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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/3245]

Catalog #

Size

20 ug
100 ug
100 ug

Price (USD)

199.00
429.00
429.00

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)

199.00
429.00
429.00
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Formalin-fixed, paraffin-embedded human Skeletal Muscle stained with Dystrophin Monospecific Mouse Monoclonal Antibody (DMD/3245).

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SDS-PAGE Analysis Purified Dystrophin Monospecific Mouse Monoclonal Antibody (DMD/3245). Confirmation of Purity and Integrity of Antibody.

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Analysis of Protein Array containing more than 19,000 full-length human proteins using Dystrophin Monospecific Mouse Monoclonal Antibody (DMD/3245).

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

ELISA, IHC-P         More Details

ELISA (For coating, order Ab without BSA)
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

A recombinant fragment (around aa 114-263) of human DMD protein (exact sequence is proprietary)

Cellular Localization

Cell Surface and Cytoplasmic

Species Reactivity

Human.

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. Blake DJ, et al. (2002) Function and genetics of dystrophin and dystrophin related proteins in muscle. Physiol Rev 82: 291-329.

Bioinformatics

  •  Entrez Gene ID: 1756
  •  Gene Symbol: DMD
  •  SwissProt: P11532
  •  Chromosome Location: Xp21.2
  •  Unigene: 495912
NBT03245