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Products Monoclonal Anti-Dystrophin
| Catalogue number: | MA1037 |
| Price: | $200.00 |
| Reactivities: | Human, Mouse, Rat, Rabbit |
| Applications: | Immunohistochemistry - frozen, Western Blot |
| Size: | 100ug/vial |
| Gene: | Human(1756), Mouse(13405), Rat(24907) |
| Swiss prot: | Human(P11532), Mouse(P11531), Rat(P11530) |
| Form: | Lyophilized |
| Format: | 1.2% sodium acetate or neutral PBS. If 1ml of PBS is used, the antibody concentration will be 100ug/ml. |
| Target synonym: | BMD; CMD3B; DXS142; DXS164; DXS206; DXS230; DXS239; DXS268; DXS269; DXS270; DXS272 |
| Storage temp: | At -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for a longer time. Avoid repeated freezing and thawing. |
| Primary protein: | Dystrophin |
| Scientific background: | 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. |
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