Clone | DYS-48 |
Size | 100μg/vial |
Form | lyophilized |
Ig type | mouse IgG2b |
Immunogen | Recombinant human dystrophin fragment. |
Application | WB; IHC-F |
Cross Reactivity | Human, mouse, rat, chicken. |
Reconstitution | 1.2% sodium acetate or neutral PBS. If 1ml of PBS is used. |
The antibody concentration will be 100μg/ml. | |
Storage | At -20˚C for one year. After reconstitution, at 4˚C for one month. |
It can also be aliquotted and stored frozen at -20˚C for a longer time. | |
Avoid repeated freezing and thawing. | |
Dystrophin(DMD) gene has 79 exons spanning at least 2,300 kb (2.3 Mb). The C terminus of the dystrophin protein is encoded by a highly conserved, alternatively spliced region of the gene. beta-dystroglycan binding activity is expressed by the dystrophin fragment spanning amino acids 3026-3345 containing the ZZ domain. DMD transcript is formed by at least 60 exons; the first half of the transcript is formed by a minimum of 33 exons spanning nearly 1000 kb, and the remaining portion has at least 27 exons that may spread over a similar distance. Dystrophin gene is expressed at a higher level in primary cultures of neuronal cells than in astro-glial cells derived from adult mouse brain. overexpression of dystrophin prevents the development of the abnormal mechanical properties associated with dystrophic muscle without causing deleterious side effects. |