PNPO

PNPO 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.

Full Name

Pyridoxamine 5'-Phosphate Oxidase

Function

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

Biological Process

Pyridoxal phosphate biosynthetic processManual Assertion Based On ExperimentIDA:CAFA
Pyridoxine biosynthetic processIEA:UniProtKB-KW

Cellular Location

Cytosol

Involvement in disease

Pyridoxine-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,.

Anti-PNPO antibodies

Mouse Anti-PNPO Recombinant Antibody (2C7) (CBMAB-P2279-YC)

Datasheet

SDS

Target: PNPO

Host: Mouse

Antibody Isotype: IgG1, κ

Specificity: Human

Clone: 2C7

Application*: WB, E

Mouse Anti-PNPO Recombinant Antibody (1G9) (CBMAB-P2278-YC)

Datasheet

SDS

Target: PNPO

Host: Mouse

Antibody Isotype: IgG2a

Specificity: Human

Clone: 1G9

Application*: WB, F, IH

For Research Use Only. Not For Clinical Use.

(P): Predicted

* Abbreviations

IFImmunofluorescence

IHImmunohistochemistry

IPImmunoprecipitation

WBWestern Blot

EELISA

MMicroarray

CIChromatin Immunoprecipitation

FFlow Cytometry

FNFunction Assay

IDImmunodiffusion

RRadioimmunoassay

TCTissue Culture

GSGel Supershift

NNeutralization

BBlocking

AActivation

IInhibition

DDepletion

ESELISpot

DBDot Blot

MCMass Cytometry/CyTOF

CTCytotoxicity

SStimulation

AGAgonist

APApoptosis

IMImmunomicroscopy

BABioassay

CSCostimulation

EMElectron Microscopy

IEImmunoelectrophoresis

PAPeptide Array

ICImmunocytochemistry

PEPeptide ELISA

MDMeDIP

SHIn situ hybridization

IAEnzyme Immunoassay

SEsandwich ELISA

PLProximity Ligation Assay

ECELISA(Cap)

EDELISA(Det)

BIBioimaging

IOImmunoassay

LFLateral Flow Immunoassay

LALuminex Assay

CImmunohistochemistry-Frozen Sections

PImmunohistologyp-Paraffin Sections

ISIntracellular Staining for Flow Cytometry

MSElectrophoretic Mobility Shift Assay

RIRNA Binding Protein Immunoprecipitation (RIP)