Cytosolic pterin-4-alpha-carbinolamine dehydratase (PCDB1) catalyzes the reaction of 4a-hydroxytetrahydrobiopterin to form q-dihydrobiopterin and water (Hauer et al. 1993. The active enzyme is a homotetramer (Ficner et al. 1995); mutations in the PCDB1 gene are associated with mild hyperphenylalanemia in vivo (Citron et al. 1993).
Hauer, CR, Rebrin, I, Blau, N, Ghisla, S, Hunziker, P, Heizmann, CW, Thony, B, Neuheiser, F, Curtius, HC
Greene, CL, Davis, MD, Citron, BA, Naylor, EW, Milstien, S, Kaufman, S
Suck, D, Sauer, UH, Stier, G, Ficner, R
4-alpha-hydroxytetrahydrobiopterin dehydratase activity of PCBD1 tetramer [cytosol]
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