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| Product Name | Dysferlin [JF05-54] |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | DMAT antibody DYSF antibody DYSF_HUMAN antibody Dysferlin antibody Dysferlin limb girdle muscular dystrophy 2B (autosomal recessive) antibody Dysferlin limb girdle muscular dystrophy 2B antibody Dystrophy associated fer 1 like 1 antibody Dystrophy associated fer 1 like protein antibody Dystrophy associated fer1 like 1 antibody Dystrophy associated fer1 like protein antibody Dystrophy-associated fer-1-like protein antibody Fer 1 like protein 1 antibody Fer-1-like protein 1 antibody Fer1 like protein 1 antibody FER1L1 antibody FLJ00175 antibody FLJ90168 antibody LGMD 2B antibody LGMD2B antibody Limb girdle muscular dystrophy 2B (autosomal recessive) antibody Limb girdle muscular dystrophy 2B antibody Miyoshi myopathy antibody MM antibody MMD1 antibody |
| Product description | 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. |
| Immunogen | recombinant protein |
| Clonality | Monoclonal |
|---|---|
| Isotype | IgG |
| Host Species | Recombinant rabbit |
| Tested Applications | WBICC/IFIHC |
| WB:1:1,000-1:5,000 ICC:1:50-1:200 IHC:1:50-1:200 | |
| Species Reactivity | HumanMouse |
| Concentration | 1mg/ml |
| Alternative Names | DMAT antibody DYSF antibody DYSF_HUMAN antibody Dysferlin antibody Dysferlin limb girdle muscular dystrophy 2B (autosomal recessive) antibody Dysferlin limb girdle muscular dystrophy 2B antibody Dystrophy associated fer 1 like 1 antibody Dystrophy associated fer 1 like protein antibody Dystrophy associated fer1 like 1 antibody Dystrophy associated fer1 like protein antibody Dystrophy-associated fer-1-like protein antibody Fer 1 like protein 1 antibody Fer-1-like protein 1 antibody Fer1 like protein 1 antibody FER1L1 antibody FLJ00175 antibody FLJ90168 antibody LGMD 2B antibody LGMD2B antibody Limb girdle muscular dystrophy 2B (autosomal recessive) antibody Limb girdle muscular dystrophy 2B antibody Miyoshi myopathy antibody MM antibody MMD1 antibody |
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| Molecular Weight(MW) | 237 kDa |
| Cellular Localization | Cell membrane, Cytoplasmic vesicle membrane. |
Application
Fig1: Western blot analysis of Dysferlin on human skeletal muscle lysates using anti-Dysferlin antibody at 1/1,000 dilution.
Application
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.
Application
Fig3: Immunohistochemical analysis of paraffin-embedded human spleen tissue using anti-Dysferlin antibody. Counter stained with hematoxylin.
Application
Fig4: Immunohistochemical analysis of paraffin-embedded mouse skeletal muscle tissue using anti-Dysferlin antibody. Counter stained with hematoxylin.
Application
Fig5: Immunohistochemical analysis of paraffin-embedded mouse spleen tissue using anti-Dysferlin antibody. Counter stained with hematoxylin.
Application
Fig6: Immunohistochemical analysis of paraffin-embedded mouse heart tissue using anti-Dysferlin antibody. Counter stained with hematoxylin.| Positive Control | SW480, human skeletal muscle tissue, human spleen tissue, mouse skeletal muscle tissue, mouse heart tissue, mouse spleen tissue. |
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| Application Notes | WB:1:1,000-1:5,000 ICC:1:50-1:200 IHC:1:50-1:200 |
| Form | Liquid |
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| Storage Instructions | Store at +4℃ after thawing. Aliquot store at -20℃ or -80℃. Avoid repeated freeze / thaw cycles. |
| Storage Buffer | 1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide. |
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