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Methionine
38%
166/436
Valine
6%
28/436
Threonine
25/436
Isoleucine
8%
37/436
Phenylalanine
39%
169/436
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The affected cells of this patient are displaying characteristics of proprionic acidemia. Phenylalanine metabolism is unaffected in this disorder. Propionic acidemia is an autosomal recessive disorder due to a deficiency in propionyl-CoA carboxylase in the propionic acid cycle. This deficiency results in the inability to metabolize several molecules (methionine, threonine, isoleucine, valine, odd-chain fatty acid acids, and proprionic acid) via the propionic acid cycle. Patients present with ketoacidosis and have elevated blood levels of propionic acid, methyl citrate, and hydroxypropionic acid. Incorrect Answers: Answer 1-4: Methionine, valine, isoleucine, and threonine cannot be broken down in propionic acidemia as they are metabolized by the priopionic acid cycle.
3.9
(14)
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