Description
Product Usage Information
Western Blotting: 1:1000
Immunohistochemistry (Paraffin): 1:50 - 1:200
Storage
Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/mL BSA, 50% glycerol, and less than 0.02% sodium azide. Store at -20°C. Do not aliquot the antibody.
Protocol
Available protocols: Western Blotting, Immunohistochemistry (Paraffin)
Specificity / Sensitivity
Dystrophin (E4F3S) Rabbit Monoclonal Antibody recognizes endogenous levels of total dystrophin protein.
Species Reactivity: Human, Rat
Source / Purification
Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues near the carboxy terminus of human dystrophin protein.
Background
Dystrophin is a large, multi-domain protein that plays a critical role in muscle function that anchors the extracellular matrix to the cytoskeleton via F-actin (1). The protein is encoded in the (Duchenne muscular dystrophy) gene located on the X chromosome. Dystrophin is part of a larger complex called the dystrophin-associated glycoprotein complex (DGC), which accumulates at the neuromuscular junction and synapses (2). The DGC also plays a structural function in muscle fibers by stabilizing the sarcolemma and protecting muscle cell membranes during muscle contractions (3). Mutations in the gene, which is the largest known gene in humans, are genetically linked to Duchenne muscular dystrophy and Becker muscular dystrophy. Dystrophin may play a role in the proper function of central nervous system synapses, as neurological disorders are observed in Duchenne muscular dystrophy patients (4). Moreover, mutations in the gene are linked to Alzheimer's Disease, suggesting a role in neurodegeneration (5).
Alternate Names
BMD; CMD3B; DMD; DXS142; DXS164; DXS206; DXS230; DXS239; DXS268; DXS269; DXS270; DXS272; Dystrophin; MRX85; truncated dystrophin
