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OXCT1
Key enzyme for ketone body catabolism. Transfers the CoA moiety from succinate to acetoacetate. Formation of the enzyme-CoA intermediate proceeds via an unstable anhydride species formed between the carboxylate groups of the enzyme and substrate. Defects in OXCT1 are a cause of succinyl-CoA-3-ketoacid- CoA transferase deficiency (SCOTD). A disorder of ketone body metabolism, characterized by episodic ketoacidosis. Patients are usually asymptomatic between episodes. Belongs to the 3-oxoacid CoA-transferase family. Note: This description may include information from UniProtKB.
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| Protein type: Transferase; Carbohydrate Metabolism - butanoate; EC 2.8.3.5; Mitochondrial; Lipid Metabolism - synthesis and degradation of ketone bodies; Amino Acid Metabolism - valine, leucine and isoleucine degradation |
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Cellular Component: mitochondrion; mitochondrial matrix
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Molecular Function: protein homodimerization activity; 3-oxoacid CoA-transferase activity
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Biological Process: ketone body catabolic process; cellular lipid metabolic process; ketone body metabolic process
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Reference #:
P55809 (UniProtKB)
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| Alt. Names/Synonyms: 3-oxoacid CoA transferase 1; 3-oxoacid-CoA transferase 1; OXCT; OXCT1; SCOT; SCOT-s; SCOT1; somatic-type succinyl CoA:3-oxoacid CoA-transferase; Somatic-type succinyl-CoA:3-oxoacid-CoA-transferase; succinyl CoA:3-oxoacid CoA transferase; succinyl-CoA:3-ketoacid-CoA transferase; Succinyl-CoA:3-ketoacid-coenzyme A transferase 1, mitochondrial |
| Gene Symbols: OXCT1 |
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Molecular weight: 56,158 Da
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Basal Isoelectric point: 7.13
Predict pI for various phosphorylation states
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