Monoclonal Anti-Dystrophin

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SPECIFICATIONS

Price

200.00 USD

Catalog Number

MA1037

Size

100ug/vial

Applications

IHC-Fr, WB

Reactivities

Hum, Mouse, Rat, Rabbit

Form

Lyophilized

Format

1.2% sodium acetate or neutral PBS. If 1ml of PBS is used, the antibody concentration will be 100ug/ml.

Gene Id

Human(1756), Mouse(13405), Rat(24907)

Swiss Prot

Human(P11532), Mouse(P11531), Rat(P11530)

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.

Target Synonym

BMD; CMD3B; DXS142; DXS164; DXS206; DXS230; DXS239; DXS268; DXS269; DXS270; DXS272\n

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.