Term
| Which are a component of all membranes but are particularly abundant in the myelin sheath??? |
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Definition
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Term
Sphingomyelins are......that are also ....... |
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Definition
- sphingolipids
- phospholipids.
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Term
•What are important structural lipid components of nerve cell membranes??? |
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Definition
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Term
•The predominant sphingomyelins contain |
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Definition
| palmitic or stearic acid N-acylated at carbon 2 of sphingosine. |
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Term
| The sphingolipids, like the phospholipids, are composed of |
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Definition
- a polar head group
- two nonpolar tails.
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Term
| The core of sphingolipids is |
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Definition
| the long-chain amino alcohol, sphingosine |
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Term
| Amino acylation, with a long chain fatty acid, at carbon 2 of sphingosine yields ..... |
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Definition
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Term
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Definition
- the sphingomyelins
- glycosphingolipids (the cerebrosides, sulfatides, globosides and gangliosides).
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Term
| Sphingomyelins are ......that are also ...... |
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Definition
sphingolipids
phospholipids |
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Term
•The sphingomyelins are synthesized by the transfer of phosphorylcholine from phosphatidylcholine to a ceramide in a reaction catalyzed by what enzyme? |
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Definition
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Term
Sphingomyelin is degraded by.......... a lysosomal enzyme that hydrolytically removes phosphorylcholine, leaving a ceramide. |
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Definition
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Term
•Defects in the enzyme acid sphingomyelinase result in the lysosomal storage disease known as : |
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Definition
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Term
There are at least how many related disorder identified as Niemann-Pick disease??? |
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Definition
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Term
•4 related disorders identified as Niemann-Pick
disease are |
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Definition
Type A
Type B
Type C
Type D |
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Term
Type A and B of Niemann-Pick disease
result from |
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Definition
| defects in acid sphingomyelinase |
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Term
Type C1, C2 and D of Niemann-Pick disease
|
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Definition
| do not result from defects in acid sphingomyelinase |
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Term
| Glycosphingolipids, or glycolipids are composed of |
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Definition
| a ceramide backbone with a wide variety of carbohydrate groups (mono- or oligosaccharides) attached to carbon 1 of sphingosine. |
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Term
| The four principal classes of glycosphingolipids are |
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Definition
- cerebrosides
- sulfatides
- globosides
- gangliosides
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Term
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Definition
| a single sugar group linked to ceramide |
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Term
| The most common of Cerebrosides is |
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Definition
- galactose (galactocerebrosides)
- with a minor level of glucose(glucocerebrosides).
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Term
•Galactocerebrosides are found |
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Definition
| are found predominantly in neuronal cell membranes |
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Term
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Definition
-
not normally found in membranes, especially neuronal membranes
-
They represent intermediates in the synthesis or degradation of more complex glycosphingolipids.
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Term
| Galactocerebrosides are synthesized from |
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Definition
| ceramide and UDP-galactose |
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Term
| Glucocerebrosides are synthesized from |
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Definition
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Term
Fabry disease is characterized by
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Definition
-
accumulation of globosides
-
a reddish-purple skin rash
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kidney and heart failure
-
burning pain in the lower extremities.
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Term
Gaucher’s disease is characterized
by |
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Definition
- accumulation of glucocerebroside
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liver and spleen enlargement
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erosion of long bones and pelvis
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mental retardation in infantile form only.
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Term
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Definition
| N-acetylneuraminic acid or sialic acid. |
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Term
•The linkage of NANA always involves ........on the number ......... carbon of the carbohydrate. |
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Definition
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Term
•Gangliosides are very similar to globosides except that
|
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Definition
| they also contain NANA in varying amounts. |
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Term
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Definition
| more than 50% of the sialic acid is in gangliosides. |
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