Cell Biology Antibodies 17

Anti-PNPO Antibody (CAB18495)

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SKU:
CAB18495
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Cell Biology

Description

system_update_altDatasheetsystem_update_altMSDS
Antibody Name:Anti-PNPO Antibody
Antibody SKU:CAB18495
Antibody Size:20uL, 50uL, 100uL
Application:WB IF
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant protein of human PNPO.
Application:WB IF
Recommended Dilution:WB 1:500 - 1:2000 IF 1:50 - 1:200
Reactivity:Human, Mouse, Rat
Positive Samples:HepG2, 293T, Mouse brain, Mouse kidney, Mouse liver, Rat brain, Rat kidney, Rat liver
Immunogen:Recombinant protein of human PNPO.
Purification Method:Affinity purification
Storage Buffer:Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:Email for sequence
Gene ID:55163
Uniprot:Q9NVS9
Cellular Location:
Calculated MW:
Observed MW:30kDa
Anti-PNPO Antibody (CAB18495)Western blot - PNPO Polyclonal Antibody (CAB18495)
Synonyms:HEL-S-302, PDXPO, PNPO
Background:
UniProt Protein Function:PNPO: Catalyzes the oxidation of either pyridoxine 5'- phosphate (PNP) or pyridoxamine 5'-phosphate (PMP) into pyridoxal 5'-phosphate (PLP). Defects in PNPO are the cause of pyridoxine-5'-phosphate oxidase deficiency (PNPO deficiency); 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. Belongs to the pyridoxamine 5'-phosphate oxidase family.
UniProt Protein Details:

Protein type:Oxidoreductase; EC 1.4.3.5; Cofactor and Vitamin Metabolism - vitamin B6

Chromosomal Location of Human Ortholog: 17q21.32

Cellular Component: cytoplasm; cytosol; nucleoplasm

Molecular Function:pyridoxamine-phosphate oxidase activity

Biological Process: positive regulation of defense response to virus by host; pyridoxal phosphate biosynthetic process; vitamin B6 metabolic process

Disease: Pyridoxamine 5-prime-phosphate Oxidase Deficiency

NCBI Summary:The enzyme encoded by this gene 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. [provided by RefSeq, Oct 2008]
UniProt Code:Q9NVS9
NCBI GenInfo Identifier:37082126
NCBI Gene ID:55163
NCBI Accession:Q9NVS9.1
UniProt Secondary Accession:Q9NVS9,B4E0V0, B4E152, B4E1D7, D3DTT9,
UniProt Related Accession:Q9NVS9
Molecular Weight:25,200 Da
NCBI Full Name:Pyridoxine-5'-phosphate oxidase
NCBI Synonym Full Names:pyridoxamine 5'-phosphate oxidase
NCBI Official Symbol:PNPO  
NCBI Official Synonym Symbols:PDXPO; HEL-S-302  
NCBI Protein Information:pyridoxine-5'-phosphate oxidase
UniProt Protein Name:Pyridoxine-5'-phosphate oxidase
UniProt Synonym Protein Names:Pyridoxamine-phosphate oxidase
Protein Family:Pyridoxine-5'-phosphate oxidase
UniProt Gene Name:PNPO  
UniProt Entry Name:PNPO_HUMAN
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