Recombinant Human Succinyl-CoA:3-ketoacid-coenzyme A transferase 1, mitochondrial (OXCT1)

Uniprot Id

P55809

Target Species

Human

Target Name

OXCT1

Target Full Name

Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial

Target 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.

Target Involvement

Succinyl-CoA:3-oxoacid CoA transferase deficiency (SCOTD)

Target Subcellular Location

Mitochondrion matrix.

Target Protein Families

3-oxoacid CoA-transferase family

Target 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.

Target Research Area

Cancer

Target Synonyms

3 oxoacid CoA transferase 1; 3-oxoacid CoA-transferase 1; EC 2.8.3.5; OTTHUMP00000120012; OTTHUMP00000221550; OXCT; Oxct1; SCOT; Scot S; Scot-S; SCOT1_HUMAN; Somatic type succinyl CoA:3 oxoacid CoA transferase; Somatic-type succinyl-CoA:3-oxoacid CoA-transferase; Succinyl CoA:3 ketoacid CoA transferase; Succinyl CoA:3 ketoacid coenzyme A transferase 1 mitochondrial; Succinyl CoA:3 oxoacid CoA transferase; Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial

Target Background

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.