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MilliporeSigma™ Mouse anti-Dysferlin/DYSF, Clone: 10H3.1,

Catalog No. MABC974MI Shop All MilliporeSigma Products
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MABC974MI 100 μg
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Mouse Monoclonal Antibody

Specifically detects Dysferlin/DYSF clone: 10H3.1 in Human samples, and it is validated for Immunohistochemistry (Paraffin), Western Blotting

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).
TRUSTED_SUSTAINABILITY

Specifications

Antigen Dysferlin/DYSF
Applications Immunohistochemistry (Paraffin), Western Blot
Classification Monoclonal
Clone 10H3.1
Formulation Purified mouse monoclonal IgG2bκ antibody in buffer containing 0.1M Tris-Glycine (pH 7.4), 150mM NaCl with 0.05% sodium azide.
Gene Accession No. O75923
Gene Symbols DYSF; FER1L1
Host Species Mouse
Immunogen GST-tagged recombinant protein corresponding to the N-terminal of human Dysferlin/DYSF.
Purification Method Protein G Purified
Quantity 100 μg
Regulatory Status RUO
Research Discipline Apoptosis, Cancer
Primary or Secondary Primary
Gene ID (Entrez) NP_003485
Target Species Human
Content And Storage Stable for one year at 2°C to 8°C from date of receipt.
Form Purified
Isotype IgG2b κ
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