Entity Details

Primary name PNPO_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionQ9NVS9
EntryNamePNPO_HUMAN
FullNamePyridoxine-5'-phosphate oxidase
TaxID9606
Evidenceevidence at protein level
Length261
SequenceStatuscomplete
DateCreated2003-09-26
DateModified2021-06-02

Ontological Relatives

GenesPNPO

GO terms

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GOName
GO:0004733 pyridoxamine-phosphate oxidase activity
GO:0005829 cytosol
GO:0008615 pyridoxine biosynthetic process
GO:0010181 FMN binding
GO:0030170 pyridoxal phosphate binding
GO:0042803 protein homodimerization activity
GO:0042816 vitamin B6 metabolic process
GO:0042823 pyridoxal phosphate biosynthetic process

Subcellular Location

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Domains

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DomainNameCategoryType
IPR000659 Pyridoxamine 5'-phosphate oxidaseFamilyFamily
IPR011576 Pyridoxamine 5'-phosphate oxidase, putativeDomainDomain
IPR012349 FMN-binding split barrelFamilyHomologous superfamily
IPR019576 Pyridoxine 5'-phosphate oxidase, dimerisation, C-terminalDomainDomain
IPR019740 Pyridoxamine 5'-phosphate oxidase, conserved siteSiteConserved site

Diseases

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Disease IDSourceNameDescription
610090 OMIMPyridoxine-5'-phosphate oxidase deficiency (PNPOD)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,. The disease is caused by variants affecting the gene represented in this entry.

Drugs

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DrugNameSourceType
DB00114 Pyridoxal phosphateDrugbanksmall molecule
DB02209 Pyridoxine phosphateDrugbanksmall molecule
DB03247 Flavin mononucleotideDrugbanksmall molecule
DB03345 MercaptoethanolDrugbanksmall molecule

Interactions

5 interactions