Product Name :Dysferlin Recombinant Rabbit mAb (KU)
Applications :WB,IHC,ICC/IF
Application_all :WB: 1:1000<br />IHC: 1:100<br />ICC/IF: 1:50
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]
Product :Store at -20°C. Supplied in 50mM Tris-Glycine(pH 7.4), 0.15M NaCl, 40%Glycerol, 0.01% sodium azide and 0.05% BSA. Stable for 12 months from date of receipt.
Purification&Purity :Affinity Purification
Storage&Stability :Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Note :For research use only, not for use in diagnostic procedure.
Alternative Name :MMD1; FER1L1; LGMD2B
Immunogen :A synthetic peptide of human Dysferlin
Modification :Unmodification
