Dystrophin Antibody / DMD

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]

Product Specifications

CAS Number

9007-83-4

Specifications

Immunohistochemistry (FFPE) : 1-2 µg/mL for 30 min at RT, Prediluted IHC only format: incubate for 30 min at RT (1)

UniProt

P11532

Host

Mouse

Reactivity

Human

Immunogen

Amino acids 114-263 from the human protein were used as the immunogen for the Dystrophin antibody.

Clonality

Monoclonal

Isotype

IgG1 k

Clone

DSTN-1

Applications

IHC-P

Purity

Protein G affinity chromatography

Format

Purified

Buffer

0.2 mg/ml in 1X PBS with 0.1 mg/ml BSA (US sourced) and 0.05% sodium azide

Reconstitution

Store the Dystrophin antibody at 2-8oC (with azide) or aliquot and store at -20oC or colder (without azide).

Limitations

This Dystrophin antibody is available for research use only.

Storage Conditions

Store the Dystrophin antibody at 2-8°C (with azide) or aliquot and store at -20°C or colder (without azide) .

Formulation

0.2 mg/mL in 1X PBS with 0.1 mg/mL BSA (US sourced) and 0.05% sodium azide

Location

Cell surface, cytoplasmic

Image Legend

IHC staining of FFPE skeletal muscle with Dystrophin antibody (clone DSTN-1) . HIER: boil tissue sections in pH 9 10mM Tris with 1mM EDTA for 10-20 min and allow to cool before testing.