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 Ab without BSA for coating, Immunohistochemistry (FFPE) : 1-2 µg/mL for 30 minutes at RT

UniProt

P11532

Host

Mouse

Reactivity

Human

Immunogen

A portion of amino acids 114-263 from the human protein was used as the immunogen for the Dystrophin antibody.

Clonality

Monoclonal

Isotype

IgG κ

Clone

DMD/3677

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), 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

Applications Notes

Optimal dilution of the Dystrophin antibody should be determined by the researcher.

Location

Cell surface, cytoplasmic

Image Legend

IHC staining of FFPE human cardiac muscle with Dystrophin antibody (clone DMD/3677) . HIER: boil tissue sections in pH 9 10mM Tris with 1mM EDTA for 20 min and allow to cool before testing.