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Single amino acid supplementation in aminoacidopathies: a systematic review.

ABSTRACT: Aminoacidopathies are a group of rare and diverse disorders, caused by the deficiency of an enzyme or transporter involved in amino acid metabolism. For most aminoacidopathies, dietary management is the mainstay of treatment. Such treatment includes severe natural protein restriction, combined with protein substitution with all amino acids except the amino acids prior to the metabolic block and enriched with the amino acid that has become essential by the enzymatic defect. For some aminoacidopathies, supplementation of one or two amino acids, that have not become essential by the enzymatic defect, has been suggested. This so-called single amino acid supplementation can serve different treatment objectives, but evidence is limited. The aim of the present article is to provide a systematic review on the reasons for applications of single amino acid supplementation in aminoacidopathies treated with natural protein restriction and synthetic amino acid mixtures.

SUBMITTER: van Vliet D

PROVIDER: S-EPMC3895659 | biostudies-literature | 2014

REPOSITORIES: biostudies-literature

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Single amino acid supplementation in aminoacidopathies: a systematic review.

van Vliet Danique D Derks Terry G J TG van Rijn Margreet M de Groot Martijn J MJ MacDonald Anita A Heiner-Fokkema M Rebecca MR van Spronsen Francjan J FJ

Orphanet journal of rare diseases 20140113

Aminoacidopathies are a group of rare and diverse disorders, caused by the deficiency of an enzyme or transporter involved in amino acid metabolism. For most aminoacidopathies, dietary management is the mainstay of treatment. Such treatment includes severe natural protein restriction, combined with protein substitution with all amino acids except the amino acids prior to the metabolic block and enriched with the amino acid that has become essential by the enzymatic defect. For some aminoacidopat ...[more]

PMID: 24422943

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Project description:Poultry and swine farming are large contributors to environmental impacts, such as climate change, eutrophication, acidification, and air and water pollution. Feed production and manure management are identified as the main sources of these impacts. Reducing dietary crude protein levels is a nutritional strategy recognized to both decrease the use of high-impact feed ingredients and alter manure composition, reducing emissions of harmful components. For a successful implementation of this technique, feed-grade amino acid supplementation is crucial to maintaining animal performance. Reducing crude protein lowers nitrogen excretion, especially excess nitrogen excreted in urea or uric acid form, improving nitrogen efficiency. At the feed-gate, low-crude protein diets can reduce the carbon footprint of feed production through changes in raw material inclusion. The magnitude of this reduction mainly depends on the climate change impact of soybean meal and its land-use change on the feed-grade amino acids used. Reducing dietary crude protein also lowers the environmental impact of manure management in housing, storage, and at spreading: nitrogen emissions from manure (ammonia, nitrates, nitrous oxide) are reduced through reduction of nitrogen excretion. Moreover, synergetic effects exist with nitrogen form, water excretion, and manure pH, further reducing emissions. Volatilization of nitrogen is more reduced in poultry than in pigs, but emissions are more studied and better understood for pig slurry than poultry litter. Ammonia emissions are also more documented than other N-compounds. Low-crude protein diets supplemented with amino acids is a strategy reducing environmental impact at different stages of animal production, making life cycle assessment the best-suited tool to quantify reduction of environmental impacts. Recent studies report an efficient reduction of environmental impacts with low-crude protein diets. However, more standardization of limits and methods used is necessary to compare results. This review summarizes the current knowledge on mitigation of environmental impacts with low-crude protein diets supplemented with amino acids in poultry and swine, its quantification, and the biological mechanisms involved. A comparison between pigs and poultry is also included. It provides concrete information based on quantified research for decision making for the livestock industry and policy makers.

Project description:Mammals in northern regions chronically suffer from low temperatures during autumn-winter seasons. The aim of this study was to investigate the response of intestinal amino acid transport and the amino acid pool in muscle to chronic cold exposure via Min pig models (cold adaptation) and Yorkshire pig models (non-cold adaptation). Furthermore, this study explored the beneficial effects of glucose supplementation on small intestinal amino acid transport and amino acid pool in muscle of cold-exposed Yorkshire pigs. Min pigs (Exp. 1) and Yorkshire pigs (Exp. 2) were divided into a control group (17 °C, n = 6) and chronic cold exposure group (7 °C, n = 6), respectively. Twelve Yorkshire pigs (Exp. 3) were divided into a cold control group and cold glucose supplementation group (8 °C). The results showed that chronic cold exposure inhibited peptide transporter protein 1 (PepT1) and excitatory amino acid transporter 3 (EAAT3) expression in ileal mucosa and cationic amino acid transporter-1 (CAT-1) in the jejunal mucosa of Yorkshire pigs (P < 0.05). In contrast, CAT-1, PepT1 and EAAT3 expression was enhanced in the duodenal mucosa of Min pigs (P < 0.05). Branched amino acids (BCAA) in the muscle of Yorkshire pigs were consumed by chronic cold exposure, accompanied by increased muscle RING-finger protein-1 (MuRF1) and muscle atrophy F-box (atrogin-1) expression (P < 0.05). More importantly, reduced concentrations of dystrophin were detected in the muscle of Yorkshire pigs (P < 0.05). However, glycine concentration in the muscle of Min pigs was raised (P < 0.05). In the absence of interaction between chronic cold exposure and glucose supplementation, glucose supplementation improved CAT-1 expression in the jejunal mucosa and PepT1 expression in the ileal mucosa of cold-exposed Yorkshire pigs (P < 0.05). It also improved BCAA and inhibited MuRF1 and atrogin-1 expression in muscle (P < 0.05). Moreover, dystrophin concentration was improved by glucose supplementation (P < 0.05). In summary, chronic cold exposure inhibits amino acid absorption in the small intestine, depletes BCAA and promotes protein degradation in muscle. Glucose supplementation ameliorates the negative effects of chronic cold exposure on amino acid transport and the amino acid pool in muscle.

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Single amino acid supplementation in aminoacidopathies: a systematic review.

Publications

Single amino acid supplementation in aminoacidopathies: a systematic review.

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