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saposin

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acid ceramidase

acid ceramidase  

EC 3.5.1.23; other name: N‐acylsphingosine deacylase; a lysosomal enzyme that catalyses the hydrolyis of ceramide to sphingosine and a fatty acid. Its activity requires sphingolipid activator ...
Gaucher activator

Gaucher activator  

ora glycosylated polypeptide derived by appropriate proteolytic cleavage of prosaposin. It is an activator of lysosomal glucosylceramidase. Deficiency of prosaposin or saposin C, due to mutation in ...
Gaucher disease

Gaucher disease  

A genetically determined (autosomal recessive) disease resulting from the deposition of glucocerebrosides (see cerebroside) in the brain and other tissues (especially bone). It results in mental ...
glucosylceramidase

glucosylceramidase  

EC 3.2.1.45; other names: β‐glucocerebrosidase; acid β‐glucosidase; systematic name: d‐glucosyl‐N‐acylsphingosine glucohydrolase; a lysosomal glycoprotein enzyme that catalyses the hydrolysis of ...
granulysin

granulysin  

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A saposin-like protein (129 aa) present in cytotoxic granules of cytolytic T cells and NK cells and released upon antigen stimulation.
Krabbe's disease

Krabbe's disease  

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A disorder involving the white matter of the central and peripheral nervous system caused by mutation in the galactosylceramidase (galactocerebrosidase) gene. An atypical form is caused by deficiency ...
leukodystrophy

leukodystrophy  

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A duplication of the laminB1 gene (LMNB1) is associated with adult-onset autosomal dominant leukodystrophy, a slowly progressive neurological disorder in which there is symmetrical and widespread ...
metachromatic leukodystrophy

metachromatic leukodystrophy  

Any of a group of hereditary diseases of humans characterized by disintegration of myelin and the accumulation of metachromatic lipid – galactosyl sulfatide (cerebroside sulfate) – in the white ...
prosaposin

prosaposin  

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The glycoprotein (sphingolipid activator protein, sulphated glycoprotein-1, SGP-1, 524 aa) produced in testis, spleen, and brain from which saposins are produced by proteolysis. There are two ...
saposin

saposin   Reference library

Oxford Dictionary of Biochemistry and Molecular Biology (2 ed.)

Reference type:
Subject Reference
Current Version:
2008
Subject:
Science and technology, Life Sciences
Length:
156 words

any of a group of peptide cofactors of enzymes for the lysosomal degradation of sphingolipids. They stimulate various enzymes, including

sortilin

sortilin  

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A lysosomal sorting receptor, important for the sorting and trafficking of sphingolipid activator proteins (saposins).
sphingolipid

sphingolipid  

A structural lipid in which an acyl chain is attached to sphingosine to form a ceramide. Further modification (glycosylation) gives rise to cerebrosides and gangliosides, major glycolipids in the ...
swaposin

swaposin  

A feature of some proteins in which domains, characteristically found in one protein, are found arranged in a different order in other proteins. The phenomenon was first commented on in ...
Tay-Sachs disease

Tay-Sachs disease  

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(amaurotic familial idiocy) an inherited disorder of lipid metabolism (see lipidosis) in which abnormal accumulation of lipid in the brain leads to blindness, mental retardation, and death in ...

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