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BMPR2 Rabbit pAb (bs-21476R)  
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產品編號 bs-21476R
英文名稱 BMPR2 Rabbit pAb
中文名稱 骨形態發生蛋白2受體抗體
別    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.  
研究領域 心血管  免疫學  信號轉導  干細胞  生長因子和激素  細胞表面分子  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應 Mouse (predicted: Human,Rat,Rabbit,Pig,Dog,Horse)
產品應用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 112 kDa
檢測分子量
細胞定位 細胞核 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human BMPR2: 28-100/1038 <Extracellular>
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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.

SWISS:
Q13873

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



產品圖片
ample: Heart (Mouse) Lysate at 40 ug Primary: Anti-BMPR2 (bs-21476R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 112 kD Observed band size: 112 kD
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