<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：細胞培養 微生物學 分子生物 生物化學 
    

    

    
	
    首頁>>免疫學>>一抗>>芳基硫酸酯酶A抗體  
    

    

    
	
    
    	
    
        	
    芳基硫酸酯酶A抗體
     
    
            
    
            	
    
            	
    
                	
      
                    	
    • 
                        	
      產品貨號：
      
                            
      BN40989R
      
                        
      • 
                    	
    • 
                        	
      中文名稱：
      
                            
      芳基硫酸酯酶A抗體 
      
                        
      • 
                                            	
    • 
                        	
      英文名稱：
      
                            
      Rabbit anti-ARSA Polyclonal antibody 
      
                        
      • 
                                                                    	
    • 
                        	
      品牌：
      
                            
      Biorigin 
      
                        
      • 
                    
    
                
    
            
    
            
    
                    
    
                        
      
                            
    • 
                                
      貨號
      
                                
      產品規格
      
                                
      售價
      
                                
      備注
      
                            
      • 
                                                        
    • 
                                
      BN40989R-100ul
      
                                
      100ul
      
                                
      ￥2360.00
      
                                
      交叉反應：Mouse,Rat(predicted:Human,Dog,Pig,Cow,Horse,Rabbit)   推薦應用：WB,IHC-P,IHC-F,IF,ELISA
      
                            
      • 
                                                        
    • 
                                
      BN40989R-200ul
      
                                
      200ul
      
                                
      ￥3490.00
      
                                
      交叉反應：Mouse,Rat(predicted:Human,Dog,Pig,Cow,Horse,Rabbit)   推薦應用：WB,IHC-P,IHC-F,IF,ELISA
      
                            
      • 
                                                    
    
                    
    
              
    
            
    
            	
    
                	
    產品描述
    
                
    
                
    
                	
    英文名稱ARSA
    中文名稱芳基硫酸酯酶A抗體
    別    名As 2; As2; ASA; metachromatic leucodystrophy; TISP73; arylsulfatase A; AS A; MLD.  
    研究領域免疫學  神經生物學  
    抗體來源Rabbit
    克隆類型Polyclonal
    交叉反應Mouse, Rat,  (predicted: Human, Dog, Pig, Cow, Horse, Rabbit, )
    產品應用WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復）
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    分 子 量45/54kDa
    細胞定位細胞漿 
    性    狀Liquid
    濃    度1mg/ml
    免 疫 原KLH conjugated synthetic peptide derived from human ARSA :368-412/507 
    亞    型IgG
    純化方法affinity purified by Protein A
    儲 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
    產品介紹The protein encoded by this gene hydrolyzes cerebroside sulfate to cerebroside and sulfate. Defects in this gene lead to metachromatic leucodystrophy (MLD), a progressive demyelination disease which results in a variety of neurological symptoms and ultimately death. Alternatively spliced transcript variants have been described for this gene. [provided by RefSeq, Dec 2010].

    Function:
    Hydrolyzes cerebroside sulfate.

    Subunit:
    Homodimer at neutral pH and homooctamer at acidic pH. Exists both as a single chain of 58 kDa (component A) or as a chain of 50 kDa (component B) linked by disulfide bond(s) to a 7 kDa chain (component C). Interacts with SUMF1.

    Subcellular Location:
    Lysosome.

    Post-translational modifications:
    The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity. This post-translational modification is severely defective in multiple sulfatase deficiency (MSD).

    DISEASE:
    Defects in ARSA are a cause of leukodystrophy metachromatic (MLD) [MIM:250100]. MLD is a disease due to a lysosomal storage defect. It is characterized by intralysosomal storage of cerebroside-3-sulfate in neural and non-neural tissues, with a diffuse loss of myelin in the central nervous system. Progressive demyelination causes a variety of neurological symptoms, including gait disturbances, ataxias, optical atrophy, dementia, seizures, and spastic tetraparesis. Three forms of the disease can be distinguished according to the age at onset: late-infantile, juvenile and adult.
    Arylsulfatase A activity is defective in multiple sulfatase deficiency (MSD) [MIM:272200]. A clinically and biochemically heterogeneous disorder caused by the simultaneous impairment of all sulfatases, due to defective post-translational modification and activation. It combines features of individual sulfatase deficiencies such as metachromatic leukodystrophy, mucopolysaccharidosis, chondrodysplasia punctata, hydrocephalus, ichthyosis, neurologic deterioration and developmental delay. Note=Arylsulfatase A activity is impaired in multiple sulfatase deficiency due to mutations in SUMF1. SUMF1 mutations result in defective post-translational modification of ARSA at residue Cys-69 that is not converted to 3-oxoalanine.

    Similarity:
    Belongs to the sulfatase family.

    SWISS:
    P15289

    Gene ID:
    410

    Database links:
    Entrez Gene: 410 Human
    Entrez Gene: 11883 Mouse
    Entrez Gene: 315222 Rat
    Omim: 607574 Human
    SwissProt: P15289 Human
    SwissProt: P50428 Mouse
    Unigene: 88251 Human
    Unigene: 620 Mouse
    Unigene: 23323 Rat


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

    ArsA蛋白是存在于微生物細胞膜上的一種亞砷酸根陰離子泵的水溶性部分。在亞砷酸根存在的情況下。ArsA具有ATP酶活力。它水解ATP,發生構象變化.芳基硫酯酶A（ArylsulfataseA， ARSA）的缺陷，使溶酶體內腦硫酯水解受阻，沉積于中樞神經系統的白質、周圍神經系統及其它內臟組織，導致異染性腦白質營養不良（Metachromatic Leukodystrophy，MLD），他是一種較常見的腦白質營養不良，也是一種最常見的溶酶體病，為常染色體隱性遺傳。






















    image.png
    image.png