抗人 PNPO 抗体对
01AB00906
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.
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技术参数
- 靶点基因名
- PNPO
- 蛋白别名
- pyridoxamine 5'-phosphate oxidase; PNPO
- UniProt
- Q9NVS9
- 物种
- Human
产品详情
- 靶点名称
- PNPO
- 蛋白名称
- pyridoxamine 5'-phosphate oxidase
- 蛋白别名
- Pyridoxine-5'-phosphate oxidase; Pyridoxamine-phosphate oxidase
- 靶点基因名
- PNPO
- UniProt
- Q9NVS9
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
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.