Dystrophin Antibody / DMD

Dystrophin-glycoprotein complex (DGC) connects the F-Actin cytoskeleton on the inner surface of muscle fibers to the surrounding extracellular matrix, through the cell membrane interface. A deficiency in this protein contributes to Duchenne (DMD) and Becker (BMD) muscular dystrophies. The human dystrophin gene measures 2.4 megabases, has more than 80 exons, produces a 14 kb mRNA and contains at least 8 independent tissue-specific promoters and 2 poly A sites. The dystrophin mRNA can undergo differential splicing and produce a range of transcripts that encode a large set of proteins. Dystrophin represents approximately 0.002% of total striated muscle protein and localizes to triadic junctions in skeletal muscle, where it is thought to influence calcium ion homeostasis and force transmission.

Product Specifications

CAS Number

9007-83-4

Specifications

ELISA (order BSA/sodium azide-free format for coating), Immunohistochemistry (FFPE) : 1-2 µg/mL for 30 min at RT

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

DMD/3241

Applications

ELISA, 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 DMD/3241) . HIER: boil tissue sections in pH6, 10mM citrate buffer, for 10-20 min and allow to cool before testing.