AAH09001.1
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UniProt Secondary Accession #
UniProt Related Accession #
NCBI Official Full Name
3-oxoacid CoA transferase 1
NCBI Official Synonym Full Names
3-oxoacid CoA transferase 1
NCBI Official Synonym Symbols
NCBI Protein Information
succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial; SCOT-s; 3-oxoacid CoA-transferase 1; 3-oxoacid-CoA transferase 1; succinyl CoA:3-oxoacid CoA transferase; succinyl-CoA:3-ketoacid-CoA transferase; somatic-type succinyl CoA:3-oxoacid CoA-transferase; somatic-type succinyl-CoA:3-oxoacid CoA-transferase; somatic-type succinyl-CoA:3-oxoacid-CoA-transferase; succinyl-CoA:3-ketoacid-coenzyme A transferase 1, mitochondrial
UniProt Protein Name
Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial
UniProt Synonym Protein Names
3-oxoacid CoA-transferase 1; Somatic-type succinyl-CoA:3-oxoacid CoA-transferase; SCOT-s
UniProt Synonym Gene Names
UniProt Entry Name
SCOT1_HUMAN
NCBI Summary for OXCT1
This gene encodes a member of the 3-oxoacid CoA-transferase gene family. The encoded protein is a homodimeric mitochondrial matrix enzyme that plays a central role in extrahepatic ketone body catabolism by catalyzing the reversible transfer of coenzyme A from succinyl-CoA to acetoacetate. Mutations in this gene are associated with succinyl CoA:3-oxoacid CoA transferase deficiency. [provided by RefSeq, Jul 2008]
UniProt Comments for OXCT1
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.
Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; Mitochondrial; Transferase; Lipid Metabolism - synthesis and degradation of ketone bodies; EC 2.8.3.5; Carbohydrate Metabolism - butanoate
Chromosomal Location of Human Ortholog: 5p13.1
Cellular Component: mitochondrion; mitochondrial matrix
Molecular Function: protein homodimerization activity; 3-oxoacid CoA-transferase activity
Biological Process: response to drug; response to starvation; response to ethanol; heart development; ketone body catabolic process; response to hormone stimulus; ketone catabolic process; cellular lipid metabolic process; brain development; ketone body metabolic process; response to activity; response to nutrient
Disease: Succinyl-coa:3-oxoacid Coa Transferase Deficiency
Research Articles on OXCT1
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Products associated with anti-OXCT1 antibody
Pathways associated with anti-OXCT1 antibody
Diseases associated with anti-OXCT1 antibody
Organs/Tissues associated with anti-OXCT1 antibody
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