The in situ degradation of ceramide, a potential lipid mediator, is not completely impaired in Farber disease

FEBS Letters

Tempesta, Marie-Caroline¹  Salvayre, Robert¹  Levade, Thierry¹ 

[1] Laboratoire de Biochimie, ‘Maladies Métaboliques’, CJF INSERM 9206, Institut Louis Bugnard, CHU Rangueil, Toulouse, France

关键词: Ceramide;    Lysosome;    Farber disease;    Ceramidase;    Sphingomyelin;    Lipid targetting;    SPM;    sphingomyelin;    LDL;    low density lipoprotein;    TLC;    thin-layer chromatography;   

DOI  :  10.1016/0014-5793(93)80243-N

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 摘 要 】

The time course of degradation of a radiolabelled natural ceramide has been studied in intact, living lymphoid cells and skin fibroblasts from normal individuals and from patients affected with Farber disease, an inborn disorder of ceramide metabolism due to deficient activity of lysosomal ceramidase. The hydrolysis of ceramide in lysosomes was selectively followed by examining the turnover of an LDL-associated radioactive sphingomyelin. This permitted to estimate accurately the effective lysosomal ceramidase activity and to demonstrate: (i) a very active catabolism of ceramide in normal cells; and (ii) the absence of a complete block of ceramide degradation in Farber cells. The possible implication of ceramide as a lipid mediator of the pathogenesis of Farber disease is discussed.

【 授权许可】

Unknown