Dysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive) (DYSF) antibody

Antigen: Dysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive) (DYSF)

Synonyms: FER1L1, LGMD2B, FLJ00175, FLJ90168, D6Pas3, AI604795, mFLJ00175, 2310004N10Rik, DYSF, MGC152169, fb73b05, wu:fb73b05, si:rp71-50c18.1, DKFZp459E1226

Clonality: Polyclonal

Host: Rabbit

Reactivity: Human

Application: Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

Catalog no.: ABIN125666

Additional Information

Alternative name: Dysferlin

Immunogen: A synthetic peptide corresponding to C-terminal of human Dysferlin.

Format: Ig-fraction

Specificity: Dysferlin 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. This antibody cross reacts with human.

Application Details

Application Notes: Titer/Working Dilution: This antibody may be diluted to a titer of 1:50-1:100 in an ABC method. However, depending on the staining conditions employed, we suggest that final dilution should be determined by the user. Staining Protocol: We suggest an incubation period of 30 minutes at room temperature. However, depending upon the fixation conditions and the staining system employed, optimal incubation conditions should be determined by the user. Formalin fixed paraffin embedded tissue sections require high temperature antigen unmasking with 10 mM citrate buffer, pH 6.0 prior to immunostaining. Positive Control: Skeletal muscle. Cellular localization: Cytoplasmic, cell membrane

Concentration: 100ug/ml approximately

Buffer: Purified immunoglobulin fraction of rabbit antiserum against Dysferlin protein containing sodium azide as preservative.

Storage: Store the antibody at 2-8o c. Do not use it beyond the expiration date stated on the label.

Restrictions: For Research Use. CE-certified for In Vitro Diagnostics (IVD) Use in the European Union

Images

Formalin fixed paraffin embedded human skeletal muscle stained with Dysferlin antibody