Human PCBD1 ELISA Kit (HUFI07313)

Built by Assay Genie for human PCBD1 research, this immunoassay kit quantifies Pterin-4-alpha-carbinolamine dehydratase, the dimerization cofactor of HNF1 (DCOH) involved in tetrahydrobiopterin (BH4) regeneration. The 96 Assays format supports in vitro studies of phenylalanine metabolism, BH4 biochemistry, and HNF1-mediated transcriptional regulation across laboratory workflows.

PCBD1 is a small bifunctional cytosolic protein that dehydrates pterin-4a-carbinolamine during BH4 recycling, the essential cofactor used by phenylalanine hydroxylase and the aromatic amino acid hydroxylases. Mutations in PCBD1 cause a recessive form of BH4-deficient hyperphenylalaninemia, sometimes accompanied by elevated proline, and altered PCBD1 levels have been examined in renal tubular cysts, fibrotic liver specimens, and certain epithelial cancer models.

The kit ships with pre-coated microplate wells, biotinylated detection antibody, streptavidin-HRP conjugate, calibrated recombinant standards, wash buffer, substrate, and stop solution, all formatted for a standard 96 Assays plate workflow. Reagents are intended for research use only, and a lot-specific certificate of analysis is supplied with each shipment from Assay Genie.