PMID- 9553048
OWN - NLM
STAT- MEDLINE
DCOM- 19980528
LR  - 20190516
IS  - 0890-9369 (Print)
IS  - 0890-9369 (Linking)
VI  - 12
IP  - 8
DP  - 1998 Apr 15
TI  - Defective peroxisomal catabolism of branched fatty acyl coenzyme A in mice 
      lacking the sterol carrier protein-2/sterol carrier protein-x gene function.
PG  - 1189-201
AB  - Gene targeting in mice was used to investigate the unknown function of Scp2, 
      encoding sterol carrier protein-2 (SCP2; a peroxisomal lipid carrier) and sterol 
      carrier protein-x (SCPx; a fusion protein between SCP2 and a peroxisomal 
      thiolase). Complete deficiency of SCP2 and SCPx was associated with marked 
      alterations in gene expression, peroxisome proliferation, hypolipidemia, impaired 
      body weight control, and neuropathy. Along with these abnormalities, catabolism 
      of methyl-branched fatty acyl CoAs was impaired. The defect became evident from 
      up to 10-fold accumulation of the tetramethyl-branched fatty acid phytanic acid 
      in Scp2(-/-) mice. Further characterization supported that the gene disruption 
      led to inefficient import of phytanoyl-CoA into peroxisomes and to defective 
      thiolytic cleavage of 3-ketopristanoyl-CoA. These results corresponded to 
      high-affinity binding of phytanoyl-CoA to the recombinant rat SCP2 protein, as 
      well as high 3-ketopristanoyl-CoA thiolase activity of the recombinant rat SCPx 
      protein.
FAU - Seedorf, U
AU  - Seedorf U
AD  - Institute for Arteriosclerosis Research, Westfalian Wilhelms-University, D-48129 
      M-unster, Germany. seedorf@ear002.uni-muenster.de
FAU - Raabe, M
AU  - Raabe M
FAU - Ellinghaus, P
AU  - Ellinghaus P
FAU - Kannenberg, F
AU  - Kannenberg F
FAU - Fobker, M
AU  - Fobker M
FAU - Engel, T
AU  - Engel T
FAU - Denis, S
AU  - Denis S
FAU - Wouters, F
AU  - Wouters F
FAU - Wirtz, K W
AU  - Wirtz KW
FAU - Wanders, R J
AU  - Wanders RJ
FAU - Maeda, N
AU  - Maeda N
FAU - Assmann, G
AU  - Assmann G
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Genes Dev
JT  - Genes & development
JID - 8711660
RN  - 0 (Acyl Coenzyme A)
RN  - 0 (Carrier Proteins)
RN  - 0 (Plant Proteins)
RN  - 0 (Sterols)
RN  - 0 (sterol carrier proteins)
RN  - 150-86-7 (Phytol)
RN  - EC 2.3.1.9 (Acetyl-CoA C-Acetyltransferase)
SB  - IM
MH  - Acetyl-CoA C-Acetyltransferase/genetics/physiology
MH  - Acyl Coenzyme A/*metabolism
MH  - Amino Acid Sequence
MH  - Animals
MH  - Carrier Proteins/*genetics/*physiology
MH  - Female
MH  - Gene Deletion
MH  - Gene Expression Regulation
MH  - Male
MH  - Mice
MH  - Mice, Inbred C57BL
MH  - Mice, Knockout
MH  - Microbodies/*metabolism
MH  - Molecular Sequence Data
MH  - Phytol/metabolism
MH  - *Plant Proteins
MH  - Sterols/*metabolism
PMC - PMC316706
EDAT- 1998/05/30 00:00
MHDA- 1998/05/30 00:01
PMCR- 1998/10/15
CRDT- 1998/05/30 00:00
PHST- 1998/05/30 00:00 [pubmed]
PHST- 1998/05/30 00:01 [medline]
PHST- 1998/05/30 00:00 [entrez]
PHST- 1998/10/15 00:00 [pmc-release]
AID - 10.1101/gad.12.8.1189 [doi]
PST - ppublish
SO  - Genes Dev. 1998 Apr 15;12(8):1189-201. doi: 10.1101/gad.12.8.1189.