Dysferlin Recombinant Rabbit monoclonal Antibody IgG

SKU: BA112048-100µl
$279.00Price

Fig1: Western blot analysis of Dysferlin on human skeletal muscle lysates using anti-Dysferlin antibody at 1/1,000 dilution.

Fig2: ICC staining Dysferlin in SW480 cells (red). The nuclear counter stain is DAPI (blue). Cells were fixed in paraformaldehyde, permeabilised with 0.25% Triton X100/PBS.

Fig3: Immunohistochemical analysis of paraffin-embedded human spleen tissue using anti-Dysferlin antibody. Counter stained with hematoxylin.

Bon Opus Cat. #BA112048
Size
  • Host Species; Species Reactivity

    Rabbit; Human, Mouse
  • Immunogen

    Recombinant protein
  • Application Summary

    WB, ICC/IF, IHC
  • Purification; Formulation

    ProA affinity purified; 1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide.; Liquid form.
  • ALTnames

    Dysferlin, Dystrophy-associated fer-1-like protein, Fer-1-like protein 1
  • Background

    Dysferlin is a muscle-specific protein that is essential for normal muscle function and development. Mutations in the human dysferlin gene, DYSF, which maps to chromosome 2p13.2, are associated with limb girdle muscular dystrophy-2B (LGMD-2B) and a related, adult-onset, distal dystrophy known as Miyoshi myopathy (MM). Dysferlin localizes to the muscle fiber membrane, but is absent in MM and LGMD-2B muscle. Dysferlin is detected in 5-6 week embryos, when limbs begin to form regional differentiation. Although it is not essential for initial myogenesis, dysferlin appears to be critical for sustained normal function in mature muscle. It has been suggested that the absence of dysferlin during development gives rise to the disease phenotype in adulthood. Identical mutations in the dysferlin gene can produce more than one myopathy phenotype, indicating that additional genes and/or other factors are also involved in the clinical phenotype. The DYSF gene has no homology to any other known mammalian gene, but the protein product is related to the spermatogenesis factor fer-1 of Caenorhabditis elegans. The name “dysferlin” combines the role of the gene in producing muscular dystrophy with its homology to C. elegans.(ET1702-45)

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