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Gene Review

PNPO  -  pyridoxamine 5'-phosphate oxidase

Homo sapiens

Synonyms: HEL-S-302, PDXPO, Pyridoxamine-phosphate oxidase, Pyridoxine-5'-phosphate oxidase
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Disease relevance of PNPO


High impact information on PNPO


Chemical compound and disease context of PNPO


Biological context of PNPO


Anatomical context of PNPO


Associations of PNPO with chemical compounds

  • Other studies demonstrate that some children with intractable seizures respond to pyridoxal phosphate rather than pyridoxine, including a rare form of neonatal epileptic encephalopathy shown to be due to mutations in the PNPO gene for pyridox(am)ine 5'-phosphate oxidase [5].
  • Based on double immunofluorescence study, PLK and PNPO immunoreactive cells in the strata oriens and radiatum were identified as GABAergic cells [10].
  • Therefore, decreases of PLK and PNPO in the hippocampal CA1 region of aged brains may be involved in aging processes related with gamma-aminobutyric acid (GABA) function [10].

Other interactions of PNPO

  • Significant age-dependent changes in PLK and PNPO immunoreactivities were found in the CA1 region, but not in the CA2/3 region [10].


  1. Absence of pyridoxine-5'-phosphate oxidase (PNPO) activity in neoplastic cells: isolation, characterization, and expression of PNPO cDNA. Ngo, E.O., LePage, G.R., Thanassi, J.W., Meisler, N., Nutter, L.M. Biochemistry (1998) [Pubmed]
  2. Genomic organization, tissue distribution and deletion mutation of human pyridoxine 5'-phosphate oxidase. Kang, J.H., Hong, M.L., Kim, D.W., Park, J., Kang, T.C., Won, M.H., Baek, N.I., Moon, B.J., Choi, S.Y., Kwon, O.S. Eur. J. Biochem. (2004) [Pubmed]
  3. The molecular structure of Rv2074, a probable pyridoxine 5'-phosphate oxidase from Mycobacterium tuberculosis, at 1.6 angstroms resolution. Biswal, B.K., Au, K., Cherney, M.M., Garen, C., James, M.N. Acta Crystallograph. Sect. F Struct. Biol. Cryst. Commun. (2006) [Pubmed]
  4. Neonatal epileptic encephalopathy caused by mutations in the PNPO gene encoding pyridox(am)ine 5'-phosphate oxidase. Mills, P.B., Surtees, R.A., Champion, M.P., Beesley, C.E., Dalton, N., Scambler, P.J., Heales, S.J., Briddon, A., Scheimberg, I., Hoffmann, G.F., Zschocke, J., Clayton, P.T. Hum. Mol. Genet. (2005) [Pubmed]
  5. Pyridoxine-dependent seizures: new genetic and biochemical clues to help with diagnosis and treatment. Gospe, S.M. Curr. Opin. Neurol. (2006) [Pubmed]
  6. Vigabatrin inhibits pyridoxine-5'-phosphate oxidase, not pyridoxal kinase in the hippocampus of seizure prone gerbils. An, S.J., Park, S.K., Hwang, I.K., Choi, S.Y., Kwon, O.S., Won, M.H., Kang, T.C. Neurochem. Int. (2004) [Pubmed]
  7. Chronological changes in pyridoxine-5'-phosphate oxidase immunoreactivity in the seizure-sensitive gerbil hippocampus. Kang, T.C., Park, S.K., Hwang, I.K., An, S.J., Bahn, J.H., Kim, A.Y., Choi, S.Y., Kwon, O.S., Baek, N.I., Lee, H.Y., Won, M.H. J. Neurosci. Res. (2002) [Pubmed]
  8. Structure of Escherichia coli pyridoxine 5'-phosphate oxidase in a tetragonal crystal form: insights into the mechanistic pathway of the enzyme. Safo, M.K., Musayev, F.N., Schirch, V. Acta Crystallogr. D Biol. Crystallogr. (2005) [Pubmed]
  9. A simple fluorimetric assay for pyridoxamine phosphate oxidase in erythrocyte haemolysates: effects of riboflavin supplementation and of glucose 6-phosphate dehydrogenase deficiency. Bates, C.J., Powers, H.J. Human nutrition. Clinical nutrition. (1985) [Pubmed]
  10. Age-dependent changes of pyridoxal phosphate synthesizing enzymes immunoreactivities and activities in the gerbil hippocampal CA1 region. Hwang, I.K., Kim, D.W., Jung, J.Y., Yoo, K.Y., Cho, J.H., Kwon, O.S., Kang, T.C., Choi, S.Y., Kim, Y.S., Won, M.H. Mech. Ageing Dev. (2005) [Pubmed]
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