| 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) |
| sphingomyelin | <biochemistry> A sphingolipid in which the head group is phosphoryl choline. A close analogue of phosphatidylcholine. In many cells the concentration of sphingomyelin and phosphatidylcholine in the plasma membrane seems to bear a reciprocal relationship. (18 Nov 1997) |
| sphingomyelin deacylase | <enzyme> Forms sphingosylphosphocholine Registry number: EC 3.5.1.- Synonym: sm-deacylase (26 Jun 1999) |
| sphingomyelin lipidosis | <disease> A family of severe lysosomal storage diseases resulting in an accumulation of sphingomyelin and other phospholipids in the reticuloendothelial system. The best studied forms are due to deficiency of sphingomyelinase and it is more common in Ashkenazi Jews than other groups. Clinical signs include foam cells in the blood and marrow, hepatosplenomegaly and neurologic degeneration. Diagnosis is confirmed by enzyme assay on leukocytes or fibroblasts and specific mutations in the gene are now recognised. (29 Dec 1997) |
| sphingomyelin phosphodiesterase | <enzyme> An enzyme that catalyses the hydrolysis of sphingomyelin to ceramide (n-acylsphingosine) plus choline phosphate. A defect in this enzyme leads to niemann-pick disease. Chemical name: Sphingomyelin cholinephosphohydrolase Registry number: EC 3.1.4.12 (12 Dec 1998) |
| sphingomyelinase | <enzyme> An enzyme that catalyses the hydrolysis of sphingomyelin to ceramide (n-acylsphingosine) plus choline phosphate. A defect in this enzyme leads to niemann-pick disease. Chemical name: Sphingomyelin cholinephosphohydrolase Registry number: EC 3.1.4.12 (12 Dec 1998) |
| sphingomyelins | A group of phospholipids, found in brain, spinal cord, kidney, and egg yolk, containing 1-phosphocholine (choline O-phosphate) combined with a ceramide (a long-chain fatty acid linked to the nitrogen of a long-chain base, such as sphingosine). Synonym: ceramide 1-phosphorylcholine, phosphosphingosides. (05 Mar 2000) |
| sphingosine | <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) |
| sphingosine CoA-independent transacetylase | <enzyme> Transfers the acetate group from platelet-activating factor (paf) to sphingosine forming n-acetylsphingosine (c2-ceramide) Registry number: EC 2.3.1.- Synonym: platelet-activating factor-sphingosine transacetylase, paf-sphingosine transacetylase, paf-sph transacetylase (26 Jun 1999) |
| sphingosine N-methyltransferase | <enzyme> Converts sphingosine to n,n-dimethylsphingosine Registry number: EC 2.1.1.- (26 Jun 1999) |