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Carbohydrates
biological carbo-protein properties
15
Medical
Graduate
08/22/2007

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Term
sphingosine sythesis
Definition
synthesized from 16C palmitic acid and the amino acid serine
Term
sphingosine
Definition
the backbone of sphingolipids
Term
ceramide
Definition
base molecule of sphingolipids, the N-acyl derivative of sphingosine
Term
glycoshingolipids production
Definition
addition of sugars to ceramide yields
Term
sphingolipidoses
Definition
result from defects in catabolism of sphingolipids
Term
autosomal recessive disorder
Definition
accumlation of sphingolipid in tissue
Term
cerebroside
Definition
myelin-specific lipid. defects in catabolism of this cause Krabbe Ds
Term
sulfatide
Definition
myelin-specific lipid, defects in the catabolism of this results in metachromatic leukodystrophy (MLD) and destroys myelin
Term
psychosine
Definition
normally minor toxic metabolite that is importnat in Krabbe's Ds
Term
gangliosides
Definition
contain glucosyl-ceramide and N-acetylneuraminic acid (NANA or sialic acid)

concentrated in neurons.

defective catabolism causes Tay-Sachs
Term
blood group antigens
Definition
glycosphingolipids which are present on erthyrocytes give rise to...
Term
O-linked glycoproteins (eg.)
Definition
forms of ABO antigens
Term
sphingomyelin
Definition
choline-P-ceramide

enriched in myelin

defects in catabolism cause Niemann-Pick A&B diseases
Term
Niemann Pick C Disease
Definition
intracellular trafficking of cholesterol deficiency.
Term
Hunter's

Hurler's Syndrom
Definition
caused by defects in degredation of GAGs such as heparin.

can lead to severe mental retardation/structural deformities.
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