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27 Cards in this Set
- Front
- Back
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inner membrane of mitochondria location of what process?
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oxidative phosphorylation, all other functions in matrix
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which enzyme in kreb cycle is stimulated by insulin?
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citrate synthase
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five drugs that inhibit ATP synthesis and location of their MOA?
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amobarbital-complex I
antimycin A- complex III CO,CN- complex IV dinitrophenol- inner mito mem oligomycin- ATP synthase |
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defect in NADH dehydrogenase (complex I), causing central vision loss and eventual blindness
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leber' hereditary optic neuropathy
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brown fat MOA?
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uncoupling of the proton gradient in ATP synthesis causing an increase in body temp.
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which enzyme has bifuncitonal activity depending on the insulin:glucagon ratio?
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PFK-2
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role of PFK-2?
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fructose to fructose 2,6-BP a activator of PFK-1
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fructose intolerance caused by def. in which enzyme?
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aldolase B
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essential fructosuria caused by def. in which enzyme?
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fructokinase
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rate limiting step of glycogen synthesis?
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glycogen synthase
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rate limiting enzyme of glycogenolysis?
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glycogen phosphorylase
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MR, cardiomegaly and death by 2y/o indicate which glycogen storage disease? adult form has skeletal myopathy
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pompe's (alpha 1,4 glucosidase)
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muscle cramping and fatigue with no increaes in lactic acid indicate which disease?
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mcardle
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lactose metabolism to galactose and glucose is done where?
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intestinal epithelium
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rate limiting enzyme of galactose metabolism?
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GALT
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rate limiting enzyme of fructose metabolism?
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aldolase B
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rate limiting enzyme of pentose phosphate pathway?
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G6PD
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purpose of pentose-phosphate pathway?
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provide NADPH and ribose 5-phosphate
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severe MR, coarse facial features, hepatosplenomegaly, corneal clouding, CAD, and vacuoles in lysosomes indicate?
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hurler's (alpha-L-iduronidase def.), auto recessive
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FA synthesis rate limiting enzyme?
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acetyl CoA carboxylase
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what must acetyle CoA be converted to prior to leaving mitochondria?
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citrate
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what substrate inhibits carnitine acyltransferase I in order to avoid futile FA production?
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malonyl CoA
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end product of FA synthesis that inhibits acetyl CoA carboxylase?
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palmitate
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lipase inhibited by which hormone? activated by?
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insulin; GH and epi
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three organs that use ketone bodies?
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muscle, brain, kidney
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production of ketone bodies?
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mitochondria of liver
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cholestyramine MOA?
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increasing cholesterol excretion through bile acids, upregulating LDL receptors to increased synthesis
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