Product Datasheet  
Alas1 Antibody  
Catalog Number: 48613  
Technical:tech@swbio.com  
Information:info@swbio.com  
Description  
  • host_species：  
  • Rabbit
  • Amount：  
  • 100μgμg
  • Swiss-Prot No.：  
  • Swiss-Prot#:P13196
  • Form of Antibody：  
  • 1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide.
  • Storage：  
  • Store at -20˚C
  • Immunogen：  
  • recombinant protein
  • reactivity：  
  • Hu
  • appl_detail：  
  • WB: 1:1,000-5,000

    ICC: 1:50-1:200

  • other_names：  
  • 5 aminolevulinate synthase antibody
    5 aminolevulinate synthase nonspecific mitochondrial antibody
    5 aminolevulinic acid synthase antibody
    5-aminolevulinate synthase antibody
    5-aminolevulinic acid synthase 1 antibody
    Alas 1 antibod
  • Purification：  
  • ProA affinity purified
  • Specificity：  
  • Applications：  
  • WB, ICC/IF
  • Background：  
  • 5-aminolevulinate synthase 1 (ALAS-H) and 2 (ALAS-E) are two isoforms of ALAS, an enzyme catalyzing the first step of the heme biosynthetic pathway in mammals. The erythroid-specific isoenzyme, ALAS-E, regulates the first step of hematopoietic cell differentation and iron metabolism in the liver. ALAS-H is a housekeeping protein which mediates synthesis of early heme in the mitochondria of most cells. Succinyl CoA associates with ALAS-E in protein conformation change and translocation of ALAS-E into the mitochondria and does not interact with ALAS-H. The ALAS-E 5'-flanking region contains binding sites for nuclear activators such as GATA-1, NF-E2 and EKLF. Since the ALAS gene maps to the X chromosome, mutation of the gene leads to the pyridoxine-refractory X-linked sideroblastic anemia.



 
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