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HIBCH
Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite. Has high activity toward isobutyryl-CoA. Could be an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Also hydrolyzes 3-hydroxypropanoyl-CoA. Belongs to the enoyl-CoA hydratase/isomerase family. Highly expressed in liver and kidney, also detected in heart, muscle and brain (at protein level). Not detected in lung. 2 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
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| Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - propanoate; EC 3.1.2.4; Hydrolase; Mitochondrial; Other Amino Acids Metabolism - beta-alanine |
| Chromosomal Location of Human Ortholog: 2q32.2 |
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Cellular Component:
mitochondrial matrix
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Molecular Function:
3-hydroxyisobutyryl-CoA hydrolase activity
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Biological Process:
branched-chain amino acid catabolic process; valine catabolic process
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Disease: Beta-hydroxyisobutyryl Coa Deacylase Deficiency
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Reference #:
Q6NVY1
(UniProtKB)
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Alt. Names/Synonyms: 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial; 3-hydroxyisobutyryl-coenzyme A hydrolase; HIB-CoA hydrolase; HIBCH; HIBYL-CoA-H
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Gene Symbols: HIBCH
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Molecular weight:
43,482 Da
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Basal Isoelectric point:
8.38
Predict pI for various phosphorylation states
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