PMID- 10198260
OWN - NLM
STAT- MEDLINE
DCOM- 19990524
LR  - 20171116
IS  - 0006-291X (Print)
IS  - 0006-291X (Linking)
VI  - 257
IP  - 2
DP  - 1999 Apr 13
TI  - Human very-long-chain acyl-CoA synthetase: cloning, topography, and relevance to 
      branched-chain fatty acid metabolism.
PG  - 615-21
AB  - Very-long-chain acyl-CoA synthetases (VLCS) activate very-long-chain fatty acids 
      (VLCFA) containing 22 or more carbons to their CoA derivatives. We cloned the
      human ortholog (hVLCS) of the gene encoding the rat liver enzyme (rVLCS). Both
      hVLCS and rVLCS contain 620 amino acids, are expressed primarily in liver and
      kidney, and have a potential peroxisome targeting signal 1 (-LKL) at their
      carboxy termini. When expressed in COS-1 cells, hVLCS activated the VLCFA
      lignoceric acid (C24:0), a long-chain fatty acid (C16:0), and two branched-chain 
      fatty acids, phytanic acid and pristanic acid. Immunofluorescence and immunoblot 
      studies localized hVLCS to both peroxisomes and endoplasmic reticulum. In
      peroxisomes of HepG2 cells, hVLCS was topographically oriented facing the matrix 
      and not the cytoplasm. This orientation, coupled with the observation that hVLCS 
      activates branched-chain fatty acids, suggests that hVLCS could play a role in
      the intraperoxisomal reactivation of pristanic acid produced via alpha-oxidation 
      of phytanic acid.
CI  - Copyright 1999 Academic Press.
FAU - Steinberg, S J
AU  - Steinberg SJ
AD  - Kennedy Krieger Research Institute, Johns Hopkins University School of Medicine, 
      Baltimore, Maryland, 21205, USA.
FAU - Wang, S J
AU  - Wang SJ
FAU - Kim, D G
AU  - Kim DG
FAU - Mihalik, S J
AU  - Mihalik SJ
FAU - Watkins, P A
AU  - Watkins PA
LA  - eng
SI  - GENBANK/AF096290
GR  - DK51149/DK/NIDDK NIH HHS/United States
GR  - HD10981/HD/NICHD NIH HHS/United States
GR  - NS10533/NS/NINDS NIH HHS/United States
PT  - Journal Article
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - United States
TA  - Biochem Biophys Res Commun
JT  - Biochemical and biophysical research communications
JID - 0372516
RN  - 0 (Biomarkers)
RN  - 0 (Fatty Acids)
RN  - 14721-66-5 (Phytanic Acid)
RN  - 5FMQ2908AP (pristanic acid)
RN  - EC 6.2.1.- (Coenzyme A Ligases)
RN  - RK3VCW5Y1L (lignoceric acid)
SB  - IM
MH  - Amino Acid Sequence
MH  - Animals
MH  - Base Sequence
MH  - Biomarkers/analysis
MH  - Cell Line
MH  - Cloning, Molecular
MH  - Coenzyme A Ligases/analysis/chemistry/*genetics/metabolism
MH  - Endoplasmic Reticulum/chemistry
MH  - Fatty Acids/*metabolism
MH  - Fluorescent Antibody Technique, Indirect
MH  - Humans
MH  - Liver/enzymology
MH  - Microbodies/chemistry/metabolism
MH  - Molecular Sequence Data
MH  - Organ Specificity
MH  - Phytanic Acid/metabolism
MH  - Rats
MH  - Sequence Homology, Amino Acid
MH  - Substrate Specificity
MH  - Transfection
EDAT- 1999/04/13 00:00
MHDA- 1999/04/13 00:01
CRDT- 1999/04/13 00:00
PHST- 1999/04/13 00:00 [pubmed]
PHST- 1999/04/13 00:01 [medline]
PHST- 1999/04/13 00:00 [entrez]
AID - S0006-291X(99)90510-7 [pii]
AID - 10.1006/bbrc.1999.0510 [doi]
PST - ppublish
SO  - Biochem Biophys Res Commun. 1999 Apr 13;257(2):615-21. doi:
      10.1006/bbrc.1999.0510.