Pts - 6-pyruvoyl-tetrahydropterin synthase

Enables 6-pyruvoyltetrahydropterin synthase activity and identical protein binding activity. Acts upstream of or within tetrahydrobiopterin biosynthetic process. Located in mitochondrion. Is expressed in several structures, including alimentary system; central nervous system; genitourinary system; respiratory system; and sensory organ. Used to study BH4-deficient hyperphenylalaninemia A. Human ortholog(s) of this gene implicated in BH4-deficient hyperphenylalaninemia A and phenylketonuria. Orthologous to human PTS (6-pyruvoyltetrahydropterin synthase). [provided by Alliance of Genome Resources, Apr 2022]