In:
FEBS Letters, Wiley, Vol. 508, No. 1 ( 2001-11-09), p. 107-110
Kurzfassung:
As previously shown for [ 3 H‐galactosyl]ceramide, the breakdown of [ 3 H‐galactosyl]sphingosine was reduced in prosaposin‐deficient skin fibroblast homogenates. Galactosylsphingosine hydrolysis was also deficient in cell homogenates from Krabbe's disease (β‐galactocerebrosidase‐deficient) patients, but not acid β‐galactosidase‐deficient patients. Moreover, hydrolysis of galactosylsphingosine in the prosaposin‐deficient cell homogenates could be partially restored by adding pure saposin A or C, thereby identifying these saposins as essential facilitators of galactosylsphingosine hydrolysis. By contrast, saposins B and D had little effect on galactosylsphingosine hydrolysis in the prosaposin‐deficient cells. The reduced galactosylsphingosine turnover in prosaposin‐deficiency suggests that there could be a pathogenetic cerebral accumulation of galactosylsphingosine in this disorder.
Materialart:
Online-Ressource
ISSN:
0014-5793
,
1873-3468
DOI:
10.1016/S0014-5793(01)03044-7
Sprache:
Englisch
Verlag:
Wiley
Publikationsdatum:
2001
ZDB Id:
1460391-3
SSG:
12
