Synonyms :
Synonyms : Sphingomyelinase C
Synonyms :
Synonyms : 4-Sphingenine, 4 Sphingenine
Synonyms : Ceramide Synthetase, Fatty Acyl-CoA - Sphingosine Acyltransferase, Sphingosine Acyltransferase, Acyltransferase, Sphingosine, Fatty Acyl CoA Sphingosine Acyltransferase, N-Acyltransferase, Sphingosine, Sphingosine N Acyltransferase, Synthetase, Ceramide
| 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.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
|
|---|---|
| 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.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
|
| sphingolipidosis |
any of various lysosomal storage diseases characterized by abnormal storage of sphingolipids.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
|
| sphingolipodystrophy |
sphingolipidosis.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
|
| sphingomyelin l. |
Niemann-Pick disease.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
|