Cedrick
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Newborn with severe acidosis,vomiting,hypotonia and neurologic deficits
lab. analysis reveals elevated levels of lactae and alanine
these observations suggest a deficiency of which of the following enzymes ?
a.-alanine aminotransferase
b.-glutamate dehydrogenase
c.-lactate dehydrogenase
d.-phenylethanolamine N-methyltransferase
e.-pyruvate dehydrogenase
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| Cedrick
Forum Fanatic
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Tricky one
e.-pyruvate dehydrogenase
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| blueocean
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In the absence of PDH pyruvate will be converted to alanine via alanine aminotransferase and to lactate via lactate dehydrogenase
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| drpkaur
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E,,,
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| docsrinu
Forum Elite
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yup E..
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| young_doc
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E. for sure.
We should remember also that the PDH enzyme is a complex, and relies on many co-factors. So deficiency in any of the co-factors could also be possible, instead of a deficiency of the enzyme as a whole.
So to extend this thread, what are the FIVE co-factors of PDH...
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| eytanraz
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CoA, Lipoic Acid, FAD, NAD+, tHIAMINE (B1)
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| young_doc
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awesome!
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| drbin
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remember this is one of the causes for hypoglycemia in alcoholics,
def of B1 leads to decreased PDH activity and accumulation of pyruvate so the substrates for gluconeogenesis ie lactate and alanine is not utilised.
other substrate for gluconeogenesis ie DHAP also cant be used bs of high NADH/NAD ratio
other causes r,
decreased oxidation of glucose due to defective AKGA (def of B1),
poor nutrition,
high NADH/NAD ratio leading to decreased beta oxidation of fatty acids.
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