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succinyl-CoA:3-ketoacid-coenzyme A transferase 1(somatic-type succinyl CoA:3-oxoacid CoA-transferase, Scot-S, OXCT1, OXCT, SCOT)
Function:
1) ketone body catabolism, ketolysis, 1st step
2) transfers the CoA moiety from succinate to acetoacetate
3) 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
General
mitochondrial protein
transferase
Properties
SIZE: MW = 56 kD
entity length = 520 aa
COMPARTMENT: mitochondrial matrix
MOTIF: binding site
SITE: 62-68
FOR-BINDING-OF: coenzyme A
glutamate residue {E344}
Database Correlations
OMIM 245050
UniProt P55809
Pfam PF01144
ENZYME 2.8.3.5
References
- UniProt :accession P55809
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=OXCT