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  • sphingolipidosis
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  • sphingomyelin
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  • sphingomyelinase
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  • sphingomyelinosis
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  • sphingosine
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  • sphingolipidosis
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  • sphingolipidosis
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  • sphingomyelin
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  • sphingomyelinase
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  • sphingomyelinosis
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  • sphingosine
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  • sphingolipid activator protein I deficiency
  • sphingolipidosis
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  • sphingomyelin
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  • sphingomyelin
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  • sphingomyelinase
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  • sphingomyelinase deficiency
  • sphingomyelinosis
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  • sphingosine
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  • sphingoid
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  • sphingolipid
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  • sphingolipidosis
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  • sphingolipodystrophy
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  • sphingomyelin
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  • sphingophospholipid
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  • sphingosine
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  • E75.3
    Sphingolipidosis, unspecified
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CancerWEB ¿µ¿µ ÀÇÇлçÀü ¸ÂÃã °Ë»ö °á°ú : 15 ÆäÀÌÁö: 1
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)
MeSH(Medical Subject Headings) ¸ÂÃã °Ë»ö (http://www.nlm.nih.gov) °á°ú : 5 ÆäÀÌÁö: 1
  • Sphingobacterium - »õâ A genus of gram-negative, chemoorganotrophic bacteria in the family Sphingobacteriaceae. They lack FLAGELLA but some species exhibit gliding motility.
    Synonyms :
  • Sphingolipid Activator Proteins - »õâ A family of glycoprotein cofactors that are required for the efficient catabolization of SPHINGOLIPIDS by specific acid hydrolases such as GLUCOSYLCERAMIDASE; GALACTOCEREBROSIDASE; BETA-N-ACETYLHEXOSAMINIDASE; and CEREBROSIDE-SULFATASE.
    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
  • Sphingolipidoses - »õâ A group of inherited metabolic disorders characterized by the intralysosomal accumulation of SPHINGOLIPIDS primarily in the CENTRAL NERVOUS SYSTEM and to a variable degree in the visceral organs. They are classified by the enzyme defect in the degradation pathway and the substrate accumulation (or storage). Clinical features vary in subtypes but neurodegeneration is a common sign.
    Synonyms : Sphingolipid Storage Diseases, Sphingolipidosis, Sphingolipid Storage Disease, Storage Disease, Sphingolipid, Storage Diseases, Sphingolipid
  • Sphingolipids - »õâ A class of membrane lipids that have a polar head and two nonpolar tails. They are composed of one molecule of the long-chain amino alcohol sphingosine (4-sphingenine) or one of its derivatives, one molecule of a long-chain acid, a polar head alcohol and sometimes phosphoric acid in diester linkage at the polar head group. (Lehninger et al, Principles of Biochemistry, 2nd ed)
    Synonyms :
  • Sphingomonadaceae - »õâ A family of gram-negative, asporogenous rods or ovoid cells, aerobic or facultative anaerobic chemoorganotrophs. They are commonly isolated from SOIL, activated sludge, or marine environments.
    Synonyms :
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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. ...
Ãâó: www.mercksource.com/pp/us/cns/cns_hl_dorlands.jspz...
sphingomyelin A type of lipid.
Ãâó: www.lsdn.com/glance_glossary.shtml
sphingo- a combining form denoting relationship to sphingosine or a sphingolipid.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
sphingogalactoside a substance composing part of the material characteristic of the spleen in Gaucher's disease.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
sphingoin a leukomaine from the substance of the brain.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
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