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13 Cards in this Set

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  • Back
What is cardiolipin? Why is it important?
Two phosphatidic acid groups esterified to glycerol. Cardiolipin is important for inner mitochondrial membrane and bacterial membranes.
What is the major component of lung surfactant?
dipalmitoyl-PC
How do you get RDS in adults?
immunosuppresivte meds or chemotherapeutic drugs which kill pneumocytes
What can you apply to prevent RDS in premature newborns? What ratio is calculated to predict RDS?
Administer corticosteroids to inhibit phospholipase A2 (enzyme which decreases surfactant production). This stimulates lung surfactant production. PC to SM ratio is calculated.
What are gangliosides made of?
acid sphingoglycolipids that contain NANA (negatively charged molecule)
Gm1 means the ganglioside has how many sugars?
5-1=4. 4 sugars.
Why is cholera toxin toxic?
Binds to Gm1 ganglioside on intestinal mucosal cells
Defect in Tay Sach's?
HexA deficient, mental retardation, liver enlargement, death by age 2.
Defect in Fabry's Disease?
a-galactosidase A. mental retardation, blindness, death by age 3.
Defect in Sandhoff's disease?
HexA and B deficient. A vs. B startoff. sandoff. Similar to Tay Sach's but more rapid progression.
Defect in Gaucher's?
glucocerebrosidase deficiency; liver and sleepn enlargement, mental retardation
Defect in Niemann Pick?
sphingomyelinase. hepatosplenomegaly, mental retardation
Krabbe's disease
galactocerebrosidase. loss of myelin, mental retardation, death by age 2.