BCHM 10000 Lecture Notes - Lecture 7: Phenylpyruvic Acid, Phenylalanine, Transamination
3 Jul 2018
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●In the absence of phe hydroxylation to tyr in PKU patients, phe is transaminated to
phenylpyruvate, which inhibits the mitochondrial pyruvate transporter
●Phenylpyruvate inhibits the mitochondrial pyruvate transporter because of its structural
similarity
●Pyruvate transport into the mitochondria is blocked and ATP generation is severely
reduced
●Metabolism involves both anabolic and catabolic steps
●Catabolism generally liberates energy that can be captured and used by the organism
either to drive anabolic processes or to do work
