Rabbit polyclonal to DysferlinDysferlin is the protein product of the 2p13 gene that is defective in patients with Limb-Girdle Muscular Dystrophy type 2B (LGMD2B) and Miyoshi Myopathy (MM). Dysferlin is normally localized to the muscle plasma membrane. In patients with LGMD2B and MM, 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.
Immunogen
Synthetic peptide (unfortunately, the amino acid sequence is considered to be commercially sensitive) (Human) (C terminal).
Key Feature
Clonality
Polyclonal
Isotype
IgG
Host Species
Rabbit
Tested Applications
IHCIHC-PWB
IHC-P: Use at a dilution of 1/50, for 10 minutes. Staining of formalin-fixed tissues requires boiling tissue sections in 10mM Citrate, pH 6.0, for 10 minutes followed by cooling at room emperature for 20 minutes. WB: Use at a concentration of 1 μg/ml. Predicted molecular weight: 231 kDa. Optimal dilutions/concentrations should be determined by the end user.:
Human Skeletal Muscle stained with anti-Dysferlin antibody
Application
staining Dysferlin in human skeletal muscle by Immunohistochemistry (FFPE-sections).
Application Notes
IHC-P: Use at a dilution of 1/50, for 10 minutes. Staining of formalin-fixed tissues requires boiling tissue sections in 10mM Citrate, pH 6.0, for 10 minutes followed by cooling at room emperature for 20 minutes. WB: Use at a concentration of 1 μg/ml. Predicted molecular weight: 231 kDa. Optimal dilutions/concentrations should be determined by the end user.:
Additional Information
Form
Liquid
Storage Instructions
Keep as concentrated solution, aliquot and store at 4°C. Do not freeze.