Anti-PNPO Antibody (CAB18495)
- SKU:
- CAB18495
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
Frequently bought together:
Description
| 抗体名: | Anti-PNPO Antibody |
| 抗体コード: | CAB18495 |
| 抗体サイズ: | 20uL, 50uL, 100uL |
| 申し込み: | WB IF |
| 反応性: | Human, Mouse, Rat |
| 宿主種: | Rabbit |
| 免疫原: | Recombinant protein of human PNPO. |
| 申し込み: | WB IF |
| 推奨希釈: | WB 1:500 - 1:2000 IF 1:50 - 1:200 |
| 反応性: | Human, Mouse, Rat |
| ポジティブサンプル: | HepG2, 293T, Mouse brain, Mouse kidney, Mouse liver, Rat brain, Rat kidney, Rat liver |
| 免疫原: | Recombinant protein of human PNPO. |
| 精製方法: | Affinity purification |
| ストレージバッファ: | Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| アイソタイプ: | IgG |
| 順序: | Email for sequence |
| 遺伝子ID: | 55163 |
| Uniprot: | Q9NVS9 |
| セルラーロケーション: | |
| 計算された分子量: | |
| 観察された分子量: | 30kDa |
 | Western blot - PNPO Polyclonal Antibody (CAB18495) |
| 同義語: | HEL-S-302, PDXPO, PNPO |
| バックグラウンド: |
| 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 |