Exam 3 Biochem-Lecture 30 – Flashcards
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| Which are a component of all membranes but are particularly abundant in the myelin sheath??? |
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| Sphingolipids |
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Sphingomyelins are......that are also ....... |
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•What are important structural lipid components of nerve cell membranes??? |
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| Sphingomyelins |
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•The predominant sphingomyelins contain |
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| palmitic or stearic acid N-acylated at carbon 2 of sphingosine. |
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| The sphingolipids, like the phospholipids, are composed of |
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| The core of sphingolipids is |
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| the long-chain amino alcohol, sphingosine |
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| Amino acylation, with a long chain fatty acid, at carbon 2 of sphingosine yields ..... |
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| a ceramide. |
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•Sphingolipids include |
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| Sphingomyelins are ......that are also ...... |
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sphingolipids phospholipids |
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•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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| sphingomyelin synthase |
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Sphingomyelin is degraded by.......... a lysosomal enzyme that hydrolytically removes phosphorylcholine, leaving a ceramide. |
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| sphingomyelinase |
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•Defects in the enzyme acid sphingomyelinase result in the lysosomal storage disease known as : |
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| Niemann-Pick disease. |
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There are at least how many related disorder identified as Niemann-Pick disease??? |
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| 4 related disorders |
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•4 related disorders identified as Niemann-Pick disease are |
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Type A Type B Type C Type D |
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Type A and B of Niemann-Pick disease result from |
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| defects in acid sphingomyelinase |
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Type C1, C2 and D of Niemann-Pick disease |
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| do not result from defects in acid sphingomyelinase |
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| Glycosphingolipids, or glycolipids are composed of |
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| a ceramide backbone with a wide variety of carbohydrate groups (mono- or oligosaccharides) attached to carbon 1 of sphingosine. |
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| The four principal classes of glycosphingolipids are |
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•Cerebrosides have |
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| a single sugar group linked to ceramide |
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| The most common of Cerebrosides is |
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•Galactocerebrosides are found |
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| are found predominantly in neuronal cell membranes |
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| Gluco-cerebrosides are |
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| Galactocerebrosides are synthesized from |
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| ceramide and UDP-galactose |
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| Glucocerebrosides are synthesized from |
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| ceramine and UDP-glucose |
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Fabry disease is characterized by
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Gaucher’s disease is characterized by |
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•NANA is |
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| N-acetylneuraminic acid or sialic acid. |
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•The linkage of NANA always involves ........on the number ......... carbon of the carbohydrate. |
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the OH group 2 |
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| they also contain NANA in varying amounts. |
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•CNS is unique in that |
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| more than 50% of the sialic acid is in gangliosides. |
