Center for Bioinformatics
Oxidoreductases | Transferases | Hydrolases | Lyases | Isomerases | Ligases

Basic Information

Enzyme Number

1.4.3.5

Official Name

pyridoxal 5'-phosphate synthase

Name from literature

pyridoxamine 5'-phosphate oxidase

Pathway from literature

pyridoxal 5 phosphate (PLP, vitamin B(6)) synthesis

Pathway from KEGG

Metabolism of Cofactors and Vitamins; Vitamin B6 metabolism; map00750

Organisms

Human (9606)

Genome localization

17q21.32[55163 ],

Comments

A flavoprotein (FMN). In Escherichia coli, the coenzyme pyridoxal 5'-phosphate is synthesized de novo by a pathway that involves EC 1.2.1.72 (erythrose-4-phosphate dehydrogenase), EC 1.1.1.290 (4-phosphoerythronate dehydrogenase), EC 2.6.1.52 (phosphoserine transaminase), EC 1.1.1.262 (4-hydroxythreonine-4-phosphate dehydrogenase), EC 2.6.99.2 (pyridoxine 5'-phosphate synthase) and EC 1.4.3.5 (with pyridoxine 5'-phosphate as substrate). N4'-Substituted pyridoxamine derivatives are also oxidized in reaction (1) to form pyridoxal 5'-phosphate and the corresponding primary amine.

Rate-limiting Description

"In particular, pyridoxine- 5'-phosphate oxidase (PNPO), the rate-limiting enzyme in pyridoxal 5'-phosphate (PLP) biosynthesis, is absent in liver and neurally-derived tumors." (9601034)

" We hypothesized that the gene PNPO (pyridoxine 5'-phosphatase oxidase gene) might be a candidate for susceptibility to schizophrenia because PNPO encodes pyridoxamine 5'-phosphate oxidase (EC 1.4.3.5), a rate-limiting enzyme in pyridoxal 5'-phosphate (PLP, vitamin B(6)) synthesis." (17851041)

Regulatory Information

Regulatory type

Detail

phosphorylation;

Q9NVS9:from_uniprot:165_Phosphoserine

phosphorylation;

Q9NVS9:from_uniprot:241_Phosphoserine

phosphorylation;

Q9NVS9:from_uniprot:40_Phosphoserine

phosphorylation;

Q9NVS9

Gene ontology

Gene ontology

GO:0008615 (P) pyridoxine biosynthetic process [Q9NVS9 ];
GO:0055114 (P) oxidation reduction [Q9NVS9 ];
GO:0010181 (F) FMN binding [Q9NVS9 ];
GO:0004733 (F) pyridoxamine-phosphate oxidase activity [Q9NVS9 ];

Disease relevance

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. Seizures can cease with the administration of PLP, being resistant to treatment with pyridoxine, [Q9NVS9 ];

Links

SwissProt

Q9NVS9

Entrez Gene

55163

HPRD

04476



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  Last Modified: 2009-03-24  
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