succinyl-CoA:3-ketoacid-coenzyme A transferase 1(somatic-type succinyl CoA:3-oxoacid CoA-transferase, Scot-S, OXCT1, OXCT, SCOT)

Function

• ketone body catabolism, ketolysis, 1st step
• transfers the CoA moiety from succinate to acetoacetate
• formation of the enzyme-CoA intermediate proceeds via an unstable anhydride species formed between carboxylate groups of enzyme & substrate

Succinyl-CoA + 3-oxo acid <--> succinate + 3-oxoacyl-CoA

Structure

homodimer

Compartment

mitochondrial matrix

Expression

• expressed in heart > kidney, brain, muscle
• expressed in leukocytes, fibroblasts

Pathology

• defects in OXCT1 results in ketoacidosis

More general terms

• transferase
• mitochondrial protein

Database

• OMIM: https://mirror.omim.org/entry/245050
• UniProt: http://www.uniprot.org/uniprot/P55809.html