CRAT transfers ISB to CAR

Stable Identifier
R-HSA-9971123
Type
Reaction [transition]
Species
Homo sapiens
Compartment
mitochondrial matrix
Synonyms
2-methylpropanoyl-CoA + (R)-carnitine => O-isobutanoyl-(R)-carnitine + CoA
ReviewStatus
5/5
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CRAT transfers ISB to CAR
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Carnitine O-acetyltransferase (CRAT) transfers the isobutyryl (ISB) moiety from ISB-CoA to (R)-carnitine, producing isobutyrylcarnitine (ISB-CAR) (Violante, 2013).
Literature References
PubMed ID Title Journal Year
23485643 Substrate specificity of human carnitine acetyltransferase: Implications for fatty acid and branched-chain amino acid metabolism

Violante, S, Ijlst, L, Ruiter, J, Koster, J, Van Lenthe, H, Duran, M, de Almeida, IT, Wanders, RJ, Houten, SM, Ventura, FV

Biochim Biophys Acta 2013
Participants
Input
Output
Participates
as an event of
Catalyst Activity

carnitine O-acyltransferase activity of CRAT [mitochondrial inner membrane]

Physical Entity
CRAT [mitochondrial inner membrane] (Homo sapiens)
Activity
carnitine O-acyltransferase activity (GO:0016406)
Orthologous Events
CRAT transfers ISB to CAR (Bos taurus)
CRAT transfers ISB to CAR (Caenorhabditis elegans)
CRAT transfers ISB to CAR (Canis familiaris)
CRAT transfers ISB to CAR (Danio rerio)
CRAT transfers ISB to CAR (Drosophila melanogaster)
CRAT transfers ISB to CAR (Gallus gallus)
CRAT transfers ISB to CAR (Mus musculus)
CRAT transfers ISB to CAR (Rattus norvegicus)
CRAT transfers ISB to CAR (Saccharomyces cerevisiae)
CRAT transfers ISB to CAR (Sus scrofa)
CRAT transfers ISB to CAR (Xenopus tropicalis)
Cross References
RHEA
Authored
Stephan, R  (2025-11-01)
Reviewed
D'Eustachio, P  (2026-02-10)
Created
Stephan, R  (2025-11-04)
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