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On the presence of C2-ceramide in mammalian tissues: possible relationship to etherphospholipids and phosphorylation by ceramide kinase.

[zellweger syndrome]

C(2)-ceramide (N-acetyl-sphingenine) is often used as an analog to study ceramide-mediated cellular processes. According to Lee et al. [J. Biol. Chem. 271 (1996), 209-217], C(2)-ceramide is formed by an acetyl transfer from platelet-activating factor (PAF, 1-O-alkyl-2-acetyl-sn-glycero-3-phosphocholine) to sphingenine. To substantiate these unconfirmed findings, we (i) developed a method to quantify C(2)-ceramide and (ii) analyzed C(2)-ceramide levels in Pex5(-/-) mice, a model for Zellweger syndrome, in which the synthesis of ether lipids such as PAF is impaired. The presence of C(2)-ceramide could be established in brain (+/-10 pmol/g) and liver (+/-25 pmol/g) from control mice, and was approximately 5000-fold less than the main long-chain ceramide species. In Pex5(-/-) mice, C(2)-ceramide levels did not differ significantly compared to control tissues. Given the presence of a ceramide kinase in mammals, phosphorylation of C(2)-ceramide by human ceramide kinase (HsCERK) was tested. C(2)-ceramide appears to be a good substrate when albumin is used as carrier. In CHO cells overexpressing HsCERK, phosphorylation of exogenously added C(2)-ceramide could also be demonstrated. Our data indicate that C(2)-ceramide is present in mammalian tissues and can be converted to C(2)-ceramide-1-phosphate, in addition to other documented metabolic alterations, but does not seem to be linked to ether lipid metabolism.

Diseases presenting "but does not seem to be linked to ether lipid metabolism" symptom

  • zellweger syndrome

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