Background: Dysferlin is a muscle-specific protein that is essential for normal muscle function and development. 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. Dyferlin is highly expressed in skeletal muscle and commonly localized to the muscle plasma membrane. A mutation that causes muscular dystrophy weakens calcium dependent binding of the C2 domain of dyferlin to phospholipid. No or low immunoreactivity to dysferlin is seen in Limb-Girdle Muscular Dystrophy type 2B (LGMD2B) and Miyoshi Myopathy (MM).
Description: Rabbit polyclonal to Dysferlin
Immunogen: KLH conjugated synthetic peptide derived from Dysferlin
Specificity: ·Reacts with Human, Mouse, Dog, Rabbit and Rat.
·Isotype: IgG
Application: ·Western blotting: 1/100-500. Predicted Mol wt: 231 kDa;
·Immunohistochemistry (Frozen/paraffin tissue section): 1/100-500;
·Immunocytochemistry: 1/100-200;
·ELISA: 1/500;
·Optimal working dilutions must be determined by the end user.
