiovascular Risks Factors and their Relationship with Disorders of Carbohydrate and Lipid Metabolism

    Directory of Open Access Journals (Sweden)

    Lilian Leguen Gulgar

    2014-12-01

    Full Text Available Background: cardiovascular disease has been the leading cause of death in Cuba, where studies on emerging cardiovascular risk factors as predictors of cardiovascular risk are scarce. Objective: to determine the association between cardiovascular risk factors and disorders of carbohydrate and lipid metabolism. Methods: a correlational study was conducted with a sample of 105 men and women selected from a total of 346 workers of the University of Medical Sciences of Cienfuegos from June 2011 through July 2012. The variables analyzed were age, sex, blood pressure, waist circumference, tobacco use, fasting blood glucose, triglycerides, cholesterol, HDL cholesterol, apolipoprotein A and B, TC/HDL ratio and apo B/apo AI ratio. Results: women older than 45 years had a higher prevalence of elevated waist circumference (60.0 %, hypertension (46.7 % and type 2 diabetes mellitus (54.3 % with hypertriglyceridemia (43.3 %, low HDLc levels (36.7 % and were 2.8 times more likely to develop elevated waist circumference; 66.7 % of the diabetic patients had low HDLc levels, 33.3 % developed hypertriglyceridemia and 66.7 % had a high total cholesterol/HDL cholesterol ratio. Conclusions: an association between age older than 45 years, female sex, obesity, hypertension and type 2 diabetes mellitus was observed. There was a higher prevalence of hypertriglyceridemia and low HDL cholesterol levels in obese and diabetic patients. Increased risk of low HDL cholesterol and high total cholesterol / HDL cholesterol ratio were also found.

  20. Role of Leu-enkephalin in the regulation of carbohydrate metabolism

    Energy Technology Data Exchange (ETDEWEB)

    Zoloev, G.K.

    1987-10-01

    The aim of this investigation was to study the possible role of Leuenkephalin (LE) is the regulation of carbohydrate metabolism. Experiments were carried out on 166 mole albino rats weighing 180-220 g. Opioid peptides, namely LE, D-Ala/sup 2/-Leu/sup 5/-Arg/sup 6/-enkephalin, and d-Ala/sup 2/-D-Leu/sup 5/-D-Arg/sup 6/-enkephalin were injected intraperitoneally in a dose of 500 ..mu..g/kg, naloxone, a blocker of opiate receptors, was injected in a dose of 100 ..mu..g/kg, and the pharmacopoeial preparations Parathyroidin in a dose of 10 U/kg and adrenalin hydrochloride in a dose of 500 ..mu..g/kg. Animals of the control group were given injections of 0.2 ml of physiological saline. The rats were decapitated under superficial ether anesthesia 1 h after injection of the drugs. Insulin levels were determined by radioimmunoassay. Radioactivity was counted on a gamma-spectrometer. The glycogen concentration in the samples was determined spectrophotometrically and the cAMP concentration by radioimmunoassay. Radioactivity was counted on a Mark III scintillation counter.

  1. Role of Leu-enkephalin in the regulation of carbohydrate metabolism

    International Nuclear Information System (INIS)

    The aim of this investigation was to study the possible role of Leuenkephalin (LE) is the regulation of carbohydrate metabolism. Experiments were carried out on 166 mole albino rats weighing 180-220 g. Opioid peptides, namely LE, D-Ala2-Leu5-Arg6-enkephalin, and d-Ala2-D-Leu5-D-Arg6-enkephalin were injected intraperitoneally in a dose of 500 μg/kg, naloxone, a blocker of opiate receptors, was injected in a dose of 100 μg/kg, and the pharmacopoeial preparations Parathyroidin in a dose of 10 U/kg and adrenalin hydrochloride in a dose of 500 μg/kg. Animals of the control group were given injections of 0.2 ml of physiological saline. The rats were decapitated under superficial ether anesthesia 1 h after injection of the drugs. Insulin levels were determined by radioimmunoassay. Radioactivity was counted on a gamma-spectrometer. The glycogen concentration in the samples was determined spectrophotometrically and the cAMP concentration by radioimmunoassay. Radioactivity was counted on a Mark III scintillation counter

  2. Postprandial de novo lipogenesis and metabolic changes induced by a high-carbohydrate, low-fat meal in lean and overweight men

    OpenAIRE

    I. Marques-Lopes; D. Ansorena; Astiasaran, I. (Iciar); L. Forga; J. A. Martinez

    2001-01-01

    BACKGROUND: Adjustments of carbohydrate intake and oxidation occur in both normal-weight and overweight individuals. Nevertheless, the contribution of carbohydrates to the accumulation of fat through either reduction of fat oxidation or stimulation of fat synthesis in obesity remains poorly investigated. OBJECTIVE: The objective of this study was to assess the postprandial metabolic changes and the fractional hepatic de novo lipogenesis (DNL) induced by a high-carbohydrate, low-fat...

  3. Cerebellar involvement in metabolic disorders: a pattern-recognition approach

    International Nuclear Information System (INIS)

    Inborn errors of metabolism can affect the cerebellum during development, maturation and later during life. We have established criteria for pattern recognition of cerebellar abnormalities in metabolic disorders. The abnormalities can be divided into four major groups: cerebellar hypoplasia (CH), hyperplasia, cerebellar atrophy (CA), cerebellar white matter abnormalities (WMA) or swelling, and involvement of the dentate nuclei (DN) or cerebellar cortex. CH can be an isolated typical finding, as in adenylsuccinase deficiency, but is also occasionally seen in many other disorders. Differentiation from CH and CA is often difficult, as in carbohydrate deficient glycoprotein syndrome or 2-l-hydroxyglutaric acidaemia. In cases of atrophy the relationship of cerebellar to cerebral atrophy is important. WMA may be diffuse or patchy, frequently predominantly around the DN. Severe swelling of white matter is present during metabolic crisis in maple syrup urine disease. The DN can be affected by metabolite deposition, necrosis, calcification or demyelination. Involvement of cerebellar cortex is seen in infantile neuroaxonal dystrophy. Changes in DN and cerebellar cortex are rather typical and therefore most helpful; additional features should be sought as they are useful in narrowing down the differential diagnosis. (orig.)

  4. Alcoholic Stem Extract of Coscinium fenestratum Regulates Carbohydrate Metabolism and Improves Antioxidant Status in Streptozotocin–Nicotinamide Induced Diabetic Rats

    OpenAIRE

    Punitha, I. R.; Rajendran, K.; Arun Shirwaikar; Annie Shirwaikar

    2005-01-01

    Alcoholic extract of the stems of Coscinium fenestratum, a medicinal plant indigenous to India and Sri Lanka used in ayurveda and siddha medicine for treating diabetes, was studied for its carbohydrate metabolism effect and antioxidant status in streptozotocin–nicotinamide induced type 2 diabetic rats. Oral administration of C. fenestratum stem extract in graded doses caused a significant increase in enzymatic antioxidants such as catalase, superoxide dismutase, glutathione synthetase, pero...

  5. Risk factors of disturbances in carbohydrate and lipid metabolism and some pleiotropic effects of antihypertensive therapy in pregnant women

    Directory of Open Access Journals (Sweden)

    A V Padyganova

    2013-03-01

    Full Text Available The any kind of hypertension developing during the pregnancy, associated with high cardiovascular risk in the future. At the pregnancy complicated by development by arterial hypertension, there are more expressed changes of a carbohydrate and lipide metabolism, than it is peculiar to normally proceeding pregnancy, were by important pathogenetic links of obesity, diabetes, cardiovascular complications. Identification of new mechanisms of action of antihypertensive means associated with positive influences on exchange processes, is represented very demanded, considering pandemic nature of prevalence of metabolic violations in modern population.

  6. Adherence Issues in Inherited Metabolic Disorders Treated by Low Natural Protein Diets

    NARCIS (Netherlands)

    MacDonald, A.; van Rijn, M.; Feillet, F.; Lund, A. M.; Bernstein, L.; Bosch, A. M.; Gizewska, M.; van Spronsen, F. J.

    2012-01-01

    Common inborn errors of metabolism treated by low natural protein diets [amino acid (AA) disorders, organic acidemias and urea cycle disorders] are responsible for a collection of diverse clinical symptoms, each condition presenting at different ages with variable severity. Precursor-free or essenti

  7. The metabolism of vitamin B6 in relation to genetic disease

    NARCIS (Netherlands)

    Albersen, M.

    2013-01-01

    Over the past years, interest in vitamin B6 has increased, since its essential role in the brain has been recognized and specific inborn errors of metabolism resulting in functional vitamin B6 deficiency have been identified. Patients suffering from vitamin B6 deficiency present with epilepsy and, f

  8. Effect of training in the fasted state on metabolic responses during exercise with carbohydrate intake

    DEFF Research Database (Denmark)

    De Bock, K; Derave, W; Eijnde, B O;

    2008-01-01

    program (6 wk, 3 day/wk, 1-2 h, 75% of peak Vo(2)) in moderately active males. They trained in the fasted (F; n = 10) or carbohydrate-fed state (CHO; n = 10) while receiving a standardized diet [65 percent of total energy intake (En) from carbohydrates, 20%En fat, 15%En protein]. Before and after the...... training period, substrate use during a 2-h exercise bout was determined. During these experimental sessions, all subjects were in a fed condition and received extra carbohydrates (1 g.kg body wt(-1) .h(-1)). Peak Vo(2) (+7%), succinate dehydrogenase activity, GLUT4, and hexokinase II content were...... elicits similar adaptations in peak Vo(2) whether carried out in the fasted or carbohydrate-fed state. Although there was a decrease in exercise-induced glycogen breakdown and an increase in proteins involved in fat handling after fasting training, fat oxidation during exercise with carbohydrate intake...

  9. Cyclin-Dependent Kinase Co-Ordinates Carbohydrate Metabolism and Cell Cycle in S. cerevisiae.

    Science.gov (United States)

    Zhao, Gang; Chen, Yuping; Carey, Lucas; Futcher, Bruce

    2016-05-19

    Cyclin-dependent kinases (CDKs) control cell division in eukaryotes by phosphorylating proteins involved in division. But successful proliferation requires co-ordination between division and cellular growth in mass. Previous proteomic studies suggested that metabolic proteins, as well as cell division proteins, could potentially be substrates of cyclin-dependent kinases. Here we focus on two metabolic enzymes of the yeast S. cerevisiae, neutral trehalase (Nth1) and glycogen phosphorylase (Gph1), and show that their activities are likely directly controlled by CDK activity, thus allowing co-ordinate regulation of carbohydrate metabolism with cell division processes. In this case, co-ordinate regulation may optimize the decision to undertake a final cell division as nutrients are being exhausted. Co-regulation of cell division processes and metabolic processes by CDK activity may be a general phenomenon important for co-ordinating the cell cycle with growth. PMID:27203179

  10. Lysosomal cobalamin accumulation in fibroblasts from a patient with an inborn error of cobalamin metabolism (cblF complementation group): Visualization by electron microscope radioautography

    International Nuclear Information System (INIS)

    Cobalamin (Cbl, vitamin B12) bound to transcobalamin II (TCII) enters cultured fibroblasts by receptor-mediated endocytosis. Following degradation of the TCII, Cbl is subsequently found in either the cytoplasm bound to methionine synthase or in the mitochondria bound to methylmalonyl CoA mutase. In fibroblasts from patients belonging to the cblF complementation group, Cbl is found free in the cell and is not transferred to the above two target enzymes. Quantitative Em radioautography was utilized to visualize intracellular Cbl in fibroblasts from cblF patients and from normal subjects. In cblF cells, 60% of all silver grains were assigned to lysosomes, with only 12.6% over cytoplasm and 1.2% over mitochondria. Subcellular fractionation showed that in cblF cells, the majority of label was associated with clearly recognizable lysosomes. These studies conclusively demonstrate that secondary lysosomes accumulate Cbl in cblF disease

  11. Exogenous classic phytohormones have limited regulatory effects on fructan and primary carbohydrate metabolism in perennial ryegrass (Lolium perenne L.

    Directory of Open Access Journals (Sweden)

    Anna Gasperl

    2016-01-01

    Full Text Available Fructans are polymers of fructose and one of the main constituents of water-soluble carbohydrates in forage grasses and cereal crops of temperate climates. Fructans are involved in cold and drought resistance, regrowth following defoliation and early spring growth, seed filling, have beneficial effects on human health and are used for industrial processes. Perennial ryegrass (Lolium perenne L. serves as model species to study fructan metabolism. Fructan metabolism is under the control of both synthesis by fructosyltransferases (FTs and breakdown through fructan exohydrolases (FEHs. The accumulation of fructans can be triggered by high sucrose levels and abiotic stress conditions such as drought and cold stress. However, detailed studies on the mechanisms involved in the regulation of fructan metabolism are scarce. Since different phytohormones, especially abscisic acid (ABA, are known to play an important role in abiotic stress responses, the possible short term regulation of the enzymes involved in fructan metabolism by the five classical phytohormones was investigated. Therefore, the activities of enzymes involved in fructan synthesis and breakdown, the expression levels for the corresponding genes and levels for water-soluble carbohydrates were determined following pulse treatments with ABA, auxin (AUX, ethylene (ET, gibberellic acid (GA or kinetin (KIN. The most pronounced fast effects were a transient increase of FT activities by AUX, KIN, ABA and ET, while minor effects were evident for 1-FEH activity with an increased activity in response to KIN and a decrease by GA. Fructan and sucrose levels were not affected. This observed discrepancy demonstrates the importance of determining enzyme activities to obtain insight into the physiological traits and ultimately the plant phenotype. The comparative analyses of activities for seven key enzymes of primary carbohydrate metabolism revealed no co-regulation between enzymes of the fructan and

  12. Expression of genes associated with carbohydrate metabolism in cotton stems and roots

    Directory of Open Access Journals (Sweden)

    Scheffler Jodi

    2009-01-01

    Full Text Available Abstract Background Cotton (Gossypium hirsutum L is an important crop worldwide that provides fiber for the textile industry. Cotton is a perennial plant that stores starch in stems and roots to provide carbohydrates for growth in subsequent seasons. Domesticated cotton makes these reserves available to developing seeds which impacts seed yield. The goals of these analyses were to identify genes and physiological pathways that establish cotton stems and roots as physiological sinks and investigate the role these pathways play in cotton development during seed set. Results Analysis of field-grown cotton plants indicated that starch levels peaked about the time of first anthesis and then declined similar to reports in greenhouse-grown cotton plants. Starch accumulated along the length of the stem and the shape and size of the starch grains from stems were easily distinguished from transient starch. Microarray analyses compared gene expression in tissues containing low levels of starch with tissues rapidly accumulating starch. Statistical analysis of differentially expressed genes indicated increased expression among genes associated with starch synthesis, starch degradation, hexose metabolism, raffinose synthesis and trehalose synthesis. The anticipated changes in these sugars were largely confirmed by measuring soluble sugars in selected tissues. Conclusion In domesticated cotton starch stored prior to flowering was available to support seed production. Starch accumulation observed in young field-grown plants was not observed in greenhouse grown plants. A suite of genes associated with starch biosynthesis was identified. The pathway for starch utilization after flowering was associated with an increase in expression of a glucan water dikinase gene as has been implicated in utilization of transient starch. Changes in raffinose levels and levels of expression of genes controlling trehalose and raffinose biosynthesis were also observed in vegetative

  13. Carbohydrate metabolism teaching strategy for the Pharmacy course, applying active teaching methodology

    Directory of Open Access Journals (Sweden)

    Uderlei Donizete Silveira Covizzi

    2012-12-01

    Full Text Available The traditional teaching method has been widely questioned on the development of skills and abilities in training healthcare professionals. In the traditional methodology the main transmitter of knowledge is the teacher while students assume passive spectator role. Some Brazilian institutions broke with this model, structuring the curriculum to student-centered learning. Some medical schools have adopted the Problem Based Learning (PBL, a methodology that presents problem questions, to be encountered by future physicians, for resolution in small tutorial groups. Our work proposes to apply an active teaching-learning methodology addressing carbohydrate metabolism during the discipline of biochemistry for under graduation students from pharmacy course. Thus, the academic content was presented through brief and objective talks. Later, learners were split into tutorial groups for the resolution of issues in context. During the activities, the teacher drove the discussion to the issues elucidation. At the end of the module learners evaluated the teaching methodology by means of an applied questionnaire and the developed content was evaluated by an usual individual test. The questionnaire analysis indicates that students believe they have actively participated in the teaching-learning process, being encouraged to discuss and understand the theme. The answers highlight closer ties between students and tutor. According to the professor, there is a greater student engagement with learning. It is concluded that an innovative methodology, where the primary responsibility for learning is centered in the student himself, besides to increase the interest in learning, facilitates learning by cases discussion in groups. The issues contextualization establishes a narrowing between theory and practice.

  14. Ferulic Acid Alleviates Changes in a Rat Model of Metabolic Syndrome Induced by High-Carbohydrate, High-Fat Diet

    Directory of Open Access Journals (Sweden)

    Ketmanee Senaphan

    2015-08-01

    Full Text Available Metabolic syndrome is a cluster of metabolic abnormalities characterized by obesity, insulin resistance, hypertension and dyslipidemia. Ferulic acid (FA is the major phenolic compound found in rice oil and various fruits and vegetables. In this study, we examined the beneficial effects of FA in minimizing insulin resistance, vascular dysfunction and remodeling in a rat model of high-carbohydrate, high-fat diet-induced metabolic changes, which is regarded as an analogue of metabolic syndrome (MS in man. Male Sprague-Dawley rats were fed a high carbohydrate, high fat (HCHF diet and 15% fructose in drinking water for 16 weeks, where control rats were fed with standard chow diet and tap water. FA (30 or 60 mg/kg was orally administered to the HCHF and control rats during the last six weeks of the study. We observed that FA significantly improved insulin sensitivity and lipid profiles, and reduced elevated blood pressure, compared to untreated controls (p < 0.05. Moreover, FA also improved vascular function and prevented vascular remodeling of mesenteric arteries. The effects of FA in HCHF-induced MS may be realized through suppression of oxidative stress by down-regulation of p47phox, increased nitric oxide (NO bioavailability with up-regulation of endothelial nitric oxide synthase (eNOS and suppression of tumor necrosis factor-α (TNF-α. Our results suggest that supplementation of FA may have health benefits by minimizing the cardiovascular complications of MS and alleviating its symptoms.

  15. Seaweed Supplements Normalise Metabolic, Cardiovascular and Liver Responses in High-Carbohydrate, High-Fat Fed Rats

    Directory of Open Access Journals (Sweden)

    Senthil Arun Kumar

    2015-02-01

    Full Text Available Increased seaweed consumption may be linked to the lower incidence of metabolic syndrome in eastern Asia. This study investigated the responses to two tropical green seaweeds, Ulva ohnoi (UO and Derbesia tenuissima (DT, in a rat model of human metabolic syndrome. Male Wistar rats (330–340 g were fed either a corn starch-rich diet or a high-carbohydrate, high-fat diet with 25% fructose in drinking water, for 16 weeks. High-carbohydrate, high-fat diet-fed rats showed the signs of metabolic syndrome leading to abdominal obesity, cardiovascular remodelling and non-alcoholic fatty liver disease. Food was supplemented with 5% dried UO or DT for the final 8 weeks only. UO lowered total final body fat mass by 24%, systolic blood pressure by 29 mmHg, and improved glucose utilisation and insulin sensitivity. In contrast, DT did not change total body fat mass but decreased plasma triglycerides by 38% and total cholesterol by 17%. UO contained 18.1% soluble fibre as part of 40.9% total fibre, and increased magnesium, while DT contained 23.4% total fibre, essentially as insoluble fibre. UO was more effective in reducing metabolic syndrome than DT, possibly due to the increased intake of soluble fibre and magnesium.

  16. Impact of motor transport emissions on carbohydrate metabolism in leaves of ornamental flower plants

    OpenAIRE

    V. P. Bessonova; О. P. Pryimak

    2012-01-01

    Investigation of carbohydrate exchange in leaves of Salvia sрlendens and Tagetes patula under conditions of environmental pollution by gaseous emission and lead is described in the article. Species differences of glucose quantity under the influence of ingredients of vehicle emissions are presented. Changes in maintenance of non-structural forms of carbohydrates took place as a result of dependences of enzymes activity from pollutants.

  17. Impact of motor transport emissions on carbohydrate metabolism in leaves of ornamental flower plants

    Directory of Open Access Journals (Sweden)

    V. P. Bessonova

    2012-02-01

    Full Text Available Investigation of carbohydrate exchange in leaves of Salvia sрlendens and Tagetes patula under conditions of environmental pollution by gaseous emission and lead is described in the article. Species differences of glucose quantity under the influence of ingredients of vehicle emissions are presented. Changes in maintenance of non-structural forms of carbohydrates took place as a result of dependences of enzymes activity from pollutants.

  18. Metabolic emergencies and the emergency physician.

    Science.gov (United States)

    Fletcher, Janice Mary

    2016-02-01

    Fifty percent of inborn errors of metabolism are present in later childhood and adulthood, with crises commonly precipitated by minor viral illnesses or increased protein ingestion. Many physicians only consider IEM after more common conditions (such as sepsis) have been considered. In view of the large number of inborn errors, it might appear that their diagnosis requires precise knowledge of a large number of biochemical pathways and their interrelationship. As a matter of fact, an adequate diagnostic approach can be based on the proper use of only a few screening tests. A detailed history of antecedent events, together with these simple screening tests, can be diagnostic, leading to life-saving, targeted treatments for many disorders. Unrecognised, IEM can lead to significant mortality and morbidity. Advice is available 24/7 through the metabolic service based at the major paediatric hospital in each state and Starship Children's Health in New Zealand. PMID:27062628

  19. Carbohydrate metabolism is essential for the colonization of Streptococcus thermophilus in the digestive tract of gnotobiotic rats.

    Directory of Open Access Journals (Sweden)

    Muriel Thomas

    Full Text Available Streptococcus thermophilus is the archetype of lactose-adapted bacterium and so far, its sugar metabolism has been mainly investigated in vitro. The objective of this work was to study the impact of lactose and lactose permease on S. thermophilus physiology in the gastrointestinal tract (GIT of gnotobiotic rats. We used rats mono-associated with LMD-9 strain and receiving 4.5% lactose. This model allowed the analysis of colonization curves of LMD-9, its metabolic profile, its production of lactate and its interaction with the colon epithelium. Lactose induced a rapid and high level of S. thermophilus in the GIT, where its activity led to 49 mM of intra-luminal L-lactate that was related to the induction of mono-carboxylic transporter mRNAs (SLC16A1 and SLC5A8 and p27(Kip1 cell cycle arrest protein in epithelial cells. In the presence of a continuous lactose supply, S. thermophilus recruited proteins involved in glycolysis and induced the metabolism of alternative sugars as sucrose, galactose, and glycogen. Moreover, inactivation of the lactose transporter, LacS, delayed S. thermophilus colonization. Our results show i/that lactose constitutes a limiting factor for colonization of S. thermophilus, ii/that activation of enzymes involved in carbohydrate metabolism constitutes the metabolic signature of S. thermophilus in the GIT, iii/that the production of lactate settles the dialogue with colon epithelium. We propose a metabolic model of management of carbohydrate resources by S. thermophilus in the GIT. Our results are in accord with the rationale that nutritional allegation via consumption of yogurt alleviates the symptoms of lactose intolerance.

  20. Substrate kinetics in patients with disorders of skeletal muscle metabolism.

    Science.gov (United States)

    Ørngreen, Mette Cathrine

    2016-07-01

    The main purpose of the following studies was to investigate pathophysiological mechanisms in fat and carbohydrate metabolism and effect of nutritional interventions in patients with metabolic myopathies and in patients with severe muscle wasting. Yet there is no cure for patients with skeletal muscle disorders. The group of patients is heterozygous and this thesis is focused on patients with metabolic myopathies and low muscle mass due to severe muscle wasting. Disorders of fatty acid oxidation (FAO) are, along with myophosphorylase deficiency (McArdle disease), the most common inborn errors of metabolism leading to recurrent episodes of rhabdomyolysis in adults. Prolonged exercise, fasting, and fever are the main triggering factors for rhabdomyolysis in these conditions, and can be complicated by acute renal failure. Patients with low muscle mass are in risk of loosing their functional skills and depend on a wheel chair and respiratory support. We used nutritional interventions and metabolic studies with stable isotope technique and indirect calorimetry in patients with metabolic myopathies and patients with low muscle mass to get information of the metabolism of the investigated diseases, and to gain knowledge of the biochemical pathways of intermediary metabolism in human skeletal muscle. We have shown that patients with fat metabolism disorders in skeletal muscle affecting the transporting enzyme of fat into the mitochondria (carnitine palmitoyltransferase II deficiency) and affecting the enzyme responsible for breakdown of the long-chain fatty acids (very long chain acyl-CoA dehydrogenase deficiency) have a normal fatty acid oxidation at rest, but enzyme activity is too low to increase fatty acid oxidation during exercise. Furthermore, these patients benefit from a carbohydrate rich diet. Oppositely is exercise capacity worsened by a fat-rich diet in these patients. The patients also benefit from IV glucose, however, when glucose is given orally just before

  1. Leaf Responses of Micropropagated Apple Plants to Water Stress: Changes in Endogenous Hormones and Their Influence on Carbohydrate Metabolism

    Institute of Scientific and Technical Information of China (English)

    LI Tian-hong; LI Shao-hua

    2007-01-01

    The changes in the concentrations of endogenous hormones and their influence on carbohydrate metabolism in leaves of micropropagated Fuji apple plants were studied under water deficiency stress. The results showed that water stress induced a rapid increase in the concentration of abscisic acid (ABA) and led to a decrease in concentrations of both zeatin and gibberellins (GAs). The concentration of indole-3-acetic acid (IAA) changed in an independent manner, which was not correlated with the different levels of water stress. With regard to the carbohydrates, the contents of sorbitol and sucrose increased, whereas the content of starch decreased. The increase in the concentration of ABA was significantly correlated with both the increase in the activity of aldose-6-phosphate reductase (A6PR) and the decrease in the activity of sorbitol dehydrogenase (SDH), indicating that ABA played a regulatory role in sorbitol metabolism. The concentration of ABA was positively correlated to the activity of sucrose-phosphate synthase (SPS) but negatively correlated to the activities of acid invertase (AI) and ADP-glucose-pyrophosphorylase (ADPGppase) in water-stressed plants, which indicated that ABA promoted sucrose synthesis and inhibited sucrose degradation and starch synthesis at the same time. Under conditions of water stress, the decrease in the level of zeatin was accompanied by a decrease in the activities of SDH and ADPGPPase. GAs concentration showed positive correlation with ADPGPPase activity. IAA showed no significant correlation with any of the enzymes tested in this study. The results of this study suggested that ABA might be one of the key factors regulating the distribution of carbohydrates under water stress. The metabolism of sorbitol and starch under conditions of water stress might be regulated by the combined action of many plant hormones.

  2. Effect of Low Dietary Zinc Intake and Experimental Diabetes on the Zinc and Carbohydrate Metabolism in Rats

    OpenAIRE

    KECHRID, Zine

    2002-01-01

    To investigate the effect of low dietary zinc intake and experimental diabetes (IDDM) on the zinc and carbohydrate metabolism, 8-week-old male wealing normal albino (Wistar) rats were fed diets containing either adequate (54mg/kg) or low zinc (1mg/kg) quantities for one week. Ten rats from each group (n=20) were then intraperitoneally injected with alloxan to induce diabetes. The rats were sacrificed after a further three weeks. Body weight gain and food intake were recorded regularly. On day...

  3. Determining pathogenetic connection between disorders of lipid and carbohydrate metabolism and non-malignant pathology of thyroid gland in children , born from parents, Chernobyl accident survivors

    International Nuclear Information System (INIS)

    The 92 children aged 12-17 years were examined with the purpose to study the links between carbohydrate and lipid metabolic abnormalities and non-malignant thyroid disorders in descendants of the Chernobyl accident survivors. Clinical, anthropometrical studies and hormonal assays were applied. Carbohydrate and lipid metabolic abnormalities were revealed in every third case of thyroid disease. It confirms our supposition of such a possibility being due to the fact that radiation impact even in low doses can result in pronounced metabolic disorders lading to entire endocrine disregulation. It is relevant in children of the puberty age

  4. Influence of nutrient supply on growth, carbohydrate, and nitrogen metabolic relations in Typha angustifolia

    Czech Academy of Sciences Publication Activity Database

    Steibachová; Vojtíšková, L.; Tylová, E.; Soukup, A.; Novická, H.; Votrubová, O.; Lipavslá, H.; Čížková, Hana

    2006-01-01

    Roč. 57, - (2006), s. 246-257. ISSN 0098-8472 Institutional research plan: CEZ:AV0Z60870520 Keywords : Wetland plant * Nitrogen * Phosphorus * Storage carbohydrates * Starch * C/N balance * Eutrophic ation Subject RIV: EF - Botanics Impact factor: 1.820, year: 2006

  5. Sugars, Stable Isotopes, and Spectrometry: New Methods for the Analysis of Carbohydrate Metabolism

    Science.gov (United States)

    Structural analysis of carbohydrates involves three parameters: composition, linkage, and conformation, and tends to rely on the various forms of two techniques; mass spectrometry (MS) and nuclear magnetic resonance (NMR) spectroscopy. These techniques are enhanced and extended by the use of stable...

  6. Metabolic flux in carbohydrate biosynthesis. New methods using stable isotopes, mass spectrometry, and NMR

    Science.gov (United States)

    Structural analysis of carbohydrates involves three parameters: composition, linkage, and conformation, and tends to rely on the various forms of two techniques; mass spectrometry (MS) and nuclear magnetic resonance (NMR) spectroscopy. These techniques are enhanced and extended by the use of stable...

  7. Proteomics Profiling Reveals Carbohydrate Metabolic Enzymes and 14-3-3 Proteins Play Important Roles for Starch Accumulation during Cassava Root Tuberization.

    Science.gov (United States)

    Wang, Xuchu; Chang, Lili; Tong, Zheng; Wang, Dongyang; Yin, Qi; Wang, Dan; Jin, Xiang; Yang, Qian; Wang, Liming; Sun, Yong; Huang, Qixing; Guo, Anping; Peng, Ming

    2016-01-01

    Cassava is one of the most important root crops as a reliable source of food and carbohydrates. Carbohydrate metabolism and starch accumulation in cassava storage root is a cascade process that includes large amounts of proteins and cofactors. Here, comparative proteomics were conducted in cassava root at nine developmental stages. A total of 154 identified proteins were found to be differentially expressed during starch accumulation and root tuberization. Many enzymes involved in starch and sucrose metabolism were significantly up-regulated, and functional classification of the differentially expressed proteins demonstrated that the majority were binding-related enzymes. Many proteins were took part in carbohydrate metabolism to produce energy. Among them, three 14-3-3 isoforms were induced to be clearly phosphorylated during storage root enlargement. Overexpression of a cassava 14-3-3 gene in Arabidopsis thaliana confirmed that the older leaves of these transgenic plants contained higher sugar and starch contents than the wild-type leaves. The 14-3-3 proteins and their binding enzymes may play important roles in carbohydrate metabolism and starch accumulation during cassava root tuberization. These results not only deepened our understanding of the tuberous root proteome, but also uncovered new insights into carbohydrate metabolism and starch accumulation during cassava root enlargement. PMID:26791570

  8. Slow-release carbohydrates: growing evidence on metabolic responses and public health interest. Summary of the symposium held at the 12th European Nutrition Conference (FENS 2015)

    OpenAIRE

    Vinoy, Sophie; Laville, Martine; Feskens, Edith

    2016-01-01

    To draw attention to the necessity of considering differences in the digestibility of carbohydrates, and more specifically of starch, a symposium was held at the 12th European Nutrition Conference (FENS), which took place in Berlin from October 20 to 23, 2015. The purpose of this session was to present the consolidated knowledge and recent advances regarding the relationship between slow-release carbohydrates, metabolic responses, and public health issues. Three main topics were presented: 1)...

  9. Effect of carbon/nitrogen ratio on carbohydrate metabolism and light energy dissipation mechanisms in Arabidopsis thaliana.

    Science.gov (United States)

    Huarancca Reyes, Thais; Scartazza, Andrea; Lu, Yu; Yamaguchi, Junji; Guglielminetti, Lorenzo

    2016-08-01

    Carbon (C) and nitrogen (N) nutrient sources are essential elements for metabolism, and their availability must be tightly coordinated for the optimal growth and development in plants. Plants are able to sense and respond to different C/N conditions via specific partitioning of C and N sources and the regulation of a complex cellular metabolic activity. We studied how the interaction between C and N signaling could affect carbohydrate metabolism, soluble sugar levels, photochemical efficiency of photosystem II (PSII) and the ability to drive the excess energy in Arabidopsis seedlings under moderated and disrupted C/N-nutrient conditions. Invertase and sucrose synthase activities were markedly affected by C/N-nutrient status depending on the phosphorylation status, suggesting that these enzymes may necessarily be modulated by their direct phosphorylation or phosphorylation of proteins that form complex with them in response to C/N stress. In addition, the enzymatic activity of these enzymes was also correlated with the amount of sugars, which not only act as substrate but also as signaling compounds. Analysis of chlorophyll fluorescence in plants under disrupted C/N condition suggested a reduction of electron transport rate at PSII level associated with a higher capacity for non-radiative energy dissipation in comparison with plants under moderated C/N condition. In conclusion, the tight coordination between C and N not only affects the carbohydrates metabolism and their concentration within plant tissues, but also the partitioning of the excitation energy at PSII level between radiative (electron transport) and non-radiative (heat) dissipation pathways. PMID:27108206

  10. Identification of behavioural and metabolic factors predicting adiposity sensitivity to both high fat and high carbohydrate diets in rats.

    Directory of Open Access Journals (Sweden)

    PatrickChristianEven

    2011-12-01

    Full Text Available Individuals exhibit a great variation in their body weight gain response to a high-fat diet. Identification of predictive factors would enable better directed intervention towards susceptible individuals to treat obesity, and uncover potential mechanisms for treatment targeting. We set out to identify predictive behavioural and metabolic factors in an outbred rat model. 12 rats were analysed in metabolic cages for a period of 5 days during both high carbohydrate diet (HCD, and transition to a high fat diet (HFD. After a recovery period, rats were given a HFD for 6 days to identify those resistant or sensitive to it according to body weight gain. Rats were dissected at the end of the study to analyse body composition. This showed that in rats fed a HCD during most of their life, small differences in final body weight hid large variations in adiposity, allowing separation of rats into a second classification of carbohydrate-sensitive or -resistant. Meal size and meal number were found to be good predictors of sensitivity to a HFD, intensity of motor activity and ingestion speed good predictors of sensitivity to a HCD. Rats that were sensitive to the HCD could be resistant to the HFD and vice versa. This contributes to the idea that to be “obesity prone” does not necessarily need a HFD, it can also happen under a HCD, and be a hidden adiposity change with stable body weight.

  11. Is a Calorie Really a Calorie? Metabolic Advantage of Low-Carbohydrate Diets

    Directory of Open Access Journals (Sweden)

    Manninen Anssi H

    2004-12-01

    Full Text Available Abstract The first law of thermodynamics dictates that body mass remains constant when caloric intake equals caloric expenditure. It should be noted, however, that different diets lead to different biochemical pathways that are not equivalent when correctly compared through the laws of thermodynamics. It is inappropriate to assume that the only thing that counts in terms of food consumption and energy balance is the intake of dietary calories and weight storage. Well-controlled studies suggest that calorie content may not be as predictive of fat loss as is reduced carbohydrate consumption. Biologically speaking, a calorie is certainly not a calorie. The ideal weight loss diet, if it even exists, remains to be determined, but a high-carbohydrate/low-protein diet may be unsatisfactory for many obese individuals.

  12. Effect Of Oleanolic Acid Isolated From Garlic Leaves On Carbohydrate Metabolizing Enzymes, In Vitro

    OpenAIRE

    G.A. Meshram; S. S. Khamkar

    2014-01-01

    Post-prandial hyperglycemia can be controlled by retarding the absorption of glucose through inhibition of the two main enzymes, α-amylase and glucoamylase. This action delays carbohydrate digestion causing reduction in the rate of glucose absorption and degradation of glycogen during starvation. Allium sativum (Garlic) is a medicinal plant used worldwide for flavoring as well as medicinal purpose. However, chemical examination and biological activity of compounds present in the leaves are le...

  13. Identification of the first insulin-like peptide in the disease vector Rhodnius prolixus: Involvement in metabolic homeostasis of lipids and carbohydrates.

    Science.gov (United States)

    Defferrari, Marina S; Orchard, Ian; Lange, Angela B

    2016-03-01

    Insulin-like peptides (ILPs) are functional analogs of insulin and have been identified in many insect species. The insulin cell signaling pathway is a conserved regulator of metabolism, and in insects, as well as in other animals, can modulate physiological functions associated with the metabolism of lipids and carbohydrates. In the present study, we have identified the first ILP from the Rhodnius prolixus genome (termed Rhopr-ILP) and investigated its involvement in energy metabolism of unfed and recently fed fifth instars. We have cloned the cDNA sequence and analyzed the expression profile of the transcript, which is predominantly present in neurosecretory cells in the brain, similar to other insect ILPs. Using RNAi, we have reduced the expression of this peptide transcript by 90% and subsequently measured the carbohydrate and lipid levels in the hemolymph, fat body and leg muscles. Reduced levels of Rhopr-ILP transcript induced increased carbohydrate and lipid levels in the hemolymph and increased lipid content in the fat body, in unfed insects and recently fed insects. Also their fat bodies displayed enlarged lipid droplets within the cells. On the other hand, the carbohydrate content in the fat body and in the leg muscles of unfed insects were decreased when compared to control insects. Our results indicate that Rhopr-ILP is a modulator of lipid and carbohydrate metabolism, probably through signaling the presence of available energy and nutrients in the hemolymph. PMID:26742603

  14. Deep insights into carbohydrate metabolism in the rumen of Mehsani buffalo at different diet treatments

    Directory of Open Access Journals (Sweden)

    Nidhi R. Parmar

    2015-12-01

    Full Text Available Ruminants are known to harbour a vast and diverse microbial community that functions in utilizing the fibrous and starchy feedstuffs. The microbial fermentation of fibrous and starchy feed is carried out by different groups of microbiota, which function in synergistic mechanism. The exploration of the shift in carbohydrate utilizing microbial community with the change in diet will reveal the efficient role of that group of microbial community in particular carbohydrate utilization. The present study explains the shifts in microbial enzymes for carbohydrate utilization with the change in the feed proportions and its correlation with the microbial community abundance at that particular treatment. The sequencing data of the present study is submitted to NCBI SRA with experiment accession IDs (ERX162128, ERX162129, ERX162130, ERX162131, ERX162139, ERX162134, ERX162140, ERX162141, ERX197218, ERX197219, ERX197220, ERX197221, ERX162158, ERX162159, ERX162160, ERX162161, ERX162176, ERX162164, ERX162165, ERX162166, ERX162167, ERX162168, ERX162169, ERX162177.

  15. AMP-activated protein kinase: an emerging drug target to regulate imbalances in lipid and carbohydrate metabolism to treat cardio-metabolic diseases.

    Science.gov (United States)

    Srivastava, Rai Ajit K; Pinkosky, Stephen L; Filippov, Sergey; Hanselman, Jeffrey C; Cramer, Clay T; Newton, Roger S

    2012-12-01

    The adenosine monophosphate-activated protein kinase (AMPK) is a metabolic sensor of energy metabolism at the cellular as well as whole-body level. It is activated by low energy status that triggers a switch from ATP-consuming anabolic pathways to ATP-producing catabolic pathways. AMPK is involved in a wide range of biological activities that normalizes lipid, glucose, and energy imbalances. These pathways are dysregulated in patients with metabolic syndrome (MetS), which represents a clustering of major cardiovascular risk factors including diabetes, lipid abnormalities, and energy imbalances. Clearly, there is an unmet medical need to find a molecule to treat alarming number of patients with MetS. AMPK, with multifaceted activities in various tissues, has emerged as an attractive drug target to manage lipid and glucose abnormalities and maintain energy homeostasis. A number of AMPK activators have been tested in preclinical models, but many of them have yet to reach to the clinic. This review focuses on the structure-function and role of AMPK in lipid, carbohydrate, and energy metabolism. The mode of action of AMPK activators, mechanism of anti-inflammatory activities, and preclinical and clinical findings as well as future prospects of AMPK as a drug target in treating cardio-metabolic disease are discussed. PMID:22798688

  16. The metabolism of vitamin B6 in relation to genetic disease

    OpenAIRE

    M. Albersen

    2013-01-01

    Over the past years, interest in vitamin B6 has increased, since its essential role in the brain has been recognized and specific inborn errors of metabolism resulting in functional vitamin B6 deficiency have been identified. Patients suffering from vitamin B6 deficiency present with epilepsy and, frequently, developmental delay. Disturbances of vitamin B6 metabolism may be missed by biochemical profiling of the secondary effects of functional vitamin B6 deficiency. Direct analysis of the dif...

  17. Azospirillum brasilense locus coding for phosphoenolpyruvate:fructose phosphotransferase system and global regulation of carbohydrate metabolism.

    OpenAIRE

    Chattopadhyay, S; Mukherjee, A.; Ghosh, S.

    1993-01-01

    Mutants of Azospirillum brasilense unable to grow on fructose include ones affected only on fructose (Fru-) and others impaired on many or all carbohydrates through interference with induction of their specific pathways (Car-). Both types of mutants could be complemented by a cosmid in broad-host-range vector pLAFR1 containing a 27.5-kb genomic insert, Car(-)-complementing activity depending on a 2.2-kb fragment, and Fru(-)-complementing activity depending on an overlapping 9.6-kb fragment.

  18. 水生龟鳖类糖代谢的研究进展%Research Advances in Carbohydrate Metabolism in Aquatic Turtles

    Institute of Scientific and Technical Information of China (English)

    刘海燕; 杨振才

    2013-01-01

    水生龟鳖类是一种以摄食动物性蛋白质饲料为主的爬行动物,对糖的利用能力不高.本文综述了水生龟鳖类对糖的利用能力、糖代谢模式及调控机制的研究现状,并结合哺乳动物和鱼类的糖代谢机制比较其中的联系与区别,提出水生龟鳖类糖代谢研究中存在的问题及今后的研究方向.%Aquatic turtles are a kind of reptile mainly ingesting the animal protein feed, and they have a low a-bility in utilization of carbohydrate. This review summarizes the recent advances about utilization ability of carbohydrate , carbohydrate metabolism model and regulation mechanism for aquatic turtles, and compares the relation and difference of mammals and fish with turtles in the carbohydrate metabolism mechanism in order to put forward the questions and directions of the carbohydrate metabolism study in aquatic turtles.

  19. Biochemical studies of effects of alcohol consumption on fat and carbohydrate metabolism in rats fed different levels of proteins

    International Nuclear Information System (INIS)

    Alcohol, ethanol and ethyl alcohol are synonymously used during the present dissertation. Alcohol probably was among the first psychoactive substances to be used by man (Winger et al., 1992). Ethanol is mainly oxidized to acetaldehyde in the liver (Ugarte and Peresa, 1978) by alcohol dehydrogenase (ADH). Alcohol is associated with many metabolic disorders inside the body (Thayer and Rubin, 1979; Forsander and Poso, 1988; Poso and Hirsimaki, 1991; Bernal, et al., 1992). The nutritional factors which received little attention have an important role in alcoholic metabolizing alterations. Morphologically and biochemically, an increase in hepatic lipid was demonstrated when ethanol was given either as a supplement or as an iso caloric substitute for carbohydrate together with an otherwise nutritionally adequate diet. Low-protein diets have been shown to diminish hepatic alcohol dehydrogenase (ADH) levels in rats and to slow down the metabolism of ethanol considerably (Wilson et al., 1986). Hepatic steatosis was produced, even with a high-protein, vitamin-supplemented diet and was accompanied by major ultrastructural liver changes and by elevations of hepatic transaminases in blood (Lieber et al., 1963 and 1965 and Lane and Lieber, 1966). If dietary fat was reduced from 35 to 25% of total calories, hepatic triglyceride accumulation greatly decreased (Lieber and DeCarli, 970)

  20. Carbohydrate metabolism in ripening banana and its alteration on gamma irradiation in relation to delay in ripening

    International Nuclear Information System (INIS)

    Ripening, of climacteric class of fruits like banana, is accompanied with an upsurge in respiration, indicating a change in metabolism from hexose monophosphate (HMP) shunt pathway to glycolytic pathway. The key enzyme in glycolytic pathway, namely, phosphofructokinase, is activated and this activation paralleled with the increase in respiration rate. The enhancement in the activity of enzymes of glycolytic and Kreb's cycle help the fruit to assimilate energy as ATP produced from the breakdown and oxidation of storage starch. The demand for energy supply is great for the different ripening processes. Gamma irradiation of the fruit at the preclimacteric stage delayed the onset of climacteric to about 7 to 8 days, thereby extending the ripening to 15-20 days. This delay was brought about by the alterations in the metabolism of carbohydrate. There is a predominance of HMP pathway in irradiated banana. This along with the activation of phosphatases like FDPase and F-6-Pase restricted the entrance of sugar phosphate esters to Kreb's cycle for oxidation. The functioning of Kreb's cycle is also affected by the inhibition of succinic dehydrogenase. But activation of glyoxylate shunt pathway helped to maintain the levels of Kreb's cycle intermediates, like citrate and malate, although energy production is reduced. Finally the activation of gluconeogenic pathway helps in channelling the metabolites back to sugars. All these metabolic changes cause a considerable depletion in the production of ATP. (auth.)

  1. Effect of aspirin and prostaglandins on the carbohydrate metabolism in albino rats.: glucose oxidation through different pathways and glycolytic enzymes

    International Nuclear Information System (INIS)

    The effect of chronic and acute doses of aspirin and prostaglandins F2α and E2 individually on the oxidation of glucose through Embden Meyerhof-TCA cycle and pentose phosphate pathways and some key glycolytic enzymes of liver were studied in male albino rats. Studies were extended to find the combined effect of PGF2α and E2 with an acute dose of aspirin. There was increased utilisation of both 1-14C glucose and 6-14C glucose on aspirin treatment. However, the metabolism through the EM-TCA pathway was more pronounced as shown by a reduced ratio of 14CO2 from 1-14C and 6-14C glucose. Two hepatic key glycolytic enzymes viz. hexokinase and pyruvate kinase were increased due to aspirin treatment. Withdrawal of aspirin corrected the above impaired carbohydrate metabolism in liver. Prostaglandin F2α also caused a reduction in the utilisation of 1-14C glucose, while PGE2 recorded an increase in the utilisation of both 1-14C and 6-14C glucose when compared to controls, indicating that different members of prostaglandins could affect metabolisms and differently. Administration of the PGs and aspirin together showed an increase in the utilisation of 6-14C glucose. (auth.)

  2. Alcoholic Stem Extract of Coscinium fenestratum Regulates Carbohydrate Metabolism and Improves Antioxidant Status in Streptozotocin–Nicotinamide Induced Diabetic Rats

    Directory of Open Access Journals (Sweden)

    I. R. Punitha

    2005-01-01

    Full Text Available Alcoholic extract of the stems of Coscinium fenestratum, a medicinal plant indigenous to India and Sri Lanka used in ayurveda and siddha medicine for treating diabetes, was studied for its carbohydrate metabolism effect and antioxidant status in streptozotocin–nicotinamide induced type 2 diabetic rats. Oral administration of C. fenestratum stem extract in graded doses caused a significant increase in enzymatic antioxidants such as catalase, superoxide dismutase, glutathione synthetase, peroxidase, and glutathione peroxidase and in the nonenzymatic antioxidants ascorbic acid, ceruloplasmin and tocopherol. Effects of alcoholic extract on glycolytic enzymes such as glucose-6-phosphate dehydrogenase, lactate dehydrogenase and hexokinase showed a significant increase in their levels, whereas a significant decrease was observed in the levels of gluconeogenic enzyme, glucose-6-phosphatase and alanine aminotransferase in treated diabetic rats. Serum creatinine and urea levels also declined significantly. This investigation demonstrates significant antidiabetic activity of C. fenestratum.

  3. Consequences of exchanging carbohydrates for proteins in the cholesterol metabolism of mice fed a high-fat diet.

    Directory of Open Access Journals (Sweden)

    Frédéric Raymond

    Full Text Available Consumption of low-carbohydrate, high-protein, high-fat diets lead to rapid weight loss but the cardioprotective effects of these diets have been questioned. We examined the impact of high-protein and high-fat diets on cholesterol metabolism by comparing the plasma cholesterol and the expression of cholesterol biosynthesis genes in the liver of mice fed a high-fat (HF diet that has a high (H or a low (L protein-to-carbohydrate (P/C ratio. H-P/C-HF feeding, compared with L-P/C-HF feeding, decreased plasma total cholesterol and increased HDL cholesterol concentrations at 4-wk. Interestingly, the expression of genes involved in hepatic steroid biosynthesis responded to an increased dietary P/C ratio by first down-regulation (2-d followed by later up-regulation at 4-wk, and the temporal gene expression patterns were connected to the putative activity of SREBF1 and 2. In contrast, Cyp7a1, the gene responsible for the conversion of cholesterol to bile acids, was consistently up-regulated in the H-P/C-HF liver regardless of feeding duration. Over expression of Cyp7a1 after 2-d and 4-wk H-P/C-HF feeding was connected to two unique sets of transcription regulators. At both time points, up-regulation of the Cyp7a1 gene could be explained by enhanced activations and reduced suppressions of multiple transcription regulators. In conclusion, we demonstrated that the hypocholesterolemic effect of H-P/C-HF feeding coincided with orchestrated changes of gene expressions in lipid metabolic pathways in the liver of mice. Based on these results, we hypothesize that the cholesterol lowering effect of high-protein feeding is associated with enhanced bile acid production but clinical validation is warranted. (246 words.

  4. Polymorphism of CD36 gene, carbohydrate metabolism and plasma CD36 concentration in obese children. A preliminary study 

    Directory of Open Access Journals (Sweden)

    Monika E. Rać

    2012-11-01

    Full Text Available Introduction:CD36 may play an important role in removal of oxidized LDLs from plasma, protein glycation, the pathogenesis of insulin resistance, type 2 diabetes, and diabetic micro- and macroangiopathy. Some reports have pointed to decreased expression of macrophages in association with mutations of the CD36 gene in hyperglycemic and obese subjects. The aim of the study was to search for an association between CD36 gene polymorphism and carbohydrate metabolism disturbances or variability of plasma soluble CD36 concentrations in obese children.Material/Methods:The study included 60 children aged 10 to 15 years: 30 with (study group and 30 without (control group obesity. Each patient’s glycated hemoglobin, weight, height, waist and hip circumference, and systolic and diastolic blood pressure were measured, BMI, WHR and MAP were calculated, and oral glucose tolerance test was performed with glucose and insulin concentration measurements. Amplicons of exons 4–6 of CD36 were studied using DHPLC technique. The PCR products with alterations were bidirectionally sequenced. Plasma concentrations of human antigen CD36 was measured using a commercially available enzyme-linked immunosorbent assay (ELISA.Results:We found two intronic alterations: IVS3-6 T/C (rs3173798 and IVS4-10 G/A (rs3211892, one nonsynonymous substitution: G367A (Glu123Lys, rs183461468 in exon 5 and two synonymous transitions in exon 6: G573A (Pro191Pro, rs5956 and A591T (Thr197Thr, rs141680676. There were no significant differences in any biochemical or morphometric parameters between genotype groups.Discussion:The polymorphisms of the studied fragment of CD36 are not associated with carbohydrate metabolism disturbances or the variability of plasma soluble CD36 concentrations in obese children, but further research is necessary to assess their functional implications. 

  5. Gallic acid ameliorates hyperglycemia and improves hepatic carbohydrate metabolism in rats fed a high-fructose diet.

    Science.gov (United States)

    Huang, Da-Wei; Chang, Wen-Chang; Wu, James Swi-Bea; Shih, Rui-Wen; Shen, Szu-Chuan

    2016-02-01

    Herein, we investigated the hypoglycemic effect of plant gallic acid (GA) on glucose uptake in an insulin-resistant cell culture model and on hepatic carbohydrate metabolism in rats with a high-fructose diet (HFD)-induced diabetes. Our hypothesis is that GA ameliorates hyperglycemia via alleviating hepatic insulin resistance by suppressing hepatic inflammation and improves abnormal hepatic carbohydrate metabolism by suppressing hepatic gluconeogenesis and enhancing the hepatic glycogenesis and glycolysis pathways in HFD-induced diabetic rats. Gallic acid increased glucose uptake activity by 19.2% at a concentration of 6.25 μg/mL in insulin-resistant FL83B mouse hepatocytes. In HFD-induced diabetic rats, GA significantly alleviated hyperglycemia, reduced the values of the area under the curve for glucose in an oral glucose tolerance test, and reduced the scores of the homeostasis model assessment of insulin resistance index. The levels of serum C-peptide and fructosamine and cardiovascular risk index scores were also significantly decreased in HFD rats treated with GA. Moreover, GA up-regulated the expression of hepatic insulin signal transduction-related proteins, including insulin receptor, insulin receptor substrate 1, phosphatidylinositol-3 kinase, Akt/protein kinase B, and glucose transporter 2, in HFD rats. Gallic acid also down-regulated the expression of hepatic gluconeogenesis-related proteins, such as fructose-1,6-bisphosphatase, and up-regulated expression of hepatic glycogen synthase and glycolysis-related proteins, including hexokinase, phosphofructokinase, and aldolase, in HFD rats. Our findings indicate that GA has potential as a health food ingredient to prevent diabetes mellitus. PMID:26547672

  6. Carbohydrate oxidation coupled to Fe(III) reduction, a novel form of anaerobic metabolism.

    Science.gov (United States)

    Coates, J D; Councell, T; Ellis, D J; Lovley, D R

    1998-12-01

    An isolate, designated GC-29, that could incompletely oxidize glucose to acetate and carbon dioxide with Fe(III) serving as the electron acceptor was recovered from freshwater sediments of the Potomac River, Maryland. This metabolism yielded energy to support cell growth. Strain GC-29 is a facultatively anaerobic, gram-negative motile rod which, in addition to glucose, also used sucrose, lactate, pyruvate, yeast extract, casamino acids or H2 as alternative electron donors for Fe(III) reduction. Stain GC-29 could reduce NO3(-), Mn(IV), U(VI), fumarate, malate, S2O3(2-), and colloidal S0 as well as the humics analog, 2,6-anthraquinone disulfonate. Analysis of the almost complete 16S rRNA sequence indicated that strain GC-29 belongs in the Shewanella genus in the epsilon subdivision of the Proteobacteria. The name Shewanella saccharophilia is proposed. Shewanella saccharophilia differs from previously described fermentative microorganisms that metabolize glucose with the reduction of Fe(III) because it transfers significantly more electron equivalents to Fe(III); acetate and carbon dioxide are the only products of glucose metabolism; energy is conserved from Fe(III) reduction; and glucose is not metabolized in the absence of Fe(III). The metabolism of organisms like S. saccharophilia may account for the fact that glucose is metabolized primarily to acetate and carbon dioxide in a variety of sediments in which Fe(III) reduction is the terminal electron accepting process. PMID:16887653

  7. Abdominal ultrasonography in inheredited diseases of carbohydrate metabolism; Ecografia dell'addome nelle malattie ereditarie del metabolismo dei carboidrati

    Energy Technology Data Exchange (ETDEWEB)

    Pozzato, Carlo; Curti, Alessandra; Cornalba, Gianpaolo [Milano Univ., Ospedale San Paolo, Milano (Italy). Unita' Operativa di Radiologia Diagnostica ed Interventistica, Istituto di Scienze Radiologiche; Radaelli, Giovanni [Milano Univ., Ospedale San Paolo, Milano (Italy). Unita' Operativa di Statistica Medica; Fiori, Laura; Rossi, Samantha; Riva, Enrica [Milano Univ., Ospedale San Paolo, Mialno (Italy). Dipartimento di Pediatria

    2005-02-01

    Purpose: To determine the usefulness of abdominal sonography in inherited diseases of carbohydrate metabolism. Materials and methods: Thirty patients (age range, 4 months to 27 years) with glycogen storage diseases, galactosemia, disorders of fructose metabolism were studied with sonography. Echogenicity of the liver, sonographic dimensions of liver, kidneys and spleen were evaluated. Plasma blood parameters (ALT, AST, total cholesterol, triglycerides) were determined. Results: Liver was enlarged in 21/22 patients (95.4%) with glycogen storage diseases, in both subjects with disorders of fructose metabolism, and in 2/6 patients (33.3%) with galactosemia. Hepatic echogenicity was increased in 20/22 patients (90.9%) with glycogen storage diseases, and in the subject with hereditary fructose intolerance. Patients with galactosemia did not show increased liver echogenicity. Both kidney were enlarged in 8/17 patients (47.0%) with glycogen storage disease type I. Subjects with increased hepatic echogenicity exhibited higher plasma concentrations of any blood parameter than the others with normal echogenicity (p<0.05). Conclusions: Sonography can be useful in identification of inherited diseases of carbohydrate metabolism even if further examinations are necessary for an ultimate diagnosis. [Italian] Scopo: Determinare l'utilita' dell'ecografia addominale nelle malattie ereditarie del metabolismo dei carboidrati. Materiale e metodi: Di 30 pazienti (eta' compresa tra 4 mesi e 27 anni), affetti da malattie di accumulo di glicogeno (glicogenosi), galattosemia, disordini del metabolismo del fruttosio, sono stati valutati tramite ecografia l'ecogenicita' epatica e le dimensioni ecografiche di fegato, reni e milza. Sono stati determinati alcuni parametri ematici (ALT, AST, colesterolo totale, trigliceridi). Risultati: Il fegato e' risultato ingrandito in 21/22 pazienti (95,4%) con malattie da accumolo di glicogeno, in entrambi i soggetti con

  8. Phylloxera (Daktulosphaira vitifoliae Fitch) alters the carbohydrate metabolism in root galls to allowing the compatible interaction with grapevine (Vitis ssp.) roots

    OpenAIRE

    Griesser, Michaela; Lawo, Nora Caroline; Crespo-Martinez, Sara; Schoedl-Hummel, Katharina; Wieczorek, Krzysztof; Gorecka, Miroslawa; Liebner, Falk; Zweckmair, Thomas; Stralis Pavese, Nancy; Kreil, David; Forneck, Astrid

    2015-01-01

    Gall forming phylloxera may compete for nutrients with meristematic tissues and develop heterotrophic structures that act as carbon sinks. In this work, we studied the underlying starch metabolism, sink-source translocation of soluble sugars towards and within root galls. We demonstrated that nodosities store carbohydrates by starch accumulation and monitored the expression of genes involved in the starch metabolic. Thereby we proved that the nodosity is symplastically connected to the source...

  9. Simple and robust determination of the activity signature of key carbohydrate metabolism enzymes for physiological phenotyping in model and crop plants

    Czech Academy of Sciences Publication Activity Database

    Jammer, A.; Gapserl, A.; Luschin-Ebengreuth, N.; Heyneke, E.; Chu, H.; Cantero-Navarro, E.; Grosskinsky, D. K.; Albacete, A.; Stabentheiner, E.; Franzaring, J.; Fangmeier, A.; van der Graaff, E.; Roitsch, Thomas

    2015-01-01

    Roč. 66, č. 18 (2015), s. 5531-5542. ISSN 0022-0957 Institutional support: RVO:67179843 Keywords : Carbohydrate metabolism * dialysis * enzyme activities * kinetic assay * physiological phenotyping * physiological state * protein extraction * signatures Subject RIV: EH - Ecology, Behaviour Impact factor: 5.526, year: 2014

  10. Low-carbohydrate/high-protein diet improves diastolic cardiac function and the metabolic syndrome in overweight-obese patients with type 2 diabetes

    Directory of Open Access Journals (Sweden)

    H. von Bibra

    2014-03-01

    Conclusions: These data indicate, that a low-glycaemic/high-protein but not a low-fat/high-carbohydrate nutrition modulates diastolic dysfunction in overweight T2D patients, improves insulin resistance and may prevent or delay the onset of diabetic cardiomyopathy and the metabolic syndrome.

  11. [Effect of vibration, noise, physical exertion and unfavorable microclimate on carbohydrate metabolism in workers engaged into mining industry and machine building].

    Science.gov (United States)

    Lapko, I V; Kir'iakov, V A; Antoshina, L I; Pavlovskaia, N A; Kondratovich, S V

    2014-01-01

    The authors studied influence of vibration, noise, physical overexertion and microclimate on carbohydrates metabolism and insulin resistance in metal mining industry workers. Findings are that vibration disease appeared to have maximal effect on insulin resistance test results and insulin level. The authors suggested biomarkers for early diagnosis of insulin resistance disorders in metal mining industry workers. PMID:25282800

  12. Open Label Extension of ISIS 301012 (Mipomersen) to Treat Familial Hypercholesterolemia

    Science.gov (United States)

    2013-12-02

    Lipid Metabolism, Inborn Errors; Hypercholesterolemia, Autosomal Dominant; Hyperlipidemias; Metabolic Diseases; Hyperlipoproteinemia Type II; Metabolism, Inborn Errors; Genetic Diseases, Inborn; Infant, Newborn, Diseases; Metabolic Disorder; Congenital Abnormalities; Hypercholesterolemia; Hyperlipoproteinemias; Dyslipidemias; Lipid Metabolism Disorders

  13. Study to Assess the Safety and Efficacy of ISIS 301012 (Mipomersen) in Homozygous Familial Hypercholesterolemia

    Science.gov (United States)

    2014-10-27

    Lipid Metabolism, Inborn Errors; Hypercholesterolemia, Autosomal Dominant; Hyperlipidemias; Metabolic Diseases; Hyperlipoproteinemia Type II; Metabolism, Inborn Errors; Genetic Diseases, Inborn; Infant, Newborn, Diseases; Metabolic Disorder; Congenital Abnormalities; Hypercholesterolemia; Hyperlipoproteinemias; Dyslipidemias; Lipid Metabolism Disorders

  14. An Open-label Extension Study to Assess the Long-term Safety and Efficacy of ISIS 301012 (Mipomersen) in Patients With Familial Hypercholesterolemia or Severe-Hypercholesterolemia

    Science.gov (United States)

    2015-11-17

    Lipid Metabolism, Inborn Errors; Hypercholesterolemia, Autosomal Dominant; Hyperlipidemias; Metabolic Diseases; Hyperlipoproteinemia Type II; Metabolism, Inborn Errors; Genetic Diseases, Inborn; Infant, Newborn, Diseases; Metabolic Disorder; Congenital Abnormalities; Hypercholesterolemia; Hyperlipoproteinemias; Dyslipidemias; Lipid Metabolism Disorders

  15. Errors

    International Nuclear Information System (INIS)

    Data indicates that about one half of all errors are skill based. Yet, most of the emphasis is focused on correcting rule and knowledge based errors leading to more programs, supervision, and training. None of this corrective action applies to the 'mental lapse' error. Skill based errors are usually committed in performing a routine and familiar task. Workers went to the wrong unit or component, or wrong something. Too often some of these errors result in reactor scrams, turbine trips, or other unwanted actuation. The workers do not need more programs, supervision, or training. They need to know when they are vulnerable and they need to know how to think. Self check can prevent errors, but only if it is practiced intellectually, and with commitment. Skill based errors are usually the result of using habits and senses instead of using our intellect. Even human factors can play a role in the cause of an error on a routine task. Personal injury also, is usually an error. Sometimes they are called accidents, but most accidents are the result of inappropriate actions. Whether we can explain it or not, cause and effect were there. A proper attitude toward risk, and a proper attitude toward danger is requisite to avoiding injury. Many personal injuries can be avoided just by attitude. Errors, based on personal experience and interviews, examines the reasons for the 'mental lapse' errors, and why some of us become injured. The paper offers corrective action without more programs, supervision, and training. It does ask you to think differently. (author)

  16. Teaching Arrangements of Carbohydrate Metabolism in Biochemistry Curriculum in Peking University Health Science Center

    Science.gov (United States)

    Chen, Hao; Ni, Ju-Hua

    2013-01-01

    Biochemistry occupies a unique place in the medical school curricula, but the teaching of biochemistry presents certain challenges. One of these challenges is facilitating students' interest in and mastery of metabolism. The many pathways and modes of regulation can be overwhelming for students to learn and difficult for professors to teach…

  17. The Effects of Space Flight on Some Liver Enzymes Concerned with Carbohydrate and Lipid Metabolism in Rats

    Science.gov (United States)

    Abraham, S.; Lin, C. Y.; Klein, H. P.; Volkmann, C.

    1978-01-01

    The activities of about 30 enzymes concerned with carbohydrate and lipid metabolism and the levels of glycogen and of individual fatty acids were measured in livers of rats ex- posed to prolonged space flight (18.5 days) aboard COSMOS 986 Biosatellite. When flight stationary, (FS) and flight centrifuged (FC) rats were compared at recovery (R(sub 0)), decrceases in the activities of glycogen phosphorylase, alpha glycerphosphate, acyl transferase, diglyceride acyl transferase, acconitase and Epsilon-phosphogluconate dehydrogenase were noted in the weightless group (FS). The significance of these findings was strengthened since all activities, showing alterations at R(sub 0), returned to normal 25 days post-flight. Differences were also seen in levels of two liver constituents. When glycogen and total fatty acids of the two groups of flight animals were determined, differences that could be attributed to reduced gravity were observed, the FS group at R(sub 0) contained, on the average, more than twice the amount of glycogen than did controls ad a remarkable shift in the ratio of palmitate to palmitoleate were noted. These metabolic alterations appear to be unique to the weightless condition. Our data justify the conclusion that centrifugation during space flight is equivalent to terrestrial gravity.

  18. Adipose tissue transcriptional response of lipid metabolism genes in growing Iberian pigs fed oleic acid v. carbohydrate enriched diets.

    Science.gov (United States)

    Benítez, R; Núñez, Y; Fernández, A; Isabel, B; Rodríguez, C; Daza, A; López-Bote, C; Silió, L; Óvilo, C

    2016-06-01

    Diet influences animal body and tissue composition due to direct deposition and to the nutrients effects on metabolism. The influence of specific nutrients on the molecular regulation of lipogenesis is not well characterized and is known to be influenced by many factors including timing and physiological status. A trial was performed to study the effects of different dietary energy sources on lipogenic genes transcription in ham adipose tissue of Iberian pigs, at different growth periods and on feeding/fasting situations. A total of 27 Iberian male pigs of 28 kg BW were allocated to two separate groups and fed with different isocaloric feeding regimens: standard diet with carbohydrates as energy source (CH) or diet enriched with high oleic sunflower oil (HO). Ham subcutaneous adipose tissue was sampled by biopsy at growing (44 kg mean BW) and finishing (100 kg mean BW) periods. The first sampling was performed on fasted animals, while the last sampling was performed twice, with animals fasted overnight and 3 h after refeeding. Effects of diet, growth period and feeding/fasting status on gene expression were explored quantifying the expression of a panel of key genes implicated in lipogenesis and lipid metabolism processes. Quantitative PCR revealed several differentially expressed genes according to diet, with similar results at both timings: RXRG, LEP and FABP5 genes were upregulated in HO group while ME1, FASN, ACACA and ELOVL6 were upregulated in CH. The diet effect on ME1 gene expression was conditional on feeding/fasting status, with the higher ME1 gene expression in CH than HO groups, observed only in fasting samples. Results are compatible with a higher de novo endogenous synthesis of fatty acids (FA) in the carbohydrate-supplemented group and a higher FA transport in the oleic acid-supplemented group. Growth period significantly affected the expression of most of the studied genes, with all but PPARG showing higher expression in finishing pigs according to

  19. Does gibberellin biosynthesis play a critical role in the growth of Lolium perenne? Evidence from a transcriptional analysis of gibberellin and carbohydrate metabolic genes after defoliation.

    Directory of Open Access Journals (Sweden)

    Qianhe eLiu

    2015-11-01

    Full Text Available Global meat and milk production depends to a large extent on grazed pastures, with Lolium perenne being the major forage grass in temperate regions. Defoliation and subsequent regrowth of leaf blades is a major and essential event with respect to L. perenne growth and productivity. Following defoliation, carbohydrates (mainly fructans and sucrose have to be mobilised from heterotrophic tissues to provide energy and carbon for regrowth of photosynthetic tissues. This mobilisation of reserve carbohydrates requires a substantial change in the expression of genes coding for enzymes involved in carbohydrate metabolism. Here we tested the hypothesis that gibberellins (GA are at the core of the processes regulating the expression of these genes. Thus, we examined the transcript profiles of genes involved in carbohydrate and GA metabolic pathways across a time course regrowth experiment. Our results show that following defoliation, the immediate reduction of carbohydrate concentrations in growing tissues is associated with a concomitant increase in the expression of genes encoding carbohydrate mobilising invertases, and was also associated with a strong decrease in the expression of fructan synthesising fructosyltransferase genes. We also show that the decrease in fructan levels is preceded by increased expression of the GA activating gene GA3-oxidase and decreased expression of the GA inactivating gene GA2-oxidase in sheaths. GA3-oxidase expression was negatively, while GA2-oxidase positively linked to sucrose concentrations. This study provides indicative evidence that gibberellins might play a role in L. perenne regrowth following defoliation and we hypothesise that there is a link between gibberellin regulation and sugar metabolism in L. perenne.

  20. Effects of Low-Carbohydrate Diets Versus Low-Fat Diets on Metabolic Risk Factors: A Meta-Analysis of Randomized Controlled Clinical Trials

    OpenAIRE

    Hu, Tian; Mills, Katherine T.; Yao, Lu; Demanelis, Kathryn; Eloustaz, Mohamed; Yancy, William S; Kelly, Tanika N.; He, Jiang; Bazzano, Lydia A.

    2012-01-01

    The effects of low-carbohydrate diets (≤45% of energy from carbohydrates) versus low-fat diets (≤30% of energy from fat) on metabolic risk factors were compared in a meta-analysis of randomized controlled trials. Twenty-three trials from multiple countries with a total of 2,788 participants met the predetermined eligibility criteria (from January 1, 1966 to June 20, 2011) and were included in the analyses. Data abstraction was conducted in duplicate by independent investigators. Both low-carb...

  1. Regulation of carbohydrate metabolism by 2,5-anhydro-D-mannitol.

    OpenAIRE

    Riquelme, P T; Wernette-Hammond, M E; Kneer, N M; Lardy, H A

    1983-01-01

    In hepatocytes isolated from fasted rats, 2,5-anhydromannitol inhibits gluconeogenesis from lactate plus pyruvate and from substrates that enter the gluconeogenic pathway as triose phosphate. This fructose analog has no effect, however, on gluconeogenesis from xylitol, a substrate that enters the pathway primarily as fructose 6-phosphate. The sensitivity of gluconeogenesis to 2,5-anhydromannitol depends on the substrate metabolized; concentrations of 2,5-anhydromannitol required for 50% inhib...

  2. Regulation of Lactobacillus plantarum contamination on the carbohydrate and energy related metabolisms of Saccharomyces cerevisiae during bioethanol fermentation.

    Science.gov (United States)

    Dong, Shi-Jun; Lin, Xiang-Hua; Li, Hao

    2015-11-01

    During the industrial bioethanol fermentation, Saccharomyces cerevisiae cells are often stressed by bacterial contaminants, especially lactic acid bacteria. Generally, lactic acid bacteria contamination can inhibit S. cerevisiae cell growth through secreting lactic acid and competing with yeast cells for micronutrients and living space. However, whether are there still any other influences of lactic acid bacteria on yeast or not? In this study, Lactobacillus plantarum ATCC 8014 was co-cultivated with S. cerevisiae S288c to mimic the L. plantarum contamination in industrial bioethanol fermentation. The contaminative L. plantarum-associated expression changes of genes involved in carbohydrate and energy related metabolisms in S. cerevisiae cells were determined by quantitative real-time polymerase chain reaction to evaluate the influence of L. plantarum on carbon source utilization and energy related metabolism in yeast cells during bioethanol fermentation. Contaminative L. plantarum influenced the expression of most of genes which are responsible for encoding key enzymes involved in glucose related metabolisms in S. cerevisiae. Specific for, contaminated L. plantarum inhibited EMP pathway but promoted TCA cycle, glyoxylate cycle, HMP, glycerol synthesis pathway, and redox pathway in S. cerevisiae cells. In the presence of L. plantarum, the carbon flux in S. cerevisiae cells was redistributed from fermentation to respiratory and more reducing power was produced to deal with the excess NADH. Moreover, L. plantarum contamination might confer higher ethanol tolerance to yeast cells through promoting accumulation of glycerol. These results also highlighted our knowledge about relationship between contaminative lactic acid bacteria and S. cerevisiae during bioethanol fermentation. PMID:26279142

  3. Metabolic profile of 4 h cycling in the field with varying amounts of carbohydrate supply.

    Science.gov (United States)

    Meyer, T; Gabriel, H H W; Auracher, M; Scharhag, J; Kindermann, W

    2003-01-01

    Several laboratory studies have demonstrated a performance-enhancing effect of carbohydrate (CHO) supplementations during endurance sessions of long duration. However, the transferability of these results to real training and competition circumstances has not been conclusively shown. Therefore, we tried to test the influence of graded CHO substitution on substrate utilization and selected physiological parameters under standardized but practically orientated field conditions. Fourteen endurance-trained male subjects [mean (SD): 25 (5) years, 72 (9) kg, .VO(2max) 67 (6) ml.min(-1).kg(-1), individual anaerobic threshold (IAT) 269 (30) W] after a stepwise increasing pre-test had to perform three 4-h endurance rides on their own bicycles with simultaneous spiroergometry: constant workload 70% IAT (monitoring by SRM-System). Before and during exercise, solutions without (0%), with 6% or 12% CHO were administered double-blindly and in randomized order (total volume: 50 ml.kg(-1)). After cessation of exercise, significant differences between 0% and both CHO concentrations were detected for blood glucose (GLU; 75 mg dl(-1) for 0% vs 101 mg dl(-1) for 6% vs 115 mg dl(-1) for 12%; PFree fatty acids (0.19 vs 0.16 vs 0.10 mmol l(-1)) and glycerol (0.41 vs 0.22 vs 0.12 mmol l(-1)) were significantly different between the endurance trials in a dose-dependent manner (both P<0.001). Lactate concentration ( P=0.42) and heart rate ( P=0.12) had no significant influence from CHO substitution. We conclude that CHO substitution during 4-h endurance training inhibits lipolysis in a dose-dependent manner and enhances aerobic glycolysis. This proves that earlier laboratory findings can be replicated under field conditions using modern portable equipment. PMID:12527974

  4. Fat and carbohydrates in the diet: Its metabolic contribution to obesity in Chilean women

    International Nuclear Information System (INIS)

    The effects of changes in dietary fatty acid composition on macronutrient oxidation were studied on controls and 6 obese women, 30-45 years old. Anthropometry, body composition, physical activity, continuous indirect calorimetry (200 min), plasma fatty acids, serum insulin and glucose were measured. The study was performed using a crossover design: baseline and three-period 2wk each: canola oil supplementation, washout and sunflower oil supplementation. Subjects were provided with commercially available oil (1 L/wk) either canola or sunflower during the corresponding period. Oil treatment produced significant modifications of plasma fatty acid profile, according to the type of oil consumed. In both groups, most of the fat provided was oxidised (ratio oxidised/intake: 79-102%) in the two dietary regimes, no differences were found between groups. Controls however, had a higher fat oxidation (mg/kg fat-free mass) with sunflower compared to canola treatment. Changes in plasma polyunsaturated/saturated ratio (P/S) from canola to sunflower treatment were not associated to fat oxidation. Changes in plasma n6/n3 ratio from canola to sunflower treatment showed a positive association with fat oxidation in controls (r=0. 72) and a negative association in obese (r=-0.79). Carbohydrate oxidation was on average 20-29% of CHO intake. Glucose oxidation was not associated to n6/n3 ratio from canola to sunflower treatment, but it was inversely correlated to PIS ratio changes in both groups. With sunflower treatment obese showed a higher CHO oxidation (mg/kg fat-free mass) associated to a greater insulin response compared to controls. This study showed that the type of oil can induce differences in substrate oxidation. Canola oil intake could be stimulated based on its smaller insulin response in subjects predisposed to hyperinsulinemia and insulin resistance. (author)

  5. Fat and carbohydrates in the diet: Its metabolic contribution to obesity in Chilean women

    International Nuclear Information System (INIS)

    It has been found that children and adults in the Chilean population are getting obese in a rapidly raising proportion. There is a cohort of children less than six years old, which are regularly controlled by the Ministry of Health. From this information and studies carried out at INTA, it is known that the prevalence is raising continuously. Unfortunately, this can not be ascertained in adults where the nutritional situation is assessed only in small groups, which are not representative of the general population. The problem with adults is that the healthy population does not attend to the medical clinics unless they are already ill. The studies conducted in Chilean adults have found that >40% of low socio-economic status (SES) women are suffering from obesity. A intriguing aspect in our situation is that although sedentarism is frequent in adult women (as a possible cause of positive energy balance), their intake is based on a high proportion of carbohydrates (CHO) but not much fat (50-70 g on average). It may be suggested that the excess CHO can be converted into fat through denovo lipogenesis but this process is less important as cause of obesity in humans. A more plausible cause of this problem is likely to be related to the diet. The oxidation hierarchy of macronutrients shows that whenever CHO and fat are available, the former will be firstly oxidised. This way, fat can be spared even when eaten in small amounts, accumulating in the mid-long term. Another important dietary aspect is provided by its fatty acids composition that according to animal studies, seems to modulate fat oxidation. In addition to these, glycemic effects of CHO eaten in combination with the same meal can further potentiate fat storage. This proposal aims to test the dietary effects mentioned above by using indirect calorimetry in tandem with stable isotopes methodologies in a group of normal weight and obese women. (author)

  6. Rare inborn errors associated with chronic hepatitis B virus infection

    DEFF Research Database (Denmark)

    Zhao, Qiang; Peng, Liang; Huang, Weijun;

    2012-01-01

    Chronic hepatitis B (CHB) is a major global health issue. The role of rare genetic variants in CHB has not been elucidated. We aimed to identify rare allelic variants predisposing to CHB. We performed exome sequencing in 50 CHB patients who had no identifiable risk factors for CHB and 40 controls...

  7. 3-Phosphoglycerate dehydrogenase deficiency: an inborn error of serine biosynthesis.

    OpenAIRE

    Jaeken, J; Detheux, M.; Van Maldergem, L; Foulon, M.; Carchon, H; van Schaftingen, E.

    1996-01-01

    Serine concentrations were markedly decreased in the cerebrospinal fluid of two brothers with congenital microcephaly, profound psychomotor retardation, hypertonia, epilepsy, growth retardation, and hypogonadism. The youngest boy also had congenital bilateral cataract. Magnetic resonance imaging of the brain showed evidence of dysmyelination. Plasma serine as well as plasma and cerebrospinal fluid glycine concentrations were also decreased but to a lesser extent. Treatment with oral serine in...

  8. [Inborn error of cholesterol biosynthesis: Smith-Lemli-Opitz syndrome].

    Science.gov (United States)

    Koczok, Katalin; V Oláh, Anna; P Szabó, Gabriella; Oláh, Éva; Török, Olga; Balogh, István

    2015-10-18

    Smith-Lemli-Opitz syndrome is an autosomal recessive mental retardation and multiple malformation syndrome caused by deficiency of the 7-dehydrocholesterol reductase, the enzyme catalyzing the last step in cholesterol biosynthesis. The authors summarize the pathophysiology, epidemiology, clinical picture, diagnostics and therapy of the disease based on a review of the international literature. Since 2004, fourteen patients have been diagnosed with Smith-Lemli-Opitz syndrome in Hungary, which suggests an underdiagnosis of the disease based upon estimated incidence data. Due to deficiency of the 7-dehydrocholesterol reductase, serum cholesterol concentration is low and 7-dehydrocholesterol concentration is elevated in blood and tissues; the latter being highly specific for the syndrome. Detection of disease-causing mutations makes the prenatal diagnosis possible. The clinical spectrum is wide, the most common symptom is syndactyly of the second and third toes. Standard therapy is cholesterol supplementation. Recent publications suggest that oxidative compounds of 7-dehydrocholesterol may play a role in the pathophysiology of the disease as well. PMID:26551309

  9. Sink filling, inulin metabolizing enzymes and carbohydrate status in field grown chicory (Cichorium intybus L.).

    Science.gov (United States)

    van Arkel, Jeroen; Vergauwen, Rudy; Sévenier, Robert; Hakkert, Johanna C; van Laere, André; Bouwmeester, Harro J; Koops, Andries J; van der Meer, Ingrid M

    2012-10-15

    Inulin is a fructose-based polymer that is isolated from chicory (Cichorium intybus L.) taproots. The degree of polymerization (DP) determines its application and hence the value of the crop. The DP is highly dependent on the field conditions and harvest time. Therefore, the present study was carried out with the objective to understand the regulation of inulin metabolism and the process that determines the chain length and inulin yield throughout the whole growing season. Metabolic aspects of inulin production and degradation in chicory were monitored in the field and under controlled conditions. The following characteristics were determined in taproots: concentrations of glucose, fructose and sucrose, the inulin mean polymer length (mDP), yield, gene expression and activity of enzymes involved in inulin metabolism. Inulin synthesis, catalyzed by sucrose:sucrose 1-fructosyltransferase (EC 2.4.1.99) (1-SST) and fructan:fructan 1-fructosyltransferase (EC 2.4.1.100) (1-FFT), started at the onset of taproot development. Inulin yield as a function of time followed a sigmoid curve reaching a maximum in November. Inulin reached a maximum mDP of about 15 in September, than gradually decreased. Based on the changes observed in the pattern of inulin accumulation, we defined three different phases in the growing season and analyzed product formation, enzyme activity and gene expression in these defined periods. The results were validated by performing experiments under controlled conditions in climate rooms. Our results show that the decrease in 1-SST that starts in June is not regulated by day length and temperature. From mid-September onwards, the mean degree of polymerization (mDP) decreased gradually although inulin yield still increased. The decrease in mDP combined with increased yield results from fructan exohydrolase activity, induced by low temperature, and the back transfer activity of 1-FFT. Overall, this study provides background information on how to improve

  10. Leptin and Adiponectin Levels in Patients with Chronic Hepatitis C with Carbohydrate and Lipid Metabolism Disorders

    Directory of Open Access Journals (Sweden)

    T. V. Antonova

    2014-01-01

    Full Text Available Aim: to analyze leptin and adiponectin serum levels in patients with chronic hepatitis C in comparison with metabolic syndrome components, biochemical features and stage of hepatitis.Materials and methods: In 93 patients with chronic HCV in age 20-55 with a few symptomatic HCV-infection and minimal liver fibrosis stage serum leptin and adiponectin was measured. Associations between leptin, adiponectin and metabolic abnormalities, biochemical features, and hepatic fibrosis were determined.Results: Abdominal obesity was revealed at 40% patients, overweight – at 41%, insulin resistance – at 36,6% cases. The leptin and adiponectin levels were within normal limits range at most patients. Patients with minimal liver fibrosis had higher index of leptin by comparison to patients with moderate and severe fibrosis (r= – 0,402, р= 0,018. In patients with HCV genotype 3a the adiponectin level was below, than in HCV genotype 1b. Patients with abdominal obesity and overweight had higher leptin and lower adiponectin indexes by comparison to patients without these metabolic abnormalities. Direct cross-correlation between the leptin level and body mass index (r=0,358, p=0,001, waist circumference (r=0,292, p=0,01; negative cross-correlation between the adiponectin level and body mass index (r=- 0,435, р <0,021, waist circumference (r=- 0,386, р =0,001 were displayed.Conclusion: Leptin and adiponectin blood levels in HCVpatientis associated with abdominal obesity and overweight. The connection of leptin level and liver fibrosis stage was revealed. Difference of adiponectin level in HCV-patients with 3a and 1b genotypes of virus was found.

  11. Influence of glycorazmulin on the parameters of carbohydrate metabolism in alloxane diabetes

    Directory of Open Access Journals (Sweden)

    Ziyoda Fayzieva

    2010-09-01

    Full Text Available There was studied effect of glycorazmulin on the morphological structure of the liver and pancreatic gland under the conditions of alloxane diabetes. The study found that that glycorazmulin eliminates pathomorphological changes that occur in alloxane diabetes in the liver and pancreatic gland, and stimulates reparative processes in these organs. The effect of this preparation is mainly directed to stimulation of the regeneration of β-cells. The elimination of histostructural changes resulted in compensation of the damaged metabolic processes in diabetes mellitus. Besides, marked increase in C-peptide in the blood is the confirmation of the insulin secretion stimulation under the effect of this preparation.

  12. Altered carbohydrate, lipid, and xenobiotic metabolism by liver from rats flown on Cosmos 1887

    Science.gov (United States)

    Merrill, A. H. Jr; Hoel, M.; Wang, E.; Mullins, R. E.; Hargrove, J. L.; Jones, D. P.; Popova, I. A.; Merrill AH, J. r. (Principal Investigator)

    1990-01-01

    To determine the possible biochemical effects of prolonged weightlessness on liver function, samples of liver from rats that had flown aboard Cosmos 1887 were analyzed for protein, glycogen, and lipids as well as the activities of a number of key enzymes involved in metabolism of these compounds and xenobiotics. Among the parameters measured, the major differences were elevations in the glycogen content and hydroxymethylglutaryl-CoA (HMG-CoA) reductase activities for the rats flown on Cosmos 1887 and decreases in the amount of microsomal cytochrome P-450 and the activities of aniline hydroxylase and ethylmorphine N-demethylase, cytochrome P-450-dependent enzymes. These results support the earlier finding of differences in these parameters and suggest that altered hepatic function could be important during spaceflight and/or the postflight recovery period.

  13. Prolonged postdiapause: influence on some indicators of carbohydrate and lipid metabolism of the red mason bee, Osmia rufa.

    Science.gov (United States)

    Dmochowska, Kamila; Giejdasz, Karol; Fliszkiewicz, Monika; Zółtowska, Krystyna

    2013-01-01

    Bees of the genus Osmia are being used in crop pollination at an increasing rate. However, a short life expectancy of adult individuals limits the feasibility of their use. Cocoons of the red mason bee, Osmia rufa L. (Hymenoptera: Megachilidae), can be stored at 4° C in a postdiapause state, and adult bees can be used for pollination outside their natural flight period. The period of storage in this form has an unfavorable influence on the survival rate, life expectancy, and fertility of the bee. It was suggested that the negative results are connected with exhaustion of energy reserves. To test this hypothesis, the present study examined the contents of protein, carbohydrates, lipids, and the activities of some enzymes, and their degradation in red mason bees that emerged in spring according to their biological clock and in summer after elongated diapause. It was found that postdiapause artificially elongated by 3 months caused significant decreases in body weight, total sugar, glycogen, lipids, and protein content in O. rufa. Glucose level was highest in bees that emerged in the summer, which was coincident with increased activities of maltase and trehalase. The activities of sucrase and cellobiase were not changed, while amylase activity was considerably decreased. The activities of triacylglycerols lipase and C2, C4, C10 carboxylesterases were highest in bees that emerged in July. Low temperatures restrict O. rufa emergence, and during prolonged postdiapause, metabolic processes lead to significant reductions of structural and energetic compounds. PMID:24219557

  14. Yuanhuapine-induced intestinal and hepatotoxicity were correlated with disturbance of amino acids, lipids, carbohydrate metabolism and gut microflora function: A rat urine metabonomic study.

    Science.gov (United States)

    Chen, Yanyan; Duan, Jin-Ao; Guo, Jianming; Shang, Erxin; Tang, Yuping; Qian, Yefei; Tao, Weiwei; Liu, Pei

    2016-07-15

    This research was designed to study metabonomic characteristics of the toxicity induced by yuanhuapine, a major bioactive diterpenoid in a well-known traditional Chinese medicine-Genkwa Flos. General observation, blood biochemistry and histopathological examination were used to reflect yuanhuapine-induced toxicity. Urine samples from rats in control and yuanhuapine treated rats were analyzed by ultra-performance liquid chromatography tandem quadrupole time-of-flight mass spectrometry (UPLC-Q-TOF/MS). Pattern recognition methods including principal components analysis (PCA), partial least-squared discriminant analysis (PLS-DA), orthogonal partial least-squared discriminant analysis (OPLS-DA) and computational system analysis were integrated to obtain comprehensive metabonomic profiling and pathways of the biological data sets. The results suggested that yuanhuapine could induce intestinal and liver damage. And 14 endogenous metabolites as biomarkers related to the amino acids metabolism, lipids metabolism, carbohydrate metabolism and gut microflora were significantly changed in the urine of yuanhuapine treated rats, which were firstly constructed the metabolomic feature profiling and metabolite interaction network of yuanhuapine-induced injury using pattern recognition methods and Ingenuity Pathway Analysis (IPA) approach. The present study showed that yuanhuapine-induced intestinal and hepatic toxicity were correlated with disturbance of amino acids metabolism, lipids metabolism, carbohydrate metabolism and gut microflora. PMID:26341729

  15. Simple and robust determination of the activity signature of key carbohydrate metabolism enzymes for physiological phenotyping in model and crop plants

    DEFF Research Database (Denmark)

    Jammer, Alexandra; Gasperl, Anna; Luschin-Ebengreuth, Nora;

    2015-01-01

    shown to be strongly associated with growth performance, crop yield, and quality, as well as stress responses. A simple, fast, and cost-effective method to determine activities for 13 key enzymes involved in carbohydrate metabolism has been established, mainly based on coupled spectrophotometric kinetic......The analysis of physiological parameters is important to understand the link between plant phenotypes and their genetic bases, and therefore is needed as an important element in the analysis of model and crop plants. The activities of enzymes involved in primary carbohydrate metabolism have been...... and adapted for a semi-high-throughput 96-well assay format. These assays proved to be robust and are thus suitable for physiological phenotyping, enabling the characterization and diagnosis of the physiological state. The potential of the determination of distinct enzyme activity signatures as part...

  16. Protective effect of bioflavonoid myricetin enhances carbohydrate metabolic enzymes and insulin signaling molecules in streptozotocin–cadmium induced diabetic nephrotoxic rats

    International Nuclear Information System (INIS)

    Diabetic nephropathy is the kidney disease that occurs as a result of diabetes. The present study was aimed to evaluate the therapeutic potential of myricetin by assaying the activities of key enzymes of carbohydrate metabolism, insulin signaling molecules and renal function markers in streptozotocin (STZ)–cadmium (Cd) induced diabetic nephrotoxic rats. After myricetin treatment schedule, blood and tissue samples were collected to determine plasma glucose, insulin, hemoglobin, glycosylated hemoglobin and renal function markers, carbohydrate metabolic enzymes in the liver and insulin signaling molecules in the pancreas and skeletal muscle. A significant increase of plasma glucose, glycosylated hemoglobin, urea, uric acid, creatinine, blood urea nitrogen (BUN), urinary albumin, glycogen phosphorylase, glucose-6-phosphatase, and fructose-1,6-bisphosphatase and a significant decrease of plasma insulin, hemoglobin, hexokinase, glucose-6-phosphate dehydrogenase, glycogen and glycogen synthase with insulin signaling molecule expression were found in the STZ–Cd induced diabetic nephrotoxic rats. The administration of myricetin significantly normalizes the carbohydrate metabolic products like glucose, glycated hemoglobin, glycogen phosphorylase and gluconeogenic enzymes and renal function markers with increase insulin, glycogen, glycogen synthase and insulin signaling molecule expression like glucose transporter-2 (GLUT-2), glucose transporter-4 (GLUT-4), insulin receptor-1 (IRS-1), insulin receptor-2 (IRS-2) and protein kinase B (PKB). Based on the data, the protective effect of myricetin was confirmed by its histological annotation of the pancreas, liver and kidney tissues. These findings suggest that myricetin improved carbohydrate metabolism which subsequently enhances glucose utilization and renal function in STZ–Cd induced diabetic nephrotoxic rats. - Highlights: • Diabetic rats are more susceptible to cadmium nephrotoxicity. • Cadmium plays as a cumulative

  17. The effect of hexavalent chromium at different pH values on the carbohydrate metabolism of Tilapia sparrmanii (Cichlidae)

    OpenAIRE

    V. Wepener; J. H. J. van Vuuren; H.H. du Preez

    1992-01-01

    The effect of hexavalent chromium at different pH values on the carbohydrate metabolism of Tilapia sparrmanii was investigated. The indicator organisms were exposed to Cr(V1) for short and long-term exposures, after which the blood glucose levels and activities of pyruvate kinase, isocitrate dehydrogenase and glucose-6-phosphate dehydrogenase were determined. It was found that short-term exposures of Cr(V1) at an acidic medium caused an increase in energy production and glucose concentrati...

  18. Protective effect of bioflavonoid myricetin enhances carbohydrate metabolic enzymes and insulin signaling molecules in streptozotocin–cadmium induced diabetic nephrotoxic rats

    Energy Technology Data Exchange (ETDEWEB)

    Kandasamy, Neelamegam; Ashokkumar, Natarajan, E-mail: npashokkumar1@gmail.com

    2014-09-01

    Diabetic nephropathy is the kidney disease that occurs as a result of diabetes. The present study was aimed to evaluate the therapeutic potential of myricetin by assaying the activities of key enzymes of carbohydrate metabolism, insulin signaling molecules and renal function markers in streptozotocin (STZ)–cadmium (Cd) induced diabetic nephrotoxic rats. After myricetin treatment schedule, blood and tissue samples were collected to determine plasma glucose, insulin, hemoglobin, glycosylated hemoglobin and renal function markers, carbohydrate metabolic enzymes in the liver and insulin signaling molecules in the pancreas and skeletal muscle. A significant increase of plasma glucose, glycosylated hemoglobin, urea, uric acid, creatinine, blood urea nitrogen (BUN), urinary albumin, glycogen phosphorylase, glucose-6-phosphatase, and fructose-1,6-bisphosphatase and a significant decrease of plasma insulin, hemoglobin, hexokinase, glucose-6-phosphate dehydrogenase, glycogen and glycogen synthase with insulin signaling molecule expression were found in the STZ–Cd induced diabetic nephrotoxic rats. The administration of myricetin significantly normalizes the carbohydrate metabolic products like glucose, glycated hemoglobin, glycogen phosphorylase and gluconeogenic enzymes and renal function markers with increase insulin, glycogen, glycogen synthase and insulin signaling molecule expression like glucose transporter-2 (GLUT-2), glucose transporter-4 (GLUT-4), insulin receptor-1 (IRS-1), insulin receptor-2 (IRS-2) and protein kinase B (PKB). Based on the data, the protective effect of myricetin was confirmed by its histological annotation of the pancreas, liver and kidney tissues. These findings suggest that myricetin improved carbohydrate metabolism which subsequently enhances glucose utilization and renal function in STZ–Cd induced diabetic nephrotoxic rats. - Highlights: • Diabetic rats are more susceptible to cadmium nephrotoxicity. • Cadmium plays as a cumulative

  19. Counting carbohydrates

    Science.gov (United States)

    Carb counting; Carbohydrate-controlled diet; Diabetic diet; Diabetes-counting carbohydrates ... goal is not to limit carbohydrates in the diet completely, but to make ... with diabetes can better control their blood sugar if they ...

  20. Surfactant Metabolism Dysfunction and Childhood Interstitial Lung Disease (chILD)

    OpenAIRE

    McFetridge, Lynne; McMorrow, Aoife; Morrison, Patrick J.; Shields, Michael D.

    2009-01-01

    Surfactant deficiency and the resultant respiratory distress syndrome (RDS) seen in preterm infants is a major cause of respiratory morbidity in this population. Until recently, the contribution of surfactant to respiratory morbidity in infancy was limited to the neonatal period. It is now recognised that inborn errors of surfactant metabolism leading to surfactant dysfunction account for around 10% of childhood interstitial lung disease (chILD). These abnormalities can be detected by blood s...

  1. Insulin and carbohydrate dysregulation.

    Science.gov (United States)

    Gelato, Marie C

    2003-04-01

    Patients with human immunodeficiency virus receiving highly active antiretroviral therapy (HAART) may experience abnormal body composition changes as well as metabolic abnormalities, including dyslipidemia, increases in triglycerides, low high-density lipoprotein cholesterol levels, and abnormal carbohydrate metabolism, ranging from insulin resistance with and without glucose intolerance to frank diabetes. Whether the body composition changes (i.e., increased visceral adiposity and fat wasting in the peripheral tissues) are linked to abnormalities in carbohydrate metabolism is unclear. The use of HAART with and without therapy with protease inhibitors (PIs) is related to carbohydrate abnormalities and changes in body composition. Regimens that include PIs appear to have a higher incidence of insulin resistance (up to 90%) and diabetes mellitus (up to 40%). The etiology of these abnormalities is not well understood; what is known about insulin and carbohydrate dysregulation with HAART is discussed. PMID:12652377

  2. Continuous light increases growth, daily carbon gain, antioxidants, and alters carbohydrate metabolism in a cultivated and a wild tomato species.

    Science.gov (United States)

    Haque, Mohammad S; Kjaer, Katrine H; Rosenqvist, Eva; Ottosen, Carl-Otto

    2015-01-01

    Cultivated tomato species develop leaf injury while grown in continuous light (CL). Growth, photosynthesis, carbohydrate metabolism and antioxidative enzyme activities of a cultivated (Solanum lycopersicum L. 'Aromata') and a wild tomato species (Solanum pimpinellifolium L.) were compared in this study aiming to analyze the species-specific differences and thermoperiod effects in responses to CL. The species were subjected to three photoperiodic treatments for 12 days in climate chambers: 16-h photoperiod with a light/dark temperature of 26/16°C (P16D10 or control); CL with a constant temperature of 23°C (P24D0); CL with a variable temperature of 26/16°C (P24D10). The results showed that both species grown in CL had higher dry matter production due to the continuous photosynthesis and a subsequent increase in carbon gain. In S. lycopersicum, the rate of photosynthesis and the maximum photochemical efficiency of photosystem II declined in CL with the development of leaf chlorosis, reduction in the leaf chlorophyll content and a higher activity of antioxidative enzymes. The normal diurnal patterns of starch and sugar were only present under control conditions. The results demonstrated that CL conditions mainly affected the photosynthetic apparatus of a cultivated species (S. lycopersicum), and to a less degree to the wild species (S. pimpinellifolium). The negative effects of the CL could be alleviated by diurnal temperature variations, but the physiological mechanisms behind these are less clear. The results also show that the genetic potential for reducing the negative effects of CL does exist in the tomato germplasm. PMID:26217371

  3. Continuous light increases growth, daily carbon gain, antioxidants and alters carbohydrate metabolism in a cultivated and a wild tomato species

    Directory of Open Access Journals (Sweden)

    Mohammad Sabibul Haque

    2015-07-01

    Full Text Available Cultivated tomato species develop leaf injury while grown in continuous light (CL. Growth, photosynthesis, carbohydrate metabolism and antioxidative enzyme activities of a cultivated (Solanum lycopersicum L. ‘Aromata’ and a wild tomato species (Solanum pimpinellifolium L. were compared in this study aiming to analyse the species-specific differences and thermoperiod effects in responses to CL. The species were subjected to three photoperiodic treatments for 12 days in climate chambers: 16-h photoperiod with a light/dark temperature of 26/16ºC (P16D10 or control; CL with a constant temperature of 23ºC (P24D0; CL with a variable temperature of 26/16ºC (P24D10. The results showed that both species grown in CL had higher dry matter production due to the continuous photosynthesis and a subsequent increase in carbon gain. In S. lycopersicum, the rate of photosynthesis and the maximum photochemical efficiency of photosystem II declined in CL with the development of leaf chlorosis, reduction in the leaf chlorophyll content and a higher activity of antioxidative enzymes. The normal diurnal patterns of starch and sugar were only present under control conditions. The results demonstrated that CL conditions mainly affected the photosynthetic apparatus of a cultivated species (S. lycopersicum, and to a less degree to the wild species (S. pimpinellifolium. The negative effects of the CL could be alleviated by diurnal temperature variations, but the physiological mechanisms behind these are less clear. The results also show that the genetic potential for reducing the negative effects of CL does exist in the tomato germplasm.

  4. Effects of recombinant human growth hormone therapy on carbohydrate, lipid and protein metabolisms of children with Turner syndrome

    Science.gov (United States)

    Qi, Weibin; Li, Shuxian; Shen, Qiong; Guo, Xiuxia; Rong, Huijuan

    2014-01-01

    Objective: To study the effect of recombinant human growth hormone (rhGH) therapy on carbohydrate, lipid and protein metabolisms of Turner syndrome (TS). Metho d s: Total 45 patients with TS admitted between Jul. 2008 and Jun. 2011 were involved in this study. All patients received the clinical evaluation of body fat, plasma lipids, proteins and oral glucose tolerance test (OGTT) before and after rhGH therapy. Results : Our results indicated a significant decrease of body fat (FAT%) from 23.56±4.21 to 18.71±2.23 but no obvious change on the level of fat mass (FM) (p>0.05) was observed after rhGH therapy. We also detected significant changes on plasma high-density lipoprotein cholesterol (HDL-C) from (1.65±0.58 mmol/L) to (2.20±0.65 mmol/L) and low-density lipoprotein cholesterol (LDH-C) from (2.55±0.55 mmol/L) to (2.10±0.54 mmol/L) after rhGH exposure. However, no statistical significance was detected on the level of plasma triglyceride (TG), cholesterol (CHO). Interestingly, the levels of plasma retinol binding protein (RbP) (32.55±4.28mg/L), transferrin (TRF) (2.95±0.40 mg/L), serum albumin (PRE) (250.00±45.50 mg/L) and albumin (propagated) (33.58±4.25 mg/L) were significantly increased. When it goes to the oral glucose tolerance test (OGTT) test, there were 10 impaired glucose tolerance (IGT) cases among all patients before and after rhGH therapy. No significant change was observed on homeostasis model assessment- insulin resistance (HOMA-IR) level during rhGH intervention. Conclusion : Abnormal lipid and protein metabolisms of the children with TS can be improved with rhGH therapy for 6 months. PMID:25097506

  5. Slow-release carbohydrates: growing evidence on metabolic responses and public health interest. Summary of the symposium held at the 12th European Nutrition Conference (FENS 2015)

    Science.gov (United States)

    Vinoy, Sophie; Laville, Martine; Feskens, Edith J M

    2016-01-01

    To draw attention to the necessity of considering differences in the digestibility of carbohydrates, and more specifically of starch, a symposium was held at the 12th European Nutrition Conference (FENS), which took place in Berlin from October 20 to 23, 2015. The purpose of this session was to present the consolidated knowledge and recent advances regarding the relationship between slow-release carbohydrates, metabolic responses, and public health issues. Three main topics were presented: 1) the definition of, sources of, and recognised interest in the glycaemic response to slowly digestible starch (SDS); 2) clinical evidence regarding the physiological effects of slow-release carbohydrates from cereal foods; and 3) interest in reducing the postprandial glycaemic response to help prevent metabolic diseases. Foods with the highest SDS content induce the lowest glycaemic responses, as the starch is protected from gelatinisation during processing. In humans, high-SDS food consumption induces slower glucose release, lower postprandial insulinaemia, and stimulation of gut hormones. Moreover, postprandial hyperglycaemia is an independent risk factor for type two diabetes mellitus (T2DM) and cardiovascular disease (CVD). Therefore, given the plausible aetiologic mechanisms, we argue that postprandial glucose levels are relevant for health and disease and represent a meaningful target for intervention, for example, through dietary factors. This symposium was organised by Mondelez International R&D. PMID:27388153

  6. Long-term consequences of small doses ionizing radiation effect on carbohydrate metabolism indices and their interrelation with incretory function on pancreas

    International Nuclear Information System (INIS)

    Upon inspection of 100 liquidators of the Chernobyl accident consequences it was established that main pathogenic factors of the damaging effect of small dose ionizing radiation is its indirect action realized through the infringement of hormonal mechanisms of metabolism regulation. Carbohydrates metabolism disorders are considered from viewpoint of the hormonal status fluctuations in the liquidators of the Chernobyl accident consequences observed in the form of basal immunoreactive insulin level reduction and simultaneous increase of counter-insular hormones concentration in blood. Potential disorders in the glucose tolerance test have been observed in 32 % of the patients, decrease of immunoreactive insulin in 89 % and also increase of counter-insular hormones concentration in 52,8 %. Interrelation of revealed hormonal and metabolic changes with radiation factor in its long-term period was established. Marked changes are caused in the organism and stress effects of the radiation factor. By means of multifractal dispersion analysis it has been found that the contribution of the radiation factor in the propagation of the described changes constitutes 86,5 %. Sulphate-ion is shown to be able to taking incorporated long-living radionuclides from the organism along with the mitigation of their toxic effect. Application of the method of glucose tolerance test estimation by the generalized criterion allows to specify risk groups of the carbohydrates metabolism disorders propagation among the liquidators followed by the endocrinological observation, hormonal status control and application of preventive measures. (author)

  7. Phylloxera (Daktulosphaira vitifoliae Fitch) alters the carbohydrate metabolism in root galls to allowing the compatible interaction with grapevine (Vitis ssp.) roots.

    Science.gov (United States)

    Griesser, Michaela; Lawo, Nora Caroline; Crespo-Martinez, Sara; Schoedl-Hummel, Katharina; Wieczorek, Krzysztof; Gorecka, Miroslawa; Liebner, Falk; Zweckmair, Thomas; Stralis Pavese, Nancy; Kreil, David; Forneck, Astrid

    2015-05-01

    Gall forming phylloxera may compete for nutrients with meristematic tissues and develop heterotrophic structures that act as carbon sinks. In this work, we studied the underlying starch metabolism, sink-source translocation of soluble sugars towards and within root galls. We demonstrated that nodosities store carbohydrates by starch accumulation and monitored the expression of genes involved in the starch metabolic. Thereby we proved that the nodosity is symplastically connected to the source tissues through its development and that the starch metabolism is significantly affected to synthesize and degrade starch within the gall. Genes required for starch biosynthesis and degradation are up-regulated. Among the carbohydrate transporters the expression of a glucose-6-phosphate translocater, one sucrose transporter and two SWEET proteins were increases, whereas hexose transporters, tonoplast monosaccharide transporter and Erd6-like sugar transporters were decreased. We found general evidence for plant response to osmotic stress in the nodosity as previously suggested for gall induction processes. We conclude that nodosities are heterogenous plant organs that accumulate starch to serve as temporary storage structure that is gradually withdrawn by phylloxera. Phylloxera transcriptionally reprograms gall tissues beyond primary metabolism and included downstream secondary processes, including response to osmotic stress. PMID:25804808

  8. Non-alcoholic fatty liver disease and the metabolic syndrome: Effects of weight loss and a review of popular diets. Are low carbohydrate diets the answer?

    Institute of Scientific and Technical Information of China (English)

    Harjot K Gill; George Y Wu

    2006-01-01

    Non-alcoholic fatty liver disease (NAFLD) encompasses a wide spectrum of fat-induced liver injury, ranging from relatively benign steatosis to cirrhosis and liver failure.The presence of obesity and insulin resistance is strongly associated with non-alcoholic fatty liver and confers on it a greater risk of histologically advanced disease. There is a growing concern in the medical profession as the prevalence of this disease continues to rise in parallel with the rise in obesity and the metabolic syndrome.Treatment options are limited and dietary weight loss is often advised. Low fat diets are difficult to adhere to and recent studies have shown the potential of low carbohydrate diets for weight loss and improving insulin resistance. Thus far, no study has evaluated the effect of low carbohydrate diets on NAFLD. Future studies will be required to address this question and others with regards to the nutritional adequacy and long-term side effects of these diets.

  9. Natural History Study of Children With Metachromatic Leukodystrophy

    Science.gov (United States)

    2016-04-19

    Lipid Metabolism Disorders; Metachromatic Leukodystrophy (MLD); Nervous System Diseases; Brain Diseases; Central Nervous System Diseases; Demyelinating Diseases; Metabolism, Inborn Errors; Genetic Diseases, Inborn; Sphingolipidoses; Hereditary Central Nervous System Demyelinating Diseases; Metabolic Inborn Brain Diseases; Lysosomal Storage Diseases; Metabolic Diseases; Sulfatidosis

  10. The Influence of Carbohydrate Status and Low Temperature on the Respiratory Metabolism of Mitochondria from Etiolated Leaves of Winter Wheat

    Directory of Open Access Journals (Sweden)

    Olga A. Borovik

    2014-12-01

    Full Text Available The separate and combined effect of sucrose (12%, 7 days and low temperature (2 °С, 7 days on the growth of plants, the content of carbohydrates in the leaves and oxidative activity of mitochondria isolated from them has been studied on the etiolated plants of winter wheat (Triticum aestivum L.. It has been shown that sucrose and low temperature cause inhibition of the growth and increasing of the carbohydrates content. Using the different oxidation substrates (malate, malate + rotenone, succinate, NADH and NADPH have been identified changes in the mitochondrial oxidative activity and the functioning of alternative oxidase and rotenone-insensitive NAD(PH dehydrogenases. It has been determined that activity of the alternative oxidase and “external” rotenone-insensitive NAD(PH dehydrogenases in the mitochondria of etiolated leaves depends on the carbohydrate status of the plant, regardless of the growth temperature.

  11. Differences between the Bud End and Stem End of Potatoes in Dry Matter Content, Starch Granule Size, and Carbohydrate Metabolic Gene Expression at the Growing and Sprouting Stages.

    Science.gov (United States)

    Liu, Bailin; Zhang, Guodong; Murphy, Agnes; De Koeyer, David; Tai, Helen; Bizimungu, Benoit; Si, Huaijun; Li, Xiu-Qing

    2016-02-10

    Potatoes usually have the tuber bud end dominance in growth during tuber bulking and in tuber sprouting, likely using carbohydrates from the tuber stem end. We hypothesized that the tuber bud end and tuber stem end coordination in carbohydrate metabolism gene expression is different between the bulking dominance and sprouting dominance of the tuber bud end. After comparing the growing tubers at harvest from a green vine and the stage that sprouts just started to emerge after storage of tubers at room temperature, we found the following: (1) Dry matter content was higher in the tuber stem end than the tuber bud end at both stages. (2) The starch granule size was larger in the tuber bud end than in the tuber stem end. (3) The tuber bud end had higher gene expression for starch synthesis but a lower gene expression of sucrose transporters than the tuber stem end during tuber growing. (4) The tuber stem end at the sprouting stage showed more active gene expression in both starch degradation and resynthesis, suggesting more active export of carbohydrates, than the tuber bud end. The results indicate that the starch accumulation mechanism in the tuber bud end was different between field growing and post-harvest sprouting tubers and that tubers already increased dry matter and average starch granule sizes in the tuber bud end prior to the rapid growth of sprouts. PMID:26760673

  12. Acclimatory responses of the Daphnia pulex proteome to environmental changes. I. Chronic exposure to hypoxia affects the oxygen transport system and carbohydrate metabolism

    Directory of Open Access Journals (Sweden)

    Madlung Johannes

    2009-04-01

    Full Text Available Abstract Background Freshwater planktonic crustaceans of the genus Daphnia show a remarkable plasticity to cope with environmental changes in oxygen concentration and temperature. One of the key proteins of adaptive gene control in Daphnia pulex under hypoxia is hemoglobin (Hb, which increases in hemolymph concentration by an order of magnitude and shows an enhanced oxygen affinity due to changes in subunit composition. To explore the full spectrum of adaptive protein expression in response to low-oxygen conditions, two-dimensional gel electrophoresis and mass spectrometry were used to analyze the proteome composition of animals acclimated to normoxia (oxygen partial pressure [Po2]: 20 kPa and hypoxia (Po2: 3 kPa, respectively. Results The comparative proteome analysis showed an up-regulation of more than 50 protein spots under hypoxia. Identification of a major share of these spots revealed acclimatory changes for Hb, glycolytic enzymes (enolase, and enzymes involved in the degradation of storage and structural carbohydrates (e.g. cellubiohydrolase. Proteolytic enzymes remained constitutively expressed on a high level. Conclusion Acclimatory adjustments of the D. pulex proteome to hypoxia included a strong induction of Hb and carbohydrate-degrading enzymes. The scenario of adaptive protein expression under environmental hypoxia can be interpreted as a process to improve oxygen transport and carbohydrate provision for the maintenance of ATP production, even during short episodes of tissue hypoxia requiring support from anaerobic metabolism.

  13. Carbohydrate metabolism and metabolic disorders in horses Metabolismo de carboidratos e disfunções metabólicas em equinos

    Directory of Open Access Journals (Sweden)

    Rhonda M. Hoffman

    2009-07-01

    Full Text Available Horses evolved consuming primarily fermentable forage carbohydrates, but forage diets have been traditionally supplemented with grain meals rich in starch and sugar in order to provide additional calories, protein and micronutrients. Starch and sugar are important for performance horses, but the consumption starch-rich meals may cause equine digestive and metabolic disorders. The critical capacity for preileal starch digestibility appears to be 0.35 to 0.4% but may be as little, depending on the source of starch. Small intestinal absorption of simple sugars is limited by the activity and expression of two classes of glucose carrier proteins, which are affected by chronic intake of hydrolyzable carbohydrate but may be sluggish to respond to abrupt changes in diet, further exacerbating the risk of overload. The most rapid fermentation occurs during starch overload or in the presence of fructans. Rapid fermentation perturbs the microbial and pH balance of the cecum and colon, favoring proliferation of Lactobacillus spp and acid production and increasing the risk of colic and laminitis. In addition to digestive disturbances, feeding grain concentrates rich in hydrolyzable carbohydrate may increase the risk of insulin resistance, which has been associated with obesity, laminitis and chronic founder, developmental orthopedic disease, and Cushing's disease in horses. This threshold concentration of starch intake may be a starting point for horse owners, feed manufacturers and veterinarians that may be claimed to be "low" enough to reduce risk in insulin resistant horses sensitive to grain-associated disorders.Equinos desenvolvem-se consumindo primordialmente os carboidratos fermentáveis das forragens, porém as dietas a base de forragens vem sendo suplementadas com dietas a base de grãos, ricas em amido e açúcar, visando fornecer adicionais calorias, proteínas e micronutrientes. Amido e açucares são importantes para os equinos atletas, porém o

  14. Doubling the CO{sub 2} concentration enhanced the activity of carbohydrate-metabolism enzymes, source carbohydrate production, photoassimilate transport, and sink strength for Opuntia ficus-indica

    Energy Technology Data Exchange (ETDEWEB)

    Wang, Ning; Nobel, P.S. [Univ. of California, Los Angeles, CA (United States)

    1996-03-01

    After exposure to a doubled CO{sub 2} concentration of 750 {mu}mol mol{sup -1} air for about 3 months, glucose and starch in the chlorenchyma of basal cladodes of Opuntia ficus-indica increased 175 and 57%, respectively, compared with the current CO{sub 2} concentration of 370 {mu}mol mol{sup -1}, but sucrose content was virtually unaffected. Doubling the CO{sub 2} concentration increased the noncturnal malate production in basal cladodes by 75%, inorganic phosphate (Pi) by 32% soluble starch synthase activity by 30%, and sucrose-Pi synthase activity by 146%, but did not affect the activity of hexokinase. Doubling CO{sub 2} accelerated phloem transport of sucrose out of the basal cladodes, resulting in a 73% higher dry weight for the daughter cladodes. Doubling CO{sub 2} increased the glucose content in 14-d-old daughter cladodes by 167%, increased nocturnal malate production by 22%, decreased total amino acid content by 61%, and increased soluble starch synthase activity by 30% and sucrose synthase activity by 62%. No downward acclimation of photosynthesis during long-term exposure to elevated CO{sub 2} concentrations occurs for O. ficus-indica, consistent with its higher source capacity and sink strength than under current CO{sub 2}. These changes apparently do not result in Pi limitation of photosynthesis or suppression of genes governing photosynthesis for this perennial Crassulacean acid metabolism species, as occur for some annual crops.

  15. UCB Transplant of Inherited Metabolic Diseases With Administration of Intrathecal UCB Derived Oligodendrocyte-Like Cells

    Science.gov (United States)

    2016-07-27

    Adrenoleukodystrophy; Batten Disease; Mucopolysaccharidosis II; Leukodystrophy, Globoid Cell; Leukodystrophy, Metachromatic; Neimann Pick Disease; Pelizaeus-Merzbacher Disease; Sandhoff Disease; Tay-Sachs Disease; Brain Diseases, Metabolic, Inborn

  16. Revealing the molecular relationship between type 2 diabetes and the metabolic changes induced by a very-low-carbohydrate low-fat ketogenic diet

    Directory of Open Access Journals (Sweden)

    Naval Jordi

    2010-12-01

    Full Text Available Abstract Background The prevalence of type 2 diabetes is increasing worldwide, accounting for 85-95% of all diagnosed cases of diabetes. Clinical trials provide evidence of benefits of low-carbohydrate ketogenic diets in terms of clinical outcomes on type 2 diabetes patients. However, the molecular events responsible for these improvements still remain unclear in spite of the high amount of knowledge on the primary mechanisms of both the diabetes and the metabolic state of ketosis. Molecular network analysis of conditions, diseases and treatments might provide new insights and help build a better understanding of clinical, metabolic and molecular relationships among physiological conditions. Accordingly, our aim is to reveal such a relationship between a ketogenic diet and type 2 diabetes through systems biology approaches. Methods Our systemic approach is based on the creation and analyses of the cell networks representing the metabolic state in a very-low-carbohydrate low-fat ketogenic diet. This global view might help identify unnoticed relationships often overlooked in molecule or process-centered studies. Results A strong relationship between the insulin resistance pathway and the ketosis main pathway was identified, providing a possible explanation for the improvement observed in clinical trials. Moreover, the map analyses permit the formulation of some hypothesis on functional relationships between the molecules involved in type 2 diabetes and induced ketosis, suggesting, for instance, a direct implication of glucose transporters or inflammatory processes. The molecular network analysis performed in the ketogenic-diet map, from the diabetes perspective, has provided insights on the potential mechanism of action, but also has opened new possibilities to study the applications of the ketogenic diet in other situations such as CNS or other metabolic dysfunctions.

  17. Carbohydrate metabolism of Xylella fastidiosa: Detection of glycolytic and pentose phosphate pathway enzymes and cloning and expression of the enolase gene

    Directory of Open Access Journals (Sweden)

    Facincani Agda Paula

    2003-01-01

    Full Text Available The objective of this work was to assess the functionality of the glycolytic pathways in the bacterium Xylella fastidiosa. To this effect, the enzymes phosphoglucose isomerase, aldolase, glyceraldehyde-3-phosphate dehydrogenase and pyruvate kinase of the glycolytic pathway, and glucose 6-phosphate dehydrogenase of the Entner-Doudoroff pathway were studied, followed by cloning and expression studies of the enolase gene and determination of its activity. These studies showed that X. fastidiosa does not use the glycolytic pathway to metabolize carbohydrates, which explains the increased duplication time of this phytopatogen. Recombinant enolase was expressed as inclusion bodies and solubilized with urea (most efficient extractor, Triton X-100, and TCA. Enolase extracted from X. fastidiosa and from chicken muscle and liver is irreversibly inactivated by urea. The purification of enolase was partial and resulted in a low yield. No enzymatic activity was detected for either recombinant and native enolases, aldolase, and glyceraldehyde-3-phosphate dehydrogenase, suggesting that X. fastidiosa uses the Entner-Doudoroff pathway to produce pyruvate. Evidence is presented supporting the idea that the regulation of genes and the presence of isoforms with regulation patterns might make it difficult to understand the metabolism of carbohydrates in X. fastidiosa.

  18. Role of a critical visceral adipose tissue threshold (CVATT) in metabolic syndrome: implications for controlling dietary carbohydrates: a review

    OpenAIRE

    Freedland ES

    2004-01-01

    Abstract There are likely many scenarios and pathways that can lead to metabolic syndrome. This paper reviews mechanisms by which the accumulation of visceral adipose tissue (VAT) may contribute to the metabolic syndrome, and explores the paradigm of a critical VAT threshold (CVATT). Exceeding the CVATT may result in a number of metabolic disturbances such as insulin resistance to glucose uptake by cells. Metabolic profiles of patients with visceral obesity may substantially improve after onl...

  19. Acarbose, lente carbohydrate, and prebiotics promote metabolic health and longevity by stimulating intestinal production of GLP-1.

    Science.gov (United States)

    McCarty, Mark F; DiNicolantonio, James J

    2015-01-01

    The α-glucosidase inhibitor acarbose, which slows carbohydrate digestion and blunts postprandial rises in plasma glucose, has long been used to treat patients with type 2 diabetes or glucose intolerance. Like metformin, acarbose tends to aid weight control, postpone onset of diabetes and decrease risk for cardiovascular events. Acarbose treatment can favourably affect blood pressure, serum lipids, platelet aggregation, progression of carotid intima-media thickness and postprandial endothelial dysfunction. In mice, lifetime acarbose feeding can increase median and maximal lifespan-an effect associated with increased plasma levels of fibroblast growth factor 21 (FGF21) and decreased levels of insulin-like growth factor-I (IGF-I). There is growing reason to suspect that an upregulation of fasting and postprandial production of glucagon-like peptide-1 (GLP-1)-stemming from increased delivery of carbohydrate to L cells in the distal intestinal tract-is largely responsible for the versatile health protection conferred by acarbose. Indeed, GLP-1 exerts protective effects on vascular endothelium, the liver, the heart, pancreatic β cells, and the brain which can rationalise many of the benefits reported with acarbose. And GLP-1 may act on the liver to modulate its production of FGF21 and IGF-I, thereby promoting longevity. The benefits of acarbose are likely mimicked by diets featuring slowly-digested 'lente' carbohydrate, and by certain nutraceuticals which can slow carbohydrate absorption. Prebiotics that promote colonic generation of short-chain fatty acids represent an alternative strategy for boosting intestinal GLP-1 production. The health benefits of all these measures presumably would be potentiated by concurrent use of dipeptidyl peptidase 4 inhibitors, which slow the proteolysis of GLP-1 in the blood. PMID:25685364

  20. AMP-activated protein kinase and ATP-citrate lyase are two distinct molecular targets for ETC-1002, a novel small molecule regulator of lipid and carbohydrate metabolism.

    Science.gov (United States)

    Pinkosky, Stephen L; Filippov, Sergey; Srivastava, Rai Ajit K; Hanselman, Jeffrey C; Bradshaw, Cheryl D; Hurley, Timothy R; Cramer, Clay T; Spahr, Mark A; Brant, Ashley F; Houghton, Jacob L; Baker, Chris; Naples, Mark; Adeli, Khosrow; Newton, Roger S

    2013-01-01

    ETC-1002 (8-hydroxy-2,2,14,14-tetramethylpentadecanedioic acid) is a novel investigational drug being developed for the treatment of dyslipidemia and other cardio-metabolic risk factors. The hypolipidemic, anti-atherosclerotic, anti-obesity, and glucose-lowering properties of ETC-1002, characterized in preclinical disease models, are believed to be due to dual inhibition of sterol and fatty acid synthesis and enhanced mitochondrial long-chain fatty acid β-oxidation. However, the molecular mechanism(s) mediating these activities remained undefined. Studies described here show that ETC-1002 free acid activates AMP-activated protein kinase in a Ca(2+)/calmodulin-dependent kinase β-independent and liver kinase β 1-dependent manner, without detectable changes in adenylate energy charge. Furthermore, ETC-1002 is shown to rapidly form a CoA thioester in liver, which directly inhibits ATP-citrate lyase. These distinct molecular mechanisms are complementary in their beneficial effects on lipid and carbohydrate metabolism in vitro and in vivo. Consistent with these mechanisms, ETC-1002 treatment reduced circulating proatherogenic lipoproteins, hepatic lipids, and body weight in a hamster model of hyperlipidemia, and it reduced body weight and improved glycemic control in a mouse model of diet-induced obesity. ETC-1002 offers promise as a novel therapeutic approach to improve multiple risk factors associated with metabolic syndrome and benefit patients with cardiovascular disease. PMID:23118444

  1. The action of p-synephrine on hepatic carbohydrate metabolism and respiration occurs via both Ca(2+)-mobilization and cAMP production.

    Science.gov (United States)

    de Oliveira, Andrea Luiza; Comar, Jurandir Fernando; de Sá-Nakanishi, Anacharis Babeto; Peralta, Rosane Marina; Bracht, Adelar

    2014-03-01

    Citrus aurantium extracts, which contain large amounts of p-synephrine, are widely used for weight loss purposes and as appetite suppressants. In the liver, C. aurantium (bitter orange) extracts affect hemodynamics, carbohydrate metabolism, and oxygen uptake. The purpose of the present work was to quantify the action of p-synephrine and also to obtain indications about its mechanism of action, a task that would be difficult to accomplish with C. aurantium extracts due to their rather complex composition. The experimental system was the isolated perfused rat liver. p-Synephrine significantly stimulated glycogenolysis, glycolysis, gluconeogenesis, and oxygen uptake. The compound also increased the portal perfusion pressure and the redox state of the cytosolic NAD(+)/NADH couple. A Ca(2+)-dependency for both the hemodynamic and the metabolic effects of p-synephrine was found. p-Synephrine stimulated both cAMP overflow and the initial Ca(2+) release from the cellular stores previously labeled with (45)Ca(2+). The metabolic and hemodynamic actions of p-synephrine were strongly inhibited by α-adrenergic antagonists and moderately affected by β-adrenergic antagonists. The results allow to conclude that p-synephrine presents important metabolic and hemodynamic effects in the liver. These effects can be considered as both catabolic (glycogenolysis) and anabolic (gluconeogenesis), they are mediated by both α- and β-adrenergic signaling, require the simultaneous participation of both Ca(2+) and cAMP, and could be contributing to the overall stimulation of metabolism that usually occurs during weight loss periods. PMID:24287564

  2. Carbohydrate metabolism during long-term growth hormone (GH) treatment and after discontinuation of GH treatment in girls with Turner syndrome participating in a randomized dose-response study. Dutch Advisory Group on Growth Hormone

    NARCIS (Netherlands)

    T.C.J. Sas (Theo); S.M.P.F. de Muinck Keizer-Schrama (Sabine); Th. Stijnen (Theo); H-J. Aanstoot (Henk-Jan); S.L.S. Drop (Stenvert)

    2000-01-01

    textabstractTo assess possible side-effects of GH treatment with supraphysiological doses on carbohydrate (CH) metabolism in girls with Turner syndrome (TS) during long term GH treatment and after discontinuation of GH treatment, the results of oral glucose tolerance te

  3. Carbohydrate microarrays

    DEFF Research Database (Denmark)

    Park, Sungjin; Gildersleeve, Jeffrey C; Blixt, Klas Ola; Shin, Injae

    2012-01-01

    In the last decade, carbohydrate microarrays have been core technologies for analyzing carbohydrate-mediated recognition events in a high-throughput fashion. A number of methods have been exploited for immobilizing glycans on the solid surface in a microarray format. This microarray-based technol......In the last decade, carbohydrate microarrays have been core technologies for analyzing carbohydrate-mediated recognition events in a high-throughput fashion. A number of methods have been exploited for immobilizing glycans on the solid surface in a microarray format. This microarray......-based technology has been widely employed for rapid analysis of the glycan binding properties of lectins and antibodies, the quantitative measurements of glycan-protein interactions, detection of cells and pathogens, identification of disease-related anti-glycan antibodies for diagnosis, and fast assessment of...

  4. Energy metabolism in young mink kits (Neovison vison) affected by protein and carbohydrate level in the diet

    DEFF Research Database (Denmark)

    Hellwing, Anne Louise Frydendahl; Hansen, NE; Tauson, A-H

    The mink is a strict carnivore and mink diets usually have a high content of protein. The energy metabolism in young minks in the transition period from milk to solid food is not investigated in detail, and the protein requirement is poorly defined. The substrate oxidation can give useful informa...

  5. Expression Patterns, Activities and Carbohydrate-Metabolizing Regulation of Sucrose Phosphate Synthase, Sucrose Synthase and Neutral Invertase in Pineapple Fruit during Development and Ripening

    Science.gov (United States)

    Zhang, Xiu-Mei; Wang, Wei; Du, Li-Qing; Xie, Jiang-Hui; Yao, Yan-Li; Sun, Guang-Ming

    2012-01-01

    Differences in carbohydrate contents and metabolizing-enzyme activities were monitored in apical, medial, basal and core sections of pineapple (Ananas comosus cv. Comte de paris) during fruit development and ripening. Fructose and glucose of various sections in nearly equal amounts were the predominant sugars in the fruitlets, and had obvious differences until the fruit matured. The large rise of sucrose/hexose was accompanied by dramatic changes in sucrose phosphate synthase (SPS) and sucrose synthase (SuSy) activities. By contrast, neutral invertase (NI) activity may provide a mechanism to increase fruit sink strength by increasing hexose concentrations. Furthermore, two cDNAs of Ac-sps (accession no. GQ996582) and Ac-ni (accession no. GQ996581) were first isolated from pineapple fruits utilizing conserved amino-acid sequences. Homology alignment reveals that the amino acid sequences contain some conserved function domains. Transcription expression analysis of Ac-sps, Ac-susy and Ac-ni also indicated distinct patterns related to sugar accumulation and composition of pineapple fruits. It suggests that differential expressions of multiple gene families are necessary for sugar metabolism in various parts and developmental stages of pineapple fruit. A cycle of sucrose breakdown in the cytosol of sink tissues could be mediated through both Ac-SuSy and Ac-NI, and Ac-NI could be involved in regulating crucial steps by generating sugar signals to the cells in a temporally and spatially restricted fashion. PMID:22949808

  6. Various Terpenoids Derived from Herbal and Dietary Plants Function as PPAR Modulators and Regulate Carbohydrate and Lipid Metabolism

    OpenAIRE

    Tsuyoshi Goto; Nobuyuki Takahashi; Shizuka Hirai; Teruo Kawada

    2010-01-01

    Several herbal plants improve medical conditions. Such plants contain many bioactive phytochemicals. Terpenoids (also called “isoprenoids”) constitute one of the largest families of natural products accounting for more than 40,000 individual compounds of both primary and secondary metabolisms. In particular, terpenoids are contained in many herbal plants, and several terpenoids have been shown to be available for pharmaceutical applications, for example, artemisinin and taxol as malaria and c...

  7. Inactivation of nitrate reductase alters metabolic branching of carbohydrate fermentation in the cyanobacterium Synechococcus sp. strain PCC 7002.

    Science.gov (United States)

    Qian, Xiao; Kumaraswamy, G Kenchappa; Zhang, Shuyi; Gates, Colin; Ananyev, Gennady M; Bryant, Donald A; Dismukes, G Charles

    2016-05-01

    To produce cellular energy, cyanobacteria reduce nitrate as the preferred pathway over proton reduction (H2 evolution) by catabolizing glycogen under dark anaerobic conditions. This competition lowers H2 production by consuming a large fraction of the reducing equivalents (NADPH and NADH). To eliminate this competition, we constructed a knockout mutant of nitrate reductase, encoded by narB, in Synechococcus sp. PCC 7002. As expected, ΔnarB was able to take up intracellular nitrate but was unable to reduce it to nitrite or ammonia, and was unable to grow photoautotrophically on nitrate. During photoautotrophic growth on urea, ΔnarB significantly redirects biomass accumulation into glycogen at the expense of protein accumulation. During subsequent dark fermentation, metabolite concentrations--both the adenylate cellular energy charge (∼ATP) and the redox poise (NAD(P)H/NAD(P))--were independent of nitrate availability in ΔnarB, in contrast to the wild type (WT) control. The ΔnarB strain diverted more reducing equivalents from glycogen catabolism into reduced products, mainly H2 and d-lactate, by 6-fold (2.8% yield) and 2-fold (82.3% yield), respectively, than WT. Continuous removal of H2 from the fermentation medium (milking) further boosted net H2 production by 7-fold in ΔnarB, at the expense of less excreted lactate, resulting in a 49-fold combined increase in the net H2 evolution rate during 2 days of fermentation compared to the WT. The absence of nitrate reductase eliminated the inductive effect of nitrate addition on rerouting carbohydrate catabolism from glycolysis to the oxidative pentose phosphate (OPP) pathway, indicating that intracellular redox poise and not nitrate itself acts as the control switch for carbon flux branching between pathways. PMID:26479976

  8. Effects of sub-chronic exposure to SO{sub 2} on lipid and carbohydrate metabolism in rats

    Energy Technology Data Exchange (ETDEWEB)

    Lovati, M.R. [Institute of Pharmacological Sciences, Milan (Italy); Manzoni, C. [Institute of Pharmacological Sciences, Milan (Italy); Daldossi, M. [Institute of Pharmacological Sciences, Milan (Italy); Spolti, S. [Institute of Pharmacological Sciences, Milan (Italy); Sirtori, C.R. [Institute of Pharmacological Sciences, Milan (Italy)

    1996-01-01

    Sulfur dioxide (SO{sub 2}) is a ubiquitous air pollutant, present in low concentrations in the urban air, and in higher concentrations in the working environment. While toxicological reports on SO{sub 2} have extensively dealt with the pulmonary system, essentially no data are available on the effects of chronic exposure to this pollutant on intermediary metabolism, although some biochemical changes in lipid metabolism have been detected. The present investigation was aimed at evaluating the effects of sub-chronic exposure to SO{sub 2} on concentrations of serum lipids/lipoproteins and on glucose metabolism, in animal models of hypercholesterolemia and diabetes. A specially designed controlinert atmosphere chamber was used, where male Sprague-Dawley rats fed on either standard or cholesterol enriched (HC) diets, as well as streptozotocin diabetics, were exposed to SO{sub 2} at 5 and 10 ppm, 24 h per day for 14 days. In rats, both on a standard diet and on a HC regimen, SO{sub 2} exposure determined a significant dose-dependent increase in plasma triglycerides, up to +363% in the 10 ppm HC exposed animals. This same gas concentration significantly reduced HDL cholesterol levels. In contrast, exposure of diabetic animals to 10 ppm SO{sub 2} resulted in a fall (-41%) of plasma and liver triglycerides and in a concomitant increase (+62%) of plasma HDL cholesterol. This discrepancy could apparently be related to diverging effects of SO{sub 2} exposure on plasma insulin levels in the different animal groups. Kinetic analyses of triglyceride synthesis carried out in rats on a standard diet revealed, in exposed animals, a significant reduction in the secretory rate, in spite of the concomitant hypertriglyceridemia. These findings suggest that SO{sub 2} exposure can markedly modify major lipid and glycemic indices, also indicating a differential response in normo/hyperlipidemic versus diabetic animals. (orig.)

  9. AMP-activated protein kinase and ATP-citrate lyase are two distinct molecular targets for ETC-1002, a novel small molecule regulator of lipid and carbohydrate metabolism[S

    Science.gov (United States)

    Pinkosky, Stephen L.; Filippov, Sergey; Srivastava, Rai Ajit K.; Hanselman, Jeffrey C.; Bradshaw, Cheryl D.; Hurley, Timothy R.; Cramer, Clay T.; Spahr, Mark A.; Brant, Ashley F.; Houghton, Jacob L.; Baker, Chris; Naples, Mark; Adeli, Khosrow; Newton, Roger S.

    2013-01-01

    ETC-1002 (8-hydroxy-2,2,14,14-tetramethylpentadecanedioic acid) is a novel investigational drug being developed for the treatment of dyslipidemia and other cardio-metabolic risk factors. The hypolipidemic, anti-atherosclerotic, anti-obesity, and glucose-lowering properties of ETC-1002, characterized in preclinical disease models, are believed to be due to dual inhibition of sterol and fatty acid synthesis and enhanced mitochondrial long-chain fatty acid β-oxidation. However, the molecular mechanism(s) mediating these activities remained undefined. Studies described here show that ETC-1002 free acid activates AMP-activated protein kinase in a Ca2+/calmodulin-dependent kinase β-independent and liver kinase β 1-dependent manner, without detectable changes in adenylate energy charge. Furthermore, ETC-1002 is shown to rapidly form a CoA thioester in liver, which directly inhibits ATP-citrate lyase. These distinct molecular mechanisms are complementary in their beneficial effects on lipid and carbohydrate metabolism in vitro and in vivo. Consistent with these mechanisms, ETC-1002 treatment reduced circulating proatherogenic lipoproteins, hepatic lipids, and body weight in a hamster model of hyperlipidemia, and it reduced body weight and improved glycemic control in a mouse model of diet-induced obesity. ETC-1002 offers promise as a novel therapeutic approach to improve multiple risk factors associated with metabolic syndrome and benefit patients with cardiovascular disease. PMID:23118444

  10. Extended exenatide administration enhances lipid metabolism and exacerbates pancreatic injury in mice on a high fat, high carbohydrate diet.

    Directory of Open Access Journals (Sweden)

    Rodney Rouse

    Full Text Available This study expanded upon a previous study in mice reporting a link between exenatide treatment and exocrine pancreatic injury by demonstrating temporal and dose responses and providing an initial mechanistic hypothesis. The design of the present study included varying lengths of exenatide exposure (3, 6 weeks to 12 weeks at multiple concentrations (3, 10, or 30 µg/kg with multiple endpoints (histopathology evaluations, immunoassay for cytokines, immunostaining of the pancreas, serum chemistries and measurement of trypsin, amylase, and, lipase, and gene expression profiles. Time- and dose-dependent exocrine pancreatic injury was observed in mice on a high fat diet treated with exenatide. The morphological changes identified in the pancreas involved acinar cell injury and death (autophagy, apoptosis, necrosis, and atrophy, cell adaptations (hypertrophy and hyperplasia, and cell survival (proliferation/regeneration accompanied by varying degrees of inflammatory response leading to secondary injury in pancreatic blood vessels, ducts, and adipose tissues. Gene expression profiles indicated increased signaling for cell survival and altered lipid metabolism in exenatide treated mice. Immunohistochemistry supported gene expression findings that exenatide caused and/or exacerbated pancreatic injury in a high fat diet environment potentially by further increasing high fat diet exacerbated lipid metabolism and resulting oxidative stress. Further investigation is required to confirm these findings and determine their relevance to human disease.

  11. Structure of Bacteroides thetaiotaomicron BT2081 at 2.05 Å resolution: the first structural representative of a new protein family that may play a role in carbohydrate metabolism

    International Nuclear Information System (INIS)

    The crystal structure of BT2081 from B. thetaiotaomicron reveals a two-domain protein with a putative carbohydrate-binding site in the C-terminal domain. BT2081 from Bacteroides thetaiotaomicron (GenBank accession code NP-810994.1) is a member of a novel protein family consisting of over 160 members, most of which are found in the different classes of Bacteroidetes. Genome-context analysis lends support to the involvement of this family in carbohydrate metabolism, which plays a key role in B. thetaiotaomicron as a predominant bacterial symbiont in the human distal gut microbiome. The crystal structure of BT2081 at 2.05 Å resolution represents the first structure from this new protein family. BT2081 consists of an N-terminal domain, which adopts a β-sandwich immunoglobulin-like fold, and a larger C-terminal domain with a β-sandwich jelly-roll fold. Structural analyses reveal that both domains are similar to those found in various carbohydrate-active enzymes. The C-terminal β-jelly-roll domain contains a potential carbohydrate-binding site that is highly conserved among BT2081 homologs and is situated in the same location as the carbohydrate-binding sites that are found in structurally similar glycoside hydrolases (GHs). However, in BT2081 this site is partially occluded by surrounding loops, which results in a deep solvent-accessible pocket rather than a shallower solvent-exposed cleft

  12. ACTIVE TEACHING-LEARNING METHODOLOGY TO APPROACH CARBOHYDRATE AND LIPID METABOLISM: An interdisciplinary strategy that involved the Moodle tool in the development of Problem Based Learning

    Directory of Open Access Journals (Sweden)

    J. M.P. Borges

    2015-08-01

    Full Text Available Highlight the relevance of topics studied for professional practice and associate approaches provided by different areas of knowledge are pointed as essential aspects for significant learning. Contextualize the study of metabolic pathways, linking the clinical use and expanding the vision with the approach of cellular and molecular biology discipline was the motivation for the development of the strategy described and evaluated here. In this work, starting from the concept of active methodology of teaching and learning was developed a methodological strategy to approach the carbohydrate and lipid metabolism. This strategy included: questioning the content through the clinical case study on diabetes mellitus and hypercholesterolemia, individual and collective study in the external space the classroom with the help of Moodle tool, classroom discussion accompanied by the teacher, preparation of seminar, evaluation of the content through individual written test and evaluation of the method. Analysis of student involvement with method indicates an average frequency of 98% in the practical class of Biochemistry discipline, effective participation in the preparation of seminars, an increase of 2 points in average of individual written evaluation. As for the fact that the cases were studied in two curricular components, the answers show that 92% of students feel more compression. Only 6% of students think  unnecessary to interdisciplinary approach. As for the different steps of the method, the answers show that 99% of students consider how relevant the initial self-study and discussions in class. However, only 50% of students appreciated the use of Moodle tool. Thus, student responses indicated the perception of the effectiveness of the method for their ability to: stimulate interest in learning, stimulate the search for answers through research and the building of learning.

  13. Effect of a vitamin D3-based nutritional supplement ('Videchol') on carbohydrate metabolism of rats following chronic low dose-rate irradiation

    International Nuclear Information System (INIS)

    In this study we evaluated the effects of the administration of a vitamin D3 preparation 'Videchol' to chronically irradiated rats (1 cGy day-1) by the assessment of the activities of several glycolytic enzymes: lactic dehydrogenase (LDH) (EC 1.1.1.28), pyruvate kinase (PK) (EC 2.7.1.40) and hexokinase (HK) (EC 2.7.1.1), in populations of erythroid and myeloid bone marrow cells. Videchol treatment of irradiated rats led to the normalisation of HK and LDH activity at cumulative doses of around 30 cGy in granulocyte-monocyte cells and to normalisation of LDH and PK activities in erythroid cells starting at 20 cGy in comparison with irradiated rats who did not receive Videchol. The reaction kinetic parameters of LDH in erythrocytes changed according to the redistribution pattern of the isozymes throughout the different stages of the experiment. The administration of Videchol to irradiated rats led to a rearrangement of the LDH isozymes ratio characterised by kinetic properties more comparable to those of the controls. Thus, vitamin D3 appears to induce a normalisation of carbohydrate metabolism in rats chronically irradiated with low dose-rate ionising radiation. (author)

  14. Dietary carbohydrates and endurance exercise.

    Science.gov (United States)

    Evans, W J; Hughes, V A

    1985-05-01

    Antecedent diet can greatly influence both substrate utilization during exercise and exercise performance itself. A number of studies have convincingly demonstrated that short-term (three to seven days) adaptation to a low carbohydrate diet results in greatly reduced liver and muscle glycogen stores. While carbohydrate utilization after such a diet is reduced, the limited glycogen stores can severely limit endurance exercise performance. High carbohydrate diets on the other hand expand carbohydrate stores which can limit performance. However, long-term adaptation to a low carbohydrate diet can greatly alter muscle and whole body energy metabolism to drastically limit the oxidation of limited carbohydrate stores with no adverse effect on performance. Glycogen loading techniques can result in supercompensation of muscle stores. Exercise induced depletion of muscle glycogen is the most important single factor in this phenomenon. Following the exercise a low carbohydrate diet for two to three days after which a high carbohydrate diet is eaten seemingly has the same effect on increasing muscle glycogen stores as simply eating a high carbohydrate diet. The form of the dietary carbohydrate during glycogen loading should be high in complex carbohydrates; however, the type of dietary starch that effects the greatest rate of resynthesis has not been investigated. Rapid resynthesis of glycogen following exercise is at least in part due to increased insulin sensitivity. The enhanced glucose transport caused by the increased sensitivity provides substrate for glycogen synthase. How rapidly this enhanced sensitivity returns to pre-exercise levels in humans is uncertain.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:3993621

  15. Understanding Carbohydrates

    Science.gov (United States)

    ... Low-calorie sweeteners are also called artificial sweeteners, sugar substitutes or non-nutritive sweeteners. They can be used to sweeten food and drinks for less calories and carbohydrate when they replace sugar. Sugar and Desserts With diabetes, it's important to ...

  16. Healthy carbohydrates

    Science.gov (United States)

    Functional foods include dietary fiber consisting of health-promoting carbohydrates. We have produced novel prebiotics from orange peel and observed that they extend the shelf life of probiotic bacteria in synbiotics. Some pectic-oligosaccharides and xyloglucan-oligosaccharides also have anti-adhesi...

  17. Adherence issues in inherited metabolic disorders treated by low natural protein diets

    DEFF Research Database (Denmark)

    MaCdonald, A; van Rijn, M; Feillet, F;

    2012-01-01

    Common inborn errors of metabolism treated by low natural protein diets [amino acid (AA) disorders, organic acidemias and urea cycle disorders] are responsible for a collection of diverse clinical symptoms, each condition presenting at different ages with variable severity. Precursor...... on their neuropsychological profile. There are little data about their ability to self-manage their own diet or the success of any formal educational programs that may have been implemented. Trials conducted in non-phenylketonuria (PKU) patients are rare, and the development of specialist L-AAs for non-PKU AA disorders has...

  18. Construction of 12 EST libraries and characterization of a 12,226 EST dataset for chicory (Cichorium intybus) root, leaves and nodules in the context of carbohydrate metabolism investigation

    OpenAIRE

    Boutry Marc; Watillon Bernard; Purnelle Bénédicte; Muys Céline; Mingeot Dominique; Dauchot Nicolas; Van Cutsem Pierre

    2009-01-01

    Abstract Background The industrial chicory, Cichorium intybus, is a member of the Asteraceae family that accumulates fructan of the inulin type in its root. Inulin is a low calories sweetener, a texture agent and a health promoting ingredient due to its prebiotic properties. Average inulin chain length is a critical parameter that is genotype and temperature dependent. In the context of the study of carbohydrate metabolism and to get insight into the transcriptome of chicory root and to visua...

  19. Effects of starvation, refeeding, and insulin on energy-linked metabolic processes in catfish (Rhamdia hilarii) adapted to a carbohydrate-rich diet

    Energy Technology Data Exchange (ETDEWEB)

    Machado, C.R.; Garofalo, M.A.; Roselino, J.E.; Kettelhut, I.C.; Migliorini, R.H.

    1988-09-01

    The effects of starvation and of a short period of refeeding on energy-linked metabolic processes, as well as the effects of insulin administration, were investigated in an omnivorous fish (catfish, Rhamdia hilarii) previously adapted to a carbohydrate-rich diet. Following food deprivation blood sugar levels declined progressively to about 50% of fed values after 30 days. During the same period plasma free fatty acid (FFA) concentration increased twofold. Starvation resulted in reduced concentrations of lipid and glycogen in the liver and of glycogen, lipid, and protein in white muscle. However, taking into account the initial and final concentrations of tissue constituents, the liver weight, and the large fractions of body weight represented by muscle, it could be estimated that most of the energy utilized during starvation derived from the catabolism of muscle lipid and protein. Refeeding starved fishes for 48 hr induced several-fold increases in the rates of in vivo and in vitro incorporation of (14C)glucose into liver and muscle lipid and of (14C)glycine into liver and muscle protein. Incorporation of (14C)glucose into liver glycogen was also increased. However; refeeding did not affect the incorporation of labeled glucose into muscle glycogen, neither in vivo nor in vitro. Administration of pharmacological doses of insulin to normally fed catfishes resulted in marked increases in the in vivo incorporation of 14C from glucose into lipid and protein in both liver and muscle. In contrast, labeled glucose incorporation into muscle glycogen was not affected by insulin and label incorporation into liver glycogen was actually lower than that in noninjected controls.

  20. PANCREATIC AND EXTRA-PANCREATIC EFFECTS OF INCRETINS AND PERSPECTIVES FOR STUDYING ENTEROINSULIN HORMONAL SYSTEM DURING GESTATIONAL DISORDER OF CARBOHYDRATE METABOLISM

    Directory of Open Access Journals (Sweden)

    T. V. Saprina

    2013-01-01

    Full Text Available The absence of an ideal medicine for the treatment of patients with type 2 diabetes, that would be able to provide not only high quality and constant monitoring of glycemia without increasing body weight, with no risk of hypoglycemia, with no negative impact on the heart, kidneys, liver, but could also ensure the preservation of the secretory function of β-cells, makes scientists continue to search for new opportunities to influence the occurrence and progression of T2D.Gastric inhibitory polypeptide (GIP and glucagon-like peptide-1 (GLP-1 are the two primary incretin hormones secreted from the intestine on ingestion of glucose or nutrients to stimulate insulin secretion from pancreatic β-cells. Within the pancreas, GIP and GLP-1 together promote β-cell proliferation and inhibit apoptosis, thereby expanding pancreatic β-cell mass, while GIP enhances postprandial glucagon response and GLP-1 suppresses it. In adipose tissues, GIP but not GLP-1 facilitates fat deposition. In bone, GIP promotes bone formation while GLP-1 inhibits bone absorption. In the brain, both GIP and GLP-1 are thought to be involved in memory formation as well as the control of appetite. In addition to these differences, secretion of GIP and GLP-1 and their insulinotropic effects on β-cells have been shown to differ in patients with type 2 diabetes compared to healthy subjects.Enteroinsulin hormones' role in the development of gestational disorder of carbohydrate metabolism is poorly understood.In a review article we analyze the publications that summarize what is known about the pancreatic and extra-pancreatic GIP and GLP-1-effects compared with healthy subjects and type 2 diabetes patients. The aspects of gestational diabetes pathophysiology and the perspectives for studying enteroinsulin hormonal system during pregnancy are also discussed in the article.

  1. Effects of starvation, refeeding, and insulin on energy-linked metabolic processes in catfish (Rhamdia hilarii) adapted to a carbohydrate-rich diet

    International Nuclear Information System (INIS)

    The effects of starvation and of a short period of refeeding on energy-linked metabolic processes, as well as the effects of insulin administration, were investigated in an omnivorous fish (catfish, Rhamdia hilarii) previously adapted to a carbohydrate-rich diet. Following food deprivation blood sugar levels declined progressively to about 50% of fed values after 30 days. During the same period plasma free fatty acid (FFA) concentration increased twofold. Starvation resulted in reduced concentrations of lipid and glycogen in the liver and of glycogen, lipid, and protein in white muscle. However, taking into account the initial and final concentrations of tissue constituents, the liver weight, and the large fractions of body weight represented by muscle, it could be estimated that most of the energy utilized during starvation derived from the catabolism of muscle lipid and protein. Refeeding starved fishes for 48 hr induced several-fold increases in the rates of in vivo and in vitro incorporation of [14C]glucose into liver and muscle lipid and of [14C]glycine into liver and muscle protein. Incorporation of [14C]glucose into liver glycogen was also increased. However; refeeding did not affect the incorporation of labeled glucose into muscle glycogen, neither in vivo nor in vitro. Administration of pharmacological doses of insulin to normally fed catfishes resulted in marked increases in the in vivo incorporation of 14C from glucose into lipid and protein in both liver and muscle. In contrast, labeled glucose incorporation into muscle glycogen was not affected by insulin and label incorporation into liver glycogen was actually lower than that in noninjected controls

  2. Added value of next generation gene panel analysis for patients with elevated methylmalonic acid and no clinical diagnosis following functional studies of vitamin B12 metabolism.

    Science.gov (United States)

    Pupavac, Mihaela; Tian, Xia; Chu, Jordan; Wang, Guoli; Feng, Yanming; Chen, Stella; Fenter, Remington; Zhang, Victor W; Wang, Jing; Watkins, David; Wong, Lee-Jun; Rosenblatt, David S

    2016-03-01

    Next generation sequencing (NGS) based gene panel testing is increasingly available as a molecular diagnostic approach for inborn errors of metabolism. Over the past 40years patients have been referred to the Vitamin B12 Clinical Research Laboratory at McGill University for diagnosis of inborn errors of cobalamin metabolism by functional studies in cultured fibroblasts. DNA samples from patients in which no diagnosis was made by these studies were tested by a NGS gene panel to determine whether any molecular diagnoses could be made. 131 DNA samples from patients with elevated methylmalonic acid and no diagnosis following functional studies of cobalamin metabolism were analyzed using the 24 gene extended cobalamin metabolism NGS based panel developed by Baylor Miraca Genetics Laboratories. Gene panel testing identified two or more variants in a single gene in 16/131 patients. Eight patients had pathogenic findings, one had a finding of uncertain significance, and seven had benign findings. Of the patients with pathogenic findings, five had mutations in ACSF3, two in SUCLG1 and one in TCN2. Thus, the NGS gene panel allowed for the presumptive diagnosis of 8 additional patients for which a diagnosis was not made by the functional assays. PMID:26827111

  3. Construction of 12 EST libraries and characterization of a 12,226 EST dataset for chicory (Cichorium intybus root, leaves and nodules in the context of carbohydrate metabolism investigation

    Directory of Open Access Journals (Sweden)

    Boutry Marc

    2009-01-01

    Full Text Available Abstract Background The industrial chicory, Cichorium intybus, is a member of the Asteraceae family that accumulates fructan of the inulin type in its root. Inulin is a low calories sweetener, a texture agent and a health promoting ingredient due to its prebiotic properties. Average inulin chain length is a critical parameter that is genotype and temperature dependent. In the context of the study of carbohydrate metabolism and to get insight into the transcriptome of chicory root and to visualize temporal changes of gene expression during the growing season, we obtained and characterized 10 cDNA libraries from chicory roots regularly sampled in field during a growing season. A leaf and a nodule libraries were also obtained for comparison. Results Approximately 1,000 Expressed Sequence Tags (EST were obtained from each of twelve cDNA libraries resulting in a 12,226 EST dataset. Clustering of these ESTs returned 1,922 contigs and 4,869 singlets for a total of 6,791 putative unigenes. All ESTs were compared to public sequence databases and functionally classified. Data were specifically searched for sequences related to carbohydrate metabolism. Season wide evolution of functional classes was evaluated by comparing libraries at the level of functional categories and unigenes distribution. Conclusion This chicory EST dataset provides a season wide outlook of the genes expressed in the root and to a minor extent in leaves and nodules. The dataset contains more than 200 sequences related to carbohydrate metabolism and 3,500 new ESTs when compared to other recently released chicory EST datasets, probably because of the season wide coverage of the root samples. We believe that these sequences will contribute to accelerate research and breeding of the industrial chicory as well as of closely related species.

  4. Effect of Zinc Deficiency on Zinc and Carbohydrate Metabolism in Genetically Diabetic (C57BL/Ksj Db+/Db+) and Non-Diabetic Original Strain (C57BL/Ksj) Mice

    OpenAIRE

    KECHRID, Zine; Bouzerna, Noureddine

    2004-01-01

    Our aim was to investigate the effect of low dietary zinc intake on zinc and carbohydrate metabolism in type II diabetes. Male, 4–5-week-old, genetically diabetic (C57B/LKsJdb+/db+) and nondiabetic original strain (C57BL/KsJ) mice were fed a diet containing 1 mg of Zn/kg (low zinc groups) or 54 mg of Zn/kg (control groups) for 27 days. Food intake and body weight gain were recorded regularly. On day 28, after an overnight fast, the animals were sacrificed and blood glucose, serum in...

  5. Analysis and validation of carbohydrate three-dimensional structures

    International Nuclear Information System (INIS)

    The article summarizes the information that is gained from and the errors that are found in carbohydrate structures in the Protein Data Bank. Validation tools that can locate these errors are described. Knowledge of the three-dimensional structures of the carbohydrate molecules is indispensable for a full understanding of the molecular processes in which carbohydrates are involved, such as protein glycosylation or protein–carbohydrate interactions. The Protein Data Bank (PDB) is a valuable resource for three-dimensional structural information on glycoproteins and protein–carbohydrate complexes. Unfortunately, many carbohydrate moieties in the PDB contain inconsistencies or errors. This article gives an overview of the information that can be obtained from individual PDB entries and from statistical analyses of sets of three-dimensional structures, of typical problems that arise during the analysis of carbohydrate three-dimensional structures and of the validation tools that are currently available to scientists to evaluate the quality of these structures

  6. In vivo neuro MR spectroscopy: a non-invasive insight into cerebral metabolism

    International Nuclear Information System (INIS)

    In addition to conventional magnetic resonance imaging (MRI) for examining anatomical structure, in vivo proton magnetic resonance spectroscopy (MRS) is currently being used as a non-invasive clinical tool for monitoring altered brain metabolism. Conditions such as head injury, dementia, multiple sclerosis, tumour, stroke, epilepsy and inborn errors of metabolism are all presently being investigated with MRS. At the Centre for Magnetic Resonance, we are currently undertaking a longitudinal study of dementia progression in Alzheimer's disease (AD) utilising both MRS and volumetric MRI techniques. The aim is to identify metabolic differences between this patient group and normal older adults and to correlate these measures with cognitive function. Cerebral artrophy, or loss of brain matter, together with ventricular enlargement , or enlargement of normally occuring cavities, is clearly present on MRI exams in patients with moderate and severe AD

  7. [Carbohydrates and fiber].

    Science.gov (United States)

    Lajolo, F M; de Menezes, E W; Filisetti-Cozzi, T M

    1988-09-01

    Dietary carbohydrates comprise two fractions that may be classified as digestible, and which are useful as energy sources (simple and complex carbohydrates) and fiber, which is presumed to be of no use to the human body. There are insufficient epidemiologic data on the metabolic effects of simple carbohydrates and it is not advisable to make quantitative recommendations of intake. It is questionable to recommend in developing countries that a fixed proportion of dietary energy be derived from simple sugars, due to the high prevalence of deficient energy intake, cultural habits, and regional differences in food intake and physical activity. In relation to recommendations of complex carbohydrates, it should be considered that their absorption is influenced by many factors inherent to the individual and to the foods. Fiber is defined as a series of different substances derived from tissue structures, cellular residues and undigested chemical substances that may be partially utilized after intestinal bacteria have acted on them. There is not a clear definition of the chemical composition of fiber, but it consists mainly of polysaccharides (such as cellulose, hemicellulose and pectins), lignin and end products of the interactions of various food components. The effects of fiber, such as control of food intake, regulation of gastrointestinal transit, post-prandial blood concentrations of cholesterol, glucose and insulin, flatulence and alterations in nutrient bioavailability are due to various physical properties inherent to its chemical components. Impairment of nutrient absorption may be harmful, mainly among populations whose food intake is lower than their energy needs, and with a high fiber content. This may be particularly important in pregnant women, growing children and the elderly, and should be considered when making nutrient recommendations. A precise knowledge of fiber is also important to calculate the real energy value of foods, mainly for two reasons: 1

  8. [PULMONARY COMPLICATIONS IN CHILDREN, OPERATED ON FOR INBORN HEART FAILURES IN THE ARTIFICIAL BLOOD CIRCULATION ENVIRONMENT].

    Science.gov (United States)

    Moshkivska, L V; Nastenko, E A; Golovenko, O S; Lazoryshynets, V V

    2015-11-01

    The risk factors of pulmonary complications occurrence were analyzed in children, operated on for inborn heart failures in atrificial blood circulation environment. Pulmonary complications rate and the risk factors of their occurrence were analyzed. PMID:26939427

  9. Progress on carbohydrate metabolism regulating antioxidant capacity of postharvest Chinese bayberry fruit%糖代谢调控杨梅果实采后抗氧化活性机制研究进展

    Institute of Scientific and Technical Information of China (English)

    施丽愉; 陈伟; 苏新国; 杨震峰

    2013-01-01

    There are accumulated data indicating that the natural antioxidant compounds from Chinese bayberry fruits have biological properties which can enhance human health. Since antioxidant capacity is be-coming an important quality parameter for postharvest fruit, it is focusing on maintaining and improvement of antioxidant activity in fruit during postharvest storage. Carbohydrate metabolism is one of the most important physiological activities of postharvest fruit, and is also closely related to the biosynthesis and metabolism of anthocyanin and phenolic. This paper introduced the antioxidant properties of postharvest Chinese bayberry fruit, and focused on the research progress of carbohydrate metabolism regulating antioxidant capacity in post-harvest Chinese bayberry fruit. Moreover, this paper also indicated the future research highlights to the specific mechanism of carbohydrate metabolism in the biosynthesis and metabolism of anthocyanin and phenolic in bayberry fruit.%杨梅果实中天然抗氧化物质对人类健康的作用日益受到人们的重视,抗氧化活性的大小已成为衡量果实采后品质的一个重要指标,维持和提高果实采后抗氧化能力已成为果实采后贮运保鲜研究中的热点。糖代谢是果实采后主要的生理活动之一,与果实采后花色苷和酚类物质的代谢存在密切的联系。本文简要介绍了杨梅果实的抗氧化特性,重点综述了糖代谢调控果实采后抗氧化活性机制的研究进展,提出了糖代谢调控杨梅果实采后花色苷和酚类物质合成代谢机制的研究展望。

  10. Influence of high carbohydrate versus high fat diet in ozone induced pulmonary injury and systemic metabolic impairment in a Brown Norway (BN) rat model of healthy aging

    Science.gov (United States)

    Rationale: Air pollution has been recently linked to the increased prevalence of metabolic syndrome. It has been postulated that dietary risk factors might exacerbate air pollution-induced metabolic impairment. We have recently reported that ozone exposure induces acute systemic ...

  11. Nonalcoholic fatty liver disease: Regulation of glucose and fat metabolism in the liver by Carbohydrate Response Element Binding Protein (ChREBP) and impact of dietary influence

    OpenAIRE

    Elkatry, Haiam Omar Mohamed

    2011-01-01

    Deregulationen in der Leberlipidsynthese sind häufig mit Adipositas und Diabetes Typ 2 verbunden und daher ist ein detailliertes Verständnis der beteiligten, regulierenden Stoffwechselwege sehr wichtig, um künftig potentielle therapeutische Targets zu identifizieren. Die Leber ist der wichtigste Ort für den Kohlenhydratstoffwechsel (Glykolyse und Glykogen-Synthese) sowie Triglycerid-Synthese (Lipogenese). Carbohydrate-responsive element-binding protein (ChREBP) wurden in die Regulation durch ...

  12. Does gibberellin biosynthesis play a critical role in the growth of Lolium perenne? Evidence from a transcriptional analysis of gibberellin and carbohydrate metabolic genes after defoliation.

    OpenAIRE

    Qianhe eLiu; Jones, Chris S; Parsons, Anthony J.; Hong eXue; Susanne eRasmussen

    2015-01-01

    Global meat and milk production depends to a large extent on grazed pastures, with Lolium perenne being the major forage grass in temperate regions. Defoliation and subsequent regrowth of leaf blades is a major and essential event with respect to L. perenne growth and productivity. Following defoliation, carbohydrates (mainly fructans and sucrose) have to be mobilised from heterotrophic tissues to provide energy and carbon for regrowth of photosynthetic tissues. This mobilisation of reserve c...

  13. Does gibberellin biosynthesis play a critical role in the growth of Lolium perenne? Evidence from a transcriptional analysis of gibberellin and carbohydrate metabolic genes after defoliation

    OpenAIRE

    Liu, Qianhe; Jones, Chris S; Parsons, Anthony J.; Xue, Hong; Rasmussen, Susanne

    2015-01-01

    Global meat and milk production depends to a large extent on grazed pastures, with Lolium perenne being the major forage grass in temperate regions. Defoliation and subsequent regrowth of leaf blades is a major and essential event with respect to L. perenne growth and productivity. Following defoliation, carbohydrates (mainly fructans and sucrose) have to be mobilized from heterotrophic tissues to provide energy and carbon for regrowth of photosynthetic tissues. This mobilization of reserve c...

  14. Transaldolase Deficiency: Liver Cirrhosis Associated with a New Inborn Error in the Pentose Phosphate Pathway

    OpenAIRE

    Verhoeven, Nanda M; Huck, Jojanneke H. J.; Roos, Birthe; Struys, Eduard A.; Salomons, Gajja S; Douwes, Adriaan C.; van der Knaap, Marjo S.; Jakobs, Cornelis

    2001-01-01

    This article describes the first patient with a deficiency of transaldolase (TALDO1 [E.C.2.2.1.2]). Clinically, the patient presented with liver cirrhosis and hepatosplenomegaly during early infancy. In urine and plasma, elevated concentrations of ribitol, d-arabitol, and erythritol were found. By incubating the patient's lymphoblasts and erythrocytes with ribose-5-phosphate and subsequently analyzing phosphate sugar metabolites, we discovered a deficiency of transaldolase. Sequence analysis ...

  15. HUMAN INVASIVE DERMATOPHYTIC DISEASE IS CAUSED BY INBORN ERRORS OF CARD9

    OpenAIRE

    Lanternier, F.; Pathan, S.; Vincent, Q.; L. Liu; Cypowij, S.; Prando, C; Migaud, M.; TAIBI, L.; Ammar-Khodja, A.; Stambouli, O.; Boudghene; Guellil, B.; Jacobs, F.; Goffard, J.C; Shepers, K.

    2012-01-01

    Dermatophytic disease is an invasive, sometimes life-threatening, fungal infection caused by dermatophytes, in which there is extensive cutaneous and subcutaneous tissue involvement, frequent dissemination to the lymph nodes and occasionally to the central nervous system. This condition, which is different from banal superficial dermatophyte infection (dermatophytosis), has mostly been reported in North African consanguineous multiplex families, strongly suggesting a Mendelian genetic etiolog...

  16. Defects in leaf carbohydrate metabolism compromise acclimation to high light and lead to a high chlorophyll fluorescence phenotype in Arabidopsis thaliana

    Directory of Open Access Journals (Sweden)

    Schmitz Jessica

    2012-01-01

    Full Text Available Abstract Background We have studied the impact of carbohydrate-starvation on the acclimation response to high light using Arabidopsis thaliana double mutants strongly impaired in the day- and night path of photoassimilate export from the chloroplast. A complete knock-out mutant of the triose phosphate/phosphate translocator (TPT; tpt-2 mutant was crossed to mutants defective in (i starch biosynthesis (adg1-1, pgm1 and pgi1-1; knock-outs of ADP-glucose pyrophosphorylase, plastidial phosphoglucomutase and phosphoglucose isomerase or (ii starch mobilization (sex1-3, knock-out of glucan water dikinase as well as in (iii maltose export from the chloroplast (mex1-2. Results All double mutants were viable and indistinguishable from the wild type when grown under low light conditions, but - except for sex1-3/tpt-2 - developed a high chlorophyll fluorescence (HCF phenotype and growth retardation when grown in high light. Immunoblots of thylakoid proteins, Blue-Native gel electrophoresis and chlorophyll fluorescence emission analyses at 77 Kelvin with the adg1-1/tpt-2 double mutant revealed that HCF was linked to a specific decrease in plastome-encoded core proteins of both photosystems (with the exception of the PSII component cytochrome b559, whereas nuclear-encoded antennae (LHCs accumulated normally, but were predominantly not attached to their photosystems. Uncoupled antennae are the major cause for HCF of dark-adapted plants. Feeding of sucrose or glucose to high light-grown adg1-1/tpt-2 plants rescued the HCF- and growth phenotypes. Elevated sugar levels induce the expression of the glucose-6-phosphate/phosphate translocator2 (GPT2, which in principle could compensate for the deficiency in the TPT. A triple mutant with an additional defect in GPT2 (adg1-1/tpt-2/gpt2-1 exhibited an identical rescue of the HCF- and growth phenotype in response to sugar feeding as the adg1-1/tpt-2 double mutant, indicating that this rescue is independent from the

  17. Newborn Screening for inherited metabolic disorders; news and views

    Directory of Open Access Journals (Sweden)

    Morteza Pourfarzam

    2013-01-01

    Full Text Available Newborn screening is important for the early detection of many congenital genetic and metabolic disorders, aimed at the earliest possible recognition and management of affected newborns, to prevent the morbidity, mortality, and disabilities associated with an inherited metabolic disorder. This comprehensive system includes; testing, education, follow up, diagnosis, treatment, management, and evaluation. There are major differences among many of the disorders being considered for inclusion in newborn screening programs. In recent times, advances in laboratory technology such as tandem mass spectrometry (MS/MS, which is more specific, sensitive, reliable, and comprehensive than traditional assays, has increased the number of genetic conditions that can be diagnosed through neonatal screening programs at birth. With a single dried filter paper blood spot, MS/MS can identify more than 30 inherited metabolic disorders in around two to three minutes. Advances in the diagnosis and treatment and an increased understanding of the natural history of inborn errors of metabolism have produced pressure to implement expanded newborn screening programs in many countries. Even as many countries throughout the world have made newborn screening mandatory, in Iran, nationwide newborn screening for inherited metabolic disorders other than hypothyroidism has not been initiated, hence, there is little information about these diseases. This article aims to review the recent advances in newborn metabolic screening and its situation in Iran and other countries.

  18. Mastering ectomycorrhizal symbiosis: the impact of carbohydrates.

    Science.gov (United States)

    Nehls, Uwe

    2008-01-01

    Mycorrhiza formation is the consequence of a mutualistic interaction between certain soil fungi and plant roots that helps to overcome nutritional limitations faced by the respective partners. In symbiosis, fungi contribute to tree nutrition by means of mineral weathering and mobilization of nutrients from organic matter, and obtain plant-derived carbohydrates as a response. Support with easily degradable carbohydrates seems to be the driving force for fungi to undergo this type of interaction. As a consequence, the fungal hexose uptake capacity is strongly increased in Hartig net hyphae of the model fungi Amanita muscaria and Laccaria bicolor. Next to fast carbohydrate uptake and metabolism, storage carbohydrates are of special interest. In functional A. muscaria ectomycorrhizas, expression and activity of proteins involved in trehalose biosynthesis is mainly localized in hyphae of the Hartig net, indicating an important function of trehalose in generation of a strong carbon sink by fungal hyphae. In symbiosis, fungal partners receive up to approximately 19 times more carbohydrates from their hosts than normal leakage of the root system would cause, resulting in a strong carbohydrate demand of infected roots and, as a consequence, a more efficient plant photosynthesis. To avoid fungal parasitism, the plant seems to have developed mechanisms to control carbohydrate drain towards the fungal partner and link it to the fungus-derived mineral nutrition. In this contribution, current knowledge on fungal strategies to obtain carbohydrates from its host and plant strategies to enable, but also to control and restrict (under certain conditions), carbon transfer are summarized. PMID:18272925

  19. Production of methane from kallar grass grown on saline sodic lands: metabolism of carbohydrates, methylated amines and methane precursors during digestion of kallar grass

    International Nuclear Information System (INIS)

    Mesophilic anaerobic digestion of kallar grass on saline sodic land was carried out in batch culture at laboratory scale with enriched culture from biogas plant. Analysis were made to determine biogas volume, gas composition and distribution of volatile fatty acids. The fermentation of kallar grass treated with 2% NaOH showed an increase in digestibility from 20-50% and the conversion efficiency approached 80-95% of theoretical yield which was fold higher than the one obtained from untreated substrates. The contents of methane in bio gas was markedly low during the first day of digestion but increased from 75 to 91% at the end of 20 days retention time. Low pH values (6.2) showed the accumulation of acetic butyric and propionic acids. Addition of carbohydrates, methylated amines and other methane precursors were found to stimulate methanogensis at low concentration (0.1%) carbohydrates, (1mM) methylated amines and methane precursors as compared to their higher concentrations. The phase contrast and fluorescence microscopic examinations showed the diverse microbial population of digestor contents. (author)

  20. Effects of in ovo feeding of carbohydrates and arginine on hatchability, body weight, energy metabolism and perinatal growth in duck embryos and neonates.

    Science.gov (United States)

    Tangara, M; Chen, W; Xu, J; Huang, F R; Peng, J

    2010-10-01

    1. The objective of this study was to test the hypothesis that in ovo feeding of carbohydrates and arginine into the duck amnion may improve the glycogen store and perinatal growth. At 23 d of incubation, fertile eggs were injected with 1·2 ml of sodium chloride (NaCl), sucrose + maltose (CHO), arginine (Arg) or sucrose + maltose + arginine (CHO + Arg), with controls not injected. Body weight, liver and muscle glycogen levels, and hepatic glucose-6-phosphatase activity were determined at 25 d of incubation, at hatch, and at 3 and 7 d posthatch. 2. At hatch and 7 d of age, the body weights were greater in the in ovo-feeding treatments than the controls. Arg and CHO + Arg significantly enhanced liver glycogen level at hatch compared with controls. CHO and CHO + Arg significantly increased muscle glycogen level at 25 d of incubation over controls. CHO and Arg decreased glucose-6-phosphatase at 25 d of incubation, whereas NaCl and CHO + Arg increased glucose-6-phosphatase at hatch relative to controls. 3. In ovo feeding of carbohydrates and arginine at 23 d of incubation may improve glycogen reserves, which may, in turn, provide the energy needed for perinatal growth. PMID:21058062

  1. iTRAQ Protein Profile Differential Analysis of Dormant and Germinated Grassbur Twin Seeds Reveals that Ribosomal Synthesis and Carbohydrate Metabolism Promote Germination Possibly Through the PI3K Pathway.

    Science.gov (United States)

    Zhang, Guo-Liang; Zhu, Yue; Fu, Wei-Dong; Wang, Peng; Zhang, Rui-Hai; Zhang, Yan-Lei; Song, Zhen; Xia, Gui-Xian; Wu, Jia-He

    2016-06-01

    Grassbur is a destructive and invasive weed in pastures, and its burs can cause gastric damage to animals. The strong adaptability and reproductive potential of grassbur are partly due to a unique germination mechanism whereby twin seeds develop in a single bur: one seed germinates, but the other remains dormant. To investigate the molecular mechanism of seed germination in twin seeds, we used isobaric tags for relative and absolute quantitation (iTRAQ) to perform a dynamic proteomic analysis of germination and dormancy. A total of 1,984 proteins were identified, 161 of which were considered to be differentially accumulated. The differentially accumulated proteins comprised 102 up-regulated and 59 down-regulated proteins. These proteins were grouped into seven functional categories, ribosomal proteins being the predominant group. The authenticity and accuracy of the results were confirmed by enzyme-linked immunosorbent assay (ELISA) and quantitative real-time reverse transcription-PCR (qPCR). A dynamic proteomic analysis revealed that ribosome synthesis and carbohydrate metabolism affect seed germination possibly through the phosphoinositide 3-kinase (PI3K) pathway. As the PI3K pathway is generally activated by insulin, analyses of seeds treated with exogenous insulin by qPCR, ELISA and iTRAQ confirmed that the PI3K pathway can be activated, which suppresses dormancy and promotes germination in twin grassbur seeds. Together, these results show that the PI3K pathway may play roles in stimulating seed germination in grassbur by modulating ribosomal synthesis and carbohydrate metabolism. PMID:27296714

  2. Interaction of Some Commercial Teas with Some Carbohydrate Metabolizing Enzymes Linked with Type-2 Diabetes: A Dietary Intervention in the Prevention of Type-2 Diabetes

    Directory of Open Access Journals (Sweden)

    Ganiyu Oboh

    2014-01-01

    (3-Ethylbenzo-Thiazoline~6-sulfonate “ABTS” scavenging ability and ferric reducing antioxidant property, and inhibition of pancreatic-α-amylase and intestinal-α-glucosidase in vitro. The study revealed that GT had the highest total phenol content, ascorbic acid content, ABTS* scavenging ability, and ferric reducing ability. Furthermore, all the teas inhibited Fe2+ and sodium nitroprusside induced lipid peroxidation in pancreas, with GT having the highest inhibitory effect. Conversely, there was no significant difference (P>0.05 in the inhibitory effects of the teas on α-amylase and α-glucosidase. The antidiabetic property of the teas could be attributed to their inhibitory effect on carbohydrate hydrolyzing enzymes implicated in diabetes and their antioxidant activities.

  3. Metabolism

    Science.gov (United States)

    ... also influenced by body composition — people with more muscle and less fat generally have higher BMRs. previous continue Things That Can Go Wrong With Metabolism Most of the time your metabolism works effectively ...

  4. MR diffusion imaging and MR spectroscopy of maple syrup urine disease during acute metabolic decompensation

    Energy Technology Data Exchange (ETDEWEB)

    Jan, Wajanat; Wang, Zhiyue J. [Department of Radiology, University of Pennsylvania School of Medicine, Children' s Hospital of Philadelphia, Pennsylvania (United States); Zimmerman, Robert A. [Department of Radiology, University of Pennsylvania School of Medicine, Children' s Hospital of Philadelphia, Pennsylvania (United States); Department of Radiology, Children' s Hospital of Philadelphia, 34th Street and Civic Center Boulevard, PA 19104, Philadelphia (United States); Berry, Gerard T.; Kaplan, Paige B.; Kaye, Edward M. [Department of Pediatrics, University of Pennsylvania School of Medicine, The Children' s Hospital of Philadelphia, Philadelphia, Pennsylvania (United States)

    2003-06-01

    Maple syrup urine disease (MSUD) is an inborn error of amino acid metabolism, which affects the brain tissue resulting in impairment or death if untreated. Imaging studies have shown reversible brain edema during acute metabolic decompensation. The purpose of this paper is to describe the diffusion-weighted imaging (DWI) and spectroscopy findings during metabolic decompensation and to assess the value of these findings in the prediction of patient outcome. Six patients with the diagnosis of MSUD underwent conventional MR imaging with DWI during acute presentation with metabolic decompensation. Spectroscopy with long TE was performed in four of the six patients. Follow-up examinations were performed after clinical and metabolic recovery. DWI demonstrated marked restriction of proton diffusion compatible with cytotoxic or intramyelinic sheath edema in the brainstem, basal ganglia, thalami, cerebellar and periventricular white matter and the cerebral cortex. This was accompanied by the presence of an abnormal branched-chain amino acids (BCAA) and branched-chain alpha-keto acids (BCKA) peak at 0.9 ppm as well as elevated lactate on proton spectroscopy in all four patients. The changes in all six patients were reversed with treatment without evidence of volume loss or persistent tissue damage. The presence of cytotoxic or intramyelinic edema as evidenced by restricted water diffusion on DWI, with the presence of lactate on spectroscopy, could imply imminent cell death. However, in the context of metabolic decompensation in MSUD, it appears that changes in cell osmolarity and metabolism can reverse completely after metabolic correction. (orig.)

  5. MR diffusion imaging and MR spectroscopy of maple syrup urine disease during acute metabolic decompensation

    International Nuclear Information System (INIS)

    Maple syrup urine disease (MSUD) is an inborn error of amino acid metabolism, which affects the brain tissue resulting in impairment or death if untreated. Imaging studies have shown reversible brain edema during acute metabolic decompensation. The purpose of this paper is to describe the diffusion-weighted imaging (DWI) and spectroscopy findings during metabolic decompensation and to assess the value of these findings in the prediction of patient outcome. Six patients with the diagnosis of MSUD underwent conventional MR imaging with DWI during acute presentation with metabolic decompensation. Spectroscopy with long TE was performed in four of the six patients. Follow-up examinations were performed after clinical and metabolic recovery. DWI demonstrated marked restriction of proton diffusion compatible with cytotoxic or intramyelinic sheath edema in the brainstem, basal ganglia, thalami, cerebellar and periventricular white matter and the cerebral cortex. This was accompanied by the presence of an abnormal branched-chain amino acids (BCAA) and branched-chain alpha-keto acids (BCKA) peak at 0.9 ppm as well as elevated lactate on proton spectroscopy in all four patients. The changes in all six patients were reversed with treatment without evidence of volume loss or persistent tissue damage. The presence of cytotoxic or intramyelinic edema as evidenced by restricted water diffusion on DWI, with the presence of lactate on spectroscopy, could imply imminent cell death. However, in the context of metabolic decompensation in MSUD, it appears that changes in cell osmolarity and metabolism can reverse completely after metabolic correction. (orig.)

  6. Metabolism

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    2008255 Serum adiponectin level declines in the elderly with metabolic syndrome.WU Xiaoyan(吴晓琰),et al.Dept Geriatr,Huashan Hosp,Fudan UnivShanghai200040.Chin J Geriatr2008;27(3):164-167.Objective To investigate the correlation between ser-um adiponectin level and metabolic syndrome in the elderly·Methods Sixty-one subjects with metabolic syndrome and140age matched subjects without metabolic

  7. The use of /sup 13/C-glucose and NMR to study cerebral carbohydrate metabolism in vivo in the rat and the rhesus monkey

    International Nuclear Information System (INIS)

    This report describes some preliminary work in the development of a method for the determination of the cerebral intermediary glucose metabolism in humans in vivo. The procedure makes use of specifically labeled [/sup 13/C]glucose and NMR and incorporates some of the theoretical considerations, equations and algorithms of a method the authors proposed in a recent publication for the determination of cerebral metabolism in humans in vivo using specifically labeled /sup 11/C-glucose and positron emission transverse tomography (PETT). Both the method for PETT and NMR make use of the enrichment of carbon atoms of glutamate in the brain with the label from intravenously administered isotopic glucose. Previously, the authors proposed a scheme for human cerebral metabolism of glucose in vivo which included in the tricarboxylic acid cycle (TCA) a small metabolically active pool of glutamate, γ-aminobutyrate and succinic semialdehyde. The small pool of glutamate in the TCA cycle was considered to be in slow equilibrium with a large metabolically inactive glutamate pool in the brain

  8. Transformation with TT8 and HB12 RNAi Constructs in Model Forage (Medicago sativa, Alfalfa) Affects Carbohydrate Structure and Metabolic Characteristics in Ruminant Livestock Systems.

    Science.gov (United States)

    Li, Xinxin; Zhang, Yonggen; Hannoufa, Abdelali; Yu, Peiqiang

    2015-11-01

    Lignin, a phenylpropanoid polymer present in secondary cell walls, has a negative impact on feed digestibility. TT8 and HB12 genes were shown to have low expression levels in low-lignin tissues of alfalfa, but to date, there has been no study on the effect of down-regulation of these two genes in alfalfa on nutrient chemical profiles and availability in ruminant livestock systems. The objectives of this study were to investigate the effect of transformation of alfalfa with TT8 and HB12 RNAi constructs on carbohydrate (CHO) structure and CHO nutritive value in ruminant livestock systems. The results showed that transformation with TT8 and HB12 RNAi constructs reduced rumen, rapidly degraded CHO fractions (RDCA4, P = 0.06; RDCB1, P neutral detergent fiber (NDF) at 30 h of incubation (ivNDF30) compared to the control (P alfalfa with TT8 and HB12 RNAi constructs induced molecular structure changes. Different CHO functional groups had different sensitivities and different responses to the transformation. The CHO molecular structure changes induced by the transformation were associated with predicted CHO availability. Compared with HB12 RNAi, transformation with TT8 RNAi could improve forage quality by increasing the availability of both NDF and DM. Further study is needed on the relationship between the transformation-induced structure changes at a molecular level and nutrient utilization in ruminant livestock systems when lignification is much higher. PMID:26492548

  9. Carbohydrates in Supramolecular Chemistry.

    Science.gov (United States)

    Delbianco, Martina; Bharate, Priya; Varela-Aramburu, Silvia; Seeberger, Peter H

    2016-02-24

    Carbohydrates are involved in a variety of biological processes. The ability of sugars to form a large number of hydrogen bonds has made them important components for supramolecular chemistry. We discuss recent advances in the use of carbohydrates in supramolecular chemistry and reveal that carbohydrates are useful building blocks for the stabilization of complex architectures. Systems are presented according to the scaffold that supports the glyco-conjugate: organic macrocycles, dendrimers, nanomaterials, and polymers are considered. Glyco-conjugates can form host-guest complexes, and can self-assemble by using carbohydrate-carbohydrate interactions and other weak interactions such as π-π interactions. Finally, complex supramolecular architectures based on carbohydrate-protein interactions are discussed. PMID:26702928

  10. ACTIVE TEACHING-LEARNING METHODOLOGY TO APPROACH CARBOHYDRATE AND LIPID METABOLISM: An interdisciplinary strategy that involved the Moodle tool in the development of Problem Based Learning

    OpenAIRE

    J. M.P. Borges; R. El Bacha; Costa, M. F.; Freire, E

    2015-01-01

    Highlight the relevance of topics studied for professional practice and associate approaches provided by different areas of knowledge are pointed as essential aspects for significant learning. Contextualize the study of metabolic pathways, linking the clinical use and expanding the vision with the approach of cellular and molecular biology discipline was the motivation for the development of the strategy described and evaluated here. In this work, starting from the concept of active methodolo...

  11. WHAT CAN WE EXPECT USING ACE INHIBITOR RAMIPRIL IN PERSONS WITH HIGH CARDIOVASCULAR RISK AND EARLY DISORDERS OF CARBOHYDRATE METABOLISM? LESSONS OF DREAM TRIAL

    OpenAIRE

    M. N. Mamedov; M. B. Stroeva

    2016-01-01

    Primary prevention of diabetes in persons with high cardiovascular risk is an actual problem. Results of DREAM trial are discussed. Influence of ACE inhibitor, ramipril, on risk of diabetes onset in patients with pre-diabetes and low cardiovascular risk is focused. Metabolic effects of other groups of antihypertensive drugs and their ability to prevent diabetes onset are compared. Ramipril three years therapy resulted in normalization in glucose level but did not have effect on frequency of d...

  12. Effect of exposure to sublethal concentrations of sodium cyanide on the carbohydrate metabolism of the Indian Major Carp Labeo rohita (Hamilton, 1822

    Directory of Open Access Journals (Sweden)

    Praveen N. Dube

    2013-07-01

    Full Text Available Experiments were designed to study in-vivo effects of sodium cyanide on biochemical endpoints in the freshwater fish Labeo rohita. Fish were exposed to two sublethal concentrations (0.106 and 0.064mg/L for a period of 15 days. Levels of glycogen, pyruvate, lactate and the enzymatic activities of lactate dehydrogenase (LDH, succinate dehydrogenase (SDH, glucose-6-phosphate dehydrogenase (G6PDH, phosphorylase, alkaline phosphatase (ALP, acid phosphatase (AcP were assessed in different tissues (liver, muscle and gills. Result indicated a steady decrease in glycogen, pyruvate, SDH, ALP and AcP activity with a concomitant increase in the lactate, phosphorylase, LDH and G6PD activity in all selected tissues. The alterations in all the above biochemical parameters were significantly (p<0.05 time and dose dependent. In all the above parameters, liver pointing out the intensity of cyanide intoxication compare to muscle and gills. Study revealed change in the metabolic energy by means of altered metabolic profile of the fish. Further, these observations indicated that even sublethal concentrations of sodium cyanide might not be fully devoid of deleterious influence on metabolism in L. rohita.

  13. Carbohydrate Content in the GDM Diet: Two Views: View 1: Nutrition Therapy in Gestational Diabetes: The Case for Complex Carbohydrates.

    Science.gov (United States)

    Hernandez, Teri L

    2016-05-01

    IN BRIEF Restriction of dietary carbohydrate has been the cornerstone for treatment of gestational diabetes mellitus (GDM). However, there is evidence that a balanced liberalization of complex carbohydrate as part of an overall eating plan in GDM meets treatment goals and may mitigate maternal adipose tissue insulin resistance, both of which may promote optimal metabolic outcomes for mother and offspring. PMID:27182176

  14. Newborn screening of inherited metabolic diseases by tandem mass spectrometry%串联质谱仪在新生儿遗传代谢病筛查中的应用

    Institute of Scientific and Technical Information of China (English)

    Chun-li YU; 顾学范

    2006-01-01

    Application of TMS technology in newborn screening has resulted in major expansion of disorder panel for metabolic diseases in recent years. This automated, multiplex testing methodology detects multiple analytes from single analysis of one blood spot, which leads to detection of 30-35 disorders of amino acids, organic acids, and fatty acids metabolism. The early identification of persons affected with inborn errors of metabolism has led to unexpected discoveries related to the natural history of the disorder or options for therapy. This article summarized (1) the basic principles of this technology and methodology. (2) Current status of application of this methodology in the United States, European countries and in China. (3) The positive impacts on the public health and advances in medical genetics. Finally (4) Challenges, issues and possible solutions. The purpose of this article aimed at introducing new technology and exploring the possibilities of implementing into developing countries where medical genetics is not developed and foreseeing the possible problems and obstacles.

  15. Mitochondrial dysfunction remodels one-carbon metabolism in human cells

    Science.gov (United States)

    Bao, Xiaoyan Robert; Ong, Shao-En; Goldberger, Olga; Peng, Jun; Sharma, Rohit; Thompson, Dawn A; Vafai, Scott B; Cox, Andrew G; Marutani, Eizo; Ichinose, Fumito; Goessling, Wolfram; Regev, Aviv; Carr, Steven A; Clish, Clary B; Mootha, Vamsi K

    2016-01-01

    Mitochondrial dysfunction is associated with a spectrum of human disorders, ranging from rare, inborn errors of metabolism to common, age-associated diseases such as neurodegeneration. How these lesions give rise to diverse pathology is not well understood, partly because their proximal consequences have not been well-studied in mammalian cells. Here we provide two lines of evidence that mitochondrial respiratory chain dysfunction leads to alterations in one-carbon metabolism pathways. First, using hypothesis-generating metabolic, proteomic, and transcriptional profiling, followed by confirmatory experiments, we report that mitochondrial DNA depletion leads to an ATF4-mediated increase in serine biosynthesis and transsulfuration. Second, we show that lesioning the respiratory chain impairs mitochondrial production of formate from serine, and that in some cells, respiratory chain inhibition leads to growth defects upon serine withdrawal that are rescuable with purine or formate supplementation. Our work underscores the connection between the respiratory chain and one-carbon metabolism with implications for understanding mitochondrial pathogenesis. DOI: http://dx.doi.org/10.7554/eLife.10575.001 PMID:27307216

  16. Carbohydrate Nutrition and Team Sport Performance.

    Science.gov (United States)

    Williams, Clyde; Rollo, Ian

    2015-11-01

    The common pattern of play in 'team sports' is 'stop and go', i.e. where players perform repeated bouts of brief high-intensity exercise punctuated by lower intensity activity. Sprints are generally 2-4 s long and recovery between sprints is of variable length. Energy production during brief sprints is derived from the degradation of intra-muscular phosphocreatine and glycogen (anaerobic metabolism). Prolonged periods of multiple sprints drain muscle glycogen stores, leading to a decrease in power output and a reduction in general work rate during training and competition. The impact of dietary carbohydrate interventions on team sport performance have been typically assessed using intermittent variable-speed shuttle running over a distance of 20 m. This method has evolved to include specific work to rest ratios and skills specific to team sports such as soccer, rugby and basketball. Increasing liver and muscle carbohydrate stores before sports helps delay the onset of fatigue during prolonged intermittent variable-speed running. Carbohydrate intake during exercise, typically ingested as carbohydrate-electrolyte solutions, is also associated with improved performance. The mechanisms responsible are likely to be the availability of carbohydrate as a substrate for central and peripheral functions. Variable-speed running in hot environments is limited by the degree of hyperthermia before muscle glycogen availability becomes a significant contributor to the onset of fatigue. Finally, ingesting carbohydrate immediately after training and competition will rapidly recover liver and muscle glycogen stores. PMID:26553494

  17. Secondary psychosis induced by metabolic disorders

    Directory of Open Access Journals (Sweden)

    Olivier eBonnot

    2015-05-01

    Full Text Available Metabolic disorders are not well recognized by psychiatrists as a possible source of secondary psychoses. Inborn errors of metabolism (IEMs are not frequent. Although, their prompt diagnosis may lead to suitable treatments. IEMs are well known to paediatricians, in particular for their most serious forms, having an early expression most of the time. Recent years discoveries have unveiled later expression forms, and sometimes, very discreet first physical signs. There is a growing body of evidence that supports the hypothesis that IEMs can manifest as atypical psychiatric symptoms, even in the absence of clear neurological symptoms. In the present review, we propose a detailed overview at schizophrenia-like and autism-like symptoms that can lead practitioners to bear in mind an IEM. Other psychiatric manifestations are also found, as behavioral., cognitive, learning and mood disorders. However, they are less frequent. Ensuring an accurate IEM diagnosis, in front of these psychiatric symptoms should be a priority, in order to grant suitable and valuable treatment for these pathologies.

  18. Metabolism

    Science.gov (United States)

    ... a particular food provides to the body. A chocolate bar has more calories than an apple, so ... More Common in People With Type 1 Diabetes Metabolic Syndrome Your Child's Weight Healthy Eating Endocrine System Blood ...

  19. Inborn Stress Reactivity Shapes Adult Behavioral Consequences of Early-Life Maternal Separation Stress

    OpenAIRE

    Rana, Samir; Pugh, Phyllis C.; Jackson, Nateka; Clinton, Sarah M.; Kerman, Ilan A.

    2014-01-01

    Early-life experience strongly impacts neurodevelopment and stress susceptibility in adulthood. Maternal separation (MS), an established model of early-life adversity, has been shown to negatively impact behavioral and endocrine responses to stress in adulthood. However, the impact of MS in rats with heightened inborn stress susceptibility has not been fully explored. To address this issue we conducted MS in Wistar-Kyoto (WKY) rats, an animal model of comorbid depression and anxiety, and Wist...

  20. A Neurological Enigma: The Inborn Numerical Competence of Humans and Animals

    Science.gov (United States)

    Gross, Hans J.

    2012-03-01

    "Subitizing" means our ability to recognize and memorize object numbers precisely under conditions where counting is impossible. This is an inborn archaic process which was named after the Latin "subito" = suddenly, immediately, indicating that the objects in question are presented to test persons only for the fraction of a second in order to prevent counting. Sequential counting, however, is an outstanding cultural achievement of mankind and means to count "1, 2, 3, 4, 5, 6, 7, 8 ..." without a limit. In contrast to inborn "subitizing", counting has to be trained, beginning in our early childhood with the help of our fingers. For humans we know since 140 years that we can "subitize" only up to 4 objects correctly and that mistakes occur from 5 objects on. Similar results have been obtained for a number of non-human vertebrates from salamanders to pigeons and dolphins. To our surprise, we have detected this inborn numerical competence for the first time in case of an invertebrate, the honeybee which recognizes and memorizes 3 to 4 objects under rigorous test conditions. This common ability of humans and honeybees to "subitize" up to 4 objects correctly and the miraculous but rare ability of persons with Savant syndrome to "subitize" more than hundred objects precisely raises a number of intriguing questions concerning the evolution and the significance of this biological enigma.

  1. Relation of bud dormancy to carbohydrate metabolism in Cerasus humilis (Bge.) Sok.%休眠期欧李碳水化合物代谢与休眠关系的分析

    Institute of Scientific and Technical Information of China (English)

    李秀珍; 陈苏丹; 李天忠

    2012-01-01

    This paper is concerned on the relation of bud dormancy to carbohydrate metabolism, The annual shoots from underground stem was used as the experimental materials. The components of carbohydrates and the enzymes activities were measured in phloem and bud of dormant Cerasus humilis (Bge.) Sok. The results indicated that soluble sugar content was higher than starch in phloem and bud in dormancy period. The highest ratio of soluble sugar and starch reached to 4.77. Most sugar was sucrose and then fructose, sorbitol and glucose. A few amount of fructose in bud were detected in natural dormancy stage. But its content increased rapidly in enhanced dormancy stage and in germination period,reaching to 2.05% in germination period. The changes in enzyme activities in phloem and bud were in line with that of soluble sugar. The activity of SPS was higher than that of other enzymes, but quickly decreased in natural dormancy stage,and reached to minimum at the end of the natural dormancy. The reduction in bud and phloem was 94. 18% and 84.78% respectively. The activity of p-amylase was higher that of a-amylase. Based on the integrated analyses above,the main accumulated carbohydrate in dormant Chinese dwarf cherry was soluble sugar. The relief of natural dormancy was obviously linked with the change of sucrose and reduction of the SPS activity. Change in the fructose contents was related to the dormancy and germination of bud.%为探讨休眠期欧李碳水化合物代谢与休眠的关系,以休眠期欧李1年生基生枝为试材,采用气相色谱等方法,分析了韧皮部、芽中碳水化合物含量及其相关酶活性变化。结果表明:休眠阶段欧李韧皮部和芽中可溶性糖含量均高于淀粉,二者比值最高可达4.77,其中含量最高的为蔗糖,其次是果糖、山梨醇和葡萄糖;自然休眠期芽中检查不到果糖含量,但在被迫休眠期和芽萌动期迅速增加,至芽萌动期质量分数达2.05%;休眠期韧皮部和芽中

  2. Comparison of Carbohydrate Metabolism after Anthesis in the Leaves of Two Tomato Types%两种不同类型番茄叶片中碳水化合物代谢的比较

    Institute of Scientific and Technical Information of China (English)

    王利; 崔娜; 范海延; 苗青; 曲波; 李天来

    2012-01-01

    Two kinds of tomato Solanum chmielewskii and Micro-Tom were used to studcy the dynamic changes of carbohydrate in their leaves at different growth stages and define the carbohydrate metabolism after anthesis, providing a referance for the study of sugar metabolism of Micro-Tom and the utilization of wild tomato resources. The main results showed that the contents of fructose and glucose in the leaves of Micro -Tom were significantly 247% and 290% higher than those of Solanum chmielewskii at 25 days after anthesis and the sucrose contents in the leaves of Micro-Tom were 111%, 96% and 169% higher than those of Solanum chmielewskii at 25 days, 35 days and 45 days after anthesis, respectively; the content of starch in the leaves of Solanum chmielewskii was 142% higher than that of Micro-Tom. Activities of invertases including acid invertase and neutral invertase in Micro-Tom were 348% and 138% higher than those in Solanum chmielewskii at 25 days after anthesis; sucrose synthase in Micro-Tom was always higher than that in Solanum chmielewskii at the same stage, however, sucrose phosphate synthase in Solanum chmielewskii was always higher during the whole development of fruits.%选用野生型番茄克梅留斯基(Solanum chmielewskii)和普通栽培型番茄Micro-Tom(Solanum lycopersicum)为试验材料,比较两种不同类型番茄花后不同生长阶段叶片中碳水化合物代谢的动态变化,明确其在开花后叶片碳水化合物代谢的规律,为以Micro-Tom为试材的糖代谢研究及野生型番茄资源的利用服务.结果表明:在番茄开花后25d,叶片中果糖、葡萄糖含量Micro-Tom明显高于克梅留斯基,分别高出247%和290%;花后25,35d,45d Micro-Tom叶片中蔗糖含量高于克梅留斯基,分别高出111%、96%和169%;而克梅留斯基在花后15d,叶片中淀粉含量明显高于Micro-Tom,为142%.转化酶(酸性转化酶和中性转化酶)活性在花后25d Micro-Tom叶片明显高

  3. Neonatal seizures: the overlap between diagnosis of metabolic disorders and structural abnormalities. Case report

    Directory of Open Access Journals (Sweden)

    Freitas Alessandra

    2003-01-01

    Full Text Available Inborn metabolic errors (IME and cortical developmental malformations are uncommon etiologies of neonatal seizures, however they may represent treatable causes of refractory epilepsy and for this reason must be considered as possible etiological factors. This case report aims to demonstrate the importance of neuroimaging studies in one patient with neonatal seizures, even when there are clues pointing to a metabolic disorder. CASE REPORT: A previously healthy 14 day-old child started presenting reiterated focal motor seizures (FMS which evolved to status epilepticus. Exams showed high serum levels of ammonia and no other abnormalities. A metabolic investigation was conducted with normal results. During follow-up, the patient presented developmental delay and left side hemiparesia. Seizures remained controlled with anti-epileptic drugs for four months, followed by relapse with repetitive FMS on the left side. Temporary improvement was obtained with anti-epileptic drug adjustment. At the age of 6 months, during a new episode of status epilepticus, high ammonia levels were detected. Other metabolic exams remained normal. The child was referred to a video-electroencephalographic monitoring and continuous epileptiform discharges were recorded over the right parasagittal and midline regions, with predominance over the posterior quadrant. A new neuroimaging study was performed and displayed a malformation of cortical development. Our case illustrates that because newborns are prone to present metabolic disarrangement, an unbalance such as hyperammonemia may be a consequence of acute events and conduct to a misdiagnosis of IME.

  4. Diarrhea caused by carbohydrate malabsorption.

    Science.gov (United States)

    Hammer, Heinz F; Hammer, Johann

    2012-09-01

    This article will focus on the role of the colon in the pathogenesis of diarrhea in carbohydrate malabsorption or physiologically incomplete absorption of carbohydrates, and on the most common manifestation of carbohydrate malabsorption, lactose malabsorption. In addition, incomplete fructose absorption, the role of carbohydrate malabsorption in other malabsorptive diseases, and congenital defects that lead to malabsorption will be covered. The article concludes with a section on diagnostic tools to evaluate carbohydrate malabsorption. PMID:22917167

  5. Effect of low temperature on phytohormones and carbohydrates metabolism in Ber-muda grass%低温胁迫对狗牙根激素和碳水化合物代谢的影响

    Institute of Scientific and Technical Information of China (English)

    杨勇; 娄燕宏; 杨知建; 向佐湘; 徐庆国; 胡龙兴

    2016-01-01

    以杂交狗牙根品种天堂419,天堂328,老鹰草,运动百慕大和普通狗牙根品种保定狗牙根为试验材料,分析了人工模拟昼夜温度为适温(30℃/25℃)、亚适温(18℃/10℃)、冷害(8℃/4℃)和冻害(4℃/-4℃)等4种梯度降温冷驯化条件下,低温胁迫对狗牙根叶片细胞膜稳定性、叶绿素含量、内源激素(ABA、IAA,GA3和 tZR)以及可溶性糖、淀粉、果聚糖、总非结构性糖等碳水化合物代谢的影响。结果表明:随着温度的降低,狗牙根叶片的电导率显著升高,叶绿素含量下降,内源激素 ABA 含量升高,而 IAA,GA3和 tZR 含量均下降;碳水化合物中可溶性糖、果糖和总非结构性糖含量在5个品种中均呈不同程度的升高,但不同品种在冷驯化过程中不同温度处理下其变化差异较大,如天堂328、老鹰草和运动百慕大的淀粉含量下降;天堂419的淀粉含量变化不大,而保定狗牙根的淀粉含量则呈上升趋势。综合分析各生理指标的变化,5个狗牙根品种的抗寒能力强弱为:保定狗牙根最弱,而天堂419,老鹰草、天堂328和运动百慕大耐寒能力依次较强。低温胁迫下积累或维持较高的内源激素 ABA、GA3、IAA、tZR和碳水化合物可溶性糖和果聚糖可能是耐寒性较强的主要原因,这些代谢物的积累或维持有助于狗牙根细胞内渗透平衡和细胞膜稳定性的维持,延缓叶片的枯黄衰老和诱导抗性相关基因或蛋白的表达从而提高了狗牙根品种的抗寒能力。%To investigate the effects of modulated cold acclimation on the leaf membrane stability,chlorophyll content,endogenous hormones (ABA,IAA,GA3 ,tZR),and carbohydrate (soluble sugars,starch,fructan, total nonstructural carbohydrates)metabolism,five Bermuda grass cultivars,Tifway,Tifgreen,Tifsport, Tifeagle and Baoding were selected and treated under a range of temperatures

  6. Metabolic enzymes link morphine withdrawal with metabolic disorder

    Institute of Scientific and Technical Information of China (English)

    Xi Jiang; Jing Li; Lan Ma

    2007-01-01

    @@ Energy metabolism is a fundamental biological process that is vital for the survival of all species. Disorders in the metabolic system result in deficiency or redundancy of certain nutrients, including carbohydrates, lipids, amino acids, etc. Abnormality of the energy metabolism system leads to a number of metabolic diseases, such as the metabolic syndrome. Broadly speaking, the term "metabolic diseases" now tends to be widened to the category that refers to all diseases with metabolism disorder.

  7. Regulation of carbohydrate metabolism during Giardia encystment

    NARCIS (Netherlands)

    Vliegenthart, J.F.G.; Jarroll, E.L.; Macechko, P.T.; Steimle, P.A.; Bulik, D.; Karr, C.D.; Keulen, Harry van; Paget, P.A.

    2001-01-01

    Giardia intestinalis trophozoites encyst when they are exposed to bile. During encystment, events related to the inducible synthesis of a novel N-acetyl-d-galactosamine (GalNAc) homopolymer, occur. Within the first 6 h of encystment, mRNA for glucosamine 6-P isomerase (GPI), the first inducible enzy

  8. Defective carbohydrate metabolism in multiple sclerosis

    OpenAIRE

    Mathur, Deepali

    2015-01-01

    La esclerosis múltiple (EM) es una enfermedad crónica del sistema nervioso central (SNC) en el que episodios repetidos de inflamación (bortes), dan lugar a inflamación que conduce a la interrupción de la vaina de mielina por daños producidos en la misma. Junto a este fenómeno de inflación focal, existe una inflamación difusa en el SNC, que unida a la anterior, dará lugar a que aparezca un proceso de neurodegeneración, que será el responsable último de la afectación axonal y neuronal difusa qu...

  9. Protein,carbohydrate and lipid metabolism

    Institute of Scientific and Technical Information of China (English)

    2010-01-01

    2010295 Relation of serum leptin and adiponectin to cardiovascular risk factors in older adults:a Guangzhou biobank cohort study-CVD. BAO Bei(鲍蓓),et al. Guangzhou No 12 Hosp,Guangzhou 510620.Chin J Epidemiol 2010;31(2):121-125. Objective To study the serum leptin and adiponectin levels among relatively healthy older people and their association with

  10. A Metabolic Signature of Mitochondrial Dysfunction Revealed through a Monogenic Form of Leigh Syndrome

    Directory of Open Access Journals (Sweden)

    Julie Thompson Legault

    2015-11-01

    Full Text Available A decline in mitochondrial respiration represents the root cause of a large number of inborn errors of metabolism. It is also associated with common age-associated diseases and the aging process. To gain insight into the systemic, biochemical consequences of respiratory chain dysfunction, we performed a case-control, prospective metabolic profiling study in a genetically homogenous cohort of patients with Leigh syndrome French Canadian variant, a mitochondrial respiratory chain disease due to loss-of-function mutations in LRPPRC. We discovered 45 plasma and urinary analytes discriminating patients from controls, including classic markers of mitochondrial metabolic dysfunction (lactate and acylcarnitines, as well as unexpected markers of cardiometabolic risk (insulin and adiponectin, amino acid catabolism linked to NADH status (α-hydroxybutyrate, and NAD+ biosynthesis (kynurenine and 3-hydroxyanthranilic acid. Our study identifies systemic, metabolic pathway derangements that can lie downstream of primary mitochondrial lesions, with implications for understanding how the organelle contributes to rare and common diseases.

  11. Vida útil e metabolismo de carboidratos em raízes de mandioquinha-salsa sob refrigeração e filme de PVC Shelf life and carbohydrate metabolism of arracacha roots stored under refrigeration and PVC film

    Directory of Open Access Journals (Sweden)

    Rosilene Antonio Ribeiro

    2007-04-01

    Full Text Available O objetivo deste trabalho foi avaliar a influência da temperatura de armazenamento e do uso do filme de cloreto de polivinila (PVC sobre a perda de matéria fresca e água, incidência de danos causados por frio e metabolismo pós-colheita dos carboidratos, em raízes tuberosas de mandioquinha-salsa (Arracacia xanthorrhiza. O filme de PVC reduziu a perda de matéria fresca e manteve o teor de água das raízes, durante o armazenamento por 60 dias a 5 e 10ºC. Os danos causados por frio foram inibidos nas raízes embaladas em filme de PCV, em ambas as temperaturas de armazenamento. As baixas temperaturas induziram o acúmulo de açúcares solúveis e a degradação de amido e, para as raízes armazenadas sem PVC, o aumento do conteúdo dos açúcares solúveis foi transiente e a taxa de degradação de amido foi superior à das raízes armazenadas com PVC.The objective of this work was to evaluate the influence of the storage temperature and stretch polyvinylchloride (PVC film on the loss of fresh weight and water, on the development of chilling injury symptoms, and on the postharvest metabolism of carbohydrates, in arracacha tuber roots (Arracacia xanthorrhiza. The PVC film reduced the fresh weight loss and kept water content in the roots during 60-day storage period at 5 and 10ºC. PVC film in both storage temperatures inhibited the development of external and internal chilling injury symptoms. The low temperatures induced the increase of soluble sugar content and decrease of starch concentration, where the increase in soluble sugar was transient in roots stored without PVC film, and the rate of starch degradation was higher compared to the roots stored with PVC.

  12. Effect of Candidatus Liberibacter asiaticus Infection on Carbohydrate Metabolism in Citrus sinensis%柑橘黄龙病菌侵染对甜橙叶片糖代谢的影响

    Institute of Scientific and Technical Information of China (English)

    吴越; 苏华楠; 黄爱军; 周彦; 李中安; 刘金香; 周常勇

    2015-01-01

    具有显著相关性(P<0.05)。蔗糖分解相关基因中,蔗糖合成酶基因 SuSy 在感病不同时期的表达差异不大,变化幅度小;细胞壁酸性转化酶基因 CSCWI 在不同时期均上调表达,最高上调约7.4倍,且表达量一直维持在较高水平;与 CSCWI 相比,液泡酸性转化酶基因 CUAI1的表达水平较低。淀粉合成相关酶 ADP-葡萄糖焦磷酸化酶基因最高上调3倍左右;淀粉分解相关基因 BAM3、MEX1和 DPE2在不同时期有所下调,达到显著性差异水平(P<0.05)。【结论】柑橘黄龙病感染甜橙后,对植株叶片中光合产物的形成与运转产生影响,扰乱宿主糖代谢平衡,与宿主淀粉积累及后期症状的产生有密切关系。%[Objective]The objective of this experiment is to study the effect of Huanglongbing (HLB) infection on carbohydrate metabolism in Citrus sinensis, elucidate the relationship between HLB infection and starch accumulation, and provide a theoretical basis for HLB pathogenesis. [Method]C. sinensis were used as the experimental materials, plants were inoculated by grafting three bark pieces onto the rootstock portion of each plant. For infected plants, bark pieces were derived from trees infected with Candidatus Liberibacter asiaticus, which were confirmed positive for Ca. L. asiaticus by PCR. For non-infected plants, bark pieces were obtained from healthy trees. Three plants in good growth state were chosen for successive test, mature leaves were collected every month until October. DNA and RNA were extracted, the content of soluble sugar and starch were measured immediately, the other leaf tissue was snap-frozen with liquid nitrogen and stored at -80℃ for testing carbohydrate metabolism related key enzyme activities. At the same time, the expression levels were compared using real-time quantitative PCR analysis.[Result]The content of soluble sugar and starch tended to increase with time extended, and their peak value were respectively

  13. Carbohydrates and dietary fiber.

    Science.gov (United States)

    Suter, P M

    2005-01-01

    The most widely spread eating habit is characterized by a reduced intake of dietary fiber, an increased intake of simple sugars, a high intake of refined grain products, an altered fat composition of the diet, and a dietary pattern characterized by a high glycemic load, an increased body weight and reduced physical activity. In this chapter the effects of this eating pattern on disease risk will be outlined. There are no epidemiological studies showing that the increase of glucose, fructose or sucrose intake is directly and independently associated with an increased risk of atherosclerosis or coronary heart disease (CHD). On the other hand a large number of studies has reported a reduction of fatal and non-fatal CHD events as a function of the intake of complex carbohydrates--respectively 'dietary fiber' or selected fiber-rich food (e.g., whole grain cereals). It seems that eating too much 'fast' carbohydrate [i.e., carbohydrates with a high glycemic index (GI)] may have deleterious long-term consequences. Indeed the last decades have shown that a low fat (and consecutively high carbohydrate) diet alone is not the best strategy to combat modern diseases including atherosclerosis. Quantity and quality issues in carbohydrate nutrient content are as important as they are for fat. Multiple lines of evidence suggest that for cardiovascular disease prevention a high sugar intake should be avoided. There is growing evidence of the high impact of dietary fiber and foods with a low GI on single risk factors (e.g., lipid pattern, diabetes, inflammation, endothelial function etc.) as well as also the development of the endpoints of atherosclerosis especially CHD. PMID:16596802

  14. Hereditary fructose intolerance in Brazilian patients

    OpenAIRE

    Eugênia Ribeiro Valadares; Ana Facury da Cruz; Talita Emile Ribeiro Adelino; Viviane de Cássia Kanufre; Maria do Carmo Ribeiro; Maria Goretti Moreira Guimarães Penido; Luciano Amedee Peret Filho; Valadares, Laís Maria Santos Valadares e

    2015-01-01

    Introduction: Hereditary fructose intolerance (HFI) is a rare inborn error of carbohydrate metabolism, autosomal recessive, caused by mutations in the gene ALDOB, leading to deficiency of aldolase B. Symptoms begin in the first months of life with the introduction of complementary foods containing fructose, sucrose or sorbitol, often with vomiting, feeding problems and failure to thrive. Prolonged exposure may cause liver and kidney failure, which can lead to death. Treatment consists in remo...

  15. Endocrine manifestations related to inherited metabolic diseases in adults

    Directory of Open Access Journals (Sweden)

    Vantyghem Marie-Christine

    2012-01-01

    Full Text Available Abstract Most inborn errors of metabolism (IEM are recessive, genetically transmitted diseases and are classified into 3 main groups according to their mechanisms: cellular intoxication, energy deficiency, and defects of complex molecules. They can be associated with endocrine manifestations, which may be complications from a previously diagnosed IEM of childhood onset. More rarely, endocrinopathies can signal an IEM in adulthood, which should be suspected when an endocrine disorder is associated with multisystemic involvement (neurological, muscular, hepatic features, etc.. IEM can affect all glands, but diabetes mellitus, thyroid dysfunction and hypogonadism are the most frequent disorders. A single IEM can present with multiple endocrine dysfunctions, especially those involving energy deficiency (respiratory chain defects, and metal (hemochromatosis and storage disorders (cystinosis. Non-autoimmune diabetes mellitus, thyroid dysfunction and/or goiter and sometimes hypoparathyroidism should steer the diagnosis towards a respiratory chain defect. Hypogonadotropic hypogonadism is frequent in haemochromatosis (often associated with diabetes, whereas primary hypogonadism is reported in Alström disease and cystinosis (both associated with diabetes, the latter also with thyroid dysfunction and galactosemia. Hypogonadism is also frequent in X-linked adrenoleukodystrophy (with adrenal failure, congenital disorders of glycosylation, and Fabry and glycogen storage diseases (along with thyroid dysfunction in the first 3 and diabetes in the last. This is a new and growing field and is not yet very well recognized in adulthood despite its consequences on growth, bone metabolism and fertility. For this reason, physicians managing adult patients should be aware of these diagnoses.

  16. The case for low carbohydrate diets in diabetes management

    Directory of Open Access Journals (Sweden)

    McFarlane Samy I

    2005-07-01

    Full Text Available Abstract A low fat, high carbohydrate diet in combination with regular exercise is the traditional recommendation for treating diabetes. Compliance with these lifestyle modifications is less than satisfactory, however, and a high carbohydrate diet raises postprandial plasma glucose and insulin secretion, thereby increasing risk of CVD, hypertension, dyslipidemia, obesity and diabetes. Moreover, the current epidemic of diabetes and obesity has been, over the past three decades, accompanied by a significant decrease in fat consumption and an increase in carbohydrate consumption. This apparent failure of the traditional diet, from a public health point of view, indicates that alternative dietary approaches are needed. Because carbohydrate is the major secretagogue of insulin, some form of carbohydrate restriction is a prima facie candidate for dietary control of diabetes. Evidence from various randomized controlled trials in recent years has convinced us that such diets are safe and effective, at least in short-term. These data show low carbohydrate diets to be comparable or better than traditional low fat high carbohydrate diets for weight reduction, improvement in the dyslipidemia of diabetes and metabolic syndrome as well as control of blood pressure, postprandial glycemia and insulin secretion. Furthermore, the ability of low carbohydrate diets to reduce triglycerides and to increase HDL is of particular importance. Resistance to such strategies has been due, in part, to equating it with the popular Atkins diet. However, there are many variations and room for individual physician planning. Some form of low carbohydrate diet, in combination with exercise, is a viable option for patients with diabetes. However, the extreme reduction of carbohydrate of popular diets (

  17. Carbohydrate catabolic diversity of bifidobacteria and lactobacilli of human origin.

    Science.gov (United States)

    McLaughlin, Heather P; Motherway, Mary O'Connell; Lakshminarayanan, Bhuvaneswari; Stanton, Catherine; Paul Ross, R; Brulc, Jennifer; Menon, Ravi; O'Toole, Paul W; van Sinderen, Douwe

    2015-06-16

    Because increased proportions of particular commensal bacteria such as bifidobacteria and lactobacilli have been linked to human health through a variety of mechanisms, there is corresponding interest in identifying carbohydrates that promote growth and metabolic activity of these bacteria. We evaluated the ability of 20 carbohydrates, including several commercially available carbohydrates that are sold as prebiotic ingredients, to support growth of 32 human-derived isolates belonging to the genera Bifidobacterium and Lactobacillus, including those isolated from healthy elderly subjects. In general, bifidobacterial strains were shown to display more diverse carbohydrate utilization profiles compared to the tested Lactobacillus species, with several bifidobacterial strains capable of metabolizing xylo-oligosaccharide (XOS), arabinoxylan, maltodextrin, galactan and carbohydrates containing fructo-oligosaccharide (FOS) components. In contrast, maltodextrin, galactan, arabinogalactan and galactomannan did not support robust growth (≥0.8 OD600 nm) of any of the Lactobacillus strains assessed. Carbohydrate fermentation was variable among strains tested of the same species for both genera. This study advances our knowledge of polysaccharide utilization by human gut commensals, and provides information for the rational design of selective prebiotic food ingredients. PMID:25817019

  18. Ribose-5-Phosphate Isomerase Deficiency: New Inborn Error in the Pentose Phosphate Pathway Associated with a Slowly Progressive Leukoencephalopathy

    OpenAIRE

    Huck, Jojanneke H. J.; Verhoeven, Nanda M; Struys, Eduard A.; Salomons, Gajja S; Jakobs, Cornelis; van der Knaap, Marjo S.

    2004-01-01

    The present article describes the first patient with a deficiency of ribose-5-phosphate isomerase (RPI) (Enzyme Commission number 5.3.1.6) who presented with leukoencephalopathy and peripheral neuropathy. Proton magnetic resonance spectroscopy of the brain revealed highly elevated levels of the polyols ribitol and D-arabitol, which were subsequently also found in high concentrations in body fluids. Deficient activity of RPI, one of the pentose-phosphate-pathway (PPP) enzymes, was demonstrated...

  19. Gastric emptying, glucose metabolism and gut hormones

    DEFF Research Database (Denmark)

    Vermeulen, Mechteld A R; Richir, Milan C; Garretsen, Martijn K;

    2011-01-01

    To study the gastric-emptying rate and gut hormonal response of two carbohydrate-rich beverages. A specifically designed carbohydrate-rich beverage is currently used to support the surgical patient metabolically. Fruit-based beverages may also promote recovery, due to natural antioxidant and carb......To study the gastric-emptying rate and gut hormonal response of two carbohydrate-rich beverages. A specifically designed carbohydrate-rich beverage is currently used to support the surgical patient metabolically. Fruit-based beverages may also promote recovery, due to natural antioxidant...... and carbohydrate content. However, gastric emptying of fluids is influenced by its nutrient composition; hence, safety of preoperative carbohydrate loading should be confirmed. Because gut hormones link carbohydrate metabolism and gastric emptying, hormonal responses were studied....

  20. Effects of Carbohydrate Consumption Case Study: carbohydrates in Bread

    Directory of Open Access Journals (Sweden)

    Neacsu N.A.

    2014-12-01

    Full Text Available Carbohydrates perform numerous roles in living organisms; they are an important source of energy. The body uses carbohydrates to make glucose which is the fuel that gives it energy and helps keep everything going. However, excess carbohydrate consumption has negative health effects. Bread is a basic product in our nutrition and it also is a product with a high content of carbohydrates. So, it is important to find out more information on bread and on the recommended bread type best for consumption.

  1. Acute metabolic decompensation due to influenza in a mouse model of ornithine transcarbamylase deficiency

    Directory of Open Access Journals (Sweden)

    Peter J. McGuire

    2014-02-01

    types of inborn errors of metabolism.

  2. Organizing multivalency in carbohydrate recognition.

    Science.gov (United States)

    Müller, Christian; Despras, Guillaume; Lindhorst, Thisbe K

    2016-06-01

    The interactions of cell surface carbohydrates as well as of soluble glycoconjugates with their receptor proteins rule fundamental processes in cell biology. One of the supramolecular principles underlying and regulating carbohydrate recognition is multivalency. Many multivalent glycoconjugates have therefore been synthesized to study multivalency effects operative in glycobiology. This review is focused on smaller multivalent structures such as glycoclusters emphasizing carbohydrate-centered and heteromultivalent glycoconjugates. We are discussing primary, secondary and tertiary structural aspects including approaches to organize multivalency. PMID:27146554

  3. Carbohydrate loading in the preoperative setting.

    Science.gov (United States)

    Hill, L T; Miller, M G A

    2015-03-01

    Nutrition support is an evolving field, and modern clinical nutrition practice should actively incorporate strategies to enhance various clinical outcomes. In surgical patients, clinical benefits can be maximised by nutritional support protocols that minimise and manage the perioperative fasting period. This approach, which includes the perioperative provision of clear carbohydrate-containing fluids, has been shown to be safe, is evidence based, and is supported by many professional societies. Such a strategy has been shown to aid the anaesthetic process and maintain an optimal metabolic state, including improved insulin sensitivity and blunted muscle catabolic activity. Some important consequences of this improved metabolic control include shorter hospital stay and fewer postoperative complications. A proactive multidisciplinary team approach is essential to use this nutrition support strategy with success across a hospital's surgical service. PMID:26294840

  4. Carbohydrates and Diabetes (For Parents)

    Science.gov (United States)

    ... of diet foods. These foods may contain extra sugar as a substitute for fat calories. Try to include your child or teen as you evaluate and select healthy carbohydrate-containing foods. With ... blood sugar. By taking a smart approach to balancing carbohydrates, ...

  5. Catalytic Conversion of Carbohydrates

    DEFF Research Database (Denmark)

    Osmundsen, Christian Mårup

    a renewable route to aromatics. The conversion of biomass by high temperature processes is a desirable prospect due to the high volumetric production rates which can be achieved, and the ability of these types of processes to convert a wide range of substrates. Current processes however typically...... process could prove to be an efficient initial conversion step in the utilization of biomass for chemicals production. The shift from an oil based chemical industry to one based on renewable resources is bound to happen sooner or later, however the environmental problems associated with the burning of...... production of commodity chemicals from the most abundantly available renewable source of carbon, carbohydrates. The production of alkyl lactates by the Lewis acid catalyzed conversion of hexoses is an interesting alternative to current fermentation based processes. A range of stannosilicates were...

  6. CARBOHYDRATE INTAKE CONSIDERATIONS FOR YOUNG ATHLETES

    Directory of Open Access Journals (Sweden)

    Veronica Montfort-Steiger

    2007-09-01

    Full Text Available Good nutritional practices are important for exercise performance and health during all ages. Athletes and especially growing children engaged in heavy training have higher energy and nutrient requirements compared to their non-active counterparts. Scientific understanding of sports nutrition for the young athlete is lacking behind the growing number of young athletes engaged in sports. Most of the sports nutrition recommendations given to athletic children and adolescents are based on adult findings due to the deficiency in age specific information in young athletes. Therefore, this review reflects on child specific sports nutrition, particularly on carbohydrate intake and metabolism that distinguishes the child athlete from the adult athlete. Children are characterised to be in an insulin resistance stage during certain periods of maturation, have different glycolytic/metabolic responses during exercise, have a tendency for higher fat oxidation during exercise and show different heat dissipation mechanisms compared to adults. These features point out that young athletes may need different nutritional advice on carbohydrate for exercise to those from adult athletes. Sport drinks for example may need to be adapted to children specific needs. However, more research in this area is warranted to clarify sports nutrition needs of the young athlete to provide better and healthy nutritional guidance to young athletes

  7. Evaluation of a simple intervention to reduce exchange transfusion rates among inborn and outborn neonates in Myanmar, comparing pre- and post-intervention rates

    OpenAIRE

    Arnolda, G.; Thein, A. A.; Trevisanuto, D; Aung, N.; Nwe, H. M; Thin, A. A.; Aye, N. S. S.; Defechereux, T.; Kumara, D.; Moccia, L.

    2015-01-01

    Background In Myanmar, approximately half of all neonatal hospital admissions are for hyperbilirubinaemia, and tertiary facilities report high rates of Exchange Transfusion (ET). The aim of this study was to evaluate the effectiveness of the pilot program in reducing ET, separately of inborn and outborn neonates. Methods The study was conducted in the Neonatal Care Units of four national tertiary hospitals: two exclusively treating inborn neonates, and two solely for outborn neonates. Prior t...

  8. Protein and leucine metabolism in maple syrup urine disease

    International Nuclear Information System (INIS)

    Constant infusions of [13C]leucine and [2H5]phenylalanine were used to trace leucine and protein kinetics, respectively, in seven children with maple syrup urine disease (MSUD) and eleven controls matched for age and dietary protein intake. Despite significant elevations of plasma leucine (mean 351 mumol/l, range 224-477) in MSUD subjects, mean whole body protein synthesis [3.78 +/- 0.42 (SD) g.kg-1. 24 h-1] and catabolism (4.07 +/- 0.46) were similar to control values (3.69 +/- 0.50 and 4.09 +/- 0.50, respectively). The relationship between phenylalanine and leucine fluxes was also similar in MSUD subjects (mean phenylalanine-leucine flux ratio 0.35 +/- 0.07) and previously reported adult controls (0.33 +/- 0.02). Leucine oxidation was undetectable in four of the MSUD subjects and very low in the other three (less than 4 mumol.kg-1.h-1; controls 13-20). These results show that persistent elevation in leucine concentration has no effect on protein synthesis. The marked disturbance in leucine metabolism in MSUD did not alter the relationship between rates of catabolism of protein to phenylalanine and leucine, which provides further support for the validity of the use of a single amino acid to trace whole body protein metabolism. The minimal leucine oxidation in MSUD differs from findings in other inborn metabolic errors and indicates that in patients with classical MSUD there is no significant route of leucine disposal other than through protein synthesis

  9. A Moderate Low-Carbohydrate Low-Calorie Diet Improves Lipid Profile, Insulin Sensitivity and Adiponectin Expression in Rats

    Directory of Open Access Journals (Sweden)

    Jie-Hua Chen

    2015-06-01

    Full Text Available Calorie restriction (CR via manipulating dietary carbohydrates has attracted increasing interest in the prevention and treatment of metabolic syndrome. There is little consensus about the extent of carbohydrate restriction to elicit optimal results in controlling metabolic parameters. Our study will identify a better carbohydrate-restricted diet using rat models. Rats were fed with one of the following diets for 12 weeks: Control diet, 80% energy (34% carbohydrate-reduced and 60% energy (68% carbohydrate-reduced of the control diet. Changes in metabolic parameters and expressions of adiponectin and peroxisome proliferator activator receptor γ (PPARγ were identified. Compared to the control diet, 68% carbohydrate-reduced diet led to a decrease in serum triglyceride and increases inlow density lipoprotein-cholesterol (LDL-C, high density lipoprotein-cholesterol (HDL-C and total cholesterol; a 34% carbohydrate-reduced diet resulted in a decrease in triglycerides and an increase in HDL-cholesterol, no changes however, were shown in LDL-cholesterol and total cholesterol; reductions in HOMA-IR were observed in both CR groups. Gene expressions of adiponectin and PPARγ in adipose tissues were found proportionally elevated with an increased degree of energy restriction. Our study for the first time ever identified that a moderate-carbohydrate restricted diet is not only effective in raising gene expressions of adiponectin and PPARγ which potentially lead to better metabolic conditions but is better at improving lipid profiles than a low-carbohydrate diet in rats.

  10. In-Born Radio Frequency Identification Devices for Safeguards Use at Gas-Centrifuge Enrichment Plants

    International Nuclear Information System (INIS)

    Global expansion of nuclear power has made the need for improved safeguards measures at Gas Centrifuge Enrichment Plants (GCEPs) imperative. One technology under consideration for safeguards applications is Radio Frequency Identification Devices (RFIDs). RFIDs have the potential to increase IAEA inspector's efficiency and effectiveness either by reducing the number of inspection visits necessary or by reducing inspection effort at those visits. This study assesses the use of RFIDs as an integral component of the 'Option 4' safeguards approach developed by Bruce Moran, U.S. Nuclear Regulatory Commission (NRC), for a model GCEP [1]. A previous analysis of RFIDs was conducted by Jae Jo, Brookhaven National Laboratory (BNL), which evaluated the effectiveness of an RFID tag applied by the facility operator [2]. This paper presents a similar evaluation carried out in the framework of Jo's paper, but it is predicated on the assumption that the RFID tag is applied by the manufacturer at the birth of the cylinder, rather than by the operator. Relevant diversion scenarios are examined to determine if RFIDs increase the effectiveness and/ or efficiency of safeguards in these scenarios. Conclusions on the benefits offered to inspectors by using in-born RFID tagging are presented.

  11. Effects of Indigestible Carbohydrates and GI of Cereal Products on Glucose Metabolism, Satiety and Cognitive Function in Healthy Subjects; Emphasising mechanisms for glycaemic regulation at the acute, second and third meal

    OpenAIRE

    Nilsson, Anne

    2007-01-01

    The metabolic syndrome includes a cluster of dysfunctions that identifies subjects at high risk of developing type 2 diabetes and cardiovascular disease. The prevalence of the metabolic syndrome has increased markedly over the last two decades. Central to this syndrome is insulin resistance and hyperglycaemia, but also other manifestations, e.g. central obesity, dyslipidaemia, elevated blood pressure, imbalance in lipoproteins and pro-thrombotic factors, or sub-clinical inflammation, are invo...

  12. Transition metals in carbohydrate chemistry

    DEFF Research Database (Denmark)

    Madsen, Robert

    1997-01-01

    This review describes the application of transition metal mediated reactions in carbohydrate synthesis. The different metal mediated transformations are divided into reaction types and illustrated by various examples on monosaccharide derivatives. Carbon-carbon bond forming reactions are further ...

  13. Carbohydrates, Sugar, and Your Child

    Science.gov (United States)

    ... are: simple carbohydrates (or simple sugars): these include fructose, glucose, and lactose, which also are found in nutritious ... look at the ingredient list for sugar, corn syrup or sweetener, dextrose, fructose, honey, or molasses, to name just a few. ...

  14. Arabinogalactan proteins: focus on carbohydrate active enzymes

    Directory of Open Access Journals (Sweden)

    Eva eKnoch

    2014-06-01

    Full Text Available Arabinogalactan proteins (AGPs are a highly diverse class of cell surface proteoglycans that are commonly found in most plant species. AGPs play important roles in many cellular processes during plant development, such as reproduction, cell proliferation, pattern formation and growth, and in plant-microbe interaction. However, little is known about the molecular mechanisms of their function. Numerous studies using monoclonal antibodies that recognize different AGP glycan epitopes have shown the appearance of a slightly altered AGP glycan in a specific stage of development in plant cells. Therefore, it is anticipated that the biosynthesis and degradation of AGP glycan is tightly regulated during development. Until recently, however, little was known about the enzymes involved in the metabolism of AGP glycans. In this review, we summarize recent discoveries of carbohydrate active enzymes (CAZy; http://www.cazy.org/ involved in the biosynthesis and degradation of AGP glycans, and we discuss the biological role of these enzymes in plant development.

  15. Carbohydrates Through Animation: Preliminary Step

    Directory of Open Access Journals (Sweden)

    J.K. Sugai

    2004-05-01

    Full Text Available Methods of education are changing, so the educational tools must change too. The developmentof the systems of information and communication gave the opportunity to bring new technology tothe learning process. Modern education needs interactive programs that may be available to theacademic community, in order to ease the learning process and sharing of the knowledge. Then,an educational software on Carbohydrates is being developed using concept maps and FLASH-MXanimations program, and approached through six modules. The introduction of Carbohydrates wasmade by the module Carbohydrates on Nature, which shows the animations gures of a teacher andstudents, visiting a farm, identifying the carbohydrates found in vegetables, animals, and microor-ganisms, integrated by links containing short texts to help understanding the structure and functionof carbohydrates. This module was presented, as pilot experiment, to teachers and students, whichdemonstrated satisfaction, and high receptivity, by using animation and interactivitys program asstrategy to biochemistrys education. The present work is part of the project Biochemistry throughanimation, which is having continuity.

  16. Obesity, metabolism, and hypertension.

    OpenAIRE

    Landsberg, L

    1989-01-01

    The relationship between obesity and hypertension is complex and poorly understood. A developing body of information suggests that metabolic factors related to the obese state are importantly involved. The pertinent observations include: (1) Diet influences sympathetic nervous system activity. Fasting suppresses, while carbohydrate and fat feeding stimulate, sympathetic activity. (2) Dietary-induced changes in sympathetic activity contribute to the changes in metabolic rate that accompany cha...

  17. Fermentation characteristics of several carbohydrate sources for dog diets using the in vitro gas production technique

    OpenAIRE

    Serena Calabrò; Aulus C. Carciofi; Nadia Musco; Raffaella Tudisco; Gomes, Marcia O. S.; Monica I. Cutrignelli

    2013-01-01

    Fermentable carbohydrates are an important part of the canine diet. They can improve gastrointestinal health by modifying gut microbial population and metabolic activity. The present study compared the fermentation characteristics and kinetic patterns of 10 carbohydrate sources using the in vitro gas production technique (IVGPT) with dog faecal inoculum. The substrates tested were: pure cellulose (PC), carboxymethylcellulose (CMC), sugar-cane fibre (SCF), beet pulp (BP), wheat bran (WB), fruc...

  18. Seasonal dynamics and age of stemwood nonstructural carbohydrates in temperate forest trees.

    OpenAIRE

    Richardson, AD; Carbone, MS; Keenan, TF; Czimczik, CI; Hollinger, DY; Murakami, P; Schaberg, PG; Xu, X.

    2013-01-01

    Nonstructural carbohydrate reserves support tree metabolism and growth when current photosynthates are insufficient, offering resilience in times of stress. We monitored stemwood nonstructural carbohydrate (starch and sugars) concentrations of the dominant tree species at three sites in the northeastern United States. We estimated the mean age of the starch and sugars in a subset of trees using the radiocarbon ((14) C) bomb spike. With these data, we then tested different carbon (C) allocatio...

  19. Fate of Carbohydrates and Lignin during Composting and Mycelium Growth of Agaricus bisporus on Wheat Straw Based Compost

    OpenAIRE

    Edita Jurak; Arjen M Punt; Wim Arts; Mirjam A Kabel; Harry Gruppen

    2015-01-01

    In wheat straw based composting, enabling growth of Agaricus bisporus mushrooms, it is unknown to which extent the carbohydrate-lignin matrix changes and how much is metabolized. In this paper we report yields and remaining structures of the major components. During the Phase II of composting 50% of both xylan and cellulose were metabolized by microbial activity, while lignin structures were unaltered. During A. bisporus' mycelium growth (Phase III) carbohydrates were only slightly consumed a...

  20. Refractive Errors

    Science.gov (United States)

    ... the eye keeps you from focusing well. The cause could be the length of the eyeball (longer or shorter), changes in the shape of the cornea, or aging of the lens. Four common refractive errors are Myopia, or nearsightedness - clear vision close up ...

  1. Medication Errors

    Science.gov (United States)

    ... Proprietary Names (PDF - 146KB) Draft Guidance for Industry: Best Practices in Developing Proprietary Names for Drugs (PDF - 279KB) ... or (301) 796-3400 druginfo@fda.hhs.gov Human Drug ... in Medication Errors Resources for You Agency for Healthcare Research and Quality: ...

  2. Very low-carbohydrate diets in the management of diabetes revisited.

    Science.gov (United States)

    Schofield, Grant Martin; Henderson, George; Thornley, Simon

    2016-01-01

    Humans can derive energy from carbohydrate, fat, or protein. The metabolism of carbohydrate requires by far the highest secretion of insulin. The central pathology of diabetes is the inability to maintain euglycaemia because of a deficiency in either the action or secretion of insulin. That is, because of either insulin resistance often accompanied by hyperinsulinaemia, or insulin deficiency caused by pancreatic beta cell failure. In individuals dependent on insulin and other hypoglycaemic medication, the difficulty of matching higher intakes of carbohydrates with the higher doses of medication required to maintain euglycaemia increases the risk of adverse events, including potentially fatal hypoglycaemic episodes. Thus, mechanistically it has always made sense to restrict carbohydrate (defined as sugar and starch, but not soluble and insoluble fibre) in the diets of people with diabetes. Randomised clinical trials have confirmed that this action based on first principles is effective. The continued recommendation of higher-carbohydrate, fat-restricted diets has been criticised by some scientists, practitioners and patients. Such protocols when compared with very low-carbohydrate diets provide inferior glycaemic control, and their introduction and subsequent increase in carbohydrate allowances has never been based on strong evidence. The trend towards highercarbohydrate diets for people with diabetes may have played a part in the modern characterisation of type 2 diabetes as a chronic condition with a progressive requirement for multiple medications. Here we will introduce some of the evidence for very low-carbohydrate diets in diabetes management and discuss some of the common objections to their use. PMID:27356254

  3. Influence of Single-bout Resistance Training and Carbohydrate-Protein Supplementation on Urine Androgen Metabolism of Male Boxers and Bodybuilders%急性抗阻运动及不同比例糖和蛋白营养干预对男子力量项目运动员尿雄激素代谢的影响

    Institute of Scientific and Technical Information of China (English)

    文安; 王启荣; 方子龙; 邵晶; 陈成亮; 周钰杰

    2012-01-01

    目的:观察急性抗阻运动及不同比例糖和蛋白营养干预对男子力量项目运动员尿雄激素变化的影响.方法:18名男子力量项目(拳击、健美)运动员,分为糖加高蛋白补充组(HP组,糖和蛋白比例为3∶1)、糖加低蛋白补充组(LP组,糖和蛋白补充比例为6∶1)及纯糖补充对照组(C组),所有受试者进行相同的抗阻运动,先进行4组70%1RM深蹲动作,每组8次,组间休息60 s,第4组完成后休息90 s;随后完成4组50%1RM深蹲动作,第1、2组重复8次,后两组重复至不能完成动作为止,组间休息60 s,第4组完成后休息150 s;接下来完成4组70% 1RM卧推动作,组间休息60 s,第4组完成后休息90 s;最后完成4组50% 1RM卧推动作,第1、2组重复8次,后两组重复至不能完成动作为止,组间休息60 s.采用气相质谱色谱仪(GC-MS)测定运动前、运动后即刻及次日晨尿雄激素.结果:急性运动后即刻各组尿本胆烷醇酮及雄酮比运动前显著升高(P<0.05),组间无差异.运动后次日晨,HP组和LP组尿5α-雄烷二醇和5β-雄烷二醇比运动前显著升高(P<0.05),组间比较HP组5α-雄烷二醇和5β-雄烷二醇显著高于LP、C组(P<0.05).结论:急性抗阻运动及糖和蛋白营养干预能加速男子力量项目运动员性腺雄激素和肾上腺雄激素转化和代谢,促进睾酮向双氢睾酮转化,有利于脂肪分解和糖酵解,促进机体运动后疲劳恢复.%Objective To investigate the effects of carbohydrate-protein supplementations with different ratios on the body recovery process after acute resistance training and urine androgen metabolism of male boxers and bodybuilders. Methods Eighteen male boxers and bodybuilders were supplied with high-protein and low-carbohydrate (group HP, carbohydrate:protein = 3:1) ,low-protein and high-carbohydrate (group LP,carbohydraterprotein = 6:1) and carbohydrate only (group C). All subjects completed 4 sets of 8-repetition squats at 70% of

  4. Carbohydrates of human immunodeficiency virus

    DEFF Research Database (Denmark)

    Hansen, J E

    1992-01-01

    Elucidation of the mechanism by which viral infection induces the appearance of carbohydrate neoantigens is highly important. Results from such studies could be expected to be significant for a general understanding of the regulation of glycosylation, and perhaps especially important for the unde...

  5. Ketone body metabolism and cardiovascular disease

    OpenAIRE

    Cotter, David G.; Schugar, Rebecca C.; Crawford, Peter A.

    2013-01-01

    Ketone bodies are metabolized through evolutionarily conserved pathways that support bioenergetic homeostasis, particularly in brain, heart, and skeletal muscle when carbohydrates are in short supply. The metabolism of ketone bodies interfaces with the tricarboxylic acid cycle, β-oxidation of fatty acids, de novo lipogenesis, sterol biosynthesis, glucose metabolism, the mitochondrial electron transport chain, hormonal signaling, intracellular signal transduction pathways, and the microbiome. ...

  6. Carbohydrates - Multiple Languages: MedlinePlus

    Science.gov (United States)

    ... Supplements Videos & Tools You Are Here: Home → Multiple Languages → All Health Topics → Carbohydrates URL of this page: https://medlineplus.gov/languages/carbohydrates.html Other topics A-Z A B ...

  7. Tumor Metabolism of Malignant Gliomas

    OpenAIRE

    Deliang Guo; Arnab Chakravarti; Williams, Terence M.; Peng Ru

    2013-01-01

    Constitutively activated oncogenic signaling via genetic mutations such as in the EGFR/PI3K/Akt and Ras/RAF/MEK pathways has been recognized as a major driver for tumorigenesis in most cancers. Recent insights into tumor metabolism have further revealed that oncogenic signaling pathways directly promote metabolic reprogramming to upregulate biosynthesis of lipids, carbohydrates, protein, DNA and RNA, leading to enhanced growth of human tumors. Therefore, targeting cell metabolism has become a...

  8. Fluorous-based carbohydrate quartz crystal microbalance.

    Science.gov (United States)

    Chen, Lei; Sun, Pengfei; Chen, Guosong

    2015-03-20

    Fluorous chemistry has brought many applications from catalysis to separation science, from supramolecular materials to analytical chemistry. However, fluorous-based quartz crystal microbalance (QCM) has not been reported so far. In the current paper, fluorous interaction has been firstly utilized in QCM, and carbohydrate-protein interaction and carbohydrate-carbohydrate interaction have been detected afterward. PMID:25541017

  9. Interactions of carbohydrates and proteins by fluorophore-assisted carbohydrate electrophoresis

    Indian Academy of Sciences (India)

    Gang-Liang Huang; Xin-Ya Mei; Peng-George Wang

    2006-06-01

    A sensitive, specific, and rapid method for the detection of carbohydrate-protein interactions is demonstrated by fluorophore-assisted carbohydrate electrophoresis (FACE). The procedure is simple and the cost is low. The advantage of this method is that carbohydrate-protein interactions can be easily displayed by FACE, and the carbohydrates do not need to be purified.

  10. Nutrition and metabolic syndrome.

    Directory of Open Access Journals (Sweden)

    Albornoz López, Raúl

    2012-12-01

    Full Text Available The metabolic syndrome comprises a cluster of metabolic abnormalities that increase the risk for cardiovascular disease and type 2 diabetes mellitus. The exact etiology is unclear, although it is known thatthere is a complex interaction between genetic, metabolic and environmental factors. Among the environmental factors, dietary habits play an important role in the treatment and prevention of this condition. General classic recommendations include control of obesity, increased physical activity, decreased intake of saturated fat and cholesterol, reduced intake of simple sugars and increased intake of fruits and vegetables. It has been studied the influence of diets low in carbohydrates, diets rich in polyunsaturated and monounsaturated fatty acids, fiber intake, the Mediterranean diet and the glycemic index in relation to metabolic syndrome.Other nutrients recently studied are the micronutrients (magnesium and calcium, soy and other phytochemicals. Evidence suggests that a healthy diet like the Mediterranean protects against metabolic syndrome,caracterized for a low content in saturated and trans fat, high in monounsaturated and polyunsaturated fatty acids, balanced intake of carbohydrates and high in fiber, fruits and vegetables. There is more controversy about the type of diet of choice for the control ofmetabolic syndrome (low-carbohydrate diets or lowfat, needing more studies on the role of soy and other phytochemicals.

  11. On the Gate of Life Be the Inborn Origin%论命门为先天之本

    Institute of Scientific and Technical Information of China (English)

    储全根

    2001-01-01

    针对明代医家李中梓提出并影响至今的“肾为先天之本”说提出质疑,认为“肾为先天之本”论实乃李氏对其他医家研究成果的否定和抹杀,带来了中医理论的诸多矛盾和问题。肾脏作为后天脏器,不应视作先天之本。《难经》所倡导而明代医家又着力阐发的命门概念有丰富的内涵,应当确定其先天之本的重要地位,这不仅解决了中医理论上的一些矛盾,且确为完善理论所必需,并可拓展今后中医基础研究的思路和空间。%The theory of “Kidney is the inborn origin of the body”whose influence has existed even to this day advocated by Li Zhongzi,the medical scientist in Ming Dynasty is questioned in this article.The author holds that Li's theory is actually the negation of the achievements in medical research gained by the other medical scientists in chinese history and it also brings about various contradictions and problems in the theory of Traditional Chinese Medicine.As the postnatal organs of the body,kidney itself cannot be regarded as the inborn origin.The concept of gate of life advocated in The book of The Classcic on Difficulty and elucidated by the medical scientists in Ming Dynasty with great efforts contains rich connotation and its important position as the real inborn origin of the body should be detined.By do so,some contradictions in the theory of Traditional Chinese Medicine can be solved and hence,the theory is necessarily improved,and meanwhile,the method and scope of thinking in the research of the fundamental theory of Traditional Chinese Medicine can also be developed and widened.

  12. Carbohydrate Microarrays in Plant Science

    DEFF Research Database (Denmark)

    Fangel, Jonatan Ulrik; Pedersen, H.L.; Vidal-Melgosa, S.;

    2012-01-01

    Almost all plant cells are surrounded by glycan-rich cell walls, which form much of the plant body and collectively are the largest source of biomass on earth. Plants use polysaccharides for support, defense, signaling, cell adhesion, and as energy storage, and many plant glycans are also important...... industrially and nutritionally. Understanding the biological roles of plant glycans and the effective exploitation of their useful properties requires a detailed understanding of their structures, occurrence, and molecular interactions. Microarray technology has revolutionized the massively high...... plant research and can be used to map glycan populations across large numbers of samples to screen antibodies, carbohydrate binding proteins, and carbohydrate binding modules and to investigate enzyme activities....

  13. Carbohydrates Through Animation: Preliminary Step

    OpenAIRE

    J.K. Sugai; M.S.R. Figueiredo; R.V. Antônio; P.M Oliveira; V.A Cardoso; Ricardo, J.; Merino, E; Figueiredo, L. F.; D.N. Heidrich

    2004-01-01

    Methods of education are changing, so the educational tools must change too. The developmentof the systems of information and communication gave the opportunity to bring new technology tothe learning process. Modern education needs interactive programs that may be available to theacademic community, in order to ease the learning process and sharing of the knowledge. Then,an educational software on Carbohydrates is being developed using concept maps and FLASH-MXanimations program, and approach...

  14. Effect of carbohydrate restriction and high carbohydrates diets on men with chemical diabetes.

    Science.gov (United States)

    Anderson, J W

    1977-03-01

    The influence of low carbohydrate (CHO) diets, starvation, and high CHO diets on glucose tolerance tests (GTT) and plasma insulin response of men with chemical diabetes was studied. The GTT and insulin responses of these seven lean diabetic men were unchanged when the carbohydrate content of the diet was reduced from 44 to 20% of calories. After a 48-hr fast a significant deterioration of the GTT was observed in these diabetic men but the percentage change was identical to that reported previously for normal men. Thus these studies indicate that changes in glucose mtes are quite similar to those reported previously for normal men. The fasting plasma glucose values of seven lean and four obese men with chemical diabetes were significantly lower after one week on a 75% CHO diet than values on a 44% CHO diet. The 75% CHO diet also was accompanied by slight improvements in the oral and intravenous GTT and by slightly lower plasma insulin responses. The improvement in glucose metabolism on high CHO diets appears to results from increased insulin sensitivity. Serum triglyceride values were approximately 55% higher on the 75% CHO diet than values on the 44% CHO diet for the 11 men but these differences were not statistically significant. These studies support previous observations and suggest that high CHO diets may be beneficial in the management of certain diabetic patients. However, further studies are required to determine the long-term effects of high CHO diets containing natural foods on the glucose and lipid metabolism of diabetic patients. PMID:842491

  15. The relationship between carbohydrate and the mealtime insulin dose in type 1 diabetes.

    Science.gov (United States)

    Bell, Kirstine J; King, Bruce R; Shafat, Amir; Smart, Carmel E

    2015-01-01

    A primary focus of the nutritional management of type 1 diabetes has been on matching prandial insulin therapy with carbohydrate amount consumed. Different methods exist to quantify carbohydrate including counting in one gram increments, 10g portions or 15g exchanges. Clinicians have assumed that counting in one gram increments is necessary to precisely dose insulin and optimize postprandial control. Carbohydrate estimations in portions or exchanges have been thought of as inadequate because they may result in less precise matching of insulin dose to carbohydrate amount. However, studies examining the impact of errors in carbohydrate quantification on postprandial glycemia challenge this commonly held view. In addition it has been found that a single mealtime bolus of insulin can cover a range of carbohydrate intake without deterioration in postprandial control. Furthermore, limitations exist in the accuracy of the nutrition information panel on a food label. This article reviews the relationship between carbohydrate quantity and insulin dose, highlighting limitations in the evidence for a linear association. These insights have significant implications for patient education and mealtime insulin dose calculations. PMID:26422396

  16. Effect of Different Rice-Crab Coculture Modes on Soil Carbohydrates

    Institute of Scientific and Technical Information of China (English)

    YAN Ying; LIU Ming-da; YANG Dan; ZHANG Wei; AN Hui; WANG Yao-jing; XIE Hong-tu; ZHANG Xu-dong

    2014-01-01

    Traditional agricultural systems have contributed to food and livelihood security. Rice-crab coculture (RC) is an important eco-agricultural process in rice production in northern China. Recognizing the soil fertility in RC may help develop novel sustainable agriculture. Soil carbohydrates are important factors in determining soil fertility in different culture modes. In this study, soil carbohydrates were analyzed under three different culture modes including rice monoculture (RM), conventional rice-crab coculture (CRC) and organic rice-crab coculture (ORC). Results showed that the contents of soil organic carbon and carbohydrates were signiifcantly higher in the ORC than those in RM. The increasing effect was greater with increased organic manure. Similar tendency was found in CRC, but the overall effect was less pronounced compared with ORC. Carbohydrates were more sensitive to RC mode and manure amendment than soil organic carbon. Compare to RM, the (Gal+Man)/(Ara+Xyl) ratio decreased in all the RC modes, indicating a relative enrichment in plant-derived carbohydrates due to the input of crab feed and manure. While the increasing (Gal+Man)/(Ara+Xyl) ratio in ORC modes with increased organic manure suggested that crab activity and metabolism induced microbially derived carbohydrates accumulation. The lower GluN/MurA ratio in ORC indicated an enhancement of bacteria contribution to SOM turnover in a short term. The ifndings reveal that the ORC mode could improve the quantity and composition of soil carbohydrates, effectively, to ensure a sustainable use of paddy soil.

  17. Abnormal sterol metabolism in holoprosencephaly.

    Science.gov (United States)

    Haas, Dorothea; Muenke, Maximilian

    2010-02-15

    Holoprosencephaly (HPE) is the most common structural malformation of the developing forebrain in humans. The HPE phenotype is extremely variable and the etiology is heterogeneous. Among a variety of embryological toxins that can induce HPE, inhibitors, and other pertubations of cholesterol biosynthesis have been shown to be important factors, most likely because cholesterol is required in the Sonic hedgehog signaling cascade. Decreased levels of maternal cholesterol during pregnancy increase the risk for preterm delivery, but they are not associated with congenital malformations. However, if the fetus is affected by an inborn error of endogenous cholesterol synthesis, a reduction of maternal cholesterol concentration and cholesterol transport over the placenta aggravates the phenotypic expression. Exposure to lipophilic statins in early pregnancy may be associated with a substantial risk for structural CNS defects. PMID:20104605

  18. Biochemical, Metabolic, and Behavioral Characteristics of Immature Chronic Hyperphenylalanemic Rats.

    Science.gov (United States)

    Dienel, Gerald A; Cruz, Nancy F

    2016-02-01

    Phenylketonuria and hyperphenylalanemia are inborn errors in metabolism of phenylalanine arising from defects in steps to convert phenylalanine to tyrosine. Phe accumulation causes severe mental retardation that can be prevented by timely identification of affected individuals and their placement on a Phe-restricted diet. In spite of many studies in patients and animal models, the basis for acquisition of mental retardation during the critical period of brain development is not adequately understood. All animal models for human disease have advantages and limitations, and characteristics common to different models are most likely to correspond to the disorder. This study established similar levels of Phe exposure in developing rats between 3 and 16 days of age using three models to produce chronic hyperphenylalanemia, and identified changes in brain amino acid levels common to all models that persist for ~16 h of each day. In a representative model, local rates of glucose utilization (CMRglc) were determined at 25-27 days of age, and only selective changes that appeared to depend on Phe exposure were observed. CMRglc was reduced in frontal cortex and thalamus and increased in hippocampus and globus pallidus. Behavioral testing to evaluate neuromuscular competence revealed poor performance in chronically-hyperphenylalanemic rats that persisted for at least 3 weeks after cessation of Phe injections and did not occur with mild or acute hyperphenylalanemia. Thus, the abnormal amino acid environment, including hyperglycinemia, in developing rat brain is associated with selective regional changes in glucose utilization and behavioral abnormalities that are not readily reversed after they are acquired. PMID:26224289

  19. Effect of Low ph on Carbohydrate Production by a Marine Planktonic Diatom (Chaetoceros muelleri)

    International Nuclear Information System (INIS)

    Rising carbon dioxide (CO2) concentrations in the atmosphere due to human activity are causing the surface ocean to become more acidic. Diatoms play a pivotal role in biogeochemical cycling and ecosystem function in the ocean. ph affected the quantum efficiency of photosystem II and carbohydrate metabolism in a planktonic diatom (Chaetoceros muelleri), representative of a widely distributed genus. In batch cultures grown at low ph, the proportion of total carbohydrate stored within the cells decreased and more dissolved carbohydrates were exuded from the cells into the surrounding medium. Changes in productivity and the way in which diatoms allocate carbon into carbohydrates may affect ecosystem function and the efficiency of the biological carbon pump in a low ph ocean.

  20. Effect of Low pH on Carbohydrate Production by a Marine Planktonic Diatom (Chaetoceros muelleri

    Directory of Open Access Journals (Sweden)

    Daniel C. O. Thornton

    2009-01-01

    Full Text Available Rising carbon dioxide (CO2 concentrations in the atmosphere due to human activity are causing the surface ocean to become more acidic. Diatoms play a pivotal role in biogeochemical cycling and ecosystem function in the ocean. pH affected the quantum efficiency of photosystem II and carbohydrate metabolism in a planktonic diatom (Chaetoceros muelleri, representative of a widely distributed genus. In batch cultures grown at low pH, the proportion of total carbohydrate stored within the cells decreased and more dissolved carbohydrates were exuded from the cells into the surrounding medium. Changes in productivity and the way in which diatoms allocate carbon into carbohydrates may affect ecosystem function and the efficiency of the biological carbon pump in a low pH ocean.

  1. Transformation of acetate carbon into carbohydrate and amino acid metabilites during decomposition in soil

    DEFF Research Database (Denmark)

    Sørensen, Lasse Holst; Paul, E. A.

    1971-01-01

    Carbon-14-labelled acetate was added to a heavy clay soil of pH 7.6 to study the transformation of acetate carbon into carbohydrate and amino acid metabolites during decomposition. The acetate was totally metabolized after 6 days of incubation at 25°C when 70% of the labelled carbon had been...... evolved as CO2. Maximum incorporation of trace-C into the various organic fractions was observed after 4 days when 19% of residual, labelled carbon in the soil was located in carbohydrates, 29 % in amino acids and 21 % in the insoluble residue of the soil. The curves showing the amounts of labelled carbon...... days of incubation, 2.2% of the labelled carbon originally added to the soil was located in carbohydrate metabolites, 7% in amino acid metabolites and 5% in the insoluble residue. The carbon in these fractions accounted for 77% of the total, residual, labelled carbon in the soil; 12% in carbohydrates...

  2. Reduced limbic metabolism and fronto-cortical volume in rats vulnerable to alcohol addiction

    OpenAIRE

    Gozzi, Alessandro; Agosta, Federica; Massi, Maurizio; Ciccocioppo, Roberto; Bifone, Angelo

    2012-01-01

    Alcohol abuse is associated with long-term reductions in fronto-cortical volume and limbic metabolism. However, an unanswered question in alcohol research is whether these alterations are the sole consequence of chronic alcohol use, or contain heritable contributions reflecting biological propensity toward ethanol addiction. Animal models of genetic predisposition to alcohol dependence can be used to investigate the role of inborn brain abnormalities in the aetiology of alcoholism. Here we us...

  3. Analysis of Carbohydrate Metabolism Genes of Spongospora subterranea Using 454 Pyrosequencing / Análisis de Genes del Metabolismo de Carbohidratos de Spongospora subterranea Utilizando Pirosecuenciación 454

    Directory of Open Access Journals (Sweden)

    Pablo Andrés Gutiérrez Sánchez

    2014-06-01

    Full Text Available Abstract. Spongospora subterranea, the causal agent of Potato powdery scab, is an important soil-borne obligate protozoan commonly found in Andean soils. This is a serious problem that causes cosmetic damage on the skin of tubers and induces root gall formation, diminishing the yield and commercial value of the potato. Genetic studies on S. subterranea are difficult due to its obligate parasitism, which explains the lack of available knowledge on its basic biology. S. subterranea is a member of the Plasmodiophorida order, a protist taxa that includes other important plant pathogens such as Plasmodiophora brassicae and Spongospora nasturtii. Little is known about the genomes of Plasmodiophorida; however, with the use of Next-GenerationSequencing technologies combined with appropriate bioinformatic techniques, it is possible to obtain genomic sequences from obligate pathogens such as S. subterranea. To gain a better understanding of the biology of this pathogen and Plasmodiophorida in general, DNA sequences from a cystosori-enriched sample of S. subterranea were obtained using 454 pyrosequencing technology. As a first step in understanding the nutritional requirements ofS. subterranea as well as its infective and resistance structures, we present a bioinformatic analysis of 24 contigs related to genes involved in the glycolysis, starch, celullose and chitin metabolism. Intron structure and codon usage is also discussed. The genes analyzed in this study are a good source of information for studies aimed at characterizing these enzymes in vitro, as well as the generation of new methods for the molecular detection of S. subterranea in either soils or infected plants. / Resumen. Spongospora subterranea, el agente causal de la sarna polvosa de la papa, es un protozoo y patógeno obligado presente en los suelos andinos. Esta enfermedad es un serio problema para el cultivo de papa, al causar lesiones cosméticas en la piel de los

  4. Dietary protein and carbohydrate affect feeding behavior and metabolic regulation in hummingbirds (Melanotrochilus fuscus Las proteínas y carbohidratos dietarios afectan la conducta de alimentación y la regulación metabólica en picaflores (Melanotrochilus fuscus

    Directory of Open Access Journals (Sweden)

    FLAVIA P. ZANOTTO

    2005-06-01

    Full Text Available The objective of this work was to link hummingbird feeding behavior with metabolic regulation and in addition to assess whether dietary composition would affect entrance into torpor. Hummingbirds were fed a combination of diets with contrasting amounts of protein and carbohydrate. The diets were composed of the following: 2.4 % protein (P - 12 % sucrose (S and 0.8 % protein (P - 36 % sucrose (S. The main findings showed that periods of feeding on each of the diets could be distinguished as separate bouts or feeding events. Hummingbirds presented to high protein-low carbohydrate diets (2.4P-12S ingested a larger volume of diet, fed for longer (both around 1.7x and increased the interval between feedings compared with hummingbirds fed diets 0.8P-36S. Physiological regulation between feeding events, on the other hand, was achieved through an increase in metabolic rate for low protein-high sugar diets (0.8P-36S. This response could probably be related to high sucrose assimilation rates through the digestive system of hummingbirds, a process already known to be very efficient in these birds. Additionally, there was a steeper decrease in oxygen consumption for hummingbirds fed diets 2.4P-12S during fasting and a suggestion of a higher torpor incidence in birds fed these dietsEl objetivo de este trabajo fue unir la conducta de alimentación de picaflores con su regulación metabólica y además determinar como la composición dietaria podría afectar la entrada en sopor. Los picaflores fueron alimentados con una combinación de dietas con cantidades contrastantes de proteínas y carbohidratos. La composición dietaria fue: 2,4 % proteína (P - 12 % sacarosa (S y 0.8 % proteína (P - 36 % (sacarosa (S. Se observó que para cada dieta, los períodos de alimentación se pueden distinguir como eventos separados de alimentación. Cuando se enfrentan a dietas de alta proteína-bajo carbohidratos (2,4P-12S, los picaflores ingieren grandes volúmenes de

  5. Inborn anemias in mice. Progress report, 1 August 1979-15 July 1980

    Energy Technology Data Exchange (ETDEWEB)

    Bernstein, S.E.; Russell, E.S.

    1980-08-01

    Four macrocytic anemias, four hemolytic anemias, nonhemolytic microcytic anemia, transitory siderocytic anemia, sex-linked iron-transport anemia, an ..cap alpha..-thalassemia, and a new target-cell anemia are under investigation in mice. Each of these blood dyscrasias is caused by the action of a unique mutant gene, which determines the structure of different intracellular molecules, and thus controls a different metabolic process. Thus the wide range of different hereditary anemias has considerable potential for uncovering many different aspects of hemopoietic homeostatic mechanisms in the mouse. Each anemia is studied through: (a) characterization of peripheral blood values; (b) determinations of radiosensitivity under a variety of conditions; (c) measurements of iron metabolism and heme synthesis; (d) histological and biochemical study of blood-forming tissue; (e) functional tests of the stem cell component; (f) examination of responses to erythroid stimuli; and (g) transplantation of tissue between individuals of differently affected genotypes.

  6. Genetic and bibliographic information: TXNIP [GenLibi

    Lifescience Database Archive (English)

    Full Text Available TXNIP thioredoxin interacting protein human Familial combined hyperlipidemia; Hyperlip...Genetic Diseases, Inborn (C16.320) > Metabolism, Inborn Errors (C16.320.565) > Lipid Metabolism, Inborn Erro...rs (C16.320.565.398) > Hyperlipidemia, Familial Combined (C16.320.565.398.450) Nu...tritional and Metabolic Diseases (C18) > Metabolic Diseases (C18.452) > Lipid Metabolism Disorders (C18.452.584) > Dyslip...idemias (C18.452.584.500) > Hyperlipidemias (C18.452.584.500.500) > Hyperlipidemia, Familial Co

  7. Different adipose tissue development in mice pair fed fish oil and different carbohydrates

    OpenAIRE

    Jarlsby, Ragnhild Helene

    2011-01-01

    Obesity causes several health problems, including insulin resistance, Type 2 diabetes, heart diseases, disrupted energy metabolism and low-grade inflammation. Being obese increases the risk of getting metabolic syndrome. A high intake of certain carbohydrates is known to cause rapid rise in blood glucose, which increases insulin secretion and over time causes insulin resistance and hyperglycemia. A high consumption of sucrose and glucose is considered harmful beyond being a source of energ...

  8. Reducing substances in urine: a paradigm for changes in a standard test.

    Science.gov (United States)

    Naumova, Nadia N; Schappert, Joseph; Kaplan, Lawrence A

    2006-01-01

    Detection of reducing substances in urine has been a standard laboratory procedure for about 50 yr. It is used as a screening test for inborn errors of carbohydrate metabolism. Although the test has poor specificity and most states perform mandatory newborn screening for the common genetic defects, most clinical laboratories still perform this as a reflex test on all pediatric urine samples. We suggest that laboratories should perform this test only at the specific order of a physician and that they should review their test menu frequently to delete tests that no longer have a clinical rationale. PMID:17127733

  9. A cereal-based evening meal rich in indigestible carbohydrates increases plasma butyrate the next morning

    DEFF Research Database (Denmark)

    Nilsson, Anne C; Östman, Elin M; Knudsen, Knud Erik Bach;

    2010-01-01

    Epidemiological studies have shown an inverse relation between a whole grain consumption and risk of type-2 diabetes and cardiovascular disease. One tentative mechanism relates to colonic metabolism of indigestible carbohydrates. In a previous study, we reported a positive relation between coloni...

  10. Perfil metabólico de Aegla platensis Schmitt, (Crustacea, Aeglidae, Anomura submetida a dietas ricas em carboidratos ou proteínas Metabolic profile of Aegla platensis Schmitt, (Crustacea, Anomura submitted to carbohydrate-rich or high-protein diets

    Directory of Open Access Journals (Sweden)

    Bibiana Della P. Ferreira

    2005-03-01

    Full Text Available O objetivo desse estudo foi avaliar o efeito de uma dieta rica em carboidratos e uma dieta rica em proteínas sobre o metabolismo de Aegla platensis, um caranguejo anomuro de água doce. Os animais foram coletados no Arroio do Mineiro, município de Taquara, Rio Grande do Sul, em agosto/2002 e janeiro/2003, e foram alimentados durante 15 dias com carne bovina crua e arroz branco, em aquários aerados. O fotoperíodo foi controlado: 12 horas claro - 12 horas escuro. Glicose, proteínas, lipídios e triglicerídios foram dosados na hemolinfa; glicogênio e lipídios foram dosados no hepatopâncreas, brânquias e músculo. Em Aegla platensis, mantida em condições de laboratório, em todos os tecidos estudados pode ser observado que o aporte regular de alimento, bem como o tipo de dieta administrada determinaram um perfil diferenciado de resposta metabólica entre os sexos e o período de realização dos experimentos (inverno e verão.The objective of this study was to assess the effect of a carbohydrate-rich and a high-protein diets on metabolism of Aegla platensis, a freshwater anomuram crab. The animals were collected in Mineiro Creek, Taquara city, Rio Grande do Sul, at august/2002 and january/2003, and were feed during 15 days with crude bovine meat and white rice, in an aerated aquarium. The photoperiod was controlled: 12 hours day - 12 hours night. Glucose, proteins, lipids and triglycerides were dosed in haemolymph; glycogen and lipids were dosed in hepatopancreas, gills and muscle tissues. In Aegla platensis, maintained in laboratory conditions, in all studied tissues can be seen that the regular food intake, as well as the kind of administered diet determinated a differential profile of metabolic response between sexes and the period of the experiments performance (winter and summer.

  11. The factors affecting on estimation of carbohydrate content of meals in carbohydrate counting.

    Science.gov (United States)

    Kawamura, Tomoyuki; Takamura, Chihiro; Hirose, Masakazu; Hashimoto, Tomomi; Higashide, Takashi; Kashihara, Yoneo; Hashimura, Kayako; Shintaku, Haruo

    2015-10-01

    The objective of this study was to identify factors affecting on errors in carbohydrate (CHO) content estimation during CHO counting. Thirty-seven type 1 diabetes patients and 22 of their parents and 28 physicians/dieticians were enrolled in this study. CHO counting was counted in "Carb", with 1 Carb defined as 10 g of CHO. To evaluate the accuracy of CHO counting, 80 real-size photographs of cooked meals were presented to the subjects for Carb estimation. Carbs tended to be overestimated for foods containing relatively small amounts of Carbs. On the other hands, Carbs tended to be underestimated for foods with higher than 6 Carbs. Accurate estimation of the Carbs in food containing a large amount of rice was particularly difficult even in the subjects having the CHO counting experience. The Carb contents of high-calorie foods such as meats, fried foods, and desserts tended to be overestimated. This error was smaller in subjects having the CHO counting experience. In conclusion, misunderstanding of high-calorie dishes containing high amounts of CHO was observed in inexperienced subjects, indicating the efficacy of the current methodology of CHO counting. On the other hand it was difficult even for experienced subjects to assess the amount of seasoned rice, suggesting the need for a new methodology for accurate estimation. PMID:26568656

  12. Carbohydrate Microarrays in Plant Science

    DEFF Research Database (Denmark)

    Fangel, Jonatan Ulrik; Pedersen, H.L.; Vidal-Melgosa, S.; Ahl, Louise Isager; Salmean, A.A.; Egelund, Jack; Rydahl, Maja Gro; Clausen, M.H.; Willats, William George Tycho

    2012-01-01

    Almost all plant cells are surrounded by glycan-rich cell walls, which form much of the plant body and collectively are the largest source of biomass on earth. Plants use polysaccharides for support, defense, signaling, cell adhesion, and as energy storage, and many plant glycans are also importa...... plant research and can be used to map glycan populations across large numbers of samples to screen antibodies, carbohydrate binding proteins, and carbohydrate binding modules and to investigate enzyme activities.......Almost all plant cells are surrounded by glycan-rich cell walls, which form much of the plant body and collectively are the largest source of biomass on earth. Plants use polysaccharides for support, defense, signaling, cell adhesion, and as energy storage, and many plant glycans are also important...... industrially and nutritionally. Understanding the biological roles of plant glycans and the effective exploitation of their useful properties requires a detailed understanding of their structures, occurrence, and molecular interactions. Microarray technology has revolutionized the massively high...

  13. Glycosyltransferase engineering for carbohydrate synthesis.

    Science.gov (United States)

    McArthur, John B; Chen, Xi

    2016-02-01

    Glycosyltransferases (GTs) are powerful tools for the synthesis of complex and biologically-important carbohydrates. Wild-type GTs may not have all the properties and functions that are desired for large-scale production of carbohydrates that exist in nature and those with non-natural modifications. With the increasing availability of crystal structures of GTs, especially those in the presence of donor and acceptor analogues, crystal structure-guided rational design has been quite successful in obtaining mutants with desired functionalities. With current limited understanding of the structure-activity relationship of GTs, directed evolution continues to be a useful approach for generating additional mutants with functionality that can be screened for in a high-throughput format. Mutating the amino acid residues constituting or close to the substrate-binding sites of GTs by structure-guided directed evolution (SGDE) further explores the biotechnological potential of GTs that can only be realized through enzyme engineering. This mini-review discusses the progress made towards GT engineering and the lessons learned for future engineering efforts and assay development. PMID:26862198

  14. Effects of nitrogen source and water availability on stem carbohydrates and cellulosic bioethanol traits of alfalfa plants

    OpenAIRE

    Fiasconaro, M. Laura; Gogorcena Aoiz, Yolanda; Muñoz, Fernando; Andueza, Donato; Sánchez-Díaz, Manuel; Antolín Bellver, M. Carmen

    2012-01-01

    Symbiotic association of legumes with rhizobia frequently results in higher photosynthesis and soluble carbohydrates in comparison with nitrate-fed plants, which might improve its potential for biomass conversion into bioethanol. A greenhouse experiment was conducted to examine the effects of nitrogen source and water availability on stem characteristics and on relationships between carbohydrates, phenolic metabolism activity and cell wall composition in alfalfa (Medicago sativa L. cv. Aragón...

  15. Carbohydrate regulation of photosynthesis and respiration from branch girdling in four species of wet tropical rain forest trees.

    Science.gov (United States)

    Asao, Shinichi; Ryan, Michael G

    2015-06-01

    How trees sense source-sink carbon balance remains unclear. One potential mechanism is a feedback from non-structural carbohydrates regulating photosynthesis and removing excess as waste respiration when the balance of photosynthesis against growth and metabolic activity changes. We tested this carbohydrate regulation of photosynthesis and respiration using branch girdling in four tree species in a wet tropical rainforest in Costa Rica. Because girdling severs phloem to stop carbohydrate export while leaving xylem intact to allow photosynthesis, we expected carbohydrates to accumulate in leaves to simulate a carbon imbalance. We varied girdling intensity by removing phloem in increments of one-quarter of the circumference (zero, one--quarter, half, three-quarters, full) and surrounded a target branch with fully girdled ones to create a gradient in leaf carbohydrate content. Light saturated photosynthesis rate was measured in situ, and foliar respiration rate and leaf carbohydrate content were measured after destructive harvest at the end of the treatment. Girdling intensity created no consistent or strong responses in leaf carbohydrates. Glucose and fructose slightly increased in all species by 3.4% per one-quarter girdle, total carbon content and leaf mass per area increased only in one species by 5.4 and 5.5% per one-quarter girdle, and starch did not change. Only full girdling lowered photosynthesis in three of four species by 59-69%, but the decrease in photosynthesis was unrelated to the increase in glucose and fructose content. Girdling did not affect respiration. The results suggest that leaf carbohydrate content remains relatively constant under carbon imbalance, and any changes are unlikely to regulate photosynthesis or respiration. Because girdling also stops the export of hormones and reactive oxygen species, girdling may induce physiological changes unrelated to carbohydrate accumulation and may not be an effective method to study carbohydrate feedback

  16. Carbohydrate availability of arroz caldo with lambda-carrageenan.

    Science.gov (United States)

    Dumelod, B D; Ramirez, R P; Tiangson, C L; Barrios, E B; Panlasigui, L N

    1999-07-01

    Total available carbohydrate (sugars and starches) and total dietary fiber (soluble and insoluble) make up the total carbohydrate content of a food. Soluble fiber decreases the availability of glucose by delaying its absorption in the proximal small intestine, thus reducing the postprandial glucose levels (Jenkins et al., 1978; Schneeman, 1987a). Carrageenan, a seaweed extract, is a good source of soluble fiber (Montaño et al., 1985). This study aimed to determine the effect of carrageenan incorporation into arroz caldo on carbohydrate availability by monitoring the postprandial blood glucose levels of normal subjects. Control and experimental arroz caldo samples were prepared and subjected to proximate analysis and feeding studies. The total dietary fiber (TDF) content of the experimental (2.03%) was about thrice that of the control (0.68%). Using randomized crossover design, preweighed 55 g available carbohydrate serving portions of control and experimental arroz caldo samples, with 3.45 and 14.84 g TDF, respectively, were fed to ten fasting normal subjects then their postprandial blood glucose levels were determined at 15, 30, 45, 60 and 90 min intervals. Results of the short-term in vivo study showed that the mean postprandial glycaemic responses of subjects after consuming the experimental sample were significantly lower than the levels after consuming the control at 15, 45, and 90 min (P arroz caldo than control (147.29 +/- 53.34). The hypoglycaemic effect of carrageenan may prove useful in the prevention and management of metabolic conditions such as diabetes. PMID:10719574

  17. Biochemical software: Carbohydrates on Laboratory

    Directory of Open Access Journals (Sweden)

    D.N. Heidrich

    2005-07-01

    Full Text Available Educators around  the  world  are  being  challenged  to  develop  and  design  better and  more  effective strategies for student learning  using a variety  of modern  resources.  In this  present  work, an educa- tional  hypermedia  software  was constructed as a support tool to biochemistry teaching.  Occurrence, structure, main  characteristics and  biological  function  of the  biomolecule  Carbohydrates were pre- sented  through  modules.  The  software was developed  using concept  maps,  ISIS-Draw,  and  FLASH- MX animation program.  The chapter  Carbohydrates on Laboratory illustrates experimental methods of carbohydrates characterization, through  animation of a laboratory scenery.   The  subject was de- veloped showing reactions  as Bial, Benedict, Selliwanoff, Barfoed, Phenol  Sulphuric,  and Iodines, and also enzymatic  reactions  as glucose oxidase and amylase.  There are also links with short texts  in order to help the understanding of the contents  and principles of laboratory practice  as well as background reactions. Application of the software to undergraduate students and high school teachers  showed an excellent  acceptance.   All of them  considered  the  software  a very good learning  tool.  Both  teachers and students welcomed this program  as it is more flexible, and allows the learning in a more individual rhythm. In addition, application of the software would be suitable  to a more effective learning  and it is less expensive than conventional experimental teaching.

  18. Metabolic Factors in Fatigue

    Institute of Scientific and Technical Information of China (English)

    Mark Hargreaves

    2006-01-01

    Increased non-oxidative and oxidative ATP production via metabolic pathways in skeletal muscle is essential for the maintenance of force and power production during exercise. However, substrate depletion and accumulation of metabolic byproducts are potential causes of fatigue. Reduced PCr availability can limit power production during sprint exercise, whereas carbohydrate depletion is a major limitation to endurance performance. During sprint exercise increased Pi and H+ may contribute to fatigue, and during prolonged strenuous exercise, the accumulation of NH3, reactive oxygen species, and heat can limit performance. Appropriate training programs and nutritional interventions are potential strategies to enhance fatigue resistance and exercise performance.

  19. Medication errors: prescribing faults and prescription errors

    OpenAIRE

    Velo, Giampaolo P; Minuz, Pietro

    2009-01-01

    Medication errors are common in general practice and in hospitals. Both errors in the act of writing (prescription errors) and prescribing faults due to erroneous medical decisions can result in harm to patients.Any step in the prescribing process can generate errors. Slips, lapses, or mistakes are sources of errors, as in unintended omissions in the transcription of drugs. Faults in dose selection, omitted transcription, and poor handwriting are common.Inadequate knowledge or competence and ...

  20. Carbohydrate clearance receptors in transfusion medicine

    DEFF Research Database (Denmark)

    Sørensen, Anne Louise Tølbøll; Clausen, Henrik; Wandall, Hans H

    2012-01-01

    Complex carbohydrates play important functions for circulation of proteins and cells. They provide protective shields and refraction from non-specific interactions with negative charges from sialic acids to enhance circulatory half-life. For recombinant protein therapeutics carbohydrates are espe...

  1. Biochemical software: Carbohydrates on Laboratory

    OpenAIRE

    D.N. Heidrich; M.S.R.B. Figueiredo; R.V. Antonio,; da Costa, J. G.; P.B. Arantes; Figueiredo, L. F.; J.K. Sugai

    2005-01-01

    Educators around  the  world  are  being  challenged  to  develop  and  design  better and  more  effective strategies for student learning  using a variety  of modern  resources.  In this  present  work, an educa- tional  hypermedia  software  was constructed as a support tool to biochemistry teaching.  Occurrence, structure, main  characteristics and  biological  function  of the  biomolecule  Carbohydrates were pre- sented  through  modules.  The  software was developed  using concept  map...

  2. Facultative thermogenesis induced by carbohydrate

    DEFF Research Database (Denmark)

    Astrup, A; Bülow, J; Christensen, N J;

    1986-01-01

    In addition to the obligatory thermogenesis due to processing and storage, carbohydrate ingestion is accompanied by a facultative thermogenesis mediated by catecholamines via beta-adrenoceptors. The anatomical origin of facultative thermogenesis has hitherto not been determined. The possible...... involvement of skeletal muscle was examined in lean, healthy subjects by measuring the response in forearm oxygen consumption to an oral glucose load. The study demonstrates an early component of skeletal muscle thermogenesis coinciding with the local glucose uptake, followed by a late facultative...... thermogenesis. The arterial epinephrine concentration increased to a maximum of 200% above base-line values 4 h after glucose. This value greatly exceeds the physiological threshold for the thermogenic action of epinephrine. In forearm venous blood the corresponding increase in epinephrine was only...

  3. Genetic and bibliographic information: ASAH1 [GenLibi

    Lifescience Database Archive (English)

    Full Text Available ASAH1 N-acylsphingosine amidohydrolase (acid ceramidase) 1 human Farber lipogranulomatosis (MeSH ... C10) > Central Nervous System Diseases (C10.228) > Brain ... Diseases (C10.228.140) > Brain ... Diseases, Metabolic ... (C10.228.140.163) > Brain ... Diseases, Metabolic, Inborn (C10.228.140.163.100) ... 6.320) > Metabolism, Inborn Errors (C16.320.565) > Brain ... Diseases, Metabolic, Inborn (C16.320.565.189) > Ly ... ic Diseases (C18) > Metabolic Diseases (C18.452) > Brain ... Diseases, Metabolic (C18.452.132) > Brain ... Diseases ...

  4. Effect of Cyolane on carbohydrate and lipid metabolism in rat

    International Nuclear Information System (INIS)

    Cyolane was orally administrated daily for 1-5 weeks (1 mg/kg body weight), induced a marked increase in liver glycogen content reaching its highest values after 1,2,3 and 15 weeks. In kidney and brain the glycogen contents showed a significant increase after 10, 12 and 15 weeks of intoxication. Serum glucose content was increased after 2, 3 and 8 weeks. It has been found that Cyolane caused a fluctuation in liver, kidney and brain total lipid through the first 8 weeks, thereafter it exhibited a significant increase after 10, 12 and 15 weeks. Serum total lipid exhibited a highly significant increase after 2-12 weeks, reaching its maximum value (288.66%) after 15 weeks. 3 figs., 4 tabs

  5. Influence of Carbohydrates on Secondary Metabolism in Fusarium avenaceum

    Directory of Open Access Journals (Sweden)

    Jens Laurids Sørensen

    2013-09-01

    Full Text Available Fusarium avenaceum is a widespread pathogen of important crops in the temperate climate zones that can produce many bioactive secondary metabolites, including moniliformin, fusarin C, antibiotic Y, 2-amino-14,16-dimethyloctadecan-3-ol (2-AOD-3-ol, chlamydosporol, aurofusarin and enniatins. Here, we examine the production of these secondary metabolites in response to cultivation on different carbon sources in order to gain insight into the regulation and production of secondary metabolites in F. avenaceum. Seven monosaccharides (arabinose, xylose, fructose, sorbose, galactose, mannose, glucose, five disaccharides (cellobiose, lactose, maltose, sucrose and trehalose and three polysaccharides (dextrin, inulin and xylan were used as substrates. Three F. avenaceum strains were used in the experiments. These were all able to grow and produce aurofusarin on the tested carbon sources. Moniliformin and enniatins were produced on all carbon types, except on lactose, which suggest a common conserved regulation mechanism. Differences in the strains was observed for production of fusarin C, 2-AOD-3-ol, chlamydosporol and antibiotic Y, which suggests that carbon source plays a role in the regulation of their biosynthesis.

  6. Influence of Carbohydrates on Secondary Metabolism in Fusarium avenaceum

    OpenAIRE

    Jens Laurids Sørensen; Henriette Giese

    2013-01-01

    Fusarium avenaceum is a widespread pathogen of important crops in the temperate climate zones that can produce many bioactive secondary metabolites, including moniliformin, fusarin C, antibiotic Y, 2-amino-14,16-dimethyloctadecan-3-ol (2-AOD-3-ol), chlamydosporol, aurofusarin and enniatins. Here, we examine the production of these secondary metabolites in response to cultivation on different carbon sources in order to gain insight into the regulation and production of secondary metabolites in...

  7. Developmental changes in carbohydrate metabolism during early tuberisation of potato

    OpenAIRE

    Appeldoorn, N.

    1999-01-01

    The potato tuber is a vegetative storage organ, which is initiated at the tip of an underground diagravitropic growing stem, called a stolon. The process of tuber formation comprises the induction, initiation and growth of a stolon, cessation of the longitudinal growth of the stolon, followed by the induction, initiation and growth of the tuber. Tuber initiation is characterized by swelling of the stolon tip. The formation of tubers is accompanied by a large accumulation of starch and of a sp...

  8. Developmental changes in carbohydrate metabolism during early tuberisation of potato

    NARCIS (Netherlands)

    Appeldoorn, N.

    1999-01-01

    The potato tuber is a vegetative storage organ, which is initiated at the tip of an underground diagravitropic growing stem, called a stolon. The process of tuber formation comprises the induction, initiation and growth of a stolon, cessation of the longitudinal growth of the stolon, followed by the

  9. Diversity of carbohydrate metabolism in species of A spergillus

    DEFF Research Database (Denmark)

    Vesth, Tammi Camilla; Hainaut, Matthieu; Rasmussen, Jane Lind Nybo; Theobald, Sebastian; Frisvad, Jens Christian; Nielsen, Kristian Fog; Lyhne, Ellen Kirstine; Kogle, Martin Engelhard; Kuo, Alan; Riley, Robert; de Vries, R.P.; Grigoriev, Igor V.; Mortensen, Uffe Hasbro; Henrissat, Bernard; Baker, Scott E.; Andersen, Mikael Rørdam

    The filamentous fungus Aspergillus niger and its close relatives in Aspergillus section Nigri are of broad interest to the scientific community including applied, medical and basic research. The fungi are prolific producers of native and heterologous proteins, organic acids (in particular citrate......), and secondary metabolites (including bioactives and toxins such as ochratoxin A).Because of these abilities they represent a substantial economic interests in bioenergy applications. In a project collaboration with the US Joint Genome Institute and JBEI we are sequencing 300 different species of...

  10. Characterization of Carbohydrate Active Enzymes Involved in Arabinogalactan Protein Metabolism

    DEFF Research Database (Denmark)

    Knoch, Eva

    accounting for up to 90% of the total mass. The glycan contains mostly galactose and arabinose, but glucoronic acid and other less-abundant sugars like rhamnose, xylose and fucose are also found. Although AGPs are important for normal plant growth and are found throughout the plant in virtually all organs...

  11. Role of carbohydrate metabolism in hormonal regulation of spermatogenesis

    NARCIS (Netherlands)

    N.H.P.M. Jutte (Nicolet)

    1982-01-01

    textabstractIn mammals the testis, the site of male germ cell development, can be divided morphologically in two cellular compartments, viz. the seminiferous tubules and the interstitial tiSsue between the tubules. The seminiferous tubules contain developing germ cells and Sertoli cells and are surr

  12. Carbohydrate intolerance and kidney stones in children in the Goldfields.

    Science.gov (United States)

    Baldwin, D N; Spencer, J L; Jeffries-Stokes, C A

    2003-07-01

    Renal stones have been reported as a common finding in Australian Aboriginal children. The stones are predominantly urate in composition. We report on five children with nephrolithiasis from the Goldfields region of Western Australia. All were diagnosed when under 5 years of age, the majority being under 3 years. All five children also had lactose intolerance, and we postulate that carbohydrate malabsorption, together with the ensuing chronic diarrhoea and intraluminal breakdown of sugars by enteric bacteria may result in a situation of chronic metabolic acidosis. Chronic metabolic acidosis can lead to protein catabolism, increased urate excretion and the formation of renal stones. Carbohydrate intolerance may be an aetiological factor in the development of renal stones and possibly chronic renal disease, particularly in Aboriginal Australians. Renal disease represents one of the most significant factors affecting the health of Australian Aboriginal people. The incidence of end stage renal failure in this population exceeds that of non-Aboriginals by a factor of 13:1, and this disproportionate figure is increasing. It is likely that chronic renal damage is multifactorial; however, it is probable that at least some aetiological factors have their onset during childhood. PMID:12887672

  13. Tumor Metabolism of Malignant Gliomas

    Directory of Open Access Journals (Sweden)

    Deliang Guo

    2013-11-01

    Full Text Available Constitutively activated oncogenic signaling via genetic mutations such as in the EGFR/PI3K/Akt and Ras/RAF/MEK pathways has been recognized as a major driver for tumorigenesis in most cancers. Recent insights into tumor metabolism have further revealed that oncogenic signaling pathways directly promote metabolic reprogramming to upregulate biosynthesis of lipids, carbohydrates, protein, DNA and RNA, leading to enhanced growth of human tumors. Therefore, targeting cell metabolism has become a novel direction for drug development in oncology. In malignant gliomas, metabolism pathways of glucose, glutamine and lipid are significantly reprogrammed. Moreover, molecular mechanisms causing these metabolic changes are just starting to be unraveled. In this review, we will summarize recent studies revealing critical gene alterations that lead to metabolic changes in malignant gliomas, and also discuss promising therapeutic strategies via targeting the key players in metabolic regulation.

  14. Tumor Metabolism of Malignant Gliomas

    Energy Technology Data Exchange (ETDEWEB)

    Ru, Peng; Williams, Terence M.; Chakravarti, Arnab; Guo, Deliang, E-mail: deliang.guo@osumc.edu [Department of Radiation Oncology, Ohio State University Comprehensive Cancer Center & Arthur G James Cancer Hospital, Columbus, OH 43012 (United States)

    2013-11-08

    Constitutively activated oncogenic signaling via genetic mutations such as in the EGFR/PI3K/Akt and Ras/RAF/MEK pathways has been recognized as a major driver for tumorigenesis in most cancers. Recent insights into tumor metabolism have further revealed that oncogenic signaling pathways directly promote metabolic reprogramming to upregulate biosynthesis of lipids, carbohydrates, protein, DNA and RNA, leading to enhanced growth of human tumors. Therefore, targeting cell metabolism has become a novel direction for drug development in oncology. In malignant gliomas, metabolism pathways of glucose, glutamine and lipid are significantly reprogrammed. Moreover, molecular mechanisms causing these metabolic changes are just starting to be unraveled. In this review, we will summarize recent studies revealing critical gene alterations that lead to metabolic changes in malignant gliomas, and also discuss promising therapeutic strategies via targeting the key players in metabolic regulation.

  15. Metabolismo de carboidratos durante o amadurecimento do mamão (Carica papaya L. Cv. Solo: influência da radiação gama Influence of gamma radiation on carbohydrates metabolism of ripening papaya (Carica papaya L. cv. Solo

    Directory of Open Access Journals (Sweden)

    M. L. P. A. GOMEZ

    1999-05-01

    Full Text Available A irradiação de alimentos é um tratamento pós-colheita bastante promissor, capaz de atuar tanto na desinfestação como no aumento da vida útil de frutos. O mamão é um fruto com curta vida pós-colheita, levando de 6 a 12 dias para atingir a senescência. Além disto, a infestação por larvas de mosca diminui sensivelmente o seu potencial de exportação. O objetivo deste trabalho foi verificar a ação de uma dose de 0,5 kGy de irradiação gama, normalmente utilizada para desinfestação e aumento de vida útil dos frutos, sobre o comportamento dos carboidratos solúveis durante o amadurecimento do mamão (Carica papaya L. cv. Solo, e sobre as enzimas relacionadas ao metabolismo da sacarose: sacarose sintase (SS, sacarose-fosfato sintase (SPS e invertases ácida e neutra. Os resultados demonstraram que a irradiação não exerce efeito sobre o teor de glicose e frutose, ou sobre a respiração e atividade da SS. Entretanto, a composição dos açúcares solúveis totais e da sacarose, e a atividade da SPS e das invertases foram afetados.Food irradiation is one of the most promising treatments that can be utilized for fruits desinfestation and extension of shelf life. The authors studied the influence of 0,5 kGy of Gamma irradiation on the soluble carbohydrates composition of papaya (Carica papaya L. cv. Solo fruit, and on sucrose metabolizing enzymes: sucrose synthase (SS, sucrose-phosphate synthase (SPS, acid and neutral invertases activities, during ripening. The results demonstred that ethylene production, total soluble sugars, sucrose content, and sucrose-phosphate synthase and invertases activities were affected by irradiation, but not respiration, glucose and fructose content, and SS activity.

  16. Carbohydrate to protein ratio in food and cognitive performance in the morning.

    Science.gov (United States)

    Fischer, Karina; Colombani, Paolo C; Langhans, Wolfgang; Wenk, Caspar

    2002-03-01

    The effect of different carbohydrate to protein ratios in food on cognitive functions and the relation between postprandial metabolic and cognitive changes were studied in 15 healthy male students. Subjects were tested in three sessions, separated by 1 week, for short-term changes in mood states, objective cognitive functions, blood parameters, and indirect calorimetry using a repeated-measures, counterbalanced cross-over design. Measurements were made after an overnight fast before and hourly during 3.5 h after test meal ingestion. The isoenergetic (1670 kJ) test meals consisted of three carbohydrate to protein ratios, i.e. a carbohydrate-rich (CHO[4:1]), balanced (BAL[1:1]), and protein-rich (PRO[1:4]) meal, respectively. Overall accuracy in short-term memory was best after the PRO[1:4] meal concomitant to the least variation in glucose metabolism and glucagon to insulin ratio (GIR). Related to changes in glucose metabolism and/or in the ratios of large neutral amino acids (LNAA), respectively, attention and decision times were transiently improved within the first hour after the CHO[4:1] meal, whereas after the first hour the BAL[1:1] and PRO[1:4] meal resulted in improved performance. Overall reaction times of a central task were fastest after the BAL[1:1] meal concomitant to the highest overall tyrosine (Tyr) to LNAA ratio. Our findings suggest that the carbohydrate to protein ratio in food specifically influences higher cognitive functions in the morning. Except for a transient positive effect of rising blood glucose after a carbohydrate-rich meal, a protein-rich or balanced meal seems to result in better overall cognitive performance presumably because of less variation in glucose metabolism and/or higher modulation in LNAA ratios indicated by the overall GIR. PMID:11897269

  17. Carbohydrates - Guidelines on Parenteral Nutrition, Chapter 5

    OpenAIRE

    Bolder, U; Ebener, C.; Hauner, H.; Jauch, KW; Kreymann, G.; Ockenga, J.; Traeger, K.; Working group for developing the guidelines for parenteral nutrition of The German Association for Nutritional Medicine

    2009-01-01

    The main role of carbohydrates in the human body is to provide energy. Carbohydrates should always be infused with PN (parenteral nutrition) in combination with amino acids and lipid emulsions to improve nitrogen balance. Glucose should be provided as a standard carbohydrate for PN, whereas the use of xylite is not generally recommended. Fructose solutions should not be used for PN. Approximately 60% of non-protein energy should be supplied as glucose with an intake of 3.0-3.5 g/kg body weigh...

  18. Molecular simulations of carbohydrate-protein complexes

    OpenAIRE

    Eid, Sameh Mansour Abbas

    2013-01-01

    I. Generation and validation of a free-energy model for carbohydrate binding. Carbohy-drates play a key role in a variety of physiological and pathological processes and, hence, represent a rich source for the development of novel therapeutic agents. Being able to predict binding mode and binding affinity is an essential, yet lacking, aspect of the stru-cture-based design of carbohydrate-based ligands. To this end, we assembled a diverse data set of 316 carbohydrate–protein crystal structu...

  19. Utilization of carbohydrates by radiation processing

    Science.gov (United States)

    Kume, T.; Nagasawa, N.; Yoshii, F.

    2002-03-01

    Upgrading and utilization of carbohydrates such as chitosan, sodium alginate, carrageenan, cellulose, pectin have been investigated for recycling these bio-resources and reducing the environmental pollution. These carbohydrates were easily degraded by irradiation and various kinds of biological activities such as anti-microbial activity, promotion of plant growth, suppression of heavy metal stress, phytoalexins induction, etc. were induced. On the other hand, some carbohydrate derivatives, carboxymethylcellulose and carboxymethylstarch, could be crosslinked under certain radiation condition and produce the biodegradable hydrogel for medical and agricultural use.

  20. 20℃下急性缺氧胁迫对红耳龟糖类代谢和抗氧化防御系统的影响%EFFECTS OF ACUTE HYPOXIA STRESS ON CARBOHYDRATE METABOLISM AND ANTIOXIDANT DEFENSE AT 20 IN THE ℃ TURTLE TRACHEMYS SCRIPTS ELEGANS

    Institute of Scientific and Technical Information of China (English)

    叶双; 黄来旺; 崔琳; 王梦霞; 黄晶晶; 冯照军

    2011-01-01

    淡水红耳龟Trachemys scripts elegans 是研究耐缺氧的模式动物之一,其对缺氧胁迫具有较完善的应激机制.该文研究了20℃下急性缺氧胁迫对红耳龟的糖类代谢和抗氧化防御系统的影响,以探讨红耳龟对急性缺氧胁迫的适应.采用生物化学方法对不同组织中的乳酸、糖原、血糖、Na+-K+-ATP 酶、超氧化物歧化酶(SOD)、过氧化氢酶(CAT)和丙二醛(MDA)进行测定.结果显示,随着缺氧时间的延长,肝脏和骨骼肌糖原逐渐被消耗,肝脏、心肌和骨骼肌乳酸含量逐渐升高,血乳酸和血糖含量急速上升,ATP 酶活性有所下降;SOD 和CAT 活性先升高后回落,MDA 含量也相应地变化.结果提示,龟在20℃急性缺氧环境中,糖类以无氧呼吸方式快速分解,抗氧化防御系统及时启动清除氧自由基.%The survival strategy of animals in environment stress is one of ecology hot projects for scholars to study nowadays, the problem that biochemical physiology responses of animals to hypoxia environment is focused by scholars for long-term. As a famous model, the freshwater red-eared turtle, Trachemys scripts elegans, is one of the hypoxia-tolerant animals for studying in biology and medicine field, especially at lower temperature, and it has more excellent mechanisms to adapt hypoxia stress. Effects of acute hypoxia stress on carbohydrate metabolism and antioxidant defense of red-eared turtle was studied at 20 °C, and the adaptation of the turtle to acute hypoxia stress was discussed in this paper. Within the thermostat at 20°C, the red-eared turtles were randomly divided into four test groups (at 3, 6, 12 and 24h respectively of hypoxia in the containers with oxygen-poor water) and one control group (at Oh). We took out five turtles respectively on time, the blood was collected from its heart, then the liver, cardiac muscle and skeletal muscle were cut, and tissue homogenate solution was prepared as normal method. The lactate

  1. Carbohydrate Catabolism in Azospirillum amazonense

    OpenAIRE

    Martínez-Drets, G.; Fabiano, E.; Cardona, A

    1985-01-01

    The nitrogen fixer Azospirillum amazonense grew on the various disaccharides, hexoses, and pentoses tested in this study but not on polyols and on some tricarboxylic acid cycle intermediates. An active transport system was detected for sucrose and glucose but not for mannitol and 2-ketoglutarate. Six A. amazonense strains were examined for 16 carbon-metabolizing enzymes, and the results indicate that these strains employ the Entner-Doudoroff pathway to catabolize sucrose, fructose, and glucos...

  2. Metabolic Syndrome – Epidemiological Problem?

    OpenAIRE

    Gierach, Marcin; Junik, Roman

    2013-01-01

    The main components of the metabolic syndrome (MetS) are abdominal obesity, hypertension, lipid disorders such as aterogenic dyslipidemia and carbohydrates disorders such as impaired fasting glucose or diabetes mellitus type 2 The first definition of MetS was described by Reaven in 1988 and was named as syndrome X. In the United States of America, the metabolic syndrome affects about 25 % of US adults. In Poland, data based on NATPOL PLUS study, according to NCEP-ATP III criteria, metabolic s...

  3. Workshop to establish databases of carbohydrate spectra

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    1995-12-31

    The workshop was organized to formulate guidelines for establishing spectral databases of complex carbohydrates. The databases will enable the scientific community to avoid the great waste of research effort and funds that frequently occurs when carbohydrate chemists are forced to duplicate the structural characterization of previously characterized complex carbohydrates. Chemists waste their effort on repetitive characterizations because in the absence of spectral databases they are unaware they are analyzing a known molecule until they have completely determined its structure. Chemists will be able to avoid much of this wasted effort when the collections of mass and of nuclear magnetic resonance (NMR) spectra initiated at the workshop are subsequently developed into searchable databases. Then scientists only need query the databases with the spectrum or with information defining the spectrum of an unidentified carbohydrate to find out if it has been previously characterized.

  4. Dietary Carbohydrates and Childhood Functional Abdominal Pain.

    Science.gov (United States)

    Chumpitazi, Bruno P; Shulman, Robert J

    2016-01-01

    Childhood functional gastrointestinal disorders (FGIDs) affect a large number of children throughout the world. Carbohydrates (which provide the majority of calories consumed in the Western diet) have been implicated both as culprits for the etiology of symptoms and as potential therapeutic agents (e.g., fiber) in childhood FGIDs. In this review, we detail how carbohydrate malabsorption may cause gastrointestinal symptoms (e.g., bloating) via the physiologic effects of both increased osmotic activity and increased gas production from bacterial fermentation. Several factors may play a role, including: (1) the amount of carbohydrate ingested; (2) whether ingestion is accompanied by a meal or other food; (3) the rate of gastric emptying (how quickly the meal enters the small intestine); (4) small intestinal transit time (the time it takes for a meal to enter the large intestine after first entering the small intestine); (5) whether the meal contains bacteria with enzymes capable of breaking down the carbohydrate; (6) colonic bacterial adaptation to one's diet, and (7) host factors such as the presence or absence of visceral hypersensitivity. By detailing controlled and uncontrolled trials, we describe how there is a general lack of strong evidence supporting restriction of individual carbohydrates (e.g., lactose, fructose) for childhood FGIDs. We review emerging evidence suggesting that a more comprehensive restriction of fermentable oligosaccharides, disaccharides, monosaccharides and polyols (FODMAP) may be effective. Finally, we review how soluble fiber (a complex carbohydrate) supplementation via randomized controlled intervention trials in childhood functional gastrointestinal disorders has demonstrated efficacy. PMID:27355647

  5. Partial characterization of the carbohydrates of the eggs (oncospheres) of the tapeworm, Hymenolepis diminuta.

    Science.gov (United States)

    Pappas, P W; Durka, G M

    1995-02-01

    The carbohydrate constituents of whole eggs and fractions derived from the eggs of Hymenolepis diminuta were partly characterized. Whole eggs contained 9.6 ng of carbohydrate (phenol-sulphuric acid-positive material) per egg, and 3.6 ng of glucose. Analysis of hydrolysed eggs by HPLC demonstrated the presence of glucose, galactose, glycerol, N-acetylgalactosamine, and 2-deoxyribose. Isolated egg shells and the KOH-stable, ethanol-precipitable fraction (putative glycogen) of eggs contained 26 and 76%, respectively, of the total carbohydrate associated with eggs. Glucose and galactose were present in both of these samples, but only glucose was present in the KOH-stable, ethanol-precipitable fraction isolated from chemically deshelled eggs; thus, the source of the galactose in the KOH-stable, ethanol-precipitable fraction isolated from whole eggs was the egg-shells. These data confirm earlier reports that the shells contain carbohydrate; the data demonstrate further that only two monosaccharides (glucose and galactose) are present in the shells, and that they are present as high molecular weight polymers. The ethanol-soluble fraction of eggs contained 2.5% of the total carbohydrate in eggs, and glucose accounted for < 1% of the ethanol-soluble carbohydrate. Analysis of the ethanol-soluble fraction by HPLC demonstrated that the predominant monosaccharides were glycerol and mannose, with smaller quantities of 2-deoxyribose and 2-deoxyglucose. The absence of a 'free pool' of glucose in eggs, and the presence of large amounts of glycerol and mannose, suggest that the pathways of intermediary carbohydrate metabolism in eggs might be very different from those in adult tapeworms. PMID:7885741

  6. Design, synthesis and evaluation of 2-deoxy-2-iodovinyl-branched carbohydrates as potential brain imaging agents

    International Nuclear Information System (INIS)

    Radioiodinated carbohydrates such as 2-deoxy-2-iodo-D-glucose and 3-deoxy-3-iodo-D-glucose undergo facile chemical or in vivo deiodination which precludes their use as radiotracers of glucose metabolism in tissues. To overcome the problems resulting from in vivo deiodination, we explored the concept of stabilizing radioiodide on a model carbohydrate, (E)-C-3-iodovinyl-D-allose (10) as an iodovinyl moiety. This agent did not exhibit brain specificity but showed low in vivo deiodination which demonstrated for the first time that radioiodide can be stabilized on a carbohydrate. The goal of this study was to develop a deoxy-branched carbohydrate with radioiodide stabilized as a vinyliodide with the objective of achieving high brain uptake. 10 refs., 1 fig., 1 tab

  7. Design, synthesis and evaluation of 2-deoxy-2-iodovinyl-branched carbohydrates as potential brain imaging agents

    Energy Technology Data Exchange (ETDEWEB)

    Goodman, M.M.; Callahan, A.P.; Knapp, F.F. Jr.

    1986-01-01

    Radioiodinated carbohydrates such as 2-deoxy-2-iodo-D-glucose and 3-deoxy-3-iodo-D-glucose undergo facile chemical or in vivo deiodination which precludes their use as radiotracers of glucose metabolism in tissues. To overcome the problems resulting from in vivo deiodination, we explored the concept of stabilizing radioiodide on a model carbohydrate, (E)-C-3-iodovinyl-D-allose (10) as an iodovinyl moiety. This agent did not exhibit brain specificity but showed low in vivo deiodination which demonstrated for the first time that radioiodide can be stabilized on a carbohydrate. The goal of this study was to develop a deoxy-branched carbohydrate with radioiodide stabilized as a vinyliodide with the objective of achieving high brain uptake. 10 refs., 1 fig., 1 tab.

  8. Sequence Classification: 890773 [

    Lifescience Database Archive (English)

    Full Text Available oline as sole nitrogen source; deficiency of the human homolog causes HPII, an autosomal recessive inborn error of metabolism; Put2p || http://www.ncbi.nlm.nih.gov/protein/6321826 ...

  9. Genetics Home Reference: congenital sucrase-isomaltase deficiency

    Science.gov (United States)

    ... down into glucose and another simple sugar called fructose, and maltose is broken down into two glucose ... MedlinePlus Encyclopedia: Inborn errors of metabolism MedlinePlus Encyclopedia: Malabsorption These resources from MedlinePlus offer information about the ...

  10. Disease: H01032 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available -acetylglutamate is the absolutely required allosteric activator of the first urea cycle enzyme carbamoylpho...S) deficiency is a rare inborn error of metabolism affecting ammonia detoxification in the urea cycle. The N

  11. The Acute Temporary Peritoneal Dialysis in Neonates: A Five-Year Experience

    Directory of Open Access Journals (Sweden)

    Seyedeh Fatemeh Khatami

    2015-06-01

    Conclusion: PD is an effective treatment for seriously ill newborns with life-threatening conditions such as acute renal failure or certain inborn errors of metabolism. However, mortality rate remains high due to the severity of underlying diseases and comorbidities.

  12. Neonatal lactic acidosis, complex I/IV deficiency, and fetal cerebral disruption

    NARCIS (Netherlands)

    van Straaten, HLM; van Tintelen, JP; Trijbels, JMF; van den Heuvel, LP; Troost, D; Rozemuller, JM; Duran, M; de Vries, LS; Schuelke, M; Barth, PG

    2005-01-01

    Cerebral developmental abnormalities occur in various inborn errors of metabolism including peroxisomal deficiencies, pyruvate dehydrogenase complex deficiency and others. Associations with abnormalities of the respiratory chain are rare. Here we report male and female siblings with microcephaly, a

  13. Skeletal muscle carnitine loading increases energy expenditure, modulates fuel metabolism gene networks and prevents body fat accumulation in humans

    OpenAIRE

    Stephens, Francis B; Wall, Benjamin T.; Marimuthu, Kanagaraj; Shannon, Chris E; Constantin-Teodosiu, Dumitru; Macdonald, Ian A.; Greenhaff, Paul L

    2013-01-01

    Twelve weeks of daily L-carnitine and carbohydrate feeding in humans increases skeletal muscle total carnitine content, and prevents body mass accrual associated with carbohydrate feeding alone. Here we determined the influence of L-carnitine and carbohydrate feeding on energy metabolism, body fat mass andmuscle expression of fuel metabolism genes. Twelve males exercised at 50% maximal oxygen consumption for 30 min once before and once after 12 weeks of twice daily feeding of 80 g carbohyd...

  14. THE IMPORTANCE OF DIETARY CARBOHYDRATE IN HUMAN EVOLUTION.

    Science.gov (United States)

    Hardy, Karen; Brand-Miller, Jennie; Brown, Katherine D; Thomas, Mark G; Copeland, Les

    2015-09-01

    ABSTRACT We propose that plant foods containing high quantities of starch were essential for the evolution of the human phenotype during the Pleistocene. Although previous studies have highlighted a stone tool-mediated shift from primarily plant-based to primarily meat-based diets as critical in the development of the brain and other human traits, we argue that digestible carbohydrates were also necessary to accommodate the increased metabolic demands of a growing brain. Furthermore, we acknowledge the adaptive role cooking played in improving the digestibility and palatability of key carbohydrates. We provide evidence that cooked starch, a source of preformed glucose, greatly increased energy availability to human tissues with high glucose demands, such as the brain, red blood cells, and the developing fetus. We also highlight the auxiliary role copy number variation in the salivary amylase genes may have played in increasing the importance of starch in human evolution following the origins of cooking. Salivary amylases are largely ineffective on raw crystalline starch, but cooking substantially increases both their energy-yielding potential and glycemia. Although uncertainties remain regarding the antiquity of cooking and the origins of salivary amylase gene copy number variation, the hypothesis we present makes a testable prediction that these events are correlated. PMID:26591850

  15. Learning from Errors

    OpenAIRE

    Martínez-Legaz, Juan Enrique; Soubeyran, Antoine

    2003-01-01

    We present a model of learning in which agents learn from errors. If an action turns out to be an error, the agent rejects not only that action but also neighboring actions. We find that, keeping memory of his errors, under mild assumptions an acceptable solution is asymptotically reached. Moreover, one can take advantage of big errors for a faster learning.

  16. Physiogenomic analysis of weight loss induced by dietary carbohydrate restriction

    Directory of Open Access Journals (Sweden)

    Wood Richard J

    2006-05-01

    Full Text Available Abstract Background Diets that restrict carbohydrate (CHO have proven to be a successful dietary treatment of obesity for many people, but the degree of weight loss varies across individuals. The extent to which genetic factors associate with the magnitude of weight loss induced by CHO restriction is unknown. We examined associations among polymorphisms in candidate genes and weight loss in order to understand the physiological factors influencing body weight responses to CHO restriction. Methods We screened for genetic associations with weight loss in 86 healthy adults who were instructed to restrict CHO to a level that induced a small level of ketosis (CHO ~10% of total energy. A total of 27 single nucleotide polymorphisms (SNPs were selected from 15 candidate genes involved in fat digestion/metabolism, intracellular glucose metabolism, lipoprotein remodeling, and appetite regulation. Multiple linear regression was used to rank the SNPs according to probability of association, and the most significant associations were analyzed in greater detail. Results Mean weight loss was 6.4 kg. SNPs in the gastric lipase (LIPF, hepatic glycogen synthase (GYS2, cholesteryl ester transfer protein (CETP and galanin (GAL genes were significantly associated with weight loss. Conclusion A strong association between weight loss induced by dietary CHO restriction and variability in genes regulating fat digestion, hepatic glucose metabolism, intravascular lipoprotein remodeling, and appetite were detected. These discoveries could provide clues to important physiologic adaptations underlying the body mass response to CHO restriction.

  17. Effect of salidroside on carbohydrate metabolism and serum levels of inflammatory cytokines in rats under negative psychological stress induced by experimental navigation and intensive exercise%红景天苷对负性心理应激下高强度运动大鼠糖代谢及炎症因子水平的影响

    Institute of Scientific and Technical Information of China (English)

    彭丽; 王扬天; 王群; 孙玲君; 王坚; 李洁

    2012-01-01

    Objective To determine the effects of salidroside on the carbohydrate metabolism and the serum levels of inflammatory cytokines in rats under negative psychological stress induced by experimental navigation and intensive exercise. Methods A total of 30 6-week Sprague-Dawley rats (SD rats) were randomly and equally allocated into 3 groups, quiescent control group (group A), training control group (group B) and salidroside treatment group (group C). Rats from group A received no stimulation, and administered a 7-day intragastrical injection of normal saline. Rats from group B underwent a 10-day run training with increasing load on treadmill and received 7-day intragastrical administration of normal saline. Group C were subjected to the same procedure of run training as group B, and received 7-day intragastrical administration of 0.4g/kg salidroside. Then, all the rats underwent experimental navigation and intensive exercise. Blood samples were immediately obtained for detection of blood glucose and the levels of inflammatory response-related cytokines, high sensitive-C reactive protein (hs-CRP), tumor necrosis factor a (TNF-α), interleukin 6(IL-6), and endothelin 1 (ET-1) by ELISA. Results As compared with group A, the levels of blood glucose, hs-CRP, TNF-α, IL-6, ET-1 was significantly increased in group B (P<0.05), while, the levels of blood glucose was significantly increased (P<0.05), and those of hs-CRP, IL-6, and ET-1 were significantly decreased in group C (P<0.05). The levels of hs-CRP, TNF-a, IL-6, ET-1 in group C were also significantly decreased than in group B (P<0.05). The level of blood glucose in group C showed a decreasing tendency. Conclusion Negative psychological stress and intensive exercise lead to disorders of glucose metabolism and increase the production of inflammatory cytokines in rats. Salidroside therapy reduces the levels of blood glucose and inflammatory cytokines in rats under stress, and thus improve glucose metabolism and

  18. Effect of carbohydrate ingestion subsequent to carbohydrate supercompensation on endurance performance.

    Science.gov (United States)

    Kang, J; Robertson, R J; Denys, B G; DaSilva, S G; Visich, P; Suminski, R R; Utter, A C; Goss, F L; Metz, K F

    1995-12-01

    This investigation determined whether carbohydrate ingestion during prolonged moderate-intensity exercise enhanced endurance performance when the exercise was preceded by carbohydrate supercompensation. Seven male trained cyclists performed two trials at an initial power output corresponding to 71 +/- 1% of their peak oxygen consumption. During the trials, subjects ingested either a 6% glucose/sucrose (C) solution or an equal volume of artificially flavored and sweetened placebo (P) every 20 min throughout exercise. Both C and P were preceded by a 6-day carbohydrate supercompensation procedure in which subjects undertook a depletion-taper exercise sequence in conjunction with a moderate- and high-carbohydrate diet regimen. Statistical analysis of time to exhaustion, plasma glucose concentration, carbohydrate oxidation rate, fat oxidation rate, and plasma glycerol concentration indicated that in spite of a carbohydrate supercompensation procedure administered prior to exercise, carbohydrate ingestion during exercise can exert an additional ergogenic effect by preventing a decline in blood glucose levels and maintaining carbohydrate oxidation during the later stages of moderate-intensity exercise. PMID:8605519

  19. Carbohydrate mouth rinse and caffeine improves high-intensity interval running capacity when carbohydrate restricted.

    Science.gov (United States)

    Kasper, Andreas M; Cocking, Scott; Cockayne, Molly; Barnard, Marcus; Tench, Jake; Parker, Liam; McAndrew, John; Langan-Evans, Carl; Close, Graeme L; Morton, James P

    2016-08-01

    We tested the hypothesis that carbohydrate mouth rinsing, alone or in combination with caffeine, augments high-intensity interval (HIT) running capacity undertaken in a carbohydrate-restricted state. Carbohydrate restriction was achieved by performing high-intensity running to volitional exhaustion in the evening prior to the main experimental trials and further refraining from carbohydrate intake in the post-exercise and overnight period. On the subsequent morning, eight males performed 45-min steady-state (SS) exercise (65% [Formula: see text]) followed by HIT running to exhaustion (1-min at 80% [Formula: see text]interspersed with 1-min walking at 6 km/h). Subjects completed 3 trials consisting of placebo capsules (administered immediately prior to SS and immediately before HIT) and placebo mouth rinse at 4-min intervals during HIT (PLACEBO), placebo capsules but 10% carbohydrate mouth rinse (CMR) at corresponding time-points or finally, caffeine capsules (200 mg per dose) plus 10% carbohydrate mouth rinse (CAFF + CMR) at corresponding time-points. Heart rate, capillary glucose, lactate, glycerol and NEFA were not different at exhaustion during HIT (P > 0.05). However, HIT capacity was different (P mouth rinsing and caffeine ingestion improves exercise capacity undertaken in carbohydrate-restricted states. Such nutritional strategies may be advantageous for those athletes who deliberately incorporate elements of training in carbohydrate-restricted states (i.e. the train-low paradigm) into their overall training programme in an attempt to strategically enhance mitochondrial adaptations of skeletal muscle. PMID:26035740

  20. Efeitos do implante subdérmico de acetato de nomegestrol sobre o metabolismo de carboidratos, lipoproteínas séricas e função hepática The effects of nomegestrol acetate subdermal implant on carbohydrate metabolism, serum lipoproteins and on hepatic function

    Directory of Open Access Journals (Sweden)

    Ione Barbosa

    1998-07-01

    implant. A significant decrease in total cholesterol (p<0.05 was observed in the third month and of HDL-C (p<0.01 in the sixth month. All lipoprotein alterations were inconsistent and values were within the normal range. Significant increases in fast glucose (p<0.05 and p<0.01 were observed in the third and sixth months, respectively. Significant SGOT decreases (p<0.05, p<0.01 and p<0.05 were observed in months 6, 18 and 24, respectively, and of SGPT (p<0.05 in month 18. Significant bilirubin increase (p<0.05 was observed only in the third month of implant use. All these variations remained within the normal range. Conclusions: these results show that, within the normal range, fasting glucose variations do not correlate with alterations in insulin levels. The slight serum lipoprotein, SGTO, SGPT and insulin alterations were transient. No clinical effects could be observed regarding lipoproteins, carbohydrate metabolism, insulin levels and liver function among the users during the two years.

  1. Resultados clínicos e metabólicos da abreviação do jejum com carboidratos na revascularização cirúrgica do miocárdio Clinical and metabolic results of fasting abbreviation with carbohydrates in coronary artery bypass graft surgery

    Directory of Open Access Journals (Sweden)

    Gibran Roder Feguri

    2012-03-01

    Full Text Available INTRODUÇÃO: Existe pouca informação sobre abreviação do jejum pré-operatório com oferta de líquidos ricos em carboidratos (CHO nas operações cardiovasculares. OBJETIVOS: Avaliar variáveis clínicas, segurança do método e efeitos no metabolismo de pacientes submetidos à abreviação do jejum na cirurgia de revascularização do miocárdio (CRVM. MÉTODOS: Quarenta pacientes submetidos à CRVM foram randomizados para receberem 400 ml (6 horas antes e 200 mL (2 horas antes de maltodextrina a 12,5% (Grupo I, n=20 ou apenas água (Grupo II, n=20 antes da indução anestésica. Foram avaliadas diversas variáveis clínicas no perioperatório e também a resistência insulínica (RI pelo índice de Homa-IR e pela necessidade de insulina exógena; além da função excretora da célula beta pancreática pelo Homa-Beta e controle glicêmico por exames de glicemia capilar. RESULTADOS: Não ocorreram óbitos, broncoaspiração, mediastinite, infarto agudo do miocárdio ou acidente vascular encefálico perioperatórios. Fibrilação atrial ocorreu em dois pacientes de cada grupo e complicações infecciosas não diferiram entre os grupos (P=0,611. Pacientes do Grupo I apresentaram dois dias a menos de internação hospitalar (P=0,025 e um dia a menos na UTI (P0,05. Declínio da produção endógena de insulina ocorreu em ambos os grupos (PINTRODUCTION: Limited information is available about preoperative fasting abbreviation with administration of liquid enriched with carbohydrates (CHO in cardiovascular surgeries. OBJECTIVES: To evaluate clinical variables, security of the method and effects on the metabolism of patients undergoing fasting abbreviation in coronary artery bypass graft (CABG surgery. METHODS: Forty patients undergoing CABG were randomized to receive 400 ml (6 hours before and 200 ml (2 hours before of maltodextrin at 12.5% (Group I, n=20 or just water (Group II, n=20 before anesthetic induction. Perioperative clinical variables

  2. Effects of polymeric carbohydrates on growth and development

    DEFF Research Database (Denmark)

    Knudsen, Knud Erik Bach

    largest energy contributor. The 2 types of polysaccharides, however, have different fates and functions in the gastrointestinal tract and lead to different metabolites upon digestion. Pancreatic and mucosal enzymes in the small intestine break down the majority of starch, while NSP primarily are degraded...... by the microflora in the large intestine. Starch degradation leads to the release of glucose which is absorbed by an active absorption process that triggers the release of insulin from the pancreas, whereas the fermentation of NSP to short-chain fatty acids (SCFA: acetate, propionate and butyrate...... (digestible, metabolized and net energy); available energy relative to protein is crucial for performance and carcass quality; second, the proportion of starch to NSP will influence rate and type of metabolites (glucose vs. SCFA) deriving from carbohydrate assimilation, and finally, type of starch (types A, B...

  3. Should we use carbohydrate deficient transferrin as a marker for alcohol abusers?

    OpenAIRE

    Das, Subir Kumar; Vasudevan, D. M.

    2004-01-01

    Carbohydrate deficient transferrin (CDT) is one of the conventional markers for chronic alcohol consumption, is used by researchers and clinicians. A number of enzymes are affected by ethanol intake. The induction or inhibition of sialyl transferase and plasma sialidase may be involved in the CDT level elevation. An alteration of protein transport during post-translational modification could be a primary mechanism in the impairment of protein metabolism associated with chronic alcohol abuse. ...

  4. The effect of dietary carbohydrates in women with polycystic ovary syndrome

    DEFF Research Database (Denmark)

    Frary, Johanna M; Bjerre, Kamilla P.; Glintborg, D;

    2016-01-01

    AIM: Weight loss improves ovulation, testosterone levels and insulin resistance in women with polycystic ovarian syndrome (PCOS), but the optimal diet composition is disputed. A diet low in carbohydrates (LCD) may be superior to a standard diet in terms of improving fertility, endocrine/metabolic......, hyperandrogenemia, glucoseand insulin levels, insulin resistance and satiety hormones, whereas diet composition is of less importance. A LCD has an additional effect to caloric restriction in terms of weight loss. Conclusions are summarized as evidence-based recommendations....

  5. Potential effect of ultrasound on carbohydrates.

    Science.gov (United States)

    Bera, Smritilekha; Mondal, Dhananjoy; Martin, Jacob T; Singh, Man

    2015-06-17

    The use of ultrasound has emerged as one of the most useful alternative energy sources for the synthesis of carbohydrate-derived biologically and pharmaceutically potential compounds. Spectacular advances have been made in the field of sonication-assisted organic reactions, which are known for producing superior yields, enhanced reactivity of the reactant, improved stereoselectivity, and shortened reaction times. Orthogonal protection-deprotection reactions and/or modification and manipulation of functional groups in carbohydrates are common synthetic steps in carbohydrate chemistry. These reaction steps can be driven by the ultrasonic energy generated by acoustic cavitation via the formation and subsequent collapse of ultrasound-induced bubbles. The ultrasound-assisted synthesis of differently functionalised monosaccharides is useful in a wide variety of applications of carbohydrate chemistry such as the glycosylation of oligosaccharides, one pot domino reactions, thioglycoside syntheses, azidoglycoside syntheses, 1,3-dipolar cycloaddition reactions, and syntheses of natural products. This review article covers ultrasound-mediated reactions on carbohydrates that have been described in the literature since 2000. PMID:25954862

  6. Efficacy and outcome of expanded newborn screening for metabolic diseases - Report of 10 years from South-West Germany *

    Directory of Open Access Journals (Sweden)

    Mengel Eugen

    2011-06-01

    Full Text Available Abstract Background National newborn screening programmes based on tandem-mass spectrometry (MS/MS and other newborn screening (NBS technologies show a substantial variation in number and types of disorders included in the screening panel. Once established, these methods offer the opportunity to extend newborn screening panels without significant investment and cost. However, systematic evaluations of newborn screening programmes are rare, most often only describing parts of the whole process from taking blood samples to long-term evaluation of outcome. Methods In a prospective single screening centre observational study 373 cases with confirmed diagnosis of a metabolic disorder from a total cohort of 1,084,195 neonates screened in one newborn screening laboratory between January 1, 1999, and June 30, 2009 and subsequently treated and monitored in five specialised centres for inborn errors of metabolism were examined. Process times for taking screening samples, obtaining results, initiating diagnostic confirmation and starting treatment as well as the outcome variables metabolic decompensations, clinical status, and intellectual development at a mean age of 3.3 years were evaluated. Results Optimal outcome is achieved especially for the large subgroup of patients with medium-chain acyl-CoA dehydrogenase deficiency. Kaplan-Meier-analysis revealed disorder related patterns of decompensation. Urea cycle disorders, organic acid disorders, and amino acid disorders show an early high and continuous risk, medium-chain acyl-CoA dehydrogenase deficiency a continuous but much lower risk for decompensation, other fatty acid oxidation disorders an intermediate risk increasing towards the end of the first year. Clinical symptoms seem inevitable in a small subgroup of patients with very early disease onset. Later decompensation can not be completely prevented despite pre-symptomatic start of treatment. Metabolic decompensation does not necessarily result in

  7. Temperature-mediated phase transformation, pore geometry and pore hysteresis transformation of borohydride derived in-born porous zirconium hydroxide nanopowders

    Science.gov (United States)

    Nayak, Nadiya B.; Nayak, Bibhuti B.

    2016-05-01

    Development of in-born porous nature of zirconium hydroxide nanopowders through a facile hydrogen (H2) gas-bubbles assisted borohydride synthesis route using sodium borohydride (NaBH4) and novel information on the temperature-mediated phase transformation, pore geometry as well as pore hysteresis transformation of in-born porous zirconium hydroxide nanopowders with the help of X-ray diffraction (XRD), Brunauer–Emmett–Teller (BET) isotherm and Transmission Electron Microscopy (TEM) images are the main theme of this research work. Without any surfactants or pore forming agents, the borohydride derived amorphous nature of porous powders was stable up to 500 °C and then the seed crystals start to develop within the loose amorphous matrix and trapping the inter-particulate voids, which led to develop the porous nature of tetragonal zirconium oxide at 600 °C and further sustain this porous nature as well as tetragonal phase of zirconium oxide up to 800 °C. The novel hydrogen (H2) gas-bubbles assisted borohydride synthesis route led to develop thermally stable porous zirconium hydroxide/oxide nanopowders with an adequate pore size, pore volume, and surface area and thus these porous materials are further suggested for promising use in different areas of applications.

  8. Differential effects of two fermentable carbohydrates on central appetite regulation and body composition.

    Directory of Open Access Journals (Sweden)

    Tulika Arora

    Full Text Available BACKGROUND: Obesity is rising at an alarming rate globally. Different fermentable carbohydrates have been shown to reduce obesity. The aim of the present study was to investigate if two different fermentable carbohydrates (inulin and β-glucan exert similar effects on body composition and central appetite regulation in high fat fed mice. METHODOLOGY/PRINCIPAL FINDINGS: Thirty six C57BL/6 male mice were randomized and maintained for 8 weeks on a high fat diet containing 0% (w/w fermentable carbohydrate, 10% (w/w inulin or 10% (w/w β-glucan individually. Fecal and cecal microbial changes were measured using fluorescent in situ hybridization, fecal metabolic profiling was obtained by proton nuclear magnetic resonance ((1H NMR, colonic short chain fatty acids were measured by gas chromatography, body composition and hypothalamic neuronal activation were measured using magnetic resonance imaging (MRI and manganese enhanced MRI (MEMRI, respectively, PYY (peptide YY concentration was determined by radioimmunoassay, adipocyte cell size and number were also measured. Both inulin and β-glucan fed groups revealed significantly lower cumulative body weight gain compared with high fat controls. Energy intake was significantly lower in β-glucan than inulin fed mice, with the latter having the greatest effect on total adipose tissue content. Both groups also showed an increase in the numbers of Bifidobacterium and Lactobacillus-Enterococcus in cecal contents as well as feces. β-Glucan appeared to have marked effects on suppressing MEMRI associated neuronal signals in the arcuate nucleus, ventromedial hypothalamus, paraventricular nucleus, periventricular nucleus and the nucleus of the tractus solitarius, suggesting a satiated state. CONCLUSIONS/SIGNIFICANCE: Although both fermentable carbohydrates are protective against increased body weight gain, the lower body fat content induced by inulin may be metabolically advantageous. β-Glucan appears to suppress

  9. Response of C57Bl/6 mice to a carbohydrate-free diet

    Directory of Open Access Journals (Sweden)

    Borghjid Saihan

    2012-07-01

    Full Text Available Abstract High fat feeding in rodents generally leads to obesity and insulin resistance whereas in humans this is only seen if dietary carbohydrate is also high, the result of the anabolic effect of poor regulation of glucose and insulin. A previous study of C57Bl/6 mice (Kennedy AR, et al.: Am J Physiol Endocrinol Metab (2007 262 E1724-1739 appeared to show the kind of beneficial effects of calorie restriction that is seen in humans but that diet was unusually low in protein (5%. In the current study, we tested a zero-carbohydrate diet that had a higher protein content (20%. Mice on the zero-carbohydrate diet, despite similar caloric intake, consistently gained more weight than animals consuming standard chow, attaining a dramatic difference by week 16 (46.1 ± 1.38 g vs. 30.4 ± 1.00 g for the chow group. Consistent with the obese phenotype, experimental mice had fatty livers and hearts as well as large fat deposits in the abdomino-pelvic cavity, and showed impaired glucose clearance after intraperitoneal injection. In sum, the response of mice to a carbohydrate-free diet was greater weight gain and metabolic disruptions in distinction to the response in humans where low carbohydrate diets cause greater weight loss than isocaloric controls. The results suggest that rodent models of obesity may be most valuable in the understanding of how metabolic mechanisms can work in ways different from the effect in humans.

  10. Minimally refined biomass fuel. [carbohydrate-water-alcohol mixture

    Energy Technology Data Exchange (ETDEWEB)

    Pearson, R.K.; Hirschfeld, T.B.

    1981-03-26

    A minimally refined fluid composition, suitable as a fuel mixture and derived from biomass material, is comprised of one or more water-soluble carbohydrates such as sucrose, one or more alcohols having less than four carbons, and water. The carbohydrate provides the fuel source; water-solubilizes the carbohydrate; and the alcohol aids in the combustion of the carbohydrate and reduces the viscosity of the carbohydrate/water solution. Because less energy is required to obtain the carbohydrate from the raw biomass than alcohol, an overall energy savings is realized compared to fuels employing alcohol as the primary fuel.

  11. Field error lottery

    Energy Technology Data Exchange (ETDEWEB)

    Elliott, C.J.; McVey, B. (Los Alamos National Lab., NM (USA)); Quimby, D.C. (Spectra Technology, Inc., Bellevue, WA (USA))

    1990-01-01

    The level of field errors in an FEL is an important determinant of its performance. We have computed 3D performance of a large laser subsystem subjected to field errors of various types. These calculations have been guided by simple models such as SWOOP. The technique of choice is utilization of the FELEX free electron laser code that now possesses extensive engineering capabilities. Modeling includes the ability to establish tolerances of various types: fast and slow scale field bowing, field error level, beam position monitor error level, gap errors, defocusing errors, energy slew, displacement and pointing errors. Many effects of these errors on relative gain and relative power extraction are displayed and are the essential elements of determining an error budget. The random errors also depend on the particular random number seed used in the calculation. The simultaneous display of the performance versus error level of cases with multiple seeds illustrates the variations attributable to stochasticity of this model. All these errors are evaluated numerically for comprehensive engineering of the system. In particular, gap errors are found to place requirements beyond mechanical tolerances of {plus minus}25{mu}m, and amelioration of these may occur by a procedure utilizing direct measurement of the magnetic fields at assembly time. 4 refs., 12 figs.

  12. Error And Error Analysis In Language Study

    OpenAIRE

    Zakaria, Teuku Azhari

    2015-01-01

    Students make mistakes during their language learning course; orally, written, listening or reading comprehension. Making mistakes is inevitable and considered natural in ones’ inter-lingual process. Believed to be part of the learning process, making error and mistake are not bad thing; as everybody would experience the same. Both students and teacher will benefit from the event as both will learn what has been done well and what needs to be reviewed and rehearsed. Understanding error and th...

  13. The Error in Total Error Reduction

    OpenAIRE

    Witnauer, James E.; Urcelay, Gonzalo P.; Miller, Ralph R.

    2013-01-01

    Most models of human and animal learning assume that learning is proportional to the discrepancy between a delivered outcome and the outcome predicted by all cues present during that trial (i.e., total error across a stimulus compound). This total error reduction (TER) view has been implemented in connectionist and artificial neural network models to describe the conditions under which weights between units change. Electrophysiological work has revealed that the activity of dopamine neurons i...

  14. Medical errors in neurosurgery

    Directory of Open Access Journals (Sweden)

    John D Rolston

    2014-01-01

    23.7-27.8% were technical, related to the execution of the surgery itself, highlighting the importance of systems-level approaches to protecting patients and reducing errors. Conclusions: Overall, the magnitude of medical errors in neurosurgery and the lack of focused research emphasize the need for prospective categorization of morbidity with judicious attribution. Ultimately, we must raise awareness of the impact of medical errors in neurosurgery, reduce the occurrence of medical errors, and mitigate their detrimental effects.

  15. Programming Errors in APL.

    Science.gov (United States)

    Kearsley, Greg P.

    This paper discusses and provides some preliminary data on errors in APL programming. Data were obtained by analyzing listings of 148 complete and partial APL sessions collected from student terminal rooms at the University of Alberta. Frequencies of errors for the various error messages are tabulated. The data, however, are limited because they…

  16. Assessment of (patho)physiologic alterations in equine muscle metabolism

    NARCIS (Netherlands)

    Westermann, C.M.

    2008-01-01

    This thesis focussed on the diagnostic use of metabolic products and enzymes found in plasma, urine and muscle of the horse, the identification of which can reveal physiological or pathological changes in muscle metabolism. In this thesis analyses of carbohydrate-, lipid- and protein metabolites hav

  17. TISSUE SLICES IN THE STUDY OF LUNG METABOLISM AND TOXICOLOGY

    Science.gov (United States)

    Lung tissue slices are model systems for the study of pulmonary metabolism. Because of the speed and simplicity of slice preparation, lung slices have been used in studies of oxygen, amino acid, carbohydrate and lipid utilization and adenine nucleotide metabolism. Dose-response c...

  18. Crystallization of carbohydrate oxidase from Microdochium nivale

    Czech Academy of Sciences Publication Activity Database

    Dušková, Jarmila; Dohnálek, Jan; Skálová, Tereza; Ostergaard, L. H.; Fuglsang, C. C.; Kolenko, Petr; Štěpánková, Andrea; Hašek, Jindřich

    2009-01-01

    Roč. 65, č. 6 (2009), s. 638-640. ISSN 1744-3091 R&D Projects: GA AV ČR IAA500500701; GA ČR GA305/07/1073 Institutional research plan: CEZ:AV0Z40500505 Keywords : carbohydrate oxidase * crystallization * data processing Subject RIV: CD - Macromolecular Chemistry Impact factor: 0.551, year: 2009

  19. The diagenesis of carbohydrates by hydrogen sulfide

    Science.gov (United States)

    Mango, Frank D.

    1983-08-01

    Carbohydrates react with hydrogen sulfide under low temperature (100° to 200°C) yielding a variety of organosulfur compounds including thiophenes, thiols, sulfides and sulfones. A polymer is also produced, whose elemental composition is within the range of natural coals. When reductive dehydration is carried out in the presence of hydrocarbon, organosulfur compounds are formed in the carbon number range of the hydrocarbon used. In these processes, an active hydrogen transfer catalyst is produced which facilitates the passage of hydrogen between normal paraffins and saccharide units, distributing sulfur between these two families primarily in the form of thiophene rings. The simplicity of these systems - H 2S, carbohydrates, H 2O, hydrocarbon - and the facility of the chemistry would suggest that the carbohydrates and hydrogen sulfide may be important agents in the diagenetic processes leading to petroleum and coal. Carbohydrate reduction by hydrogen sulfide may constitute an important route through which certain organosulfur compounds found in petroleum and coal entered these materials in early diagenesis.

  20. The clinical impact of carbohydrate malabsorption.

    Science.gov (United States)

    Born, Peter

    2011-03-01

    Malabsorption of carbohydrates such as fructose, lactose or sorbitol can often be detected among patients suffering from so-called non specific abdominal complaints. Sometimes the differential diagnosis may be difficult. So far successful treatment consists of dietary interventions only. Nevertheless, many questions are remaining still unanswered. PMID:21429446

  1. Separation and quantification of microalgal carbohydrates.

    Science.gov (United States)

    Templeton, David W; Quinn, Matthew; Van Wychen, Stefanie; Hyman, Deborah; Laurens, Lieve M L

    2012-12-28

    Structural carbohydrates can constitute a large fraction of the dry weight of algal biomass and thus accurate identification and quantification is important for summative mass closure. Two limitations to the accurate characterization of microalgal carbohydrates are the lack of a robust analytical procedure to hydrolyze polymeric carbohydrates to their respective monomers and the subsequent identification and quantification of those monosaccharides. We address the second limitation, chromatographic separation of monosaccharides, here by identifying optimum conditions for the resolution of a synthetic mixture of 13 microalgae-specific monosaccharides, comprised of 8 neutral, 2 amino sugars, 2 uronic acids and 1 alditol (myo-inositol as an internal standard). The synthetic 13-carbohydrate mix showed incomplete resolution across 11 traditional high performance liquid chromatography (HPLC) methods, but showed improved resolution and accurate quantification using anion exchange chromatography (HPAEC) as well as alditol acetate derivatization followed by gas chromatography (for the neutral- and amino-sugars only). We demonstrate the application of monosaccharide quantification using optimized chromatography conditions after sulfuric acid analytical hydrolysis for three model algae strains and compare the quantification and complexity of monosaccharides in analytical hydrolysates relative to a typical terrestrial feedstock, sugarcane bagasse. PMID:23177152

  2. Nutrient and carbohydrate partitioning in sorghum stover

    International Nuclear Information System (INIS)

    Sorghum [Sorghum bicolor (L.) Moench] stover has been demonstrated to be a potential biomass energy source. Complete aboveground crop removal, however, can result in soil degradation. Differential dry matter, nutrient, and carbohydrate partitioning by sorghum cultivars may allow management strategies that return certain parts to the field while removing other portions for alternative uses, such as energy production. A field study was conducted to determine N,P,K, nonstructural carbohydrate, cellulose hemicellulose, and lignin distributions in stover of three diverse sorghum cultivars of differing harvest indices. Determinations were based on total vegetative biomass; total blades; total stalks; and upper middle, and lower blades and stalks. Concentrations of N and P were higher in blades than stalks and generally declines from upper to lower stover parts. Large carbohydrate and lignin concentration differences were observed on the basis of cultivar and stover part. Greater nutrient partitioning to the upper third of the intermediate and forage-type sorghum stovers was observed as compared to the conventional grain cultivar. Stover carbohydrates for all cultivars were mainly contained in the lower two-thirds of the stalk fraction. A system was proposed for returning upper stover portion to soil, while removing remaining portions for alternative uses

  3. General Properties, Occurrence, and Preparation of Carbohydrates

    Science.gov (United States)

    Robyt, John F.

    D-Glucose and its derivatives and analogues, N-acetyl-D-glucosamine, N-acetyl-D-muramic acid, D-glucopyranosyl uronic acid, and D-glucitol represent 99.9% of the carbohydrates on the earth. D-Glucose is found in the free state in human blood and in the combined state in disaccharides, sucrose, lactose, and α,α-trehalose, in cyclic dextrins, and in polysaccharides, starch, glycogen, cellulose, dextrans; N-acetyl-D-glucosamine and an analogue N-acetyl-D-muramic acid are found in bacterial cell wall polysaccharide, murein, along with teichoic acids made up of poly-glycerol or -ribitol phosphodiesters. Other carbohydrates, D-mannose, D-mannuronic acid, D-galactose, N-acetyl-D-galactosamine, D-galacturonic acid, D-iduronic acid, L-guluronic acid, L-rhamnose, L-fucose, D-xylose, and N-acetyl-D-neuraminic acid are found in glycoproteins, hemicelluloses, glycosaminoglycans, and polysaccharides of plant exudates, bacterial capsules, alginates, and heparin. D-Ribofuranose-5-phosphate is found in many coenzymes and is the backbone of RNAs (ribonucleic acid), and 2-deoxy-D-ribofuranose-5-phosphate is the backbone of DNA (deoxyribonucleic acid). D-Fructofuranose is found in sucrose, inulin, and levan. The general properties and occurrence of these carbohydrates and general methods of isolation and preparation of carbohydrates are presented.

  4. 'Biomoleculas': cellular metabolism didactic software

    International Nuclear Information System (INIS)

    'Biomoleculas' is a software that deals with topics such as the digestion, cellular metabolism and excretion of nutrients. It is a pleasant, simple and didactic guide, made by and for students. In this program, each biomolecule (carbohydrates, lipids and proteins) is accompanied until its degradation and assimilation by crossing and interrelating the different metabolic channels to finally show the destination of the different metabolites formed and the way in which these are excreted. It is used at present as a teaching-learning process tool by the chair of Physiology and Biophysics at the Facultad de Ingenieria - Universidad Nacional de Entre Rios

  5. Fate of Carbohydrates and Lignin during Composting and Mycelium Growth of Agaricus bisporus on Wheat Straw Based Compost.

    Science.gov (United States)

    Jurak, Edita; Punt, Arjen M; Arts, Wim; Kabel, Mirjam A; Gruppen, Harry

    2015-01-01

    In wheat straw based composting, enabling growth of Agaricus bisporus mushrooms, it is unknown to which extent the carbohydrate-lignin matrix changes and how much is metabolized. In this paper we report yields and remaining structures of the major components. During the Phase II of composting 50% of both xylan and cellulose were metabolized by microbial activity, while lignin structures were unaltered. During A. bisporus' mycelium growth (Phase III) carbohydrates were only slightly consumed and xylan was found to be partially degraded. At the same time, lignin was metabolized for 45% based on pyrolysis GC/MS. Remaining lignin was found to be modified by an increase in the ratio of syringyl (S) to guaiacyl (G) units from 0.5 to 0.7 during mycelium growth, while fewer decorations on the phenolic skeleton of both S and G units remained. PMID:26436656

  6. Fate of Carbohydrates and Lignin during Composting and Mycelium Growth of Agaricus bisporus on Wheat Straw Based Compost.

    Directory of Open Access Journals (Sweden)

    Edita Jurak

    Full Text Available In wheat straw based composting, enabling growth of Agaricus bisporus mushrooms, it is unknown to which extent the carbohydrate-lignin matrix changes and how much is metabolized. In this paper we report yields and remaining structures of the major components. During the Phase II of composting 50% of both xylan and cellulose were metabolized by microbial activity, while lignin structures were unaltered. During A. bisporus' mycelium growth (Phase III carbohydrates were only slightly consumed and xylan was found to be partially degraded. At the same time, lignin was metabolized for 45% based on pyrolysis GC/MS. Remaining lignin was found to be modified by an increase in the ratio of syringyl (S to guaiacyl (G units from 0.5 to 0.7 during mycelium growth, while fewer decorations on the phenolic skeleton of both S and G units remained.

  7. Metabolic Adaptation to Muscle Ischemia

    Science.gov (United States)

    Cabrera, Marco E.; Coon, Jennifer E.; Kalhan, Satish C.; Radhakrishnan, Krishnan; Saidel, Gerald M.; Stanley, William C.

    2000-01-01

    Although all tissues in the body can adapt to varying physiological/pathological conditions, muscle is the most adaptable. To understand the significance of cellular events and their role in controlling metabolic adaptations in complex physiological systems, it is necessary to link cellular and system levels by means of mechanistic computational models. The main objective of this work is to improve understanding of the regulation of energy metabolism during skeletal/cardiac muscle ischemia by combining in vivo experiments and quantitative models of metabolism. Our main focus is to investigate factors affecting lactate metabolism (e.g., NADH/NAD) and the inter-regulation between carbohydrate and fatty acid metabolism during a reduction in regional blood flow. A mechanistic mathematical model of energy metabolism has been developed to link cellular metabolic processes and their control mechanisms to tissue (skeletal muscle) and organ (heart) physiological responses. We applied this model to simulate the relationship between tissue oxygenation, redox state, and lactate metabolism in skeletal muscle. The model was validated using human data from published occlusion studies. Currently, we are investigating the difference in the responses to sudden vs. gradual onset ischemia in swine by combining in vivo experimental studies with computational models of myocardial energy metabolism during normal and ischemic conditions.

  8. A functional genomics approach to establish the complement of carbohydrate transporters in Streptococcus pneumoniae.

    Directory of Open Access Journals (Sweden)

    Alessandro Bidossi

    Full Text Available The aerotolerant anaerobe Streptococcus pneumoniae is part of the normal nasopharyngeal microbiota of humans and one of the most important invasive pathogens. A genomic survey allowed establishing the occurrence of twenty-one phosphotransferase systems, seven carbohydrate uptake ABC transporters, one sodium:solute symporter and a permease, underlining an exceptionally high capacity for uptake of carbohydrate substrates. Despite high genomic variability, combined phenotypic and genomic analysis of twenty sequenced strains did assign the substrate specificity only to two uptake systems. Systematic analysis of mutants for most carbohydrate transporters enabled us to assign a phenotype and substrate specificity to twenty-three transport systems. For five putative transporters for galactose, pentoses, ribonucleosides and sulphated glycans activity was inferred, but not experimentally confirmed and only one transport system remains with an unknown substrate and lack of any functional annotation. Using a metabolic approach, 80% of the thirty-two fermentable carbon substrates were assigned to the corresponding transporter. The complexity and robustness of sugar uptake is underlined by the finding that many transporters have multiple substrates, and many sugars are transported by more than one system. The present work permits to draw a functional map of the complete arsenal of carbohydrate utilisation proteins of pneumococci, allows re-annotation of genomic data and might serve as a reference for related species. These data provide tools for specific investigation of the roles of the different carbon substrates on pneumococcal physiology in the host during carriage and invasive infection.

  9. A Functional Genomics Approach to Establish the Complement of Carbohydrate Transporters in Streptococcus pneumoniae

    Science.gov (United States)

    Bidossi, Alessandro; Mulas, Laura; Decorosi, Francesca; Colomba, Leonarda; Ricci, Susanna; Pozzi, Gianni; Deutscher, Josef; Viti, Carlo; Oggioni, Marco Rinaldo

    2012-01-01

    The aerotolerant anaerobe Streptococcus pneumoniae is part of the normal nasopharyngeal microbiota of humans and one of the most important invasive pathogens. A genomic survey allowed establishing the occurrence of twenty-one phosphotransferase systems, seven carbohydrate uptake ABC transporters, one sodium∶solute symporter and a permease, underlining an exceptionally high capacity for uptake of carbohydrate substrates. Despite high genomic variability, combined phenotypic and genomic analysis of twenty sequenced strains did assign the substrate specificity only to two uptake systems. Systematic analysis of mutants for most carbohydrate transporters enabled us to assign a phenotype and substrate specificity to twenty-three transport systems. For five putative transporters for galactose, pentoses, ribonucleosides and sulphated glycans activity was inferred, but not experimentally confirmed and only one transport system remains with an unknown substrate and lack of any functional annotation. Using a metabolic approach, 80% of the thirty-two fermentable carbon substrates were assigned to the corresponding transporter. The complexity and robustness of sugar uptake is underlined by the finding that many transporters have multiple substrates, and many sugars are transported by more than one system. The present work permits to draw a functional map of the complete arsenal of carbohydrate utilisation proteins of pneumococci, allows re-annotation of genomic data and might serve as a reference for related species. These data provide tools for specific investigation of the roles of the different carbon substrates on pneumococcal physiology in the host during carriage and invasive infection. PMID:22428019

  10. Error-prone signalling.

    Science.gov (United States)

    Johnstone, R A; Grafen, A

    1992-06-22

    The handicap principle of Zahavi is potentially of great importance to the study of biological communication. Existing models of the handicap principle, however, make the unrealistic assumption that communication is error free. It seems possible, therefore, that Zahavi's arguments do not apply to real signalling systems, in which some degree of error is inevitable. Here, we present a general evolutionarily stable strategy (ESS) model of the handicap principle which incorporates perceptual error. We show that, for a wide range of error functions, error-prone signalling systems must be honest at equilibrium. Perceptual error is thus unlikely to threaten the validity of the handicap principle. Our model represents a step towards greater realism, and also opens up new possibilities for biological signalling theory. Concurrent displays, direct perception of quality, and the evolution of 'amplifiers' and 'attenuators' are all probable features of real signalling systems, yet handicap models based on the assumption of error-free communication cannot accommodate these possibilities. PMID:1354361

  11. Carbohydrate deficient transferrin (CDT) in alcoholic cirrhosis: a kinetic study

    DEFF Research Database (Denmark)

    Henriksen, Jens Henrik Sahl; Grønbaek, M; Møller, Søren;

    1997-01-01

    controls (n = 8), which indicates a slow turnover rate of carbohydrate deficient transferrin. Food ingestion did not affect the circulating level of carbohydrate deficient transferrin, and the analysis of carbohydrate deficient transferrin was almost unaffected by the presence of ethanol in plasma within...... alcohol intake, but the overlap is substantial in patients with cirrhosis. Carbohydrate deficient transferrin has a low turnover rate in both patients with cirrhosis and normals....

  12. 五味子对模拟航渡及高强度运动大鼠垂体-睾丸轴及糖代谢的影响%Effects of fructus schisandrae on the function of the pituitary-testis axis and carbohydrate metabolism in rats undergoing experimental navigation and high-intensity exercise

    Institute of Scientific and Technical Information of China (English)

    夏萍; 孙玲君; 王坚

    2011-01-01

    目的:研究五味子对实验性模拟航渡及高强度运动雄性大鼠睾丸轴功能、超微结构及糖代谢的影响.方法:选取34只SD雄性大鼠,分为安静对照组(A组)、应激对照组(B组)、五味子处理组(C组),B组和C组参照Bedford的训练模式进行训练,分别以生理盐水和五味子灌胃1周,每日2次.实验结束后,取血以放免法测定血清睾酮(T)、皮质酮(CORT)、血清黄体生成素(LH)、葡萄糖(Glu)水平,同时随机取垂体、睾丸组织在电子显微镜下观察其超微结构.结果:①A组血Glu为(5.22±2.13)mmol/L,T为(0.61±0.68)ng/ml,CORT为(4.67±1.19)ng/ml;B组血Glu为(9.41±2.56)mmol/L,较A组水平升高(P0.05);C组血CORT为(3.55±3.52)ng/ml,Glu为(5.09±1.64)mmol/L,较B组水平均显著降低(P0.05);LH在3组之间均无显著变化(P>0.05).②电子显微镜观察发现,B组垂体细胞胞质中分泌颗粒较A组显著减少,c组与B组相比胞质中分泌颗粒的数目明显增多,B组睾丸Leydig细胞线粒体肿胀,电子密度增高,嵴减少或消失,C组睾丸Leydig细胞中线粒体结构趋向于正常,可见大小不等深染的分泌颗粒.结论:模拟航渡及高强度运动训练抑制睾丸功能并影响细胞内超微结构,灌服五味子对垂体-睾丸轴具有保护作用,同时可降低应激大鼠的血糖水平.%Objective: To investigate the influence of fructus schisandrae (FS) on the function of the pituitary-testis axis and carbohydrate metabolism in male rats undergoing experimental navigation and strenuous exercise. Methods: Thirty-four SD rats were randomly allocated into three groups, quiescent control (A), stress control (B) and F3 (C). Those in Groups B and C received 10 days of Benford's high-intensity training, followed by 7 days of intrngastric administration of normal saline and FS, respectively. Blood samples were immediately obtained at the end of the experiment for the measurement of the levels of serum testosterone ( T), corticosterone

  13. Investigation on Carbohydrate Counting Method in Type 1 Diabetic Patients

    Directory of Open Access Journals (Sweden)

    Osman Son

    2014-01-01

    Full Text Available Objective. The results from Diabetes Control and Complications Trial (DCCT have propounded the importance of the approach of treatment by medical nutrition when treating diabetes mellitus (DM. During this study, we tried to inquire carbohydrate (Kh count method’s positive effects on the type 1 DM treatment’s success as well as on the life quality of the patients. Methods. 22 of 37 type 1 DM patients who applied to Eskişehir Osmangazi University, Faculty of Medicine Hospital, Department of Endocrinology and Metabolism, had been treated by Kh count method and 15 of them are treated by multiple dosage intensive insulin treatment with applying standard diabetic diet as a control group and both of groups were under close follow-up for 6 months. Required approval was taken from the Ethical Committee of Eskişehir Osmangazi University, Medical Faculty, as well as informed consent from the patients. The body weight of patients who are treated by carbohydrate count method and multiple dosage intensive insulin treatment during the study beginning and after 6-month term, body mass index, and body compositions are analyzed. A short life quality and medical research survey applied. At statistical analysis, t-test, chi-squared test, and Mann-Whitney U test were used. Results. There had been no significant change determined at glycemic control indicators between the Kh counting group and the standard diabetic diet and multiple dosage insulin treatment group in our study. Conclusion. As a result, Kh counting method which offers a flexible nutrition plan to diabetic individuals is a functional method.

  14. Natural Selection at the Brush-Border: Adaptations to Carbohydrate Diets in Humans and Other Mammals.

    Science.gov (United States)

    Pontremoli, Chiara; Mozzi, Alessandra; Forni, Diego; Cagliani, Rachele; Pozzoli, Uberto; Menozzi, Giorgia; Vertemara, Jacopo; Bresolin, Nereo; Clerici, Mario; Sironi, Manuela

    2015-09-01

    Dietary shifts can drive molecular evolution in mammals and a major transition in human history, the agricultural revolution, favored carbohydrate consumption. We investigated the evolutionary history of nine genes encoding brush-border proteins involved in carbohydrate digestion/absorption. Results indicated widespread adaptive evolution in mammals, with several branches experiencing episodic selection, particularly strong in bats. Many positively selected sites map to functional protein regions (e.g., within glucosidase catalytic crevices), with parallel evolution at SI (sucrase-isomaltase) and MGAM (maltase-glucoamylase). In human populations, five genes were targeted by positive selection acting on noncoding variants within regulatory elements. Analysis of ancient DNA samples indicated that most derived alleles were already present in the Paleolithic. Positively selected variants at SLC2A5 (fructose transporter) were an exception and possibly spread following the domestication of specific fruit crops. We conclude that agriculture determined no major selective event at carbohydrate metabolism genes in humans, with implications for susceptibility to metabolic disorders. PMID:26319403

  15. Natural Selection at the Brush-Border: Adaptations to Carbohydrate Diets in Humans and Other Mammals

    Science.gov (United States)

    Pontremoli, Chiara; Mozzi, Alessandra; Forni, Diego; Cagliani, Rachele; Pozzoli, Uberto; Menozzi, Giorgia; Vertemara, Jacopo; Bresolin, Nereo; Clerici, Mario; Sironi, Manuela

    2015-01-01

    Dietary shifts can drive molecular evolution in mammals and a major transition in human history, the agricultural revolution, favored carbohydrate consumption. We investigated the evolutionary history of nine genes encoding brush-border proteins involved in carbohydrate digestion/absorption. Results indicated widespread adaptive evolution in mammals, with several branches experiencing episodic selection, particularly strong in bats. Many positively selected sites map to functional protein regions (e.g., within glucosidase catalytic crevices), with parallel evolution at SI (sucrase-isomaltase) and MGAM (maltase-glucoamylase). In human populations, five genes were targeted by positive selection acting on noncoding variants within regulatory elements. Analysis of ancient DNA samples indicated that most derived alleles were already present in the Paleolithic. Positively selected variants at SLC2A5 (fructose transporter) were an exception and possibly spread following the domestication of specific fruit crops. We conclude that agriculture determined no major selective event at carbohydrate metabolism genes in humans, with implications for susceptibility to metabolic disorders. PMID:26319403

  16. Postprandial Responses to Lipid and Carbohydrate Ingestion in Repeated Subcutaneous Adipose Tissue Biopsies in Healthy Adults

    Directory of Open Access Journals (Sweden)

    Aimee L. Dordevic

    2015-07-01

    Full Text Available Adipose tissue is a primary site of meta-inflammation. Diet composition influences adipose tissue metabolism and a single meal can drive an inflammatory response in postprandial period. This study aimed to examine the effect lipid and carbohydrate ingestion compared with a non-caloric placebo on adipose tissue response. Thirty-three healthy adults (age 24.5 ± 3.3 year (mean ± standard deviation (SD; body mass index (BMI 24.1 ± 3.2 kg/m2, were randomised into one of three parallel beverage groups; placebo (water, carbohydrate (maltodextrin or lipid (dairy-cream. Subcutaneous, abdominal adipose tissue biopsies and serum samples were collected prior to (0 h, as well as 2 h and 4 h after consumption of the beverage. Adipose tissue gene expression levels of monocyte chemoattractant protein-1 (MCP-1, interleukin 6 (IL-6 and tumor necrosis factor-α (TNF-α increased in all three groups, without an increase in circulating TNF-α. Serum leptin (0.6-fold, p = 0.03 and adipose tissue leptin gene expression levels (0.6-fold, p = 0.001 decreased in the hours following the placebo beverage, but not the nutrient beverages. Despite increased inflammatory cytokine gene expression in adipose tissue with all beverages, suggesting a confounding effect of the repeated biopsy method, differences in metabolic responses of adipose tissue and circulating adipokines to ingestion of lipid and carbohydrate beverages were observed.

  17. Sodium-Glucose Cotransporter 2 Inhibitor and a Low Carbohydrate Diet Affect Gluconeogenesis and Glycogen Content Differently in the Kidney and the Liver of Non-Diabetic Mice

    OpenAIRE

    Atageldiyeva, Kuralay; Fujita, Yukihiro; Yanagimachi, Tsuyoshi; Mizumoto, Katsutoshi; Takeda, Yasutaka; Honjo, Jun; Takiyama, Yumi; Abiko, Atsuko; Makino, Yuichi; Haneda, Masakazu

    2016-01-01

    A low carbohydrate diet (LCHD) as well as sodium glucose cotransporter 2 inhibitors (SGLT2i) may reduce glucose utilization and improve metabolic disorders. However, it is not clear how different or similar the effects of LCHD and SGLT2i are on metabolic parameters such as insulin sensitivity, fat accumulation, and especially gluconeogenesis in the kidney and the liver. We conducted an 8-week study using non-diabetic mice, which were fed ad-libitum with LCHD or a normal carbohydrate diet (NCH...

  18. Investigations into selective metabolic aspects of bifidobacteria: carbohydrate metabolism, fatty acid biosynthesis and plasmid biology

    OpenAIRE

    O'Connell, Kerry Joan

    2014-01-01

    The gastrointestinal tract (GIT) is a diverse ecosystem, and is colonised by a diverse array of bacteria, of which bifidobacteria are a significant component. Bifidobacteria are Gram-positive, saccharolytic, non-motile, non-sporulating, anaerobic, Y-shaped bacteria, which possess a high GC genome content. Certain bifidobacteria possess the ability to produce conjugated linoleic acid (CLA) from linoleic acid (LA) by a biochemical pathway that is hypothesised to be achieved via a linoleic isome...

  19. Uncorrected refractive errors

    OpenAIRE

    Naidoo, Kovin S; Jyoti Jaggernath

    2012-01-01

    Global estimates indicate that more than 2.3 billion people in the world suffer from poor vision due to refractive error; of which 670 million people are considered visually impaired because they do not have access to corrective treatment. Refractive errors, if uncorrected, results in an impaired quality of life for millions of people worldwide, irrespective of their age, sex and ethnicity. Over the past decade, a series of studies using a survey methodology, referred to as Refractive Error S...

  20. Carbohydrate plasma expanders for passive tumor targeting

    DEFF Research Database (Denmark)

    Hoffmann, Stefan; Caysa, Henrike; Kuntsche, Judith;

    2013-01-01

    The objective of this study was to investigate the suitability of carbohydrate plasma volume expanders as a novel polymer platform for tumor targeting. Many synthetic polymers have already been synthesized for targeted tumor therapy, but potential advantages of these carbohydrates include...... inexpensive synthesis, constant availability, a good safety profile, biodegradability and the long clinical use as plasma expanders. Three polymers have been tested for cytotoxicity and cytokine activation in cell cultures and conjugated with a near-infrared fluorescent dye: hydroxyethyl starches (HES 200 k......Da and HES 450 kDa) and dextran (DEX 500 kDa). Particle size and molecular weight distribution were determined by asymmetric flow field-flow fractionation (AF4). The biodistribution was investigated non-invasively in nude mice using multispectral optical imaging. The most promising polymer conjugate was...

  1. Fine carbohydrate recognition of Euphorbia milii lectin.

    Science.gov (United States)

    Irazoqui, Fernando J; Vozari-Hampe, Magdolna M; Lardone, Ricardo D; Villarreal, Marcos A; Sendra, Victor G; Montich, Guillermo G; Trindade, Vera M; Clausen, Henrik; Nores, Gustavo A

    2005-10-14

    Glycans are key structures involved in biological processes such as cell attachment, migration, and invasion. Information coded on cell-surface glycans is frequently deciphered by proteins, as lectins, that recognize specific carbohydrate topology. Here, we describe the fine carbohydrate specificity of Euphorbia milii lectin (EML). Competitive assays using various sugars showed that GalNAc was the strongest inhibitor, and that the hydroxyl axial position of C4 and acetamido on C2 of GalNAc are critical points of EML recognition. A hydrophobic locus adjacent to GalNAc is also an important region for EML binding. Direct binding assays of EML revealed a stereochemical requirement for a structure adjacent to terminal GalNAc, showing that GalNAc residue is a necessary but not sufficient condition for EML interaction. The capacity of EML to bind epithelial tumor cells makes it a potentially useful tool for study of some over-expressed GalNAc glycoconjugates. PMID:16122701

  2. Correction for quadrature errors

    DEFF Research Database (Denmark)

    Netterstrøm, A.; Christensen, Erik Lintz

    1994-01-01

    In high bandwidth radar systems it is necessary to use quadrature devices to convert the signal to/from baseband. Practical problems make it difficult to implement a perfect quadrature system. Channel imbalance and quadrature phase errors in the transmitter and the receiver result in error signals......, which appear as self-clutter in the radar image. When digital techniques are used for generation and processing or the radar signal it is possible to reduce these error signals. In the paper the quadrature devices are analyzed, and two different error compensation methods are considered. The practical...

  3. Error coding simulations

    Science.gov (United States)

    Noble, Viveca K.

    1993-01-01

    There are various elements such as radio frequency interference (RFI) which may induce errors in data being transmitted via a satellite communication link. When a transmission is affected by interference or other error-causing elements, the transmitted data becomes indecipherable. It becomes necessary to implement techniques to recover from these disturbances. The objective of this research is to develop software which simulates error control circuits and evaluate the performance of these modules in various bit error rate environments. The results of the evaluation provide the engineer with information which helps determine the optimal error control scheme. The Consultative Committee for Space Data Systems (CCSDS) recommends the use of Reed-Solomon (RS) and convolutional encoders and Viterbi and RS decoders for error correction. The use of forward error correction techniques greatly reduces the received signal to noise needed for a certain desired bit error rate. The use of concatenated coding, e.g. inner convolutional code and outer RS code, provides even greater coding gain. The 16-bit cyclic redundancy check (CRC) code is recommended by CCSDS for error detection.

  4. Lysophosphatidylinositol Signalling and Metabolic Diseases.

    Science.gov (United States)

    Arifin, Syamsul A; Falasca, Marco

    2016-01-01

    Metabolism is a chemical process used by cells to transform food-derived nutrients, such as proteins, carbohydrates and fats, into chemical and thermal energy. Whenever an alteration of this process occurs, the chemical balance within the cells is impaired and this can affect their growth and response to the environment, leading to the development of a metabolic disease. Metabolic syndrome, a cluster of several metabolic risk factors such as abdominal obesity, insulin resistance, high cholesterol and high blood pressure, and atherogenic dyslipidaemia, is increasingly common in modern society. Metabolic syndrome, as well as other diseases, such as diabetes, obesity, hyperlipidaemia and hypertension, are associated with abnormal lipid metabolism. Cellular lipids are the major component of cell membranes; they represent also a valuable source of energy and therefore play a crucial role for both cellular and physiological energy homeostasis. In this review, we will focus on the physiological and pathophysiological roles of the lysophospholipid mediator lysophosphatidylinositol (LPI) and its receptor G-protein coupled receptor 55 (GPR55) in metabolic diseases. LPI is a bioactive lipid generated by phospholipase A (PLA) family of lipases which is believed to play an important role in several diseases. Indeed LPI can affect various functions such as cell growth, differentiation and motility in a number of cell-types. Recently published data suggest that LPI plays an important role in different physiological and pathological contexts, including a role in metabolism and glucose homeostasis. PMID:26784247

  5. A rapid stereoselective synthesis of fluorinated carbohydrates

    International Nuclear Information System (INIS)

    Acetyl hypofluorite has been added to six unsaturated carbohydrates which contain the vinyl ether moiety. All reactions were rapid (less than 5 min.) at -78 degrees C and gave, with one exception, high yields of isomerically pure products. The hypofluorite was shown to add exclusively in a cis mode and with a strong preference for a particular 'face' of the double bond. As well as the syntheses, NMR data and preferred conformations for the fluorinated products are also discussed

  6. Intake of carbohydrates during pregnancy in obese women is associated with fat mass in the newborn offspring

    DEFF Research Database (Denmark)

    Renault, Kristina M; Carlsen, Emma M; Nørgaard, Kirsten;

    2015-01-01

    BACKGROUND: Transmission of obesity across generations is of concern. Offspring of obese women have short- and long-term increased morbidities. A high intake of carbohydrate during pregnancy combined with impaired glucose tolerance is postulated to result in high birth weight, which is linked to...... subsequent metabolic disease. OBJECTIVE: The objective was to examine the association between carbohydrate intake in obese pregnant women and their offspring's body composition. DESIGN: Secondary analyses were performed in an observational setting of 222 pregnant women with a pregestational BMI (in kg/m(2...... with the offspring's relative fat mass in late (P-trend = 0.006) but not early (P-trend = 0.15) pregnancy. A comparison of mothers in the highest (median: 238 g/d) compared with the lowest (median: 188 g/d) quartile of digestible carbohydrate intake showed a mean adjusted higher value in the offspring...

  7. Glycaemic index of different kinds of carbohydrates in type 2 diabetes.

    Science.gov (United States)

    Donduran, S; Hamulu, F; Cetinkalp, S; Colak, B; Horozoğlu, N; Tüzün, M

    1999-12-01

    An adequate diet provides good metabolic control in diabetics. Since 1981 when Jenkins showed that complex carbohydrates are digested more slowly and raise blood glucose less than simple sugars, many studies have been performed in this field. In this study, seven kinds of carbohydrate-rich food were compared with glucose in 52 Type 2 diabetic patients and 31 normal volunteers. The subject consumed either macaroni, white rice, potatoes, tarhana soup (tarhana includes wheat flour, yoghurt, tomato and green pepper), noodle soup, white or whole wheat bread, or glucose at one-week intervals after an overnight fast. The glycaemic index (GI) of each food was calculated from the area under its glycaemic response curve (AUC) expressed as a percentage of the mean response to glucose. The results showed that the foods ranked from the highest to the lowest GI as follows: white bread; whole wheat bread; macaroni; tarhana soup; white rice; potatoes; noodle soup. PMID:10728183

  8. Starch metabolism in leaves.

    Science.gov (United States)

    Orzechowski, Sławomir

    2008-01-01

    Starch is the most abundant storage carbohydrate produced in plants. The initiation of transitory starch synthesis and degradation in plastids depends mainly on diurnal cycle, post-translational regulation of enzyme activity and starch phosphorylation. For the proper structure of starch granule the activities of all starch synthase isoenzymes, branching enzymes and debranching enzymes are needed. The intensity of starch biosynthesis depends mainly on the activity of AGPase (adenosine 5'-diphosphate glucose pyrophosphorylase). The key enzymes in starch degradation are beta-amylase, isoamylase 3 and disproportionating enzyme. However, it should be underlined that there are some crucial differences in starch metabolism between heterotrophic and autotrophic tissues, e.g. is the ability to build multiprotein complexes responsible for biosynthesis and degradation of starch granules in chloroplasts. The observed huge progress in understanding of starch metabolism was possible mainly due to analyses of the complete Arabidopsis and rice genomes and of numerous mutants with altered starch metabolism in leaves. The aim of this paper is to review current knowledge on transient starch metabolism in higher plants. PMID:18787712

  9. Cell surface carbohydrates as prognostic markers in human carcinomas

    DEFF Research Database (Denmark)

    Dabelsteen, Erik

    1996-01-01

    cell types; within a given tissue, variation in expression may be related to cell maturation. Tumour-associated carbohydrate structures often reflect a certain stage of cellular development; most of these moieties are structures normally found in other adult or embryonic tissues. There is no unique......Tumour development is usually associated with changes in cell surface carbohydrates. These are often divided into changes related to terminal carbohydrate structures, which include incomplete synthesis and modification of normally existing carbohydrates, and changes in the carbohydrate core...... tumour carbohydrate structure, since certain structures which are tumour-related in one organ may be normal constituents of other tissues. Tumour-associated carbohydrate changes have been used in the diagnosis of human cancers. Recently, however, it has been demonstrated that the expression of some...

  10. The Intricate Role of Growth Hormone in Metabolism

    OpenAIRE

    DerekLeroith

    2011-01-01

    Growth hormone (GH), a master regulator of somatic growth, also regulates carbohydrate and lipid metabolism via complex interactions with insulin and insulin-like growth factor-1 (IGF-1). Data from human and rodent studies reveal the importance of GH in insulin synthesis and secretion, lipid metabolism and body fat remodeling. In this review, we will summarize the tissue-specific metabolic effects of GH, with emphasis on recent targets identified to mediate these effects. Furthermore, we will...

  11. Global microarray analysis of carbohydrate use in alkaliphilic hemicellulolytic bacterium Bacillus sp. N16-5.

    Directory of Open Access Journals (Sweden)

    Yajian Song

    Full Text Available The alkaliphilic hemicellulolytic bacterium Bacillus sp. N16-5 has a broad substrate spectrum and exhibits the capacity to utilize complex carbohydrates such as galactomannan, xylan, and pectin. In the monosaccharide mixture, sequential utilization by Bacillus sp. N16-5 was observed. Glucose appeared to be its preferential monosaccharide, followed by fructose, mannose, arabinose, xylose, and galactose. Global transcription profiles of the strain were determined separately for growth on six monosaccharides (glucose, fructose, mannose, galactose, arabinose, and xylose and four polysaccharides (galactomannan, xylan, pectin, and sodium carboxymethylcellulose using one-color microarrays. Numerous genes potentially related to polysaccharide degradation, sugar transport, and monosaccharide metabolism were found to respond to a specific substrate. Putative gene clusters for different carbohydrates were identified according to transcriptional patterns and genome annotation. Identification and analysis of these gene clusters contributed to pathway reconstruction for carbohydrate utilization in Bacillus sp. N16-5. Several genes encoding putative sugar transporters were highly expressed during growth on specific sugars, suggesting their functional roles. Two phosphoenolpyruvate-dependent phosphotransferase systems were identified as candidate transporters for mannose and fructose, and a major facilitator superfamily transporter was identified as a candidate transporter for arabinose and xylose. Five carbohydrate uptake transporter 1 family ATP-binding cassette transporters were predicted to participate in the uptake of hemicellulose and pectin degradation products. Collectively, microarray data improved the pathway reconstruction involved in carbohydrate utilization of Bacillus sp. N16-5 and revealed that the organism precisely regulates gene transcription in response to fluctuations in energy resources.

  12. Correlation of Lactobacillus rhamnosus Genotypes and Carbohydrate Utilization Signatures Determined by Phenotype Profiling.

    Science.gov (United States)

    Ceapa, Corina; Lambert, Jolanda; van Limpt, Kees; Wels, Michiel; Smokvina, Tamara; Knol, Jan; Kleerebezem, Michiel

    2015-08-15

    Lactobacillus rhamnosus is a bacterial species commonly colonizing the gastrointestinal (GI) tract of humans and also frequently used in food products. While some strains have been studied extensively, physiological variability among isolates of the species found in healthy humans or their diet is largely unexplored. The aim of this study was to characterize the diversity of carbohydrate utilization capabilities of human isolates and food-derived strains of L. rhamnosus in relation to their niche of isolation and genotype. We investigated the genotypic and phenotypic diversity of 25 out of 65 L. rhamnosus strains from various niches, mainly human feces and fermented dairy products. Genetic fingerprinting of the strains by amplified fragment length polymorphism (AFLP) identified 11 distinct subgroups at 70% similarity and suggested niche enrichment within particular genetic clades. High-resolution carbohydrate utilization profiling (OmniLog) identified 14 carbon sources that could be used by all of the strains tested for growth, while the utilization of 58 carbon sources differed significantly between strains, enabling the stratification of L. rhamnosus strains into three metabolic clusters that partially correlate with the genotypic clades but appear uncorrelated with the strain's origin of isolation. Draft genome sequences of 8 strains were generated and employed in a gene-trait matching (GTM) analysis together with the publicly available genomes of L. rhamnosus GG (ATCC 53103) and HN001 for several carbohydrates that were distinct for the different metabolic clusters: l-rhamnose, cellobiose, l-sorbose, and α-methyl-d-glucoside. From the analysis, candidate genes were identified that correlate with l-sorbose and α-methyl-d-glucoside utilization, and the proposed function of these genes could be confirmed by heterologous expression in a strain lacking the genes. This study expands our insight into the phenotypic and genotypic diversity of the species L. rhamnosus

  13. SIRT1 and metabolic syndrome

    Directory of Open Access Journals (Sweden)

    Katarzyna Mac-Marcjanek

    2011-04-01

    Full Text Available Both obesity and type 2 diabetes mellitus, two major components of metabolic syndrome, become healthepidemics in the world. Over the past decade, advances in understanding the role of some regulators participatingin lipid and carbohydrate homeostasis have been made.Of them, SIRT1, the mammalian orthologue of the yeast Sir2 protein has been identified. SIRT1 is a nuclearNAD+-dependent deacetylase that targets many transcriptional modulators, including PPAR-α and -γ (peroxisomeproliferator-activated receptors α and γ, PGC-1α (PPAR-γ coactivator-1α, FOXO (forkhead box O proteins,and nuclear factor κB (NF-κB, thereby this enzyme mediates a wide range of physiological processes like apoptosis,fat metabolism, glucose homeostasis, and neurodegeneration.In this article, we discuss how SIRT1 regulates lipid and carbohydrate metabolism, and insulin secretion indifferent metabolic organs/tissue, including liver, muscle, pancreas, and fat. Additionally, the role of this enzymein reduction of inflammatory signalling is highlighted.

  14. The effects of high fat, low carbohydrate and low fat, high carbohydrate diets on tumor necrosis factor superfamily proteins and proinflammatory cytokines in C57BL/6 mice.

    Directory of Open Access Journals (Sweden)

    Mahshid Sirjani

    2014-08-01

    Full Text Available There has been considerable inconsistency regarding the potential relationship between dyslipidemia and bone metabolism. The inflammatory stimulation through the receptor activator of the nuclear factor kappa-B ligand (RANKL/ receptor activator of the nuclear factor kappa-B (RANK/ osteoprotegerin (OPG pathway could be the infrastructural mechanism for hypercholesterolemia-induced bone loss.In this study, we investigated the effect of dyslipidemia on RANKL and OPG alongside with pro-inflammatory cytokines. Thirty male C57Bl/6 mice (4 weeks old were randomized to two purified diet groups (15 animals in each group, high fat, low carbohydrate diet (HFLCD and its matched low fat, high carbohydrate diet (LFHCD. After 12 weeks of feeding in standard situations, the plasma concentration of lipid profile, interleukin (IL 1Beta, IL-6, tumor necrosis factor-alpha (TNF-α and RANKL, OPG, and RANKL: OPG ratio were measured.In the present study, although the body weight significantly increased during 12 weeks in HFLCD and LFHCD groups, there were no significant differences in food intake, food efficiency ratio and weight gain between the two groups. The LFHCD group had significantly higher median RANKL and RANKL/OPG ratio. There was no significant difference in plasma IL-1β, IL-6 and TNF-α concentration between LFHCD and HFLCD groups.These unexpected findings from LFHCD, that seem to be as a result of its higher carbohydrate proportion in comparison to HFLCD, implicate dietary carbohydrate rather than dietary fat as a more significant nutritional factor contributing to change in RANKL level and RANKL: OPG ratio.

  15. The 3-methylglutaconic acidurias: what’s new?

    OpenAIRE

    Wortmann, Saskia B.; Kluijtmans, Leo A.; Engelke, Udo F. H.; Wevers, Ron A.; MORAVA, EVA

    2010-01-01

    The heterogeneous group of 3-methylglutaconic aciduria (3-MGA-uria) syndromes includes several inborn errors of metabolism biochemically characterized by increased urinary excretion of 3-methylglutaconic acid. Five distinct types have been recognized: 3-methylglutaconic aciduria type I is an inborn error of leucine catabolism; the additional four types all affect mitochondrial function through different pathomechanisms. We provide an overview of the expanding clinical spectrum of the 3-MGA-ur...

  16. Metabolism of oligosaccharides and starch in lactobacilli: a review

    Directory of Open Access Journals (Sweden)

    MichaelGänzle

    2012-09-01

    Full Text Available Oligosaccharides, compounds that are composed of 2 – 10 monosaccharide residues, are major carbohydrate sources in habitats populated by lactobacilli. Moreover, oligosaccharide metabolism is essential for ecological fitness of lactobacilli. Disaccharide metabolism by lactobacilli is well understood; however, few data on the metabolism of higher oligosaccharides are available. Research on the ecology of intestinal microbiota as well as the commercial application of prebiotics has shifted the interest from (digestible disaccharides to (indigestible higher oligosaccharides. This review provides an overview on oligosaccharide metabolism in lactobacilli. Emphasis is placed on maltodextrins, isomalto-oligosaccharides, fructo-oligosaccharides, galacto-oligosaccharides, and raffinose-family oligosaccharides. Starch is also considered. Metabolism is discussed on the basis of metabolic studies related to oligosaccharide metabolism, information on the cellular location and substrate specificity of carbohydrate transport systems, glycosyl hydrolases and phorphorylases, and the presence of metabolic genes in genomes of 28 strains of lactobacilli. Metabolic pathways for disaccharide metabolism often also enable the metabolism of tri- and tetrasaccharides. However, with the exception of amylase and levansucrase, metabolic enzymes for oligosaccharide conversion are intracellular and oligosaccharide metabolism is limited by transport. This general restriction to intracellular glycosyl hydrolases differentiates lactobacilli from other bacteria that adapted to intestinal habitats, particularly Bifidobacterium spp.

  17. Metabolic acidosis

    Science.gov (United States)

    Acidosis - metabolic ... Metabolic acidosis occurs when the body produces too much acid. It can also occur when the kidneys are not ... the body. There are several types of metabolic acidosis. Diabetic acidosis develops when acidic substances, known as ...

  18. Medical error and disclosure.

    Science.gov (United States)

    White, Andrew A; Gallagher, Thomas H

    2013-01-01

    Errors occur commonly in healthcare and can cause significant harm to patients. Most errors arise from a combination of individual, system, and communication failures. Neurologists may be involved in harmful errors in any practice setting and should familiarize themselves with tools to prevent, report, and examine errors. Although physicians, patients, and ethicists endorse candid disclosure of harmful medical errors to patients, many physicians express uncertainty about how to approach these conversations. A growing body of research indicates physicians often fail to meet patient expectations for timely and open disclosure. Patients desire information about the error, an apology, and a plan for preventing recurrence of the error. To meet these expectations, physicians should participate in event investigations and plan thoroughly for each disclosure conversation, preferably with a disclosure coach. Physicians should also anticipate and attend to the ongoing medical and emotional needs of the patient. A cultural change towards greater transparency following medical errors is in motion. Substantial progress is still required, but neurologists can further this movement by promoting policies and environments conducive to open reporting, respectful disclosure to patients, and support for the healthcare workers involved. PMID:24182370

  19. Differential usage of storage carbohydrates in the CAM bromeliad Aechmea 'Maya' during acclimation to drought and recovery from dehydration.

    Science.gov (United States)

    Ceusters, Johan; Borland, Anne M; Londers, Elsje; Verdoodt, Veerle; Godts, Christof; De Proft, Maurice P

    2009-02-01

    CAM requires a substantial investment of resources into storage carbohydrates to account for nocturnal CO(2) uptake, thereby restricting carbohydrate partitioning to other metabolic activities, including dark respiration, growth and acclimation to abiotic stress. Flexible modulation of carbon flow to the different competing sinks under changing environmental conditions is considered a key determinant for the growth, productivity and ecological success of the CAM pathway. The aim of the present study was to examine how shifts in carbohydrate partitioning could assure maintenance of photosynthetic integrity and a positive carbon balance under conditions of increasing water deprivation in CAM species. Measurements of gas exchange, leaf water relations, malate, starch and soluble sugar (glucose, fructose and sucrose) contents were made in leaves of the CAM bromeliad Aechmea 'Maya' over a 6-month period of drought and subsequently over a 2-month period of recovery from drought. Results indicated that short-term influences of water stress were minimized by elevating the level of respiratory recycling, and carbohydrate pools were maintained at the expense of export for growth while providing a comparable nocturnal carbon gain to that in well-watered control plants. Longer term drought resulted in a disproportionate depletion of key carbohydrate reserves. Sucrose, which was of minor importance for providing substrate for the dark reactions under well-watered conditions, became the major source of carbohydrate for nocturnal carboxylation as drought progressed. Flexibility in terms of the major carbohydrate source used to sustain dark CO(2) uptake is therefore considered a crucial factor in meeting the carbon and energy demands under limiting environmental conditions. Recovery from CAM-idling was found to be dependent on the restoration of the starch pool, which was used predominantly for provision of substrate for nocturnal carboxylation, while net carbon export was limited

  20. "Most people are simply not designed to eat pasta": evolutionary explanations for obesity in the low-carbohydrate diet movement.

    Science.gov (United States)

    Knight, Christine

    2011-09-01

    Low-carbohydrate diets, notably the Atkins Diet, were particularly popular in Britain and North America in the late 1990s and early 2000s. On the basis of a discourse analysis of bestselling low-carbohydrate diet books, I examine and critique genetic and evolutionary explanations for obesity and diabetes as they feature in the low-carbohydrate literature. Low-carbohydrate diet books present two distinct neo-Darwinian explanations of health and body-weight. First, evolutionary nutrition is based on the premise that the human body has adapted to function best on the diet eaten in the Paleolithic era. Second, the thrifty gene theory suggests that feast-or-famine conditions during human evolutionary development naturally selected for people who could store excess energy as body fat for later use. However, the historical narratives and scientific arguments presented in the low-carbohydrate literature are beset with generalisations, inconsistencies and errors. These result, I argue, from the use of the primitive as a discursive "blank slate" onto which to project ideals perceived to be lacking in contemporary industrialised life. PMID:22164708