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A set of small Trp-free, multifunctional glycoproteins (co-β-glucosidase, A1 activator, glucosylceramidase activator, SAP-A–D, 81 aa) derived from a single precursor, prosaposin, that act as sphingolipid activator proteins (SAPs) and assist in the lysosomal hydrolysis of sphingolipids.Other functions include neuritogenic/neuroprotection effects and induction of membrane fusion. Deficiency of saposin A causes atypical Krabbe's disease, deficiency of saposin B causes metachromatic leukodystrophy, deficiency of saposin C causes an atypical form of Gaucher's disease. Saposin-like proteins (SAPLIPs) include surfactant protein B (SP-B), Entamoeba histolytica pore-forming peptide, granulysin, NK-lysin, acid sphingomyelinase, and acyloxyacyl hydrolase.
From: saposins in A Dictionary of Biomedicine »
Subjects: Chemistry — Medicine and Health.
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