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Sample records for aminoisocaproic acid-alpha

  1. Carbohydrate content of acid alpha-glucosidase (gamma-amylase) from human liver.

    Science.gov (United States)

    Belen'ky, D M; Mikhajlov, V I; Rosenfeld, E L

    1979-05-01

    The presence of carbohydrates in homogeneous preparations of human liver acid alpha-glucosidase has been established and the carbohydrate content of the enzyme determined. The enzyme was purified with the specific purpose of removing all low-molecular-weight carbohydrates. It was specifically adsorbed on Concanavalin A-Sepharose, eluted with methyl-alpha-D-mannopyranoside and gave a positive reaction with the phenol-sulphuric acid reagent. These facts taken together provide evidence that the enzyme studied is a glycoprotein. The analysis of the carbohydrate content of human liver acid alpha-glucosidase showed that there were 8.3 glucosamine, 13.2 mannose and possibly 3--4 glucose residues per molecule of the enzyme with a molecular weight of 98,000. PMID:376187

  2. Ascorbic acid, alpha-tocopherol, and oregano supplements reduce stress-induced deterioration of chicken meat quality.

    Science.gov (United States)

    Young, J F; Stagsted, J; Jensen, S K; Karlsson, A H; Henckel, P

    2003-08-01

    In order to ameliorate a negative effect of stress on meat quality characteristics, chickens were fed a diet supplemented with a combination of ascorbic acid (1,000 ppm) and alpha-tocopherol (200 ppm) or oregano (3%), which has a high content of antioxidants. Chickens were slaughtered by cervical dislocation in the stable (no stress) or after transport and electrical stunning at the slaughter plant (stress). Activities of antioxidative enzymes (catalase, superoxide dismutase, and glutathion peroxidase) in pectoralis major (PM), iliotibialis (IL), and liver were unaffected by supplementation. However, erythrocyte stability, which is a more complex model system for determining oxidative status, increased with ascorbic acid-alpha-tocopherol supplementation and tended to increase after oregano supplementation. In nonstressed birds, this improved antioxidative status was reflected in decreased TBA-reactive substances (TBARS) in PM and liver of ascorbic acid-alpha-tocopherol-supplemented chickens and likewise in liver from oregano-supplemented chickens compared to that of nonstressed control birds. However, postmortem temperature, pH, and water-holding capacity were not affected by supplementation. Drip loss from oregano-supplemented chickens showed increased protein oxidation in specific bands, but this did not relate to water-holding capacity or antioxidative status. When exposed to stress, the concentration of TBARS in the control animals increased in PM and IL. Ascorbic acid-alpha-tocopherol supplementation protected IL, and oregano supplementation protected PM from stress-induced increases in TBARS. This differential effect between muscles may indicate differences in protection mechanisms. In conclusion, ascorbic acid-alpha-tocopherol and oregano supplements to chickens protect against stress-induced increase in TBARS, in different muscles. PMID:12943308

  3. An NMR and ab initio quantum chemical study of acid-base equilibria for conformationally constrained acidic alpha-amino acids in aqueous solution

    DEFF Research Database (Denmark)

    Nielsen, Peter Aadal; Jaroszewski, Jerzy W.; Norrby, Per-Ola;

    2001-01-01

    The protonation states of a series of piperidinedicarboxylic acids (PDAs), which are conformationally constrained acidic alpha -amino acids, have been studied by C-13 NMR titration in water. The resulting data have been correlated with theoretical results obtained by HF/6-31+G* calculations using...

  4. An NMR and ab initio quantum chemical study of acid-base equilibria for conformationally constrained acidic alpha-amino acids in aqueous solution

    DEFF Research Database (Denmark)

    Nielsen, Peter Aadal; Jaroszewski, Jerzy W.; Norrby, Per-Ola; Liljefors, Tommy

    2001-01-01

    The protonation states of a series of piperidinedicarboxylic acids (PDAs), which are conformationally constrained acidic alpha -amino acids, have been studied by C-13 NMR titration in water. The resulting data have been correlated with theoretical results obtained by HF/6-31+G* calculations using...... the polarizable continuum model (PCM) fore the description of water. The PDAs are highly ionizable and contain one or two possible internal hydrogen bonds. In the present study, we show that the PCM model is able to reproduce the relative stabilities of the different protonation states of the PDAs...

  5. Effects of Ascorbic Acid, Alpha-Tocopherol and Allopurinol on Ischemia-Reperfusion Injury in Rabbit Skeletal Muscle: An Experimental Study

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    Bilgehan Erkut

    2007-01-01

    Full Text Available Purpose: Ischemia reperfusion injury to skeletal muscle, following an acute arterial occlusion is important cause of morbidity and mortality. The aim of the present study was to determine and evaluate the effects of ascorbic acide, alpha-tocopherol and allopurinol on ischemia reperfusion injury in rabbit skeletal muscle.Methods: Forty-eight New Zealand white rabbits, all male, weighing between 2.5 to 3.0 (mean 2.8 kg, were used in the study. They were separated into four groups. Group I was the control group without any drugs. The other groups were treatment groups (groups II, III, and IV. Group II rabbits administrated 50 mg/kg ascorbic acide and 100 mg/kg alpha-tocopherol 3 days prior to ischemia, group III rabbits received 50 mg/kg allopurinol 2 days prior to ischemia, and group IV rabbits were administrated both 50 mg/kg ascorbic acide, 100 mg/kg alpha-tocopherol 3 days prior to ischemia and 50 mg/kg allopurinol 2 days prior to ischemia. Two hours ischemia and 2 hours reperfusion were underwent to the treatment groups. At the end of the reperfusion periods, muscle samples were taken from rectus femoris muscle for determination of superoxide dismutase, catalase and glutathione peroxidase activities as antioxidant enzymes, and malondialdehyde as an indicator of lipid peroxidation and xanthine oxidase levels as source hydroxyl radical. Besides, histopathological changes (edema, inflammation, ring formation and splitting formation were evaluated in the muscle specimens. Results: In the treatment groups; superoxide dismutase (U/mgprotein, catalase (U/mgprotein, and glutathione peroxidise (U/mgprotein levels increased, malondialdehyde (nmol/mgprotein and xanthine oksidase (mU/mgprotein levels decreased compared to control I (p < 0.05. Increase of superoxide dismutase, catalase, and glutathione peroxidase levels were the highest and decrease of malondialdehyde and xanthine oxidase levels were the highest in group IV compared to groups II and III

  6. Possible involvement of phospholipase C and protein kinase C in stimulatory actions of L-leucine and its keto acid, alpha-ketoisocaproic acid, on protein synthesis in RLC-16 hepatocytes.

    Science.gov (United States)

    Yagasaki, Kazumi; Morisaki-Tsuji, Naoko; Miura, Atsuhito; Funabiki, Ryuhei

    2002-11-01

    Effects of leucine and related compounds on protein synthesis were studied in RLC-16 hepatocytes. The incorporation of [(3)H] tyrosine into cellular protein was measured as an indexof protein synthesis. In leucine-depleted RLC-16 cells, L-leucineand its keto acid, alpha-ketoisocaproic acid (KIC), stimulated protein synthesis, while D-leucine did not. Mepacrine, an inhibitor of both phospholipase A(2) and C canceled stimulatory actions of L-leucine and KIC on protein synthesis, suggesting a possible involvement of either arachidonic acid metabolism by phospholipase A(2), cyclooxygenase or lipoxygenase, or phosphatidylinositol degradation by phospholipase C in the stimulatory actions of L-leucine and KIC.Neither indomethacin, an inhibitor of cyclooxygenase, nor caffeic acid, an inhibitor of lipoxygenase, diminished their stimulatory actions, suggesting no involvement of arachidonic acid metabolism. Conversely, 1-O-hexadecyl-2-O-methylglycerol, an inhibitor of protein kinase C, significantly canceled the stimulatory actions of L-leucine and KIC on protein synthesis, suggesting an involvement of phosphatidylinositol degradation and activation of protein kinase C. These results strongly suggest that both L-leucine and KIC stimulate protein synthesis in RLC-16 cells via activation of phospholipase C and production of diacylglycerol and inositol triphosphate from phosphatidylinositol, which in turn activate protein kinase C. PMID:19003115

  7. Relato do primeiro paciente brasileiro com a forma infantil da doença de Pompe tratado com alfa-glicosidase recombinante humana Report of the first Brazilian infantile Pompe disease patient to be treated with recombinant human acid alpha-glucosidase

    Directory of Open Access Journals (Sweden)

    Sandra J. Pereira

    2008-06-01

    Full Text Available Objetivo: Relatar o primeiro caso de forma infantil da doença de Pompe tratado no Brasil. Descrição: Trata-se de doença de depósito lisossomal que se caracteriza por defeitos da enzima alfa-glicosidase ácida, com acúmulo intracelular de glicogênio, principalmente nos músculos. São descritas a forma infantil e tardia. Desde 2006, está disponível tratamento com enzima recombinante humana. Descreve-se o primeiro caso de forma infantil da doença tratado no Brasil. Trata-se de menina com 2,5 meses de idade e progressão rápida da doença, com perda dos movimentos dos membros, miocardiopatia hipertrófica e insuficiência respiratória aos 7 meses de idade. Após 10 meses de tratamento, apresentou boa resposta clínica, com remissão da insuficiência respiratória, recuperação parcial dos movimentos dos membros e melhora importante do quadro cardiológico. Comentários: Apesar de pouco freqüente, a forma infantil da doença de Pompe é letal. A disponibilidade de tratamento eficaz aumenta a necessidade de conhecimento e diagnóstico precoce da doença.Objective: To describe the first case of infantile Pompe disease to be treated in Brazil. Description: Pompe disease is a glycogen storage disease related to defects in the acid alpha-glucosidase enzyme, leading to an intracellular accumulation of glycogen, mainly in muscles. Two forms are described: infantile and juvenile. Since 2006, treatment with recombinant human acid alpha-glucosidase has been available. This article describes the first case of infantile Pompe disease treated in Brazil. A girl presented at 2.5 months of age with rapid disease progression, exhibiting severe hypotonia, loss of movements in both upper and lower limbs and hypertrophic cardiomyopathy, progressing to respiratory failure by the age of 7 months. After 10 months of treatment, she exhibited a good clinical response, with remission of the respiratory failure, partial recovery of arm and leg movements and

  8. Determinação sérica de haptoglobina, ceruloplasmina e alfa-glicoproteína ácida em cães com gastrenterite hemorrágica Determination of serum haptoglobin, ceruloplasmin and acid alpha-glycoprotein in dogs with haemorrhagic gastroenteritis

    Directory of Open Access Journals (Sweden)

    Márcia Mery Kogika

    2003-06-01

    Full Text Available As proteínas de fase aguda (PFA são proteínas plasmáticas, cujo estímulo à síntese ocorre de forma rápida e marcante em resposta à injúria tecidual. Estas proteínas permitem o diagnóstico de processos inflamatórios em animais com supressão ou depressão medular. Além disso, são úteis na monitorização da resolução tecidual de traumas ou inflamação e também na avaliação da resposta orgânica ao tratamento. Uma vez que a leucopenia é observada nos estádios iniciais da parvovirose canina, a dosagem das PFA pode permitir a avaliação do processo inflamatório sob estas condições. Considerando-se estas hipóteses, foram determinados os níveis séricos das PFA (haptoglobina, ceruloplasmina e alfa-glicoproteína ácida em 11 cães saudáveis e 11 cães leucopênicos com gastrenterite hemorrágica, com suspeita clínica de parvovirose canina. A avaliação estatística mostrou diferença significativa, com intervalo de confiabilidade de 99% (PAcute phase proteins (APP are serum proteins whose stimulus for the synthesis happens in a quick and intense manner in response to tissue injury. Those proteins allow the diagnosis of inflammatory process in animals with bone marrow depression and, also, they are useful in the follow up of tissue resolution of traumas or inflammation, as well as in the evaluation of the organic response of the treatment. As leukopenia is observed in the initial stage of the canine parvovirus infection, the dosage of APP can allow the evaluation of the inflammatory process under these conditions. According to these hypothesis, serum APP levels (haptoglobin, ceruloplasm and a-acid-glycoprotein in 11 healthy dogs and 11 leukopenic dogs with haemorrhagic gastroenteritis, clinically suspected of canine parvovirus infection, were measured. There was a significant difference, with confidence interval of 99% (P <0.01 for the haptoglobin (p <0.0064 and the acid alpha-glycoprotein (p <0.0042 and 95% (P <0.05 of

  9. A specific acid [alpha]-glucosidase in lamellar bodies of the human lung

    NARCIS (Netherlands)

    Vries, A.C.J. de; Schram, A.W.; Tager, J.M.; Batenburg, J.J.

    2006-01-01

    In the present investigation, we have demonstrated that three lysosomal-type hydrolases, alpha-glucosidase, alpha-mannosidase and a phosphatase, are present in lamellar bodies isolated from adult human lung. The hydrolase activities that were studied, all showed an acidic pH optimum, which is charac

  10. Characterization of a chromosomally encoded 2,4-dichlorophenoxyacetic acid/alpha-ketoglutarate dioxygenase from Burkholderia sp. strain RASC.

    OpenAIRE

    Suwa, Y.; Wright, A D; Fukimori, F; Nummy, K A; Hausinger, R P; Holben, W E; Forney, L J

    1996-01-01

    The findings of previous studies indicate that the genes required for metabolism of the pesticide 2,4-dichlorophenoxyacetic acid (2,4-D) are typically encoded on broad-host-range plasmids. However, characterization of plasmid-cured strains of Burkholderia sp. strain RASC, as well as mutants obtained by transposon mutagenesis, suggested that the 2,4-D catabolic genes were located on the chromosome of this strain. Mutants of Burkholderia strain RASC unable to degrade 2,4-D (2,4-D- strains) were...

  11. Effet de la dose d’acide alpha-linolénique alimentaire sur le métabolisme lipidique

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    Morise Anne

    2005-09-01

    Full Text Available The purpose of our study was to investigate the effects of high doses of ALA provided by linseed oil (containing 50% ALA on its own bioavailability and that of its derivatives as well as on lipid metabolism. We investigated in male hamsters the dose/response effects of ALA over a broad range of supply as linseed oil (1, 10, 20 and 41% of total fatty acids, FA, or 0.4, 3.6, 6.7 and 14.6% of total energy intake. ALA was substituted for oleic acid in order to keep constant linoleic acid (LA and saturated fatty acids which could interfere with the metabolism of n-3 PUFA and lipids, respectively. The capacity of ALA absorption, transport, storage and conversion into EPA had no limitation over the chosen range of dietary intake. However, dietary ALA failed to increase DHA content in plasma phospholipids. In parallel to the increase in EPA, arachidonic acid content decreased, resulting in an improved balance of 20 carbons FA. Moreover, in our atherogenic conditions, triglyceridemia decreased by 45% in response to 10% dietary ALA and was not affected by higher intakes. It was associated with lower hepatic activities of acetyl-CoA-carboxylase (up to – 29% and malic enzyme (up to – 42%, which were negatively correlated to ALA intake (r2 = 0.33 and r2 = 0.38, respectively. Substitution of 10% ALA for oleic acid increased cholesterolemia by 15% but, as in TG, higher ALA intakes did not amplify the response. The highest ALA intake (40% modified dramatically hepatobiliary metabolism of sterols. Thus, replacing 10% oleic acid by ALA is sufficient to improve its bioavailability and that of EPA, and to exert a beneficial hypotriglyceridemic effect, that may be counteracted by the slight increase in cholesterolemia. Higher intakes did not modify these parameters, but a very high dose resulted in adverse effects on sterol metabolism and does not seem appropriate for humans.

  12. Redox Cycles of Caffeic Acid, alpha-Tocopherol, and Ascorbate: Implications for Protection of Low-Density Lipoproteins Against Oxidation

    OpenAIRE

    Laranjinha, João; Cadenas, Enrique

    1999-01-01

    This study addresses the dynamic interactions among alpha-tocopherol, caffeic acid, and ascorbate in terms of a sequence of redox cycles aimed at accomplishing optimal synergistic antioxidant protection. Several experimental models were designed to examine these interactions: UV irradiation of alpha-tocopherol-containing sodium dodecyl sulfate micelles, one-electron oxidations catalyzed by the hypervalent state of myoglobin, ferrylmyoglobin, and autoxidation at appropriate pHs. These models w...

  13. Enhancing cosmetic outcomes by combining superficial glycolic acid (alpha-hydroxy acid) peels with nonablative lasers, intense pulsed light, and trichloroacetic acid peels.

    Science.gov (United States)

    Effron, Cheryl; Briden, M Elizabeth; Green, Barbara A

    2007-01-01

    Nonablative lasers, intense pulsed light (IPL), and trichloroacetic acid (TCA) peels are cosmetic rejuvenation techniques used to remodel skin and provide improved skin texture, firmness, and even pigmentation. Glycolic acid is an alpha-hydroxy acid that can be used as a topical skin peel to provide important complementary benefits to nonablative lasers, IPL, and TCA peels. Superficial glycolic acid peels provide both epidermal and dermal antiaging benefits, can be used to smooth the stratum corneum to reduce light scattering, and can enable the use of lower concentrations of TCA during a peel procedure. When used with these procedures, glycolic acid peels can enhance skin benefits and perceived patient outcomes. Methods of combining nonablative lasers, IPL, and TCA peels with glycolic acid peels were discussed at a dermatologist roundtable event and are summarized in this article. PMID:17455887

  14. Studies in lipid histochemistry. XIII. The OPA (osmiumtetroxide-periodic acid-alpha-naphthylamine) method for the detection of apolar lipids.

    Science.gov (United States)

    Elleder, M

    1975-09-29

    A new procedure for the detection of apolar lipids is described. It is a modification of the OTAN method (Adams, 1959) using periodic acid which oxidatively removes lower osmium derivatives from polar sites only, leaving those in apolar lipids intact and demonstrable with alpha-naphthylamine. Control steps for the exclusion of the possible interference of some less polar complex lipids and of lipopigments are described. The described technic is superior to the conventionally used sudan dyes due partly to the fact that only aqueous solutions are employed thus excluding any extraction of lipids, partly to the more distinct coloration. PMID:171245

  15. Capture of a catabolic plasmid that encodes only 2,4-dichlorophenoxyacetic acid:alpha-ketoglutaric acid dioxygenase (TfdA) by genetic complementation.

    OpenAIRE

    Top, E. M.; Maltseva, O V; Forney, L J

    1996-01-01

    The modular pathway for the metabolism of 2,4-dichlorophenoxyacetic acid (2,4-D) encoded on plasmid pJP4 of Alcaligenes eutrophus JMP134 appears to be an example in which two genes, tfdA and tfdB, have been recruited during the evolution of a catabolic pathway. The products of these genes act to convert 2,4-D to a chloro-substituted catechol that can be further metabolized by enzymes of a modified ortho-cleavage pathway encoded by tfdCDEF. Given that modified ortho-cleavage pathways are compa...

  16. Evidence for Acquisition in Nature of a Chromosomal 2,4-Dichlorophenoxyacetic Acid/(alpha)-Ketoglutarate Dioxygenase Gene by Different Burkholderia spp

    OpenAIRE

    Matheson, V. G.; Forney, L J; Suwa, Y.; Nakatsu, C. H.; A. J. Sexstone; Holben, W E

    1996-01-01

    We characterized the gene required to initiate the degradation of 2,4-dichlorophenoxyacetic acid (2,4-D) by the soil bacterium Burkholderia sp. strain TFD6, which hybridized to the tfdA gene of the canonical 2,4-D catabolic plasmid pJP4 under low-stringency conditions. Cleavage of the ether bond of 2,4-D by cell extracts of TFD6 proceeded by an (alpha)-ketoglutarate-dependent reaction, characteristic of TfdA (F. Fukumori and R. P. Hausinger, J. Bacteriol. 175:2083-2086, 1993). The TFD6 tfdA g...

  17. The pharmacological chaperone AT2220 increases the specific activity and lysosomal delivery of mutant acid alpha-glucosidase, and promotes glycogen reduction in a transgenic mouse model of Pompe disease.

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    Richie Khanna

    Full Text Available Pompe disease is an inherited lysosomal storage disorder that results from a deficiency in acid α-glucosidase (GAA activity due to mutations in the GAA gene. Pompe disease is characterized by accumulation of lysosomal glycogen primarily in heart and skeletal muscles, which leads to progressive muscle weakness. We have shown previously that the small molecule pharmacological chaperone AT2220 (1-deoxynojirimycin hydrochloride, duvoglustat hydrochloride binds and stabilizes wild-type as well as multiple mutant forms of GAA, and can lead to higher cellular levels of GAA. In this study, we examined the effect of AT2220 on mutant GAA, in vitro and in vivo, with a primary focus on the endoplasmic reticulum (ER-retained P545L mutant form of human GAA (P545L GAA. AT2220 increased the specific activity of P545L GAA toward both natural (glycogen and artificial substrates in vitro. Incubation with AT2220 also increased the ER export, lysosomal delivery, proteolytic processing, and stability of P545L GAA. In a new transgenic mouse model of Pompe disease that expresses human P545L on a Gaa knockout background (Tg/KO and is characterized by reduced GAA activity and elevated glycogen levels in disease-relevant tissues, daily oral administration of AT2220 for 4 weeks resulted in significant and dose-dependent increases in mature lysosomal GAA isoforms and GAA activity in heart and skeletal muscles. Importantly, oral administration of AT2220 also resulted in significant glycogen reduction in disease-relevant tissues. Compared to daily administration, less-frequent AT2220 administration, including repeated cycles of 4 or 5 days with AT2220 followed by 3 or 2 days without drug, respectively, resulted in even greater glycogen reductions. Collectively, these data indicate that AT2220 increases the specific activity, trafficking, and lysosomal stability of P545L GAA, leads to increased levels of mature GAA in lysosomes, and promotes glycogen reduction in situ. As such, AT2220 may warrant further evaluation as a treatment for Pompe disease.

  18. Secondary liquefaction in ethanol production

    DEFF Research Database (Denmark)

    2007-01-01

    The invention relates to a method of producing ethanol by fermentation, said method comprising a secondary liquefaction step in the presence of a themostable acid alpha-amylase or, a themostable maltogenic acid alpha-amylase.......The invention relates to a method of producing ethanol by fermentation, said method comprising a secondary liquefaction step in the presence of a themostable acid alpha-amylase or, a themostable maltogenic acid alpha-amylase....

  19. Influence du taux d’acide alpha-linolénique de l’aliment sur la teneur en oméga-3 et les caractéristiques hédoniques de la viande de lapin. Revue bibliographique

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    Teillet Benoît

    2013-01-01

    Full Text Available Several publications are dealing with the influence of the omega 3 level in the feed, more particularly the alpha-linolenic acid level, on the rabbit meat level in this fatty acid, but only few of them deal with the use of the flaxseed. This review is a synthesis of the effects of the level of alpha-linolenic acid brought by the flaxseed on the omega 3 level of the rabbit meat. It shows a direct connection between the alpha-linolenic acid level in the feed and the omega 3 content of the rabbit meat. Besides, this enrichment decreases the saturated fatty acids level, the omega 6 level and consequently the omega 6/omega 3 ratio below 4. In consequence, the contribution of around 0.65% of alpha-linolenic acid in the feed by incorporation of flaxseed in the feed allows to bring in average 967 mg of omega 3 for 100 g of back or 459 mg for 100 kcal and 1004 mg of omega 3 for 100 g of shoulder or 483 mg for 100 kcal and consequently to claim the allegation “rich in omega 3”. This enrichment doesn’t modify the hedonic characteristics of the rabbit meat.

  20. Acute Myelogenous Leukemia without Maturation with a Retinoic Alpha-Receptor Deletion: A Case Report

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    Christopher Trosclair

    2014-06-01

    Full Text Available Acute promyelocytic leukemia (APL is characterized by a t(15;17 which fuses the 17q retinoic acid alpha-receptor sequence to the 15q PML gene sequence. The resulting fusion product plays a role in the development and maintenance of APL, and is very rarely found in other acute myeloid leukemia (AML subtypes. Rare complex APL genomic rearrangements have retinoic acid alpha-receptor sequence deletions. Here we report a retinoic acid alpha-receptor sequence deletion in a case of AML without differentiation. To our knowledge, this is the first example of a retinoic acid alpha-receptor sequence deletion in this AML subtype.

  1. Linkages in thermal copolymers of lysine

    Science.gov (United States)

    Fox, S. W.; Suzuki, F.

    1976-01-01

    The thermal copolymerization of lysine with other alpha-amino acids has been studied further. The identity of the second amino acid influences various properties of the polymer obtained, including the proportion of alpha and epsilon linkages of lysine. A review of linkages in proteinoids indicates alpha and beta linkages for aspartic acid, alpha and gamma linkages for glutamic acid, alpha and epsilon linkages for lysine, and alpha linkages for other amino acids. Thermal proteinoids are thus more complex in types of linkage than are proteins

  2. Distribution of sialic acid receptors and influenza A viruses of avian and swine origin and in experimentally infected pigs

    DEFF Research Database (Denmark)

    Trebbien, Ramona; Larsen, Lars Erik; Viuff, Birgitte M.

    2011-01-01

    Background: Pigs are considered susceptible to influenza A virus infections from different host origins because earlier studies have shown that they have receptors for both avian (sialic acid-alpha-2,3-terminal saccharides (SAalpha- 2,3)) and swine/human (SA-alpha-2,6) influenza viruses in the...

  3. 4-hydroxy-1,2,5-oxadiazol-3-yl moiety as bioisoster of the carboxy function. Synthesis, ionization constants, and molecular pharmacological characterization at ionotropic glutamate receptors of compounds related to glutamate and its homologues

    DEFF Research Database (Denmark)

    Lolli, Marco L; Giordano, Cecilia; Pickering, Darryl S;

    2010-01-01

    In order to investigate the 4-hydroxy-1,2,5-oxadiazol-3-yl moiety as a carboxylic acid bioisoster at ionotropic glutamate receptors (iGluRs), a series of acidic alpha-aminocarboxylic acids in which the distal carboxy group was replaced by the 4-hydroxy-1,2,5-oxadiazol-3-yl group was synthesized. ...

  4. Characterizing the Glycocalyx of Poultry Spermatozoa: II. Low Temperature Storage of Turkey Semen and Sperm Mobility Phenotype Impact the Carbohydrate Component of Membrane Glycoconjugates

    Science.gov (United States)

    The turkey sperm glycocalyx is known to contain residues of sialic acid, alpha-mannose/alpha-glucose, alpha- and beta-galactose, alpha-fucose, alpha- and beta-N-acetyl-galactosamine, monomers and dimers of N-acetyl-glucosamine and N-acetyl-lactosamine. Potential changes in these carbohydrates during...

  5. beta-carotene does not change markers of enzymatic and nonenzymatic antioxidant activity in human blood

    DEFF Research Database (Denmark)

    Castenmiller, J.J.M.; Lauridsen, Søren T.; Dragsted, Lars O.;

    1999-01-01

    = 12) or with a spinach product (n = 12 per group), i.e., whole-leaf, minced, liquefied or liquefied spinach plus added dietary fiber. After 3 wk of dietary intervention, changes in serum or plasma concentrations of ascorbic acid, alpha-tocopherol, FRAP (ferric reducing ability of plasma) and uric acid...

  6. A RARE METABOLIC DISORDER: POMPE’S DISEASE

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    Nazeer Ahmed

    2015-10-01

    Full Text Available Pompe disease is an autosomal recessive metabolic disorder caused by the buildup of a sugar called glycogen in the body’s cells.1,2 It is caused by an accumulation of glycogen in the lysosome due to deficiency or absence of the enzyme acid alpha-glucosidase (GAA. The enzyme GAA is used to breakdown glycogen into a simpler sugar, glucose.3 It is characterised by progressive weakness in the muscles used for mobility and breathing. In infants with Pompe disease, the heart muscles are often severely affected as well.4,7 The cells of the heart and skeletal muscles are affected the most.It is caused by a mutation in a gene (Acid alpha-glucosidase: also known as acid maltase on long arm of chromosome 17 at 17q25.2-q25.3.. Without treatment the disease is particularly lethal in infants and young children.8

  7. Preparation and characterization of chelating fibers based on natural wool for removal of Hg(II), Cu(II) and Co(II) metal ions from aqueous solutions

    Energy Technology Data Exchange (ETDEWEB)

    Monier, M., E-mail: monierchem@yahoo.com [Chemistry Department, Faculty of Science, Mansoura University, 35516 (Egypt); Nawar, N., E-mail: nnawar@mans.edu.eg [Chemistry Department, Faculty of Science, Mansoura University, 35516 (Egypt); Abdel-Latif, D.A. [Chemistry Department, Faculty of Science, Mansoura University, 35516 (Egypt)

    2010-12-15

    The graft copolymerization of acrylonitrile (AN) onto natural wool fibers initiated by KMnO{sub 4} and oxalic acid combined redox initiator system in limited aqueous medium was carried out in heterogeneous media. Moreover, modification of the grafted wool fibers was done by changing the nitrile group (-CN) into cyano-acetic acid {alpha}-amino-acrylic-hydrazide through the reaction with hydrazine hydrate followed by ethylcyanoacetate which eventually produce wool-grafted-poly(cyano-acetic acid {alpha}-amino-acrylic-hydrazide) (wool-g-PCAH) chelating fibers. The application of the modified fibers for metal ion uptake was studied using Hg{sup 2+}, Cu{sup 2+} and Co{sup 2+}. The modified chelating fibers were characterized using FTIR spectroscopy, SEM and X-ray diffraction.

  8. Structure-function relationships of non-cyclic dioxygenase products from polyunsaturated fatty acids: Poxytrins as a class of bioactive derivatives.

    OpenAIRE

    Lagarde, Michel; Véricel, Evelyne; Liu, Miao; Chen, Ping; Guichardant, Michel

    2014-01-01

    : More and more attention is paid to omega-3 fatty acids because of their potential activities in preventing cardiovascular events. In this brief review, we focus on the lipoxygenase end-metabolites of two relevant nutrients belonging to the omega-3 family fatty acids: alpha-linolenic and docosahexaenoic acids, the latter being a prominent component of brain lipids. Dihydroxylated derivatives are described as well as their inhibitory effects on platelet aggregation and cyclooxygenase activiti...

  9. Lack of robust satellite cell activation and muscle regeneration during the progression of Pompe disease

    OpenAIRE

    Schaaf, Gerben J.; van Gestel, Tom JM; Brusse, Esther; Verdijk, Robert M.; de Coo, Irenaeus FM; Doorn Van, Pieter A; Ploeg, Ans T van der; Pijnappel, WWM Pim

    2015-01-01

    Introduction Muscle stem cells termed satellite cells are essential for muscle regeneration. A central question in many neuromuscular disorders is why satellite cells are unable to prevent progressive muscle wasting. We have analyzed muscle fiber pathology and the satellite cell response in Pompe disease, a metabolic myopathy caused by acid alpha-glucosidase deficiency and lysosomal glycogen accumulation. Pathology included muscle fiber vacuolization, loss of cross striation, and immune cell ...

  10. Analysis of the biotechnological production of 2-ketomethylvalerate with Corynebacterium glutamicum

    OpenAIRE

    Hagmann, Christian Thomas

    2012-01-01

    The current work deals with the biotechnological synthesis of alpha-ketomethylvalerate (KMV) with Corynebacterium glutamicum. KMV together with the two other branched-chain alpha-keto acids alpha-ketoisovalerate (KIV) and alpha-ketoisocaproate (KIC) is used as a pharmaceutical agent and also as ingredient of functional food. So far KMV has only been produced by chemical synthesis and the aim of this work was to initiate the development of a fermentative KMV production. When C. glutamicum was ...

  11. An electron microscopic and biochemical study of the effects of cyclic 3', 5'- AMP, ergotamine or propanolol on the lysosomes of newborn rat hepatocytes

    OpenAIRE

    Kotoulas, Angeliki O.; Kotoulas, Othon B.; Kalamidas, Stefanos

    1991-01-01

    The effects of cyclic 3', 5'-AMP, ergotamine or propranolol on newborn rat liver were studied by using biochemical assays, electron microscopy and quantitative morphometry. Cyclic AMP enhanced the normal postnatal rise in the glycogen-hydrolysing activity of acid alpha 1,4 glucosidase but had no effect on the maltose-hydrolysing activity of the enzyme. The results suggest that these activities may be due to different enzymes. Propranolol prevented the postnatal...

  12. Neuroprotective effects of α-lipoic acid on the development of oxidative stress and astrogliosis in the brain of STZ-diabetic rats

    OpenAIRE

    Kyrychenko, S.; I. Prishchepa; Lagoda, V.; M. Velika; V. Nedzvetsky

    2014-01-01

    The aim of this study was to examine whether the antioxidant alpha-lipoic acid protects neurons from diabetic-reperfusion injury. The streptozotocin (STZ) rat model was used to study the glial reactivity and prevention of gliosis by alpha-lipoic acid (alpha-LA) administration. The expression of glial fibrillary acidic protein (GFAP) was determined, as well as lipid peroxidation (LPO) and glu-tathione (GSH) levels in some brain tissues. We observed significant increasing of lipid peroxidation ...

  13. Bioactive phytochemicals in flaxseed

    OpenAIRE

    Johnsson, Pernilla

    2009-01-01

    Flaxseed (Linum usitatissimum L.) is rich in health-promoting bioactive compounds. Among plant foods, flaxseed has the highest content of lignans, mainly in the form of secoisolariciresinol diglucoside (SDG). Flaxseed oil also has a very high concentration of the essential omega-3 fatty acid alpha-linolenic acid (ALA). This thesis presents studies on both SDG and ALA. An HPLC method for quantification of SDG in hydrolysed flaxseed extracts was developed and used to compare the SDG content in ...

  14. Pompe disease gene therapy

    OpenAIRE

    Barry J Byrne; Falk, Darin J.; Pacak, Christina A; Nayak, Sushrusha; Herzog, Roland W; Elder, Melissa E.; Collins, Shelley W.; Conlon, Thomas J.; Clement, Nathalie; Cleaver, Brian D.; Cloutier, Denise A.; Porvasnik, Stacy L; Islam, Saleem; ElMallah, Mai K; Martin, Anatole

    2011-01-01

    Pompe disease is an autosomal recessive metabolic myopathy caused by the deficiency of the lysosomal enzyme acid alpha-glucosidase and results in cellular lysosomal and cytoplasmic glycogen accumulation. A wide spectrum of disease exists from hypotonia and severe cardiac hypertrophy in the first few months of life due to severe mutations to a milder form with the onset of symptoms in adulthood. In either condition, the involvement of several systems leads to progressive weakness and disabilit...

  15. Chemical constituents of Hyptidendron canum (Pohl ex Benth.) R. Harley (Lamiaceae); Constituintes quimicos de Hyptidendron canum (Pohl ex Benth.) R. Harley (Lamiaceae)

    Energy Technology Data Exchange (ETDEWEB)

    Lemes, Geralda de Fatima; Ferri, Pedro Henrique, E-mail: geralda@quimica.ufg.b [Universidade Federal de Goias (UFG), Goiania, GO (Brazil). Inst. de Quimica; Lopes, Marcia Nasser [Universidade Estadual Paulista (IQ/UNESP), Araraquara, SP (Brazil). Inst. de Quimica

    2011-07-01

    Chemical investigation of Hyptidendron canum stems resulted in the isolation of betulinic, ursolic and euscaphic acids. From the leaves were isolated 3{beta}-O-{beta}-galactopiranosilsitosterol, ursolic aldehyde, and mixtures of maslinic acid and 2{alpha}-hydroxy ursolic acid, {alpha} and {beta}-amyrin, uvaol and erythrodiol, sitosterol and stigmasterol, spathulenol and globulol. Hexane and chloroform leave fractions as well as ursolic and betulinic acids showed antifungal activities against the yeast form of Paracoccidioides brasiliensis. (author)

  16. A cross-sectional single-centre study on the spectrum of Pompe disease, German patients: molecular analysis of the GAA gene, manifestation and genotype-phenotype correlations

    Directory of Open Access Journals (Sweden)

    Herzog Andreas

    2012-06-01

    Full Text Available Abstract Background Pompe disease (Glycogen storage disease type II, GSD II, acid alpha-glucosidase deficiency, acid maltase deficiency, OMIM # 232300 is an autosomal-recessive lysosomal storage disorder due to a deficiency of acid alpha-glucosidase (GAA, acid maltase, EC 3.2.1.20, Swiss-Prot P10253. Clinical manifestations are dominated by progressive weakness of skeletal muscle throughout the clinical spectrum. In addition, the classic infantile form is characterised by hypertrophic cardiomyopathy. Methods In a cross-sectional single-centre study we clinically assessed 3 patients with classic infantile Pompe disease and 39 patients with non-classic presentations, measured their acid alpha-glucosidase activities and analysed their GAA genes. Results Classic infantile patients had nearly absent residual enzyme activities and a typical clinical course with hypertrophic cardiomyopathy until the beginning of therapy. The disease manifestations in non-classic patients were heterogeneous. There was a broad variability in the decline of locomotive and respiratory function. The age of onset ranged from birth to late adulthood and correlated with enzyme activities. Molecular analysis revealed as many as 33 different mutations, 14 of which are novel. All classic infantile patients had two severe mutations. The most common mutation in the non-classic group was c.-32-13 T > G. It was associated with a milder course in this subgroup. Conclusions Disease manifestation strongly correlates with the nature of the GAA mutations, while the variable progression in non-classic Pompe disease is likely to be explained by yet unknown modifying factors. This study provides the first comprehensive dataset on the clinical course and the mutational spectrum of Pompe disease in Germany.

  17. Adult onset glycogen storage disease type II (adult onset Pompe disease): report and magnetic resonance images of two cases

    Energy Technology Data Exchange (ETDEWEB)

    Del Gaizo, Andrew [Emory University School of Medicine, Radiology Resident, Atlanta, GA (United States); Banerjee, Sima [Emory University School of Medicine, Musculoskeletal Radiology Department, Atlanta, GA (United States); Terk, Michael [Emory University School of Medicine, Radiology, Division of Musculoskeletal Imaging, Atlanta, GA (United States)

    2009-12-15

    Glycogen storage disease type II (GSDII), also referred to as Pompe disease or acid maltase deficiency, is a rare inherited condition caused by a deficiency in acid alpha-glucosidase (GAA) enzyme activity. The condition is often classified by age of presentation, with infantile and late onset variants (Laforet et al. J Neurology 55:1122-8, 2000). Late onset tends to present with progressive proximal muscle weakness and respiratory insufficiency (Winkel et al. J Neurology 252:875-84, 2005). We report two cases of biopsy confirmed adult onset GSDII, along with key Magnetic Resonance (MR) images. (orig.)

  18. Infantile Onset Glycogen Storage Disease Type 2: Case Report

    Directory of Open Access Journals (Sweden)

    Serkan Bilge Koca

    2014-08-01

    Full Text Available Glycogen storage disease type 2 (Pompe’s disease is an autosomal recessive, fatal glycogen storage disease presenting with hypotonia and muscle weakness. It is known that deficiency of lysosomal acid alpha-glucosidase (acid maltase leads to progressive generalised myopathy, cardiomyopathy and death in early infancy because of respiratory muscle weakness. Excessive undegradable intracellular glycogen deposition plays a role in the pathogenesis of the disease. Here we report a 3.5 month-old girl presenting with respiratory failure due to pneumonia and hypotonia, who was later diagnosed as Pompe disease.

  19. Synthesis of glycosyl-amino acids of biological interest; Sintese de glicoaminoacidos de interesse biologico

    Energy Technology Data Exchange (ETDEWEB)

    Campo, Vanessa Leiria; Carvalho, Ivone [Universidade de Sao Paulo (USP), Ribeirao Preto, SP (Brazil). Faculdade de Ciencias Farmaceuticas]. E-mail: carronal@usp.br

    2008-07-01

    This work describes the synthesis of the glycosylated amino acids {alpha}GlcNAc-Thr, {beta}GlcNAc-Thr and {alpha}LacNAc-Thr by the glycosylation reaction of the amino acid threonine with the corresponding glycosyl donors {alpha}GlcNAcCl and {alpha}LacN3Cl. The glycosylated amino acids containing the sugar units {alpha}-D-GlcNAc and {alpha}-D-LacNAc O-linked to threonine amino acids are related to O-glycans found in mucins of the parasite Trypanosoma cruzi, while the corresponding {beta}-D-GlcNAc isomer is involved in cellular signaling events. (author)

  20. Successful combined liver/kidney transplantation from a donor with Pompe disease.

    Science.gov (United States)

    Halldorson, J; Kazi, Z; Mekeel, K; Kuo, A; Hassanein, T; Loomba, R; Austin, S; Valasek, M A; Kishnani, P; Hemming, A W

    2015-08-01

    Pompe disease results from inherited deficiency of the enzyme acid alpha-glucosidase resulting in lysosomal accumulation of glycogen primarily in skeletal muscle. Reported is the first case in which a donor with late onset Pompe disease (LOPD) was successfully used for deceased donor liver and kidney transplantation. This case demonstrates co-operative transplant surgery and genetic medicine evaluation and risk estimation for donors with inherited metabolic disorders some of which may be suitable for donation of selected organs for transplantation. PMID:26031770

  1. Insulin-tumour interrelationships in EL4-lymphoma or thymoma-bearing mice. II. Effects of dietary omega-3 and omega-6 polyunsaturated fatty acids.

    OpenAIRE

    Yam, D.; Fink, A.; NIR, I.; Budowski, P.

    1990-01-01

    Male C57BL/65 mice received a basal diet supplemented with 4% soya-bean oil, linseed oil or fish oil, in which the major polyunsaturated fatty acids were linoleic acid, alpha-linolenic acid and long chain omega-3 fatty acids, respectively. Groups of animals were injected into the right flank with EL4-lymphoma cells, others with thymoma cells. Tumour implantation caused a gradual decrease in food consumption with both types of tumour, while body weight increased, especially in the EL4-bearing ...

  2. Peptoid-Peptide hybrid backbone architectures

    DEFF Research Database (Denmark)

    Olsen, Christian Adam

    2010-01-01

    Peptidomimetic oligomers and foldamers have received considerable attention for over a decade, with beta-peptides and the so-called peptoids (N-alkylglycine oligomers) representing prominent examples of such architectures. Lately, hybrid or mixed backbones consisting of both alpha- and beta......-amino acids (alpha/beta-peptides) have been investigated in some detail as well. The present Minireview is a survey of the literature concerning hybrid structures of alpha-amino acids and peptoids, including beta-peptoids (N-alkyl-beta-alanine oligomers), and is intended to give an overview of this area...

  3. Mezinárodní obchod s chmelem a faktory, jenž ho ovlivňují

    OpenAIRE

    Francl, Petr

    2011-01-01

    According to available information, total acreage of hop-gardens in the world in 2010 was an area of 52,156 hectares, which represents a decrease of total 4591 hectares from the previous year. That represents almost 8 % decrease. In the world were harvested during the last year nearly 100,000 tonnes of raw hops. From this total amount were extracted approximately 9,475 tons of important alpha acids. Alpha acids are resinous substances, the most important part of the hops for beer brewing....

  4. Pompe disease: A case report

    Directory of Open Access Journals (Sweden)

    Abdullah Çim

    2015-12-01

    Full Text Available Pompe disease is inherited in an autosomal recessive manner, and is usually observed in the children of asymptomatic carriers. Pompe disease, known as Glycogen Storage Disorder type II, is caused by pathogenic mutations in the gene encoding lysosomal acid alpha-glucosidase (GAA. There are three types of Pompe disease: classical infantile form, non-classical infantile form and late-onset Pompe disease. Age of onset and severity of the disease determine the type of Pompe disease. We aimed to identify a mutation in GAA gene in parents who were first cousins and their baby girl was passed away due to the Pompe disease. The baby girl had reduced acid alpha-glucosidase activity, but genetic analysis had not been performed. Mutation analysis of parents was performed using high-throughput DNA sequencing method. Heterozygous mutation of c.896 T>C in exon 5 was found in parents, and prenatal diagnosis was performed for their next pregnancy. In conclusion, c.896 T>C substitution in GAA gene may lead to the severe type of Pompe disease. Using a relatively fast and reliable molecular genetic analysis method to confirm the early diagnosis of the Pompe disease is important for the management of the disease.

  5. A complex craniovertebral junction malformation in a patient with late onset glycogenosis 2

    Directory of Open Access Journals (Sweden)

    Mariasofia Cotelli

    2014-01-01

    Full Text Available Glycogenosis II (GSDII is an autosomal recessive lysosomal storage disorder resulting from deficiency of acid alpha-glucosidase and subsequent lysosomal accumulation of glycogen in skeletal, cardiac and smooth muscles. The late-onset form is characterized by wide variability of the phenotypical spectrum. Clinical findings may include muscle weakness, respiratory insufficiency, vascular abnormalities, low bone mineral density and higher risk of developing osteoporosis. Craniovertebral junction (CVJ malformations have never been described so far. We here report on a GSDII 43-year-old woman who harbored the mutations IVS1-13T>G and c.2237G>A in the acid alpha-glucosidase gene. She recurrently suffered from headache, neck pain and dizziness. Brain MRI and CT scan showed the presence of a very rare complex CVJ malformation composed of basilar invagination, basiocciput hypoplasia, partial C1 assimilation, C1 posterior arch aplasia and C1 lateral mass hypoplasia and offset. Although we cannot rule out their coincidental occurrence, the rarity of multiple CVJ malformations in the general population as well as the well-known GSDII multisystem involvement should suggest to study the CVJ in the diagnostic process of GSDII patients in order to assess the CVJ malformation frequency in GSDII population and verify a possible relationship between these two conditions.

  6. 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...

  7. Guidelines for the diagnosis, treatment and clinical monitoring of patients with juvenile and adult Pompe disease

    Directory of Open Access Journals (Sweden)

    Juan Clinton Llerena Junior

    2015-01-01

    Full Text Available ABSTRACT Pompe disease (PD is a potentially lethal illness involving irreversible muscle damage resulting from glycogen storage in muscle fiber and activation of autophagic pathways. A promising therapeutic perspective for PD is enzyme replacement therapy (ERT with the human recombinant enzyme acid alpha-glucosidase (Myozyme®. The need to organize a diagnostic flowchart, systematize clinical follow-up, and establish new therapeutic recommendations has become vital, as ERT ensures greater patient longevity. A task force of experienced clinicians outlined a protocol for diagnosis, monitoring, treatment, genetic counseling, and rehabilitation for PD patients. The study was conducted under the coordination of REBREPOM, the Brazilian Network for Studies of PD. The meeting of these experts took place in October 2013, at L’Hotel Port Bay in São Paulo, Brazil. In August 2014, the text was reassessed and updated. Given the rarity of PD and limited high-impact publications, experts submitted their views.

  8. Fungal Peptaibiotics: Assessing Potential Meteoritic Amino Acid Contamination

    Science.gov (United States)

    Elsila, J. E.; Callahan, M. P.; Glavin, D. P.; Dworkin, J. P.; Bruckner, H.

    2010-01-01

    The presence of non-protein alpha-dialkyl-amino acids such as alpha-aminoisobutyric acid (alpha-A1B) and isovaline (Iva), which are relatively rare in the terrestrial biosphere, has long been used as an indication of the indigeneity of meteoritic amino acids, however, the discovery of alpha-AIB in peptides producers by a widespread group of filamentous fungi indicates the possibility of a terrestrial biotic source for the alpha-AIB observed in some meteorites. The alpha-AIB-containing peptides produced by these fungi are dubbed peptaibiotics. We measured the molecular distribution and stable carbon and nitrogen isotopic ratios for amino acids found in the total hydrolysates of four biologically synthesized peptaibiotics. We compared these aneasurenetts with those from the CM2 carbonaceous chondrite Murchison and from three Antarctic CR2 carbonaceous chondrites in order to understand the peptaibiotics as a potential source of meteoritic contamination.

  9. Analysis of phenolic acids in barley by high-performance liquid chromatography.

    Science.gov (United States)

    Yu, J; Vasanthan, T; Temelli, F

    2001-09-01

    Phenolic acids from 30 barley varieties (combination of hulled/hulless/two-row/six-row/regular/waxy) were investigated by HPLC following four different sample treatments: (a) simple hot water extraction, (b) extraction after acid hydrolysis, (c) acid plus alpha-amylase hydrolysis, and (d) acid plus alpha-amylase plus cellulase hydrolysis treatments. The benzoic acid (p-hydroxybenzoic, vanillic, and protocatechuic acids) and cinnamic acid derivatives (coumaric, caffeic, ferulic, and chlorogenic acids) were identified, and some of the phenolic acids were quantified after each above-mentioned treatment. The data indicated that a combination of sequential acid, alpha-amylase, and cellulase hydrolysis treatments might be applicable for release of more phenolic acids from barley. PMID:11559137

  10. Calcium binding in α-amylases: An X-ray diffraction study at 2.1-angstrom resolution of two enzymes from Aspergillus

    International Nuclear Information System (INIS)

    X-ray diffraction analysis (at 2.1-angstrom resolution) of an acid alpha-amylase from Aspergillus niger allowed a detailed description of the stereochemistry of the calcium-binding sites. The primary site (which is essential in maintaining proper folding around the active site) contains a tightly bound Ca2+ with an unusually high number of eight ligands. A secondary binding site was identified at the bottom of the substrate binding cleft; it involves the residues presumed to play a catalytic role (Asp206 and Glu230). This explains the inhibitory effect of calcium observed at higher concentrations. Neutral Aspergillus oryzae (TAKA) α-amylase was also refined in a new crystal at 2.1-angstrom resolution. The structure of this homologous (over 80%) enzyme and addition kinetic studies support all the structural conclusions regarding both calcium-binding sites

  11. Chemical mechanical polishing of hard disk substrate with {alpha}-alumina-g-polystyrene sulfonic acid composite abrasive

    Energy Technology Data Exchange (ETDEWEB)

    Lei Hong, E-mail: hong_lei2005@yahoo.com.c [Research Center of Nano-science and Nano-technology, Shanghai University, Shanghai 200444 (China); Bu Naijing; Chen Ruling; Hao Ping [Research Center of Nano-science and Nano-technology, Shanghai University, Shanghai 200444 (China); Neng Sima; Tu Xifu; Yuen Kwok [Shenzhen Kaifa Magnetic Recording Co., LTD, Shenzhen, 518035 (China)

    2010-05-03

    {alpha}-Alumina-g-polystyrene sulfonic acid ({alpha}-Al{sub 2}O{sub 3}-g-PSS) composite abrasive was prepared by surface activation, graft polymerization and sulfonation, successively. The composition, dispersibility and morphology of the product were characterized by Fourier transformed infrared spectroscopy, laser particle size analysis and scanning electron microscopy, respectively. The chemical mechanical polishing (CMP) performances of the composite abrasive on hard disk substrate with nickel-phosphorous plating were investigated. The microscopy images of the polished surfaces show that {alpha}-Al{sub 2}O{sub 3}-g-PSS composite abrasive results in improved CMP and post-CMP cleaning performances than pure {alpha}-alumina abrasive under the same testing conditions.

  12. Construction of an in vitro primary lung co-culture platform derived from New Zealand white rabbits

    Energy Technology Data Exchange (ETDEWEB)

    Powell, Joshua D.; Hess, Becky M.; Hutchison, Janine R.; Straub, Tim M.

    2015-05-01

    We report the construction of an in vitro three dimensional (3D) co-culture platform consisting of differentiated lung epithelial cells and monocytes from New Zealand white rabbits. Rabbit lung epithelial cells were successfully grown at air-liquid interface, produced mucus, and expressed both sialic acid alpha-2,3 and alpha-2,6. Blood-derived CD14+ monocytes were deposited above the epithelial layer resulting in the differentiation of a subset of monocytes into CD11c+ cells within the co-culture. These proof-of-concept findings provide a convenient means to comparatively study in vitro versus in vivo rabbit lung responses as they relate to inhalation or lung-challenge studies.

  13. Alpha-linolenic acid regulates the growth of breast and cervical cancer cell lines through regulation of NO release and induction of lipid peroxidation

    Directory of Open Access Journals (Sweden)

    Ruchika Kaul-Ghanekar

    2013-02-01

    Full Text Available In the present work, we have analyzed the effect of the essential fatty acid, alpha linolenic acid (ALA on nitric oxide release as well as induction of lipid peroxidation in breast (MCF-7 and MDA-MB-231 and cervical (SiHa and HeLa cancer cell lines. ALA-treated cells showed a dose-dependent decrease in cell viability in both breast and cervical cancer cell lines without affecting the viability of non-cancerous transformed HEK 293 cells. Both types of cancer cells treated with ALA demonstrated a significant reduction in nitric oxide (NO release with a simultaneous increase in lipid peroxidation (LPO. This was followed by a decrease in the mitochondrial membrane potential as well as activation of caspase 3 leading to apoptosis. Thus, ALA regulated the growth of cancer cell lines through induction of lipid peroxidation and modulation of nitric oxide release resulting in apoptosis.

  14. Relations entre acides gras oméga-3, oméga-9, structures et fonctions du cerveau. Le point sur les dernières données. Le coût financier alimentaire des oméga-3 « Je chercherai à connaître les différences qui existent entre les huiles de faine, de colza, d’olive, de noix ». Honoré de Balzac. « Histoire de César Birotteau ».

    OpenAIRE

    Bourre Jean-Marie

    2003-01-01

    Pour ce qui concerne le cerveau, les acides gras ω3 ont été plus spécialement étudiés que les acides gras ω6 ou ω9. La carence en acide alpha-linolénique (18 :3 ω3) altère la structure et la fonction des membranes, et entraîne de légers dysfonctionnements cérébraux, comme cela a été montré sur les modèles animaux puis chez les nourrissons humains. Les résultats récents ont montré que son déficit alimentaire induit des anomalies plus marquées dans certaines structures cérébrales que dans d’aut...

  15. [Studies on the constituents of trichosanthes root. III. Constituents of roots of Trichosanthes bracteata Voigt].

    Science.gov (United States)

    Kitajima, J; Mukai, A; Masuda, Y; Tanaka, Y

    1989-04-01

    From the fresh roots of Trichosanthes bracteata Voigt., the following substances were identified: methyl palmitate, palmitic acid, suberic acid, alpha-spinasterol, stigmast-7-en-3 beta-ol, alpha-spinasterol 3-O-beta-D-glucopyranoside, stigmast-7-en-3 beta-ol 3-O-beta-D-glucopyranoside, glyceryl 1-palmitate, glyceryl 1-stearate, bryonolic acid, cucurbitacin B, isocucurbitacin B, 3-epi-isocucurbitacin B, 23,24-dihydrocucurbitacin B, 23,24-dihydroisocucurbitacin B, 23,24-dihydro-3-epi-isocucurbitacin B, cucurbitacin D, isocucurbitacin D and D-glucose. This root contains more than 6 times cucurbitacin of the root of T. kirilowii Maxim. var. japonicum Kitam. PMID:2760813

  16. Effect of dietary fatty acid intake on prospective weight change in the Heidelberg cohort of the European Prospective Investigation into Cancer and Nutrition

    DEFF Research Database (Denmark)

    Nimptsch, Katharina; Berg-Beckhoff, Gabi; Linseisen, Jakob

    2010-01-01

    . RESULTS: Stearic acid intake was linearly associated with weight gain (P <0.01) in men and women. Linear associations also existed for ALA and arachidonic acid intake, significantly so in women. In multinomial models, women in the highest tertile of ALA and stearic acid intake showed increased OR (95 % CI......OBJECTIVE: To evaluate the association between fatty acid (alpha-linolenic acid (ALA), EPA, DHA, palmitic, stearic, oleic, linoleic and arachidonic acids) intake and prospective weight change in the Heidelberg cohort of the European Prospective Investigation into Cancer and Nutrition. DESIGN...... categorised into four groups (weight loss, or =2.5 to or =7.5%/5 years). Energy-adjusted dietary fatty acid intake data were estimated from the FFQ completed at baseline. Multivariate linear regression models as well as multinomial logistic regression analyses (carbohydrate replacement models) were conducted...

  17. beta-carotene does not change markers of enzymatic and nonenzymatic antioxidant activity in human blood

    DEFF Research Database (Denmark)

    Castenmiller, J.J.M.; Lauridsen, Søren T.; Dragsted, Lars O.; Hof, K.H.V.; Linssen, J.P.H.; West, C.E.

    1999-01-01

    = 12) or with a spinach product (n = 12 per group), i.e., whole-leaf, minced, liquefied or liquefied spinach plus added dietary fiber. After 3 wk of dietary intervention, changes in serum or plasma concentrations of ascorbic acid, alpha-tocopherol, FRAP (ferric reducing ability of plasma) and uric acid...... and erythrocyte enzyme activities were assessed, and differences among experimental groups were tested. Consumption of spinach resulted in greater (P <0.01) erythrocyte glutathione reductase activity and lower (P <0.05) erythrocyte catalase activity and serum alpha-tocopherol concentration compared...... with the control group. Consumption of the carotenoid supplement led to lower alpha-tocopherol responses (P = 0.02) compared with the basic diet only. Our data suggest that the short-term changes in erythrocyte glutathione reductase activity and serum alpha-tocopherol concentration can be attributed to...

  18. Unsaturated compounds induce up-regulation of CD86 on dendritic cells in the in vitro sensitization assay LCSA.

    Science.gov (United States)

    Frohwein, Thomas Armin; Sonnenburg, Anna; Zuberbier, Torsten; Stahlmann, Ralf; Schreiner, Maximilian

    2016-04-01

    Unsaturated compounds are known to cause false-positive reactions in the local lymph node assay (LLNA) but not in the guinea pig maximization test. We have tested a panel of substances (succinic acid, undecylenic acid, 1-octyn-3-ol, fumaric acid, maleic acid, linoleic acid, oleic acid, alpha-linolenic acid, squalene, and arachidonic acid) in the loose-fit coculture-based sensitization assay (LCSA) to evaluate whether unspecific activation of dendritic cells is a confounder for sensitization testing in vitro. Eight out of 10 tested substances caused significant up-regulation of CD86 on dendritic cells cocultured with keratinocytes and would have been classified as sensitizers; only succinic acid was tested negative, and squalene had to be excluded from data analysis due to poor solubility in cell culture medium. Based on human data, only undecylenic acid can be considered a true sensitizer. The true sensitizing potential of 1-octyn-3-ol is uncertain. Fumaric acid and its isomer maleic acid are not known as sensitizers, but their esters are contact allergens. A group of 18- to 20-carbon chain unsaturated fatty acids (linoleic acid, oleic acid, alpha-linolenic acid, and arachidonic acid) elicited the strongest reaction in vitro. This is possibly due to the formation of pro-inflammatory lipid mediators in the cell culture causing nonspecific activation of dendritic cells. In conclusion, both the LLNA and the LCSA seem to provide false-positive results for unsaturated fatty acids. The inclusion of T cells in dendritic cell-based in vitro sensitization assays may help to eliminate false-positive results due to nonspecific dendritic cell activation. This would lead to more accurate prediction of sensitizers, which is paramount for consumer health protection and occupational safety. PMID:25975990

  19. Receptor specificity in human, avian, and equine H2 and H3 influenza virus isolates.

    Science.gov (United States)

    Connor, R J; Kawaoka, Y; Webster, R G; Paulson, J C

    1994-11-15

    The receptor specificity of 56 H2 and H3 influenza virus isolates from various animal species has been determined to test the relevance of receptor specificity to the ecology of influenza virus. The results show that the receptor specificity of both H2 and H3 isolates evaluated for sialic acid linkage specificity and inhibition of hemagglutination by horse serum correlates with the species of origin, as postulated earlier for H3 strains based on a limited survey of five human, three avian, and one equine strain. Elucidation of the amino acid sequence of several human H2 receptor variants and analysis of known sequences of H2 and H3 isolates revealed that receptor specificity varies in association with an amino acid change at residues 228 in addition to the change at residue 226 previously documented to affect receptor specificity of H3 but not H1 isolates. Residues 226 and 228 are leucine and serine in human isolates, which preferentially bind sialic acid alpha 2,6-galactose beta 1,4-N-acetyl glucosamine (SA alpha 2,6Gal), and glutamine and glycine in avian and equine isolates, which exhibit specificity for sialic acid alpha-2,3-galactose beta-1,3-N-acetyl galactosamine (SA alpha 2,3Gal). The results demonstrate that the correlation of receptor specificity and species of origin is maintained across both H2 and H3 influenza virus serotypes and provide compelling evidence that influenza virus hosts exert selective pressure to maintain the receptor specificity characteristics of strains isolated from that species. PMID:7975212

  20. [Enzyme replacement therapy in a patient with Pompe disease].

    Science.gov (United States)

    Fujikawa, Yoshinao; Kinoshita, Satoru; Miyamoto, Yusaku; Nakayama, Tojo; Endo, Yusaku; Sasaki, Masayuki

    2007-09-01

    Pompe disease is a rare autosomal recessive disease caused by the deficiency of acid alpha-glucosidase (GAA), which is required for the degradation of lysosomal glycogen. Glycogen accumulation in heart, muscle and liver eventually leads to muscle weakness, hepatomegaly and cardiomegaly. Although an approved therapy does not exist, the efficacy of enzyme replacement therapy (ERT) has recently been reported in multinational trials in Europe and the US. Here, we present data on the efficacy of recombinant human acid alpha-glucosidase (rhGAA) (provided by Genzyme Corporation) in a patient with Pompe disease. At 5 months of age, motor delay (could not raise his head) and cardiomegaly were observed. A definite diagnosis of Pompe disease was made at 8 months of age after the accumulation of glycogen in a muscle biopsy specimen was observed. This was confirmed by low GAA activity. Since then, motor delay predominated and he was unable to sit independently by age 2.5 years. Every 2 weeks, 20 mg/kg of rhGAA was infused intravenously. To assess the effectiveness, chest X-ray, echocardiography and auditory brain response were recorded. The patient was administered rhGAA for 26 months from 2 years and 8 months of age. Following the initiation of ERT, hepatomegaly and cardiac function (ejection fraction) were rapidly improved and motor function was gradually improved. At 4 years and 10 months, the patient could walk with support. No adverse event has been observed. It can be concluded that ERT with rhGAA is an effective and safe regimen for this case. PMID:17879614

  1. Differential expression of glycans in the hippocampus of rats trained on an inhibitory learning paradigm.

    Science.gov (United States)

    Hidalgo, Alejandra; Burgos, Valeria; Viola, Haydée; Medina, Jorge; Argibay, Pablo

    2006-12-01

    The glycan chains of glycoconjugates play important roles in cell-cell and cell-matrix interactions. In the CNS, previous studies on learning and memory suggest the importance of oligosaccharides attached to glycoconjugates in the modulation of synaptic connections. We studied the hippocampal glycan distribution of rats subject to an inhibitory avoidance task. The expression of glycans was examined by lectin-histochemistry using Vicia villosa lectin (VVL) for terminal alpha/beta N-acetylgalactosamine (alpha/beta GalNAc); Galanthus nivalus lectin (GNL) for terminal mannose alpha-1,3 (Man alpha-1,3); Peanut agglutinin (PNA) for galactose beta-1,3N-acetylgalactosamine (Gal beta-1,3 GalNAc); Erythrina cristagalli lectin (ECL) for galactose beta-1,4 N-acetylglucosamine (Gal beta-1,4 GlcNAc); Sambucus nigra lectin (SNA) for sialic acid alpha-2.6 galactose (SA alpha-2,6 Gal); Maackia amurensis lectin II (MAL II) for sialic acid alpha-2,3 (SA alpha-2,3); Wheat germ agglutinin (WGA) for terminal N-acetylglucosamine with/ without sialic acid (GlcNAc wo SA); succynilated WGA (sWGA) for terminal N-acetylglucosamine without sialic acid (terminal GlcNAc without SA); Griffonia simplicifolia lectin II (GSL II) for terminal alpha/beta N-acetylglucosamine (alpha/beta GlcNAc terminal); and Lotus tetragonolobus lectin (LTL) alpha-fucose. Two groups of 10 animals were examined: non-trained (Control) and Trained rats. ECL, sWGA and GSL II were negative for both groups in all the hippocampal subfields studied. For both groups, VVL was negative in CA4 and granular cells of the Dentate Gyrus (DG) and LTL was negative in the CA4 subfield. Expression of alpha/beta GalNAc, alpha-fucose and GlcNAc in other hippocampal subflields was positive, with no differences between groups. However, expression of Man alpha-1,3 was significantly higher in the CA1, CA2, CA3, and CA4 subfields in the Trained group. On the other hand, expression of Gal beta-1,3 GalNAc was significantly low in CA4 and DG in the

  2. Effects of intermediate metabolite carboxylic acids of TCA cycle on Microcystis with overproduction of phycocyanin.

    Science.gov (United States)

    Bai, Shijie; Dai, Jingcheng; Xia, Ming; Ruan, Jing; Wei, Hehong; Yu, Dianzhen; Li, Ronghui; Jing, Hongmei; Tian, Chunyuan; Song, Lirong; Qiu, Dongru

    2015-04-01

    Toxic Microcystis species are the main bloom-forming cyanobacteria in freshwaters. It is imperative to develop efficient techniques to control these notorious harmful algal blooms (HABs). Here, we present a simple, efficient, and environmentally safe algicidal way to control Microcystis blooms, by using intermediate carboxylic acids from the tricarboxylic acid (TCA) cycle. The citric acid, alpha-ketoglutaric acid, succinic acid, fumaric acid, and malic acid all exhibited strong algicidal effects, and particularly succinic acid could cause the rapid lysis of Microcystis in a few hours. It is revealed that the Microcystis-lysing activity of succinic acid and other carboxylic acids was due to their strong acidic activity. Interestingly, the acid-lysed Microcystis cells released large amounts of phycocyanin, about 27-fold higher than those of the control. On the other hand, the transcription of mcyA and mcyD of the microcystin biosynthesis operon was not upregulated by addition of alpha-ketoglutaric acid and other carboxylic acids. Consider the environmental safety of intermediate carboxylic acids. We propose that administration of TCA cycle organic acids may not only provide an algicidal method with high efficiency and environmental safety but also serve as an applicable way to produce and extract phycocyanin from cyanobacterial biomass. PMID:25342454

  3. Py-GC/MS, GC/MS and FTIR investigations on Late Roman-Egyptian adhesives from opus sectile: new insights into ancient recipes and technologies.

    Science.gov (United States)

    Ribechini, Erika; Orsini, Sibilla; Silvano, Flora; Colombini, Maria Perla

    2009-04-01

    An analytical protocol based on optical microscopy, Fourier transforms infrared spectroscopy (FTIR), analytical pyrolysis in the presence of hexamethyldisilazane followed by gas chromatographic/mass spectrometric analysis (Py-GC/MS) and gas chromatography/mass spectrometry after alkaline hydrolysis, solvent extraction and trimethylsilylation (GC/MS) was used in the chemical characterisation of the original adhesives used to fix monochrome and mosaic glass and stone plaques coming from the Late Roman archaeological site of Antinoopolis (Egypt). FTIR analysis demonstrated the presence of calcite fragments, and Py-GC/MS and GC/MS analyses provided detailed molecular compositions, highlighting the presence of a wide range of compound classes including diterpenoid acids, tricyclic abietanes with a high degree of aromatisation, mid- and long-chain monocarboxylic fatty acids, mono- and di-hydroxy acids, alpha,omega-dicaboxylic fatty acids, n-alkanols, and n-alkanes. Characteristic biomarkers and their distribution patterns indicated the presence of pine pitch in all the adhesives, which in some cases was admixed with beeswax and brassicaceae seed oil. The results provided new insights into the complex recipes used by artisans in ancient Egypt in the production of adhesives and in the sophisticated manufacture of opus sectile decorations. PMID:19298883

  4. [Clinical and biochemical alterations in rats treated with high doses of vitamin A].

    Science.gov (United States)

    Alarcón-Corredor, O M; Alfonso, R

    2007-09-01

    In the present work the effect of intramuscular administration of 30.000, 50.000 and 100.000 IU of vitamin A palmitate daily for seven days, respectively, on the liver enzyme activity in 45 white male Wistar rats, aged 12 weeks and weighing 180-200 g, have been studied. The group control was integrated by 15 healthy rats with similar characteristics (strain, gender, age and weight) to treated animals. Food and water consumption and body weights were recorded at the end of the experimental period. Rats were observed for clinical signs of toxicity. At the end of the study, rats were sacrificed under ether anesthesia. Liver samples were taken for the determination of enzyme activity. Administration of excess of vitamin A produced a significant (p anorexia, loss of body weight, alopecia, conjunctivitis, external and internal hemorrhages, skin abnormalities and death) and increased (p < 0.05) the activity of the following enzymes: alanine aminotransferase, aspartate aminotransferase, acid maltase (acid alpha-1,4-glucosidase), acid proteases, lactate dehydrogenase and alkaline phosphatase while glucose-6-phosphatase, glycogen phosphorylase, alpha-amylase, cholinesterase and arginase decreased (p < 0.05) as compared with untreated controls. These changes depend on the doses given of vitamin A. In conclusion, our results provide evidence that short-term administration of high doses of vitamin A determined diverse and variable clinical signs and produces a marked alteration of activity of liver enzymes. PMID:18271400

  5. Retrospective, Single Center Study of Clinical, Paraclinical and Natural Course of Infantile-Onset Pompe Disease

    Directory of Open Access Journals (Sweden)

    Noormohammad Noori

    2015-01-01

    Full Text Available Background: Infantile-onset Pompe disease is a rare genetic and lethal disorder which is caused by the lack of acid alpha-glucosidase activity (GAA. The aim of our study was to identify the demographic and clinical characteristics, and natural history of these patients. Materials and Methods: In this retrospective study, clinical file of 15 patients diagnosed with infantile-onset Pompe disease whose symptoms started before the age of 12 months were studied. Diagnosis was based on clinical history, physical examination and diagnostic parameters in chest X-ray, echocardiogram, electrocardiogram and biochemical tests after rule out the other metabolic and neuromuscular disorders. Results: Sixty percent of the patients were male and 40% were female. The mean age at the onset of symptoms was 78 days (range: 3-150 days. Most frequent clinical and paraclinical symptoms were cardiomegaly, hypotonia, hyporeflexia, macroglossia, failure to thrive, hepatomegaly, and feeding problems, respectively. The mean age at the time of death was 5.96 months (range: 4-8 months, and all patients died before one year of age. Muscle enzymes including AST, ALT, LDH, and CPK were elevated in all patients. Due to the lack of availability, enzyme replacement therapy was not possible for any patient. Conclusion: The study showed that despite the supportive measures and no specific treatment, the clinical course is not significantly different with similar studies and the overall prognosis of this form of disease is very poor and disappointing.

  6. Late-onset Pompe disease with complicated intracranial aneurysm: a Chinese case report

    Directory of Open Access Journals (Sweden)

    Zhang B

    2016-03-01

    Full Text Available Bin Zhang,1,2,* Yuying Zhao,1,3,* Junling Liu,1,4 Ling Li,1 Jingli Shan,1 Dandan Zhao,1 Chuanzhu Yan1,3 1Laboratory of Neuromuscular Disorders and Department of Neurology, Qilu Hospital of Shandong University, Jinan, Shandong, 2Department of Neurology, Liaocheng People’s Hospital, Liaocheng, Shandong, 3Department of Neurology, Qilu Hospital of Shandong University, Key Laboratory for Experimental Teratology of the Ministry of Education, Brain Science Research Institute, Shandong University, Jinan, Shandong, 4Department of Neurology, Affiliated Hospital of Weifang Medical University, Weifang, Shandong, People’s Republic of China *These authors contributed equally to this work Abstract: Pompe disease is a rare autosomal recessive hereditary disease caused by genetic defects of acid maltase. This disease could be divided into two forms: infantile and late-onset, which mainly affect cardiac, respiratory, and skeletal muscle systems. Late-onset patients mainly show symptoms of skeletal muscle involvement, but recent reports have found that the central nervous system was also affected in some patients. Herein, we report a case of a female, adolescent-onset Pompe patient, who was diagnosed with complicated intracranial aneurysm in adulthood. Keywords: Pompe disease, glycogen storage disease II, acid maltase, acid alpha-glucosidase, cerebrovascular disorders

  7. Comparison of antioxidant activity between aromatic indolinonic nitroxides and natural and synthetic antioxidants.

    Science.gov (United States)

    Damiani, Elisabetta; Belaid, Chokri; Carloni, Patricia; Greci, Lucedio

    2003-07-01

    In view of the possible employment of nitroxide compounds in various fields, it is important to know how they compare with other synthetic antioxidant compounds currently used in several industries and with naturally occurring antioxidants. To address this issue, the antioxidant activity of two aromatic indolinonic nitroxides synthesized by us was compared with both commercial phenolic antioxidants (BHT and BHA) and with natural phenolic antioxidants (alpha-hydroxytyrosol, tyrosol, caffeic acid, alpha-tocopherol). DPPH radical scavenging ability and the inhibition of both lipid and protein oxidation induced by the peroxyl-radical generator, AAPH, were evaluated. The results obtained show that overall: (i) the reduced forms of the nitroxide compounds are better scavengers of DPPH radical than butylated hydroxyanisole (BHA) and butylated hydroxytoluene (BLT) but less efficient than the natural compounds; (ii) the nitroxides inhibit both linolenic acid micelles and bovine serum albumin (BSA) oxidation to similar extents as most of the other compounds in a concentration-dependent fashion. Since the aromatic nitroxides tested in this study are less toxic than BHT, these compounds may be regarded as potential, alternative sources for several applications. The mechanisms underlying the antioxidant activity of nitroxides were further confirmed by UV-Vis absorption spectroscopy experiments and macroscale reactions in the presence of radicals generated by thermolabile azo-compounds. Distribution coefficients in octanol/buffer of the nitroxides and the other compounds were also determined as a measure of lipophilicity. PMID:12911269

  8. The Amino Acid Composition of the Sutter's Mill Carbonaceous Chondrite

    Science.gov (United States)

    Glavin, D. P.; Burton, A. S.; Elsila, J. E.; Dworkin, J. P.; Yin, Q. Z.; Cooper, G.; Jenniskens, P.

    2012-01-01

    In contrast to the Murchison meteorite which had a complex distribution of amino acids with a total C2 to Cs amino acid abundance of approx.14,000 parts-per-billion (ppb) [2], the Sutters Mill meteorite was found to be highly depleted in amino acids. Much lower abundances (approx.30 to 180 ppb) of glycine, beta-alanine, L-alanine and L-serine were detected in SM2 above procedural blank levels indicating that this meteorite sample experienced only minimal terrestrial amino acid contamination after its fall to Earth. Carbon isotope measurements will be necessary to establish the origin of glycine and beta-alanine in SM2. Other non-protein amino acids that are rare on Earth, yet commonly found in other CM meteorites such as aaminoisobutyric acid (alpha-AIB) and isovaline, were not identified in SM2. However, traces of beta-AIB (approx.1 ppb) were detected in SM2 and could be" extraterrestrial in origin. The low abundances of amino acids in the Sutter's Mill meteorite is consistent with mineralogical evidence that at least some parts of the Sutter's Mill meteorite parent body experienced extensive aqueous and/or thermal alteration.

  9. A thermogravimetric analysis (TGA) method developed for estimating the stoichiometric ratio of solid-state {alpha}-cyclodextrin-based inclusion complexes

    Energy Technology Data Exchange (ETDEWEB)

    Bai, Yuxiang; Wang, Jinpeng; Bashari, Mohanad; Hu, Xiuting [The State Key Laboratory of Food Science and Technology, School of Food Science and Technology, Jiangnan University, Wuxi 214122 (China); Feng, Tao [School of Perfume and Aroma Technology, Shanghai Institute of Technology, Shanghai 201418 (China); Xu, Xueming [The State Key Laboratory of Food Science and Technology, School of Food Science and Technology, Jiangnan University, Wuxi 214122 (China); Jin, Zhengyu, E-mail: jinlab2008@yahoo.com [The State Key Laboratory of Food Science and Technology, School of Food Science and Technology, Jiangnan University, Wuxi 214122 (China); Tian, Yaoqi, E-mail: yqtian@jiangnan.edu.cn [The State Key Laboratory of Food Science and Technology, School of Food Science and Technology, Jiangnan University, Wuxi 214122 (China)

    2012-08-10

    Highlights: Black-Right-Pointing-Pointer We develop a TGA method for the measurement of the stoichiometric ratio. Black-Right-Pointing-Pointer A series of formulas are deduced to calculate the stoichiometric ratio. Black-Right-Pointing-Pointer Four {alpha}-CD-based inclusion complexes were successfully prepared. Black-Right-Pointing-Pointer The developed method is applicable. - Abstract: An approach mainly based on thermogravimetric analysis (TGA) was developed to evaluate the stoichiometric ratio (SR, guest to host) of the guest-{alpha}-cyclodextrin (Guest-{alpha}-CD) inclusion complexes (4-cresol-{alpha}-CD, benzyl alcohol-{alpha}-CD, ferrocene-{alpha}-CD and decanoic acid-{alpha}-CD). The present data obtained from Fourier transform-infrared (FT-IR) spectroscopy showed that all the {alpha}-CD-based inclusion complexes were successfully prepared in a solid-state form. The stoichiometric ratios of {alpha}-CD to the relative guests (4-cresol, benzyl alcohol, ferrocene and decanoic acid) determined by the developed method were 1:1, 1:2, 2:1 and 1:2, respectively. These SR data were well demonstrated by the previously reported X-ray diffraction (XRD) method and the NMR confirmatory experiments, except the SR of decanoic acid with a larger size and longer chain was not consistent. It is, therefore, suggested that the TGA-based method is applicable to follow the stoichiometric ratio of the polycrystalline {alpha}-CD-based inclusion complexes with smaller and shorter chain guests.

  10. The role of topical dermocosmetics in acne vulgaris.

    Science.gov (United States)

    Araviiskaia, E; Dréno, B

    2016-06-01

    Acne is a common chronic inflammatory disease and treatment modalities based on acne severity are well established. The role of dermocosmetics in dermatology, and in particular acne, is becoming more important as more research elucidates the mechanisms of action of products in the pathogenesis of acne. Dermocosmetics have the potential to be used as monotherapy or in combination with medical treatment. Therefore, it has become important for dermatologists to understand dermocosmetics to effectively and appropriately advise patients on their use. The objective of this review was to provide new insights into the role of traditional and novel ingredients in dermocosmetics for the treatment of acne, based on the authors' objective assessment of the published literature. The type of products discussed include: those which have a sebostatic effect, such as topical antioxidants and niacinamide; agents targeting abnormal keratinization, such as salicylic acid, lipo-hydroxy acid, alpha-hydroxy acids, retinol-based products and linoleic acid; agents targeting Propionibacterium acnes, such as lauric acid; and anti-inflammatory agents such as nicotinamide, alpha-linolenic acid and zinc salts. Despite the scientific advances in understanding these cosmetic ingredients, there still remains a lack of rigorous controlled studies in this area. PMID:26916232

  11. [Alpha-linolenic acid and cardiovascular diseases].

    Science.gov (United States)

    Ristić-Medić, Danijela; Ristić, Gordana; Tepsić, Vesna

    2003-01-01

    IMPORTANCE AND METABOLISM OF ALPHA-LINOLENIC ACID: Alpha-linolenic acid is an essential fatty acid which cannot be produced in the body and must be taken by food. Both in animals and humans, alpha-linolenic acid is desaturated and elongated into eicosapentaenoic and docosahexaenoic acid. It is also incorporated into plasma and tissue lipids and its conversion is affected by levels of linoleic acid. POTENTIAL ROLE IN PATHOGENESIS OF CARDIOVASCULAR DISEASES: Diet enriched in n-3 fatty acids, especially alpha-linolenic acid, reduces the incidence of cardiac death. Studies have shown that alpha linolenic acid prevents ventricular fibrillation which is the main cause of cardiac death. Studies in rats suggest that alpha-linolenic acid may be more effective in preventing ventricular fibrillations than eicosapentaenoic and docosahexaenoic acid. Furthermore, alpha-linolenic acid is the main fatty acid decreasing platalet aggregation which is an important step in thrombosis i.e. non-fatal myocardial infarction and stroke. DIETARY SOURCES AND NUTRITION RECOMMENDATIONS: Dietary sources include flaxseed and flaxseed oil, canola oil, soybean and soybean oil, pumpkin seed and pumpkin oil, walnuts and walnut oil. Strong evidence supports beneficial effects of alpha-linolenic acid and its dietary sources should be incorporated into balanced diet for prevention of cardiovascular diseases. The recommended daily intake is 2 g with a ratio of 5/1 for linoleic/alpha-linolenic acid. PMID:15510909

  12. Fractures in children with Pompe disease: a potentiallong-term complication

    Energy Technology Data Exchange (ETDEWEB)

    Case, Laura E. [Duke University Medical Center, Division of Physical Therapy, Department of Community and Family Medicine, Durham, NC (United States); Hanna, Rabi; DeArmey, Stephanie; Mackey, Joanne; Boney, Anne; Chen, Yuan-Tsong; Kishnani, Priya S. [Duke University Medical Center, Department of Pediatrics, Durham, NC (United States); Frush, Donald P. [Duke University Medical Center, Department of Radiology, Durham, NC (United States); Krishnamurthy, Vidya [Duke University Medical Center, Department of Pediatrics and Department of Medicine, Durham, NC (United States); Morgan, Claire; Bouchard, Susan [Genzyme Corporation, Pharmacovigilance, Cambridge, MA (United States); Corzo, Deyanira [Genzyme Corporation, Cambridge, MA (United States); Weber, Thomas J. [Duke University Medical Center, Department of Medicine, Durham, NC (United States)

    2007-05-15

    Pompe disease (glycogen storage disease type II or acid maltase deficiency) is an autosomal recessive disorder caused by deficiency of the lysosomal enzyme acid {alpha}-glucosidase (GAA). Classic infantile-onset disease, characterized by cardiomegaly and profound weakness, leads to death in the first year of life from cardiorespiratory failure. Reversal of cardiomyopathy and improved motor function have been shown in clinical trials of rhGAA enzyme replacement therapy (ERT) with alglucosidase alfa (Myozyme), recently approved for clinical use. Increased survival potentially unmasks long-term complications of this previously lethal disease, including risk of skeletal fracture, recently identified at our institution and not previously reported in children with Pompe disease. To report the risk of fracture in children with Pompe disease with increased survival with ERT. We present four cases of fracture in patients with classic infantile Pompe disease treated with ERT at our institution, and review a study database for additional reports of fracture in this population. We review 19 fractures in 14 children with Pompe disease on ERT. Radiologists should be familiar with and vigilant for the association of fractures and increased survival on ERT in children with Pompe disease. We discuss potential mechanisms, implications for radiographic surveillance, potential intervention, and needs for further research. (orig.)

  13. Salinity induced changes in photosynthetic pigment and antioxidant responses in Sesuvium portulacastrum.

    Science.gov (United States)

    Sivakumar, Thirumal; Panneerselvam, Rajaram

    2011-11-01

    The production of leaf and root antioxidant changes when exposed to saline conditions were investigated in the perennial halophyte Sesuvium portulacastrum L. Plants were grown with a nonsterilized soil and sterilized soil with 50 and 100% of sterilized seawater on 25, 55 and 85 Days After Planting (DAP). The plants were harvested on 30th, 60th and 90th DAP and used for analyzing the photosynthetic pigments, antioxidant enzyme activities viz., Superoxide dismutase (SOD; EC1.15.1.1) Ascorbate peroxidase (APX, EC 1.11.1.11) and non enzymatic antioxidant contents like ascorbic acid, alpha-tocopherol, reduced glutathione were determined. Plants exposed to salinity, either alone (SSW) sterilized seawater/unsterilized soil (USS) along with higher pigments, antioxidative enzymes and Na+ ions response. This tendency was generally more marked in SSW/USS plants when compared to SSW/SS plants. The concentration of SSW/SS was negatively correlated with the antioxidative capacity of the plant, either enzymatic or non enzymatic and K+ ions. These data suggest that the enhancement of the antioxidative response is of crucial significance for S. portulacastrum plants growing under saline conditions. PMID:22514886

  14. Involvement of protein kinase C activation in L-leucine-induced stimulation of protein synthesis in l6 myotubes.

    Science.gov (United States)

    Yagasaki, Kazumi; Morisaki, Naoko; Kitahara, Yoshiro; Miura, Atsuhito; Funabiki, Ryuhei

    2003-11-01

    Effects of leucine and related compounds on protein synthesis were studied in L6 myotubes. The incorporation of [(3)H]tyrosine into cellular protein was measured as an index of protein synthesis. In leucine-depleted L6 myotubes, leucine and its keto acid, alpha-ketoisocaproic acid (KIC), stimulated protein synthesis, while D-leucine did not. Mepacrine, an inhibitor of both phospholipases A(2) and C, canceled stimulatory actions of L-leucine and KIC on protein synthesis. Neither indomethacin, an inhibitor of cyclooxygenase, nor caffeic acid, an inhibitor of lipoxygenase, diminished their stimulatory actions, suggesting no involvement of arachidonic acid metabolism. Conversely, 1-O-hexadecyl-2-O-methylglycerol, an inhibitor of proteinkinase C, significantly canceled the stimulatory actions of L-leucine and KIC on protein synthesis, suggesting an involvement of phosphatidylinositol degradation and activation of protein kinase C. L-Leucine caused a rapid activation of protein kinase C in both cytosol and membrane fractions of the cells. These results strongly suggest that both L-leucine and KIC stimulate protein synthesis in L6 myotubes through activation of phospholipase C and protein kinase C. PMID:19003213

  15. Immunomodulatory gene therapy prevents antibody formation and lethal hypersensitivity reactions in murine pompe disease.

    Science.gov (United States)

    Sun, Baodong; Kulis, Michael D; Young, Sarah P; Hobeika, Amy C; Li, Songtao; Bird, Andrew; Zhang, Haoyue; Li, Yifan; Clay, Timothy M; Burks, Wesley; Kishnani, Priya S; Koeberl, Dwight D

    2010-02-01

    Infantile Pompe disease progresses to a lethal cardiomyopathy in absence of effective treatment. Enzyme-replacement therapy (ERT) with recombinant human acid alpha-glucosidase (rhGAA) has been effective in most patients with Pompe disease, but efficacy was reduced by high-titer antibody responses. Immunomodulatory gene therapy with a low dose adeno-associated virus (AAV) vector (2 x 10(10) particles) containing a liver-specific regulatory cassette significantly lowered immunoglobin G (IgG), IgG1, and IgE antibodies to GAA in Pompe disease mice, when compared with mock-treated mice (P mast cell protease-1 (MMCP-1) followed the pattern associated with hypersensitivity reactions (P cells (Treg) were demonstrated to play a role in the tolerance induced by gene therapy as depletion of Treg led to an increase in GAA-specific IgG (P Treg depleted mice were challenged with GAA and had significantly stronger allergic reactions than mice given gene therapy without subsequent Treg depletion (temperature: P < 0.01; symptoms: P < 0.05). Ubiquitous GAA expression failed to prevent antibody formation. Thus, immunomodulatory gene therapy could provide adjunctive therapy in lysosomal storage disorders treated by enzyme replacement. PMID:19690517

  16. Indigenous amino acids in primitive CR meteorites

    CERN Document Server

    Martins, Z; Orzechowska, G E; Fogel, M L; Ehrenfreund, P

    2008-01-01

    CR meteorites are among the most primitive meteorites. In this paper, we report the first measurements of amino acids in Antarctic CR meteorites, two of which show the highest amino acid concentrations ever found in a chondrite. EET92042, GRA95229 and GRO95577 were analyzed for their amino acid content using high performance liquid chromatography with UV fluorescence detection (HPLC-FD) and gas chromatographymass spectrometry (GC-MS). Our data show that EET92042 and GRA95229 are the most amino acid-rich chondrites ever analyzed, with total amino acid concentrations ranging from 180 parts-per-million (ppm) to 249 ppm. GRO95577, however, is depleted in amino acids. The most abundant amino acids present in the EET92042 and GRA95229 meteorites are the alpha-amino acids glycine, isovaline, alpha-aminoisobutyric acid (alpha-AIB), and alanine, with delta13C values ranging from +31.6per mil to +50.5per mil. The carbon isotope results together with racemic enantiomeric ratios determined for most amino acids strongly i...

  17. Intestinal lactase (beta-galactosidase) and other glycosidase activities in suckling and adult tammar wallabies (Macropus eugenii).

    Science.gov (United States)

    Walcott, P J; Messer, M

    1980-10-01

    The activities of various glycosidases in homogenates of the small intestinal mucosa of two adult and 18 suckling tammar wallabies (M. eugenii) aged from 6 to 50 weeks were investigated. Lactase (beta-D-galactosidase), beta-N-acetylglucosaminidase, alpha-L-fucosidase and neuraminidase activities were high during the first 34 weeks post partum and then declined to very low levels. Maltase, isomaltase, sucrase and trehalase activities were very low or absent during the first 34 weeks, and then increased. The lactase activity was unusual in being greater in the distal than the middle or proximal thirds of the intestine, and in its low pH optimum (pH 4.6), inhibition by p-chloromercuribenzene sulfonate but not by Tris, and lack of cellobiase activity. These properties are those of a lysosomal acid beta-galactosidase rather than of a brush border neutral lactase. The maltase activity had the characteristics of a lysosomal acid alpha-glucosidase early in lactation and of a brush border neutral maltase in adult animals. The significance of these findings is discussed in relation to changes in dietary carbohydrates during weaning and to the mode of digestion of milk carbohydrates by the pouch young. PMID:6783021

  18. Nutrients and antioxidant molecules in yellow plums (Prunus domestica L.) from conventional and organic productions: a comparative study.

    Science.gov (United States)

    Lombardi-Boccia, Ginevra; Lucarini, Massimo; Lanzi, Sabina; Aguzzi, Altero; Cappelloni, Marsilio

    2004-01-14

    Yellow plums (Prunus domestica L) conventionally and organically grown in the same farm were selected to study the influence of different agronomic practices on antioxidant vitamins (ascorbic acid, vitamin E, beta-carotene) and phenolics (total polyphenols, phenolic acids, flavonols) concentration. Conventional plums were grown on tilled soil. Three organic cultivations were performed: tilled soil, soil covered with trifolium, and soil covered with natural meadow. Differences in macronutrients were marginal, whereas antioxidant vitamins and phenolic compounds concentration markedly differed among cultivations. Ascorbic acid, alpha-, gamma-tocopherols, and beta-carotene were higher in organic plums grown on soil covered with natural meadow. The highest phenolic acids content was detected in plums grown on soil covered with trifolium. Total polyphenols content was higher in conventional plums. Quercetin was higher in conventional plums, but myrecitin and kaempferol were higher in organic plums. Under the same cultivar and climate conditions, the type of soil management turned out of primary importance in influencing the concentration of health-promoting compounds. PMID:14709018

  19. Simultaneous quantitative determination of alpha-ketoglutaric acid and 5-hydroxymethylfurfural in human plasma by gas chromatography-mass spectrometry.

    Science.gov (United States)

    Wagner, Bernhard M; Donnarumma, Fabrizio; Wintersteiger, Reinhold; Windischhofer, Werner; Leis, Hans J

    2010-04-01

    Alpha-ketoglutaric acid (alpha-KG) and 5-hydroxymethylfurfural (5-HMF) are currently under investigation as promising cancer cell damaging agents. A method for the simultaneous quantitative determination of alpha-KG and 5-HMF in human plasma was established for screening these compounds in human plasma. Plasma samples were directly treated with O-(2,3,4,5,6-pentafluorobenzyl) hydroxylamine hydrochloride to form the corresponding oximes, thus facilitating subsequent liquid-liquid extraction. After formation of the trimethylsilyl ethers, samples were analyzed by gas chromatography with electron ionization mass spectrometry. Stable isotope labeled standards were used, the preparation of (13)C(6)-5-HMF is described. Limits of quantitation were set to 0.938 microg/mL for alpha-KG and 0.156 microg/mL for 5-HMF. Inter-day accuracy was < or = 93.7% (alpha-KG) and < or = 92.8% (5-HMF). Inter-day precision was < or = 6.0% (alpha-KG) and < or = 4.6% (5-HMF). The method has been successfully applied to pharmacokinetic profiling of the compounds after intravenous application. PMID:20155414

  20. Gamma1- and gamma2-syntrophins, two novel dystrophin-binding proteins localized in neuronal cells.

    Science.gov (United States)

    Piluso, G; Mirabella, M; Ricci, E; Belsito, A; Abbondanza, C; Servidei, S; Puca, A A; Tonali, P; Puca, G A; Nigro, V

    2000-05-26

    Dystrophin is the scaffold of a protein complex, disrupted in inherited muscular dystrophies. At the last 3' terminus of the gene, a protein domain is encoded, where syntrophins are tightly bound. These are a family of cytoplasmic peripheral membrane proteins. Three genes have been described encoding one acidic (alpha1) and two basic (beta1 and beta2) proteins of approximately 57-60 kDa. Here, we describe the characterization of two novel putative members of the syntrophin family, named gamma1- and gamma2-syntrophins. The human gamma1-syntrophin gene is composed of 19 exons and encodes a brain-specific protein of 517 amino acids. The human gamma2-syntrophin gene is composed of at least 17 exons, and its transcript is expressed in brain and, to a lesser degree, in other tissues. We mapped the gamma1-syntrophin gene to human chromosome 8q11 and the gamma2-syntrophin gene to chromosome 2p25. Yeast two-hybrid experiments and pull-down studies showed that both proteins can bind the C-terminal region of dystrophin and related proteins. We raised antibodies against these proteins and recognized expression in both rat and human central neurons, coincident with RNA in situ hybridization of adjacent sections. Our present findings suggest a differentiated role of a modified dystrophin-associated complex in the central nervous system. PMID:10747910

  1. A unique de novo interstitial deletion of chromosome 17, del(17)(q23.2q24.3) in a female newborn with multiple congenital anomalies

    Energy Technology Data Exchange (ETDEWEB)

    Levin, M.L.; Shaffer, L.G.; Lewis, R.A. [Baylor College of Medicine, Houston, TX (United States)] [and others

    1994-09-01

    Contiguous gene or microdeletion syndromes occurring on chromosome 17p include the Smith-Magenis and Miller-Dieker syndromes associated with interstitial deletions of 17p11.2 and 17p13.3, respectively. Other cytogenetically visible interstitial deletions on chromosome 17 are quite rare or unique. We describe a newborn with a novel interstitial deletion of the long arm of chromosome 17 [del(17)(q23.2q24.3)] who died on day of life 17 during a recurrent apneic episode. We have compared our patient`s phenotype and karyotype to two reported patients with deletion 17q with minor clinical overlap. The most striking clinical features of this patient were severe intrauterine growth retardation, widespread skeletal malformations (split sutures, hypoplastic acetabulae and scapulae, vertebral anomalies, and digital hypoplasia), cutis verticis gyrata, dysmorphic facial features, and oropharyngeal malformations (absent uvula and submucous cleft palate). Mild congenital heart disease and anomalous optic nerves were also present. Parental karyotyps were normal. DNA from parents and patient has been collected and cell lines established on both parents. Genes which have been previously mapped to the region that is apparently deleted in this patient include: chorionic somatomammotropin A, growth hormone (normal), acid alpha-glucosidase, apolipoprotein H, and the alpha peptide of type 4 voltage gated sodium channel. As in other clinical cytogenetic syndromes, further descriptions of patients with similar or overlapping rearrangements in this region will be necessary to delineate genotype/phenotype correlations for chromosome 17.

  2. A novel approach to analyzing fMRI and SNP data via parallel independent component analysis

    Science.gov (United States)

    Liu, Jingyu; Pearlson, Godfrey; Calhoun, Vince; Windemuth, Andreas

    2007-03-01

    There is current interest in understanding genetic influences on brain function in both the healthy and the disordered brain. Parallel independent component analysis, a new method for analyzing multimodal data, is proposed in this paper and applied to functional magnetic resonance imaging (fMRI) and a single nucleotide polymorphism (SNP) array. The method aims to identify the independent components of each modality and the relationship between the two modalities. We analyzed 92 participants, including 29 schizophrenia (SZ) patients, 13 unaffected SZ relatives, and 50 healthy controls. We found a correlation of 0.79 between one fMRI component and one SNP component. The fMRI component consists of activations in cingulate gyrus, multiple frontal gyri, and superior temporal gyrus. The related SNP component is contributed to significantly by 9 SNPs located in sets of genes, including those coding for apolipoprotein A-I, and C-III, malate dehydrogenase 1 and the gamma-aminobutyric acid alpha-2 receptor. A significant difference in the presences of this SNP component is found between the SZ group (SZ patients and their relatives) and the control group. In summary, we constructed a framework to identify the interactions between brain functional and genetic information; our findings provide new insight into understanding genetic influences on brain function in a common mental disorder.

  3. Extraterrestrial Amino Acids in the Almahata Sitta Meteorite

    Science.gov (United States)

    Glavin, Daniel P.; Aubrey, Andrew D.; Callahan, Michael P.; Dworkin, Jason P.; Elsila, Jamie E.; Parker, Eric T.; Bada, Jeffrey L.

    2009-01-01

    Amino acid analysis of a meteorite fragment of asteroid 2008 TC(sub 3) called Almahata Sitta was carried out using reverse-phase high-perfo rmance liquid chromatography coupled with UV fluorescence detection a nd time-of-flight mass spectrometry (HPLC-FD/ToF-MS) as part of a sam ple analysis consortium. HPLC analyses of hot-water extracts from the meteorite revealed a complex distribution of two- to six-carbon aliph atic amino acids and one- to three carbon amines with abundances rang ing from 0.5 to 149 parts-per-billion (ppb). The enantiomeric ratios of the amino acids alanine, Beta-amino-n-butyric acid (Beta-ABA), 2-amino-2- methylbutanoic acid (isovaline), and 2-aminopentanoic acid (no rvaline) in the meteorite were racemic (D/L approximately 1), indicat ing that these amino acids are indigenous to the meteorite and not te rrestrial contaminants. Several other non-protein amino acids were also identified in the meteorite above background levels including alpha -aminoisobutyric acid (alpha-AIB), 4-amino-2- methybutanoic acid, 4-a mino-3-methylbutanoic acid, and 3-, 4-, and 5-aminopentanoic acid. Th e total abundances of isovaline and AlB in Almahata Sitta are approximately 1000 times lower than the abundances of these amino acids found in the CM carbonaceous meteorite Murchison. The extremely love abund ances and unusual distribution of five carbon amino acids in Almahata Sitta compared to Cl, CM, and CR carbonaceous meteorites and may be due to extensive thermal alteration of amino acids on the parent aster oid by partial melting during formation or impact shock heating.

  4. Age-related macular degeneration: prevention and treatment. A review

    Directory of Open Access Journals (Sweden)

    K. A. Mirzabekova

    2014-01-01

    Full Text Available Age-related macular degeneration (AMD is a multifactorial disease. Age, light exposure, smoking, melanin levels and low-antioxidant diet are contributed to AMD development and progression. Cardiovascular disorders are of considerable importance as well. In macula, photoreceptor outer segments that are rich in polyunsaturated fatty acids (FA, particularly, docosahexaenoic acid (DHA, are susceptible to free radicals damage. High blood flow velocity and oxygen partial pressure as well as direct sunlight exposure induce oxidative processes. The source of free radicals in photoreceptor cells and retinal pigment epithelium (RPE is an extensive mitochondrial metabolism, photoreceptor outer segments phagocytosis, lipofuscin phototoxic activity and hemoglobin or protoporphyrin precursors photosensitization. Oxidative stress is considered as an universal component of cell depth in necrosis, apoptosis and toxic damage. Antioxidant protective system consists of enzymes (superoxide dismutase, glutathione peroxidase and catalase and non-enzymatic factors (ascorbic acid, alpha tocopherol, retinol, carotenoids. Specific antioxidant food supplement containing ascorbic acid (500 mg, vitamin E (400 IU and beta carotene (15 mg coupled with zinc (80 mg of zinc oxide and copper (2 mg of copper oxide results in 25 % decrease in late-stage AMD development rate. Amongst the agents that can protect retina from oxidative stress and AMD development, carotenoids are of special importance. Lutein and zeaxanthin containing in retina and lens screen blue light from central area of the retina. They also absorb blue light and inhibit free radicals generation thus preventing polyunsaturated FA light destruction. Association between lutein and zeaxanthin intake and late-stage AMD risk was revealed. Amongst the most important factors which deficiency favors macular degeneration are omega-3 FAs, i.e., DHA. DHA is the key component of visual pigment rhodopsin transformation. It

  5. Age-related macular degeneration: prevention and treatment. A review

    Directory of Open Access Journals (Sweden)

    K. A. Mirzabekova

    2014-07-01

    Full Text Available Age-related macular degeneration (AMD is a multifactorial disease. Age, light exposure, smoking, melanin levels and low-antioxidant diet are contributed to AMD development and progression. Cardiovascular disorders are of considerable importance as well. In macula, photoreceptor outer segments that are rich in polyunsaturated fatty acids (FA, particularly, docosahexaenoic acid (DHA, are susceptible to free radicals damage. High blood flow velocity and oxygen partial pressure as well as direct sunlight exposure induce oxidative processes. The source of free radicals in photoreceptor cells and retinal pigment epithelium (RPE is an extensive mitochondrial metabolism, photoreceptor outer segments phagocytosis, lipofuscin phototoxic activity and hemoglobin or protoporphyrin precursors photosensitization. Oxidative stress is considered as an universal component of cell depth in necrosis, apoptosis and toxic damage. Antioxidant protective system consists of enzymes (superoxide dismutase, glutathione peroxidase and catalase and non-enzymatic factors (ascorbic acid, alpha tocopherol, retinol, carotenoids. Specific antioxidant food supplement containing ascorbic acid (500 mg, vitamin E (400 IU and beta carotene (15 mg coupled with zinc (80 mg of zinc oxide and copper (2 mg of copper oxide results in 25 % decrease in late-stage AMD development rate. Amongst the agents that can protect retina from oxidative stress and AMD development, carotenoids are of special importance. Lutein and zeaxanthin containing in retina and lens screen blue light from central area of the retina. They also absorb blue light and inhibit free radicals generation thus preventing polyunsaturated FA light destruction. Association between lutein and zeaxanthin intake and late-stage AMD risk was revealed. Amongst the most important factors which deficiency favors macular degeneration are omega-3 FAs, i.e., DHA. DHA is the key component of visual pigment rhodopsin transformation. It

  6. Impaired Organization and Function of Myofilaments in Single Muscle Fibers from a Mouse Model of Pompe Disease

    Energy Technology Data Exchange (ETDEWEB)

    Xu, S.; Galperin, M; Melvin, G; Horowits, R; Raben, N; Plotz, P; Yu, L

    2010-01-01

    Pompe disease, a deficiency of lysosomal acid {alpha}-glucosidase, is a disorder of glycogen metabolism that can affect infants, children, or adults. In all forms of the disease, there is progressive muscle pathology leading to premature death. The pathology is characterized by accumulation of glycogen in lysosomes, autophagic buildup, and muscle atrophy. The purpose of the present investigation was to determine if myofibrillar dysfunction in Pompe disease contributes to muscle weakness beyond that attributed to atrophy. The study was performed on isolated myofibers dissected from severely affected fast glycolytic muscle in the {alpha}-glucosidase knockout mouse model. Psoas muscle fibers were first permeabilized, so that the contractile proteins could be directly relaxed or activated by control of the composition of the bathing solution. When normalized by cross-sectional area, single fibers from knockout mice produced 6.3 N/cm{sup 2} of maximum Ca{sup 2+}-activated tension compared with 12.0 N/cm{sup 2} produced by wild-type fibers. The total protein concentration was slightly higher in the knockout mice, but concentrations of the contractile proteins myosin and actin remained unchanged. Structurally, X-ray diffraction showed that the actin and myosin filaments, normally arranged in hexagonal arrays, were disordered in the knockout muscle, and a lower fraction of myosin cross bridges was near the actin filaments in the relaxed muscle. The results are consistent with a disruption of actin and myosin interactions in the knockout muscles, demonstrating that impaired myofibrillar function contributes to weakness in the diseased muscle fibers.

  7. Fiber type conversion by PGC-1α activates lysosomal and autophagosomal biogenesis in both unaffected and Pompe skeletal muscle.

    Directory of Open Access Journals (Sweden)

    Shoichi Takikita

    Full Text Available PGC-1α is a transcriptional co-activator that plays a central role in the regulation of energy metabolism. Our interest in this protein was driven by its ability to promote muscle remodeling. Conversion from fast glycolytic to slow oxidative fibers seemed a promising therapeutic approach in Pompe disease, a severe myopathy caused by deficiency of the lysosomal enzyme acid alpha-glucosidase (GAA which is responsible for the degradation of glycogen. The recently approved enzyme replacement therapy (ERT has only a partial effect in skeletal muscle. In our Pompe mouse model (KO, the poor muscle response is seen in fast but not in slow muscle and is associated with massive accumulation of autophagic debris and ineffective autophagy. In an attempt to turn the therapy-resistant fibers into fibers amenable to therapy, we made transgenic KO mice expressing PGC-1α in muscle (tgKO. The successful switch from fast to slow fibers prevented the formation of autophagic buildup in the converted fibers, but PGC-1α failed to improve the clearance of glycogen by ERT. This outcome is likely explained by an unexpected dramatic increase in muscle glycogen load to levels much closer to those observed in patients, in particular infants, with the disease. We have also found a remarkable rise in the number of lysosomes and autophagosomes in the tgKO compared to the KO. These data point to the role of PGC-1α in muscle glucose metabolism and its possible role as a master regulator for organelle biogenesis - not only for mitochondria but also for lysosomes and autophagosomes. These findings may have implications for therapy of lysosomal diseases and other disorders with altered autophagy.

  8. Pompe disease: from pathophysiology to therapy and back again

    Directory of Open Access Journals (Sweden)

    Jeong-A eLim

    2014-07-01

    Full Text Available Pompe disease is a lysosomal storage disorder in which acid alpha-glucosidase is deficient or absent. Deficiency of this lysosomal enzyme results in progressive expansion of glycogen-filled lysosomes in multiple tissues, with cardiac and skeletal muscle being the most severely affected. The clinical spectrum ranges from fatal hypertrophic cardiomyopathy and skeletal muscle myopathy in infants to relatively attenuated forms, which manifest as a progressive myopathy without cardiac involvement. The currently available enzyme replacement therapy proved to be successful in reversing cardiac but not skeletal muscle abnormalities. Although the overall understanding of the disease has progressed, the pathophysiology of muscle damage remains poorly understood. Lysosomal enlargement/rupture has long been considered a mechanism of relentless muscle damage in Pompe disease. In past years, it became clear that this simple view of the pathology is inadequate; the pathological cascade involves dysfunctional autophagy, a major lysosome-dependent intracellular degradative pathway. The autophagic process in Pompe skeletal muscle is affected at the termination stage - impaired autophagosomal-lysosomal fusion. Yet another abnormality in the diseased muscle is the accelerated production of large, unrelated to ageing, lipofuscin deposits - a marker of cellular oxidative damage and a sign of mitochondrial dysfunction. The massive autophagic buildup and lipofuscin inclusions appear to cause a greater effect on muscle architecture than the enlarged lysosomes outside the autophagic regions. Furthermore, the dysfunctional autophagy affects the trafficking of the replacement enzyme and interferes with its delivery to the lysosomes. Several new therapeutic approaches have been tested in Pompe mouse models: substrate reduction therapy, lysosomal exocytosis following the overexpression of transcription factor EB and a closely related but distinct factor E3, and genetic

  9. Extraterrestrial Amino Acids in the Almahata Sitta Meteorite

    Science.gov (United States)

    Glavin, Daniel P.; Aubrey, Andrew D.; Callahan, Michael P.; Dworkin, Jason P.; Elsila, Jamie E.; Parker, Eric T.; Bada, Jeffrey L.

    2010-01-01

    Amino acid analysis of a meteorite fragment of asteroid 2008 TC3 called Almahata Sitta was carried out using reverse-phase liquid chromatography coupled with UV fluorescence detection and time-of-flight mass spectrometry (LC-FD/ToF-MS) as part of a sample analysis consortium. LC-FD/ToF-MS analyses of hot-water extracts from the meteorite revealed a complex distribution of two- to seven-carbon aliphatic amino acids and one- to three-carbon amines with abundances ranging from 0.5 to 149 parts-per-billion (ppb). The enantiomeric ratios of the amino acids alanine, R-amino-n-butyric acid (beta-ABA), 2-amino-2-methylbutanoic acid (isovaline), and 2-aminopentanoic acid (norvaline) in the meteorite were racemic (D/L approximately 1), indicating that these amino acids are indigenous to the meteorite and not terrestrial contaminants. Several other non-protein amino acids were also identified in the meteorite above background levels including alpha-aminoisobutyric acid (alpha-AIB), 4-amino-2- methylbutanoic acid, 4-amino-3-methylbutanoic acid, and 3-, 4-, and 5-aminopentanoic acid. The total abundances of isovaline and alpha-AIB in Almahata Sitta are 1000 times lower than the abundances of these amino acids found in the CM carbonaceous chondrite Murchison. The extremely low abundances and unusual distribution of five carbon amino acids in Almahata Sitta compared to Cl, CM, and CR carbonaceous chondrites may reflect extensive thermal alteration of amino acids on the parent asteroid by partial melting during formation or subsequent impact shock heating. It is also possible that amino acids were synthesized by catalytic reactions on the parent body after asteroid 2008 TC3 cooled to lower temperatures.

  10. Dysregulation of soluble epoxide hydrolase and lipidomic profiles in anorexia nervosa.

    Science.gov (United States)

    Shih, P B; Yang, J; Morisseau, C; German, J B; Zeeland, A A Scott-Van; Armando, A M; Quehenberger, O; Bergen, A W; Magistretti, P; Berrettini, W; Halmi, K A; Schork, N; Hammock, B D; Kaye, W

    2016-04-01

    Individuals with anorexia nervosa (AN) restrict eating and become emaciated. They tend to have an aversion to foods rich in fat. Because epoxide hydrolase 2 (EPHX2) was identified as a novel AN susceptibility gene, and because its protein product, soluble epoxide hydrolase (sEH), converts bioactive epoxides of polyunsaturated fatty acid (PUFA) to the corresponding diols, lipidomic and metabolomic targets of EPHX2 were assessed to evaluate the biological functions of EPHX2 and their role in AN. Epoxide substrates of sEH and associated oxylipins were measured in ill AN, recovered AN and gender- and race-matched controls. PUFA and oxylipin markers were tested as potential biomarkers for AN. Oxylipin ratios were calculated as proxy markers of in vivo sEH activity. Several free- and total PUFAs were associated with AN diagnosis and with AN recovery. AN displayed elevated n-3 PUFAs and may differ from controls in PUFA elongation and desaturation processes. Cytochrome P450 pathway oxylipins from arachidonic acid, linoleic acid, alpha-linolenic acid and docosahexaenoic acid PUFAs are associated with AN diagnosis. The diol:epoxide ratios suggest the sEH activity is higher in AN compared with controls. Multivariate analysis illustrates normalization of lipidomic profiles in recovered ANs. EPHX2 influences AN risk through in vivo interaction with dietary PUFAs. PUFA composition and concentrations as well as sEH activity may contribute to the pathogenesis and prognosis of AN. Our data support the involvement of EPHX2-associated lipidomic and oxylipin dysregulations in AN, and reveal their potential as biomarkers to assess responsiveness to future intervention or treatment. PMID:25824304

  11. Short communication. N-(2-chloro-4-pyridyl)-N-phenylurea (4-CPPU) enhances in vitro direct shoot organogenesis of Citrus aurantium L. epicotyl segments compared to other commonly used cytokinins

    Energy Technology Data Exchange (ETDEWEB)

    Roussos, P. A.; Dimitriou, G.; Voloudakis, A. E.

    2011-07-01

    The effect of three concentrations of five different cytokinins, i.e. 6-benzylamino purine, 2-isopentyl adenine, kinetin (Kin), thidiazuron and N-(2-chloro-4-pyridyl)-N-phenylurea (4-CPPU), was evaluated on the in vitro direct shoot organogenesis of epicotyl explants of sour orange (Citrus aurantium L.). The basal medium used was that of Murashige and Tucker and epicotyl explants were incubated in medium supplemented with the prementioned cytokinins for 45 days. The addition of Kin and 4-CPPU in the medium enhanced the direct shoot organogenesis of sour orange epicotyl segments. The concentration of each of these two cytokinins which gave the best results, was combined with indole-3-acetic acid (IAA) or {alpha}-naphthalene acetic acid ({alpha}-NAA) at concentrations ranging from 0.01 mg L{sup -}1 to 0.2 mg L{sup -}1. The inclusion of IAA at 0.2 mg L{sup -}1 in the medium with 4-CPPU at 0.05 mg L{sup -}1 resulted in 100% successful direct shoot organogenesis, while the combination of Kin at 0.25 mg L{sup -}1 with IAA or {alpha}-NAA each at 0.01 mg L{sup -}1 presented equally high organogenesis percentages (91.7%). The incubation of the produced shoots, in medium supplemented with either indole-3-butyric acid or {alpha}-NAA resulted in high rooting percentages (up to 90%) and the rooted explants were successfully acclimatized under mist (85%). Although 4-CPPU has been used in in vitro culture of various species, this is the first report on its use in the direct shoot organogenesis of citrus species and could be of great value in citrus genetic transformation protocols using epicotyl segments, since this cytokinin resulted in the absolute organogenesis percentage. (Author) 20 refs.

  12. Daphnia HR96 is a promiscuous xenobiotic and endobiotic nuclear receptor

    Energy Technology Data Exchange (ETDEWEB)

    Karimullina, Elina [Environmental Toxicology Program, Clemson University, Clemson, SC 29634 (United States); Institute of Plant and Animal Ecology, Russian Academy of Sciences, Ural Branch, Yekaterinburg 620144 (Russian Federation); Li Yangchun; Ginjupalli, Gautam K. [Environmental Toxicology Program, Clemson University, Clemson, SC 29634 (United States); Baldwin, William S., E-mail: baldwin@clemson.edu [Environmental Toxicology Program, Clemson University, Clemson, SC 29634 (United States); Biological Sciences, Clemson University, Clemson, SC (United States)

    2012-07-15

    Daphnia pulex is the first crustacean to have its genome sequenced. The genome project provides new insight and data into how an aquatic crustacean may respond to environmental stressors, including toxicants. We cloned Daphnia pulex HR96 (DappuHR96), a nuclear receptor orthologous to the CAR/PXR/VDR group of nuclear receptors. In Drosophila melanogaster, (hormone receptor 96) HR96 responds to phenobarbital exposure and has been hypothesized as a toxicant receptor. Therefore, we set up a transactivation assay to test whether DappuHR96 is a promiscuous receptor activated by xenobiotics and endobiotics similar to the constitutive androstane receptor (CAR) and the pregnane X-receptor (PXR). Transactivation assays performed with a GAL4-HR96 chimera demonstrate that HR96 is a promiscuous toxicant receptor activated by a diverse set of chemicals such as pesticides, hormones, and fatty acids. Several environmental toxicants activate HR96 including estradiol, pyriproxyfen, chlorpyrifos, atrazine, and methane arsonate. We also observed repression of HR96 activity by chemicals such as triclosan, androstanol, and fluoxetine. Nearly 50% of the chemicals tested activated or inhibited HR96. Interestingly, unsaturated fatty acids were common activators or inhibitors of HR96 activity, indicating a link between diet and toxicant response. The omega-6 and omega-9 unsaturated fatty acids linoleic and oleic acid activated HR96, but the omega-3 unsaturated fatty acids alpha-linolenic acid and docosahexaenoic acid inhibited HR96, suggesting that these two distinct sets of lipids perform opposing roles in Daphnia physiology. This also provides a putative mechanism by which the ratio of dietary unsaturated fats may affect the ability of an organism to respond to a toxic insult. In summary, HR96 is a promiscuous nuclear receptor activated by numerous endo- and xenobiotics.

  13. Nutritional evaluation of the moonfish Mene maculata (Bloch & Schneider, 1801) from Parangipettai, southeast coast of India

    Institute of Scientific and Technical Information of China (English)

    Palanivel Bharadhirajan; Natarajan Periyasamy; Sambantham Murugan

    2014-01-01

    Objective: To assess the nutritions in Mene maculata (Bloch & Schneider, 1801) (M. maculata). Methods: Fishes (14-16 cm) were obtained from the landings at Parangipettai for the evaluation of biochemical composition. The present study deals with biochemical composition such as protein, carbohydrate, lipid, amino acids fatty acids, vitamins and minerals which were evaluated in the moonfish.Results:protein was high in the tissue (23.16%), followed by the carbohydrate (1.3%) and lipid (2.62%). Totally 20 essential and nonessential amino acids were present at the rate of 46.72% and 43.91%. In the analysis, the fatty acid profile by gas chromatography revealed the presence of higher amount of saturated fatty acid (palmitic acid 22.17%) than monounsaturated fatty acid (oleic acid 14.51%) and polyunsaturated fatty acid (alpha linolenic acid 16.07%). Vitamins were detected in M. maculata. Among them, vitamin A was found in higher levels (124.5 mg/g), whereas vitamin B6 was noticed as lower levels (0.34 mg/g). In the present study, totally 5 macro minerals and 2 trace minerals were reported. The macro mineral calcium (156.7 mg/g) was found at the highest level and other minerals such as sodium (31.98 mg/g), potassium (21.33 mg/g), copper (1.43 mg/g) and magnesium (0.341 mg/g) were also detected in the moonfish.Conclusions:The results of proximate composition in M. maculata showed that the percentage of The result showed that the moonfish M. maculata tissue is a valuable food recipe for human consumption, due to its high quality protein and well-balanced amino acids.

  14. Regulation of valine and. alpha. -ketoisocaproate metabolism in rat kidney mitochondria

    Energy Technology Data Exchange (ETDEWEB)

    Miller, R.H.; Harper, A.E. (Univ. of Wisconsin, Madison (USA))

    1988-10-01

    Activities of branched-chain amino acid (BCAA) aminotransferase (BCAT) and {alpha}-keto acid dehydrogenase (BCKD) were assayed in mitochondria isolated from kidneys of rats. Rates of transamination of valine and oxidation of keto acids {alpha}-ketoisocaproate (KIC) or {alpha}-ketoisovalerate (KIV) were estimated using radioactive tracers of the appropriate substrate from amounts of {sup 14}C-labeled products formed. Because of the high mitochondrial BCAT activity, an amino acceptor for BCAT, {alpha}-ketoglutarate ({alpha}-KG) or KIC, was added to the assay medium when valine was the substrate. Rates of valine transamination and subsequent oxidation of the KIV formed were determined with 0.5 mM {alpha}-KG as the amino acceptor; these rates were 5- to 50-fold those without added {alpha}-KG. Rates of CO{sub 2} evolution from valine also increased when KIC was present; however, with KIC concentrations above 0.2 mM, rates of CO{sub 2} evolution from valine declined although rates of transamination continued to rise. When 0.05 mM KIC was added to the assay medium, oxidation of KIC was suppressed by inclusion of valine or glutamate in the medium. When valine was present KIC was not oxidized preferentially, presumably because it was also serving as an amino acceptor for BCAT. These results indicate that as the supply of amino acceptor, {alpha}-KG or KIC, is increased in mitochondria not only is the rate of valine transamination stimulated but also the rate of oxidation of the KIV formed from valine. Thus the rate of oxidation of BCAA can be controlled by factors that influence the rate and direction of BCAA transamination and, thereby, the supply of substrate for BCKD.

  15. Shifts in striatal responsivity evoked by chronic stimulation of dopamine and glutamate systems.

    Science.gov (United States)

    Canales, J J; Capper-Loup, C; Hu, D; Choe, E S; Upadhyay, U; Graybiel, A M

    2002-10-01

    Dopamine and glutamate are key neurotransmitters in cortico-basal ganglia loops affecting motor and cognitive function. To examine functional convergence of dopamine and glutamate neurotransmitter systems in the basal ganglia, we evaluated the long-term effects of chronic stimulation of each of these systems on striatal responses to stimulation of the other. First we exposed rats to chronic intermittent cocaine and used early-gene assays to test the responsivity of the striatum to subsequent acute motor cortex stimulation by application of the GABA(A) (gamma-aminobutyric acid alpha subunit) receptor antagonist, picrotoxin. Reciprocally, we studied the effects of chronic intermittent motor cortex stimulation on the capacity for subsequent acute dopaminergic treatments to induce early-gene activation in the striatum. Prior treatment with chronic intermittent cocaine induced motor sensitization and significantly potentiated the striatal expression of Fos-family early genes in response to stimulation of the motor cortex. Contrary to this, chronic intermittent stimulation of the motor cortex down-regulated cocaine-induced gene expression in the striatum, but enhanced striatal gene expression induced by a full D1 receptor agonist (SKF 81297) and did not change the early-gene response elicited by a D2 receptor antagonist (haloperidol). These findings suggests that repeated dopaminergic stimulation produces long-term enhancement of corticostriatal signalling from the motor cortex, amplifying cortically evoked modulation of the basal ganglia. By contrast, persistent stimulation of the motor cortex inhibits cocaine-stimulated signalling in the striatum, but not signalling mediated by individual dopamine receptor sites, suggesting that chronic cortical hyperexcitability produces long-term impairment of dopaminergic activity and compensation at the receptor level. These findings prompt a model of the basal ganglia function as being regulated by opposing homeostatic dopamine

  16. Early administration of enzyme replacement therapy for Pompe disease: short-term follow-up results.

    Science.gov (United States)

    Hamdan, M A; Almalik, M H; Mirghani, H M

    2008-12-01

    Pompe disease (glycogen storage disease II, OMIM # 232300), is a hereditary lysosomal disorder. It is characterized by deficiency of acid alpha-glucosidase enzyme (acid maltase, GAA, OMIM *606800, EC 3.1.26.2), secondary to mutations in the GAA gene (HGNC:4065) on chromosome 17q25.2-q25.3. Absent enzyme activity in the infantile form of Pompe disease results in abnormal glycogen deposition in the skeletal, cardiac, and smooth muscles, leading to hypertrophic cardiomyopathy, feeding abnormalities, hypotonia, weakness, respiratory insufficiency, and ultimately death. Prenatal diagnosis is accomplished by enzyme assay, mutation analysis or electron microscopy of amniotic fluid cells or chorionic villus sample. However, these techniques may not always be available, and can result in perinatal morbidity and fetal loss. Early diagnosis of Pompe disease results in early institution of enzyme replacement therapy (ERT), which minimizes morbidity and prolongs survival. We report the case of a 35-week part-of-twin neonate, whose older sibling died earlier because of infantile Pompe disease. At 32 weeks of gestation, fetal echocardiography showed hypertrophic cardiomyopathy in twin 1, which persisted until birth at 35 weeks of gestation. Diagnosis was confirmed after birth by enzyme assay, and mutation analysis showing homozygosity for the sequence change 1327-2A>G (GAA intr 8). Administration of ERT at 18 h of age, resulted in normalization of cardiac abnormalities within 21 weeks of therapy, and normal neurodevelopmental assessment at 46 weeks, using Griffiths Mental Development Scales. To our knowledge, this is the youngest patient reported to receive ERT for Pompe disease, and the first report of prenatal diagnosis of Pompe disease by fetal echocardiography. PMID:19067231

  17. Radioisotopic method for the measurement of lipolysis in small samples of human adipose tissue

    International Nuclear Information System (INIS)

    To facilitate the study of adrenoreceptor response in small needle biopsy samples of human subcutaneous adipose tissue, we developed a dual radioisotopic technique for measuring lipolysis rate. Aliquots (20-75 mg) of adipose tissue fragments were incubated in a buffered albumin medium containing [3H]palmitate and [14C]glucose, each of high specific activity. In neutral glycerides synthesized in this system, [14C]glucose is incorporated exclusively into the glyceride-glycerol moiety and 3H appears solely in the esterified fatty acid. Alpha-2 and beta-1 adrenoreceptor activation of tissue incubated in this system does not alter rates of 14C-labeled glyceride accumulation, but does produce a respective increase or decrease in the specific activity of fatty acids esterified into newly synthesized glycerides. This alteration in esterified fatty acid specific activity is reflected in the ratio of 14C:3H in newly synthesized triglycerides extracted from the incubated adipose tissue. There is a high correlation (r . 0.90) between the 14C:3H ratio in triglycerides and the rate of lipolysis as reflected in glycerol release into the incubation medium. The degree of adrenoreceptor activation by various concentrations of lipolytic and anti-lipolytic substances can be assessed by comparing this ratio in stimulated tissue to that characterizing unstimulated tissue or the incubation medium. This technique permits the study of very small, unweighed tissue biopsy fragments, the only limitation on sensitivity being the specific activity of the medium glucose and palmitate. It is, therefore, useful for serial examinations of adipose tissue adrenoreceptor dose-response characteristics under a variety of clinical circumstances

  18. Soy foods, isoflavones, and the health of postmenopausal women.

    Science.gov (United States)

    Messina, Mark

    2014-07-01

    Over the past 2 decades, soy foods have been the subject of a vast amount of research, primarily because they are uniquely rich sources of isoflavones. Isoflavones are classified as both phytoestrogens and selective estrogen receptor modulators. The phytoestrogenic effects of isoflavones have led some to view soy foods and isoflavone supplements as alternatives to conventional hormone therapy. However, clinical research shows that isoflavones and estrogen exert differing effects on a variety of health outcomes. Nevertheless, there is substantial evidence that soy foods have the potential to address several conditions and diseases associated with the menopausal transition. For example, data suggest that soy foods can potentially reduce ischemic heart disease through multiple mechanisms. Soy protein directly lowers blood low-density lipoprotein-cholesterol concentrations, and the soybean is low in saturated fat and a source of both essential fatty acids, the omega-6 fatty acid linoleic acid and the omega-3 fatty acid alpha-linolenic acid. In addition, soflavones improve endothelial function and possibly slow the progression of subclinical atherosclerosis. Isoflavone supplements also consistently alleviate menopausal hot flashes provided they contain sufficient amounts of the predominant soybean isoflavone genistein. In contrast, the evidence that isoflavones reduce bone loss in postmenopausal women is unimpressive. Whether adult soy food intake reduces breast cancer risk is unclear. Considerable evidence suggests that for soy to reduce risk, consumption during childhood and/or adolescence is required. Although concerns have been raised that soy food consumption may be harmful to breast cancer patients, an analysis in 9514 breast cancer survivors who were followed for 7.4 y found that higher postdiagnosis soy intake was associated with a significant 25% reduction in tumor recurrence. In summary, the clinical and epidemiologic data indicate that adding soy foods to the

  19. NUTRITIONAL STATUS OF SUBJECTS WITH DOMINANT PLANT FOOD CONSUMPTION

    Directory of Open Access Journals (Sweden)

    Viera Pauková

    2011-07-01

    Full Text Available In three groups of apparently healthy subjects – vegetarians (plant food, dairy products, eggs, semi-vegetarians (as vegetarians with addition of white meat consumption and non-vegetarians (control group on traditional mixed diet were analyzed the dietary questionnaires of consumption frequency  and measured the values of lipid profile, insulin resistance, homocysteine with determinants (vitamins B6, B9, B12 and plasma antioxidative vitamins (C,E, beta-carotene. Vegetarians and semi-vegetarians consumed the significantly reduced amount of cholesterol, saturated fatty acids, methionine, lysine, vitamin B12 and on the other hand, they have the significantly higher daily intake of polyunsaturated fatty acids, linoleic acid, alpha-linolenic acid, fiber, plant proteins, arginine, glycine, serine, alanine, folic acid (vitamin B9, vitamin B6, vitamins C,E and beta-carotene. Alternative nutrition groups vs. non-vegetarians have the significantly reduced concentrations of total and LDL-cholesterol, triacylglycerols, insulin as well as values of atherogenic index and insulin resistance. The vegetarian (but not semi-vegetarian value of homocysteine is significantly increased as a consequence of the significantly reduced and low concentration of vitamin B12. Other two determinants of homocysteine degradation were significantly increased in serum of alternative nutrition groups. The both vegetarian groups have the significantly higher plasma concentrations of antioxidative vitamins and these values are in range of effective free radical disease reduction. The results  of favourable values of cardiovascular risk markers and antioxidants document a beneficial effect of vegetarian nutrition in prevention of degenerative age-related diseases. doi:10.5219/148

  20. The effect of Korean pine nut oil on in vitro CCK release, on appetite sensations and on gut hormones in post-menopausal overweight women

    Directory of Open Access Journals (Sweden)

    Hendriks Henk FJ

    2008-03-01

    Full Text Available Abstract Appetite suppressants may be one strategy in the fight against obesity. This study evaluated whether Korean pine nut free fatty acids (FFA and triglycerides (TG work as an appetite suppressant. Korean pine nut FFA were evaluated in STC-1 cell culture for their ability to increase cholecystokinin (CCK-8 secretion vs. several other dietary fatty acids from Italian stone pine nut fatty acids, oleic acid, linoleic acid, alpha-linolenic acid, and capric acid used as a control. At 50 μM concentration, Korean pine nut FFA produced the greatest amount of CCK-8 release (493 pg/ml relative to the other fatty acids and control (46 pg/ml. A randomized, placebo-controlled, double-blind cross-over trial including 18 overweight post-menopausal women was performed. Subjects received capsules with 3 g Korean pine (Pinus koraiensis nut FFA, 3 g pine nut TG or 3 g placebo (olive oil in combination with a light breakfast. At 0, 30, 60, 90, 120, 180 and 240 minutes the gut hormones cholecystokinin (CCK-8, glucagon like peptide-1 (GLP-1, peptide YY (PYY and ghrelin, and appetite sensations were measured. A wash-out period of one week separated each intervention day. CCK-8 was higher 30 min after pine nut FFA and 60 min after pine nut TG when compared to placebo (p This study suggests that Korean pine nut may work as an appetite suppressant through an increasing effect on satiety hormones and a reduced prospective food intake.

  1. The Ocean supplies more EPA and DHA than we can use

    Directory of Open Access Journals (Sweden)

    Ackman R.G.

    2004-03-01

    Full Text Available Nutraceutical is a modern marketing term and a source of much confusion, nowhere more obviously than in the field of polyunsaturated fatty acids. The world supply of vegetable oils includes an abundance of an unsaturated and omega 6 (or n-6 C18 fatty acid, linoleic, which has been declared for decades as “essential” for elongation to the C20 trienoic and tetraenoic n-6 fatty acids vital to the functioning of our body systems. It also keeps most of the vegetable oils liquid or semi-liquid. A frequent minor partner fatty acid is the C18 omega 3 fatty acid alpha-linolenic (18:3n-3 which was sensitive to oxidation and therefore a nuisance in salad and frying oils and margarines. Wherever possible, it was reduced or eliminated. Within the last two decades we have realized that our conversion of 18:3n-3 to the “truly essential” 20:5n-3 (EPA and especially 22:6n-3 (DHA, via 22:5n-3 (DPA, is poor, and we have depended on DHA from eggs and certain animal meats. Fish and shellfish for both were the most useful sources in our diets but were not recognized officially until 1996. Then U.K. Report No. 46 recommended eating fish twice a week, one being oily. More recently the American Heart Association has followed suit. This review shows how the microalgae of the oceans produce and concentrate generous supplies of these two highly desirable dietary fatty acids available from a truly “functional” food¡

  2. Rapid separation of lanthanides and actinides on small particle based reverse phase supports

    Energy Technology Data Exchange (ETDEWEB)

    Datta, A.; Sivaraman, N.; Srinivasan, T.G.; Vasudeva Rao, P.R. [Chemistry Group, Indira Gandhi Centre for Atomic Research, Kalpakkam (India)

    2010-07-01

    This paper presents the results on the use of short columns (3-5 cm long) with small particle size (1.8 {mu}m) for high performance liquid chromatographic separation of individual lanthanides and uranium from plutonium as well as uranium from thorium to achieve rapid separations i.e. separation time as short as 3.6 min for individual lanthanides, 1 min for thorium-uranium and 4.2 min for uranium from plutonium. These advantages can be exploited to significantly reduce analysis time, liquid waste generation as well as dose to operator when radioactive samples are analysed e.g. burn-up determination. In the present work, a dynamic ion-exchange chromatographic separation technique was employed using camphor-10-sulfonic acid (CSA) as the ion-pairing reagent and {alpha}-hydroxy isobutyric acid ({alpha}-HIBA) as the complexing reagent for the isolation of individual lanthanides as well as the separation of uranium from thorium. Uranium was separated from Pu(III) as well as Pu(IV) by reverse phase HPLC technique. The reverse phase HPLC was also investigated for the isolation and quantitative determination of uranium from thorium as well as lanthanide group from uranium. The dynamic ion-exchange technique using small particle support was demonstrated for measuring the concentrations of lanthanide fission products such as La, Ce, Pr, Nd and Sm in the dissolver solution of fast reactor fuel. Similarly, the assay of uranium in the dissolver solution of fast reactor was carried out using reverse phase HPLC technique. The rapid separation technique using reverse phase HPLC was also demonstrated for separation of lanthanides as a group from uranium matrix; samples of LiCl-KCl eutectic salt containing chlorides of lanthanides in uranium matrix (typically 1: 2000) were analysed. (orig.)

  3. Correlation of retention of lanthanide and actinide complexes with stability constants and their speciation

    Energy Technology Data Exchange (ETDEWEB)

    Datta, A.; Sivaraman, N.; Viswanathan, K.S.; Ghosh, Suddhasattwa; Srinivasan, T.G.; Vasudeva Rao, P.R. [Indira Gandhi Centre for Atomic Research, Kalpakkam (India). Chemistry Group

    2013-03-01

    The present study describes a correlation that is developed from retention of lanthanide and actinide complexes with the stability constant. In these studies, an ion-pairing reagent, camphor-10-sulphonic acid (CSA) was used as the modifier and organic acids such as {alpha}-hydroxy isobutyric acid ({alpha}-HIBA), mandelic acid, lactic acid and tartaric acid were used as complexing reagent for elution. From these studies, a correlation has been established between capacity factor of a metal ion, concentration of ion-pairing reagent and complexing agent with the stability constant of metal complex. Based on these studies, it has been shown that the stability constant of lanthanide and actinide complexes can be estimated using a single lanthanide calibrant. Validation of the method was carried out with the complexing agents such as {alpha}-HIBA and lactic acid. It was also demonstrated that data from a single chromatogram can be used for estimation of stability constant at various ionic strengths. These studies also demonstrated that the method can be applied for estimation of stability constant of actinides with a ligand whose value is not reported yet, e.g., ligands of importance in the lanthanide-actinide separations, chelation therapy etc. The chromatographic separation method is fast and the estimation of stability constant can be done in a very short time, which is a significant advantage especially in dealing with radioactive elements. The stability constant data was used to derive speciation data of plutonium in different oxidation states as well as that of americium with {alpha}-HIBA. The elution behavior of actinides such as Pu and Am from reversed phase chromatographic technique could be explained based on these studies. (orig.)

  4. Evidence for the importance of 5'-deoxy-5-fluorouridine catabolism in humans from 19F nuclear magnetic resonance spectrometry.

    Science.gov (United States)

    Malet-Martino, M C; Armand, J P; Lopez, A; Bernadou, J; Béteille, J P; Bon, M; Martino, R

    1986-04-01

    The use of a new methodology, 19F nuclear magnetic resonance, has allowed detection of all the fluorinated metabolites in the biofluids of patients treated with 5'-deoxy-5-fluorouridine (5'-dFUrd) injected i.v. at a dose of 10 g/m2 over 6 h. This technique, which requires no labeled drug, allows a direct study of the biological sample with no need for extraction or derivatization and a simultaneous identification and quantitation of all the different fluorinated metabolites. As well as the already known metabolites, unmetabolized 5'-dFUrd, 5-fluorouracil, and 5,6-dihydro-5-fluorouracil, the presence of alpha-fluoro-beta-ureidopropionic acid, alpha-fluoro-beta-alanine (FBAL), N-carboxy-alpha-fluoro-beta-alanine, and the fluoride anion F- is reported. The catabolic pathway proposed for 5'-dFUrd is analogous to that of 5-fluorouracil, completed with FBAL----F- step, and the plasmatic equilibrium of FBAL with N-carboxy-alpha-fluoro-beta-alanine, its N-carboxy derivative. The quantitative analysis of the different metabolites found in plasma and urine emphasizes the significance of the catabolic pathway. High concentrations of alpha-fluoro-beta ureidopropionic acid and FBAL are recovered in plasma from 3 h after the beginning of the perfusion to 1 h after its end. The global urinary excretion results show that there is a high excretion of 5'-dFUrd and metabolites. Unchanged 5'-dFUrd and FBAL are by far the major excretory products and are at nearly equal rates. The protocol followed in this study produces relatively low but persistent plasmatic concentrations of 5-fluorouracil throughout the perfusion. PMID:2936452

  5. Effect of an antioxidant combination on the distribution of acetylcholinesterase and adenosine triphosphatase activities in the cerebellum of in lindane-intoxicated mice

    Directory of Open Access Journals (Sweden)

    Devendra Kumar Bhatt

    2013-04-01

    Full Text Available Objective: The present investigation ascertains a protective potential of a combination of antioxidants against lindane-induced toxicity in cerebellum of mice. Methods: For the study, animals are divided into four groups. First group is control and it is given only vehicle. Second group is treated with lindane and analysed if there are any lesions in the brain. Third group is purely antioxidants treated group; four antioxidants, i.e. resveratrol, ascorbic acid, alpha lipoic acid and vitamin E, are subcutaneously administered in a suitable combination to the animals of this group. Fourth group is treated with both lindane and antioxidants. Acetylcholinesterase (AChE and adenosine triphosphatase (ATPase activities are used as histochemical markers for manifestation of lindane-induced acute toxicity. Biochemical levels of glutathione (GSH and thiobarbituric acid reactive substances (TBARS were also evaluated for different groups to confirm the toxicity of lindane in cerebellum. Results: Treatment with lindane caused decrease in AChE and ATPase activities, and GSH levels in cerebellum whereas a significant increase was recorded in TBARS. Antioxidants treatment increased the enzymatic activities. A significant rise in GSH level was recorded in the antioxidant treatment group as compared to group I and group II whereas TBARS levels were significantly reduced. GSH and TBARS levels altered significantly in group IV as compared to control group and lindane-treated group. In group III, AChE and ATPase activities increased in layers and nuclei of cerebellum as compared to control group. Conclusions: Since the use of antioxidants prevents the inhibition of AChE and ATPase, functions which are altered due to lindane-toxicity may be protected. [J Exp Integr Med 2013; 3(2.000: 103-112

  6. Surface Structure and Chemical Switching of Thioctic Acid Adsorbed on Au(111) as Observed Using Near-Edge X-ray Absorption Fine Structure

    Energy Technology Data Exchange (ETDEWEB)

    Meulenberg, R W; van Buuren, T; Vance, A L; Terminello, L J; Willey, T M; Bostedt, C; Fadley, C S

    2004-01-06

    Thioctic acid (alpha-lipoic acid) is a molecule with a large disulfide-containing base, a short alkyl-chain with four CH{sub 2} units, and a carboxyl termination. Self-assembled monolayer (SAM) films of thioctic acid adsorbed on Au(111) have been investigated with near-edge x-ray absorption fine structure (NEXAFS) spectroscopy and x-ray photoelectron spectroscopy (XPS) to determine film quality, bonding and morphology. Using standard preparation protocols for SAMs, that is, dissolving thioctic acid in ethanol and exposing gold to the solution, results in poor films. These films are highly disordered, contain a mixture of carboxyl and carboxylate terminations, have more than monolayer coverage, and exhibit unbound disulfide. Conversely, forming films by dissolving 1 mmol thioctic acid into 5% acetic acid in ethanol (as previously reported with carboxyl-terminated alkyl-thiols) forms ordered monolayers with small amounts of unbound sulfur. NEXAFS indicates tilted over endgroups with the carboxyl group normal on average 38{sup o} from the surface normal. Slight dichroism in other features indicates alkyl chains statistically more upright than prostrate on the surface. Reflection-absorption Fourier transform infrared (RA-FTIR) spectra indicate hydrogen bonding between neighboring molecules. In such well-formed monolayers, a stark reorientation occurs upon deprotonation of the endgroup by rinsing in a KOH solution. The carboxylate plane normal is now about 66{sup o} from sample normal, a much more upright orientation. Data indicate this reorientation may also cause a more upright orientation to the alkyl portion of the molecules.

  7. The effectiveness of evening primrose oil and alpha lipoic acid in recovery of nerve function in diabetic rats

    Directory of Open Access Journals (Sweden)

    Abeer Mohamed Rashad

    2011-09-01

    Full Text Available Objectives: Diabetic polyneuropathy is a serious complication of diabetes mellitus and the most frequent neuropathy worldwide. Evening primrose oil (EPO is rich in omega-6 essential fatty acid component and gamma-linolenic acid. Alpha lipoic acid (ALPA has a protective effect against lipid peroxidation and helps in scavenging free radicals. Data regarding the effect of treatment with EPO on diabetic parameters and neuropathic manifestations are conflicting. This study aimed to determine the therapeutic efficacy of EPO and ALPA in correcting diabetic parameters and functional and structural neuropathic manifestations in streptozotocin (STZ induced diabetic rats.Materials and methods: In this study, the effects of two week oral treatment with EPO (1.25 g/kg was compared to that of ALPA (100 mg/kg and insulin (2 IU/day, utilized singly or in combination.Results: Compared with untreated diabetic rats, EPO and ALPA resulted in reduction of serum levels of glucose (p<0.05, total cholesterol (p<0.01, triglycerides (p<0.01, low density lipoprotein cholesterol (p<0.01, thiobarbituric acid reactive substance (a marker of oxidative stress (p<0.05, and increased in levels of high density lipoprotein cholesterol (p<0.05 and total antioxidant capacity (p<0.05. Enhanced positive effect was observed with combination therapy.Conclusion: This work indicates that EPO and ALPA, particularly when used in combination, improve glycemic control, lipid abnormalities and antioxidant capacity, thus restore the impaired functional properties of peripheral nerves to a great extent. J Clin Exp Invest 2011; 2 (3: 245-253.

  8. Nuclear medicine program. Progress report for quarter ending June 30, 1995

    Energy Technology Data Exchange (ETDEWEB)

    Knapp, F.F. Jr.; Ambrose, K.R.; Beets, A.L. [and others

    1995-09-01

    In this report we describe the first synthesis of the (-)(-) and (-)(+) isomers of 1-azabicyclo oct-3-yl {alpha}-(1-fluoropent-5-yl)-{alpha}-hydroxy-{alpha}-phenylacetate ({open_quotes}FQNPe{close_quotes}). Earlier studies with the racemic FQNPe mixture had demonstrated high in vitro binding affinity for the muscarinic-cholinergic receptor and showed that pre-treatment of rats with this new agent significantly blocked receptor localization of subsequently injected -Z-(-,-)-IQNP. Because of the potential important use of fluorine-18-labeled analogues for clinical evaluation of changes in muscarinic-cholinergic receptors by positron emission tomography (PET), we have now synthesized the diastereomeric isomers of FQNPe. Multi-gram quantities of ethyl-{alpha}- (1-chloropent-5-yl)-{alpha}-hydroxy-{alpha}-phenylacetate were prepared and then saponified into the racemic {alpha}-(1-chloropent-5-yl)-{alpha}-hydroxy-{alpha}-phenylacetic acid mixture. The racemic acid was resolved into (-)- and (+)-{alpha}-(1-chloropent-5-yl)-{alpha}-hydroxy-{alpha}-phenylacetic acid enantiomers by isolation of the (-) salt of (S-)-(-)-{alpha}-methylbenzylamine and the (+) salt of (R)-(+)-{alpha}-methylbenzylamine. The resolved (-)- ([{alpha}]{sub D} = -12.1{degrees}, c = 5.8, chloroform) and (+)-acetic acids ([{alpha}]{sub D} = + 11.6{degrees}, c = 6.0, chloroform) were fully characterized and then converted to the enantiomeric ethyl-{alpha}-(1-fluoropent-5-yl)-{alpha}-hydroxy-{alpha}-phenylacetates by a four-step reaction sequence. The (-)- and (+)-ethyl-{alpha}-(1-fluoropent-5-yl)-{alpha}-hydroxy-{alpha}-phenylacetates were then each transesterified with (-)-quinuclidinol to form the (-)(-) FQNPe and (-)(+) FQNPe diastereomers. These diastereomeric esters will now be evaluated in in vitro studies. The availability of the substrates for preparation of the fluorine-18-labeled enantiomers will now allow evaluation of the radiolabeled compounds in animals.

  9. Nutritional evaluation of the moonfish Mene maculata (Bloch & Schneider, 1801 from Parangipettai, southeast coast of India

    Directory of Open Access Journals (Sweden)

    Palanivel Bharadhirajan

    2014-01-01

    Full Text Available Objective: To assess the nutritions in Mene maculata (Bloch & Schneider, 1801 (M. maculata. Methods: Fishes (14-16 cm were obtained from the landings at Parangipettai for the evaluation of biochemical composition. The present study deals with biochemical composition such as protein, carbohydrate, lipid, amino acids fatty acids, vitamins and minerals which were evaluated in the moonfish. Results: The results of proximate composition in M. maculata showed that the percentage of protein was high in the tissue (23.16%, followed by the carbohydrate (1.3% and lipid (2.62%. Totally 20 essential and nonessential amino acids were present at the rate of 46.72% and 43.91%. In the analysis, the fatty acid profile by gas chromatography revealed the presence of higher amount of saturated fatty acid (palmitic acid 22.17% than monounsaturated fatty acid (oleic acid 14.51% and polyunsaturated fatty acid (alpha linolenic acid 16.07%. Vitamins were detected in M. maculata. Among them, vitamin A was found in higher levels (124.5 mg/g, whereas vitamin B6 was noticed as lower levels (0.34 mg/g. In the present study, totally 5 macro minerals and 2 trace minerals were reported. The macro mineral calcium (156.7 mg/g was found at the highest level and other minerals such as sodium (31.98 mg/g, potassium (21.33 mg/g, copper (1.43 mg/g and magnesium (0.341 mg/g were also detected in the moonfish. Conclusions: The result showed that the moonfish M. maculata tissue is a valuable food recipe for human consumption, due to its high quality protein and well-balanced amino acids.

  10. Daphnia HR96 is a promiscuous xenobiotic and endobiotic nuclear receptor

    International Nuclear Information System (INIS)

    Daphnia pulex is the first crustacean to have its genome sequenced. The genome project provides new insight and data into how an aquatic crustacean may respond to environmental stressors, including toxicants. We cloned Daphnia pulex HR96 (DappuHR96), a nuclear receptor orthologous to the CAR/PXR/VDR group of nuclear receptors. In Drosophila melanogaster, (hormone receptor 96) HR96 responds to phenobarbital exposure and has been hypothesized as a toxicant receptor. Therefore, we set up a transactivation assay to test whether DappuHR96 is a promiscuous receptor activated by xenobiotics and endobiotics similar to the constitutive androstane receptor (CAR) and the pregnane X-receptor (PXR). Transactivation assays performed with a GAL4-HR96 chimera demonstrate that HR96 is a promiscuous toxicant receptor activated by a diverse set of chemicals such as pesticides, hormones, and fatty acids. Several environmental toxicants activate HR96 including estradiol, pyriproxyfen, chlorpyrifos, atrazine, and methane arsonate. We also observed repression of HR96 activity by chemicals such as triclosan, androstanol, and fluoxetine. Nearly 50% of the chemicals tested activated or inhibited HR96. Interestingly, unsaturated fatty acids were common activators or inhibitors of HR96 activity, indicating a link between diet and toxicant response. The omega-6 and omega-9 unsaturated fatty acids linoleic and oleic acid activated HR96, but the omega-3 unsaturated fatty acids alpha-linolenic acid and docosahexaenoic acid inhibited HR96, suggesting that these two distinct sets of lipids perform opposing roles in Daphnia physiology. This also provides a putative mechanism by which the ratio of dietary unsaturated fats may affect the ability of an organism to respond to a toxic insult. In summary, HR96 is a promiscuous nuclear receptor activated by numerous endo- and xenobiotics.

  11. Exploring triacylglycerol biosynthetic pathway in developing seeds of Chia (Salvia hispanica L.): a transcriptomic approach.

    Science.gov (United States)

    R V, Sreedhar; Kumari, Priya; Rupwate, Sunny D; Rajasekharan, Ram; Srinivasan, Malathi

    2015-01-01

    Chia (Salvia hispanica L.), a member of the mint family (Lamiaceae), is a rediscovered crop with great importance in health and nutrition and is also the highest known terrestrial plant source of heart-healthy omega-3 fatty acid, alpha linolenic acid (ALA). At present, there is no public genomic information or database available for this crop, hindering research on its genetic improvement through genomics-assisted breeding programs. The first comprehensive analysis of the global transcriptome profile of developing Salvia hispanica L. seeds, with special reference to lipid biosynthesis is presented in this study. RNA from five different stages of seed development was extracted and sequenced separately using the Illumina GAIIx platform. De novo assembly of processed reads in the pooled transcriptome using Trinity yielded 76,014 transcripts. The total transcript length was 66,944,462 bases (66.9 Mb), with an average length of approximately 880 bases. In the molecular functions category of Gene Ontology (GO) terms, ATP binding and nucleotide binding were found to be the most abundant and in the biological processes category, the metabolic process and the regulation of transcription-DNA-dependent and oxidation-reduction process were abundant. From the EuKaryotic Orthologous Groups of proteins (KOG) classification, the major category was "Metabolism" (31.97%), of which the most prominent class was 'carbohydrate metabolism and transport' (5.81% of total KOG classifications) followed by 'secondary metabolite biosynthesis transport and catabolism' (5.34%) and 'lipid metabolism' (4.57%). A majority of the candidate genes involved in lipid biosynthesis and oil accumulation were identified. Furthermore, 5596 simple sequence repeats (SSRs) were identified. The transcriptome data was further validated through confirmative PCR and qRT-PCR for select lipid genes. Our study provides insight into the complex transcriptome and will contribute to further genome-wide research and

  12. Inhibitory effect of polyunsaturated fatty acids on apoptosis induced by etoposide, okadaic acid and AraC in Neuro2a cells

    Directory of Open Access Journals (Sweden)

    Tomizawa,Kazuhito

    2007-06-01

    Full Text Available Neuronal apoptosis is involved in neurodegenerative diseases such as Alzheimer's disease and Parkinson.s disease. An efficient means of preventing it remains to be found. Some n-3 polyunsaturated fatty acids (PUFAs such as docosahexaenoic acid (DHA, 22 : 6n-3 and eicosapentaenoic acid (EPA, 20 : 5n-3 have been reported to be protective against the neuronal apoptosis and neuronal degeneration seen after spinal cord injury (SCI [1]. However, it is unclear which kinds of PUFAs have the most potent ability to inhibit neuronal apoptosis and whether the simultaneous treatment of PUFAs inhibits the apoptosis. In the present study, we compared the abilities of various n-3- and n-6- PUFAs to inhibit the apoptosis induced after the administration of different apoptotic inducers, etoposide, okadaic acid, and AraC, in mouse neuroblastoma cells (Neuro2a. Preincubation with DHA (22 : 6n-3, eicosapentaenoic acid (EPA, 20 : 5n-3, alpha-linolenic acid (alpha-LNA, 18 : 3n-3, linoleic acid (LA, 18 : 2n-6, arachidonic acid (AA, 20 : 4n-3, and gamma-linolenic acid (gamma-LNA, 18 : 3n-6 significantly inhibited caspase-3 activity and LDH leakage but simultaneous treatment with the PUFAs had no effect on the apoptosis of Neuro2a cells. There were no significant differences of the anti-apoptotic eff ect among the PUFAs. These results suggest that PUFAs may not be effective for inhibiting neuronal cell death after acute and chronic neurodegenerative disorders. However, dietary supplementation with PUFAs may be beneficial as a potential means to delay the onset of the diseases and/or their rate of progression.

  13. Chiral recognition with enantioselective ion exchangers based on carbamoylated cinchonan derivatives as chiral selectors for the HPLC enantioseparation

    International Nuclear Information System (INIS)

    The high-performance liquid chromatographic (HPLC) separation of enantiomers is preferentially performed using chiral stationary phases (CSPs). If the chiral auxiliary (selector, SO) contains charged or ionizable groups one gets ion exchanger type CSPs which may bind and retain oppositely charged analytes (selectands, SAs). We prepared anion exchanger type CSPs with various quinine and quinidine carbarnates as chiral SOs immobilized either on porous or non-porous silica. These CSPs are able to resolve the enantiomers of a wide spectrum of chiral carboxylic, sulfonic, phosphonic, phosphoric acids and of many other chiral acidic solutes (e.g. N-derivatized alpha-, beta- , gamma-amino acids as 2,4-dinitrophenyl, 3,5-dinitrobenzoyl, benzoyl, acetyl, formyl, t.-butoxycarbonyl, benzyloxycarbonyl, 9-fluorenylmethoxycarbonyl, dansyl amino acids and peptides, alpha-arylalkylcarboxylic acids as profens, alpha-aryloxyalkylcarboxylic acids, alpha-arylthioalkylcarboxylic acids and acidic drugs like etodolac, proglumide, acenocournarol, leucovorin, omeprazole, pantoprazole) employing buffered aqueous mobile phases or non-aqueous mobile phases with buffer dissolved in the organic solvent. The influence of mobile phase parameters and other experimental conditions on retention and enantioselectivity has been evaluated for isocratic and gradient elution techniques, aided by the commercial method development computer software DryLab. Several 'Quantitative Structure-Retention Relationships' (QSRR) have been derived, which allowed prediction of enantioselectivity of new analytes and moreover the optimization of the SO-structure. Spectroscopic investigations as H-NMR, FTIR of certain SO-SA-complexes have been exerted to unveil the mechanism of chiral recognition. (author)

  14. Relations entre acides gras oméga-3, oméga-9, structures et fonctions du cerveau. Le point sur les dernières données. Le coût financier alimentaire des oméga-3 « Je chercherai à connaître les différences qui existent entre les huiles de faine, de colza, d’olive, de noix ». Honoré de Balzac. « Histoire de César Birotteau ».

    Directory of Open Access Journals (Sweden)

    Bourre Jean-Marie

    2003-05-01

    Full Text Available Pour ce qui concerne le cerveau, les acides gras ω3 ont été plus spécialement étudiés que les acides gras ω6 ou ω9. La carence en acide alpha-linolénique (18 :3 ω3 altère la structure et la fonction des membranes, et entraîne de légers dysfonctionnements cérébraux, comme cela a été montré sur les modèles animaux puis chez les nourrissons humains. Les résultats récents ont montré que son déficit alimentaire induit des anomalies plus marquées dans certaines structures cérébrales que dans d’autres, le cortex frontal et l’hypophyse étant les plus touchés. Ces singularités sont accompagnées de perturbations comportementales qui touchent plus particulièrement certains tests (habituation, adaptation à une situation nouvelle. Les anomalies biochimiques et comportementales sont partiellement réversées par une supplémentation avec des phospholipides, notamment extraits d’oeuf enrichis en ω3 ou de cervelle de porc. Une étude effet-dose montre que les phospholipides d’origine animale sont plus efficaces que ceux d’origine végétale pour réverser les conséquences du déficit, dans la mesure, entre autres, où ils apportent les très longues chaînes pré-formées. La déficience en acide alpha-linolénique se traduit au niveau du cortex frontal de rat par des perturbations de la neurotransmission mono-aminergique. Mais ne sont touchées ni la densité ni la fonction des transporteurs de la dopamine. Chez l’animal carencé, la réversibilité dans la neurotransmission dopaminergique (dans le cortex préfrontal, entre autres n’est que partielle après arrêt du déficit alimentaire. La carence en acide alpha-linolénique diminue la perception du plaisir, en altérant légèrement l’efficacité des organes sensoriels et en affectant certaines structures cérébrales. Cette carence modifie le métabolisme énergétique du cerveau en perturbant le transport du glucose. Au cours du vieillissement, la baisse

  15. Chemical reactivity of {alpha}-isosaccharinic acid in heterogeneous alkaline systems

    Energy Technology Data Exchange (ETDEWEB)

    Glaus, M. A.; Loon, L. R. Van

    2008-11-15

    Cellulose degradation under alkaline conditions is of relevance for the mobility of many radionuclides in the near-field of a cementitious repository for radioactive waste, because metal-binding degradation products may be formed. Among these, {alpha}-isosaccharinic acid ({alpha}-ISA) is the strongest complexant. The prediction of the equilibrium concentration of {alpha}-ISA in cement pore water is therefore an important step in the assessment of the influence of cellulose degradation products on the speciation of radionuclides in such environments. The present report focuses on possible chemical transformation reactions of {alpha}-ISA in heterogeneous alkaline model systems containing either Ca(OH){sub 2} or crushed hardened cement paste. The transformation reactions were monitored by measuring the concentration of {alpha}-ISA by high performance anion exchange chromatography and the formation of reaction products by high performance ion exclusion chromatography. The overall loss of organic species from solution was monitored by measuring the concentration of non-purgeable organic carbon. The reactions were examined in diluted and compacted suspensions, either at 25 {sup o}C or 90 {sup o}C, and under anaerobic atmospheres obtained by various methods. It was found that {alpha}-ISA was transformed under all conditions tested to some extent. Reaction products, such as glycolate, formate, lactate and acetate, all compounds with less complexing strength than {alpha}-ISA, were detected. The amount of reaction products identified by the chromatographic technique applied was {approx}50 % of the amount of {alpha}-ISA reacted. Sorption of {alpha}-ISA to Ca(OH){sub 2} contributed only to a minor extent to the loss of {alpha}-ISA from the solution phase. As the most important conclusion of the present work it was demonstrated that the presence of oxidising agents had a distinctive influence on the turnover of {alpha}-ISA. Under aerobic conditions {alpha}-ISA was

  16. Investigation of the separation of scandium and rare earth elements from red mud by use of reversed-phase HPLC.

    Science.gov (United States)

    Tsakanika, Lambrini V; Ochsenkühn-Petropoulou, Maria Th; Mendrinos, Leonidas N

    2004-07-01

    A chromatographic method has been developed for separation and determination of scandium (Sc) and rare earth elements (REEs) in samples from a red mud (RM)-utilization process. Reversed-phase high-performance liquid chromatography (RP-HPLC) with post-column derivatization using 4-(2-pyridylazo)-resorcinol (PAR) and UV-visible detection at 520 nm was tested using different gradient elution profiles and pH values to optimize separation and recovery, primarily for Sc but also for yttrium and the individual lanthanides, from iron present in the samples. The separation was performed in less than 20 min by use of a mobile phase gradient. The concentration of alpha-hydroxyisobutyric acid ( alpha-HIBA), as eluent, was altered from 0.06 to 0.4 mol L(-1) (pH 3.7) and 0.01 mol L(-1) sodium salt n-octane sulfonic acid (OS) was used as modifier. Very low detection limits in the nanogram range and a good resolution for Sc and REEs except for Y/Dy were achieved. Before application of the method to the red mud samples and to the corresponding bauxites, Sc and REEs were leached from red mud with 0.6 mol L(-1) HNO(3) and mostly separated, as a group, from the main elements by ion exchange/selective elution (6 mol L(-1) HNO(3)) in accordance with a pilot-plant process developed in this laboratory. After evaporation of the eluent to dryness the extracted elements were re-dissolved in the mobile phase. By use of this chromatographic method Sc, which is the most expensive of the elements investigated and occurs in economically interesting concentrations in red mud, could be separated not only from co-existing Fe but also from Y/Dy, Yb, Er, Ho, Gd, Eu, Sm, Nd, Pr, Ce and La. All the elements investigated were individually recovered. Their recoveries were found to be nearly quantitative. PMID:15221192

  17. Direct molecular mass determination of trehalose monomycolate from 11 species of mycobacteria by MALDI-TOF mass spectrometry.

    Science.gov (United States)

    Fujita, Yukiko; Naka, Takashi; Doi, Takeshi; Yano, Ikuya

    2005-05-01

    Direct estimation of the molecular mass of single molecular species of trehalose 6-monomycolate (TMM), a ubiquitous cell-wall component of mycobacteria, was performed by matrix-assisted laser desorption/ionization time-of-flight (MALDI-TOF) mass spectrometry. When less than 1 microg TMM was analysed by MALDI-TOF mass spectrometry, quasimolecular ions [M+Na]+ of each molecular species were demonstrated and the numbers of carbons and double bonds (or cyclopropane rings) were determined. Since the introduction of oxygen atoms such as carbonyl, methoxy and ester groups yielded the appropriate shift of mass ions, the major subclasses of mycolic acid (alpha, methoxy, keto and wax ester) were identified without resorting to hydrolytic procedures. The results showed a marked difference in the molecular species composition of TMM among mycobacterial species. Unexpectedly, differing from other mycoloyl glycolipids, TMM from Mycobacterium tuberculosis showed a distinctive mass pattern, with abundant odd-carbon-numbered monocyclopropanoic (or monoenoic) alpha-mycolates besides dicyclopropanoic mycolate, ranging from C75 to C85, odd- and even-carbon-numbered methoxymycolates ranging from C83 to C94 and even- and odd-carbon-numbered ketomycolates ranging from C83 to C90. In contrast, TMM from Mycobacterium bovis (wild strain and BCG substrains) possessed even-carbon-numbered dicyclopropanoic alpha-mycolates. BCG Connaught strain lacked methoxymycolates almost completely. These results were confirmed by MALDI-TOF mass analysis of mycolic acid methyl esters liberated by alkaline hydrolysis and methylation of the original TMM. Wax ester-mycoloyl TMM molecular species were demonstrated for the first time as an intact form in the Mycobacterium avium-intracellulare group, M. phlei and M. flavescens. The M. avium-intracellulare group possessed predominantly C85 and C87 wax ester-mycoloyl TMM, while M. phlei and the rapid growers tested contained C80, C81, C82 and C83 wax ester

  18. Chemical reactivity of {alpha}-isosaccharinic acid in heterogeneous alkaline systems

    Energy Technology Data Exchange (ETDEWEB)

    Glaus, M. A.; Loon, L. R. Van

    2009-05-15

    Cellulose degradation under alkaline conditions is of relevance for the mobility of many radionuclides in the near-field of a cementitious repository for radioactive waste, because metal-binding degradation products may be formed. Among these, {alpha}- isosaccharinic acid ({alpha}-ISA) is the strongest complexant. The prediction of the equilibrium concentration of {alpha}-ISA in cement pore water is therefore an important step in the assessment of the influence of cellulose degradation products on the speciation of radionuclides in such environments. The present report focuses on possible chemical transformation reactions of {alpha}-ISA in heterogeneous alkaline model systems containing either Ca(OH){sub 2} or crushed hardened cement paste. The transformation reactions were monitored by measuring the concentration of {alpha}-ISA by high performance anion exchange chromatography and the formation of reaction products by high performance ion exclusion chromatography. The overall loss of organic species from solution was monitored by measuring the concentration of non-purgeable organic carbon. The reactions were examined in diluted and compacted suspensions, at either 25 {sup o}C or 90 {sup o}C, and under anaerobic atmospheres obtained by various methods. It was found that {alpha}-ISA was transformed under all conditions tested to some extent. Reaction products, such as glycolate, formate, lactate and acetate, all compounds with less complexing strength than {alpha}-ISA, were detected. The amount of reaction products identified by the chromatographic technique applied was {approx} 50 % of the amount of {alpha}-ISA reacted. Sorption of {alpha}-ISA to Ca(OH){sub 2} contributed only to a minor extent to the loss of {alpha}-ISA from the solution phase. As the most important conclusion of the present work it was demonstrated that the presence of oxidising agents had a distinctive influence on the turnover of {alpha}-ISA. Under aerobic conditions {alpha}-ISA was

  19. Effects of pH and Iminosugar Pharmacological Chaperones on Lysosomal Glycosidase Structure and Stability

    Energy Technology Data Exchange (ETDEWEB)

    Lieberman, Raquel L.; D’aquino, J. Alejandro; Ringe, Dagmar; Petsko, Gregory A.; (Harvard-Med); (Brandeis)

    2009-06-05

    Human lysosomal enzymes acid-{beta}-glucosidase (GCase) and acid-{alpha}-galactosidase ({alpha}-Gal A) hydrolyze the sphingolipids glucosyl- and globotriaosylceramide, respectively, and mutations in these enzymes lead to the lipid metabolism disorders Gaucher and Fabry disease, respectively. We have investigated the structure and stability of GCase and {alpha}-Gal A in a neutral-pH environment reflective of the endoplasmic reticulum and an acidic-pH environment reflective of the lysosome. These details are important for the development of pharmacological chaperone therapy for Gaucher and Fabry disease, in which small molecules bind mutant enzymes in the ER to enable the mutant enzyme to meet quality control requirements for lysosomal trafficking. We report crystal structures of apo GCase at pH 4.5, at pH 5.5, and in complex with the pharmacological chaperone isofagomine (IFG) at pH 7.5. We also present thermostability analysis of GCase at pH 7.4 and 5.2 using differential scanning calorimetry. We compare our results with analogous experiments using {alpha}-Gal A and the chaperone 1-deoxygalactonijirimycin (DGJ), including the first structure of {alpha}-Gal A with DGJ. Both GCase and {alpha}-Gal A are more stable at lysosomal pH with and without their respective iminosugars bound, and notably, the stability of the GCase-IFG complex is pH sensitive. We show that the conformations of the active site loops in GCase are sensitive to ligand binding but not pH, whereas analogous galactose- or DGJ-dependent conformational changes in {alpha}-Gal A are not seen. Thermodynamic parameters obtained from {alpha}-Gal A unfolding indicate two-state, van't Hoff unfolding in the absence of the iminosugar at neutral and lysosomal pH, and non-two-state unfolding in the presence of DGJ. Taken together, these results provide insight into how GCase and {alpha}-Gal A are thermodynamically stabilized by iminosugars and suggest strategies for the development of new pharmacological

  20. Neuroprotective effects of α-lipoic acid on the development of oxidative stress and astrogliosis in the brain of STZ-diabetic rats

    Directory of Open Access Journals (Sweden)

    S. Kyrychenko

    2014-07-01

    Full Text Available The aim of this study was to examine whether the antioxidant alpha-lipoic acid protects neurons from diabetic-reperfusion injury. The streptozotocin (STZ rat model was used to study the glial reactivity and prevention of gliosis by alpha-lipoic acid (alpha-LA administration. The expression of glial fibrillary acidic protein (GFAP was determined, as well as lipid peroxidation (LPO and glu-tathione (GSH levels in some brain tissues. We observed significant increasing of lipid peroxidation products in both hippocampus and cortex. Changesof polypeptide GFAP were observed in hippocampus and cortex. Both soluble and filamentous forms of GFAP featured the increase in hippocampus of rat with hyperthyreosis. In the filamentfractions, increase in the intensity of 49 kDa polypeptide band was found. In the same fraction of insoluble cytoskeleton proteins degraded HFKB polypeptides with molecular weight in the range of 46–41 kDa appeared. Markedincrease of degraded polypeptides was found in the soluble fraction of the brain stem. The intensity of the intact polypeptide – 49 kDa, as well as in the filament fraction, significantly increased. It is possible that increasing concentrations of soluble subunits glial filaments may be due to dissociation of own filaments during the reorganization of cytoskeleton structures. Given the results of Western blotting for filament fraction, increased content of soluble intact 49 kDa polypeptide is primarily the result of increased expression of HFKB and only partly due to redistribution of existing filament structures. Calculation and analysis of indicators showed high correlation between the increase in content and peroxidation products of HFKB.These results indicate the important role of oxidative stress in the induction of astroglial response under conditions of diabet encefalopathia. Administration of alpha-LA reduced the expression both of glial and neuronal markers. In addition, alpha-LA significantly prevented

  1. Do paradigma molecular ao impacto no prognóstico: uma visão da leucemia promielocítica aguda From the molecular model to the impact on prognosis: an overview on acute promyelocytic leukemia

    Directory of Open Access Journals (Sweden)

    Rafael Henriques Jácomo

    2008-02-01

    Full Text Available A leucemia promielocítica aguda (LPA é um modelo da aplicabilidade clínica dos conhecimentos moleculares fisiopatológicos. Caracteriza-se por alterações genéticas recorrentes que envolvem o gene do receptor alfa do ácido retinóico. A conseqüência é uma proteína com sensibilidade reduzida ao ligante, com bloqueio da diferenciação mielóide. Entretanto, doses suprafisiológicas do ácido all-trans-retinóico (ATRA são capazes de suplantar esta deficiência, e este é o princípio fundamental do tratamento da LPA, permitindo uma sobrevida livre de doença acima de 80% quando adequadamente tratada. Epidemiologicamente, difere dos demais subtipos de leucemia mielóide aguda por apresentar incidência predominante em adultos jovens e, aparentemente, maior incidência em países de colonização "latina". Contrastando com os excelentes resultados observados em países desenvolvidos, a mortalidade por LPA no Brasil ainda é alta, apesar da ampla disponibilidade das medicações no país.Acute promyelocytic leukemia (APL is a model of clinical applicability of the knowledge of molecular physiopathology. It is characterized by recurrent genetic involvement of the retinoic acid alpha receptor. The consequence is a protein with low sensibility to its ligand and a myeloid maturation arrest. However, higher doses of all-trans-retinoic acid (ATRA are able to supersede this deficiency and this is the mainstay of APL treatment leading to over 80% disease free survival, when adequately treated. Epidemiologically, it differs from other acute myeloid leukemia due to a higher incidence in young adults and in countries of "Latin" colonization. Differing from excellent results observed in developed countries, APL mortality in Brazil is still high, despite the wide availability of drugs.

  2. Exploring triacylglycerol biosynthetic pathway in developing seeds of Chia (Salvia hispanica L.: a transcriptomic approach.

    Directory of Open Access Journals (Sweden)

    Sreedhar R V

    Full Text Available Chia (Salvia hispanica L., a member of the mint family (Lamiaceae, is a rediscovered crop with great importance in health and nutrition and is also the highest known terrestrial plant source of heart-healthy omega-3 fatty acid, alpha linolenic acid (ALA. At present, there is no public genomic information or database available for this crop, hindering research on its genetic improvement through genomics-assisted breeding programs. The first comprehensive analysis of the global transcriptome profile of developing Salvia hispanica L. seeds, with special reference to lipid biosynthesis is presented in this study. RNA from five different stages of seed development was extracted and sequenced separately using the Illumina GAIIx platform. De novo assembly of processed reads in the pooled transcriptome using Trinity yielded 76,014 transcripts. The total transcript length was 66,944,462 bases (66.9 Mb, with an average length of approximately 880 bases. In the molecular functions category of Gene Ontology (GO terms, ATP binding and nucleotide binding were found to be the most abundant and in the biological processes category, the metabolic process and the regulation of transcription-DNA-dependent and oxidation-reduction process were abundant. From the EuKaryotic Orthologous Groups of proteins (KOG classification, the major category was "Metabolism" (31.97%, of which the most prominent class was 'carbohydrate metabolism and transport' (5.81% of total KOG classifications followed by 'secondary metabolite biosynthesis transport and catabolism' (5.34% and 'lipid metabolism' (4.57%. A majority of the candidate genes involved in lipid biosynthesis and oil accumulation were identified. Furthermore, 5596 simple sequence repeats (SSRs were identified. The transcriptome data was further validated through confirmative PCR and qRT-PCR for select lipid genes. Our study provides insight into the complex transcriptome and will contribute to further genome-wide research

  3. Observational clinical study of 22 adult-onset Pompe disease patients undergoing enzyme replacement therapy over 5years.

    Science.gov (United States)

    Stepien, Karolina M; Hendriksz, Christian J; Roberts, Mark; Sharma, Reena

    2016-04-01

    Pompe disease is an autosomal recessive disease resulting from deficiency of the acid alpha-glucosidase (GAA). The late-onset Pompe Disease (LOPD) patients develop muscular and respiratory complications later in life. We describe a retrospective observational cohort study including 22 patients with LOPD. The cohort was assessed at baseline before Enzyme Replacement Therapy (ERT) with alglucosidase alpha (20mg/kg biweekly) was commenced and subsequently relevant information was collected at 2, 4 and 5years later. The median age of the patients at study entry was 44years (16-64years), with median disease duration of 11.5years (4-31years). At baseline, 10 patients (45%) could walk without support, 12 (55%) could walk with unilateral or bilateral support including 3/12 were wheelchair bound. Mean predicted FVC % was 55.7 (95% CI 45-66) of predicted normal at baseline and showed no significant change after 5years (54.6 (95% CI 43-66)), (all p=0.9815). Mean FVC % supine was 41.8 (95% CI 33.8-49) of predicted normal at baseline and remained significantly unchanged at 5years (48.4 (95% CI 37-59.6)), (all p=0.8680). The overnight non-invasive ventilator dependence increased by 18.2% as compared with baseline and requirement of mobility aids increased during this period by 5.2% as compared with the baseline. Mean walking distance at 6min walk test was 411.5 (95% CI 338-485) at baseline, 266.5 (95% CI 187-346) m at 2years, 238.6 (95% CI 162-315) m at 4years and 286.8 (95% CI 203-370) m at 5years (p=0.1981; ANOVA was completed only for 14 patients). A gradual decline in FVC% predicted was noted only in four cases and a decline in FVC% supine in two other. Only one patient showed a decline in both pulmonary function tests. In all remaining cases (17/22) respiratory function remains stable. In conclusion overall pulmonary function tests and mobility remained stable for 5years in majority of patients on ERT. However, in some patients they continued to decline in spite of ERT

  4. 糖原累积病Ⅱ型(Pompe病)17例临床特点和转归%Clinical sequelae of 17 cases with glycogen storage disease type Ⅱ/Pompe disease

    Institute of Scientific and Technical Information of China (English)

    张寒冰; 张为民; 仇佳晶; 孟岩; 邱正庆

    2012-01-01

    目的 总结分析国内糖原累积病Ⅱ型(Pompe病)患者的临床表现及疾病转归的特点.方法 回顾性分析17例经酶学确诊的糖原累积病Ⅱ型患者临床资料,通过设计临床调查表进行电话随访,总结归纳临床和转归特点.结果 17例糖原累积病Ⅱ型患者中,4例为婴儿型,生后2~6个月发病,均有肌酶升高和心脏受累,伴或不伴呼吸肌受累.4例均未进行治疗.其余13例为晚发型,发病年龄2~22岁,均有肌酶升高.其中11例以肌肉无力起病,2例表现为呼吸肌受累,2例表现为脊柱侧弯,1例表现为肌酶高伴肝功能异常.除3例曾接受呼吸机辅助治疗外,其余10例未接受相关治疗.全部患者α-1,4-葡萄糖苷酶活性数值均低于正常人10%以下;14例行病理肌活检检测,9例提示糖原累积病Ⅱ型;10例行基因检测,6例检测到2个致病突变.成功电话随访12例,随访率70%.婴儿型4例中,3例在3岁之内死于呼吸衰竭,存活的1例目前1岁7个月.成功随访的晚发型8例中,4例于发病3~5年死亡,均死于呼吸衰竭,存活的4例中3例自觉肌肉无力症状加重,1例自住院时使用呼吸机治疗,至今病情无明显改变.12例随访者中7例死亡,总死亡率58%.结论 糖原累积病Ⅱ型在临床表现上存在差异.婴儿型表现为全身性肌肉无力,心脏受累明显,病情凶险,死亡率高.晚发型以慢性进行性肌肉无力,呼吸功能不全为主.经α-1,4-葡萄糖苷酶活性测定、肌肉活检以及基因检测诊断.患者的预后与发病年龄、有无呼吸肌受累相关.%Objective To analyze and summarize the characteristics of glycogen storage disease type Ⅱ ( Pompe disease) patients according to the clinical description and prognosis.Method Seventeen Chinese patients diagnosed by acid alpha-glucosidase ( GAA ) enzyme activity test were reviewed.Clinical data tables were designed.Interviews were made via phone calls.Information was collected to

  5. 210Po concentration in fish, algae, mussel and beach sand samples in Turkish coast of the Black Sea

    International Nuclear Information System (INIS)

    The natural radionuclide 210Po is the major contributor to the radiation dose received by humans from the sea food consumption. The main source of 210Po in the environment is the exhalation of 222Rn gas from the ground into the atmosphere. The radionuclides fall to the sea water and oceans together with aerosol particles by washout and sedimentation. The other source include burning of fossil fuel, uranium, phosphate and lead ore processing industries and tetraethyl lead used for car engines. The entrance of the radionuclides to the human body are via inhalation or ingestion. The major contribution for 210Po received via food consumption especially is originated from fish and shell fish by which it is strongly accumulated in relatively high concentration. This can lead to increase the radiation dose delivered to man by significant factor. In this study the concentration of 210Po were determined in algae, fish, mussel and beach sand samples collected from four different coastal zones of the Black Sea in Turkey from selected stations namely Sile, Trabzon, Sinop and Zonguldak during the period of 1998-2001. The sampling and analysis were carried out as a part of the 'Marine Environmental Assessment of the Black Sea Region' Technical Cooperation Project, RER/2/003 undertaken by the six Black Sea countries which is supported by IAEA. For the determination of 210Po in samples the standard techniques was used. Known activity of 209Po were added as an isotopic tracer. Samples were completely dissolved with mineral acids (HNO3, H2O2). When the solution was clear, concentrated HCl was added and the solution was gently evaporated to near dryness. After evaporation, polonium was plated onto silver disc in 0.5 M HCl in presence of ascorbic acid. Alpha counting of sample was performed using a silicon surface barrier detectors connected to a PC. The mean 210Po concentrations measured in mussel, algae, fish and beach sand samples from the four different locations on Turkish coast

  6. Les oméga 3 : de l’alimentation animale à la nutrition humaine

    Directory of Open Access Journals (Sweden)

    Combe Nicole

    2004-01-01

    Full Text Available La dernière édition des Apports nutritionnels conseillés (ANC- éd. 2001 souligne la nécessité d’accroître la consommation d’acide alpha-linolénique (ALA. Celle-ci devrait au minimum doubler et représenter 0,8 % (par rapport à l’énergie totale contre 0,34 % actuellement. Or, l’étude Aquitaine [1] a montré que les lipides d’origine animale contribuaient à près de 75 % de l’apport alimentaire en ALA. Ainsi, parmi les stratégies envisageables pour accroître notre consommation d’ALA, sans changer fondamentalement les habitudes alimentaires des Français, on note un intérêt croissant pour augmenter la teneur en oméga 3 des produits animaux via l’alimentation de ces derniers. Quelques exemples de modification de la composition en acides gras des produits animaux sont exposés. Chez les espèces monogastriques (volaille, porc, le profil des lipides de carcasse est un bon reflet des lipides alimentaires. Ainsi, chez le porc, le niveau d’oméga 3 peut être augmenté d’un facteur 2 à 4, lorsque les animaux reçoivent dans leur alimentation de l’huile de colza, des graines de lin ou de la farine de poissons. En revanche, chez les animaux ruminants, le transfert des acides gras polyinsaturés alimentaires vers les tissus est faible en raison de l’hydrogénation qu’ils subissent dans le rumen. Certaines stratégies qui consistent à protéger ces acides gras de la biohydrogénation permettent néanmoins d’accroître le taux d’ALA dans les tissus et le lait. Ainsi, il apparaît possible d’augmenter la consommation d’oméga 3 de la population, via la consommation de produits issus d’animaux nourris avec des sources d’oméga 3. Cependant, au niveau de la production, ces changements doivent être maîtrisés \\; ils ne peuvent se faire sans un contrôle des qualités d’une part organoleptiques, en raison du degré élevé d’oxydabilité de ces acides gras, d’autre part de texture, en raison

  7. Screening, Identification and Fermentation Property of a Yeast Strain R6 Accumulating Alpha-ketoglutaric Acid%酵母 R6中α-酮戊二酸的筛选、鉴定和发酵性能的研究

    Institute of Scientific and Technical Information of China (English)

    陈静; 张春杨; 李超

    2015-01-01

    A yeast strain R6 was obtained by the method of thiamine(VB1) auxotroph-ic negative selection from the edible oil-pol uted soil in Zibo, China. Physiological and biochemical experiments revealed that strain R6 shared common feature with Rhodotorula mucilaginosa according to the API 20 C AUX yeast identification sys-tem which has been tested previously. Furthermore, the 18S rDNA gene of strain R6 was amplified and sequenced. Phylogenetic analysis based on the 18S rDNA sequence and the relatives indicated that R6 shared 99% homologies with the members of R. mucilaginosa, suggesting that strain R6 belonged to R. mucilaginosa. Investigation showed that strain R6 possessed the capacity of accumulating exocel-lular alpha-ketoglutaric acid (alpha-KG). Final y, the fermentation conditions of R6 to accumulate alpha-KG was optimized by control ing each single fermenting variable and detected through high performance liquid chromatography (HPLC). Results showed that both VB1 and CaCO3 in fermentation medium were the key factors in-fluencing the cumulant of alpha-KG. The discovery of natural auxotrophic strain R6 not only broadened the microbial resource which can achieve lots of alpha-KG pro-duction through fermentation, but also laid a foundation for further fermentation regu-lation to achieve excessive alpha-KG accumulation.%该研究通过硫胺素( VB1)营养缺陷型负选择法,从中国淄博食用油污染的土壤中获得一株酵母属菌株 R6。该菌株具有累积胞外α-酮戊二酸(α-KG)的能力。生理学和生物化学实验表明该 R6菌株与已测试的 API 20 C AUX酵母鉴别系统中的胶红酵母具有共同特征。此外,R6菌株的18S rDNA 基因已经获得并测序,基于该序列以及同源菌属所得到的系统进化学分析表明菌株 R6与胶红酵母属成员具有99%的同源性。因此,菌株 R6被鉴别为胶红酵母属的一员。随后,本实验通过控制单一发酵变量以及高效液相

  8. Acyl chain length effects related to glycosphingolipid crypticity in phospholipid membranes: probed by 2H-NMR.

    Science.gov (United States)

    Hamilton, K S; Briere, K; Jarrell, H C; Grant, C W

    1994-03-23

    Wideline 2H-NMR was used to consider the relationships amongst glycosphingolipid and phospholipid fatty acid chain length and glycosphingolipid receptor function, in a system classically associated with crypticity. Galactosyl ceramide (GalCer), having 18- or 24-carbon fatty acid, was deuterium labelled at the conformationally-restricted fatty acid alpha-carbon (C-2). 2H-NMR spectra of N-[2,2-2H2]stearoyl and N-[2,2-2H2]lignoceroyl GalCer (GalCer with 18-vs. 24-carbon selectively deuterated fatty acid) were then compared over a range of temperatures in phosphatidylcholine/cholesterol membranes in which the host phospholipid had dimyristoyl, dipalmitoyl, or distearoyl fatty acid composition. Findings were evaluated in the light of known sensitivity of antibody interaction with GalCer to temperature and to both glycolipid fatty acid chain length and host matrix fatty acid chain length. Under the conditions of experimentation, spectra were not obtainable for glycolipids having rigid body motions that were slow on the NMR timescale (10(-4)-10(-5) s)-i.e.. motions typical of non-fluid (gel phase) membranes. The systems, DPPC/cholesterol and DSPC/cholesterol, in which the original observation was made of increased antibody binding to GalCer with long fatty acid, proved to be characterised by receptor motions that were in this slow timescale for both 18:0 and 24:0 GalCer at 22-24 degrees C. Under conditions for which spectra could be obtained, those for GalCer with [2,2-2H2]lignoceroyl (24-carbon alpha-deuterated) fatty acid were qualitatively similar to those of its 18-carbon analogue in all (fluid) membranes examined. However, spectral splittings differed quantitatively between deuterated 18:0 and 24:0 GalCer at a given temperature, dependent upon host matrix. These differences were most marked at lower temperatures and in the longer chain (more ordered) matrices, DPPC/cholesterol and DSPC/cholesterol. This suggests that maximum effects of glycolipid chain length on

  9. Examining food additives and spices for their anti-oxidant ability to counteract oxidative damage due to chronic exposure to free radicals from environmental pollutants

    Science.gov (United States)

    Martinez, Raul A., III

    The main objective of this work was to examine food additives and spices (from the Apiaceae family) to determine their antioxidant properties to counteract oxidative stress (damage) caused by Environmental pollutants. Environmental pollutants generate Reactive Oxygen species and Reactive Nitrogen species. Star anise essential oil showed lower antioxidant activity than extracts using DPPH scavenging. Dill Seed -- Anethum Graveolens -the monoterpene components of dill showed to activate the enzyme glutathione-S-transferase , which helped attach the antioxidant molecule glutathione to oxidized molecules that would otherwise do damage in the body. The antioxidant activity of extracts of dill was comparable with ascorbic acid, alpha-tocopherol, and quercetin in in-vitro systems. Black Cumin -- Nigella Sativa: was evaluated the method 1,1-diphenyl2-picrylhhydrazyl (DPPH) radical scavenging activity. Positive correlations were found between the total phenolic content in the black cumin extracts and their antioxidant activities. Caraway -- Carum Carvi: The antioxidant activity was evaluated by the scavenging effects of 1,1'-diphenyl-2-picrylhydrazyl (DPPH). Caraway showed strong antioxidant activity. Cumin -- Cuminum Cyminum - the major polyphenolic were extracted and separated by HPTLC. The antioxidant activity of the cumin extract was tested on 1,1'-diphenyl-2- picrylhydrazyl (DPPH) free radical scavenging. Coriander -- Coriandrum Sativum - the antioxidant and free-radical-scavenging property of the seeds was studied and also investigated whether the administration of seeds curtails oxidative stress. Coriander seed powder not only inhibited the process of Peroxidative damage, but also significantly reactivated the antioxidant enzymes and antioxidant levels. The seeds also showed scavenging activity against superoxides and hydroxyl radicals. The total polyphenolic content of the seeds was found to be 12.2 galic acid equivalents (GAE)/g while the total flavonoid content

  10. Cold pressed poppy seed oils: sensory properties, aromatic profiles and consumer preferences

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    Emir, D. D.

    2014-09-01

    Full Text Available The sensory descriptions, aromatic profiles and consumer preferences of poppy seed oils produced from three poppy varieties (ofis3, ofis4, and ofis8 by cold pressing were studied. Roasting and enzyme treatments were applied to the seeds prior to cold pressing. In addition, 75 different volatiles were quantified by GC-MS analysis. A flavor profile analysis was made with 9 panelists and 12 terms were identified for the description of the oil. The results shown that, only earthy term scores were different among the seed varieties, while treatments have caused differences in roasted, hazelnut, hay and sweet aromatic terms. Roasting and enzyme treatments decreased hay and increased sweet aromatic values. The enzyme treatment of the poppy seeds enhanced fermented and waxy scores in the cold press oils. 1-hexanol, 2-heptanone, 2-pentanone, 2-pentyl furan, 3-ethyl- 2-methyl 1,3-hexadiene, 2-(dimethylamino-3-phenylbenzo[b]thiophene, 3-octen-2-one, 4-hydroxyphenylacetic acid, alpha-pinene, limonene, dimethyl sulfone, mercaptoacetic acid, hexanal and nonanal were quantified as the major volatiles in all treatment groups. Consumer test results indicated that roasted samples are more liked, and the yellow (ofis4 roasted sample was identified as the most preferred (53.55% oil by consumers. This study provides the first sensory descriptive definitions and consumer preferences for poppy seed oils.En este trabajo, se analizan las descripciones sensoriales, perfiles aromáticos y preferencias de los consumidores de aceites de semillas de amapola producidosmediante prensado en frío a partir de tres variedades (ofis3, ofis4, y ofis8.Previo al prensado en frío, a las semillas se aplicóuna fase de tostado y un tratamiento enzimático. Además del análisis GC-MS donde se cuantificaron 75 compuestos volátiles, el perfil del sabor,realizado con 9 panelistas,logra identificar 12 términos descriptores. Los resultados muestran que, solamente la puntuación del t

  11. Informação nutricional de fórmulas infantis comercializadas no Estado de São Paulo: avaliação dos teores de lipídeos e ácidos graxos Nutrition facts of infant formulas sold in São Paulo state: assessment of fat and fatty acid contents

    Directory of Open Access Journals (Sweden)

    Mahyara Markievicz Mancio Kus

    2011-04-01

    Full Text Available OBJETIVO: Este trabalho teve como objetivo avaliar a informação nutricional fornecida pelos fabricantes de fórmulas infantis, comparando-as quanto aos teores obtidos na análise, ao conteúdo de lipídeos, ácidos graxos saturados, ácidos graxos trans, ácido linoleico, ácido alfa-linolênico, ácido araquidônico e ácido docosahexaenoico. MÉTODOS: Foram analisadas 14 amostras de seis marcas diferentes de fórmulas infantis comercializadas no Estado de São Paulo. A extração e a quantificação dos lipídeos foram realizadas pelo método oficial (Roese Gottlieb, e a quantificação dos ácidos graxos, pela cromatografia em fase gasosa pelo método de adição de padrão interno. As análises foram realizadas em triplicata. RESULTADOS: De acordo com os resultados, todas as amostras estavam em conformidade com a rotulagem para gordura total, uma estava em desacordo para ácidos graxos saturados, seis para ácidos graxos trans, quatro para ácido linoleico, dez para ácido alfa-linolênico, dois para ácido araquidônico e três para o ácido docosahexaenoico. As fórmulas infantis para lactentes de zero a seis meses foram as que revelaram as maiores diferenças em relação à informação nutricional fornecida pelo fabricante. CONCLUSÃO: Os resultados obtidos apontam a necessidade de um contínuo monitoramento desses produtos por meio de programas de vigilância sanitária, uma vez que a inadequação dos nutrientes da fração lipídica e outros pode afetar de maneira significativa o desenvolvimento infantil.OBJECTIVE: This study determined the contents of fats, saturated fatty acids, trans fatty acids, linoleic acid, alpha-linolenic acid, arachidonic acid and docosahexaenoic acid in infant formulas and compared them with the nutrition facts reported on the respective packaging. METHODS: Fourteen samples of six different infant formula brands sold in the state of São Paulo were analyzed. The extraction and quantification of fats were

  12. Activity of mouse liver glutathione S-transferases toward trans,trans-muconaldehyde and trans-4-hydroxy-2-nonenal.

    Science.gov (United States)

    Goon, D; Saxena, M; Awasthi, Y C; Ross, D

    1993-04-01

    This study investigated the catalytic activities of hepatic glutathione S-transferase (GST) isoenzymes isolated from CD-1 mice toward two activated alkenals of toxicological relevance: trans,trans-muconaldehyde (MA), a putative myelotoxic metabolite of benzene, and trans-4-hydroxy-2-nonenal (HNE), a highly reactive lipid peroxidation product. The activity toward 1-chloro-2,4-dinitrobenzene (CDNB) was also determined. Four isoenzymes with pI values of 9.8, 8.7, 6.4, and 5.7 were each isolated from male and female mice. The isoenzymes with pI values of 8.7 and 6.4 are pi and mu class GSTs, respectively, whereas the pI 9.8 and 5.7 GSTs are both alpha class isoenzymes. CDNB activity was greatest in the pi (pI 8.7) isoenzyme of both sexes. In addition, the CDNB activity of the pi (pI 8.7) isoenzyme from males was markedly greater than the corresponding GST from female mouse liver. In contrast to CDNB, both MA and HNE were better substrates for the acidic alpha (pI 5.7) and mu (pI 6.4) GSTs, whereas minimal activity toward either alkenal was detected in the pi (pI 8.7) and alpha (pI 9.8) isoenzymes. Maximum activity toward MA and HNE was exhibited by the alpha (pI 5.7) isoenzyme of both sexes. The level of HNE activity observed with the alpha (pI 5.7) isoenzyme was five- to sixfold greater than that reported previously for any mouse GST isoenzyme. Moreover, the specific activities of the female alpha (pI 5.7) isoenzyme toward both HNE and MA were markedly greater than those of the corresponding isoenzyme from males. A similar gender-specific difference was noted in the activity of the mu (pI 6.4) isoenzyme toward HNE, but not toward MA. These results show that both MA and HNE are substrates for the alpha (pI 5.7) and mu (pI 6.4) GSTs of murine liver, with maximum activity toward both activated alkenals exhibited by the alpha (pI 5.7) isozyme. In addition, evidence is presented that demonstrates a female-dominant sex difference in the activity of the alpha (pI 5

  13. Glicogenose hereditária em bovinos Brahman no Brasil Inherited glycogenosis in Brahman cattle in Brazil

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    P Zlotowski

    2005-12-01

    one month of age, showed progressive difficulty to follow their mother, retarded growth, muscular weakness and tremors, lethargy and poor body condition. All affected calves were sired by the same bull. Necropsy was performed on three affected calves. The only gross lesion detected was paleness of the skeletal muscles of the trunk and limbs. Cytoplasmic vacuoles, the main histological lesion, were particularly evident in skeletal muscles, myocardium and Purkinje fibers, in neurons of the brain and spinal cord. Large amounts of periodic acid Schiff (PAS positive granules were also observed in these most severely affected tissues. Pretreatment with diastase completely abolished the PAS reactivity. The 1057?TA, a lethal mutation in the gene of the acid alpha-glucosidase, which causes generalized glycogenosis in Brahman cattle, was detected by PCR in paraffin embedded tissues of affected animals on which post-mortem examination was performed. Clinical, histological and molecular findings were similar to previous descriptions of generalized glycogenosis in Brahman cattle in Australia. No previous indexed reports about generalized glycogenosis of Brahman cattle in Brazil could be found.

  14. Alimentation animale et valeur nutritionnelle induite sur les produits dérivés consommés par l’homme : Les lipides sont-ils principalement concernés ?

    Directory of Open Access Journals (Sweden)

    Bourre Jean-Marie

    2003-09-01

    sous contrôle génétique. En revanche, la nature des acides gras des triglycérides de réserve (trouvés en quantité plus ou moins importante selon les localisations anatomiques c’est-à-dire les morceaux de boucherie peut varier notablement en fonction de la nourriture reçue par les animaux. En les contrôlant, il est possible de contribuer à un meilleur état sanitaire des consommateurs. Les conséquences (qualitatives et quantitatives des modifications de la composition de l’alimentation animale sur la valeur des produits dérivés consommés par l’homme sont plus amples chez les mono-gastriques que chez les poly-gastriques. Car, par exemple, les bactéries intestinales hydrogénantes de ces derniers transforment en acides gras saturés une fraction notable des acides gras poly-insaturés présents dans leur alimentation, leur faisant par conséquent perdre leur intérêt biologique. Ainsi, dans les meilleures conditions, en nourrissant par exemple les animaux avec des graines de lin ou de colza, la teneur en acide alpha-linolénique est multipliée par environ 2 dans la viande de bœuf, par 6 dans celle de porc, par 10 dans le poulet, par 40 dans les œufs. En nourrissant les animaux avec des extraits de poissons ou d’algues (huiles, la quantité de DHA (acide cervonique, 22:6ω3 est multipliée par 2 dans la viande de bœuf, par 7 dans le poulet, par 6 dans les œufs, par 20 dans le poisson (saumon. Pour obtenir de tels résultats, il ne s’agit que de respecter la physiologie des animaux, ce qui était fréquemment le cas avec les méthodes traditionnelles. Il convient de mettre l’accent sur les poissons, dont la valeur nutritionnelle pour l’homme en termes de lipides (déterminée par la quantité d’acides gras oméga-3 peut varier considérablement selon la nature des graisses avec lesquelles les animaux sont alimentés. L’objectif de prévention de certains aspects des maladies cardio-vasculaires (et d’autres pathologies peut être

  15. 遗传代谢病类婴儿肝炎综合征的临床分析%Clinical analysis of infantile hepatitis syndrome complicated with inherited metabolic diseases

    Institute of Scientific and Technical Information of China (English)

    董红; 欧榕琼; 欧阳颖

    2015-01-01

    mass spectrometry analysis re-sults,diagnosis,treatment and clinical prognosis were recorded and analyzed.Results Physical examination revealed yellow skin and sclera in 1 0 cases.All infants presented with complications,including anemia in nine cases,cytomegalovirus (CMV)infection in four infants and epstein-barr virus (EBV)infection in two infants.Elevated levels of total bilirubin,direct bilirubin,AST,bile acid,alpha fetal protein and lactic acid were found in all 1 0 infants had.Three infants presented with hypoglycemia,six with hypoproteinemia,three with metabolic acidosis and nine with coagulation disorders.Based upon the urine GC-MS,blood MS /MS,clinical characteristics,laboratory and auxiliary examination outcomes,1 0 infants were eventually diagnosed with inher-ited metabolic diseases complicated with IHS including two cases of galactosemia,four cases of neonatal intra-hepatic cholestasis induced by Citrin deficiency,one case of acidaemia,one case of urea circulatory disorders and citrullinemia,one case of urea circulatory disorders and organic aciduria and one case of tyrosinemia.One NICCD infant had a SLC25A1 3 gene sequence of c.851 854delGTAT p.(Met285Ts).After diagnosis was confirmed,all infants received ademetionine 1 ,4-butanedisulfonate and ursodesoxycholic acid therapy.Eight infants had alleviated jaundice and were discharged,and the other two abandoned the treatment.Conclusions Infants with inherited metabolic diseases complicated with IHS had persistent and severe jaundice,and con-stantly complicated with metabolic acidosis,hypoglycemia,hyperlactatemia,hyperammonemia,high level of alpha fetal protein and hypoproteinemia,etc.Urine GC-MS and blood MS /MS were of diagnostic significance.Effective therapy should be delivered immediately after the diagnosis is confirmed.

  16. Roles of unsaturated fatty acids (especially omega-3 fatty acids) in the brain at various ages and during ageing.

    Science.gov (United States)

    Bourre, J M

    2004-01-01

    Among various organs, in the brain, the fatty acids most extensively studied are omega-3 fatty acids. Alpha-linolenic acid (18:3omega3) deficiency alters the structure and function of membranes and induces minor cerebral dysfunctions, as demonstrated in animal models and subsequently in human infants. Even though the brain is materially an organ like any other, that is to say elaborated from substances present in the diet (sometimes exclusively), for long it was not accepted that food can have an influence on brain structure, and thus on its function. Lipids, and especially omega-3 fatty acids, provided the first coherent experimental demonstration of the effect of diet (nutrients) on the structure and function of the brain. In fact the brain, after adipose tissue, is the organ richest in lipids, whose only role is to participate in membrane structure. First it was shown that the differentiation and functioning of cultured brain cells requires not only alpha-linolenic acid (the major component of the omega-3, omega3 family), but also the very long omega-3 and omega-6 carbon chains (1). It was then demonstrated that alpha-linolenic acid deficiency alters the course of brain development, perturbs the composition and physicochemical properties of brain cell membranes, neurones, oligodendrocytes, and astrocytes (2). This leads to physicochemical modifications, induces biochemical and physiological perturbations, and results in neurosensory and behavioural upset (3). Consequently, the nature of polyunsaturated fatty acids (in particular omega-3) present in formula milks for infants (premature and term) conditions the visual and cerebral abilities, including intellectual. Moreover, dietary omega-3 fatty acids are certainly involved in the prevention of some aspects of cardiovascular disease (including at the level of cerebral vascularization), and in some neuropsychiatric disorders, particularly depression, as well as in dementia, notably Alzheimer's disease. Recent

  17. 亚临界流体萃取胡麻籽低温压榨饼中油脂%Subcritical fluid extraction of oil from cold press linseed cake

    Institute of Scientific and Technical Information of China (English)

    万楚筠; 黄凤洪; 张明; 李文林; 黄庆德

    2014-01-01

    为高品质提取胡麻籽低温压榨饼残油,采用D-optimum响应面设计优化亚临界丁烷萃取胡麻饼工艺,研究萃取温度、时间和液料比对油脂提取率的影响,并将所得油与粕与正已烷工艺进行品质比较。结果表明:液料比和萃取时间对油脂提取率的影响显著(P<0.05);液料比与萃取温度交互作用显著(P<0.05);最佳工艺参数为液料比8.4 mL/g、萃取时间40 min、萃取温度26℃,此条件下胡麻籽油的提取率为96.50%。亚临界丁烷萃取的胡麻籽油红色较浅、黄色较深,酸价与过氧化值都优于正已烷工艺,磷脂质量分数约为正已烷工艺的1/10。亚临界丁烷萃取后的胡麻籽粕,中性洗涤纤维质量分数低,蛋白中可溶性氮质量分数是正已烷工艺的3.5倍。研究结果可为胡麻籽高品质加工利用提供数据参考。%Linseed (Linum usitassimum L.), also known as Flaxseed, belonging to family Lineaceae, is an annual herb that is one of the oldest cultivated crops, continues to be widely grown for oil, textile, and food. Currently, Linseed is cultivated in more than 50 countries, predominantly in the Northern hemisphere. The important Linseed growing countries include Canada, China, India, Argentina, and United States. In China, Linseed, as one of five most important kinds of oil crops, of the average production is 500 thousand tons per year. Linseed oil is an excellent source of the omega-3 fatty acid alpha linolenic acid (ALA) with typical levels of 55% in the oil making it ideal for function food and nutritional supplements because they have shown beneficial physiological effects. However, the high content of ALA in flaxseed oil is highly susceptible to oxidation, leading to rapid deterioration of quality. Because ALA is sensitive to heat, oxygen, and light, Linseed is usually cold pressed. Cold presses typically recover 80 to 90% of the oil from oilseeds. To exhaust extract the oil of

  18. Comparação entre a relação PCR/albumina e o índice prognóstico inflamatório nutricional (IPIN Comparison of PCR/albumin ratio with prognostic inflammatory nutritional index (PINI

    Directory of Open Access Journals (Sweden)

    Camila Renata Corrêa

    2002-07-01

    diferenciação G3 > (G1 = G2 e G3 > G3A. Entre todas as proteínas dosadas apenas PCR, Alb e TTR discriminaram os grupos: sendo G3 > (G1= G2 para PCR e G3 G3A (para PCR e G3D The inflammatory stress of hospitalyzed patients was quantified according to their plasma levels of acute-phase proteins (APP. The data from 54 adult (48 ± 20 yrs patients were retrospectively (1994-1998 analysed along with other 12 healthy controls. The major pathologies were peripheral vascular disease (22 , penphigus pholiaceos (7, inflammatory bowel disease (7, trauma (6 and orthognatic post surgery (3. Samples of fasting venous blood were drawn and their plasma used for positive (+ and negative (- APP by nephelometric assays. Among assayed APP+ were C-reactive protein (CRP, acid alpha-1-glycoprotein (AAG, alpha-1-antitrypsin (AAT and ceruloplasmim (CER while albumin (Alb, transthyretin (TTR, transferrin (TF and retinol-binding protein (RBP were the APP- representatives. A significant relationship (Spearman test was found between the variables CRP ´ AAG (r = 0.49, Alb ´ TTR (r = 0.60, Alb ´ RBP (0.58, Alb ´ TF (r = 0.39, TTR ´ RBP (r = 0.56 and TTR ´TF (r = 0.43. The stronger relationships between APP+ ´ APP- were found for CRP ´ Alb (r = - 0.71, CRP ´ TTR (- 0.54, CRP ´ TF (r = - 0.39 and AAG ´ Alb (r = - 0.35. By assembling the APP according to the prognostic inflammatory nutritional index (PINI proposed by Ingenbleek & Carpentier (Int. J. Vitam. Nutr. Res., 55: 91, 1985 the obtained data allowed a group distribution as healthy controls (G1, patients without (PINI 1, G3. The later was split as lower (G3A, n =16 medium (G3B, n =10 and high (G3C, n = 6 risk and mortality-risk (G3D, n =11. The PINI values differentiated (non-parametric Kruskall-Wallis test G3 > (G1= G2 and G3 > G3A. Among all assayed proteins only CRP, Alb and TTR discriminated groups as G3 > (G1= G2 for CRP or G3 G3A (for CRP and G3D < G3A (for TTR and TF. The correlation coeficient allowed only APP-- substitutions at

  19. Effet-dose de l’acide oléique alimentaire. Cet acide est-il conditionnellement essentiel ?

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    Bourre Jean-Marie

    2000-11-01

    Full Text Available Dans un travail précédent [1] nous avons montré que, globalement, au terme de la période de gestation-lactation chez des rats de 21 jours, la carence alimentaire en acide oléique entraîne une diminution de la concentration en 18:1(n-9; la synthèse endogène ne compense donc pas l’absence d’acide oléique dans les aliments. Afin de déterminer exactement l’effet de la présence et de la concentration de l’acide oléique dans l’alimentation sur la composition en acides gras de divers organes, les huiles végétales commerciales n’étant pas utilisables (car contenant toujours de l’acide oléique, des triglycérides ont été synthétisés par voie chimique et enzymologique; ils ont été formés soit d’acide oléique, soit d’acide alpha-linolénique, soit d’acide linoléique. La détermination de l’effet-dose a été réalisée avec un protocole expérimental portant sur 7 groupes de rats ayant reçu chacun des aliments de composition identique (en particulier au niveau des acides gras indispensables : les acides linoléique et alpha-linolénique mais dont la teneur, variable, en acide oléique était située entre 0 et 6 000 mg pour 100 g d’aliments. Les rates ont été nourries avec les régimes à partir de 2 semaines avant l’accouplement, leurs portées ont été sacrifiées soit à 21, soit à 60 jours. Quand la teneur de l’acide oléique augmente dans les aliments, les principales modifications observées chez les animaux de 21 jours sont les suivantes.– Concernant le 18:1(n-9 : dans le foie, le muscle, le coeur, les reins et les testicules, sa concentration constante (le plateau de la courbe est atteinte aux environs de 4 g d’acide oléique pour 100 g d’alimentation. En deçà de cette dose, la réponse est croissante. Dans le cerveau, la myéline et les terminaisons nerveuses (mais non le nerf sciatique, la teneur en acide oléique reste optimale et constante.– La concentration du 16:1(n-7 diminue

  20. Citrin缺陷所致婴儿肝内胆汁淤积症43例分析%Intrahepatic cholestasis caused by citrin deficiency:a clinical analysis of 43 infants

    Institute of Scientific and Technical Information of China (English)

    姜涛; 姚蔚; 欧阳文献; 谭艳芳; 李双杰

    2014-01-01

    Objective To analyze the clinical characteristics and prognosis of neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD)in infants.Methods The clinical data of 43 infants with NICCD in our hospital from July 201 1 to April 2014 were collected.The diagnosis was confirmed by tandem mass spectrometry (MS-MS)analysis of blood,gas chromatography-mass spectrometry (GC-MS)a-nalysis of urine,and genetic testing,and an analysis was performed with reference to clinical manifestations and laboratory results.Results Most patients with NICCD developed jaundice early,with a round,fat face,hepatomegaly,and growth retardation.Laboratory examina-tions showed increased bilirubin,total bile acids,blood lactic acid,alpha-fetoprotein,and procalcitonin in all patients,increased alanine aminotransferase,gamma-glutamyl transpeptidase,and blood ammonia,decreased albumin and blood glucose,and dyslipidemia and coag-ulation disorders in most patients,and abnormal liver fibrosis markers (20/24)and low ceruloplasmin (17/20)in some patients.Abdomi-nal ultrasound showed hepatomegaly in 32 patients.MS-MS analysis of blood samples revealed distinctive elevation of methionine,citrul-line,tyrosine,threonine,and a variety of acyl carnitine in most patients.GC-MS analysis of urine samples revealed elevated galactose and galactitol in 20 patients,elevated 4-hydroxyphenyllactic acid and 4-hydroxyphenylpyruvic acid in 10 patients,and no abnormalities in 6 patients.Genetic testing revealed 851del4,1638ins23,and IVS6+5G>A mutations,especially 851del4 mutation.After being treated by giving lactose-free diet,strengthening medium-chain fatty acid diet,protecting the liver,and relieving cholestasis,most patients had nor-malized indices 2 months later,2 cases had liver cirrhosis,and 7 cases died.Conclusion The clinical manifestations of NICCD vary in children.MS-MS analysis of blood,GC-MS analysis of urine,and genetic testing should be performed early in children with clinically suspected NICCD