| 產(chǎn)品編號(hào) | bs-12694R |
| 英文名稱 | Rabbit Anti-PKLR antibody |
| 中文名稱 | 丙酮酸激酶肝型抗體 |
| 別 名 | EC 2.7.1.40; KPYR_HUMAN; L-PK; Pk-1; PK1; PKL; Pklg; Pklr; PKR; PKRL; Pyruvate kinase 1; Pyruvate kinase isozymes R/L; Pyruvate kinase liver and blood cell; Pyruvate kinase liver and RBC; Pyruvate kinase liver and red blood cell; Pyruvate kinase liver type; Pyruvate kinase type L; Pyruvate kinase, red cell type; R type/L type pyruvate kinase; R-PK; R-type/L-type pyruvate kinase; Red cell/liver pyruvate kinase; RPK. |
| 研究領(lǐng)域 | 腫瘤 細(xì)胞生物 信號(hào)轉(zhuǎn)導(dǎo) 激酶和磷酸酶 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human,Mouse,Rat,Rabbit,Cow,Horse) |
| 產(chǎn)品應(yīng)用 | IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 62kDa |
| 細(xì)胞定位 | 細(xì)胞漿 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human PKLR: 401-500/571 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
The protein encoded by this gene is a pyruvate kinase that catalyzes the transphosphorylation of phohsphoenolpyruvate into pyruvate and ATP, which is the rate-limiting step of glycolysis. Defects in this enzyme, due to gene mutations or genetic variations, are the common cause of chronic hereditary nonspherocytic hemolytic anemia (CNSHA or HNSHA). Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008] Function: Plays a key role in glycolysis. DISEASE: Defects in PKLR are the cause of pyruvate kinase hyperactivity (PKHYP) [MIM:102900]; also known as high red cell ATP syndrome. This autosomal dominant phenotype is characterized by increase of red blood cell ATP. Defects in PKLR are the cause of pyruvate kinase deficiency of red cells (PKRD) [MIM:266200]. A frequent cause of hereditary non-spherocytic hemolytic anemia. Clinically, pyruvate kinase-deficient patients suffer from a highly variable degree of chronic hemolysis, ranging from severe neonatal jaundice and fatal anemia at birth, severe transfusion-dependent chronic hemolysis, moderate hemolysis with exacerbation during infection, to a fully compensated hemolysis without apparent anemia. Similarity: Belongs to the pyruvate kinase family. SWISS: P30613 Gene ID: 5313 Database links: Entrez Gene: 5313 Human Entrez Gene: 18770 Mouse Omim: 609712 Human SwissProt: P30613 Human SwissProt: P53657 Mouse Unigene: 95990 Human Unigene: 383180 Mouse |
