<strike id="amuic"></strike>
  • <abbr id="amuic"><option id="amuic"></option></abbr>
    <strike id="amuic"></strike>
    

  • <cite id="amuic"><source id="amuic"></source></cite>

    <cite id="amuic"><source id="amuic"></source></cite>
    • <ul id="amuic"><table id="amuic"></table></ul>
    


    
    
    
        
        
    
    
    

    

    
    
    
    
    
        
        
    
    
        
    

    

    
    
    
    
    
        
        
    
    
    
    最近搜索：細(xì)胞培養(yǎng) 微生物學(xué) 分子生物 生物化學(xué) 
    

    

    
	
    首頁(yè)>>免疫學(xué)>>一抗>>骨形態(tài)發(fā)生蛋白受體1B抗體  
    

    

    
	
    
    	
    
        	
    骨形態(tài)發(fā)生蛋白受體1B抗體
     
    
            
    
            	
    
            	
    
                	
      
                    	
    • 
                        	
      產(chǎn)品貨號(hào)：
      
                            
      BN41945R
      
                        
      • 
                    	
    • 
                        	
      中文名稱：
      
                            
      骨形態(tài)發(fā)生蛋白受體1B抗體 
      
                        
      • 
                                            	
    • 
                        	
      英文名稱：
      
                            
      Rabbit anti-BMPR1B Polyclonal antibody 
      
                        
      • 
                                                                    	
    • 
                        	
      品牌：
      
                            
      Biorigin 
      
                        
      • 
                    
    
                
    
            
    
            
    
                    
    
                        
      
                            
    • 
                                
      貨號(hào)
      
                                
      產(chǎn)品規(guī)格
      
                                
      售價(jià)
      
                                
      備注
      
                            
      • 
                                                        
    • 
                                
      BN41945R-50ul
      
                                
      50ul
      
                                
      ￥1486.00
      
                                
      交叉反應(yīng)：nan(predicted:Human,Mouse,Rat,Dog,Cow,Rabbit,Sheep)   推薦應(yīng)用：WB,ELISA
      
                            
      • 
                                                        
    • 
                                
      BN41945R-100ul
      
                                
      100ul
      
                                
      ￥2360.00
      
                                
      交叉反應(yīng)：nan(predicted:Human,Mouse,Rat,Dog,Cow,Rabbit,Sheep)   推薦應(yīng)用：WB,ELISA
      
                            
      • 
                                                        
    • 
                                
      BN41945R-200ul
      
                                
      200ul
      
                                
      ￥3490.00
      
                                
      交叉反應(yīng)：nan(predicted:Human,Mouse,Rat,Dog,Cow,Rabbit,Sheep)   推薦應(yīng)用：WB,ELISA
      
                            
      • 
                                                    
    
                    
    
              
    
            
    
            	
    
                	
    產(chǎn)品描述
    
                
    
                
    
                	
    英文名稱BMPR1B
    中文名稱骨形態(tài)發(fā)生蛋白受體1B抗體
    別    名BMPR-IB; Activin receptor like kinase 6; Acvrlk6; ALK 6; ALK6; alk6tr; BMP type-1B receptor; BMPR IB; BMPR-1B; Bmpr1b; BMPRIB; BMR1B_HUMAN; Bone morphogenetic protein receptor type 1B; Bone morphogenetic protein receptor type IB; Bone morphogenetic protein receptor type-1B; BR 1b; BR1b; CDw 293; CDw293; CDw293 antigen; CFK 43a; CFK43a; Serine/threonine receptor kinase; zALK 6; zALK6.  
    研究領(lǐng)域細(xì)胞生物  信號(hào)轉(zhuǎn)導(dǎo)  干細(xì)胞  轉(zhuǎn)錄調(diào)節(jié)因子  激酶和磷酸酶  細(xì)胞表面分子  細(xì)胞外基質(zhì)  表觀遺傳學(xué)  
    抗體來(lái)源Rabbit
    克隆類型Polyclonal
    交叉反應(yīng)(predicted: Human, Mouse, Rat, Dog, Cow, Rabbit, Sheep, .)
    產(chǎn)品應(yīng)用WB=1:500-2000 ELISA=1:5000-10000 
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    分 子 量56kDa
    細(xì)胞定位細(xì)胞膜 
    性    狀Liquid
    濃    度1mg/ml
    免 疫 原KLH conjugated synthetic peptide derived from human BMPR1B:61-160/502 <Extracellular>
    亞    型IgG
    純化方法affinity purified by Protein A
    儲(chǔ) 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
    保存條件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
    PubMedPubMed
    產(chǎn)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. Receptor for BMP7/OP-1 and GDF5.
    Involvement in disease; Defects in BMPR1B are the cause of acromesomelic chondrodysplasia with genital anomalies (AMDGA). Acromesomelic chondrodysplasias are rare hereditary skeletal disorders characterized by short stature, very short limbs, and hand/foot malformations. The severity of limb abnormalities increases from proximal to distal with profoundly affected hands and feet showing brachydactyly and/or rudimentary fingers (knob-like fingers).
    Defects in BMPR1B are a cause of brachydactyly type A2 (BDA2) [MIM:112600]. Brachydactylies (BDs) are a group of inherited malformations characterized by shortening of the digits due to abnormal development of the phalanges and/or the metacarpals. They have been classified on an anatomic and genetic basis into five groups, A to E, including three subgroups (A1 to A3) that usually manifest as autosomal dominant traits. BDA2 was described first in a large Norwegian kindred. BDA2 is caused by mutations in BMPR1B gene and studies demonstrate that these mutations function as dominant negatives in vitro and in vivo.

    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. Receptor for BMP7/OP-1 and GDF5.

    Subcellular Location:
    Membrane; Single-pass type I membrane protein.

    DISEASE:
    Defects in BMPR1B are the cause of acromesomelic chondrodysplasia with genital anomalies (AMDGA) [MIM:609441]. Acromesomelic chondrodysplasias are rare hereditary skeletal disorders characterized by short stature, very short limbs, and hand/foot malformations. The severity of limb abnormalities increases from proximal to distal with profoundly affected hands and feet showing brachydactyly and/or rudimentary fingers (knob-like fingers).
    Defects in BMPR1B are a cause of brachydactyly type A2 (BDA2) [MIM:112600]. Brachydactylies (BDs) are a group of inherited malformations characterized by shortening of the digits due to abnormal development of the phalanges and/or the metacarpals. They have been classified on an anatomic and genetic basis into five groups, A to E, including three subgroups (A1 to A3) that usually manifest as autosomal dominant traits. BDA2 was described first in a large Norwegian kindred. BDA2 is caused by mutations in BMPR1B gene and studies demonstrate that these mutations function as dominant negatives in vitro and in vivo.

    Similarity:
    Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.
    Contains 1 GS domain.
    Contains 1 protein kinase domain.

    SWISS:
    O00238

    Gene ID:
    658

    Database links:
    Entrez Gene: 658 Human
    Entrez Gene: 12167 Mouse
    Omim: 603248 Human
    SwissProt: O00238 Human
    SwissProt: P36898 Mouse
    Unigene: 598475 Human
    Unigene: 39089 Mouse


    Important Note:
    This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications

























    image.png