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BMPR-II rabbit pAb
商品貨號(hào)： PLA021293
適 應(yīng) 性： 人,小鼠,大鼠,猴
WB ELISA
￥600元
規(guī)格：
在線咨詢
MSDS
說(shuō)明書
商品描述
  • 基因名稱： BMPR2 PPH1
  • 蛋白名稱： BMPR-II
  • Human_gene_id： 659
  • Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=659
  • Human_swiss_prot_no： Q13873
  • Human_swiss_link： https://www.uniprot.org/uniprotkb/Q13873/entry
  • Mouse_gene_id： 12168
  • Mouse_gene_link： https://www.uniprot.org/uniprot/12168
  • Mouse_swiss_prot_no： O35607
  • Mouse_swiss_link： https://www.uniprot.org/uniprotkb/O35607
  • 特異性： This antibody detects endogenous levels of Human,Mouse,Rat,Monkey BMPR-II
  • 組成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 來(lái)源： Polyclonal, Rabbit,IgG
  • 稀釋： WB 1:1000-2000 ELISA 1:5000-20000
  • 純化工藝： The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 濃度： 1 mg/ml
  • 儲(chǔ)存： -15°C to -25°C/1 year(Do not lower than -25°C)
  • 說(shuō)明書： 1
  • Msds： MSDS_Antibody.pdf
  • 其他名稱： Bone morphogenetic protein receptor type-2 (BMP type-2 receptor;BMPR-2;EC 2.7.11.30;Bone morphogenetic protein receptor type II;BMP type II receptor;BMPR-II)
  • 分子量： 114kD
  • 功能： catalytic activity:ATP + [receptor-protein] = ADP + [receptor-protein] phosphate.,cofactor:Magnesium or manganese.,disease: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.,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.,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.,similarity:Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.,similarity:Contains 1 protein kinase domain.,tissue specificity:Highly expressed in heart and liver.,
  • 相關(guān)產(chǎn)品： RS0001,RS0002,YM3028,YM3306
  • 細(xì)胞定位： Cell membrane ; Single-pass type I membrane protein.
  • 組織表達(dá)： Highly expressed in heart and liver.
  • 科研貨號(hào)： PLA021293
BMPR-II rabbit pAb
Catalog No PLA021293
Product information
  • 基因名稱： BMPR2 PPH1
  • 蛋白名稱： BMPR-II
  • Human_gene_id： 659
  • Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=659
  • Human_swiss_prot_no： Q13873
  • Human_swiss_link： https://www.uniprot.org/uniprotkb/Q13873/entry
  • Mouse_gene_id： 12168
  • Mouse_gene_link： https://www.uniprot.org/uniprot/12168
  • Mouse_swiss_prot_no： O35607
  • Mouse_swiss_link： https://www.uniprot.org/uniprotkb/O35607
  • 特異性： This antibody detects endogenous levels of Human,Mouse,Rat,Monkey BMPR-II
  • 組成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 來(lái)源： Polyclonal, Rabbit,IgG
  • 稀釋： WB 1:1000-2000 ELISA 1:5000-20000
  • 純化工藝： The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 濃度： 1 mg/ml
  • 儲(chǔ)存： -15°C to -25°C/1 year(Do not lower than -25°C)
  • 說(shuō)明書： 1
  • Msds： MSDS_Antibody.pdf
  • 其他名稱： Bone morphogenetic protein receptor type-2 (BMP type-2 receptor;BMPR-2;EC 2.7.11.30;Bone morphogenetic protein receptor type II;BMP type II receptor;BMPR-II)
  • 分子量： 114kD
  • 功能： catalytic activity:ATP + [receptor-protein] = ADP + [receptor-protein] phosphate.,cofactor:Magnesium or manganese.,disease: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.,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.,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.,similarity:Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.,similarity:Contains 1 protein kinase domain.,tissue specificity:Highly expressed in heart and liver.,
  • 相關(guān)產(chǎn)品： RS0001,RS0002,YM3028,YM3306
  • 細(xì)胞定位： Cell membrane ; Single-pass type I membrane protein.
  • 組織表達(dá)： Highly expressed in heart and liver.
  • 科研貨號(hào)： PLA021293
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    E-mail：service@uptbio.com
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