PMID- 10486279
OWN - NLM
STAT- MEDLINE
DCOM- 19991021
LR  - 20171116
IS  - 0006-291X (Print)
IS  - 0006-291X (Linking)
VI  - 263
IP  - 1
DP  - 1999 Sep 16
TI  - Molecular cloning and expression of human carnitine octanoyltransferase: evidence
      for its role in the peroxisomal beta-oxidation of branched-chain fatty acids.
PG  - 213-8
AB  - To study the putative role of human carnitine octanoyltransferase (COT) in the
      beta-oxidation of branched-chain fatty acids, we identified and cloned the cDNA
      encoding human COT and expressed it in the yeast Saccharomyces cerevisiae. Enzyme
      activity measurements showed that COT efficiently converts one of the end
      products of the peroxisomal beta-oxidation of pristanic acid, 4,
      8-dimethylnonanoyl-CoA, to its corresponding carnitine ester. Production of the
      carnitine ester of this branched/medium-chain acyl-CoA within the peroxisome is
      required for its transport to the mitochondrion where further beta-oxidation
      occurs. In contrast, 4, 8-dimethylnonanoyl-CoA is not a substrate for carnitine
      acetyltransferase, another acyltransferase localized in peroxisomes, which
      catalyzes the formation of carnitine esters of the other products of pristanic
      acid beta-oxidation, namely acetyl-CoA and propionyl-CoA. Our results shed new
      light on the function of COT in fatty acid metabolism and point to a crucial role
      of COT in the beta-oxidation of branched-chain fatty acids.
CI  - Copyright 1999 Academic Press.
FAU - Ferdinandusse, S
AU  - Ferdinandusse S
AD  - Departments of Clinical Chemistry and Pediatrics, Emma Children's Hospital,
      Academic Medical Center, Amsterdam, 1100 DE, The Netherlands.
FAU - Mulders, J
AU  - Mulders J
FAU - IJlst, L
AU  - IJlst L
FAU - Denis, S
AU  - Denis S
FAU - Dacremont, G
AU  - Dacremont G
FAU - Waterham, H R
AU  - Waterham HR
FAU - Wanders, R J
AU  - Wanders RJ
LA  - eng
SI  - GENBANK/AF168793
PT  - Comparative Study
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Biochem Biophys Res Commun
JT  - Biochemical and biophysical research communications
JID - 0372516
RN  - 0 (DNA Primers)
RN  - 0 (DNA, Complementary)
RN  - 0 (Fatty Acids)
RN  - 0 (Recombinant Proteins)
RN  - 5FMQ2908AP (pristanic acid)
RN  - EC 2.3.1.- (Carnitine Acyltransferases)
RN  - EC 2.3.1.- (carnitine octanoyltransferase)
SB  - IM
MH  - Amino Acid Sequence
MH  - Animals
MH  - Base Sequence
MH  - Carnitine Acyltransferases/*genetics/*metabolism
MH  - Cattle
MH  - Cloning, Molecular
MH  - DNA Primers/genetics
MH  - DNA, Complementary/genetics
MH  - Fatty Acids/*metabolism
MH  - Gene Expression
MH  - Humans
MH  - In Vitro Techniques
MH  - Microbodies/metabolism
MH  - Molecular Sequence Data
MH  - Oxidation-Reduction
MH  - Rats
MH  - Recombinant Proteins/genetics/metabolism
MH  - Saccharomyces cerevisiae/genetics
MH  - Sequence Homology, Amino Acid
MH  - Species Specificity
EDAT- 1999/09/16 00:00
MHDA- 1999/09/16 00:01
CRDT- 1999/09/16 00:00
PHST- 1999/09/16 00:00 [pubmed]
PHST- 1999/09/16 00:01 [medline]
PHST- 1999/09/16 00:00 [entrez]
AID - 10.1006/bbrc.1999.1340 [doi]
AID - S0006-291X(99)91340-2 [pii]
PST - ppublish
SO  - Biochem Biophys Res Commun. 1999 Sep 16;263(1):213-8. doi:
      10.1006/bbrc.1999.1340.