*Optimal dilutions/concentrations should be determined by the researcher.
Freeze specimen tissue blocks in isopentane chilled in liquid nitrogen.
Not tested in other applications.
This antibody reacts strongly with the amino terminal domain (between amino acids 321 and 494) of human dystrophin. Patient immunoreactivity indicates epitope is near exons 10 to 12. Epitope mapping suggests that sequences from amino acids 308 to 351 are involved in antibody binding. This region spans the junction of exons 9 and 10 and the epitope recognised may be part of a hinge region joining the amino domain to the central rod domain. No reactivity with DMD/BMD patients deleted for exons 10 to 12.
We do not recommend use of this product for Mouse,Rat,Rabbit,Dog,Hamster,Chicken,Pig samples.
Tissue culture supernatant
Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4ºC. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.
>4 μg/ml (Please refer to the vial label for the specific concentration.)
Fusion protein containing amino acids 67 to 713.
For laboratory use only. Not for any clinical, therapeutic, or diagnostic use in humans or animals. Not for animal or human consumption.
dystrophin , BMD , CMD3B , DXS142 , DXS164 , DXS206 , DXS230 , DXS239 , DXS268 , DXS269 , DXS270 , DXS272 , MRX85
This gene spans a genomic range of greater than 2 Mb and encodes a large protein containing an N-terminal actin-binding domain and multiple spectrin repeats. The encoded protein forms a component of the dystrophin-glycoprotein complex (DGC), which bridges the inner cytoskeleton and the extracellular matrix. Deletions, duplications, and point mutations at this gene locus may cause Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), or cardiomyopathy. Alternative promoter usage and alternative splicing result in numerous distinct transcript variants and protein isoforms for this gene. [provided by RefSeq, Dec 2016]