Catabolic pathway of phe/tyr.

<p>The scheme illustrates the individual enzymatic steps and intermediates of the catabolism of phe/tyr to acetoacetate and fumarate in the cytoplasmic compartment and their further metabolization within mitochondria. PAH, phenylalanine hydroxylase; BH4, tetrahydrobiopterin; TAT, tyrosine aminotransferase; HPPD, 4-hydroxyphenylpyruvate dioxygenase; HGD, homogentisate dioxygenase; MAAI, maleylacetoacetate isomerase; FAH, fumarylacetoacetate hydrolase; DCA, dichloroacetate; TC, tricaboxylic acid cycle; OXPHOS, oxidative phosphorylation.