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.

Pterin-4-alpha-carbinolamine dehydratase
PHS
4-alpha-hydroxy-tetrahydropterin dehydratase
Dimerization cofactor of hepatocyte nuclear factor 1-alpha (DCoH; Dimerization cofactor of HNF1)
Phenylalanine hydroxylase-stimulating protein
Pterin carbinolamine dehydratase (PCD)
PCBD1
DCOH, PCBD

p61457

WB,IHC

WB 1:1000
IHC 1:1000

Human,Mouse,Rat

Predicted size: 12 kDa

Rabbit

Cytoplasm. Nucleus.

Protein A affinity purified.

Recombinant Rabbit monoclonal

IgG

Liquid

1*TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.

Store at +4°C short term. Store at -20°C long term. Avoid freeze / thaw cycle.