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Home >> Antibodies >> Dystrophin (DMD) (Marker of Duchenne and Becker Muscular Dystrophy)

Dystrophin (DMD) (Marker of Duchenne and Becker Muscular Dystrophy)

Mouse Monoclonal Antibody [Clone DMD/6270]

Catalog #

Size

20 ug
100 ug
100 ug

Price (USD)

219.00
499.00
499.00

Formulation

Purified Ab with BSA and Azide at 200ug/ml
Purified Ab with BSA and Azide at 200ug/ml
Purified Ab WITHOUT BSA and Azide at 1.0mg/ml

Catalog No

Size

20 ug
100 ug
100 ug

Formulation

Purified Ab with BSA and Azide at 200ug/ml
Purified Ab with BSA and Azide at 200ug/ml
Purified Ab WITHOUT BSA and Azide at 1.0mg/ml

Price (USD)

219.00
499.00
499.00
img

Formalin-fixed, paraffin-embedded human heart muscle stained with Dystrophin Monospecific Mouse Monoclonal Antibody (DMD/6270). HIER: Tris/EDTA, pH9.0, 45min. 2: HRP-polymer, 30min. DAB, 5min.

Product Details

Synonyms

BMD; CMD3B; Duchenne muscular dystrophy (DMD); Dystrophin; Muscular dystrophy Duchenne and Becker types

Positive Control

Human skeletal muscle and heart muscle tissues (IHC).

Known Applications & Suggested Dilutions

IF, Western Blot, IHC-P         More Details

Immunofluorescence (1-2ug/ml)
Western Blot (1-2ug/ml)
Immunohistochemistry (Formalin-fixed) (1-2ug/ml for 30 minutes at RT)
(Staining of formalin-fixed tissues requires heating tissue sections in 10mM Tris with 1mM EDTA, pH 9.0, for 45 min at 95 °C followed by cooling at RT for 20 minutes)
Optimal dilution for a specific application should be determined.

Immunogen

Recombinant fragment (around aa1700-2300) of human DMD (exact sequence is proprietary).

Cellular Localization

Cell surface. Cytoplasm.

Species Reactivity

Human. Dog. Does not react with mouse, xenopus or chick.

Host / Ig Isotype

Mouse / IgG2a, kappa

Mol. Weight of Antigen

427kDa

Specificity & Comments

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.

Related Products



200ug/ml of Ab purified from Bioreactor Concentrate by Protein A/G. Prepared in 10mM PBS with 0.05% BSA & 0.05% azide. Also available WITHOUT BSA & azide at 1.0mg/ml.

Key References

  1. BlakeDJ, et al. (2002)Function and genetics of dystrophin and dystrophin related proteins in muscle.Physiol Rev82:291 329.

Bioinformatics

  •  Entrez Gene ID: 1756
  •  Gene Symbol: DMD
  •  SwissProt: P11532
  •  Chromosome Location: Xp21.2
  •  Unigene: 495912