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Vol. 12, No. 8, pp. 1189-1201, April 15, 1998
1 Institute for Arteriosclerosis Research and
2 Institute for Clinical Chemistry and Laboratory Medicine
(Zentrallaboratorium), Westfalian Wilhelms-University, D-48129
Münster, Germany;
3 Department of Pediatrics, Academic
Medical Center, University of Amsterdam, 1105 AZ Amsterdam, The
Netherlands;
4 Center for Biomembranes and Lipid Enzymology,
University of Utrecht, De Uithof, Utrecht, The Netherlands;
5 Department of Pathology, University of North Carolina at
Chapel Hill, Chapel Hill, North Carolina 27599-7525 USA
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.
[Key Words:
Gene targeting; peroxisomes; 
-oxidation; Refsum disease; cholesterol; steroid hormones]
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