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34 terms

Lipids II

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Carnauba Wax
Hardest and most useful wax. Found in brasilian tree
Lanolin Wax
From sheep wool.
Survanta and curosurf (phospolipids) is used to treat ?
respiratory distress syndrom (RDS)
phosphatidylcholine
phospo lipid only on cytosol side. goes to outside during apoptosis.
filipase
enzyme envolved with phosphatidylcholine
phosphatidyl inositol
used in cell signaling and metabolism. PIP in periphery of cell.
Ceramide
sphingosine + fatty acid
found in animal membranes
holds skin together
functions in apoptosis
sphingomyelin
has ceramide. lower concentrations than phospatidylcholine. Most abundant ceramide.
Ether glycerophospholipids
ether linkage at C1 of glycerol. PFA and plasmalogen. found in white matter.
ethanolamine plasmalogen
found in nervous system. not in plants, fungi or bacteria.
Glycolipids = ?
cerebrosides, globoside, ganglioside
cerebroside = ?
- 1 sugar + ceramide
Galactocerebroside
- type of cerebrosdie
only found in neural tissue
Glucocerebroside
type of cerebroside
found in other tissues besides neural
globoside
has 2 sugars instead of 1
ganglioside
oligosaccharide attached instead of monosaccharide. used in cell to cell signaling.
cardiolipids
only found in mitochondria and bacteria
test for syphilis
sulfolipids
brain lipids. sulfate esters of cerebrosides.
sphingolipidoses
Lipid storage disease. passed on through x chromosomes. Most die 3-5 yo.
Tay-Sachs Disease
GM2 accumulation in brain.
Hexodsminidase deficiency
Fabry's disease
ceramide trihexoside in kidney
Alpha galactosidase deficiency
Krabbe's disease
galactocerebroside in white matter
globoid leukodystrophy
galactocerebrosidase deficiency
Gaucher's disease
glucocerebroside in the spleen and liver
lysosomal glucocerebrosidase deficiency
most common
erosion of bones
Imiglucerase (Cerezyme)
analogue of human β-glucocerebrosidase used to treat Gaucher's disease
Miglustat (Zavesca)
a glucosylceramide synthase inhibitor. prevents build up of fatty acids in Gaucher's disease.
Metachromatic leukodystrophy
accumulation of sulfogalactocerebroside in CNS
arylsulfatase A deficiency
metachromasia
nerves stain yellowish-brown with cresyl violet dye in metachromatic leukodystrophy
Generalized gangliosidosis
accumulation of ganglioside GM1
deficiency of GM1 ganglioside: b-galactosidase
Niemann-Pick disease
sphingomyelin accumulation in reticuloendothelial.
sphingomyelinase deficiency
Niemann-Pick Type A and B (I)
Missing acid sphingomyelinase (ASM)
Niemann-Pick Type C
body cannot properly break down cholesterol and other fats
Niemann-Pick Type D
problems with the movement of cholesterol between brain cells.
A antigens
B antigens
H antigens
Type A blood
Type B blood
Type O blood
Ganglioside Nomenclature
1
2
3
1. Gal-GalNAc...
2. GalNAc...
3. Gal-Glc...