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货号： bs-4237R-Bio 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-4237R-Bio
- 英文名称
- Anti-BMPR2/Biotin
- 中文名称
- 生物素标记的骨形态发生蛋白2受体抗体
- 别 名
- BMP type II receptor; BMP type-2 receptor; BMPR 2; BMPR 3; BMPR II; BMPR-2; BMPR-II; Bmpr2; BMPR2_HUMAN; BMPR3; BMPRII; BMR 2; BMR2; Bone morphogenetic protein receptor type 2; Bone morphogenetic protein receptor type II; Bone morphogenetic protein receptor type-2; Bone morphogenic protein receptor type II serine threonine kinase; BRK 3; BRK3; PPH 1; PPH1; Serine threonine kinase type II activin receptor like kinase; T ALK; TALK; Type II activin receptor like kinase.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 心血管 免疫学 信号转导 干细胞 生长因子和激素 细胞表面分子
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Chicken, Pig, Cow, Horse, Rabbit,
- 产品应用
- WB=1:50-200 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=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
- 112kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human BMPR2
- 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:
On ligand binding, forms a receptor complex consisting of two type II and two type I transmembrane serine/threonine kinases. Type II receptors phosphorylate and activate type I receptors which autophosphorylate, then bind and activate SMAD transcriptional regulators. Binds to BMP-7, BMP-2 and, less efficiently, BMP-4. Binding is weak but enhanced by the presence of type I receptors for BMPs.
Function:
On ligand binding, forms a receptor complex consisting of two type II and two type I transmembrane serine/threonine kinases. Type II receptors phosphorylate and activate type I receptors which autophosphorylate, then bind and activate SMAD transcriptional regulators. Binds to BMP-7, BMP-2 and, less efficiently, BMP-4. Binding is weak but enhanced by the presence of type I receptors for BMPs.
Subcellular Location:
Membrane.
Tissue Specificity:
Highly expressed in heart and liver.
DISEASE:
Defects in BMPR2 are the cause of primary pulmonary hypertension (PPH1) [MIM:178600]. PPH1 is a rare autosomal dominant disorder characterized by plexiform lesions of proliferating endothelial cells in pulmonary arterioles. The lesions lead to elevated pulmonary arterial pression, right ventricular failure, and death. The disease can occur from infancy throughout life and it has a mean age at onset of 36 years. Penetrance is reduced. Although familial PPH1 is rare, cases secondary to known etiologies are more common and include those associated with the appetite-suppressant drugs.
Defects in BMPR2 are a cause of pulmonary venoocclusive disease (PVOD) [MIM:265450]. PVOD is a rare form of pulmonary hypertension in which the vascular changes originate in the small pulmonary veins and venules. The pathogenesis is unknown and any link with PPH1 has been speculative. The finding of PVOD associated with a BMPR2 mutation reveals a possible pathogenetic connection with PPH1.
Similarity:
Belongs to the protein kinase superfamily.
TKL Ser/Thr protein kinase family. TGFB receptor subfamily.
Contains 1 protein kinase domain.
Database links:Entrez Gene: 659Human
Entrez Gene: 12168Mouse
Entrez Gene: 140590Rat
Omim: 600799Human
SwissProt: Q13873Human
SwissProt: O35607Mouse
Unigene: 471119Human
Unigene: 391654Mouse
Unigene: 7106Mouse
Unigene: 40848Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.