FITC标记的骨形态发生蛋白2受体抗体
产品名称： FITC标记的骨形态发生蛋白2受体抗体
英文名称： Anti-BMPR2/FITC
产品编号： HZ-4237R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： IF=1:50-200
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Rabbit Anti-BMPR2/FITC Conjugated antibody
FITC标记的骨形态发生蛋白2受体抗体
| 英文名称 | Anti-BMPR2/FITC |
| 中文名称 | FITC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 | 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: 659 Human Entrez Gene: 12168 Mouse Entrez Gene: 140590 Rat Omim: 600799 Human SwissProt: Q13873 Human SwissProt: O35607 Mouse Unigene: 471119 Human Unigene: 391654 Mouse Unigene: 7106 Mouse Unigene: 40848 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
在配体结合上，形成由两种II型和两种I型跨膜丝氨酸/苏氨酸激酶组成的受体复合物。II型受体磷酸化并激活I型受体，其自身磷酸化，然后结合并激活SMAD转录调节子。结合BMP-7，BMP-2，且效率较低，BMP-4。结合是弱的，但增强了I型受体的骨形态发生蛋白的存在。