产品中心
当前位置：首页>产品中心Anti-PKLR/RBITC
货号： bs-12694R-RBITC 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-12694R-RBITC
- 英文名称
- Anti-PKLR/RBITC
- 中文名称
- 罗丹明（RBITC）标记的丙酮酸激酶肝型抗体
- 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 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.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 肿瘤 细胞生物 信号转导 激酶和磷酸酶
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Cow, Horse, Rabbit,
- 产品应用
- ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 62kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human PKLR
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 保存条件
- Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
- 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. 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.
Database links:Entrez Gene: 5313Human
Entrez Gene: 18770Mouse
Omim: 609712Human
SwissProt: P30613Human
SwissProt: P53657Mouse
Unigene: 95990Human
Unigene: 383180Mouse
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.