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Sample records for aminopropionic acid-alpha

  1. Matrice lipidique et biodisponibilité de l’acide alpha-linolénique

    Directory of Open Access Journals (Sweden)

    Couëdelo Leslie

    2017-03-01

    Full Text Available Les acides gras poly-insaturés (AGPI de la série oméga-3 (ω3 ont un rôle important dans la prévention de certaines pathologies. En plus d’être nécessaires dans des conditions physiologiques particulières (développement pré- et post-natal, croissance (Riediger et al., 2009. J Am Diet Assoc 109 : 668–679, ils sont associés à des effets santé en termes de prévention, notamment au niveau de pathologies cardiovasculaires, inflammatoires, certains cancers et certaines maladies neuro-dégénératives (De Lorgeril et al., 1994. Lancet 343 : 1454–1459 ; Simopoulos, 2008. Exp Biol Med (Maywood, NJ 233 : 674–688. Cependant, les dernières études épidémiologiques montrent que les apports en AGPI ω3, et notamment en acide alpha-linolénique (ALA, précurseur métabolique des AGPI à longue chaîne ω3, sont deux fois inférieurs aux recommandations de l’Agence national de sécurité sanitaire, de l’alimentation, de l’environnement et du travail (ANSES, 2011. Outre la nécessité d’augmenter l’apport en ALA, il est désormais nécessaire de prendre en considération les facteurs qui améliorent sa biodisponibilité. Dans ce contexte, nous avons testé plusieurs paramètres susceptibles de moduler le devenir métabolique de l’ALA. Nos recherches ont mis en évidence que plusieurs paramètres physiques et chimiques, tels que l’émulsification d’une huile linolénique avec de la lécithine de soja, la position de l’ALA sur le squelette glycérique du triglycéride alimentaire mais aussi la composition de la matrice permettraient de moduler la biodisponibilité et le devenir métabolique de l’ALA dans l’organisme.

  2. 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...

  3. Oral delivery of Acid Alpha Glucosidase epitopes expressed in plant chloroplasts suppresses antibody formation in treatment of Pompe mice.

    Science.gov (United States)

    Su, Jin; Sherman, Alexandra; Doerfler, Phillip A; Byrne, Barry J; Herzog, Roland W; Daniell, Henry

    2015-10-01

    Deficiency of acid alpha glucosidase (GAA) causes Pompe disease in which the patients systemically accumulate lysosomal glycogen in muscles and nervous systems, often resulting in infant mortality. Although enzyme replacement therapy (ERT) is effective in treating patients with Pompe disease, formation of antibodies against rhGAA complicates treatment. In this report, we investigated induction of tolerance by oral administration of GAA expressed in chloroplasts. Because full-length GAA could not be expressed, N-terminal 410-amino acids of GAA (as determined by T-cell epitope mapping) were fused with the transmucosal carrier CTB. Tobacco transplastomic lines expressing CTB-GAA were generated through site-specific integration of transgenes into the chloroplast genome. Homoplasmic lines were confirmed by Southern blot analysis. Despite low-level expression of CTB-GAA in chloroplasts, yellow or albino phenotype of transplastomic lines was observed due to binding of GAA to a chloroplast protein that has homology to mannose-6 phosphate receptor. Oral administration of the plant-made CTB-GAA fusion protein even at 330-fold lower dose (1.5 μg) significantly suppressed immunoglobulin formation against GAA in Pompe mice injected with 500 μg rhGAA per dose, with several-fold lower titre of GAA-specific IgG1 and IgG2a. Lyophilization increased CTB-GAA concentration by 30-fold (up to 190 μg per g of freeze-dried leaf material), facilitating long-term storage at room temperature and higher dosage in future investigations. This study provides the first evidence that oral delivery of plant cells is effective in reducing antibody responses in ERT for lysosomal storage disorders facilitating further advances in clinical investigations using plant cell culture system or in vitro propagation. © 2015 Society for Experimental Biology, Association of Applied Biologists and John Wiley & Sons Ltd.

  4. Corrosion inhibition of mild steel in 1 M HCl solution by henna extract: A comparative study of the inhibition by henna and its constituents (Lawsone, Gallic acid, {alpha}-D-Glucose and Tannic acid)

    Energy Technology Data Exchange (ETDEWEB)

    Ostovari, A. [Technical Inspection Engineering Department, Petroleum University of Technology, Abadan (Iran, Islamic Republic of)], E-mail: A.Ostovari@gmail.com; Hoseinieh, S.M.; Peikari, M. [Technical Inspection Engineering Department, Petroleum University of Technology, Abadan (Iran, Islamic Republic of); Shadizadeh, S.R. [Petroleum Engineering Department, Petroleum University of Technology, Abadan (Iran, Islamic Republic of); Hashemi, S.J. [Technical Inspection Engineering Department, Petroleum University of Technology, Abadan (Iran, Islamic Republic of)

    2009-09-15

    The inhibitive action of henna extract (Lawsonia inermis) and its main constituents (lawsone, gallic acid, {alpha}-D-Glucose and tannic acid) on corrosion of mild steel in 1 M HCl solution was investigated through electrochemical techniques and surface analysis (SEM/EDS). Polarization measurements indicate that all the examined compounds act as a mixed inhibitor and inhibition efficiency increases with inhibitor concentration. Maximum inhibition efficiency (92.06%) is obtained at 1.2 g/l henna extract. Inhibition efficiency increases in the order: lawsone > henna extract > gallic acid > {alpha}-D-Glucose > tannic acid. Also, inhibition mechanism and thermodynamic parameters are discussed.

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

    Science.gov (United States)

    Nielsen, P A; Jaroszewski, J W; Norrby, P O; Liljefors, T

    2001-03-07

    The protonation states of a series of piperidinedicarboxylic acids (PDAs), which are conformationally constrained acidic alpha-amino acids, have been studied by (13)C 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) for 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. Furthermore, our results show that prediction of relative pK(a) values for two different types of ionizable functional groups covering a pK(a) range from 1.6 to 12.1 is possible with a high degree of accuracy.

  6. 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

  7. 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

  8. Syntheses of {gamma}-aminobutyric-1-{sup 14}C and of {alpha}-aminoadipic-6-{sup 14}C acid from methoxy-3 chloropropyl-magnesium and marked carbon dioxide; Syntheses de l'acide {gamma}-aminobutyrique{sup 14}C-1 et de l'acide {alpha}-aminoadipique {sup 14}C-6 a partir de methoxy-3 chloropropylmagnesium et d'anhydride carbonique marque

    Energy Technology Data Exchange (ETDEWEB)

    Phung Nhu Liem [Commissariat a l' Energie Atomique, Saclay (France). Centre d' Etudes Nucleaires, Departement des radioelements, Service des molecules marquees

    1967-04-01

    Carbonation of {gamma}-methoxypropyl-magnesium chloride by CO{sub 2} gives {gamma}-methoxy-butyric carboxylic-{sup 14}C acid with a yield of about 95 per cent. When the latter is treated successively with anhydrous HBr and with diazomethane, methyl carboxylic {gamma}-bromobutyrate-{sup 14}C is formed. This in turn gives {gamma}-amino-butyric carboxylic-{sup 14}C acid with an overall yield of 66 per cent with respect to Ba{sup 14}CO{sub 3}, when it is condensed with potassium phthalimide and hydrolyzed by acid. By reacting methyl-{gamma}-bromobutyrate-{sup 14}C with the sodium derivative of ethyl cyanacetamido-acetate in ethanol, followed by an acid hydrolysis, {alpha}-aminoadipic-6-{sup 14}C acid is obtained with an overall yield of 46 per cent with respect to Ba{sup 14}CO{sub 3}. (author) [French] La carbonatation du chlorure de {gamma}-methoxypropylmagnesium par {sup 14}CO{sub 2} donne l'acide {gamma}-methoxybutyrique carboxyle {sup 14}C avec un rendement d'environ 95 pour cent. Ce dernier traite successivement par HBr anhydre et par le diazomethane conduit au {gamma}-bromobutyrate de methyle carboxyle {sup 14}C. Celui-ci condense avec le phtalimide de potassium suivi d'une hydrolyse acide fournit l'acide {gamma}-aminobutyrique carboxyle {sup 14}C avec un rendement global de 66 pour cent par rapport a Ba{sup 14}CO{sub 3}. L'action du {gamma}-bromobutyrate de methyle {sup 14}C sur le derive sode du cyanacetamidoacetate d'ethyle dans l'ethanol suivie d'hydrolyse acide donne l'acide {alpha}-aminoadipique {sup 14}C-6 avec un rendement global de 46 pour cent par rapport a Ba{sup 14}CO{sub 3}. (auteur)

  9. A Comparative Study of Effects of Omega‑3 Fatty Acids, Alpha Lipoic ...

    African Journals Online (AJOL)

    Statistical analysis was carried out by paired t-test by using SPSS software version 11 (SPSS, Chicago, USA). Results: Analysis of baseline (V1) vs. end of treatment period (V2) parameters, showed significant decrease in HbA1c in the three treatment group. We also observed decrease in fasting blood glucose in the three ...

  10. Thermal stability of the human blood serum acid alpha(1)-glycoprotein in acidic media

    Czech Academy of Sciences Publication Activity Database

    Hofbauerová, Kateřina; Kopecký ml., Vladimír; Sýkora, J.; Karpenko, V.

    2003-01-01

    Roč. 103, č. 1 (2003), s. 25-33 ISSN 0301-4622 Grant - others:GA UK(CZ) No.220/2000/B-CH Institutional research plan: CEZ:AV0Z5011922; CEZ:MSM113100001; CEZ:MSM113200001; CEZ:MSM123100001 Keywords : orosomucoid * thermal stability * UV-spectroscopy Subject RIV: BO - Biophysics Impact factor: 1.728, year: 2003

  11. A Comparative Study of Effects of Omega‑3 Fatty Acids, Alpha Lipoic ...

    African Journals Online (AJOL)

    We also observed decrease in fasting blood glucose in the three treatment group but it was not statistically significant (Gr. I = 0.51, Gr. II = 0.05, Gr. III = 0.22,. Gr. IV = 0.88). Conclusion: ALA, Omega 3 fatty acid and vitamin E can be used as add on therapy in patients with type 2 diabetes mellitus to improve insulin sensitivity ...

  12. The application of glutamic acid alpha-decarboxylase for the valorization of glutamic acid

    NARCIS (Netherlands)

    Lammens, T.M.; Biase, De Daniela; Franssen, M.C.R.; Scott, E.L.; Sanders, J.P.M.

    2009-01-01

    Glutamic acid is an important constituent of waste streams from biofuels production. It is an interesting starting material for the synthesis of nitrogen containing bulk chemicals, thereby decreasing the dependency on fossil fuels. On the pathway from glutamic acid to a range of molecules, the

  13. Spectroscopic and thermodynamic studies on ferulic acid - Alpha-2-macroglobulin interaction

    Science.gov (United States)

    Rehman, Ahmed Abdur; Sarwar, Tarique; Arif, Hussain; Ali, Syed Saqib; Ahsan, Haseeb; Tabish, Mohammad; Khan, Fahim Halim

    2017-09-01

    Ferulic acid is a major phenolic acid found in numerous plant species in conjugated form. It binds to enzymes and oligomeric proteins and modifies their structure and function. This study was designed to examine the interaction of ferulic acid, an active ingredient of some important medicines, with α2M, a key serum proteinase, under physiological conditions. The mechanism of interaction was studied by spectroscopic techniques such as, UV-visible absorption, fluorescence spectroscopy, circular dichroism along with isothermal titration calorimetry. Fluorescence quenching of α2M by ferulic acid demonstrated the formation of α2M-ferulic acid complex by static quenching mechanism. Binding parameters calculated by Stern-Volmer method showed that ferulic acid binds to α2M with moderate affinity of the order of ∼104 M-1. The thermodynamic signatures reveal that binding was enthalpy driven and hydrogen bonding played a major role in ferulic acid-α2M binding. CD spectra analysis suggests very little conformational changes in α2M on ferulic acid binding.

  14. Acide alpha-linolénique, anti-oxydants et croissance tumorale mammaire

    Directory of Open Access Journals (Sweden)

    Chajes Véronique

    2000-01-01

    Full Text Available En France, comme dans la plupart des pays occidentaux, le cancer du sein est la première cause de mortalité, avec un nombre annuel de décès proche de 10 000. Chez les femmes, le cancer du sein est celui dont le taux d’incidence est le plus élevé dans la plupart des pays occidentaux. En France, il y a approximativement 35 000 nouveaux cas chaque année. Le taux d’incidence est de 80 pour 100 000 femmes/an. Il existe une disparité géographique dans les taux d’incidence du cancer du sein. Ils sont élevés dans tous les pays développés, à l’exception du Japon et de la Chine. Ajusté sur l’âge, il est le plus élevé aux États-Unis, et le plus bas en Chine, en Inde, au Japon. La France se situe à une position intermédiaire en Europe. L’incidence du cancer du sein est globalement plus faible dans les pays du Sud de l’Europe. Cette disparité géographique ne peut pas être expliquée uniquement par les facteurs génétiques. L’étude des populations migrantes, dont les taux d’incidence du cancer du sein se modifient pour atteindre celui du pays dans lequel elles migrent, a permis de mettre en évidence le rôle des facteurs environnementaux dans l’apparition du cancer du sein. Parmi ceux-là, la part de l’alimentation est estimée à environ 35 % (entre 20 à 60 %, en fonction du site du cancer [1]. Il existe donc un réel potentiel de prévention du cancer du sein, sous réserve que les aliments protecteurs soient mis en évidence et que les nutriments responsables de ces effets protecteurs soient individualisés.

  15. The role of amino acid alpha38 in the control of oxygen binding to human adult and embryonic haemoglobin Portland.

    OpenAIRE

    Zheng, T; Brittain, T; Watmough, N J; Weber, R E

    1999-01-01

    The role of the amino acid at position alpha(38) in haemoglobin has been probed using site-directed mutagenesis. When the Thr residue at position alpha(38) (which is totally conserved in all mammals) is changed to a Gln, the equilibrium properties of the protein are significantly altered. Equilibrium and kinetic data show that the R-state properties of the protein are essentially unaffected by the mutation whilst the allosteric equilibrium and T-state properties are changed. Mutation of the n...

  16. Phytanic acid alpha-oxidation: decarboxylation of 2-hydroxyphytanoyl-CoA to pristanic acid in human liver

    NARCIS (Netherlands)

    Verhoeven, N. M.; Wanders, R. J.; Schor, D. S.; Jansen, G. A.; Jakobs, C.

    1997-01-01

    The degradation of the first intermediate in the alpha-oxidation of phytanic acid, 2-hydroxyphytanoyl-CoA, was investigated. Human liver homogenates were incubated with 2-hydroxyphytanoyl-CoA or 2-hydroxyphytanic acid, after which formation of 2-ketophytanic acid and pristanic acid were studied.

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

    Directory of Open Access Journals (Sweden)

    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.

  18. Two forms of acid alpha-D-mannosidase in monkey brain: evidence for the co-existence of high mannose and complex oligosaccharides in one form.

    Science.gov (United States)

    Mathur, R; Alvares, K; Balasubramanian, A S

    1984-09-28

    Lysosomal alpha-D-mannosidase of monkey brain existed in two forms. One form of mannosidase was bound to the Ricinus communis agglutinin120 (RCA1)-Sepharose and could be specifically eluted with lactose. The other form did not bind to the RCA1-Sepharose. Both forms of mannosidase could bind to a similar extent to the immobilized brain lysosomal receptor protein. Both the forms were purified to apparent homogeneity. Neutral sugar analysis by GLC showed the presence of glucose, mannose and galactose in the RCA1-Sepharose bindable mannosidase and glucose and mannose in the non-bindable mannosidase. Several other brain lysosomal hydrolases did not bind to the RCA1-Sepharose. The results suggested the existence of only high mannose oligosaccharides in the RCA1 non-bindable mannosidase and both high mannose and complex oligosaccharides in the bindable mannosidase.

  19. Phytanic acid alpha-oxidation: accumulation of 2-hydroxyphytanic acid and absence of 2-oxophytanic acid in plasma from patients with peroxisomal disorders

    NARCIS (Netherlands)

    ten Brink, H. J.; Schor, D. S.; Kok, R. M.; Poll-The, B. T.; Wanders, R. J.; Jakobs, C.

    1992-01-01

    A stable isotope dilution method was developed for the measurement of 2-hydroxyphytanic acid and 2-oxophytanic acid in plasma. In plasma from healthy individuals and from patients with Refsum's disease, 2-hydroxyphytanic acid was found at levels less than 0.2 mumol/l, whereas the acid accumulated in

  20. Structure and Reactivity of the Glutathione Radical Cation: Radical Rearrangement from the Cysteine Sulfur to the Glutamic Acid alpha-Carbon Atom

    NARCIS (Netherlands)

    Osburn, S.; Berden, G.; Oomens, J.; Gulyuz, K.; Polfer, N.C.; O'Hair, R.A.J.; Ryzhov, V.

    2013-01-01

    A gas-phase radical rearrangement through intramolecular hydrogen-atom transfer (HAT) was studied in the glutathione radical cation, [-ECG](+.), which was generated by a homolytic cleavage of the protonated S-nitrosoglutathione. Ion-molecule reactions suggested that the radical migrates from the

  1. Stereochemistry of the peroxisomal branched-chain fatty acid alpha- and beta-oxidation systems in patients suffering from different peroxisomal disorders

    NARCIS (Netherlands)

    Ferdinandusse, S.; Rusch, H.; van Lint, A. E. M.; Dacremont, G.; Wanders, R. J. A.; Vreken, P.

    2002-01-01

    Phytanic acid (3,7,11,15-tetramethylhexadecanoic acid) is a branched-chain fatty acid derived from dietary sources and broken down in the peroxisome to pristanic acid (2,6,10,14-tetramethylpentadecanoic acid) via alpha-oxidation. Pristanic acid then undergoes beta-oxidation in peroxisomes. Phytanic

  2. High-performance liquid chromatographic separation of carminic acid, alpha- and beta-bixin, and alpha- and beta-norbixin, and the determination of carminic acid in foods.

    Science.gov (United States)

    Lancaster, F E; Lawrence, J F

    1996-05-03

    During a study of natural food colours, a simple and reliable high-performance liquid chromatography system was developed for use with cochineal and annato. An isocratic mobile phase, consisting of methanol and 6% aqueous acetic acid, resolved bixin and norbixin, while a gradient system was used to separate carminic acid and the annato compounds. The carminic acid contents of cochineal extract, carmine and carmine hydrosoluble were determined using an isocratic mobile phase (40:60, v/v). The detection limit for carminic acid in the various products was approximately 100 ng/g. Carminic acid was determined quantitatively in fruit beverages, yogurt and candies. It was demonstrated that, because of decomposition, carminic acid was not suitable for use in candies when manufacturing temperatures above 100 degrees C were required. Most membrane filters are not suitable for use with cochineal solutions, but a cellulose membrane filter did not adsorb carminic acid and was used successfully to remove impurities from water-based cochineal products and food extracts containing carminic acid.

  3. β-Peptoid Foldamers at Last

    DEFF Research Database (Denmark)

    Laursen, Jonas S; Engel-Andreasen, Jens; Olsen, Christian A

    2015-01-01

    in this journal in the late 1990s. Oligomers of N-alkylated 3-aminopropionic acid residues have been called β-peptoids due to their structural similarity to β-peptides and peptoids (N-alkylglycines), respectively. Because bona fide foldamer behavior has been demonstrated for both parent architectures, we wondered...

  4. Triangular prism-shaped β-peptoid helices as unique biomimetic scaffolds

    DEFF Research Database (Denmark)

    Laursen, Jonas Striegler; Harris, Pernille; Fristrup, Peter

    2015-01-01

    β-Peptoids are peptidomimetics based on N-alkylated β-aminopropionic acid residues (or N-alkyl-β-alanines). This type of peptide mimic has previously been incorporated in biologically active ligands and has been hypothesized to be able to exhibit foldamer properties. Here we show, for the first t...... of novel biomimetics that display functional groups with high accuracy in three dimensions, which has potential for development of new functional materials....

  5. 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....

  6. 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

    Directory of Open Access Journals (Sweden)

    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.

  7. Alpha-ketoadipic aciduria, a new inborn error of lysine metabolism; biochemical studies

    NARCIS (Netherlands)

    Przyrembel, Hildegard; Bachmann, Dorothea; Lombeck, Ingrid; Becker, K.; Wendel, U.; Wadman, S.K.; Bremer, H.J.

    1975-01-01

    Investigation of a psychomotorically retarded girl showed excretion of abnormal amounts of alpha-ketoadipic acid, alpha-hydroxyadipic acid, alpha-aminoadipic acid, 1,2-butenedicarboxylic acid and elevation of plasma alpha-aminoadipic acid levels. The identity of these metabolites was established by

  8. Food-Grade Synthesis of Maillard-Type Taste Enhancers Using Natural Deep Eutectic Solvents (NADES

    Directory of Open Access Journals (Sweden)

    Maximilian Kranz

    2018-01-01

    Full Text Available The increasing demand for healthier food products, with reduced levels of table salt, sugar, and mono sodium glutamate, reinforce the need for novel taste enhancers prepared by means of food-grade kitchen-type chemistry. Although several taste modulating compounds have been discovered in processed foods, their Maillard-type ex food production is usually not exploited by industrial process reactions as the yields of target compounds typically do not exceed 1–2%. Natural deep eutectic solvents (NADES are reported for the first time to significantly increase the yields of the taste enhancers 1-deoxy-ᴅ-fructosyl-N-β-alanyl-ʟ-histidine (49% yield, N-(1-methyl-4-oxoimidazolidin-2-ylidene aminopropionic acid (54% yield and N2-(1-carboxyethyl guanosine 5′-monophosphate (22% yield at low temperature (80–100 °C within a maximum reaction time of 2 h. Therefore, NADES open new avenues to a “next-generation culinary chemistry” overcoming the yield limitations of traditional Maillard chemistry approaches and enable a food-grade Maillard-type generation of flavor modulators.

  9. Walnut extract inhibits LPS-induced activation of BV-2 microglia via internalization of TLR4: possible involvement of phospholipase D2

    Science.gov (United States)

    Walnuts are a rich source of essential fatty acids, including the polyunsaturated fatty acids alpha-linolenic acid (ALA) and linoleic acid (LA). Essential fatty acids have been shown to modulate a number of cellular processes in the brain, including the activation state of microglia. Microglial acti...

  10. 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...

  11. Biosynthesis and transport of lysosomal alpha-glucosidase in the human colon carcinoma cell-line Caco-2: secretion from the apical surface

    NARCIS (Netherlands)

    Klumperman, J.; Fransen, J.A.; Boekestijn, J.C.; Oude Elferink, R.P.; Matter, K.; Hauri, H.P.; Tager, J.M.; Ginsel, L.A.

    1991-01-01

    The human adenocarcinoma cell line Caco-2 was used for studies on the biosynthesis and transport of lysosomal acid alpha-glucosidase in polarized epithelial cells. Metabolic labelling revealed that in Caco-2 cells alpha-glucosidase is synthesized as a precursor form of 110 x 10(3) Mr. This form is

  12. Human disorders of peroxisome metabolism and biogenesis

    NARCIS (Netherlands)

    Waterham, Hans R.; Ferdinandusse, Sacha; Wanders, Ronald J. A.

    2016-01-01

    Peroxisomes are dynamic organelles that play an essential role in a variety of cellular catabolic and anabolic metabolic pathways, including fatty acid alpha- and beta-oxidation, and plasmalogen and bile acid synthesis. Defects in genes encoding peroxisomal proteins can result in a large variety of

  13. Peroxisomes, lipid metabolism, and human disease

    NARCIS (Netherlands)

    Wanders, R. J.

    2000-01-01

    In the past few years, much has been learned about the metabolic functions of peroxisomes. These studies have shown that peroxisomes play a major role in lipid metabolism, including fatty acid beta-oxidation, etherphospholipid biosynthesis, and phytanic acid alpha-oxidation. This article describes

  14. Peroxisome proliferator-activated receptor mRNA levels are modified by dietary n-3 fatty acid restriction and energy restriction in the brain and liver of growing rats

    Science.gov (United States)

    Without dietary sources of long chain (LC) n-3 fatty acids, alpha-linolenic acid (ALA;18:3n-3) is the precursor for docosahexaenoic acid (DHA; 22:6n-3). It is not known how energy restriction (ER) impacts ALA conversion to DHA. We tested the hypothesis that ER reduces LCn-3 content in growing rats ...

  15. Enzyme therapy for pompe disease with recombinant human alpha-glucosidase from rabbit milk.

    NARCIS (Netherlands)

    Hout, J.M. van den; Reuser, A.J.J.; Klerk, J.B.C. de; Arts, W.F.M.; Smeitink, J.A.M.; Ploeg, A.T. van der

    2001-01-01

    Pompe disease is a metabolic myopathy caused by deficiency of lysosomal acid alpha-glucosidase. In this report we review the first 36 weeks of a clinical study on the safety and efficacy of enzyme therapy aimed at correcting the deficiency. Four patients with infantile Pompe disease were enrolled.

  16. Metabolism and disposition of [14C]brivanib alaninate after oral administration to rats, monkeys, and humans.

    Science.gov (United States)

    Gong, Jiachang; Gan, Jinping; Caceres-Cortes, Janet; Christopher, Lisa J; Arora, Vinod; Masson, Eric; Williams, Daphne; Pursley, Janice; Allentoff, Alban; Lago, Michael; Tran, Scott B; Iyer, Ramaswamy A

    2011-05-01

    Brivanib [(R)-1-(4-(4-fluoro-2-methyl-1H-indol-5-yloxy)-5-methylpyrrolo[1,2,4]triazin-6-yloxy)propan-2-ol, BMS-540215] is a potent and selective dual inhibitor of vascular endothelial growth factor (VEGF) and fibroblast growth factor (FGF) signaling pathways. Its alanine prodrug, brivanib alaninate [(1R,2S)-2-aminopropionic acid 2-[4-(4-fluoro-2-methyl-1H-indol-5-yloxy)-5-methylpyrrolo[2,1-f][1,2,4]triazin-6-yloxy]-1-methylethyl ester, BMS-582664], is currently under development as an oral agent for the treatment of cancer. This study describes the in vivo biotransformation of brivanib after a single oral dose of [(14)C]brivanib alaninate to intact rats, bile duct-cannulated (BDC) rats, intact monkeys, BDC monkeys, and humans. Fecal excretion was the primary route of elimination of drug-derived radioactivity in animals and humans. In BDC rats and monkeys, the majority of radioactivity was excreted in bile. Brivanib alaninate was rapidly and completely converted via hydrolysis to brivanib in vivo. The area under the curve from zero to infinity of brivanib accounted for 14.2 to 54.3% of circulating radioactivity in plasma in animals and humans, suggesting that metabolites contributed significantly to the total drug-related radioactivity. In plasma from animals and humans, brivanib was a prominent circulating component. All the metabolites that humans were exposed to were also present in toxicological species. On the basis of metabolite exposure and activity against VEGF and FGF receptors of the prominent human circulating metabolites, only brivanib is expected to contribute to the pharmacological effects in humans. Unchanged brivanib was not detected in urine or bile samples, suggesting that metabolic clearance was the primary route of elimination. The primary metabolic pathways were oxidative and conjugative metabolism of brivanib.

  17. 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 up......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...... acts as a mixing vessel between human and avian influenza viruses. Furthermore, it was shown that AIV prefers to infect alveolar type II epithelial cells in pigs. This corresponds with findings in humans emphasising the resemblance between the two species....

  18. Exploring Triacylglycerol Biosynthetic Pathway in Developing Seeds of Chia (Salvia hispanica L.): A Transcriptomic Approach

    OpenAIRE

    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...

  19. Studies of the acidic components of the Colorado Green River formation oil shale-Mass spectrometric identification of the methyl esters of extractable acids.

    Science.gov (United States)

    Haug, P.; Schnoes, H. K.; Burlingame, A. L.

    1971-01-01

    Study of solvent extractable acidic constituents of oil shale from the Colorado Green River Formation. Identification of individual components is based on gas chromatographic and mass spectrometric data obtained for their respective methyl esters. Normal acids, isoprenoidal acids, alpha, omega-dicarboxylic acids, mono-alpha-methyl dicarboxylic acids and methyl ketoacids were identified. In addition, the presence of monocyclic, benzoic, phenylalkanoic and naphthyl-carboxylic acids, as well as cycloaromatic acids, is demonstrated by partial identification.

  20. Synthesis and coupling reactions of alpha,alpha-dialkylated amino acids with nucleobase side chains.

    OpenAIRE

    Azumaya, I; Aebi, R; Kubik, S; Rebek, J

    1995-01-01

    Several di- and tripeptides containing protected purine (adenine) and pyrimidine (thymine) residues on their side chains were synthesized. The parent amino acids alpha, alpha-dialkylated in a symmetrical manner. An effective coupling procedure was developed for these sterically hindered amino acids: the fluoren-9-ylmethyloxycarbonyl-protected amino acid was dehydrated to its oxazolinone form, which was coupled in good yields with amino esters in hot tetrachloroethane.

  1. Omega-6 and omega-3 fatty acid nutrition amongst Malaysians are far from desirable

    OpenAIRE

    Tony Kock Wai Ng; Sivalingam Nalliah; Azlinda Hamid; Siew Rong Wong; Sim Ling Chee; Cheryl Andrea Augustine

    2012-01-01

    This paper reviews available reports on theomega-6 (linoleic acid, LA) and omega-3 fatty acid[alpha-linolenic acid (ALA) + eicosapentaenoic acid(EPA) + docosahexaenoic acid) intakes amongstMalaysians against Malaysian Recommended NutrientIntakes (RNI), focussing particularly on pregnant andlactating women because of the availability of data forthese latter vulnerable groups. Overall, the omega-6 andomega-3 fatty acid nutrition amongst Malaysians arepoor and far from desirable. The nutritional...

  2. 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.

  3. 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...... of carotenoid supplementation and spinach intake on erythrocyte enzyme antioxidant activities, serum or plasma nonenzymatic antioxidant concentrations, and concentrations of oxidatively damaged amino acids in plasma; Subjects received for 3 wk a basic diet (n = 10), a basic diet with a carotenoid supplement (n...

  4. Characterization of lysosomes and lysosomal enzymes from Chediak-Higashi-syndrome cultured fibroblasts.

    Science.gov (United States)

    Miller, A L; Stein, R; Sundsmo, M; Yeh, R Y

    1986-01-01

    Chediak-Higashi-syndrome cultured skin fibroblasts were used to study the possible involvement of lysosomal enzymes and lysosomal dysfunction in this disorder. Our evidence indicated that Chediak-Higashi fibroblasts displayed a significant decrease in the specific activity of the acidic alpha-D-mannosidase (pH 4.2) compared with normal controls. Additional studies revealed a small, but significant, decrease in the rate of degradation of 125I-labelled beta-D-glucosidase that had been endocytosed into Chediak-Higashi cells. PMID:3099770

  5. 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...

  6. 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.)

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

    International Nuclear Information System (INIS)

    Del Gaizo, Andrew; Banerjee, Sima; Terk, Michael

    2009-01-01

    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.)

  8. 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)

  9. A New Mutation Causing Severe Infantile-Onset Pompe Disease Responsive to Enzyme Replacement Therapy

    Directory of Open Access Journals (Sweden)

    Hossein Moravej

    2018-03-01

    Full Text Available Pompe disease (PD, also known as “glycogen storage disease type II (OMIM # 232300” is a rare autosomal recessive disorder characterized by progressive glycogen accumulation in cellular lysosomes. It ultimately leads to cellular damage. Infantile-onset Pompe disease (IOPD is the most severe type of this disease and is characterized by severe hypertrophic cardiomyopathy and generalized hypotonia. Mutations in the acid alpha-glucosidase (GAA gene, located at locus 17q25.3, are responsible for the disease leading to reduced activity of the acid alpha-glucosidase enzyme. To date, approximately 400 pathogenic mutations have been reported in the GAA gene. The aim of this study is to report a novel nonsense mutation in exon 4 of the GAA gene in an Iranian child suffering from IOPD. The patient was a female neonate with hypertrophic cardiomyopathy and a positive family history of IOPD. After definite diagnosis, enzyme-replacement therapy (ERT was started for the patient, who was 2 months old. Now at the age of 20 months, she has had good growth and development and her echocardiographic parameters are within the normal range. This report shows that IOPD patients with this mutation can be treated with ERT successfully.

  10. Pompe disease: clinical perspectives

    Directory of Open Access Journals (Sweden)

    Cabello JF

    2016-12-01

    Full Text Available Juan Francisco Cabello,1 Deborah Marsden21Genetics and Metabolic Disease Laboratory, Nutrition and Food Technology Institute (INTA, University of Chile, Santiago, Chile; 2Division of Genetics and Genomics, Boston Children's Hospital, Boston, MA, USA Abstract: Pompe disease (acid alpha-glucosidase deficiency, OMIM 232300 is a rare lysosomal storage disorder due to autosomal recessive mutations in the GAA gene. It has also been called acid maltase deficiency and glycogen storage disease type II. There is a broad clinical presentation: the most severe form that presents in the first few months of life with cardiomyopathy and generalized muscle weakness that rapidly progresses to death from cardio-respiratory failure in the first year of life (infant-onset Pompe disease. A more slowly progressive disease, with little or no cardiac involvement, presents with proximal myopathy and/or pulmonary insufficiency, from the second year of life to late adulthood (late-onset Pompe disease. The recent development and introduction of enzyme replacement therapy with intravenous infusion of recombinant human acid alpha-glucosidase have made a major improvement in the morbidity and mortality of this disease. New therapies are also in development. With the availability of treatment, diagnostic methods have also improved, allowing for earlier recognition and potential early therapeutic intervention. The advent of newborn screening for Pompe disease may identify patients who can be treated before significant irreversible disease has occurred. Keywords: Pompe disease, glycogen storage disease, lysosomal storage disease, enzyme replacement therapy, gene therapy, chaperone therapy, genotype/phenotype, newborn screening

  11. 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.

  12. [Clinical sequelae of 17 cases with glycogen storage disease type II/Pompe disease].

    Science.gov (United States)

    Zhang, Han-bing; Zhang, Wei-min; Qiu, Jia-jing; Meng, Yan; Qiu, Zheng-qing

    2012-06-01

    To analyze and summarize the characteristics of glycogen storage disease type II (Pompe disease) patients according to the clinical description and prognosis. 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 reach the objective. Four of 17 patients diagnosed by acid alpha-glucosidase are infantile-onset, symptoms started between 2 to 6 months after birth with increased serum creatine kinase and cardiac problems, with or without respiratory concerns. Other 13 patients were later-onset cases, and their symptoms started between 2 to 22 years of age with increased serum creatine kinase. Eleven later-onset patients started with muscle weakness, 2 patients developed respiratory insufficiency, 2 patients showed scoliosis, and 1 patient expressed increased serum creatine kinase with abnormal liver function. Just 3 of the later-onset patients were treated with mechanical ventilator and adjuvant therapy, others were not. All patients' acid alpha-glucosidase (GAA) enzyme activity analysis showed lower than 10% of normal. Fourteen patients were tested by muscle biopsy pathology, and 9 of them progressed to glycogen storage disease type II; 10 patients received genetic analysis, and 6 of them had two mutations which cause the disorder. Twelve of the 17 patients were interviewed successfully. In 3 of the infant-onset patients the disease resulted in death from respiratory failure, and 1 is still alive at the age of 1 year and 7 months. In 4 of 8 later-onset patients the disease resulted in death from respiratory failure between 3 to 5 years after onset of symptoms. Three of 4 survivors had increased muscle weakness, and 1 patient kept alive with ventilator without any changes. Seven of 12 interviewed patients died, the mortality rate was 58.3%. Glycogen storage disease type II (Pompe disease) present differently in the

  13. 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

    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....... RESULTS: Stearic acid intake was linearly associated with weight gain (P 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......) and 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...

  14. Characterization of lymphoid cells in the blood of healthy adults: sequential immunological, cytochemical and cytokinetic studies

    International Nuclear Information System (INIS)

    Hirt, A.; Wagner, H.P.

    1980-01-01

    With a new method, sequential immunological, cytochemical and cytokinetic studies were done on lymphoid cells in the peripheral blood of 12 healthy adults. Every single lymphoid cell could therefore be characterized by the following markers: surface immunoglobulins (sIg); rosetting with sheep red blood cells (E); unspecific acid alpha-naphthyl acetate esterase (ANAE); and 3HdT incorporation. Significantly more E+sIg-ANAE-cells (51% and 22% of all lymphoid cells, respectively). Of all ANAE+ cells 90% were E+, but 64% of all ANAE- cells were also E+. In all individuals a subpopulation of E+sIg+ cells was found. The esterase pattern of these cells was similar to that of E-sIg+ cells. The overall labeling index of the lymphoid cells examined was less than or equal to 0.2%

  15. [Effect of auxins on production of coumarin in a suspension culture of Angelica archangelica L].

    Science.gov (United States)

    Siatka, T; Kasparová, M

    2003-07-01

    The paper examined the effect of selected auxins (2,4-dichlorophenoxyacetic acid, alpha-naphthalene-acetic acid, beta-indoleacetic acid, beta-indoleburytic acid; each in four concentrations--0.2, 2, 10, and 20 mg/l) on the production of coumarins in the suspension culture of Angelica archangelica L. cultinated in the dark and under permanent lighting(3500 lux). The effect of the light regimen is, in comparison with auxins, less marked--the content of coumarins is mostly comparable both under permanent lighting and in the dark. The highest coumarin content was achieved with the use of alpha-naphthalene-acetic acid in a concentration of 0.2 mg/l with cultivation in the dark.

  16. 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...... and erythrocyte enzyme activities were assessed, and differences among experimental groups were tested. Consumption of spinach resulted in greater (P 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...

  17. In vitro antiplasmodial investigation of medicinal plants from El Salvador.

    Science.gov (United States)

    Köhler, Inga; Jenett-Siems, Kristina; Siems, Karsten; Hernández, Marco Antonio; Ibarra, Ricardo A; Berendsohn, Walter G; Bienzle, Ulrich; Eich, Eckart

    2002-01-01

    In vitro antiplasmodial activities of extracts from Albizia saman, Fabaceae, Calea tenuifolia (C. zacatechichi), Asteraceae, Hymenaea courbaril, Fabaceae, Jatropha curcas, Euphorbiaceae, Momordica charantia, Cucurbitaceae, and Moringa oleifera, Moringaceae were evaluated. From the lipophilic extract of C tenuifolia five active flavones were obtained. 4',5-Dihydroxy-7-methoxyflavone [genkwanin] and 5-hydroxy-4',7-dimethoxyflavone [apigenin 4',7-dimethylether] exhibited the strongest antiplasmodial activity against a chloroquine-sensitive strain (poW) and a chloroquine-resistant strain (Dd2) of Plasmodium falciparum [IC50 values: 17.1-28.5 microM). Furthermore octadeca-9,12-dienoic acid [linoleic acid] [IC50] values of 21.8 microM (poW) and 31.1 microM (Dd2)] and octadeca-9,12,15-trienoic acid (alpha-linolenic acid) were isolated.

  18. 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.

  19. Impact of a Central Scaffold on the Binding Affinity of Fragment Pairs Isolated from DNA-Encoded Self-Assembling Chemical Libraries.

    Science.gov (United States)

    Bigatti, Martina; Dal Corso, Alberto; Vanetti, Sara; Cazzamalli, Samuele; Rieder, Ulrike; Scheuermann, Jörg; Neri, Dario; Sladojevich, Filippo

    2017-11-08

    The screening of encoded self-assembling chemical libraries allows the identification of fragment pairs that bind to adjacent pockets on target proteins of interest. For practical applications, it is necessary to link these ligand pairs into discrete organic molecules, devoid of any nucleic acid component. Here we describe the discovery of a synergistic binding pair for acid alpha-1 glycoprotein and a chemical strategy for the identification of optimal linkers, connecting the two fragments. The procedure yielded a set of small organic ligands, the best of which exhibited a dissociation constant of 9.9 nm, as measured in solution by fluorescence polarization. © 2017 Wiley-VCH Verlag GmbH & Co. KGaA, Weinheim.

  20. Type of vegetable oils used in cooking and risk of metabolic syndrome among Asian Indians.

    Science.gov (United States)

    Lakshmipriya, Nagarajan; Gayathri, Rajagopal; Praseena, Kallingal; Vijayalakshmi, Parthasarathy; Geetha, Gunasekaran; Sudha, Vasudevan; Krishnaswamy, Kamala; Anjana, Ranjit Mohan; Henry, Jeyakumar; Mohan, Viswanathan

    2013-03-01

    There is little data on the type of vegetable oil used and the prevalence of metabolic syndrome (MS) in Asian Indians. Food frequency questionnaire was used to document the type of cooking oil in 1875 adults in Chennai city. MS was assessed by new harmonizing criteria. The prevalence of MS was higher among sunflower oil users (30.7%) than palmolein (23.2%) and traditional oil (17.1%, p < 0.001) users. The higher prevalence of MS in sunflower oil group persisted even when stratified according to body mass index, except in obese groups. The risk of MS was further compounded by quantity of refined cereals consumed. Higher LA%E and linoleic acid/alpha-linolenic acid ratio in sunflower oil probably contributes to increased risk of MS.

  1. L’apport de lin dans la ration des vaches laitières : Effets sur la production, la composition du lait et des produits laitiers, les émissions de méthane et les performances de reproduction

    OpenAIRE

    Brunschwig, Philippe; Hurtaud, Catherine; Chilliard, Yves; Glasser, Frederic

    2010-01-01

    La supplémentation en lin des rations des vaches laitières est une pratique qui se développe, avec pour objectifs l'amélioration de la qualité nutritionnelle du lait et la diminution des rejets de méthane. En effet, la recherche de laits moins riches en Acides Gras (AG) saturés et avec un rapport plus faible entre acide linoléique et acide linolénique, incite à utiliser des aliments riches en acides gras polyinsaturés (et en particulier en acide alpha-linolénique C18:3 n-3) pour corriger des ...

  2. 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.

  3. Caractéristiques des huiles de lin et de chanvre

    Directory of Open Access Journals (Sweden)

    Morin Odile

    2015-11-01

    Full Text Available Les huiles de lin et de chanvre appartiennent à la famille des huiles riches en acides gras polyinsaturés contenant de l’acide alpha-linolénique (ALA. Le contexte actuel du déficit d’apport en ALA, précurseur indispensable des AGPI de la série des omégas 3 (ou n-3, comme en ses dérivés métaboliques supérieurs (EPA, DHA, fonde le regain d’intérêt pour les sources apportant le précurseur de ces acides gras essentiels. Les autorisations de commercialisation de l’huile de chanvre ou l’évolution réglementaire dans le cas de l’huile de lin en France, ont tenu compte de cet atout nutritionnel pourvu que les conditions de stabilité en conservation soient garanties.

  4. Pyrolysis of amino acids - Mechanistic considerations

    Science.gov (United States)

    Ratcliff, M. A., Jr.; Medley, E. E.; Simmonds, P. G.

    1974-01-01

    Pyrolysis of several structurally different amino acids in a column at 500 C showed differences in the mechanisms and final products. The aliphatic protein amino acids decompose mainly by simple decarboxylation and condensation reactions, while the beta amino acids undergo deamination to unsaturated acids. Alpha amino acids with alpha alkyl substituents undergo an unusual intramolecular SN1 reaction with the formation of an intermediate alpha lactone which decomposes to yield a ketone. The alpha alkyl substituents appear to stabilize the developing negative charge formed by partial heterolytic cleavage of the alpha carbon - NH3 bond. The gamma and delta amino acids give 2-pyrrolidinone and 2-piperidone respectively, while the epsilon acids yield mixed products.

  5. 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.

  6. Cloning and chromosomal localization of the three human syntrophin genes

    Energy Technology Data Exchange (ETDEWEB)

    Feener, C.A.; Anderson, M.D.S.; Selig, S. [Children`s Hospital, Boston, MA (United States)] [and others

    1994-09-01

    Dystrophin, the protein product the Duchenne muscular dystrophy locus, is normally found to be associated with a complex of proteins. Among these dystrophin-associated proteins are the syntrophins, a group of 59 kDa membrane-associated proteins. When the syntrophins are purified based upon their association with dystrophin, they have been shown previously to form two distinct groups, the acidic ({alpha}) and basic ({beta}) forms. Based on peptide and rodent cDNA sequences, three separate syntrophin genes have been cloned and characterized from human tissues. The predicted amino acid sequences from these cDNA reveal that these proteins are related but are distinct with respect to charge, as predicted from their biochemistry. The family consists of one acidic ({alpha}-syntrophin, analogous to mouse syntrophin-1) and two basic ({beta}{sub 1}-syntrophin; and {beta}{sub 2}-syntrophin, analogous to mouse syntrophin-2) genes. Each of the three genes are widely expressed in a variety of human tissues, but the relative abundance of the three are unique with respect to each other. {alpha}-syntrophin is expressed primarily in skeletal muscle and heart as a single transcript. {beta}{sub 1}-syntrophin is expressed widely in up to five distinct transcript sizes, and is most abundant in brain. The human chromosomal locations of the three syntrophins are currently being mapped. {beta}{sub 1}-syntrophin maps to chromosome 8q23-24 and {beta}{sub 2}-syntrophin to chromosome 16. The {alpha}-syntrophin gene will be mapped accordingly. Although all three genes are candidates for neuromuscular diseases, the predominant expression of {alpha}-syntrophin in skeletal muscle and heart makes it a strong candidate to be involved in a neuromuscular disease.

  7. Ultrastructural immunolocalization of alpha-keratins and associated beta-proteins (beta-keratins) suggests a new interpretation on the process of hard and soft cornification in turtle epidermis.

    Science.gov (United States)

    Alibardi, L

    2013-01-01

    The epidermis of soft-shelled and hard-shelled turtles has been compared to determine the origin of the different cornification. Immunolocalization of acidic alpha-keratin (AK2) of 45-50 kDa in tonofilaments of the epidermis in Apalone spinifera and absence in the corneous layer where desquamating corneocytes are present supports the biochemical data. Corneocytes shows a weak to absent immunolabeling for beta-proteins (formerly beta-keratins) of 14-16 kDa while sparse immunolabeled corneous granules are seen in the pre-corneous layer. In the hard-shelled turtle Pseudemys nelsonii differentiating corneocytes contain small level of acidic alpha-keratin while beta-proteins of 10-17 kDa form dense aggregates of corneous material among tonofilaments. Corneocytes do not desquamate but remain tightly connected determining an increase in thickness of the corneous layer that becomes mechanically stiff and resistant. Since both species possess beta-proteins in shelled and non-shelled areas of the epidermis the difference in hardness of the corneous layer is not due to the alternation between beta-keratin versus alpha-keratin. Mechanical resilience of the corneous layer derives from the accumulation of alpha-keratins, beta- and likely of other proteins in corneocytes of the shell in hard-shelled turtles. In the softer epidermis of hard-shelled turtles and in the soft-shelled turtle a more rapid and continuous turnover of corneocytes is present and no accumulation of beta-proteins and corneocytes takes place. It is hypothesized that the dermis derived from the carapacial ridge during development remains localized underneath the shell epidermis in hard-shelled turtles and influences the formation of the hard corneous epidermis. Copyright © 2013 Elsevier Ltd. All rights reserved.

  8. Rheology as a Tool to Predict the Release of Alpha-Lipoic Acid from Emulsions Used for the Prevention of Skin Aging

    Science.gov (United States)

    Isaac, Vera Lucia Borges; Chiari-Andréo, Bruna Galdorfini; Marto, Joana Marques; Moraes, Jemima Daniela Dias; Leone, Beatriz Alves; Corrêa, Marcos Antonio; Ribeiro, Helena Margarida

    2015-01-01

    The availability of an active substance through the skin depends basically on two consecutive steps: the release of this substance from the vehicle and its subsequent permeation through the skin. Hence, studies on the specific properties of vehicles, such as their rheological behavior, are of great interest in the field of dermatological products. Recent studies have shown the influence of the rheological features of a vehicle on the release of drugs and active compounds from the formulation. In this context, the aim of this study was to evaluate the influence of the rheological features of two different emulsion formulations on the release of alpha-lipoic acid. Alpha-lipoic acid (ALA) was chosen for this study because of its antioxidant characteristics, which could be useful for the prevention of skin diseases and aging. The rheological and mechanical behavior and the in vitro release profile were assayed. The results showed that rheological features, such as viscosity, thixotropy, and compliance, strongly influenced the release of ALA from the emulsion and that the presence of a hydrophilic polymer in one of the emulsions was an important factor affecting the rheology and, therefore, the release of ALA. PMID:26788510

  9. Cloning of human basic A1, a distinct 59-kDa dystrophin-associated protein encoded on chromosome 8q23-24

    Energy Technology Data Exchange (ETDEWEB)

    Ahn, A.H. [Harvard Medical School, Boston, MA (United States); Yoshida, Mikiharu; Hagiwara, Yasuko; Ozawa, Eijiro [National Institute of Neuroscience, Ogawa Higashi, Kodaira (Japan); Anderson, M.S.; Feener, C.A.; Selig, S. [Howard Hughes Medical Institute at Children`s Hospital, Boston, MA (United States); Kunkel, L.M. [Harvard Medical School, Boston, MA (United States)]|[Howard Hughes Medical Institute at Children`s Hosptial, Boston, MA (United States)

    1994-05-10

    Duchenne and Becker muscular dystrophies are caused by defects of dystrophin, which forms a part of the membrane cytoskeleton of specialized cells such as muscle. It has been previously shown that the dystrophin-associated protein A1 (59-kDa DAP) is actually a heterogeneous group of phosphorylated proteins consisting of an acidic ({alpha}-A1) and a distinct basic ({beta}-A1) component. Partial peptide sequence of the A1 complex purified from rabbit muscle permitted the design of oligonucleotide probes that were used to isolate a cDNA for one human isoform of A1. This cDNA encodes a basic A1 isoform that is distinct from the recently described syntrophins in Torpedo and mouse and is expressed in many tissues with at least five distinct mRNA species of 5.9, 4.8, 4.3, 3.1, and 1.5 kb. A comparison of the human cDNA sequence with the GenBank expressed sequence tag (EST) data base has identified a relative from human skeletal muscle, EST25263, which is probably a human homologue of the published mouse syntrophin 2. The authors have mapped the human basic component of A1 and EST25263 genes to chromosomes 8q23-24 and 16, respectively.

  10. Prevalence of adult Pompe disease in patients with proximal myopathic syndrome and undiagnosed muscle biopsy.

    Science.gov (United States)

    Golsari, Amir; Nasimzadah, Arzoo; Thomalla, Götz; Keller, Sarah; Gerloff, Christian; Magnus, Tim

    2018-03-01

    We examined patients with limb-girdle muscle weakness and/or hyper-CKaemia and undiagnosed muscle biopsy for late onset Pompe disease (LOPD). Patients with an inconclusive limb-girdle muscle weakness who presented at our neuromuscular centre between 2005 and 2015 with undiagnosed muscle biopsies were examined by dry blood spot testing (DBS) including determination of the enzyme activity of acid alpha-glucosidase (GAA). In the case of depressed enzyme activity, additional gene testing of the GAA gene was carried out. Of the 340 evaluated muscle biopsies, 69 patients fulfilled the inclusion criteria and were examined with DBS. Among those patients, 76% showed a limb-girdle muscle weakness and 14% showed a hyper-CKaemia. A diagnosis of LOPD could be established in the case of two patients (2.9%) with reduced GAA enzyme activity and proof of mutations in the GAA gene. One of the two patients presents in the muscle biopsy suggestive features of Pompe disease including vacuoles with positive acid phosphatase reaction. In summary, our results show that a muscle biopsy can be helpful in identifying LOPD patients, but vacuolation with glycogen storage can also be absent. An inconspicuous muscle biopsy does not rule out Pompe disease. Consequently, all patients with limb-girdle muscle weakness should be examined by DBS before conducting a muscle biopsy. Copyright © 2017 Elsevier B.V. All rights reserved.

  11. Relationship of total serum sialic acid to sialylglycoprotein acute-phase reactants in malignant melanoma.

    Science.gov (United States)

    Silver, H. K.; Karim, K. A.; Salinas, F. A.

    1980-01-01

    Reported elevations of total serum sialic acid may be a result of shed tumour-related membrane sialyglycoprotein and/or concurrent elevation of non-specific, acute-phase reactant sialoglycoprotein. To clarify further the specificity and sensitivity of serum sialic acid monitoring, analyses of sialic acid by the thiobarbituric acid method and acute-phase reactants by radial immunodiffusion were made using the same malignant melanoma patients' sera. Preliminary studies of IgG, IgA, IgM, ceruloplasmin and C-reactive protein suggested that these would not be valuable monitors of tumour burden. Single serum samples from 59 melanoma patients and age- and sex-matched controls were further examined for sialic acid, alpha 1-acid glycoprotein, alpha 1-antitrypsin, haptoglobin, and alpha 2-macroglobulin. Patients were grouped according to tumour burden. In pairwise statistical tests, differences between groups tended to be greater for sialic acid than for acute-phase reactants. On discriminant analysis , sialic acid was clearly the most significant single discriminator between groups, with an F statistic of P < 0.00005. Although alpha 1-acid glycoprotein was quite strongly correlated with sialic acid, it was not such a good discriminator and did not add significantly to the predictive power of sialic acid alone. PMID:6158966

  12. ED50 AP block predictions for phenyl substituted and unsubstituted n-alkanols.

    Science.gov (United States)

    Hahin, R; Kondratiev, A

    2001-03-15

    A series of n-alkanols and phenyl-substituted n-alkanols (phi-alkanols) of increasing chain length and phenol were characterized for their ability to block action potentials (APs) in frog sciatic nerves. APs were recorded using the single sucrose-gap method. The degree of AP attenuation when the nerve was exposed to different concentrations of an alcohol was used to construct dose-response curves. The reciprocals of the half-blocking doses (ED50s) were used to obtain a measure of the potency of the alcohols. For n-alkanols and phi-alkanols, increasing the chain length by the addition of a methylene group increased the potency on average by 3.1 for both groups of alkanols. The addition of a phenyl group caused a potency increase that ranged between the values of 77 and 122. The ED50 for both groups of alkanols could not be solely predicted by the log octanol-water partition coefficient (Kow). Using linear solvation energy relations (LSER), the log ED50 could be described as a linear combination of the intrinsic (van der Waals) molar volume (VI), polarity (P), and hydrogen bond acceptor basicity (beta) and donor acidity (alpha). Size alone could not predict the ED50 for both n-alkanols and phi-alkanols. The results are consistent with the hypothesis that alkanols bind to and interact with Na channels to cause AP block. Phenyl group addition to an alkanol markedly increases the molecule's potency.

  13. Novel Synthesis of Core-Shell Silica Nanoparticles for the Capture of Low Molecular Weight Proteins and Peptides

    Directory of Open Access Journals (Sweden)

    Sergio G. Hernandez-Leon

    2017-10-01

    Full Text Available Silica nanoparticles were functionalized with immobilized molecular bait, Cibacron Blue, and a porous polymeric bis-acrylamide shell. These nanoparticles represent a new alternative to capture low molecular weight (LMW proteins/peptides, that might be potential biomarkers. Functionalized core-shell silica nanoparticles (FCSNP presented a size distribution of 243.9 ± 11.6 nm and an estimated surface charge of −38.1 ± 0.9 mV. The successful attachment of compounds at every stage of synthesis was evidenced by ATR-FTIR. The capture of model peptides was determined by mass spectrometry, indicating that only the peptide with a long sequence of hydrophobic amino acids (alpha zein 34-mer interacted with the molecular bait. FCSNP excluded the high molecular weight protein (HMW, BSA, and captured LMW proteins (myoglobin and aprotinin, as evidenced by SDS-PAGE. Functionalization of nanoparticles with Cibacron Blue was crucial to capture these molecules. FCSNP were stable after twelve months of storage and maintained a capacity of 3.1–3.4 µg/mg.

  14. Engineering oilseed plants for a sustainable, land-based source of long chain polyunsaturated fatty acids.

    Science.gov (United States)

    Damude, Howard G; Kinney, Anthony J

    2007-04-01

    Numerous clinical studies have demonstrated the cardiovascular and mental health benefits of including very long chain omega-3 polyunsaturated fatty acids, namely eicospentaenoic acid (EPA) and docosohexaenoic acid (DHA) in the human diet. Certain fish oils can be a rich source of omega-3 long chain polyunsaturated fatty acids although processed marine oils are generally undesirable as food ingredients because of the associated objectionable flavors and contaminants that are difficult and cost-prohibitive to remove. Oilseed plants rich in omega-3 fatty acids, such as flax and walnut oils, contain only the 18-carbon omega-3 polyunsaturated fatty acid alpha-linolenic acid, which is poorly converted by the human body to EPA and DHA. It is now possible to engineer common omega-6 rich oilseeds such as soybean and canola to produce EPA and DHA and this has been the focus of a number of academic and industrial research groups. Recent advances and future prospects in the production of EPA and DHA in oilseed crops are discussed here.

  15. Effects of soybean oil on plasma lipoproteins and cardiovascular risk in men and women

    Directory of Open Access Journals (Sweden)

    Lecerf Jean-Michel

    2002-03-01

    Full Text Available Indépendamment des phytostérols et des autres composants de la graine (protéines, isoflavones, l’huile de soja présente plusieurs autres atouts qui lui confèrent des effets préventifs vis-à-vis du risque cardiovasculaire et des maladies coronariennes. Elle contient 61% d’acides gras polyinsaturés et seulement 15% de saturés, et ne comporte pas d’acide gras trans : sa consommation peut donc permettre une réduction du cholestérol total et du cholestérol LDL. C’est également une huile riche en vitamine E, et particulièrement en gamma tocophérol dont l’activité antioxydante connaît aujourd’hui un regain d’intérêt. Enfin, sa teneur élevée en acide alpha-linolénique est compatible avec des propriétés antithrombotique et antiarrythmique, mais la faible biodisponibilité de cet acide gras dans l’huile de soja en limite peut-être les effets.

  16. Production of a functional human acid maltase in tobacco seeds: biochemical analysis, uptake by human GSDII cells, and in vivo studies in GAA knockout mice.

    Science.gov (United States)

    Martiniuk, Frank; Reggi, Serena; Tchou-Wong, Kam-Meng; Rom, William N; Busconi, Matteo; Fogher, Corrado

    2013-10-01

    Genetic deficiency of acid alpha glucosidase (GAA) results in glycogen storage disease type II (GSDII) or Pompe's disease. To investigate whether we could generate a functional recombinant human GAA enzyme (tobrhGAA) in tobacco seeds for future enzyme replacement therapy, we subcloned the human GAA cDNA into the plant expression plasmid-pBI101 under the control of the soybean β-conglycinin seed-specific promoter and biochemically analyzed the tobrhGAA. Tobacco seeds contain the metabolic machinery that is more compatible with mammalian glycosylation-phosphorylation and processing. We found the tobrhGAA to be enzymatically active was readily taken up by GSDII fibroblasts and in white blood cells from whole blood to reverse the defect. The tobrhGAA corrected the enzyme defect in tissues at 7 days after a single dose following intraperitoneal (IP) administration in GAA knockout (GAA(-/-)) mice. Additionally, we could purify the tobrhGAA since it bound tightly to the matrix of Sephadex G100 and can be eluted by competition with maltose. These data demonstrate indirectly that the tobrhGAA is fully functional, predominantly proteolytically cleaved and contains the minimal phosphorylation and mannose-6-phosphate residues essential for biological activity.

  17. Parameters contributing to efficient ion generation in aerosol MALDI mass spectrometry.

    Science.gov (United States)

    McJimpsey, Erica L; Jackson, William M; Lebrilla, Carlito B; Tobias, Herbert; Bogan, Michael J; Gard, Eric E; Frank, Matthias; Steele, Paul T

    2008-03-01

    The Bioaerosol Mass Spectrometry (BAMS) system was developed for the real-time detection and identification of biological aerosols using laser desorption ionization. Greater differentiation of particle types is desired; consequently MALDI techniques are being investigated. The small sample size ( approximately 1 microm3), lack of substrate, and ability to simultaneously monitor both positive and negative ions provide a unique opportunity to gain new insight into the MALDI process. Several parameters known to influence MALDI molecular ion yield and formation are investigated here in the single particle phase. A comparative study of five matrices (2,6-dihydroxyacetophenone, 2,5-dihydroxybenzoic acid, alpha-cyano-4-hydroxycinnamic acid, ferulic acid, and sinapinic acid) with a single analyte (angiotensin I) is presented and reveals effects of matrix selection, matrix-to-analyte molar ratio, and aerosol particle diameter. The strongest analyte ion signal is found at a matrix-to-analyte molar ratio of 100:1. At this ratio, the matrices yielding the least and greatest analyte molecular ion formation are ferulic acid and alpha-cyano-4-hydroxycinnamic acid, respectively. Additionally, a significant positive correlation is found between aerodynamic particle diameter and analyte molecular ion yield for all matrices. SEM imaging of select aerosol particle types reveals interesting surface morphology and structure.

  18. Relationships between botanical and chemical composition of forages: a multivariate approach to grasslands in the Western Italian Alps.

    Science.gov (United States)

    Ravetto Enri, Simone; Renna, Manuela; Probo, Massimiliano; Lussiana, Carola; Battaglini, Luca M; Lonati, Michele; Lombardi, Giampiero

    2017-03-01

    Plant composition of species-rich mountain grasslands can affect the sensorial and chemical attributes of dairy and meat products, with implications for human health. A multivariate approach was used to analyse the complex relationships between vegetation characteristics (botanical composition and plant community variables) and chemical composition (proximate constituents and fatty acid profile) in mesophilic and dry vegetation ecological groups, comprising six different semi-natural grassland types in the Western Italian Alps. Mesophilic and dry grasslands were comparable in terms of phenology, biodiversity indices and proportion of botanical families. The content of total fatty acids and that of the most abundant fatty acids (alpha-linolenic, linoleic and palmitic acids) were mainly associated to nutrient-rich plant species, belonging to the mesophilic grassland ecological group. Mesophilic grasslands showed also higher values of crude protein, lower values of fibre content and they were related to higher pastoral values of vegetation compared to dry grasslands. The proximate composition and fatty acid profile appeared mainly single species dependent rather than botanical family dependent. These findings highlight that forage from mesophilic grasslands can provide higher nutritive value for ruminants and may be associated to ruminant-derived food products with a healthier fatty acid profile. © 2016 Society of Chemical Industry. © 2016 Society of Chemical Industry.

  19. 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

  20. 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.)

  1. 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.

  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. Identification a novel mononucleotide deletion mutation in GAA in pompe disease patients

    Directory of Open Access Journals (Sweden)

    Milad Ebrahimi

    2017-01-01

    Full Text Available Background: Mutations in the acid alpha-glucosidase (GAA gene usually lead to reduced GAA activity. In this study, we analyzed the mutations of GAA and GAA enzyme activity from one sibling suspected Pompe disease and their first-degree relatives. Materials and Methods: In this cross-sectional study, GAA enzyme activity assay was assessed using tandem mass spectrometry. Polymerase chain reaction and Sanger sequencing were performed for GAA analysis. Results: GAA enzyme activity was significantly decreased in patients compared to the normal range (P = 0.02. Two individuals showed ten alterations in the GAA sequence, in which one of them (c. 1650del G has not been previously described in the literature. A single Guanine deletion (del-G was detected at codon 551 in exon 12. Conclusion: According to the literature, the detected change is a novel mutation. We hypothesized that the discovered deletion in the GAA might lead to a reduced activity of the gene product.

  4. Quinazoline antifolates inhibiting thymidylate synthase: synthesis of four oligo(L-gamma-glutamyl) conjugates of N10-propargyl-5,8-dideazafolic acid and their enzyme inhibition.

    Science.gov (United States)

    Pawelczak, K; Jones, T R; Kempny, M; Jackman, A L; Newell, D R; Krzyzanowski, L; Rzeszotarska, B

    1989-01-01

    The synthesis is described of four oligo(gamma-glutamyl) conjugates of N10-propargyl-5,8-dideazafolic acid containing a total of two, three, four, and five L-glutamic acid residues. The tert-butyl group was chosen as the carboxyl protecting group in order to obviate the use of alkali and thus the possibility of gamma----alpha transpeptidation. The starting material, di-tert-butyl glutamate, was coupled to N-(benzyloxycarbonyl)-L-glutamic acid alpha-tert-butyl ester via a mixed anhydride with isobutyl chloroformate. Hydrogenolysis of the benzyloxycarbonyl group in the product gave a carboxyl-protected diglutamate, which either was acylated with 4-[(benzyloxycarbonyl)amino] benzoyl chloride to give a protected aminobenzamide or was cycled further by using the above mixed anhydride/hydrogenolysis sequence into tri-, tetra-, and pentaglutamates. Each of the last named was also acylated, as above, to give a benzamide. The benzyloxycarbonyl group in the benzamides was removed by hydrogenolysis and the amino groups thus exposed were N-alkylated with propargyl bromide. The resulting proparglyamines were further alkylated with 2-amino-6-(bromomethyl)-4-hydroxyquinazoline hydrobromide to give the antifolate poly(t-Bu) esters. Deprotection with trifluoroacetic acid in the final step delivered the desired antifolates as their trifluoroacetate salts. The di- to pentaglutamates were, respectively, 31-, 97-, 171-, and 167-fold more inhibitory to WI-L2 human thymidylate synthase than the parent compound.

  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. Sterically hindered C(alpha, alpha)-disubstituted alpha-amino acids: synthesis from alpha-nitroacetate and incorporation into peptides.

    Science.gov (United States)

    Fu, Y; Hammarström, L G; Miller, T J; Fronczek, F R; McLaughlin, M L; Hammer, R P

    2001-10-19

    The preparation of sterically hindered and polyfunctional C(alpha,alpha)-disubstituted alpha-amino acids (alpha alpha AAs) via alkylation of ethyl nitroacetate and transformation into derivatives ready for incorporation into peptides are described. Treatment of ethyl nitroacetate with N,N-diisopropylethylamine (DIEA) in the presence of a catalytic amount of tetraalkylammonium salt, followed by the addition of an activated alkyl halide or Michael acceptor, gives the doubly C-alkylated product in good to excellent yields. Selective nitro reduction with Zn in acetic acid or hydrogen over Raney Ni gives the corresponding amino ester that, upon saponification, can be protected with the fluorenylmethyloxycarbonyl (Fmoc) group. The first synthesis of an orthogonally protected, tetrafunctional C(alpha,alpha)-disubstituted analogue of aspartic acid, 2,2-bis(tert-butylcarboxymethyl)glycine (Bcmg), is described. Also, the sterically demanding C(alpha,alpha)-dibenzylglycine (Dbg) has been incorporated into a peptide using solid-phase synthesis. It was found that once sterically congested Dbg is at the peptide N-terminus, further chain extension becomes very difficult using uronium or phosphonium salts (PyAOP, PyAOP/HOAt, HATU). However, preformed amino acid symmetrical anhydride couples to N-terminal Dbg in almost quantitative yield in nonpolar solvent (dichloroethane-DMF, 9:1).

  7. [Simultaneous determination of twenty-one organic acids in food by ion chromatography with eluent autogeneration].

    Science.gov (United States)

    Lin, Huaying; Lin, Fenghua; Sheng, Lina; Li, Yidan; Zhang, Qiong

    2007-01-01

    A novel ion chromatographic method was developed for the simultaneous determination of organic acids in food samples including quinic acid, acetic acid, pyruvic acid, ketosuccinic acid, mannitic acid, lactic acid, succinic acid, malic acid, tartaric acid, oxalic acid, fumaric acid, ascorbic acid, alpha-ketoglutaric acid, cinnamic acid, salicylic acid, citric acid, isocitric acid, ferulic acid, cis-aconitic acid, trans-aconitic acid, beta-coumaric acid. 5 - 34 mmol/L KOH produced by an EG40 eluent autogenerator could achieve a flat baseline and lower background conductance when performing gradient elution. The flow rate was 0.6 - 2.5 mL/min and the injection volume was 25 microL. The separation was performed on an IonPac AS11 column and detected by suppressed conductivity with self-regenerating suppressor mode. The samples were prepared through extraction, decoloration and filtration before analysis. Twenty-one organic acids showed good linear relationship between the mass concentration and the peak area in the measurement ranges. The correlation coefficients were above 0.999 and the detection limits were 0.011 - 0.188 mg/L, and the average recoveries were 91.5% - 101.8%. The method is simple and rapid with good accuracy and reproducibility, and has been applied to determine twenty-one organic acids in diversiform samples with satisfactory results.

  8. Nutrient content of carob pod (Ceratonia siliqua L.) flour prepared commercially and domestically.

    Science.gov (United States)

    Ayaz, Faik A; Torun, Hülya; Glew, Robert H; Bak, Zehra D; Chuang, Luther T; Presley, Jack M; Andrews, Ronnie

    2009-12-01

    Although the fruit of the carob tree (Ceratonia siliqua L. Fabaceae) is nutritious and widely available in Turkey, especially in West and South Anatolia, much remains to be learned about its nutrient composition. The main goal of our study was to determine if there are differences in the content of certain nutrients in commercially-prepared carob flour (CPCP) and domestic or home-prepared carob powder (HPCP). Sucrose was the main sugar in CPCP and HPCP. Total protein was 40% lower in CPCP than HPCP due mainly to decreases in the content of several essential amino acids. However, except for lysine in CPCP, HPCP and CPCP compared favourably to a WHO protein standard. There were large differences in terms of their content of the two essential fatty acids, linoleic and alpha-linolenic acid, and the linoleic acid/alpha-linolenic acid ratio was 3.6 for CPCP, and 6.1 for HPCP. Manganese and iron were 2.5-fold higher in HPCP than CPCP. This study demonstrates that carob flour prepared in either the household or industrially is a good source of many, but not all essential nutrients, and that commercial processing of carob fruit into flour seems to affect its content of several important nutrients.

  9. Mutation profile of the GAA gene in 40 Italian patients with late onset glycogen storage disease type II.

    Science.gov (United States)

    Montalvo, A L E; Bembi, B; Donnarumma, M; Filocamo, M; Parenti, G; Rossi, M; Merlini, L; Buratti, E; De Filippi, P; Dardis, A; Stroppiano, M; Ciana, G; Pittis, M G

    2006-10-01

    Glycogen storage disease type II (GSDII) is a recessively inherited disorder due to the deficiency of acid alpha-glucosidase (GAA) that results in impaired glycogen degradation and its accumulation in the lysosomes. We report here the complete molecular analysis of the GAA gene performed on 40 Italian patients with late onset GSDII. Twelve novel alleles have been identified: missense mutations were functionally characterized by enzyme activity and protein processing in a human GAA-deficient cell line while splicing mutations were studied by RT-PCR and in silico analysis. A complex allele was also identified carrying three different alterations in cis. The c.-32-13T > G was the most frequent mutation, present as compound heterozygote in 85% of the patients (allele frequency 42.3%), as described in other late onset GSDII Caucasian populations. Interestingly, the c.-32-13T > G was associated with the c.2237G > A (p.W746X) in nine of the 40 patients. Genotype-phenotype correlations are discussed with particular emphasis on the subgroup carrying the c.-32-13T > G/c.2237G > A genotype.

  10. Influence of postharvest hot water treatment on nutritional and functional properties of kumquat (Fortunella japonica Lour. Swingle Cv. Ovale) fruit.

    Science.gov (United States)

    Schirra, Mario; Palma, Amedeo; D'Aquino, Salvatore; Angioni, Alberto; Minello, Elisabeth V; Melis, Marinella; Cabras, Paolo

    2008-01-23

    The present study investigated the influence of a hot water dip (HWD) for 2 min at 50 degrees C, a standard and effective treatment for postharvest decay control of citrus fruit, on the nutritional and health-related properties of kumquats. The results show that most of the parameters examined, including titratable acidity, soluble solids content, maturity index, glucose, fructose, sucrose, ascorbic acid, dehydroascorbic acid, alpha- and gamma-tocopherols, beta-carotene, zeaxantin, rhoifolin, and antioxidant activity, were not significantly affected by treatment. The levels of beta-cryptoxanthin, narirutin, and total flavonoids increased after HWD, whereas lutein and total phenols decreased. The concentration of the essential oil and the relative percentage of the individual components of the essential oil were not affected by HWD except for the minor compound p-menta-1,5-dien-1-ol, which increased after HWD. After storage, lower levels of glucose, total sugars, beta-carotene, beta-cryptoxanthin and lutein were recorded in HWD fruit. A decrease in antioxidant activity and increases in alpha-tocopherol and total vitamin E were found both in control and HWD fruit. The influence of HWD at 50 degrees C for 2 min on individual nutraceuticals and health-related properties was thus generally low and may depend on storage conditions.

  11. α-linolenic acid reduces TNF-induced apoptosis in C2C12 myoblasts by regulating expression of apoptotic proteins

    Directory of Open Access Journals (Sweden)

    Felicia Carotenuto

    2016-11-01

    Full Text Available Impaired regeneration and consequent muscle wasting is a major feature of muscle degenerative diseases. Nutritional interventions as adjuvant strategy for preventing such conditions are recently gaining increasing attention. Ingestion of n3-polyunsaturated fatty acids has been suggested to have a positive impact on muscle diseases. We recently demonstrated that the dietary n3-fatty acid, alpha-linolenic acid (ALA, exerts potent beneficial effects in preserving skeletal muscle regeneration in models of muscle dystrophy. To better elucidate the underlying mechanism we investigate here on the expression level of the anti- and pro-apototic proteins, as well as caspase-3 activity, in C2C12 myoblasts challenged with pathological levels of TNF. The results demonstrated that ALA protective effect on C2C12 myoblasts was associated to an increased Bcl-2/Bax ratio. Indeed, the effect of ALA was directed to rescue Bcl-2 expression and decrease Bax expression both affected in an opposite way by TNF treatment. This effect was associated with a decrease in caspase-3 activity by ALA. TNF is a major pro-inflammatory cytokine that is expressed in damaged skeletal muscle, therefore, counteract inflammatory signals in the muscle microenvironment represents a critical strategy to ameliorate skeletal muscle pathologies

  12. Effect of nitrogen source in the fertilizing solution on nutritional quality of three members of the Portulaca oleracea aggregate.

    Science.gov (United States)

    Szalai, Gabriella; Dai, Nir; Danin, Avinoam; Dudai, Nativ; Barazani, Oz

    2010-09-01

    Portulaca oleracea (purslane) is nutritious but, in addition to the essential alpha-linolenic acid, vitamin C and tocopherols, it contains undesirable oxalic acid. Knowing the effects of nitrate and ammonium on oxalate accumulation, we tested the agronomic potential of three members of the P. oleracea aggregate under various nitrogen fertilization conditions, by measuring biomass production and accumulation of fatty acids, organic acids and tocopherol in the commercial P. sativa (Pos) and two natural members: P. nitida (Pon) and P. papillato-stellulata (Pop). With nitrate as the sole N source, we measured differences between Pon and Pos in concentrations of the essential omega-3 fatty acid alpha-linolenic acid. Pos also gained less dry biomass under these conditions, implying a higher agronomical and nutritional value for Pon. Increasing the fertilizer ammonium concentration and reducing that of nitrate significantly decreased oxalic acid by factors of up to 1.7, 2.6 and 3.4 in Pos, Pop and Pon, respectively, significantly increased concentrations of tocopherol and malic acid, had no effect on fatty acids or ascorbic acid, but reduced biomass. In spite of the recumbent growth habit of Pon, the present findings indicate its agronomic potential. Because early flowering and seed production may be the limiting factors in purslane agriculture, growing Pon in nitrate-poor conditions might be agriculturally favorable. Copyright 2010 Society of Chemical Industry.

  13. Reactions of diiron m-aminocarbyne complexes containing nitrile ligands

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    Busetto Luigi

    2003-01-01

    Full Text Available The acetonitrile ligand in the mu-aminocarbyne complexes [Fe2{mu-CN(MeR}(mu-CO(CO(NCMe(Cp2][SO 3CF3] (R = Me, 2a, CH2Ph, 2b, Xyl, 2c (Xyl = 2,6-Me2C6H3 is readily displaced by halides and cyanide anions affording the corresponding neutral species [Fe2{mu-CN(MeR}(mu-CO(CO(X(Cp2 ] (X = Br, I, CN. Complexes 2 undergo deprotonation and rearrangement of the coordinated MeCN upon treatment with organolithium reagents. Trimethylacetonitrile, that does not contain acidic alpha hydrogens has been used in place of MeCN to form the complexes [Fe2{mu-CN(MeR}(mu-CO(CO(NCCMe3 (Cp2][SO3CF3] (7a-c. Attempts to replace the nitrile ligand in 3 with carbon nucleophiles (by reaction with RLi failed, resulting in decomposition products. However the reaction of 7c with LiCºCTol (Tol = C6H4Me, followed by treatment with HSO3CF3, yielded the imino complex [Fe2{mu-CN(MeXyl}(mu-CO(CO {N(HC(CºCC6H4Me-4CMe3}(Cp 2][SO3CF3 ] (8, obtained via acetilyde addition at the coordinated NCCMe3.

  14. Nanocellulose coated with various free fatty acids can adsorb fumonisin B1, and decrease its toxicity.

    Science.gov (United States)

    Zadeh, Mohammad Hossein Balal; Shahdadi, Hossein

    2015-10-01

    The aim of this study was to evaluate the adsorption and biological properties of nanocellulose coated with free fatty acids (NCCFFAs). At first, nanocellulose was synthesized by acid hydrolysis, and then separately coated with different free fatty acids (FFAs), including lauric acid, alpha linoleic acid, oleic acid, and palmitic acid. Next, the serial concentrations of NCCFFAs (1, 10, 100, and 1000 μg/mL) was separately added to fumonisin B1 (FB1) at 1000 μg/mL, and separately incubated at 37 °C for 1, 2, and 3h. Then, the percentage of adsorption was calculated. In the next experiment, the viability of mouse liver cells was measured when they exposed to serial concentrations of NCCFFAs, FFAs, and FB1. This study showed that the increase of incubation time and concentration of NCCFFAs led to increase of FB1 adsorption. Although FFAs and NCCFFAs had no remarkable toxicity, the high toxicity was observed for FB1. Importantly, the toxicity of FB1 was highly decreased, when incubated together with FFAs or NCCFFAs. These novel adsorbents, NCCFFAs, can be used together with different foodstuffs to remove FB1. Copyright © 2015 Elsevier B.V. All rights reserved.

  15. Cupric ion reducing antioxidant capacity assay for antioxidants in human serum and for hydroxyl radical scavengers.

    Science.gov (United States)

    Apak, Reşat; Güçlü, Kubilay; Ozyürek, Mustafa; Bektaşoğlu, Burcu; Bener, Mustafa

    2010-01-01

    Tests measuring the combined antioxidant effect of the nonenzymatic defenses in biological fluids may be useful in providing an index of the organism's capability to counteract reactive species known as pro-oxidants, resist oxidative damage, and combat oxidative stress-related diseases. The selected chromogenic redox reagent for the assay of human serum should be easily accessible, stable, selective, and respond to all types of biologically important antioxidants such as ascorbic acid, alpha-tocopherol, beta-carotene, reduced glutathione (GSH), uric acid, and bilirubin, regardless of chemical type or hydrophilicity. Our recently developed cupric reducing antioxidant capacity (CUPRAC) spectrophotometric method for a number of polyphenols and flavonoids using the copper(II)-neocuproine reagent in ammonium acetate buffer is now applied to a complete series of plasma antioxidants for the assay of total antioxidant capacity of serum, and the resulting absorbance at 450 nm is recorded either directly (e.g., for ascorbic acid, alpha-tocopherol, and glutathione) or after incubation at 50 degrees C for 20 min (e.g., for uric acid, bilirubin, and albumin), quantitation being made by means of a calibration curve. The lipophilic antioxidants, alpha-tocopherol and beta-carotene, are assayed in dichloromethane. Lipophilic antioxidants of serum are extracted with n-hexane from an ethanolic solution of serum subjected to centrifugation. Hydrophilic antioxidants of serum are assayed in the centrifugate after perchloric acid precipitation of proteins. The CUPRAC molar absorptivities, linear ranges, and TEAC (trolox equivalent antioxidant capacity) coefficients of the serum antioxidants are established, and the results are evaluated in comparison with the findings of the ABTS/TEAC reference method. The intra- and inter-assay coefficients of variation (CVs) are 0.7 and 1.5%, respectively, for serum. The CUPRAC assay proved to be efficient for glutathione and thiol-type antioxidants

  16. Total antioxidant capacity assay of human serum using copper(II)-neocuproine as chromogenic oxidant: the CUPRAC method.

    Science.gov (United States)

    Apak, Reşat; Güçlü, Kubilay; Ozyürek, Mustafa; Karademir, Saliha Esin; Altun, Mehmet

    2005-09-01

    Tests measuring the combined antioxidant effect of the nonenzymatic defenses in biological fluids may be useful in providing an index of the organism's capability to counteract reactive species known as prooxidants, resist oxidative damage and combat oxidative stress-related diseases. The selected chromogenic redox reagent for the assay of human serum should be easily accessible, stable, selective, respond to all types of biologically important antioxidants such as ascorbic acid, alpha-tocopherol, beta-carotene, reduced glutathione (GSH), uric acid and bilirubin, regardless of chemical type or hydrophilicity. Currently, there is no rapid method for total antioxidant assay of human serum meeting the above criteria. Our recently developed cupric reducing antioxidant capacity (CUPRAC) spectrophotometric method for a number of polyphenols and flavonoids using the copper(II)-neocuproine reagent in ammonium acetate buffer was now applied to a complete series of plasma antioxidants for the assay of total antioxidant capacity (TAC) of serum, and the resulting absorbance at 450 nm was recorded either directly (e.g. for ascorbic acid, alpha-tocopherol and glutathione) or after incubation at 50 degrees C for 20 min (e.g. for uric acid, bilirubin and albumin), quantitation being made by means of a calibration curve. The lipophilic antioxidants, alpha-tocopherol and beta-carotene, were assayed in dichloromethane (DCM). Lipophilic antioxidants of serum were extracted with n-hexane from an ethanolic solution of serum subjected to centrifugation. Hydrophilic antioxidants of serum were assayed after perchloric acid precipitation of proteins in the centrifugate. The molar absorptivities, linear ranges and trolox equivalent antioxidant capacity (TEAC) coefficients of the serum antioxidants were established with respect to the CUPRAC spectrophotometric method, and the results (TEAC, or TEAC coefficients) were evaluated in comparison to the findings of the ABTS/TEAC reference method

  17. GENOMIQUE ET NUTRITION HUMAINE Biodisponibilité des acides gras et apports nutritionnels conseillés

    Directory of Open Access Journals (Sweden)

    Combe Nicole

    2002-03-01

    édiocre lorsqu’ils sont situés en positions sn1 et sn3, comparativement à la position sn2. Dans le cas de l’acide alpha-linolénique, dont le métabolisme oxydatif in vivo est très rapide, c’est son maintien en position interne des triglycérides plasmatiques qui pourrait lui assurer la protection contre une dégradation oxydative précoce, préjudiciable à sa fonction de précurseur d’acides gras essentiels. Sur cette base sont évoquées les capacités de l’huile de colza supérieures à celles de l’huile de soja, avec le maintien sur la position sn2 des triglycérides lymphatiques de proportions plus élevées en acide alpha-linolénique.

  18. Risk of postpartum depression in relation to dietary fish and fat intake in Japan: the Osaka Maternal and Child Health Study.

    Science.gov (United States)

    Miyake, Yoshihiro; Sasaki, Satoshi; Yokoyama, Tetsuji; Tanaka, Keiko; Ohya, Yukihiro; Fukushima, Wakaba; Saito, Kyoko; Ohfuji, Satoko; Kiyohara, Chikako; Hirota, Yoshio

    2006-12-01

    An ecological analysis found that the docosahexaenoic acid content in mother's milk and seafood intake were inversely correlated with postpartum depression. This prospective study investigated the relationship of consumption of selected high-fat foods and specific types of fatty acids with the risk of postpartum depression. The subjects were 865 Japanese women. Dietary data were obtained from a self-administered diet history questionnaire during pregnancy. The Edinburgh Postnatal Depression Scale (EPDS) was used for the evaluation of postpartum depression. Adjustment was made for age, gestation, parity, cigarette smoking, family structure, family income, education, changes in diet in the previous month, season when data at baseline were collected, body mass index, time of delivery before the second survey, medical problems in pregnancy, baby's sex and baby's birthweight. The percentage of women with high depression scores was 14.0%. No evident dose-response associations were observed between intake of fish, meat, eggs, dairy products, total fat, saturated fatty acids, monounsaturated fatty acids, n-3 polyunsaturated fatty acids, n-6 polyunsaturated fatty acids, linoleic acid, alpha-linolenic acid, arachidonic acid, eicosapentaenoic acid or docosahexaenoic acid and the ratio of n-3 to n-6 polyunsaturated fatty acids and the risk of postpartum depression. However, there was an inverted J-shaped relationship between intake of n-3 polyunsaturated fatty acids and docosahexaenoic acid and the risk of postpartum depression. This study failed to substantiate a clear inverse relationship between fish and n-3 polyunsaturated fatty acid intake and postpartum depression. Further investigations are needed to determine whether fish and n-3 polyunsaturated fatty acid consumption is preventive against postpartum depression.

  19. [Tetra-saccharide glucose as a diagnostic biomarker for Pompe disease: a study with 35 patients].

    Science.gov (United States)

    Bobillo Lobato, Joaquín; Durán Parejo, Pilar; Tejero Díez, Pedro; Jiménez Jiménez, Luis M

    2013-08-04

    Pompe disease is a disorder originating from an acid alpha-glycosidase (AAG) enzyme deficiency. This disease produces an accumulation of lysosomal glycogen in different tissues, whereby the skeletal and heart muscles are especially involved. The established diagnosis is achieved through the identification of the AAG deficiency. There are also other secondary diagnostic biomarkers, such as tetra-saccharide glucose (Glc4), which shows high levels in the urine of these patients. In this study it is highlighted the usefulness of Glc4 as a diagnostic biomarker for Pompe disease in its different forms of presentation, using a high-performance liquid chromatography with ultraviolet detection (HPLC/UV) adapted to the study. A total of 75 individuals have been analyzed: 40 healthy controls and 35 patients diagnosed with Pompe disease. Twenty-four hour samples of urine were collected from all of the patients and their Glc4 levels were determined by means of HPLC/UV. The evaluation of the urinary Glc4 shows a high discrimination ability between healthy/sick individuals. In addition, the results obtained have allowed to establish the most appropriate level of decision or cut-off point for the identification of sick people. Glc4 urinary levels are found to be high in patients suffering from Pompe disease and even though increased levels are also found in other conditions, the existence of a AAG deficiency together with a compatible clinical symptoms, prove very helpful for a correct diagnosis of this serious disease. Copyright © 2012 Elsevier España, S.L. All rights reserved.

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

    KAUST Repository

    Shih, P. B.

    2015-03-31

    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.

  1. Effects of a 12-week high-α-linolenic acid intervention on EPA and DHA concentrations in red blood cells and plasma oxylipin pattern in subjects with a low EPA and DHA status.

    Science.gov (United States)

    Greupner, Theresa; Kutzner, Laura; Nolte, Fabian; Strangmann, Alena; Kohrs, Heike; Hahn, Andreas; Schebb, Nils Helge; Schuchardt, Jan Philipp

    2018-03-01

    The essential omega-3 fatty acid alpha-linolenic acid (ALA, 18:3n3) can be converted into EPA and DHA. The aim of the present study was to determine the effect of a high-ALA diet on EPA and DHA levels in red blood cells (RBCs) and their oxylipins in the plasma of subjects with a low EPA and DHA status. Fatty acid concentrations [μg mL -1 ] and relative amounts [% of total fatty acids] in the RBCs of 19 healthy men (mean age 26.4 ± 4.6 years) were analyzed by means of GC-FID. Free plasma oxylipin concentrations were determined by LC-MS based targeted metabolomics. Samples were collected and analyzed at baseline (week 0) and after 1 (week 1), 3 (week 3), 6 (week 6), and 12 (week 12) weeks of high dietary ALA intake (14.0 ± 0.45 g day -1 ). ALA concentrations significantly (p oxylipins, the concentrations increased less rapidly and to a lesser extent compared to the concentrations of their precursor FA. LA-derived oxylipins remained unchanged and arachidonic acid and DHA oxylipin concentrations were not significantly changed. Our results confirm that the intake of ALA is not a sufficient source for the increase of EPA + DHA in subjects on a Western diet. Specifically, a high-ALA diet results in increased EPA and declined DHA concentrations. However, the changes effectively balance each other out so that ΣEPA + DHA in RBCs - which is an established marker for health protective effects of omega-3-PUFA - remains constant. The PUFA levels in RBCs reflect the concentration and its changes in plasma hydroxy- and dihydroxy-PUFA concentrations for ALA and EPA.

  2. 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

  3. Comparative sequence analyses of the major quantitative trait locus phosphorus uptake 1 (Pup1) reveal a complex genetic structure.

    Science.gov (United States)

    Heuer, Sigrid; Lu, Xiaochun; Chin, Joong Hyoun; Tanaka, Juan Pariasca; Kanamori, Hiroyuki; Matsumoto, Takashi; De Leon, Teresa; Ulat, Victor Jun; Ismail, Abdelbagi M; Yano, Masahiro; Wissuwa, Matthias

    2009-06-01

    The phosphorus uptake 1 (Pup1) locus was identified as a major quantitative trait locus (QTL) for tolerance of phosphorus deficiency in rice. Near-isogenic lines with the Pup1 region from tolerant donor parent Kasalath typically show threefold higher phosphorus uptake and grain yield in phosphorus-deficient field trials than the intolerant parent Nipponbare. In this study, we report the fine mapping of the Pup1 locus to the long arm of chromosome 12 (15.31-15.47 Mb). Genes in the region were initially identified on the basis of the Nipponbare reference genome, but did not reveal any obvious candidate genes related to phosphorus uptake. Kasalath BAC clones were therefore sequenced and revealed a 278-kbp sequence significantly different from the syntenic regions in Nipponbare (145 kb) and in the indica reference genome of 93-11 (742 kbp). Size differences are caused by large insertions or deletions (INDELs), and an exceptionally large number of retrotransposon and transposon-related elements (TEs) present in all three sequences (45%-54%). About 46 kb of the Kasalath sequence did not align with the entire Nipponbare genome, and only three Nipponbare genes (fatty acid alpha-dioxygenase, dirigent protein and aspartic proteinase) are highly conserved in Kasalath. Two Nipponbare genes (expressed proteins) might have evolved by at least three TE integrations in an ancestor gene that is still present in Kasalath. Several predicted Kasalath genes are novel or unknown genes that are mainly located within INDEL regions. Our results highlight the importance of sequencing QTL regions in the respective donor parent, as important genes might not be present in the current reference genomes.

  4. 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.

  5. 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

    2010-12-01

    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.

  6. 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.

  7. 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

  8. HYPERTROPHIC OBSTRUCTIVE CARDIOMYOPATHY IN AN 8-MONTH OLD FEMALE INFANT SUSPECTED INFANTILE ONSET POMPE DISEASE

    Directory of Open Access Journals (Sweden)

    Made Dwi Purnami

    2015-05-01

    Full Text Available Hypertrophic cardiomyopathy (HCM is an autosomal dominant cardiac disorder marked with muscular hypertrophy of the left ventricle, associated obstruction of left ventricular outflow. About 0.2% of all cases worldwide. The majority of patients are asymptomatic, and some present with severe activity- limiting symptoms. The diagnosis of HCM before the age of 2 years is rare and usually discovered by chance, during the investigation of a murmur. Progressive disease characterized by prominent cardiomegaly, cadiomyopathy, hepatomegaly, musle weakness or hypotonia, respiratory distress, feeding difficulties and failure to thrive as presenting sign and symptoms are often referred to infantile Pompe disease. A deficiency of of the enzyme acid alpha glucosidase disease, result in lysosomal accumulation of glycogen in heart and skeletal muscle. Cardiorespiratory failure is the cause of significant morbidity and mortality in the first year of life. We reported a rare case, 8 month-old female with frequent respiratory distress since 2 months before admission. Physical examination showed dyspnea with chest wall retraction, no cyanosis, with grade III systolic murmur at midclavicular line sinistra, ICS IV- V and floopy infant. Chest films showed   pneumonia and cardiomegaly. The echocardiogram demonstrated bi-ventricular and interventricular hypertrophy with left ventricular obstruction. Laboratory finding there was increased levels of glutamic oxaloacetic acid transferase, alanin aminotransferase, and lactate dehydrogenase. Patient was diagnosed with hypertrophic obstructive cardiomyopathy of suspected infantile onset pompe disease. Despite medical treatment with propanolol dan diuretics, there was no significant improvement and she was died after 26th days of treatment in intermediate ward. [MEDICINA 2014;45:108-14]    

  9. Novel method for detection of glycogen in cells.

    Science.gov (United States)

    Skurat, Alexander V; Segvich, Dyann M; DePaoli-Roach, Anna A; Roach, Peter J

    2017-05-01

    Glycogen, a branched polymer of glucose, functions as an energy reserve in many living organisms. Abnormalities in glycogen metabolism, usually excessive accumulation, can be caused genetically, most often through mutation of the enzymes directly involved in synthesis and degradation of the polymer leading to a variety of glycogen storage diseases (GSDs). Microscopic visualization of glycogen deposits in cells and tissues is important for the study of normal glycogen metabolism as well as diagnosis of GSDs. Here, we describe a method for the detection of glycogen using a renewable, recombinant protein which contains the carbohydrate-binding module (CBM) from starch-binding domain containing protein 1 (Stbd1). We generated a fusion protein containing g lutathione S-transferase, a cM c eptitope and the tbd1 BM (GYSC) for use as a glycogen-binding probe, which can be detected with secondary antibodies against glutathione S-transferase or cMyc. By enzyme-linked immunosorbent assay, we demonstrate that GYSC binds glycogen and two other polymers of glucose, amylopectin and amylose. Immunofluorescence staining of cultured cells indicate a GYSC-specific signal that is co-localized with signals obtained with anti-glycogen or anti-glycogen synthase antibodies. GYSC-positive staining inside of lysosomes is observed in individual muscle fibers isolated from mice deficient in lysosomal enzyme acid alpha-glucosidase, a well-characterized model of GSD II (Pompe disease). Co-localized GYSC and glycogen signals are also found in muscle fibers isolated from mice deficient in malin, a model for Lafora disease. These data indicate that GYSC is a novel probe that can be used to study glycogen metabolism under normal and pathological conditions. © The Author 2017. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com

  10. Analysis of gene expression in gecko digital adhesive pads indicates significant production of cysteine- and glycine-rich beta-keratins.

    Science.gov (United States)

    Hallahan, David L; Keiper-Hrynko, Natalie M; Shang, Tanya Q; Ganzke, Thaya S; Toni, Mattia; Dalla Valle, Luisa; Alibardi, Lorenzo

    2009-01-15

    Microscopic bristles (setae) present on digital pads permit the adhesion and climbing of geckos. Keratins of setae of the lizard Gekko gecko (Tokay gecko) were analyzed by the isolation of expressed mRNAs and by the generation of an EST library. Of the 510 sequences determined, 268 (52.9%) were unique. Of these, 14 appeared to encode alpha- and 111 beta-keratins. Within the beta-keratins, we identified five groups based on nucleotide sequence comparisons. Of these, one contained the bulk of beta-keratins, with 103 EST members. The mRNAs within this major group, together with two singlets, encoded cysteine-proline-serine-rich proteins of 10-14 kDa (Ge-cprp). One of the smaller groups of transcripts encoded slightly larger glycine-proline-serine-rich proteins, of 14-19 kDa (Ge-gprp). The remaining group consisted of smaller (9 kDa) serine-tyrosine-rich beta-keratins (Ge-strp). Thus three classes could be distinguished by amino acid sequence alignment. Exact matches for some of the peptide sequences obtained from setal proteins by ms/ms sequencing occur within several of these clones. Most of the beta-keratins were basic and contained a core-box region of two beta-strand sequences, with high homology to core-boxes present in avian scale and feather beta-keratins. Core-boxes are beta-folded regions that are likely responsible for polymerization into the beta-keratin filaments. The two deduced alpha-keratins of 52.7 kDa are both acidic, and contain the typical central rod region with some homology to mammalian and avian alpha-keratins, with variable N- and C-terminal regions. Basic beta-keratins and acidic alpha-keratins may interact electrostatically to form the resistant corneous material of setae. (c) 2008 Wiley-Liss, Inc.

  11. 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.

  12. 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¡

  13. Content of redox-active compounds (ie, antioxidants) in foods consumed in the United States.

    Science.gov (United States)

    Halvorsen, Bente L; Carlsen, Monica H; Phillips, Katherine M; Bøhn, Siv K; Holte, Kari; Jacobs, David R; Blomhoff, Rune

    2006-07-01

    Supplements containing ascorbic acid, alpha-tocopherol, or beta-carotene do not protect against oxidative stress-related diseases in most randomized intervention trials. We suggest that other redox-active phytochemicals may be more effective and that a combination of different redox-active compounds (ie, antioxidants or reductants) may be needed for proper protection against oxidative damage. We aimed to generate a ranked food table with values for total content of redox-active compounds to test this alternative antioxidant hypothesis. An assay that measures the total concentration of redox-active compounds above a certain cutoff reduction potential was used to analyze 1113 food samples obtained from the US Department of Agriculture National Food and Nutrient Analysis Program. Large variations in the content of antioxidants were observed in different foods and food categories. The food groups spices and herbs, nuts and seeds, berries, and fruit and vegetables all contained foods with very high antioxidant contents. Most food categories also contained products almost devoid of antioxidants. Of the 50 food products highest in antioxidant concentrations, 13 were spices, 8 were in the fruit and vegetables category, 5 were berries, 5 were chocolate-based, 5 were breakfast cereals, and 4 were nuts or seeds. On the basis of typical serving sizes, blackberries, walnuts, strawberries, artichokes, cranberries, brewed coffee, raspberries, pecans, blueberries, ground cloves, grape juice, and unsweetened baking chocolate were at the top of the ranked list. This ranked antioxidant food table provides a useful tool for investigations into the possible health benefit of dietary antioxidants.

  14. Dietary total fat and fatty acids intake, serum fatty acids and risk of breast cancer: A meta-analysis of prospective cohort studies.

    Science.gov (United States)

    Cao, Yi; Hou, Lin; Wang, Weijing

    2016-04-15

    Results from prospective cohort studies on the association between dietary total fat and fatty acids intake and risk of breast cancer remain controversial. Pertinent prospective cohort studies were identified by a search of Embase and PubMed from inception to September 2015. Study-specific relative risks (RRs) with 95% confidence intervals were pooled using a random-effect model. Between-study heterogeneity and publication bias were assessed, and sensitivity analysis was conducted. Twenty-four independent studies on dietary total fat and fatty acids intake and seven studies on serum fatty acids were included. The pooled RR of breast cancer for the highest vs. lowest category of dietary total fat intake was 1.10 (1.02-1.19); however, no association was observed in studies adjusting for traditional risk factors of breast cancer. No association was observed between animal fat, vegetable fat, saturated fatty acids (SFA), monounsaturated fatty acids (MUFA), polyunsaturated fatty acids (PUFA), n-3 PUFA, n-6 PUFA, eicosapentaenoic acid, docosahexaenoic acid, alpha-linolenic acid, oleic acid, linoleic acid and arachidonic acid and risk of breast cancer. The pooled RRs of breast cancer for the highest vs. lowest category of serum SFA, MUFA, PUFA, n-3 PUFA and n-6 PUFA were 1.00 (0.78-1.28), 1.41 (0.99-2.03), 0.59 (0.27-1.30), 0.81 (0.60-1.10) and 0.84 (0.60-1.18), respectively. Results from this meta-analysis suggested that dietary total fat and fatty acids might be not associated with risk of breast cancer. © 2015 UICC.

  15. Inhibition of steroid 5 alpha-reductase by specific aliphatic unsaturated fatty acids.

    Science.gov (United States)

    Liang, T; Liao, S

    1992-01-01

    Human or rat microsomal 5 alpha-reductase activity, as measured by enzymic conversion of testosterone into 5 alpha-dihydrotestosterone or by binding of a competitive inhibitor, [3H]17 beta-NN-diethulcarbamoyl-4-methyl-4-aza-5 alpha-androstan-3-one ([3H]4-MA) to the reductase, is inhibited by low concentrations (less than 10 microM) of certain polyunsaturated fatty acids. The relative inhibitory potencies of unsaturated fatty acids are, in decreasing order: gamma-linolenic acid greater than cis-4,7,10,13,16,19-docosahexaenoic acid = cis-6,9,12,15-octatetraenoic acid = arachidonic acid = alpha-linolenic acid greater than linoleic acid greater than palmitoleic acid greater than oleic acid greater than myristoleic acid. Other unsaturated fatty acids such as undecylenic acid, erucic acid and nervonic acid, are inactive. The methyl esters and alcohol analogues of these compounds, glycerols, phospholipids, saturated fatty acids, retinoids and carotenes were inactive even at 0.2 mM. The results of the binding assay and the enzymic assay correlated well except for elaidic acid and linolelaidic acid, the trans isomers of oleic acid and linoleic acid respectively, which were much less active than their cis isomers in the binding assay but were as potent in the enzymic assay. gamma-Linolenic acid had no effect on the activities of two other rat liver microsomal enzymes: NADH:menadione reductase and glucuronosyl transferase. gamma-Linolenic acid, the most potent inhibitor tested, decreased the Vmax. and increased Km values of substrates, NADPH and testosterone, and promoted dissociation of [3H]4-MA from the microsomal reductase. gamma-Linolenic acid, but not the corresponding saturated fatty acid (stearic acid), inhibited the 5 alpha-reductase activity, but not the 17 beta-dehydrogenase activity, of human prostate cancer cells in culture. These results suggest that unsaturated fatty acids may play an important role in regulating androgen action in target cells. PMID:1637346

  16. Lectin histochemistry of salivary glands in the giant ant-eater (Myrmecophaga tridactyla).

    Science.gov (United States)

    Meyer, W; Beyer, C; Wissdorf, H

    1993-04-01

    The submandibular and buccal glands of the Giant Ant-eater (Myrmecophaga tridactyla) have been studied by means of a series of carbohydrate histochemical methods, including a broad spectrum of PO-lectin procedures. The seromucous cells (Gl. submandibularis) and mucous cells (Gl. buccalis) of the glandular acini, as well as the secretion in the excretory duct system exhibited very strong to strong reactions for neutral and acidic glycoconjugates. The serous cells of the buccal glands and the excretory duct cells reacted rather weakly. The different controls applied particularly emphasized that sialoglycoconjugates are the predominant ingredients of the saliva secreted. Lectin histochemical differentiation demonstrated a varying pattern of saccharide residues in these substances. In the submandibular glands the glycoconjugates (mostly proteoglycans) of the seromucous cells and the luminal secretion normally contained terminal beta-galactose and minor contents of terminal alpha-N-acetylglucosamine. After sialidase digestion this cell type exhibited distinct amounts of sialic acid-beta-galactose and sialic acid-alpha-N-acetylgalactosamine. Sialic acid was also clearly present in the tough interlobular connective tissue. The buccal glands showed a similar distribution of saccharide residues in the mucous cells. In the serous cells, however, acidic glycoproteins with sialyl residues were observed, also containing terminal alpha-D-mannosyl, alpha-N-acetylgalactosaminyl, and beta-D-galactosyl residues. The cells of the excretory duct system of both gland types reacted weakly to moderately for terminal sugar residues (N-acetyl-D-glucosamine, N-acetyl-D-galactosamine, beta-D-galactose). The results obtained are discussed in view of the specific feeding mode of the Giant Ant-eater, whereby high contents of sialoglycoconjugates (proteoglycans, glycoproteins) produced by the salivary glands warrant for the main function of the non-sticky saliva; i.e., to act as an effective

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

    International Nuclear Information System (INIS)

    Karimullina, Elina; Li Yangchun; Ginjupalli, Gautam K.; Baldwin, William S.

    2012-01-01

    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.

  18. 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

  19. 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.

  20. Linear programming can help identify practical solutions to improve the nutritional quality of food aid.

    Science.gov (United States)

    Rambeloson, Zo J; Darmon, Nicole; Ferguson, Elaine L

    2008-04-01

    To assess the nutritional quality of food aid delivered by food banks in France and to identify practical modifications to improve it. National-level data were collected for all food aid distributed by French food banks in 2004, and its nutrient content per 2000 kcal was estimated and compared with French recommendations for adults. Starting with the actual donation and allowing new foods into the food aid donation, linear programming was used to identify the minimum changes required in the actual donation to achieve the French recommendations. French food-bank-delivered food aid does not achieve the French recommendations for dietary fibre, ascorbic acid, vitamin D, folate, magnesium, docosahexaenoic acid, alpha-linolenic acid and the percentage of energy from saturated fatty acids. Linear programming analysis showed that these recommendations are achievable if more fruits, vegetables, legumes and fish were collected and less cheese, refined cereals and foods rich in fat, sugar and/or salt. In addition, new foods not previously collected are needed, particularly nuts, wholemeal bread and rapeseed oil. These changes increased the total edible weight (42%) and economic value (55%) of the food aid donation, with one-third of its edible weight coming from fruits and vegetables, one-third from staples, one-quarter from dairy products and approximately a tenth from meat/fish/eggs. Important changes in the types and amounts of food collected will improve the nutritional quality of food-bank-delivered food aid in France. Such changes are recommended to improve the diets of deprived French populations.

  1. Daphnia HR96 is a Promiscuous Xenobiotic and Endobiotic Nuclear Receptor

    Science.gov (United States)

    Karimullina, Elina; Li, Yangchun; Ginjupalli, Gautam; Baldwin, William S.

    2012-01-01

    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. PMID:22466357

  2. Extended phenotype description and new molecular findings in late onset glycogen storage disease type II: a northern Italy population study and review of the literature.

    Science.gov (United States)

    Remiche, Gauthier; Ronchi, Dario; Magri, Francesca; Lamperti, Costanza; Bordoni, Andreina; Moggio, Maurizio; Bresolin, Nereo; Comi, Giacomo P

    2014-01-01

    Glycogen storage disease type II (GSDII) is a lysosomal storage disorder caused by acid alpha-1,4-glucosidase deficiency and associated with recessive mutations in its coding gene GAA. Few studies have provided so far a detailed phenotypical characterization in late onset GSDII (LO-GSDII) patients. Genotype-phenotype correlation has been previously attempted with controversial results. We aim to provide an in-depth description of a cohort (n = 36) of LO-GSDII patients coming from the north of Italy and compare our population's findings to the literature. We performed a clinical record-based retrospective and prospective study of our patients. LO-GSDII in our cohort covers a large variability of phenotype including subtle clinical presentation and did not differ significantly from previous data. In all patients, molecular analysis disclosed GAA mutations, five of them being novel. To assess potential genotype-phenotype correlations we divided IVS1-32-13T>G heterozygous patients into two groups following the severity of the mutations on the second allele. Our patients harbouring "severe" mutations (n = 21) presented a strong tendency to have more severe phenotypes and more disability, more severe phenotypes and more disability, higher prevalence of assisted ventilation and a shorter time of evolution to show it. The determination of prognostic factors is mandatory in order to refine the accuracy of prognostic information, to develop follow-up strategy and, more importantly, to improve the decision algorithm for enzyme replacement therapy administration. The demonstration of genotype-phenotype correlations could help to reach this objective. Clinical assessment homogeneity is required to overcome limitations due to the lack of power of most studies.

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

    International Nuclear Information System (INIS)

    Laemmerhofer, M.

    1996-11-01

    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)

  4. 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.

  5. 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

  6. 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.

  7. Composition en acides gras poly-insaturés de notre assiette et utilisation des matières premières agricoles en France : une amélioration lente, mais insuffisante

    Directory of Open Access Journals (Sweden)

    Duru Michel

    2017-03-01

    Full Text Available Un consensus est établi qu'un apport insuffisant en acide alpha-linolénique (ALA et un rapport LA (acide linoléique/ALA trop élevé contribuent à un accroissement de nombreuses maladies chroniques. Pour évaluer l'évolution de ces apports en France au cours des 20 dernières années (1993–2012, nous estimons les quantités apparentes consommées incluant la composition des produits animaux selon leur mode d'alimentation. Nous montrons que les consommations en LA et ALA ont respectivement diminué et augmenté (de 0,72 à 0,94 g/j pour l'ALA et que le rapport LA/ALA est passé de 18,3 à 12,4. L'évolution observée est cohérente avec celle de la composition du lait maternel. Ces valeurs sont proches de celles obtenues en 2006 par enquêtes auprès d'un échantillon de consommateurs. Elle est discutée au regard des politiques publiques de santé, des stratégies de production agricole et du comportement du consommateur. Ces lentes évolutions suggèrent que des changements radicaux restent nécessaires pour se rapprocher des recommandations (ALA = 1,8 g/j ; LA/ALA ≤ 4. Elles questionnent les marges de manœuvre dont dispose le consommateur pour choisir des produits animaux selon leur mode d'alimentation et les huiles alimentaires selon leurs apports.

  8. Face aux besoins et à la réalité des consommations, quelles sont les spécificités des différentes sources d’acides gras oméga 3 disponibles ?

    Directory of Open Access Journals (Sweden)

    Combe Nicole

    2004-03-01

    Full Text Available Les données de consommation en acides gras oméga 3 de la population française montrent les déficits d’apports, en particulier en acide alpha-linolénique (ALA, compte tenu des ANC (apports nutritionnels conseillés. En regard de ces données et des sources actuellement utilisées, les spécificités des différents vecteurs alimentaires des oméga 3 sont décrites. L’étude Aquitaine a montré que les lipides d’origine animale contribuaient à 73 % de l’apport alimentaire en ALA. Augmenter le contenu de celui-ci dans les produits d’animaux d’élevage via leur alimentation dans laquelle on introduit des sources d’ALA (colza, lin ou de DHA (huiles de poissons représente une stratégie d’optimisation. À cet égard, les résultats sont plus intéressants avec les monogastriques comparés aux polygastriques. En dépit de son potentiel, le vecteur des huiles végétales est trop peu exploité, il n’apporte actuellement que 9 % de l’apport global d’ALA. Pour couvrir les besoins en ALA, sans modification des autres habitudes alimentaires, il faudrait que l’ALA représente 8 % des acides gras totaux consommés sous forme d’huile, contre 0,5-0,6 % actuellement. Les spécificités de trois huiles linoléniques sont comparées. Par rapport aux objectifs, l’huile de colza présente les caractéristiques les plus intéressantes.

  9. Alcohol-induced blackout. Phenomenology, biological basis, and gender differences.

    Science.gov (United States)

    Rose, Mark E; Grant, Jon E

    2010-06-01

    Blackouts from acute alcohol ingestion are defined as the inability to recall events that occurred during a drinking episode and are highly prevalent in both alcoholic and nonalcoholic populations. This article reviews the clinical manifestations, epidemiology, risk factors, cognitive impairment, and neurobiology associated with alcohol-induced blackout, with special emphasis on the neurochemical and neurophysiological basis, and gender differences. Two types of blackout have been identified: en bloc, or complete inability to recall events during a time period, and fragmentary, where memory loss is incomplete. The rapidity of rise in blood alcohol concentration is the most robust predictor of blackout. Alcohol impairs different brain functions at different rates, and cognitive and memory performance are differentially impaired by ascending versus descending blood alcohol concentration. Cognitive and memory impairment occurs before motor impairment, possibly explaining how a drinker appearing fully functional can have little subsequent memory. Blackouts are caused by breakdown in the transfer of short-term memory into long-term storage and subsequent retrieval primarily through dose-dependent disruption of hippocampal CA1 pyramidal cell activity. The exact mechanism is believed to involve potentiation of gamma-aminobutyric acid-alpha-mediated inhibition and interference with excitatory hippocampal N-methyl-d-aspartate receptor activation, resulting in decreased long-term potentiation. Another possible mechanism involves disrupted septohippocampal theta rhythm activity because of enhanced medial septal area gamma-aminobutyric acid-ergic neurotransmission. Women are more susceptible to blackouts and undergo a slower recovery from cognitive impairment than men, due in part to the effect of gender differences in pharmacokinetics and body composition on alcohol bioavailability.

  10. Cornification in reptilian epidermis occurs through the deposition of keratin-associated beta-proteins (beta-keratins) onto a scaffold of intermediate filament keratins.

    Science.gov (United States)

    Alibardi, Lorenzo

    2013-02-01

    The isolation of genes for alpha-keratins and keratin-associated beta-proteins (formerly beta-keratins) has allowed the production of epitope-specific antibodies for localizing these proteins during the process of cornification epidermis of reptilian sauropsids. The antibodies are directed toward proteins in the alpha-keratin range (40-70 kDa) or beta-protein range (10-30 kDa) of most reptilian sauropsids. The ultrastructural immunogold study shows the localization of acidic alpha-proteins in suprabasal and precorneous epidermal layers in lizard, snake, tuatara, crocodile, and turtle while keratin-associated beta-proteins are localized in precorneous and corneous layers. This late activation of the synthesis of keratin-associated beta-proteins is typical for keratin-associated and corneous proteins in mammalian epidermis (involucrin, filaggrin, loricrin) or hair (tyrosine-rich or sulfur-rich proteins). In turtles and crocodilians epidermis, keratin-associated beta-proteins are synthesized in upper spinosus and precorneous layers and accumulate in the corneous layer. The complex stratification of lepidosaurian epidermis derives from the deposition of specific glycine-rich versus cysteine-glycine-rich keratin-associated beta-proteins in cells sequentially produced from the basal layer and not from the alternation of beta- with alpha-keratins. The process gives rise to Oberhäutchen, beta-, mesos-, and alpha-layers during the shedding cycle of lizards and snakes. Differently from fish, amphibian, and mammalian keratin-associated proteins (KAPs) of the epidermis, the keratin-associated beta-proteins of sauropsids are capable to form filaments of 3-4 nm which give rise to an X-ray beta-pattern as a consequence of the presence of a beta-pleated central region of high homology, which seems to be absent in KAPs of the other vertebrates. Copyright © 2012 Wiley Periodicals, Inc.

  11. Ultrastructural immunocytochemistry for the central region of keratin associated-beta-proteins (beta-keratins) shows the epitope is constantly expressed in reptilian epidermis.

    Science.gov (United States)

    Alibardi, L

    2013-08-01

    The presence of beta-proteins containing a core-box region in specific regions of reptilian epidermis has been studied by immunological methods. Alpha-keratins are detected by the antibody AK2 that recognizes a sequence toward the C-terminal of acidic alpha-keratins of 48-52kDa. Beta-proteins are recognized by an antibody directed to the core-box region specific for these proteins of 18-37kDa. The AK2 antibody labels with variable intensity alpha-keratin bundles in basal and suprabasal keratinocytes in the epidermis of representative species of reptiles but immunolabeling decreases or disappears in pre-corneous and corneous cells. As opposite, the core-box antibody only labels with variable intensity the dense beta-corneous material formed in pre-corneous and corneous layers of crocodilian and turtle epidermis. In lepidosaurian epidermis the core-box antibody labels the beta-layer while the mesos and alpha-layers are poorly or not labeled. The immunological evidence indicates that beta-proteins are synthesized in the upper spinosus and pre-corneous layers of the epidermis and replace or mask the initial alpha-keratin framework present in keratinocytes as they differentiate into cells of the beta-layer. In the specialized pad lamellae of gecko and anoline lizards charged beta-proteins accumulate in the adhesive setae and may affect the mechanism of adhesion that allows these lizards to walk vertical surfaces. The addition of beta-proteins to the alpha-keratins in upper cell layers of the epidermis recalls the process of cornification of mammalian epidermis where specific keratin-associated proteins (involucrin, loricrin and filaggrin) associate with the keratin framework in terminally differentiating keratinocytes of the stratum corneum. Copyright © 2013 Elsevier Ltd. All rights reserved.

  12. Syntheses of alpha- and gamma-substituted amides, peptides, and esters of methotrexate and their evaluation as inhibitors of folate metabolism.

    Science.gov (United States)

    Piper, J R; Montgomery, J A; Sirotnak, F M; Chello, P L

    1982-02-01

    N-[4-[[(Benzyloxy)carbonyl]methylamino]benzoyl]-L-glutamic acid alpha-benzyl ester (2) and gamma-benzyl ester (6) served as key intermediates in syntheses of precursors to amides and peptides of methotrexate (MTX) involving both the alpha- and gamma-carboxyl groupings of the glutamate moiety. Coupling of 2 and 6 at the open carboxyl grouping with amino compounds was affected by the mixed anhydride method (using isobutyl chloroformate); carboxyl groupings of amino acids coupled with 2 and 6 were protected as benzyl esters. N-[4-[[(Benzyloxy)carbonyl]methylamino]benzoyl]-L-glutamic acid gamma-methyl ester (5), a precursor to MTX gamma-methyl ester, was prepared from L-glutamic acid gamma-methyl ester and 4-[[(benzyloxy)carbonyl]methylamino]benzoyl chloride (1) in a manner similar to that used to prepare 2 and 6. The precursor to MTX alpha-methyl ester was prepared from gamma-benzyl ester 6 by treatment with MeI in DMF containing (i-Pr)2NEt. Benzyl and (benzyloxy)carbonyl protective groupings were removed by hydrogenolysis, and the deprotected side-chain precursors were converted to alpha- and gamma-substituted amides, peptides, and esters of MTX by alkylation with 6-(bromomethyl)-2,4-pteridinediamine hydrobromide (12). Biochemical-pharmacological studies on the prepared compounds aided in establishing that the alpha-carboxyl grouping of the glutamate moiety contributes to the binding of MTX to dihydrofolate reductase while the gamma-carboxyl does not. Other studies on the peptide MTX-gamma-Glu (13h) are concerned with the contribution toward antifolate activity of this metabolite of MTX. The compounds prepared were also evaluated and compared with MTX with respect to cytotoxicity toward H.Ep.-2 cells and effect on L1210 murine leukemia.

  13. 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.)

  14. 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.)

  15. 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.

  16. Profiling of Amino Acids and Their Derivatives Biogenic Amines Before and After Antipsychotic Treatment in First-Episode Psychosis

    Directory of Open Access Journals (Sweden)

    Liisa Leppik

    2018-04-01

    Full Text Available Schizophrenia (SCH is a heterogeneous disorder, deriving from a potential multitude of etiopathogenetic factors. During the past few years there has been an increasing interest in the role of circulating amino acids (AAs and biogenic amines (BAs in the pathophysiology of SCH. In the present study, we aimed to provide an insight into the potential role of alterations in levels of AAs and BAs as well as examine their more specific metabolic shifts in relation to early stage of SCH. We measured 21 AAs and 17 BAs in serum samples of patients with first-episode psychosis (FEP before and after 7-month antipsychotic treatment in comparison to control subjects (CSs. According to multivariate analysis, antipsychotic-naïve FEP patients had significantly higher levels of taurine and spermine, whereas values of proline (Pro, alpha-aminoadipic acid (alpha-AAA, kynurenine (Kyn, valine (Val, tyrosine (Tyr, citrulline (Citr, tryptophan (Trp, and histidine (His were diminished compared to CSs. Increased levels of taurine and spermine, as well as reduced levels of alpha-AAA and Kyn probably reflect the compromised function of N-methyl-D-aspartate (NMDA receptors in patients. The decreased levels of Pro (AA modulating the function of glutamate decarboxylase likely reflect the imbalanced function of gamma-aminobutyric acid (GABA system in the brain of FEP patients. The alterations in ratio between Tyr and phenylalanine (Phe can be taken as a sign of compromised function of dopaminergic system. These metabolic shifts were reinstated by 7-month antipsychotic treatment. Serum metabolic profiles can be regarded as important indicators to investigate clinical course of SCH and treatment response.

  17. Long-term observational, non-randomized study of enzyme replacement therapy in late-onset glycogenosis type II.

    Science.gov (United States)

    Bembi, Bruno; Pisa, Federica Edith; Confalonieri, Marco; Ciana, Giovanni; Fiumara, Agata; Parini, Rossella; Rigoldi, Miriam; Moglia, Arrigo; Costa, Alfredo; Carlucci, Annalisa; Danesino, Cesare; Pittis, Maria Gabriela; Dardis, Andrea; Ravaglia, Sabrina

    2010-12-01

    Type II glycogenosis (GSDII) is a lysosomal storage disorder due to acid alpha-glucosidase (GAA) deficiency. Enzyme replacement therapy (ERT) with human recombinant alpha-glucosidase (rhGAA) has been demonstrated to be effective in the treatment of infantile forms of GSDII, but little information is available concerning late-onset phenotypes. Long-term follow-up studies are not available at present. The aim of this study was to evaluate the ERT long-term effects in late-onset GSDII. Twenty-four patients, including 7 juveniles and 17 adults, received bi-weekly infusion of rhGAA (20 mg/kg) for at least 36 months. Clinical conditions, muscular function (6-min walking test, 6MWT; Walton scale, WS), respiratory function (vital capacity, VC; forced expiratory volume, FEV1; arterial pCO(2)), and muscle enzymes were assessed every 6 months. The 6MWT improved in both juvenile and adult patients (p = 0.01, p = 0.0002, respectively), as well as in patients with moderate to severe muscle function impairment (WS >3.5; p = 0.002). An overall improvement in WS was also observed (p = 0.0003). VC and FEV1 remained unchanged, while pCO(2) decreased (p = 0.017). Muscle enzymes decreased significantly (p < 0.0001). Two patients (8%) showed transient secondary events during ERT. Long-term ERT with rhGAA was shown to be safe, well tolerated, and effective in improving motor function and in stabilizing respiratory function in late-onset GSDII. The response pattern showed a progressive clinical improvement during the follow-up period in juvenile patients, while in adults it reached and maintained a plateau after the first year of treatment.

  18. The lipoxygenase pathway in tulip (Tulipa gesneriana): detection of the ketol route.

    Science.gov (United States)

    Grechkin, A N; Mukhtarova, L S; Hamberg, M

    2000-12-01

    The in vitro metabolism of [1-(14)C]linoleate, [1-(14)C]linolenate and their 9(S)-hydroperoxides was studied in cell-free preparations from tulip (Tulipa gesneriana) bulbs, leaves and flowers. Linoleate and its 9-hydroperoxide were converted by bulb and leaf preparations into three ketols: (12Z)-9-hydroxy-10-oxo-12-octadecadienoic acid (alpha-ketol), (11E)-10-oxo-13-hydroxy-11-octadecadienoic acid (gamma-ketol) and a novel compound, (12Z)-10-oxo-11-hydroxy-12-octadecadienoic acid (10,11-ketol), in the approximate molar proportions of 10:3:1. The corresponding 15, 16-dehydro alpha- and gamma-ketols were the main metabolites of [1-(14)C]linolenate and its 9-hydroperoxide. Thus bulbs and leaves possessed 9-lipoxygenase and allene oxide synthase activities. Incubations with flower preparations gave alpha-ketol hydro(pero)xides as predominant metabolites. Bulb and leaf preparations possessed a novel enzyme activity, gamma-ketol reductase, which reduces gamma-ketol to 10-oxo-13-hydroxyoctadecanoic acid (dihydro-gamma-ketol) in the presence of NADH. Exogenous linolenate 13(S)-hydroperoxide was converted mostly into chiral (9S,13S)-12-oxo-10-phytodienoate (99.5% optical purity) by bulb preparations, while [1-(14)C]linolenate was a precursor for ketols only. Thus tulip bulbs possess abundant allene oxide cyclase activity, the substrate for which is linolenate 13(S)-hydroperoxide, even though 13(S)-lipoxygenase products were not detectable in the bulbs. The majority of the cyclase activity was found in the microsomes (10(5) g pellet). Cyclase activity was not found in the other tissues examined, but only in the bulbs. The ketol route of the lipoxygenase pathway, mediated by 9-lipoxygenase and allene oxide synthase activities, has not been detected previously in the vegetative organs of any plant species.

  19. 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

  20. Plasma fatty acid ratios affect blood gene expression profiles--a cross-sectional study of the Norwegian Women and Cancer Post-Genome Cohort.

    Directory of Open Access Journals (Sweden)

    Karina Standahl Olsen

    Full Text Available High blood concentrations of n-6 fatty acids (FAs relative to n-3 FAs may lead to a "physiological switch" towards permanent low-grade inflammation, potentially influencing the onset of cardiovascular and inflammatory diseases, as well as cancer. To explore the potential effects of FA ratios prior to disease onset, we measured blood gene expression profiles and plasma FA ratios (linoleic acid/alpha-linolenic acid, LA/ALA; arachidonic acid/eicosapentaenoic acid, AA/EPA; and total n-6/n-3 in a cross-section of middle-aged Norwegian women (n = 227. After arranging samples from the highest values to the lowest for all three FA ratios (LA/ALA, AA/EPA and total n-6/n-3, the highest and lowest deciles of samples were compared. Differences in gene expression profiles were assessed by single-gene and pathway-level analyses. The LA/ALA ratio had the largest impact on gene expression profiles, with 135 differentially expressed genes, followed by the total n-6/n-3 ratio (125 genes and the AA/EPA ratio (72 genes. All FA ratios were associated with genes related to immune processes, with a tendency for increased pro-inflammatory signaling in the highest FA ratio deciles. Lipid metabolism related to peroxisome proliferator-activated receptor γ (PPARγ signaling was modified, with possible implications for foam cell formation and development of cardiovascular diseases. We identified higher expression levels of several autophagy marker genes, mainly in the lowest LA/ALA decile. This finding may point to the regulation of autophagy as a novel aspect of FA biology which warrants further study. Lastly, all FA ratios were associated with gene sets that included targets of specific microRNAs, and gene sets containing common promoter motifs that did not match any known transcription factors. We conclude that plasma FA ratios are associated with differences in blood gene expression profiles in this free-living population, and that affected genes and pathways may

  1. Alpha-mannosidosis

    Directory of Open Access Journals (Sweden)

    Nilssen Øivind

    2008-07-01

    Full Text Available Abstract Alpha-mannosidosis is an inherited lysosomal storage disorder characterized by immune deficiency, facial and skeletal abnormalities, hearing impairment, and intellectual disability. It occurs in approximately 1 of 500,000 live births. The children are often born apparently normal, and their condition worsens progressively. Some children are born with ankle equinus or develop hydrocephalus in the first year of life. Main features are immune deficiency (manifested by recurrent infections, especially in the first decade of life, skeletal abnormalities (mild-to-moderate dysostosis multiplex, scoliosis and deformation of the sternum, hearing impairment (moderate-to-severe sensorineural hearing loss, gradual impairment of mental functions and speech, and often, periods of psychosis. Associated motor function disturbances include muscular weakness, joint abnormalities and ataxia. The facial trait include large head with prominent forehead, rounded eyebrows, flattened nasal bridge, macroglossia, widely spaced teeth, and prognathism. Slight strabismus is common. The clinical variability is significant, representing a continuum in severity. The disorder is caused by lysosomal alpha-mannosidase deficiency. Alpha-mannosidosis is inherited in an autosomal recessive fashion and is caused by mutations in the MAN2B1 gene located on chromosome 19 (19 p13.2-q12. Diagnosis is made by measuring acid alpha-mannosidase activity in leukocytes or other nucleated cells and can be confirmed by genetic testing. Elevated urinary secretion of mannose-rich oligosaccharides is suggestive, but not diagnostic. Differential diagnoses are mainly the other lysosomal storage diseases like the mucopolysaccharidoses. Genetic counseling should be given to explain the nature of the disease and to detect carriers. Antenatal diagnosis is possible, based on both biochemical and genetic methods. The management should be pro-active, preventing complications and treating

  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. ED50 G(Na) block predictions for phenyl substituted and unsubstituted n-alkanols.

    Science.gov (United States)

    Kondratiev, A; Hahin, R

    2001-03-15

    To study the role the phenyl group plays in producing local anesthetic block, a sequence of n-alkanols and phenyl-substituted alkanols (phi)-alkanols) were characterized in their ability to block Na channels. The sequence of n-alkanols studied possess 3-5 carbons (propanol-pentanol). The action of phenol and 3-phi-alkanols (benzyl alcohol, phenethyl alcohol, 3-phenyl-1-propanol) were also studied. Na currents (INa) were recorded from single frog skeletal muscle fibers using the Vaseline-gap voltage clamp technique. INas were recorded prior to, during, and following the removal of the solutes in Ringer's solution. All alkanols and phenol acted to block INa in a dose-dependent manner. Effective doses to produce half block (ED50) of INa or Na conductance (GNa) were obtained from dose-response relations for all solutes used. The block of GNa depended on voltage, and could be separated into voltage-dependent and -independent components. Each solute acted to shift GNa-V relations in a depolarized direction and reduce the maximum GNa and slope of the relation. All solutes acted to speed up INa kinetics and cause hyperpolarizing shifts in steady-state inactivation. The magnitude of the kinetic changes increased with dose. Size was an important variable in determining the magnitude of the changes in INa; however, size alone was not sufficient to predict the changes in INa. ED50s for GNa and AP block could be predicted as a function of intrinsic molar volume, hydrogen bond acceptor basicity (beta) and donor acidity (alpha), and polarity (P) of the solutes. The equivalency of ED50 predictions for AP and GNa block can be explained by the fact that AP block arises from channel block and solute-induced changes in INa kinetics. phi-Alkanols were more effective at blocking and inactivating Na channels than their unsubstituted counterparts. Phenyl-substituted alkanols are more likely to interact with the channel than their unsubstituted counterparts.

  4. 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

  5. Chemistry of anti-AIDS and anticancer compounds

    Energy Technology Data Exchange (ETDEWEB)

    Yan, S.

    1992-01-01

    Several types of prodrugs of 2[prime], 3[prime]-dideoxynucleosides were designed and synthesized for evaluation as anti-AIDS drugs. These prodrugs include 5[prime]-O-acyl-2[prime], 3[prime]-dideoxynucleosides, in which the acyl groups are derived from both aromatic and aliphatic acids, [alpha]-amino acids, diacylglycerol carbonic acids, and diacylglycerol carbamic acids. By applying the pyridium-dihydropyridine redox delivery system to deliver 2[prime], 3[prime]-dideoxynucleosides to the central nervous system, 1,4-dihydropyridine-2[prime], 3[prime]-dideoxy-inosine and -adenosine compounds were synthesized. 5[prime]-Esters of 2[prime], 3[prime]-dideoxyinosine and 2[prime], 3[prime]-dideoxyadenosine were evaluated for their activity against the HIV-1 virus and for delivery to the central nervous system (CNS). The isomerization, hydrolysis, and oxidation of alkyl 1,4-dihydro-N-methylpyridine-3-carboxylates were studied by [sup 1]H and [sup 13]C NMR spectroscopy. Three intermediates, 1,4-dihydro-N-methylpyridine-3-carboxylic acid, alkyl (methyl or isopropyl) 1,6-dihydro-N-methylpyridine-3-carboxylate, and 1,6-dihydro-N-methylpyridine-3-carboxylic acid, were observed by [sup 1]H and [sup 13]C NMR spectroscopy, and their percentages in solution were determined. The structures of the 1,6-dihydropyridine intermediates were confirmed by comparison of the NMR spectra with those of an authentic model compound, methyl N-(4-chlorobenzyl)-1,6-dihydropyridine-3-carboxylate. The rate of hydrolysis of alkyl 1,4-dihydro-N-methylpyridine-3-carboxylates depends on the steric bulk of the O-alkyl group. A new type of 1,4-dihydropyridine drug delivery system with a three-carbon spacer group, 9-[2,3-di-O-acetyl-5-O-[3-(1,4-dihydro-N-methylpyridine-3-carboxamido)propionyl]-[beta]-D-arabinofuranosyl]adenine was designed, synthesized, and evaluated to deliver ara-ADA to the CNS for treatment of herpes encephalitis.

  6. Redox- and non-redox-metal-induced formation of free radicals and their role in human disease.

    Science.gov (United States)

    Valko, Marian; Jomova, Klaudia; Rhodes, Christopher J; Kuča, Kamil; Musílek, Kamil

    2016-01-01

    Transition metal ions are key elements of various biological processes ranging from oxygen formation to hypoxia sensing, and therefore, their homeostasis is maintained within strict limits through tightly regulated mechanisms of uptake, storage and secretion. The breakdown of metal ion homeostasis can lead to an uncontrolled formation of reactive oxygen species, ROS (via the Fenton reaction, which produces hydroxyl radicals), and reactive nitrogen species, RNS, which may cause oxidative damage to biological macromolecules such as DNA, proteins and lipids. An imbalance between the formation of free radicals and their elimination by antioxidant defense systems is termed oxidative stress. Most vulnerable to free radical attack is the cell membrane which may undergo enhanced lipid peroxidation, finally producing mutagenic and carcinogenic malondialdehyde and 4-hydroxynonenal and other exocyclic DNA adducts. While redox-active iron (Fe) and copper (Cu) undergo redox-cycling reactions, for a second group of redox-inactive metals such as arsenic (As) and cadmium (Cd), the primary route for their toxicity is depletion of glutathione and bonding to sulfhydryl groups of proteins. While arsenic is known to bind directly to critical thiols, other mechanisms, involving formation of hydrogen peroxide under physiological conditions, have been proposed. Redox-inert zinc (Zn) is the most abundant metal in the brain and an essential component of numerous proteins involved in biological defense mechanisms against oxidative stress. The depletion of zinc may enhance DNA damage by impairing DNA repair mechanisms. Intoxication of an organism by arsenic and cadmium may lead to metabolic disturbances of redox-active copper and iron, with the occurrence of oxidative stress induced by the enhanced formation of ROS/RNS. Oxidative stress occurs when excessive formation of ROS overwhelms the antioxidant defense system, as is maintained by antioxidants such as ascorbic acid, alpha

  7. 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

  8. Alkaline degradation studies of anion exchange polymers to enable new membrane designs

    Science.gov (United States)

    Nunez, Sean Andrew

    Current performance targets for anion-exchange membrane (AEM) fuel cells call for greater than 95% alkaline stability for 5000 hours at temperatures up to 120 °C. Using this target temperature of 120 °C, an incisive 1H NMR-based alkaline degradation method to identify the degradation products of n-alkyl spacer tetraalkylammonium cations in various AEM polymers and small molecule analogs. Herein, the degradation mechanisms and rates of benzyltrimethylammonium-, n-alkyl interstitial spacer- and n-alkyl terminal pendant-cations are studied on several architectures. These findings demonstrate that benzyltrimethylammonium- and n-alkyl terminal pendant cations are more labile than an n-alkyl interstitial spacer cation and conclude that Hofmann elimination is not the predominant mechanism of alkaline degradation. Additionally, the alkaline stability of an n-alkyl interstitial spacer cation is enhanced when combined with an n-alkyl terminal pendant. Interestingly, at 120 °C, an inverse trend was found in the overall alkaline stability of AEM poly(styrene) and AEM poly(phenylene oxide) samples than was previously shown at 80 °C. Successive small molecule studies suggest that at 120 °C, an anion-induced 1,4-elimination degradation mechanism may be activated on styrenic AEM polymers bearing an acidic alpha-hydrogen. In addition, an ATR-FTIR based method was developed to assess the alkaline stability of solid membranes and any added resistance to degradation that may be due to differential solubilities and phase separation. To increase the stability of anion exchange membranes, Oshima magnesate--halogen exchange was demonstrated as a method for the synthesis of new anion exchange membranes that typically fail in the presence of organolithium or Grignard reagents alone. This new chemistry, applied to non-resinous polymers for the first time, proved effective for the n-akyl interstitial spacer functionalization of poly(phenylene oxide) and poly(styrene- co

  9. 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.

  10. [Examination of acute phase proteins concentrations in children with allergic rhinitis].

    Science.gov (United States)

    Steiner, Iwona; Sobieska, Magdalena; Pucher, Beata; Grzegorowski, Michał; Samborski, Włodzimierz

    2006-01-01

    Allergic rhinitis is an inflammatory disorder of upper respiratory tract (about 15 per cent of the population in industrialized countries suffer from this condition), characterized by frequent sneezing and a runny or stuffy nose sometimes accompanied by watery eyes. As the most common allergic condition, allergic rhinitis affects people of all ages. Boys are twice as likely to get allergic rhinitis than girls. The median age of onset of the condition is 10 years old, meaning that equal numbers of children develop the condition before and after age 10. Symptoms usually appear in childhood first and then lessen by the age of 30 or 40. Seasonal allergic rhinitis usually results from tree, grass or weed pollen. With this type of rhinitis, symptoms will decrease with the arrival of cold weather. Perennial allergic rhinitis can cause year-round symptoms. This allergic reaction is the result of indoor irritants such as feathers, mold spores, animal dander (hair and skin shed by pets) or dust mites. It is often aggravated by a food allergy, the most common being an allergy to milk. Acute phase proteins (APP) belong to the most ancient part of the unspecific immunity and contribute markedly to the keeping of homeostasis. As much as 30 various proteins are for the moment regarded as APP. Being multifunctional regulators and effectors APP stay in multiple relations to practically all types of cells and molecules. Among APP following functional groups may be described: transport proteins (transferrin, ceruloplasmin and haptoglobin), clotting factors (fibrinogen), antiproteases (alpha1-antitrypsin, alpha1-antichymotrypsin, alpha2-macroglobulin), complement components (C3, C4) and several proteins of hardly known function, like C-reactive protein (CRP), serum amyloid A, acid alpha1-glycoprotein (AGP) and others. From a group of 32 children, aged from 5 to 14 years, with symptoms of seasonal allergic rhinitis, and from a control group of 10 healthy children sex and age matched

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

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    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

  12. 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

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    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

  13. 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

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

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    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. Avaliação da disponibilidade de ferro em ovo, cenoura e couve e em suas misturas Iron availability in egg, carrot and cabbage and in their mixtures

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    Flávia Maria Vasques Farinazzi Machado

    2006-09-01

    were analysed for the centesimal composition, iron, tannin, phytic acid, oxalic acid, alpha, beta-carotene and in vitro iron dialysability. The data was analysed using Tukey and correlation. A significant positive correlation of in vitro iron dialysability in relation to moisture and beta-carotene, negative for fat, proteins and ash was observed. The mixture of 1.5 c showed high levels of tannins and the mixtures of 1 cb and 1.5 cb high levels of oxalates and lower levels of in vitro iron dialysability in relation to the other mixtures. It can be concluded that the mixtures with larger carrot and cabbage proportions increased the iron availability and there was no interference from the antinutritional factors. The beta-carotene showed a significant positive correlation with in vitro iron dialysability influencing the decrease of the antinutritional factors effect in iron availability.

  16. 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.

  17. Enzyme replacement therapy for infantile-onset Pompe disease.

    Science.gov (United States)

    Chen, Min; Zhang, Lingli; Quan, Shuyan

    2017-11-20

    Infantile-onset Pompe disease is a rare and progressive autosomal-recessive disorder caused by a deficiency of the lysosomal enzyme acid alpha-glucosidase (GAA). Current treatment involves enzyme replacement therapy (with recombinant human alglucosidase alfa) and symptomatic therapies (e.g. to control secretions). Children who are cross-reactive immunological material (CRIM)-negative require immunomodulation prior to commencing enzyme replacement therapy.Enzyme replacement therapy was developed as the most promising therapeutic approach for Pompe disease; however, the evidence is lacking, especially regarding the optimal dose and dose frequency. To assess the effectiveness, safety and appropriate dose regimen of enzyme replacement therapy for treating infantile-onset Pompe disease. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Inborn Errors of Metabolism Trials Register, which is compiled from electronic database searches and handsearching of journals and conference abstract books. We also searched the Cochrane Central Register of Controlled Trials (CENTRAL), Embase (Ovid), PubMed and LILACS, and CBM, CNKI, VIP, and WANFANG for literature published in Chinese. In addition, we searched three online registers: WHO International Clinical Trials Registry Platform ClinicalTrials.gov, and www.genzymeclinicalresearch.com. We also searched the reference lists of relevant articles and reviews.Date of last search of the Group's Inborn Errors of Metabolism Trials Register: 24 November 2016. Randomized and quasi-randomized controlled trials of enzyme replacement therapy in children with infantile-onset Pompe disease. Two authors independently selected relevant trials, assessed the risk of bias and extracted data. We contacted investigators to obtain important missing information. We found no trials comparing the effectiveness and safety of enzyme replacement therapy to another intervention, no intervention or placebo.We found one trial (18 participants

  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

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    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. 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