PCBD1
[Pterin-4-alpha-carbinolamine dehydratase/Dimerization cofactor of HNF1]. Also: 4-alpha-hydroxy-tetrahydropterin dehydratase. The protein is known also as PCD [Pterin-4-alpha-carbinolamine dehydratase, Pterin carbinolamine dehydratase] and PHS [Phenylalanine hydroxylase-stimulating protein].
The PCBD1 gene encodes a bifunctional protein that, in the cytoplasm, acts as an enzyme in the salvage pathway for the regeneration of tetrahydrobiopterin (BH4) (Hauer et al, 1993), the cofactor for aromatic amino acid hydroxylases. In the nucleus it acts as a binding partner of the HNF1 family of transcription factors
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