PNPO, Blocking Peptide

Pyridoxine-5'-phosphate oxidase; Pyridoxamine-phosphate oxidase; PNPO

For Research Use Only. Not for use in diagnostic procedures.

Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.

Small volumes of PNPO blocking peptide vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Catalyzes the oxidation of either pyridoxine 5'- phosphate (PNP) or pyridoxamine 5'-phosphate (PMP) into pyridoxal 5'-phosphate (PLP).

25,200 Da

pyridoxamine 5'-phosphate oxidase

pyridoxine-5'-phosphate oxidase

Pyridoxine-5'-phosphate oxidase

PNPO_HUMAN

The enzyme encoded by this gene catalyzes the terminal, rate-limiting step in the synthesis of pyridoxal 5'-phosphate, also known as vitamin B6. Vitamin B6 is a required co-factor for enzymes involved in both homocysteine metabolism and synthesis of neurotransmitters such as catecholamine. Mutations in this gene result in pyridoxamine 5'-phosphate oxidase (PNPO) deficiency, a form of neonatal epileptic encephalopathy. [provided by RefSeq, Oct 2008]

PNPO: Catalyzes the oxidation of either pyridoxine 5'- phosphate (PNP) or pyridoxamine 5'-phosphate (PMP) into pyridoxal 5'-phosphate (PLP). Defects in PNPO are the cause of pyridoxine-5'-phosphate oxidase deficiency (PNPO deficiency); also known as PNPO-related neonatal epileptic encephalopathy. The main feature of neonatal epileptic encephalopathy is the onset within hours of birth of a severe seizure disorder that does not respond to anticonvulsant drugs and can be fatal. Seizures can cease with the administration of PLP, being resistant to treatment with pyridoxine. Belongs to the pyridoxamine 5'-phosphate oxidase family.

Protein type: Oxidoreductase; EC 1.4.3.5; Cofactor and Vitamin Metabolism - vitamin B6

Chromosomal Location of Human Ortholog: 17q21.32

Cellular Component: cytoplasm; cytosol; nucleoplasm

Molecular Function: pyridoxamine-phosphate oxidase activity

Biological Process: positive regulation of defense response to virus by host; pyridoxal phosphate biosynthetic process; vitamin B6 metabolic process

Disease: Pyridoxamine 5-prime-phosphate Oxidase Deficiency

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