Anti-Human PNPO Antibody Pair

Product Description: Pyridoxamine 5'-phosphate oxidase (PNPO) is a homodimeric enzyme essential for vitamin B6 metabolism, catalyzing the oxidation of pyridoxine 5'-phosphate (PNP) or pyridoxamine 5'-phosphate (PMP) into pyridoxal 5'-phosphate (PLP), the active form of vitamin B6. Structurally, each subunit features a split-barrel motif composed of both alpha-helices and beta-sheets, stabilized by disulfide bonds and salt-bridge interactions, and binds one PLP molecule tightly. The enzyme requires flavin mononucleotide (FMN) as a cofactor, which is held in a deep cleft between the subunits and is crucial for substrate binding and catalysis. The active site undergoes conformational changes upon substrate binding, shifting from an open to a partially closed state to facilitate PLP production. Mutations in the PNPO gene can lead to neonatal epileptic encephalopathy, a severe neurological disorder characterized by early-onset seizures due to impaired PLP synthesis. Protein Function: Catalyzes the oxidation of either pyridoxine 5'-phosphate (PNP) or pyridoxamine 5'-phosphate (PMP) into pyridoxal 5'-phosphate (PLP) [The Uniprot Consortium]