ID:SCOT1_HUMAN DESCRIPTION: RecName: Full=Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial; EC=2.8.3.5; AltName: Full=3-oxoacid CoA-transferase 1; AltName: Full=Somatic-type succinyl-CoA:3-oxoacid CoA-transferase; Short=SCOT-s; Flags: Precursor; 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";
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on P55809
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
Biological Process: GO:0007420 brain development GO:0007507 heart development GO:0007584 response to nutrient GO:0008152 metabolic process GO:0009725 response to hormone GO:0014823 response to activity GO:0035774 positive regulation of insulin secretion involved in cellular response to glucose stimulus GO:0042182 ketone catabolic process GO:0042493 response to drug GO:0042594 response to starvation GO:0045471 response to ethanol GO:0046950 cellular ketone body metabolic process GO:0046952 ketone body catabolic process GO:0060612 adipose tissue development