PCBD1 Recombinant Rabbit Monoclonal Antibody [JE62-75] (HA722464)

UNIPROT #

P61457

PROTEIN NAME

PCBD1

SYNONYMS

4 alpha hydroxy tetrahydropterin dehydratase antibody;4-alpha-hydroxy-tetrahydropterin dehydratase antibody;6 pyruvoyl tetrahydropterin synthase/dimerization cofactor of hepatocyte nuclear factor 1 alpha (TCF1) antibody;6 pyruvoyl tetrahydropterin synthase/dimerization cofactor of hepatocyte nuclear factor 1 alpha antibody;DCoH antibody;Dimerization cofactor of hepatic nuclear factor 1 alpha antibody;Dimerization cofactor of hepatocyte nuclear factor 1 alpha antibody;Dimerization cofactor of hepatocyte nuclear factor 1-alpha antibody;Dimerization cofactor of HNF1 antibody;Dimerizing cofactor for HNF1 antibody;PCBD 1 antibody;PCBD antibody;PCBD1 antibody;PCD antibody;Phenylalanine hydroxylase stimulating protein antibody;Phenylalanine hydroxylase-stimulating protein antibody;PHS antibody;PHS_HUMAN antibody;Pterin 4 alpha carbinolamine dehydratase antibody;Pterin 4 alpha carbinolamine dehydratase/dimerization cofactor of hepatocyte nuclear factor 1 alpha (TCF1) antibody;Pterin 4 alpha carbinolamine dehydratase/dimerization cofactor of hepatocyte nuclear factor 1 alpha antibody;Pterin carbinolamine dehydratase antibody;Pterin-4-alpha-carbinolamine dehydratase antibody

SUBCELLULAR LOCATION

Cytoplasm. Nucleus.

FUNCTION

This gene encodes a member of the pterin-4-alpha-carbinolamine dehydratase family. The encoded protein has been identified as a moonlighting protein based on its ability to perform mechanistically distinct functions. The encoded protein functions as both a dehydratase involved in tetrahydrobiopterin biosynthesis, and as a cofactor for HNF1A-dependent transcription. A deficiency of this enzyme leads to hyperphenylalaninemia. Alternative splicing results in multiple transcript variants. Involved in tetrahydrobiopterin biosynthesis (By similarity). Seems to both prevent the formation of 7-pterins and accelerate the formation of quinonoid-BH2. Coactivator for HNF1A-dependent transcription (By similarity). Regulates the dimerization of homeodomain protein HNF1A and enhances its transcriptional activity (By similarity). Also acts as a coactivator for HNF1B-dependent transcription.