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12 Cards in this Set

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Sphingolipids backbone and head group
instead of glycerol backbone it has spingosine backbone and instead of head group it has carbohydrate
Sphingosine is formed from what amino acid
serine
What is the parent compound of sphingolipids
ceramide
What is the parent compound of Ecosinoids
Arachadonic Acid
Gaucher's disease

Lipid accumulation
Enzyme deficiency
Primary organ involvement
Lipid accumulation: GLUCOCEREBROSIDE

Enzyme deficiency: GLUCOSYLCERAMIDE (B-D-GLUCOSIDASE)

Primary organ involvement: LIVER, SPLEEN, BRAIN
Nieman-Pick

Lipid accumulation
Enzyme deficiency
Primary organ involvement
Lipid accumulation:
SPHINGOMYLIN

Enzyme deficiency:
SPHINGOMYLINASE

Primary organ involvement:
BRAIN, LIVER, SPLEEN
Fabry's

Lipid accumulation
Enzyme deficiency
Primary organ involvement
Lipid accumulation:
CERAMIDE TRIHEXOSIDE

Enzyme deficiency:
ALPHA-BETA-GALACTOSIDASE

Primary organ involvement
KIDNEY
Tay Sach's

Lipid accumulation
Enzyme deficiency
Primary organ involvement
Lipid accumulation:
GANGLIOSIDE GM2

Enzyme deficiency:
B-D-HEXOSAMINIDASE A

Primary organ involvement: BRAIN
Describe Tay Sach's
Disfunction in enzyme B-D hexosaminidase A. Results in build up of gangliosides (GM2) and rapid and progressive neurodegeration. Results in blindness, cherry red macula, muscular weakness, seizures
Prevelant in eastern european jews.
Autosomal recessive.
Describe Gaucher's disease
Results from a build up of glucocerebrosides because of a faulty glucosylceramide enzyme. It is the most common lysosomal storage disease. Resutls in hepatomegly. Osteoperosis of long bones. Rare CNS involvement in juvenile and infantile forms.
Autosomal recessive.
Crumpled tissue paper appearance to cytoplasm in cells.
Describe Nieman Pick disease
Disfunction in spingomyelinase enzyme results in a build up of sphingomyelin. Results in hepatomegly and neurodegeneration.
Cells appear "foamy" with sphingomylin.
Autosomal recessive
Describe Fabry's disease
Disfunction in alpha galactosidase enzyme results in build-up of ceramide trihexoside. Results in a reddish-purple skin rash. Kidney and heart failure and burning pain in the lower extremities. X-linked recessive.