Mouse monoclonal antibody raised against partial recombinant DMD.
Recombinant protein corresponding to a region of the rod domain of human DMD.
Immunohistochemistry (1:10-1:20) The optimal working dilution should be determined by the end user.
Lyophilized from tissue culture supernatant (0.09% sodium azide)
Store at 4°C on dry atmosphere. After reconstitution with steril distilled water, Store at 4°C for at least two months. For long term storage store at -20°C or below. Aliquot to avoid repeated freezing and thawing.
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
The dystrophin gene is the largest gene found in nature, measuring 2.4 Mb. The gene was identified through a positional cloning approach, targeted at the isolation of the gene responsible for Duchenne (DMD) and Becker (BMD) Muscular Dystrophies. DMD is a recessive, fatal, X-linked disorder occurring at a frequency of about 1 in 3,500 new-born males. BMD is a milder allelic form. In general, DMD patients carry mutations which cause premature translation termination (nonsense or frame shift mutations), while in BMD patients dystrophin is reduced either in molecular weight (derived from in-frame deletions) or in expression level. The dystrophin gene is highly complex, containing at least eight independent, tissue-specific promoters and two polyA-addition sites. Furthermore, dystrophin RNA is differentially spliced, producing a range of different transcripts, encoding a large set of protein isoforms. Dystrophin (as encoded by the Dp427 transcripts) is a large, rod-like cytoskeletal protein which is found at the inner surface of muscle fibers. Dystrophin is part of the dystrophin-glycoprotein complex (DGC), which bridges the inner cytoskeleton (F-actin) and the extra-cellular matrix. [provided by RefSeq
Duchenne muscular dystrophy protein,OTTHUMP00000023117,OTTHUMP00000023124,OTTHUMP00000023125,OTTHUMP00000023126,muscular dystrophy, Duchenne and Becker types