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25 Cards in this Set
- Front
- Back
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sphingolipids
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sphingophospholipids and glycolipids
has a sphingosine backbone with a head groupd attached by a phsophate bond or a carbohydrate, respectively |
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sphingomyelin
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sphingosine backbone with a head groupd attached by a phosphate bond
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Glycolipids
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sphingosine backbone with a carbohydrate
cerebrosides, sulfatides, globosides, gangliosides |
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ceramide
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the parent compound of sphingolipids
sphingosine with a fatty acid the the C2 position |
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sphingosine
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made from serine
basic backbone for sphingolipids |
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sphingolipid synthesis
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synthesized by adding UDP activated sugar moieties or choline from phosphotidylcholine
forms sphingomyelin (phosphtidylcholine) sulfatide, globoside, ganglioside enzyme is glycosyltransferase |
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cerebrosides
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have one neutral sugar
galactosylceramide, glucosylceramide |
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galactosylceramide
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cerebroside
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glucosylceramide
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cerebroside
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gangliosides and globosides
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have two or more sugar
lactosylceramide, trihexosylceramide, globoside, hematoside(GM3), tays sachs ganglioside(GM2), GM1 |
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function of sphingolipids
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found mainly in myelin sheaths
highly concentrated in PNS and CNS ABO blood groups are characterized by different sphingolipids |
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Sphingolipidoses/degradation
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they are degradated in lysosomes
multistep enzyme hydrolysis genetic enzyme deficiency accumulation of particular sphingolipid inclusion bodies leads to splenohepatomegalay |
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I- cell diseases
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inclusion bodies formed by decreased degradation of sphingolipids
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sphingolipidoses conditions
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Gaucher's, Niemann-Pick, Krabbe's, Matachromatic leukodystrophy, Fabry's, Tay-Sachs
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Gaucher's disease
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glucocerebroside accumulation
glucosylceramide-beta-D-glucosidase dificiency involved with liver, spleen, and brain most common lysosomal storage disease hepatosplenomegalay osteoporosis of long bones Cns involvement in rare and juvenile forms has a "crumpled tissue paper apearance in cytoplasms autosomal recessive |
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Neimann-Pick disease
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accumulation of sphingomyelin
sphingomyelinase dificiency affects brain, liver, spleen has foamy appearing cell hepatosplenomegalay neurodegenartive course(type A) autosomal recessive |
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Fabry's disease
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accumulation of ceramide trihexose
alpha-D-galactosidase dificiency reddish purple skin rash kidney and heart failure burning pain in lower extremities only x-linked recessive affects kidney |
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Tay-Sachs disease
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accumulation of ganglioside GM2
Beta-D-Hexosaminidase A dificiency rapid and prgressive neurodegeneration, blindness, cherry red macula, macula weakness, seizures Affects brain |
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Krabbe's
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accumulation of galactocerebroside
galactosylceramide beta-D-galactosidase dificiency affects brain |
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metachromatic dystrophy
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accumulation of beta-sulfogalactocerebroside
sulfatide sulfatase dificiency affects brain |
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Neimann-Pick disease
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accumulation of sphingomyelin
sphingomyelinase dificiency affects brain, liver, spleen |
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Fabry's disease
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accumulation of ceramide trihexose
alpha-D-galactosidase dificiency affects kidney |
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Tay-Sachs disease
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accumulation of ganglioside GM2
Beta-D-Hexosaminidase A dificiency Affects brain |
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Krabbe's
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accumulation of galactocerebroside
galactosylceramide beta-D-galactosidase dificiency affects brain |
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metachromatic dystrophy
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accumulation of beta-sulfogalactocerebroside
sulfatide sulfatase dificiency affects brain |
