Title: Mass spectrometric analysis of ceramide perturbations in brain and fibroblasts of mice and human patients with peroxisomal disorders
Authors: Pettus, Benjamin J ×
Baes, Myriam
Busman, Mark
Hannun, Yusuf A
Van Veldhoven, Paul P #
Issue Date: Jul-2004
Series Title: Rapid communications in mass spectrometry : RCM. vol:18 issue:14 pages:1569-1574
Abstract: In this study, the levels and composition of ceramides in brains of newborn mice lacking peroxisomes (Pex5-/-, Zellweger mice) were analyzed using normal-phase high-performance liquid chromatography/atmospheric pressure chemical ionization mass spectrometry (HPLC/APCI-MS). Total ceramide compositions were found to be comparable to that of control animals. However, a minor ceramide species, containing hexacosanoic/hexacosenoic acid as the amide fatty acid, was 9-fold increased. Also, in the sphingomyelin-derived ceramides this species was elevated. Subsequent analysis of extracts from fibroblasts of Pex5-/- mice and mice with a defective peroxisomal beta-oxidation (lacking D-specific multifunctional protein 2 (MFP2)), revealed, again, a similar rise in this particular ceramide. Further, this ceramide was elevated in human X-ALD fibroblasts as well. Whether C26:1/0-ceramide is linked to some of the pathology seen in Zellweger syndrome remains to be investigated. However, an increase in this sphingolipid can be considered as a diagnostic criterion for diseases caused by defects in peroxisome biogenesis or peroxisomal beta-oxidation.
ISSN: 0951-4198
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Cell Metabolism
Laboratory of Lipid Biochemistry and Protein Interactions
× corresponding author
# (joint) last author

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