丙酮酸激酶肝型抗体
规格：1mg/1ml
英文名： PKLR
别名： 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 kin
分子量： 62kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human PKLR
交叉反应：Human, Mouse, Rat,
细胞定位：细胞浆
丙酮酸激酶肝型抗体产品介绍：background: 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丙酮酸激酶肝型抗体. 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, 丙酮酸激酶肝型抗体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. 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 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
丙酮酸激酶肝型抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 信号转导 激酶和磷酸酶
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid