Branched chain amino acid metabolism
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Branched chain amino acid metabolism requirements in healthy adult men and patients with maple syrup urine disease. by Roya Riazi

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Published .
Written in English

Book details:

Edition Notes

Thesis (Ph.D.) -- University of Toronto, 2003.

The Physical Object
Pagination225 leaves.
Number of Pages225
ID Numbers
Open LibraryOL19984705M
ISBN 100612848116

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  Increased levels of branched-chain amino acids (BCAAs) are associated with type 2 diabetes (T2D) pathogenesis. However, most metabolomic studies in T2D are limited to an analysis of plasma metabolites under fasting conditions, rather than the dynamic shift in response to a glucose challenge. Moreover, metabolomic profiles of peripheral tissues involved in glucose homeostasis are Author: Rasmus J.O. Sjögren, David Rizo-Roca, Alexander V. Chibalin, Elin Chorell, Regula Furrer, Shintaro K. Branched-chain amino acid metabolism, insulin sensitivity and liver fat response to exercise training in sedentary dysglycaemic and normoglycaemic men SindreLee1,2,3 & h4 & ite1 & FrodeNorheim1 & ThomasOlsen1 & HelgaRefsum1 & Jørgen Jensen5 & Kåre I. Birkeland2,3 & Christian A. Drevon1Author: Sindre Lee, Sindre Lee, Hanne L. Gulseth, Torgrim M. Langleite, Frode Norheim, Thomas Olsen, Helga R. Branched-chain amino acids (BCAA) are used as nutritional support for patients with a range of conditions including liver cirrhosis and in-born errors of amino acid metabolism, and they are. Genetic disorders of BCAA metabolism produce amino acidopathies and various forms of organic aciduria with severe clinical consequences. A metabolic block in the oxidative decarboxylation of BCAA caused by mutations in the mitochondrial branched-chain a-keto acid dehydrogenase complex (BCKDC) results in Maple Syrup Urine Disease (MSUD) or branched-chain ketoaciduria.

Branched chain amino acids (BCAAs), including leucine (Leu), isoleucine (Ile), and valine (Val), play critical roles in the regulation of energy homeostasis, nutrition metabolism, gut health, immunity and disease in humans and animals. As the most abundant of essential amino acids (EAAs), BCAAs are Cited by:   Branched Chain Amino Acid Metabolism and Maple Syrup Urine Disease. Metabolic Pathway of Branched Chain Amino Acids (Leucine, Isoleucine, and Valine) Transamination of Branched Chain Amino Acid - The first step in the catabolism of branched-chain amino acids including leucine, isoleucine, and valine. Valine, leucine, and isoleucine are branched-chain amino acids; deficiency of enzymes involved in their metabolism leads to accumulation of organic acids with severe metabolic acidosis. There are numerous disorders of branched-chain amino acid metabolism (see the table) as well as many other amino acid and organic acid metabolism disorders.   Accumulation of branched chain amino acids causes an impairment in transport & function of other amino acids. Protein biosynthesis is reduced. Branched chain amino acids competitively inhibit glutamate dehydrogenase. The disease results in acidosis, lethargy, convulsions, mental retardation, coma & death within one year after birth.

Maple syrup urine disease (MSUD) is a deficiency of branched-chain ketoacid dehydrogenase (Fig. , reaction 2), a mitochondrial enzyme. Decarboxylation of the branched-chain ketoacids, derived from transamination of branched-chain amino acids (BCAA), proceeds via a reaction for which the cofactors are thiamine pyrophosphate, lipoic acid, NAD, FAD and coenzyme : Marc Yudkoff. Branched-Chain Amino Acid Metabolism in Arabidopsis Thaliana Arabidopsis Book. ;8:e doi: /tab Epub Aug Author Stefan Binder 1 Cited by: Amino Acid Metabolism, 3rd Edition covers all aspects of the biochemistry and nutritional biochemistry of the amino acids. Starting with an overview of nitrogen fixation and the incorporation of inorganic nitrogen into amino acids, the book then details other major nitrogenous compounds in micro-organisms, plants and animals. Contents include a discussion of the catabolism of amino acids and Reviews: 1. This is the first volume in a 2-volume compendium that is the go-to source for both research- and practice-oriented information on the importance of branched chain amino acids in maintaining the nutritional status and overall health of individuals, especially those with certain disease conditions.