Dysferlin (DYSF) Mouse Monoclonal Antibody [Clone ID: Ham3/17B2]

CAT#: TA355285

Dysferlin Mouse Monoclonal Antibody

Specifications

Product Data

• Clone Name: Ham3/17B2
• Applications: IHC
• Recommended Dilution: 1:20 - 1:40
• Reactivities: Human
• Host: Mouse
• Isotype: IgG2b
• Clonality: Monoclonal
• Immunogen: Synthetic peptide containing amino acids 349-366, spanning exons 11 and 12, of the human dysferlin molecule
• Specificity: Reactive with the dysferlin molecule in human skeletal muscle. Also present in many non-muscle tissues
• Formulation: Lyophilized tissue culture supernatant containing sodium azide as a preservative.
• Reconstitution Method: The user is required to reconstitute the contents of the vial with the correct volume of sterile distilled water as indicated on the vial label
• Conjugation: Unconjugated
• Storage: Store at 2-8°C
• Stability: 12 months
• Gene Name: dysferlin
• Database Link:
  Entrez Gene 8291 Human
• Background: 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. Patients with other neuromuscular conditions demonstrate normal labeling patterns.
• Synonyms: dysferlin; FER1L1; FLJ00175; FLJ90168; LGMD2B; OTTHUMP00000202233; OTTHUMP00000202234; OTTHUMP00000202235; OTTHUMP00000202236; OTTHUMP00000202237; OTTHUMP00000202240