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Galactosylsphingosine hydrolysis was also deficient in cell homogenates from Krabbe's disease (\u03b2\u2010galactocerebrosidase\u2010deficient) patients, but not acid \u03b2\u2010galactosidase\u2010deficient patients. Moreover, hydrolysis of galactosylsphingosine in the prosaposin\u2010deficient 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\u2010deficient cells. The reduced galactosylsphingosine turnover in prosaposin\u2010deficiency suggests that there could be a pathogenetic cerebral accumulation of galactosylsphingosine in this disorder.<\/jats:p>","DOI":"10.1016\/s0014-5793(01)03044-7","type":"journal-article","created":{"date-parts":[[2002,7,25]],"date-time":"2002-07-25T09:53:08Z","timestamp":1027590788000},"page":"107-110","source":"Crossref","is-referenced-by-count":29,"title":["Saposins (<i>sap<\/i>) A and C activate the degradation of galactosylsphingosine"],"prefix":"10.1002","volume":"508","author":[{"given":"Klaus","family":"Harzer","sequence":"first","affiliation":[]},{"given":"Masao","family":"Hiraiwa","sequence":"additional","affiliation":[]},{"given":"Barbara C","family":"Paton","sequence":"additional","affiliation":[]}],"member":"311","published-online":{"date-parts":[[2001,11,27]]},"reference":[{"key":"e_1_2_6_2_1","unstructured":"Sandhoff K. Kolter T. and Harzer K. 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