Anti-Dystrophin Antibody [JF1-022]

HUABIO

Anti-Dystrophin Antibody [JF1-022]

7

Western Blot, ImmunoHistoChemistry, ImmunoHistoChemistry Frozen, ImmunoHistoChemistry Paraffin

WB,IHC-P

Research Use Only

Monoclonal

JF1-022

1 mg/ml

Unconjugated

DMD

dystrophin

BMD, CMD3B, DXS142, DXS164, DXS206, DXS230, DXS239, DXS268, DXS269, DXS270, DXS272, MRX85, dystrophin, mutant dystrophin

Rabbit

IgG

Dystrophin

Dystrophin is a rod-shaped cytoplasmic protein, and a vital part of a protein complex that connects the cytoskeleton of a muscle fiber to the surrounding extracellular matrix through the cell membrane. This complex is variously known as the costamere or the dystrophin-associated protein complex (DAPC). Many muscle proteins, such as alpha-dystrobrevin, syncoilin, synemin, sarcoglycan, dystroglycan, and sarcospan, colocalize with dystrophin at the costamere. It has a molecular weight of 427 kDa. Dystrophin is coded for by the DMD gene - the largest known human gene, covering 2.4 megabases (0.08% of the human genome) at locus Xp21. The primary transcript in muscle measures about 2,100 kilobases and takes 16 hours to transcribe; the mature mRNA measures 14.0 kilobases. The 79-exon muscle transcript codes for a protein of 3685 amino acid residues. Spontaneous or inherited mutations in the dystrophin gene can cause different forms of muscular dystrophy, a disease characterized by progressive muscular wasting. The most common of these disorders caused by genetic defects in dystrophin is Duchenne muscular dystrophy.

Human, Mouse, Rat

Human,Mouse,Rat

-20°C,2°C to 8°C

41116161