The DMD 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. (RefSeq)
Formulation
1 mg/ml in 1X PBS; BSA free, sodium azide free
Host
Mouse
Immunogen Region
Amino acids 114-263 from the human protein were used as the immunogen for the Dystrophin antibody.
Isotype
Mouse IgG1, kappa
Species Reactivity
Human
Note
Optimal dilution of the Dystrophin antibody should be determined by the researcher.1. The prediluted format is supplied in a dropper bottle and is optimized for use in IHC. After epitope retrieval step (if required), drip mAb solution onto the tissue section and incubate at RT for 30 min.
Uniprot
P11532
Format
Purified
Purity
Protein G affinity chromatography
Storage
Store the Dystrophin antibody at 2-8oC (with azide) or aliquot and store at -20oC or colder (without azide).
Applications
IHC-P
Description
Immunohistochemistry (FFPE): 1-2ug/ml for 30 min at RT,Prediluted IHC only format: incubate for 30 min at RT (1)