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Carnitine metabolism
Stable Identifier
R-HSA-200425
DOI
10.3180/REACT_11082.1
Type
Pathway
Species
Homo sapiens
Compartment
mitochondrion
,
cytosol
Synonyms
Import of palmitoyl-CoA into the mitochondrial matrix
ReviewStatus
5/5
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Metabolism (Homo sapiens)
Metabolism of lipids (Homo sapiens)
Fatty acid metabolism (Homo sapiens)
Carnitine metabolism (Homo sapiens)
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The mitochondrial carnitine system catalyzes the transport of long-chain fatty acids into the mitochondrial matrix where they undergo beta oxidation. This transport system consists of the malonyl-CoA sensitive carnitine palmitoyltransferase I (CPT-I) localized in the mitochondrial outer membrane, the carnitine:acylcarnitine translocase, an integral inner membrane protein, and carnitine palmitoyltransferase II localized on the matrix side of the inner membrane. (Kerner and Hoppel, 2000).
Literature References
PubMed ID
Title
Journal
Year
11257506
Molecular enzymology of carnitine transfer and transport
Gandour, RD
,
Ramsay, RR
,
van der Leij, FR
Biochim Biophys Acta
2001
Participants
Events
OCTN2 / SLC22A5 transports CAR from extracellular space to cytosol
(Homo sapiens)
CAR translocates from the cytosol to the mitochondrial intermembrane space
(Homo sapiens)
CPT1A,B transfers PALM to CAR
(Homo sapiens)
Exchange of palmitoylcarnitine and carnitine across the inner mitochondrial membrane
(Homo sapiens)
palmitoylcarnitine + CoASH => palmitoyl-CoA + carnitine
(Homo sapiens)
MID1IP1 binds THRSP
(Homo sapiens)
Btn-ACACA:2Mn2+ polymer carboxylates Ac-CoA to form Mal-CoA
(Homo sapiens)
Btn-ACACB:2Mn2+ polymer carboxylates Ac-CoA to form Mal-CoA
(Homo sapiens)
pAMPK inactivates ACACB, inhibiting malonyl-CoA synthesis
(Homo sapiens)
Participates
as an event of
Fatty acid metabolism (Homo sapiens)
Event Information
Go Biological Process
carnitine shuttle (0006853)
Orthologous Events
Carnitine metabolism (Bos taurus)
Carnitine metabolism (Caenorhabditis elegans)
Carnitine metabolism (Canis familiaris)
Carnitine metabolism (Danio rerio)
Carnitine metabolism (Dictyostelium discoideum)
Carnitine metabolism (Drosophila melanogaster)
Carnitine metabolism (Gallus gallus)
Carnitine metabolism (Mus musculus)
Carnitine metabolism (Plasmodium falciparum)
Carnitine metabolism (Rattus norvegicus)
Carnitine metabolism (Saccharomyces cerevisiae)
Carnitine metabolism (Schizosaccharomyces pombe)
Carnitine metabolism (Sus scrofa)
Carnitine metabolism (Xenopus tropicalis)
Authored
Gopinathrao, G (2007-07-30)
Reviewed
D'Eustachio, P (2007-07-31)
Created
Gopinathrao, G (2007-07-18)
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