Rabbit anti-Human PNPO Polyclonal Antibody | anti-PNPO antibody

Anti-PNPO Antibody, Rabbit Polyclonal

Cat. Num. AAA8104006

• Gene Names: PNPO; PDXPO; HEL-S-302
• Reactivity: Human
• Applications: Western Blot, Immunohistochemistry
• Purity: Protein A & Antigen Affinity
• Synonyms: PNPO; Polyclonal Antibody; Anti-PNPO Antibody; Rabbit Polyclonal; PNPO Antibody; Rabbit PAb; Antigen Affinity Purified; pyridoxamine 5'-phosphate oxidase; Anti-HEL-S-302 Antibody; Anti-PDXPO Antibody; anti-PNPO antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: Rabbit IgG
• Specificity: Human PNPO
• Purity/Purification: Protein A & Antigen Affinity
• Form/Format: Liquid; PBS, pH7.0 with 0.03% Proclin300

• Applicable Applications for anti-PNPO antibody: Western Blot (WB), Immunohistochemistry-Paraffin (IHC-P)
• Application Notes: WB: 1:500-1:2000; IHC-P: 1:200-1:1000
• Immunogen: A synthetic peptide corresponding to the center region of the Human PNPO
• Conjugation: Unconjugated
• Preparation: Produced in rabbits immunized with a synthetic peptide corresponding to the center region of the Human PNPO, and purified by antigen affinity chromatography.
• Preparation and Storage: This antibody can be stored at 2-8 degree C for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20 degree C to -80 degree C. Avoid repeated freeze-thaw cycles.

Western Blot (WB)

(Anti-PNPO rabbit polyclonal antibody at 1:500 dilution Lane A: HepG2 Whole Cell Lysate Lane B: 293 Whole Cell Lysate Lysates/proteins at 30 ug per lane. Secondary Goat Anti-Rabbit IgG (H+L)/HRP at 1/10000 dilution. Developed using the ECL technique. Performed under reducing conditions. Predicted band size:30 kDa Observed band size:30 kDa)

Immunohistochemistry (IHC)

(Immunochemical staining of human PNPO in human kidney with rabbit polyclonal antibody at 1:300 dilution, formalin-fixed paraffin embedded sections.)

Related Product Information for anti-PNPO antibody

Pyridoxamine 5'-phosphate oxidase (PNPO) is a rate-limiting enzyme in converting dietary vitamin B6 (VB6) to pyridoxal 5'-phosphate (PLP), the biologically active form of VB6 and involved in the synthesis of neurotransmitters including gamma-aminobutyric acid (GABA), dopamine, and serotonin. PLP is an active form of vitamin B6 implicated in several types of cancer. In humans, PNPO mutations have been increasingly identified in neonatal epileptic encephalopathy and more recently also in early-onset epilepsy. Neonatal epileptic encephalopathy (NEE), as a result of pyridoxine 5'-phosphate oxidase (PNPO) deficiency, is a rare neural disorder characterized by intractable seizures and usually leads to early infant death. PNPO can serve as a novel tissue biomarker of epithelial ovarian cancer (EOC) and maybe a potential target for therapeutic intervention.

References

Zhang L, et al. (2017) Pyridoxine 5'-phosphate oxidase is a novel therapeutic target and regulated by the tgf-beta signalling pathway in epithelial ovarian cancer. Cell Death Dis 8 (12): 3214.Ciapaite J, et al. (2020) Pyridox(am)ine 5'-phosphate oxidase (pnpo) deficiency in zebrafish results in fatal seizures and metabolic aberrations. Biochim Biophys Acta Mol Basis Dis 1866 (3): 165607.Chi W, et al. (2019) Pyridox (am) ine 5'-phosphate oxidase deficiency induces seizures in drosophila melanogaster. Hum Mol Genet 28 (18): 3126-3136.Chen PY, et al. (2019) Pyridoxamine supplementation effectively reverses the abnormal phenotypes of zebrafish larvae with pnpo deficiency. Front Pharmacol 10 1086.

NCBI and Uniprot Product Information

• NCBI GI #: 8922498
• NCBI GeneID: 55163
• NCBI Accession #: NP_060599.1
• NCBI GenBank Nucleotide #: NM_018129.3
• UniProt Accession #: Q9NVS9; B4E0V0; B4E152; B4E1D7; D3DTT9
• Molecular Weight: 25,200 Da
• NCBI Official Full Name: pyridoxine-5'-phosphate oxidase
• NCBI Official 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; epididymis secretory protein Li 302; pyridoxal 5'-phosphate synthase; pyridoxamine-phosphate oxidase; pyridoxine 5'-phosphate oxidase
• UniProt Protein Name: Pyridoxine-5'-phosphate oxidase
• Protein Family: Pyridoxine-5'-phosphate oxidase
• UniProt Gene Name: PNPO
• UniProt Entry Name: PNPO_HUMAN

NCBI Description

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 Description

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.

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

Chromosomal Location of Human Ortholog: 17q21.32

Cellular Component: nucleoplasm; cytoplasm; cytosol

Molecular Function: FMN binding; pyridoxamine-phosphate oxidase activity

Biological Process: vitamin metabolic process; vitamin B6 metabolic process; pyridoxine biosynthetic process; water-soluble vitamin metabolic process; pyridoxal phosphate biosynthetic process

Disease: Pyridoxamine 5-prime-phosphate Oxidase Deficiency