Overview

saposin


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Gaucher disease

Krabbe's disease

Tay-Sachs disease

 

'saposin' can also refer to...

saposin

saposins

Tissue-specific effects of saposin A and saposin B on glycosphingolipid degradation in mutant mice

Neurological deficits and glycosphingolipid accumulation in saposin B deficient mice

Saposin C mutations in Gaucher disease patients resulting in lysosomal lipid accumulation, saposin C deficiency, but normal prosaposin processing and sorting

Specific saposin C deficiency: CNS impairment and acid β-glucosidase effects in the mouse

Gaucher disease due to saposin C deficiency is an inherited lysosomal disease caused by rapidly degraded mutant proteins

Combined saposin C and D deficiencies in mice lead to a neuronopathic phenotype, glucosylceramide and α-hydroxy ceramide accumulation, and altered prosaposin trafficking

Saposin-like Proteins, a Multigene Family of Schistosoma Species, are Biomarkers for the Immunodiagnosis of Schistosomiasis Japonica

Neuronopathic Gaucher disease in the mouse: viable combined selective saposin C deficiency and mutant glucocerebrosidase (V394L) mice with glucosylsphingosine and glucosylceramide accumulation and progressive neurological deficits

Mutation in saposin D domain of sphingolipid activator protein gene causes urinary system defects and cerebellar Purkinje cell degeneration with accumulation of hydroxy fatty acid-containing ceramide in mouse

A novel mutation in the coding region of the prosaposin gene leads to a complete deficiency of prosaposin and saposins, and is associated with a complex sphingolipidosis dominated by lactosylceramide accumulation

A mutation in the saposin A domain of the sphingolipid activator protein (prosaposin) gene results in a late-onset, chronic form of globoid cell leukodystrophy in the mouse

 

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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.

Subjects: Chemistry — Medicine and Health.

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saposins
in A Dictionary of Biomedicine

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saposin
in Oxford Dictionary of Biochemistry and Molecular Biology

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