| sphingol | <biochemistry> Long chain amino alcohol that bears an approximate similarity to glycerol with a hydrophobic chain attached to the 3 carbon. Forms the class of sphingolipids when it carries an acyl group joined by an amide link to the nitrogen. Forms sphingomyelin when phosphoryl choline is attached to the 1 hydroxyl group. Gives rise to the cerebroside and ganglioside classes of glycolipids when oligosaccharides are attached to the 1 hydroxyl group. Not found in the free form. (18 Nov 1997) |
|---|---|
| sphingolipid | <biochemistry> Structural lipid of which the parent structure is sphingosine rather than glycerol. Synthesised in the Golgi complex. (18 Nov 1997) |
| sphingolipid ceramide N-deacylase | <enzyme> Hydrolyzes the n-acyl linkage between fatty acids and sphingosine bases in ceramides in various sphingolipids, as well as sphingomyelin to produce their lyso forms; mw 52 kD Registry number: EC 3.5.1.- Synonym: scdase (26 Jun 1999) |
| sphingolipidoses | Lysosomal storage diseases characterised by failure to degrade sphingolipids and resulting in their abnormal storage. (12 Dec 1998) |
| sphingolipidosis | Collective designation for a variety of diseases characterised by abnormal sphingolipid metabolism, e.g., gangliosidosis, Gaucher's disease, Niemann-Pick disease. Synonym: sphingolipodystrophy. (05 Mar 2000) |
| sphingolipodystrophy | Collective designation for a variety of diseases characterised by abnormal sphingolipid metabolism, e.g., gangliosidosis, Gaucher's disease, Niemann-Pick disease. Synonym: sphingolipodystrophy. (05 Mar 2000) |
Synonyms : Cerebroside Sulfate Activator Protein, G(M1) Ganglioside Activating Factor, GM1 Activator Protein, Ganglioside Degradation Activator Protein, Ganglioside Hydrolysis Activator Proteins, Hexosaminidase A Activating Protein, Hexosaminidase Activating Factor
Synonyms : Sphingolipid Storage Diseases, Sphingolipidosis, Sphingolipid Storage Disease, Storage Disease, Sphingolipid, Storage Diseases, Sphingolipid
Synonyms :
| 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. ...
출처: www.mercksource.com/pp/us/cns/cns_hl_dorlands.jspz...
|
|---|---|
| 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...
|
제품명 |
판매사 |
보험코드 | 성분/함량 | 구분/보험급여 |
|---|
제품명 |
판매사 |
보험코드 | 성분/함량 | 구분/보험급여 |
|---|