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. This antibody is suitable for researchers interested in Muscular Dystrophies and Dystrophin-Glycoprotein Complexes and cardiovascular research.
应用类型
ELISA,Immunohistochemistry,Western Blot,
免疫原
Dystrophin antibody was produced in mice by repeated immunizations with recombinant protein corresponding to human dystrophin fragment.
来源宿主
Mouse
反应性
H. sapiens (Human); Leporidae (Rabbit); Mus musculus (Mouse); Rattus (Rat)
保存建议
Store vial at -20° C prior to opening. Aliquot contents and freeze at -20° C or below for extended storage. Avoid cycles of freezing and thawing. Centrifuge product if not completely clear after standing at room temperature. This product is stable for several weeks at 4° C as an undiluted liquid. Dilute only prior to immediate use.
