Cholesteryl lignocerate hydrolysis in adrenoleukodystrophy.

Abstract

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.

• Authors: V V Michels, A L Beaudet
• Journal: Pediatric research (Pediatr Res) Vol. 14 Issue 1 Pg. 21-3 (Jan 1980) ISSN: 0031-3998 [Print] United States
• PMID: 6767215 (Publication Type: Comparative Study, Journal Article, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Cholesterol Esters
• Triolein
• cholesteryl oleate
• cholesteryl lignocerate

Topics

• Cholesterol Esters (metabolism)
• Diffuse Cerebral Sclerosis of Schilder (metabolism)
• Fibroblasts (metabolism)
• Humans
• Hydrolysis
• In Vitro Techniques
• Skin (metabolism)
• Triolein (metabolism)
• Xanthogranuloma, Juvenile (metabolism)