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What are the four major phospholipids
- Phosphotidylcholine
- Phosphatidylserine
- Phosphatidylethanolamine
- Phosphatidylinositol
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All phospholipids contain
Fatty acids
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Cardiolipin is important for what membrane
Mitochondrial
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What is the only glycerophospholipid that is antigenic
Cardiolipin
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Dipalmitoylphosphatidylcholine is a major component of
Lung surfactant
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What is found in snake and bee venom that makes them especially toxic
Phospholipases
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Phospholipase A2 is important for the synthesis of
Arachidonic acid
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Phospholipase A2 is found to be in high amounts of what secretions
Pancreatic
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What inhibits Phospholipase
Glucocorticoids
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Platelet activating factor (PAF) is released from phagocytic cells and has what effect
- Causes platelets to aggregate
- Causes anaphylactic responses
- Causes macrophages to produce super oxide radicals
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Phosphatidylethanolamine is found to be abundant in what tissue
Nerve tissue
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Phosphatidylcholine is found to be abundant in what tissue
Heart muscle
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Plasmalogen deficiency plays an important role in
Alzheimers disease
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What phospholipid is abundant in myelin sheaths
Sphingomyelin
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What determines blood groups
- Gangliosides
- "The Gangli guys are always the ones making decisions"
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What is the enzyme deficient in Tay-Sachs disease
Hexoamanidase A
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What is a classic clinical finding in Tay-Sachs disease
Cherry red macula
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What accumulates in Tay-Sachs
Gangliosides
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What are the symptoms for Gaucher disease
- Hepatosplenomegaly
- Mental retardation
- Osteoporosis
- "Auch!!, mental retardation, osteoporosis, and a big spleen!"
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What is deficient and accumulating in Gaucher disease
- Gluco-ceribrosidase (deficient)
- Glucocerebroside (accumulating)
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What are symptoms of Fabry disease
- Kidney/Heart failure
- Reddish purple skin rashes
- "It ain't no fable, her skin is as red as a stable"
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What is deficient and accumulating in Fabry disease
- Galactosidase (deficient)
- Globosides (accumulating)
- affecting patients "sides" with skin rashes
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Neimann Pick disease has what symptoms
- Mental retardation
- Hepatosplenomegaly
- "Don't pick retardation or hepatosplenomegaly"
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What is deficient and accumulating in Neimann Picks disease
- Sphingomyelinase (deficient)
- Sphingomyelin (accumulating)
- "Neimann marcus makes you sphing"
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What are the symptoms of Krabbes disease
- Blindness
- Deafness
- Mental deterioration
- "Krabbs are blind, deaf and deteriorated"
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What is deficient and accumulating in Krabbes disease
- B-Galactosidase (deficient)
- Galactocerebroside (accumulating)
- "Krabbs want to go to the galactic empire"
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Sandhoff disease has what symptoms
- Mental retardation
- Blindness
- Muscle weakness
- Seizures
- Same as Tay Sachs but with visceral involvement
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What is deficient and accumulating in Sandhoffs disease
- Hexoaminidase (deficient)
- Globosides (accumulate)
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What is the rate limiting step for cholesterol synthesis
- HMG-CoA reductase
- "OMG, did you just stop cholesterol synthesis!?"
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What is the rate limiting step of Bile acid synthesis
7 alpha-hydroxylase
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7 alpha-hydroxylase requires what for activation
Thyroid hormones and Vit C
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What are the secondary bile acids
- Deoxycholic acid
- Lithocholic acid
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What is the only possible mechanism for excretion of excess cholesterol in the feces
Bile acids
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What are some symptoms of an obstructed bile duct
- Pale stool
- Itching
- Jaundice
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