Dysferlin is the protein product of the 2p13 gene that is defective in patients with Limb-Girdle Muscular Dystrophy type 2B (LGMD2B) and MiyoshiMyopathy (MM). Dysferlin is normally localized to the muscle plasma membrane. Patients with LGMD2B and MM, the immunoreactivity to dysferlin is severely reduced or lost, depending on the type of mutation. This antibody is used for the characterization of LGMD2B and MM.
Applications:
Suitable for use in Western Blot and Immunohistochemistry. Other applications not tested.
Recommended Dilution:
Western Blot: 1ug/ml for 1h
Immunohistochemistry (Formalin fixed paraffin embedded): 1:50 for 10min at RT
Staining of formalin-fixed tissues requires boiling tissue sections in 10mM citrate buffer, pH 6.0 for 10min followed by cooling at RT for 20min
Optimal dilutions to be determined by the researcher.
Positive Control:
Skeletal muscle
Storage and Stability:
May be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Quick Search
Products 
