abbr.: PCD. EC 4.2.1.96; an enzyme activity of liver and other tissues that is part of a bifunctional protein (
abbr.: PCD/DCoH; 103 amino acids) that also acts as the dimerization cofactor for hepatocyte nuclear factor 1α (
abbr.: DCoH). The enzyme converts 4
α-carbinolamine tetrahydrobiopterin (formed during the conversion of phenylalanine to tyrosine) to quinonoid dihydrobiopterin by elimination of water. It also prevents nonenzymatic conversion of tetrahydrobiopterin to
primapterin. Several mutations in the protein lead to deficiency of PCD activity, with consequent hyperphenylalaninemia and primapterinuria. See
biopterin.