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Definition
| synthesized from 16C palmitic acid and the amino acid serine |
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Definition
| the backbone of sphingolipids |
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Term
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Definition
| base molecule of sphingolipids, the N-acyl derivative of sphingosine |
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Term
| glycoshingolipids production |
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Definition
| addition of sugars to ceramide yields |
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Term
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Definition
| result from defects in catabolism of sphingolipids |
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Term
| autosomal recessive disorder |
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Definition
| accumlation of sphingolipid in tissue |
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Term
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Definition
| myelin-specific lipid. defects in catabolism of this cause Krabbe Ds |
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Term
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Definition
| myelin-specific lipid, defects in the catabolism of this results in metachromatic leukodystrophy (MLD) and destroys myelin |
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Term
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Definition
| normally minor toxic metabolite that is importnat in Krabbe's Ds |
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Term
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Definition
contain glucosyl-ceramide and N-acetylneuraminic acid (NANA or sialic acid)
concentrated in neurons.
defective catabolism causes Tay-Sachs |
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Term
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Definition
| glycosphingolipids which are present on erthyrocytes give rise to... |
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Term
| O-linked glycoproteins (eg.) |
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Definition
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Term
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Definition
choline-P-ceramide
enriched in myelin
defects in catabolism cause Niemann-Pick A&B diseases |
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Term
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Definition
| intracellular trafficking of cholesterol deficiency. |
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Term
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Definition
caused by defects in degredation of GAGs such as heparin.
can lead to severe mental retardation/structural deformities. |
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