| sphingolipid activator protein |
(SAP) any of a group of non-enzymatic lysosomal proteins that stimulate the actions of specific lysosomal hydrolases by binding and solubilizing their sphingolipid substrates. SAP-1 is a glycoprotein that binds sulfatide, ganglioside GM 1 , and ceramide trihexoside, activating cerebroside sulfatase, lysosomal b-galactosidase, and a-galactosidase activities, respectively. ...
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| sphingolipid |
a lipid in which the backbone is sphingosine or a related base; the basic unit is a ceramide (q.v.) which is attached via its 1-hydroxyl group to a polar head group. The sphingolipids include sphingomyelins, cerebrosides, and gangliosides.
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| sphingolipid activator p. |
any of a group of non-enzymatic lysosomal proteins that stimulate the actions of specific lysosomal hydrolases by binding and solubilizing their sphingolipid substrates. SAP-1 is a glycoprotein that binds sulfatide, ganglioside GM1, and ceramide trihexoside, activating cerebroside sulfatase, lysosomal β-galactosidase, and α-galactosidase activities, respectively. Deficiency of SAP-1, an autosomal recessive trait, results in a disorder considered to be a form of juvenile metachromatic leukodystrophy. SAP-2 is a glycoprotein necessary for the hydrolysis of ganglioside GM2 via activation of hexosaminidases A and B; deficiency of SAP-2 results in GM2 gangliosidosis, variant AB.
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| sphingolipidosis |
any of various lysosomal storage diseases characterized by abnormal storage of sphingolipids.
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| sphingolipodystrophy |
sphingolipidosis.
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