| Predicate | Object |
|---|---|
| rdf:type | |
| biopax3:comment |
FUNCTION: 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. CATALYTIC ACTIVITY: Succinyl-CoA + a 3-oxo acid = succinate + a 3- oxoacyl-CoA. PATHWAY: Ketone metabolism; succinyl-CoA degradation; acetoacetyl- CoA from succinyl-CoA: step 1/1. SUBUNIT: Homodimer. SUBCELLULAR LOCATION: Mitochondrion matrix. TISSUE SPECIFICITY: Abundant in heart, followed in order by kidney, brain, and muscle, whereas in liver it is undetectable; also detectable in leukocytes and fibroblasts. DISEASE: Defects in OXCT1 are a cause of succinyl-CoA-3-ketoacid- CoA transferase deficiency (SCOTD) [MIM:245050]. A disorder of ketone body metabolism, characterized by episodic ketoacidosis. Patients are usually asymptomatic between episodes. SIMILARITY: Belongs to the 3-oxoacid CoA-transferase family. WEB RESOURCE: Name=GeneReviews; URL="http://www.ncbi.nlm.nih.gov/sites/GeneTests/lab/gene/OXCT1"; GENE SYNONYMS:OXCT1 OXCT SCOT. COPYRIGHT: Protein annotation is derived from the UniProt Consortium (http://www.uniprot.org/). Distributed under the Creative Commons Attribution-NoDerivs License.,
SEQUENCE 520 AA; 56158 MW; 54DA790FB8EDA546 CRC64;
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| biopax3:xref | |
| biopax3:displayName |
SCOT1_HUMAN
|
| biopax3:name |
2.8.3.5,
3-oxoacid CoA-transferase 1,
OXCT1,
SCOT-s,
Somatic-type succinyl-CoA:3-oxoacid CoA-transferase
|
| biopax3:organism | |
| biopax3:sequence |
MAALKLLSSGLRLCASARGSGATWYKGCVCSFSTSAHRHTKFYTDPVEAVKDIPDGATVLVGGFGLCGIPENLIDALLKTGVKGLTAVSNNAGVDNFGLGLLLRSKQIKRMVSSYVGENAEFERQYLSGELEVELTPQGTLAERIRAGGAGVPAFYTPTGYGTLVQEGGSPIKYNKDGSVAIASKPREVREFNGQHFILEEAITGDFALVKAWKADRAGNVIFRKSARNFNLPMCKAAETTVVEVEEIVDIGAFAPEDIHIPQIYVHRLIKGEKYEKRIERLSIRKEGDGEAKSAKPGDDVRERIIKRAALEFEDGMYANLGIGIPLLASNFISPNITVHLQSENGVLGLGPYPRQHEADADLINAGKETVTILPGASFFSSDESFAMIRGGHVDLTMLGAMQVSKYGDLANWMIPGKMVKGMGGAMDLVSSAKTKVVVTMEHSAKGNAHKIMEKCTLPLTGKQCVNRIITEKAVFDVDKKKGLTLIELWEGLTVDDVQKSTGCDFAVSPKLMPMQQIAN
|
| biopax3:standardName |
Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial
|