Rabbit Monoclonal PCBD1 Antibody

The PCBD1 (Pterin-4-alpha-carbinolamine Dehydratase 1) antibody is a tool used to study the PCBD1 protein, which plays a critical role in cellular metabolic pathways. PCBD1. also known as DCoH (Dimerization Cofactor of HNF1), is a bifunctional enzyme involved in tetrahydrobiopterin (BH4) recycling and transcriptional regulation. In the phenylalanine hydroxylation pathway, PCBD1 catalyzes the dehydration of 4-alpha-carbinolamine tetrahydrobiopterin to quinonoid dihydrobiopterin, ensuring BH4 regeneration—a cofactor essential for neurotransmitter synthesis and nitric oxide production. Additionally, PCBD1 interacts with hepatocyte nuclear factor 1 (HNF1) to modulate gene expression in the liver, pancreas, and kidneys. Dysregulation of PCBD1 is linked to metabolic disorders, including hyperphenylalaninemia, and variants in the PCBD1 gene are associated with mild forms of tetrahydrobiopterin deficiency. Antibodies targeting PCBD1 are employed in research to investigate its expression, localization, and interactions in disease models, particularly inborn errors of metabolism and diabetes. They are vital for techniques like Western blotting, immunohistochemistry, and co-immunoprecipitation, aiding in the understanding of PCBD1’s dual enzymatic and regulatory roles. Commercial PCBD1 antibodies are typically raised in rabbits or mice, with validation data ensuring specificity for accurate experimental outcomes.