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Details for Product No. ABIN267904

Dystrophin (DMD) antibody

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BMD, CMD3B, DXS142, DXS164, DXS206, DXS230, DXS239, DXS268, DXS269, DXS270, DXS272, DXSmh7, DXSmh9, Dp427, Dp71, dys, mdx, pke, RATDMD, DNADMD1, DMD, Dmd, MGC83347, DKFZp459C1629, dmd, MGC79631, DKFZp ... show more
»Alternatives Fish, Human, Mouse (Murine), Rat (Rattus)
»Alternatives Mouse
Clonality (Clone) Monoclonal ()
»Alternatives Un-conjugated
»Alternatives ELISA, Immunocytochemistry (ICC), Immunofluorescence (IF)
Pubmed 7 references available
Catalog no. ABIN267904
Quantity 0.1 mL
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Immunogen Fusion protein, corresponding to amino acids 3200-3684 of Human Dystrophin.
Isotype IgG1
Specificity The antibody is specific to dystrophin and does not react with alpha-actinin and utrophin,an autosomal homologue of dystrophin, also called dystrophin-related protein (DRP).InWestern blots, the antibody also stains the 70-75 kDa protein known asApo-Dystrophin-1 or Dp71. This is detected in the brain as well as in lymphoblastoidcells, cultures of brain astroglial and neuronal cells, liver and Hep G2 cells (humanhepatoma). Species Reactivity: Cross-reacts with Fish, Human, Mouse and Rat. Not yet tested in other species. Localization: Cell Membrane.
Cross-Reactivity Human, Mouse (Murine), Rat (Rattus)
Purification Ascites
Alternative Name Dystrophin / DMD
Background Dystrophin is a muscle membrane protein (427 kDa) which is absent, reduced or alteredas a result of mutation in Duchenne and Becker muscular dystrophies (DMD/BMD) or itshomologue in the mouse.8 Severe DMD is associated with a marked dystrophindeficiency whereas patients with the milder form of BMD show less pronouncedabnormalities of protein expression. Because abnormalities in the protein expressionoccur specifically in patients with these types of muscular dystrophy, dystrophin analysismay be used to distinguish these conditions from other neuromuscular diseases.Predictions from the sequence suggest a structural protein on the inner face of themembrane, consisting of a 25-repeat, rod-like triple-helical domain separating anN-terminal actin-binding domain from two C-terminal domains, one of which is rich incysteine.9 The large size of dystrophin and its low abundance (<0.01% of the totalmuscle protein) are a hindrance to the isolation of intact, native protein forstructure/function studies. Monoclonal antibodies against defined regions10 ofdystrophin provide a means for studying its structure and function, interactions withother proteins and the nature of the partial gene products produced in some patientscarrying deletions in the dystrophin gene. The antibodies are useful in the prenatal orpost-abortion diagnosis of muscular dystrophy carriers by immunohistologicalanalyses.11. Alternate Names: Anti-Apo dystrophin antibody, Anti-BMD antibody, Anti-CMD3B antibody, Anti-DMDantibody, Anti-DXS142 antibody, Anti-DXS164 antibody, Anti-DXS206 antibody,Anti-DXS230 antibody, Anti-DXS239 antibody, Anti-DXS268 antibody, Anti-DXS269antibody, Anti-DXS270 antibody, Anti-DXS272 antibody, Anti-Muscular dystrophyDuchenne and Becker types antibody. Related Diseases: Muscular Dystrophy
Gene ID 1756, 13405, 24907
Application Notes Immunohistochemistry-Frozen 1/100
Restrictions For Research Use only
Format Liquid
Concentration 7.5 mg/ml
Buffer Preservative: 0.1% sodium azide.
Preservative Sodium azide
Storage 4 °C
Nguyen, Ginjaar, van Ommen et al.: "Monoclonal antibodies for dystrophin analysis. Epitope mapping and improved binding to SDS-treated muscle sections." in: The Biochemical journal, Vol. 288 ( Pt 2), pp. 663-8, 1993 (PubMed).

Lederfein, Levy, Augier et al.: "A 71-kilodalton protein is a major product of the Duchenne muscular dystrophy gene in brain and other nonmuscle tissues." in: Proceedings of the National Academy of Sciences of the United States of America, Vol. 89, Issue 12, pp. 5346-50, 1992 (PubMed).

Hugnot, Gilgenkrantz, Vincent et al.: "Distal transcript of the dystrophin gene initiated from an alternative first exon and encoding a 75-kDa protein widely distributed in nonmuscle tissues." in: Proceedings of the National Academy of Sciences of the United States of America, Vol. 89, Issue 16, pp. 7506-10, 1992 (PubMed).

Gussoni, Pavlath, Lanctot et al.: "Normal dystrophin transcripts detected in Duchenne muscular dystrophy patients after myoblast transplantation." in: Nature, Vol. 356, Issue 6368, pp. 435-8, 1992 (PubMed).

Nguyen thi Man, Cartwright, Morris et al.: "Monoclonal antibodies against defined regions of the muscular dystrophy protein, dystrophin." in: FEBS letters, Vol. 262, Issue 2, pp. 237-40, 1990 (PubMed).

Ellis, Man, Morris et al.: "Specificity of dystrophin analysis improved with monoclonal antibodies." in: Lancet, Vol. 336, Issue 8719, pp. 881-2, 1990 (PubMed).

Morris, Ellis, Nguyen: "A quantitative ELISA for dystrophin." in: Journal of immunological methods, Vol. 161, Issue 1, pp. 23-8, 1993 (PubMed).

Alternatives for antigen "Dystrophin (DMD)", type "Antibodies"
Hosts (49), (33), (1)
Reactivities (64), (36), (26), (16), (14), (13), (11), (3), (2), (1), (1), (1), (1), (1)
Applications (44), (38), (19), (14), (14), (10), (10), (6), (5), (3), (3), (2), (1)
Conjugates (1), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1)
Epitopes (19), (3), (1), (1), (1), (1)