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General description
Dysferlin (UniProt O75923; also known as Dystrophy-associated fer-1-like 1 protein, Fer-1-like family member 1, Fer-1-like protein 1, Limb girdle muscular dystrophy 2B (autosomal recessive) is encoded by the DYSF (also known as FER1L1, LGMD2B, MMD1) gene (Gene ID 8291) in human. Dysferlin is a member of the ferlin family of calcium-dependent phospholipid-binding proteins involved in membrane vesicle fusion, membrane repair, T-tubule stability, and calcium homeostasis. Defects in dysferlin function/expression leads to muscular dystrophies (dysferlinopathies) that include Miyoshi myopathy (MM), limb-girdle muscular dystrophy type 2B (LGMD-2B), and distal myopathy with anterior tibial onset (DMAT).
~237 kDa observed. Uncharacterized band(s) may appear in some lysates.
Immunogen
Epitope: N-terminal
GST-tagged recombinant protein corresponding to the N-terminal of human Dysferlin/DYSF.
Application
Immunohistochemistry Analysis: A 1:50-1,000 dilution from a representative lot detected Dysferlin/DYSF in human colon tissue and human skeletal muscle myocytes.
Research Category
Apoptosis & Cancer
Research Sub Category
Apoptosis - Additional
This Anti-Dysferlin/DYSF Antibody, clone 10H3.1 is validated for use in Western Blotting, Immunohistochemistry (Paraffin) for the detection of Dysferlin/DYSF.
Biochem/physiol Actions
Expected to react with spliced isoforms 1, 3, 4, 6, 9, 10, 12, 14, and 15. Reactivity toward isoforms 2, 5, 7, 8, 11, and 13 has not been tested.
Physical form
Format: Purified
Protein G Purified
Purified mouse monoclonal IgG2bκ antibody in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
Preparation Note
Stable for 1 year at 2-8°C from date of receipt.
Analysis Note
Evaluated by Western Blotting in human fetal skeletal muscle tissue lysate.
Western Blotting Analysis: 0.5 µg/mL of this antibody detected Dysferlin/DYSF in 10 µg of human fetal skeletal muscle tissue lysate.
Other Notes
Concentration: Please refer to lot specific datasheet.
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
| biological source | mouse |
| Quality Segment | 100 |
| conjugate | unconjugated |
| antibody form | purified immunoglobulin |
| antibody product type | primary antibodies |
| clone | 10H3.1, monoclonal |
| species reactivity | human |
| technique(s) | immunohistochemistry: suitable (paraffin),western blot: suitable |
| isotype | IgG2bκ |
| NCBI accession no. | NP_003485 |
| UniProt accession no. | O75923 |
| shipped in | wet ice |
| target post-translational modification | unmodified |
| Gene Information | human ... DYSF(8291) |
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