منابع مشابه
Branched-chain amino acid metabolism in cancer
PURPOSE OF REVIEW The current review aims to provide an update on the recent biomedical interest in oncogenic branched-chain amino acid (BCAA) metabolism, and discusses the advantages of using BCAAs and expression of BCAA-related enzymes in the treatment and diagnosis of cancers. RECENT FINDINGS An accumulating body of evidence demonstrates that BCAAs are essential nutrients for cancer growth...
متن کاملDisorders of branched chain amino acid metabolism
The three essential branched-chain amino acids (BCAAs), leucine, isoleucine and valine, share the first enzymatic steps in their metabolic pathways, including a reversible transamination followed by an irreversible oxidative decarboxylation to coenzyme-A derivatives. The respective oxidative pathways subsequently diverge and at the final steps yield acetyl- and/or propionyl-CoA that enter the K...
متن کاملRegulation of branched-chain amino acid catabolism.
Catabolism of the branched-chain amino acids is regulated in part at the step catalyzed by the branched-chain alpha-ketoacid dehydrogenase complex. Previous work suggests both short-term and long-term control mechanisms are involved in regulation of the kinase responsible for phosphorylation and inactivation of the branched-chain alpha-ketoacid dehydrogenase complex. Recent work of this laborat...
متن کاملRole of mitochondrial transamination in branched chain amino acid metabolism.
Oxidative decarboxylation and transamination of 1-14C-branched chain amino and alpha-keto acids were examined in mitochondria isolated from rat heart. Transamination was inhibited by aminooxyacetate, but not by L-cycloserine. At equimolar concentrations of alpha-ketoiso[1-14C]valerate (KIV) and isoleucine, transamination was increased by disrupting the mitochondria with detergent which suggests...
متن کاملBranched-Chain Amino Acids: Metabolism, Physiological Function, and Application Lessons from Genetic Disorders of Branched-Chain Amino Acid Metabolism
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. There are presently five kn...
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ژورنال
عنوان ژورنال: The Journal of Nutrition
سال: 1998
ISSN: 0022-3166,1541-6100
DOI: 10.1093/jn/128.7.1165