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- Table of Contents
Facts about Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial.
Formation of the enzyme-CoA intermediate proceeds via an unstable anhydride species formed between the carboxylate groups of the enzyme and substrate. .
Human | |
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Gene Name: | OXCT1 |
Uniprot: | P55809 |
Entrez: | 5019 |
Belongs to: |
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3-oxoacid CoA-transferase family |
3-oxoacid CoA transferase 1,3-oxoacid CoA transferase; EC 2.8.3; EC 2.8.3.5; SCOTmitochondrial
Mass (kDA):
56.158 kDA
Human | |
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Location: | 5p13.1 |
Sequence: | 5; NC_000005.10 (41730065..41870535, complement) |
Abundant in heart, followed in order by kidney, brain, and muscle, whereas in liver it is undetectable; also detectable in leukocytes and fibroblasts.
Mitochondrion matrix.
PMID: 8751852 by Kassovska-Bratinova S., et al. Succinyl CoA:3-oxoacid CoA transferase (SCOT): human cDNA cloning, human chromosomal mapping to 5p13, and mutation detection in a SCOT- deficient patient.
PMID: 10964512 by Fukao T., et al. Succinyl-CoA:3-ketoacid CoA transferase (SCOT): cloning of the human SCOT gene, tertiary structural modeling of the human SCOT monomer, and characterization of three pathogenic mutations.