Protein basic information
| LiverAtlas Protein ID |
HuLPr30000 |
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Uniprot ID |
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Uniprot Acc |
Q9NVS9;D3DTT9; |
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Protein name |
Pyridoxine-5'-phosphate oxidase |
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Comment |
FUNCTION:Catalyzes the oxidation of either pyridoxine 5'- phosphate (PNP) or pyridoxamine 5'-phosphate (PMP) into pyridoxal 5'-phosphate (PLP).||CATALYTIC ACTIVITY:Pyridoxamine 5'-phosphate + H(2)O + O(2) = pyridoxal 5'-phosphate + NH(3) + H(2)O(2).||CATALYTIC ACTIVITY:Pyridoxine 5'-phosphate + O(2) = pyridoxal 5'- phosphate + H(2)O(2).||COFACTOR:Binds 1 FMN per subunit.||PATHWAY:Cofactor biosynthesis; B6 vitamer interconversion; pyridoxal 5'-phosphate from pyridoxamine 5'-phosphate:step 1/1.||PATHWAY:Cofactor biosynthesis; B6 vitamer interconversion; pyridoxal 5'-phosphate from pyridoxine 5'-phosphate:step 1/1.||SUBUNIT:Homodimer.||DISEASE:Defects in PNPO are the cause of pyridoxine-5'-phosphate oxidase deficiency (PNPO deficiency) [MIM:610090]; 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. |
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Gene name |
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Related liver disease name |
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Protein sequence
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MTCWLRGVTATFGRPAEWPGYLSHLCGRSAAMDLGPMRKS YRGDREAFEETHLTSLDPVKQFAAWFEEAVQCPDIGEAN AMCLATCTRDGKPSARMLLLKGFGKDGFRFFTNFESRKG KELDSNPFASLVFYWEPLNRQVRVEGPVKKLPEEEAECY FHSRPKSSQIGAVVSHQSSVIPDREYLRKKNEELEQLYQ DQEVPKPKSWGGYVLYPQVMEFWQGQTNRLHDRIVFRRG LPTGDSPLGPMTHRGEEDWLYERLAP |
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Database cross reference |
RefSeq Protein accession:NP_060599
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Liver relevance
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HCC significant Proteins |
Yes/No |
Yes |
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Quality score |
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HLPP validation |
Yes/No |
Yes |
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Project name |
Chinese Liver; |
Ontology annotation
| GO-C |
GO:0005829;C:cytosol;EXP:Reactome. |
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GO-F |
GO:0010181;F:FMN binding;IEA:InterPro. GO:0004733;F:pyridoxamine-phosphate oxidase activity;EXP:Reactome. |
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GO-P |
GO:0055114;P:oxidation-reduction process;IEA:UniProtKB-KW. GO:0008615;P:pyridoxine biosynthetic process;IEA:UniProtKB-KW. |
Post-translational modification
| LiverAtlas Protein ID |
MOD type1 |
Position1 |
Residue1 |
Source name1 |
source ID1 |
Source method |
HLPP validation1 (Yes/no) |
Quality score |
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HuLPr30000 |
PHOSPHORYLATION |
40 |
S |
Phospho-ELM |
HTP |
N |
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HuLPr30000 |
PHOSPHORYLATION |
40 |
S |
PhosphoSitePlus |
HTP |
N |
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HuLPr30000 |
PHOSPHORYLATION |
164 |
S |
PhosphoSitePlus |
HTP |
yes |
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HuLPr30000 |
PHOSPHORYLATION |
241 |
S |
PhosphoSitePlus |
HTP |
yes |
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Pathway
| Pathway name |
