Reactivity: | H,M |
Applications: | WB |
Host Species: | Rabbit |
Clonality: | Polyclonal |
Full Name: | Dysferlin rabbit polyclonal antibody |
Gene Name: | Dysferlin |
Synonyms: | Dystrophy-associated fer-1-like protein, Fer-1-like protein 1, Dysf, Fer1l1, DMAT, LGMD2B, MMD1, Miyoshi myopathy |
Immunogen: | KLH conjugated Synthetic peptide corresponding to Mouse Dysferlin |
Isotype: | IgG |
Purity: | Affinity purification |
Predicted MW. | 238 kDa |
Observed MW. | 280 kDa |
Uniprot ID: | O75923, Q9ESD7 |
Product Usage Information
Applications | Species | Dilution | Positive Sample |
WB | Human, Mouse | 1: 500-1: 1000 | heart , skeletal muscle |
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.
Images
Western blot analysis of Dysferlin (GB112212) at dilution of 1: 500 | |
Western blot analysis of Dysferlin (GB112212) at dilution of 1: 500 |
Storage
Storage | Store at -20°C for one year. Avoid repeated freeze/thaw cycles. |
Storage Buffer | PBS with 0.02%sodium azide,100 μg/ml BSA and 50% glycerol. |