Clone Name: Ham3/17B2
Application Note: IHC-P: 1:20–1:40. IHC-Fr: 1:20–1:40. *Optimal dilutions/concentrations should be determined by the researcher.Not tested in other applications.
Calculated MW: 237
Specificity/Sensitivity: Reactive with the dysferlin molecule in human skeletal muscle. Also present in many non-muscle tissues.
Form: Liquid
Buffer (with preservative): Tissue culture supernatant, 0.09% Sodium azide.
Concentration:
Background: 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. In addition, the protein encoded by this gene binds caveolin-3, a skeletal muscle membrane protein which is important in the formation of caveolae. Specific mutations in this gene have been shown to cause autosomal recessive limb girdle muscular dystrophy type 2B (LGMD2B) as well as Miyoshi myopathy. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Aug 2008]
Uniprot ID: O75923
Antigen Species: Human
Immunogen: Synthetic peptide containing amino acids 349-366, spanning exons 11 and 12, of the human dysferlin molecule.
Purification: Unpurified
Conjugation: Unconjugated
Full Name: dysferlin