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Originally published In Press as doi:10.1074/jbc.M001994200 on March 15, 2000

J. Biol. Chem., Vol. 275, Issue 21, 16329-16336, May 26, 2000
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Inactivation of the Peroxisomal Multifunctional Protein-2 in Mice Impedes the Degradation of Not Only 2-Methyl-branched Fatty Acids and Bile Acid Intermediates but Also of Very Long Chain Fatty Acids*

Myriam BaesDagger §, Steven HuygheDagger , Peter Carmeliet, Peter E. DeclercqDagger , Désiré Collen, Guy P. Mannaerts||, and Paul P. Van Veldhoven||

From the Dagger  Laboratory of Clinical Chemistry and || Laboratory of Pharmacology, K. U. Leuven, Herestraat 49 O/N, B 3000 Leuven, Belgium and the  Center for Transgene Technology and Gene Therapy, Flemish Institute for Biotechnology, Herestraat 49 O/N, B 3000 Leuven, Belgium

According to current views, peroxisomal beta -oxidation is organized as two parallel pathways: the classical pathway that is responsible for the degradation of straight chain fatty acids and a more recently identified pathway that degrades branched chain fatty acids and bile acid intermediates. Multifunctional protein-2 (MFP-2), also called D-bifunctional protein, catalyzes the second (hydration) and third (dehydrogenation) reactions of the latter pathway. In order to further clarify the physiological role of this enzyme in the degradation of fatty carboxylates, MFP-2 knockout mice were generated. MFP-2 deficiency caused a severe growth retardation during the first weeks of life, resulting in the premature death of one-third of the MFP-2-/- mice. Furthermore, MFP-2-deficient mice accumulated VLCFA in brain and liver phospholipids, immature C27 bile acids in bile, and, after supplementation with phytol, pristanic and phytanic acid in liver triacylglycerols. These changes correlated with a severe impairment of peroxisomal beta -oxidation of very long straight chain fatty acids (C24), 2-methyl-branched chain fatty acids, and the bile acid intermediate trihydroxycoprostanic acid in fibroblast cultures or liver homogenates derived from the MFP-2 knockout mice. In contrast, peroxisomal beta -oxidation of long straight chain fatty acids (C16) was enhanced in liver tissue from MFP-2-/- mice, due to the up-regulation of the enzymes of the classical peroxisomal beta -oxidation pathway. The present data indicate that MFP-2 is not only essential for the degradation of 2-methyl-branched fatty acids and the bile acid intermediates di- and trihydroxycoprostanic acid but also for the breakdown of very long chain fatty acids.

* These studies were supported by grants from Geconcerteerde Onderzoeksacties K.U. Leuven (GOA/99/09).The costs of publication of this article were defrayed in part by the payment of page charges. The article must therefore be hereby marked "advertisement" in accordance with 18 U.S.C. Section 1734 solely to indicate this fact.

§ Research associate of the "Fonds Wetenschappelijk Onderzoek Vlaanderen." To whom correspondence should be addressed: Laboratory of Clinical Chemistry, Herestraat 49 O/N, B 3000 Leuven, Belgium. Tel.: 32 16 347283; Fax: 32 16 347281; E-mail: myriam.baes@uz.kuleuven.ac.be.

Copyright © 2000 by The American Society for Biochemistry and Molecular Biology, Inc.
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