|Applications||ICC/IF, IHC, IHC-Fr, IHC-P, WB|
|Application Note||IHC-P: Use at a dilution of 1/100 for 10 minutes at RT. Staining of formalin-fixed tissues requires boiling tissue sections in 1mM EDTA, pH 8.0 for 10 minutes followed by cooling at RT for 20 minutes. WB: Use at a concentration of 1 μg/ml for 1 hour. Predicted molecular weight: 427 kDa. Optimal dilutions/concentrations should be determined by the end user.|
|Positive Controls||Skeletal muscle|
(Please refer to the vial label for the specific concentration)|
|Purification||Immunogen affinity purified|
|Product Description||Rabbit polyclonal to Dystrophin|
|Background||The dystrophin gene is the largest gene found in nature, measuring 2.4 Mb. The gene was identified through a positional cloning approach, targeted at the isolation of the gene responsible for Duchenne (DMD) and Becker (BMD) Muscular Dystrophies. DMD is a recessive, fatal, X linked disorder occurring at a frequency of about 1 in 3,500 new born males. BMD is a milder allelic form. In general, DMD patients carry mutations which cause premature translation termination (nonsense or frame shift mutations), while in BMD patients dystrophin is reduced either in molecular weight (derived from in frame deletions) or in expression level. The dystrophin gene is highly complex, containing at least eight independent, tissue specific promoters and two polyA addition sites. Furthermore, dystrophin RNA is differentially spliced, producing a range of different transcripts, encoding a large set of protein isoforms. Dystrophin (as encoded by the Dp427 transcripts) is a large, rod like cytoskeletal protein which is found at the inner surface of muscle fibers. Dystrophin is part of the dystrophin glycoprotein complex (DGC), which bridges the inner cytoskeleton (F actin) and the extracellular matrix.|
|Synonyms||Dystrophin, DXS269, DXS272, 1756, DXS268, DXS239, DXS270, DXS142, 300376, DXS206, P11532, DXS230, DMD, DXS164, BMD|
|Cellular Localization||Cell Membrane|
|Immunogen||A synthetic peptide corresponding to C-terminal of human Dystrophin.|
|Species Reactivity||Human, Mouse, Rat|
|Predicted Cross Reactivity species Predicted cross-reactivity:|
Predicted cross-reactivity is based on sequence homology, with greater than 80% immunogen sequence identity considered positive.
Please note that we are only able to guarantee products to work in applications and species in which they have been tested.
|Predict Reactivity Note||Pig(94%)|