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25 Cards in this Set

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