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