Product DescriptionMouse anti-Dystrophin Monoclonal Antibody (Unconjugated), suitable for WB, IHC-Frozen.
Application DetailsImmunohistochemistry (IHC) and Western Blotting (WB). A 1:200-1:1:000 is recommended for WB. Human dystrophin has a predicted length of 3,685 residues and MW of 427 kDa. A concentration of 1:200-1:6000 (ABC detection) is recommended to detect dystrophin fresh frozen, or acetone fixed, 2% PLP fixed sections so far tested. Not recommended for paraffin embedded tissues. Immunolocalization: Stains muscle membranes (no staining of Duchenne muscle membrane). Biosensis recommends optimal dilutions/concentrations should be determined by the end user.
SpecificityThe specificity of this antibody has been confirmed by WB and IHC against human samples.
Target Host SpeciesHuman
Species ReactivityChicken, Human, Mouse, Rabbit, Rat
Immunogen DescriptionRecombinant human dystrophin encoding amino-acids 816-1749
Reconstitution InstructionsSpin vial briefly before opening. Reconstitute in 1 mL of sterile-filtered PBS (pH 7.4) to achieve an antibody concentration of 100 µg/mL. Centrifuge to remove any insoluble material.
Storage InstructionsStore lyophilized antibody at 2-8°C. After reconstitution divide into aliquots and store at -20°C for up to six months. Store at 2-8°C short-term (up to 4 weeks). Avoid repetitive freeze/thaw cycles.
Batch NumberPlease see item label.
Expiration Date12 months after date of receipt (unopened vial).
Scientific BackgroundAnchors the extracellular matrix to the cytoskeleton via F-actin. Ligand for dystroglycan. Component of the dystrophin-associated glycoprotein complex which accumulates at the neuromuscular junction (NMJ) and at a variety of synapses in the peripheral and central nervous systems and has a structural function in stabilizing the sarcolemma. Also implicated in signaling events and synaptic transmission.
In muscle cells, sarcolemma localization requires the presence of ANK2, while localization to costameres requires the presence of ANK3. Localizes to neuromuscular junctions (NMJs). In adult muscle, NMJ localization depends upon ANK2 presence, but not in newborn animals.
Involvement in disease: Duchenne muscular dystrophy (DMD) 6 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease description: Most common form of muscular dystrophy; a sex-linked recessive disorder. It typically presents in boys aged 3 to 7 year as proximal muscle weakness causing waddling gait, toe-walking, lordosis, frequent falls, and difficulty in standing up and climbing up stairs. The pelvic girdle is affected first, then the shoulder girdle. Progression is steady and most patients are confined to a wheelchair by age of 10 or 12. Flexion contractures and scoliosis ultimately occur. About 50% of patients have a lower IQ than their genetic expectations would suggest. There is no treatment. Source: https://www.uniprot.org/uniprot/P11532
General ReferencesNguyen Thi Man, A.J. Cartwright, G.E. Morris, D. Love, J.F. Bloomfield and K.E. Davies. Monoclonal antibodies against defined regions of the muscular dystrophy protein, dystrophin. FEBS Letters, 262 (1990) 237-240.