Mammalian peroxisomes degrade fatty carboxylates via two pathways, β-oxidation and, as shown more recently, α-oxidation. The latter process consists of an activation step, followed by a hydroxylation at position 2 and cleavage of the 2-hydroxyacyl-CoA, generating formyl-CoA (precursor of formate/CO2) and, in case of phytanic acid as substrate, pristanal (precursor of pristanic acid). The stereochemistry of the overall pathway, cofactor requirements and substrate specificity of the hydroxylase and the cleavage enzyme, which is homologous with bacterial oxalyl-CoA decarboxylases, will be discussed. With regard to β-oxidation, peroxisomes contain different acyl-CoA oxidases, multifunctional proteins and thiolases. Based on substrate spectra and stereospecificities of these enzymes, a model was proposed whereby straight chain and branched compounds are degraded by separate pathways. The biochemical findings in mice lacking the D-specific multifunctional protein, however, do not fully support this model. These animals, together with the Pex5−/− mice, might be useful to pinpoint the pathological factors contributing to the brain abnormalities in Zellweger patients. Apparently, the deficit in docosahexaenoic acid, presumably formed via peroxisomal β-oxidation, is not the major cause.
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Conference Article|
May 01 2001
Further insights into peroxisomal lipid breakdown via α- and β-oxidation
P. P. Van Veldhoven;
P. P. Van Veldhoven
1
*Katholieke Universiteit Leuven, Campus Gasthuisberg, Afdeling Farmacologie, Herestraat, B-3000 Leuven, Belgium
1To whom correspondence should be addressed (e-mail paul.vanveldhoven@med.kuleuven.ac.be)
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M. Casteels;
M. Casteels
*Katholieke Universiteit Leuven, Campus Gasthuisberg, Afdeling Farmacologie, Herestraat, B-3000 Leuven, Belgium
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G. P. Mannaerts;
G. P. Mannaerts
*Katholieke Universiteit Leuven, Campus Gasthuisberg, Afdeling Farmacologie, Herestraat, B-3000 Leuven, Belgium
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M. Baes
M. Baes
†Labo Klinische Chemie, Katholieke Universiteit Leuven, Campus Gasthuisberg, Herestraat, B-3000 Leuven, Belgium
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Publisher: Portland Press Ltd
Received:
November 10 2000
Online ISSN: 1470-8752
Print ISSN: 0300-5127
© 2001 Biochemical Society
2001
Biochem Soc Trans (2001) 29 (2): 292–297.
Article history
Received:
November 10 2000
Citation
P. P. Van Veldhoven, M. Casteels, G. P. Mannaerts, M. Baes; Further insights into peroxisomal lipid breakdown via α- and β-oxidation. Biochem Soc Trans 1 May 2001; 29 (2): 292–297. doi: https://doi.org/10.1042/bst0290292
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