骨形态发生蛋白2受体抗体
规格：1mg/1ml
英文名： BMPR2
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 prot
分子量： 112kDa
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 BMPR2
交叉反应：Human, Mouse, Rat, Chicken, Pig, Cow, Horse, Rabbit,
细胞定位：细胞核
骨形态发生蛋白2受体抗体产品介绍：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骨形态发生蛋白2受体抗体 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骨形态发生蛋白2受体抗体 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. Gene ID: 659 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.
骨形态发生蛋白2受体抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1:50-300 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：心血管 **学 信号转导 干细胞 生长因子和** 细胞表面分子
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid