Sphingolipid Metabolism

Front 1

Sphingolipids backbone and head group

Back 1

instead of glycerol backbone it has spingosine backbone and instead of head group it has carbohydrate

Front 2

Sphingosine is formed from what amino acid

Back 2

serine

Front 3

What is the parent compound of sphingolipids

Back 3

ceramide

Front 4

What is the parent compound of Ecosinoids

Back 4

Arachadonic Acid

Front 5

Gaucher's disease

Lipid accumulation
Enzyme deficiency
Primary organ involvement

Back 5

Lipid accumulation: GLUCOCEREBROSIDE

Enzyme deficiency: GLUCOSYLCERAMIDE (B-D-GLUCOSIDASE)

Primary organ involvement: LIVER, SPLEEN, BRAIN

Front 6

Nieman-Pick

Lipid accumulation
Enzyme deficiency
Primary organ involvement

Back 6

Lipid accumulation:
SPHINGOMYLIN

Enzyme deficiency:
SPHINGOMYLINASE

Primary organ involvement:
BRAIN, LIVER, SPLEEN

Front 7

Fabry's

Lipid accumulation
Enzyme deficiency
Primary organ involvement

Back 7

Lipid accumulation:
CERAMIDE TRIHEXOSIDE

Enzyme deficiency:
ALPHA-BETA-GALACTOSIDASE

Primary organ involvement
KIDNEY

Front 8

Tay Sach's

Lipid accumulation
Enzyme deficiency
Primary organ involvement

Back 8

Lipid accumulation:
GANGLIOSIDE GM2

Enzyme deficiency:
B-D-HEXOSAMINIDASE A

Primary organ involvement: BRAIN

Front 9

Describe Tay Sach's

Back 9

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.

Front 10

Describe Gaucher's disease

Back 10

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.

Front 11

Describe Nieman Pick disease

Back 11

Disfunction in spingomyelinase enzyme results in a build up of sphingomyelin. Results in hepatomegly and neurodegeneration.
Cells appear "foamy" with sphingomylin.
Autosomal recessive

Front 12

Describe Fabry's disease

Back 12

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.