Cholesteryl lignocerate hydrolysis in adrenoleukodystrophy.
Because cholesteryl esters with very long chain fatty acids accumulate in Schilder adrenoleukodystrophy, the ability of extracts of such fibroblasts to hydrolyze [14C]cholesteryl lignocerate was examined. Hydrolytic activity was detected at pH 3.0, and this activity was impaired by sulfhydryl inactivating agents. Cholesteryl lignocerate hydrolysis was deficient in cells from patients with cholesteryl ester storage disease or Wolman disease due to acid lipase deficiency, but was in the control range for adrenoleukodystrophy fibroblasts. This suggests that cholesteryl lignocerate hydrolysis can be carried out by acid lipase.[1]References
- Cholesteryl lignocerate hydrolysis in adrenoleukodystrophy. Michels, V.V., Beaudet, A.L. Pediatr. Res. (1980) [Pubmed]
Annotations and hyperlinks in this abstract are from individual authors of WikiGenes or automatically generated by the WikiGenes Data Mining Engine. The abstract is from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.About WikiGenesOpen Access LicencePrivacy PolicyTerms of Useapsburg