|Reactivity||Human, Mouse, Rat|
|Calculated MW||426750 Da|
|Application & Usage||Western blotting (0.5-4 µg/ml). However, the optimal conditions should be determined individually. The antibody detects ~60 and 130 kDa of Dystrophin in samples from human, mouse and rat origins. Reactivity to other species has not been determined.|
|Other Names||DMD , DXS272 , DXS269 , DXS230 , Dystrophin, DXS268 , DXS270 , DXS239, DXS142 , CMD3B , DXS206 , DXS164 , BMD|
|Formulation||100 µg (0.5 mg/ml) antigen affinity purified rabbit anti-Dystrophin polyclonal antibody in phosphate buffered saline (PBS), pH 7.2, containing 30% glycerol and 0.01% Thimerosal.|
|Handling||The antibody solution should be gently mixed before use.|
|Reconstitution & Storage||-20 °C|
|Precautions||Dystrophin Antibody is for research use only and not for use in diagnostic or therapeutic procedures.|
|Function||Anchors 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.|
|Cellular Location||Cell membrane, sarcolemma; Peripheral membrane protein; Cytoplasmic side. Cytoplasm, cytoskeleton. Cell junction, synapse, postsynaptic cell membrane Note=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 the presence of ANK2 (By similarity).|
|Tissue Location||Expressed in muscle fibers accumulating in the costameres of myoplasm at the sarcolemma. Expressed in brain, muscle, kidney, lung and testis. Isoform 5 is expressed in heart, brain, liver, testis and hepatoma cells. Most tissues contain transcripts of multiple isoforms, however only isoform 5 is detected in heart and liver.|
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Dystrophin is one of the actin-binding proteins that are involved in anchoring the cytoskeleton to the plasma membrane. Dystrophin expression is found in muscle brain tissues, where it is located to the inner surface of the plasma membrane. It is s µggested that alternative splicing of the caboxy terminus allows dystrophin to interact with a variety of proteins. Loss of dystrophin-associated proteins in Duchenne afflicted muscle is due to the absence of dystrophin rather than to muscle degradation and lack of dystrophin results in the loss of linkage between the cytoskeleton and extracellular matrix.
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