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4.1.1.72: branched-chain-2-oxoacid decarboxylase

This is an abbreviated version!
For detailed information about branched-chain-2-oxoacid decarboxylase, go to the full flat file.

Word Map on EC 4.1.1.72

Reaction

(3S)-3-methyl-2-oxopentanoate
=
2-methylbutanal
 +
CO2

Synonyms

alpha-keto acid decarboxylase, AlsS, BCKA, BCKAD, BCKDC, branched-chain 2-keto acid decarboxylase, branched-chain 2-oxoacid decarboxylase, branched-chain alpha-keto acid decarboxylase, branched-chain alpha-ketoacid dehydrogenase complex, branched-chain keto acid decarboxylase, branched-chain oxo acid decarboxylase, decarboxylase, branched-chain oxo acid, KDC, KdcA, MtKDC, THI3, thiamine metabolism regulatory protein, YDL080c

ECTree

     4 Lyases
         4.1 Carbon-carbon lyases
             4.1.1 Carboxy-lyases
                4.1.1.72 branched-chain-2-oxoacid decarboxylase

Disease

topprint

Disease on EC 4.1.1.72 - branched-chain-2-oxoacid decarboxylase

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DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
Acidosis
Acidosis and glucocorticoids induce branched-chain amino acid catabolism.
Chronic metabolic acidosis accelerates whole body proteolysis and oxidation in awake rats.
Glucocorticoids and acidification independently increase transcription of branched-chain ketoacid dehydrogenase subunit genes.
Mechanisms contributing to muscle-wasting in acute uremia: activation of amino acid catabolism.
Mechanisms for protein catabolism in uremia: metabolic acidosis and activation of proteolytic pathways.
Metabolic acidosis accelerates whole body protein degradation and leucine oxidation by a glucocorticoid-dependent mechanism.
Tissue-specific responses of branched-chain alpha-ketoacid dehydrogenase activity in metabolic acidosis.
Brain Diseases
Living donor liver transplantation in maple syrup urine disease - Case series and world's youngest domino liver donor and recipient.
branched-chain-2-oxoacid decarboxylase deficiency
Demonstration of a new mammalian isoleucine catabolic pathway yielding an Rseries of metabolites.
Carcinoma, Hepatocellular
Regulation of expression of branched-chain alpha-keto acid dehydrogenase subunits in permanent cell lines.
Maple Syrup Urine Disease
A distinct variant of intermediate maple syrup urine disease.
Altered kinetic properties of the branched-chain alpha-keto acid dehydrogenase complex due to mutation of the beta-subunit of the branched-chain alpha-keto acid decarboxylase (E1) component in lymphoblastoid cells derived from patients with maple syrup urine disease.
Maple syrup urine disease: branched-chain keto acid decarboxylation in fibroblasts as measured with amino acids and keto acids.
Neoplasms
Administration of endotoxin, tumor necrosis factor, or interleukin 1 to rats activates skeletal muscle branched-chain alpha-keto acid dehydrogenase.
Sepsis
Administration of endotoxin, tumor necrosis factor, or interleukin 1 to rats activates skeletal muscle branched-chain alpha-keto acid dehydrogenase.
Starvation
Effect of starvation on branched-chain alpha-keto acid dehydrogenase activity in rat heart and skeletal muscle.
Tuberculosis
Amino acids allosterically regulate the thiamine diphosphate-dependent alpha-keto acid decarboxylase from Mycobacterium tuberculosis.
Uremia
Glucocorticoids and acidification independently increase transcription of branched-chain ketoacid dehydrogenase subunit genes.