Duchenne Muscular dystrophy (DMD) is the most common of the muscular dystrophies resulting in progressive muscular wasting and death. Dystrophin is the 427kD protein product of the DMD gene located on the X chromosome at position Xp21. Abnormalities in protein expression occur in patients with DMD/BMD and dystrophin analysis may be used to distinguish these conditions from other neuromuscular diseases. Severe Duchenne muscular dystrophy is associated with a marked dystrophin deficiency, whereas patients with the milder form of Becker muscular dystrophy show less pronounced abnormalities of protein expression. The immunolabeling patterns for DYS1, DYS2 and DYS3 are similar; however, the use of all three antibodies is recommended to avoid the possibility of occasional false negative results.
Dystrophin Antibodies are recommended for the detection of specific antigens of interest in normal and abnormal tissues, as an adjunct to conventional histopathology using non-immunologic histochemical stains.