Product Datasheet  
Dysferlin (Romeo) Rabbit mAb  
Catalog Number: 78837  
Technical:tech@swbio.com  
Information:info@swbio.com  
Description  
  • host_species:  
  • Rabbit
  • Amount:  
  • 100μgμg
  • Swiss-Prot No.:  
  • O75923
  • Form of Antibody:  
  • Storage:  
  • Store at -20℃. Avoid freeze / thaw cycles.|Buffer: PBS with 0.02% sodium azide,0.05% BSA,50% glycerol,pH7.3.
  • Immunogen:  
  • A synthesized peptide derived from human Dysferlin (Romeo)
  • reactivity:  
  • Human,Mouse
  • appl_detail:  
  • WB 1:500 - 1:2000
    IHC 1:50 - 1:200
  • other_names:  
  • FER1L1, LGMD2B, MMD1
  • Purification:  
  • Affinity purification
  • Specificity:  
  • Applications:  
  • WB,IHC
  • Background:  
  • The protein encoded by this gene belongs to the ferlin family and is a skeletal muscle protein found associated with the sarcolemma. It is involved in muscle contraction and contains C2 domains that play a role in calcium-mediated membrane fusion events, suggesting that it may be involved in membrane regeneration and repair. In addition, the protein encoded by this gene binds caveolin-3, a skeletal muscle membrane protein which is important in the formation of caveolae. Specific mutations in this gene have been shown to cause autosomal recessive limb girdle muscular dystrophy type 2B (LGMD2B) as well as Miyoshi myopathy. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Aug 2008]



 
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