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Saposins ( sap ) A and C activate the degradation of galactosylsphingosine
Author(s) -
Harzer Klaus,
Hiraiwa Masao,
Paton Barbara C
Publication year - 2001
Publication title -
febs letters
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.593
H-Index - 257
eISSN - 1873-3468
pISSN - 0014-5793
DOI - 10.1016/s0014-5793(01)03044-7
Subject(s) - hydrolysis , sphingosine , ceramide , chemistry , biochemistry , sphingolipid , apoptosis , receptor
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.