Dystrophin (DMD) (AA 3200-3684) antibody

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Antigen
Synonyms
DMD, Dmd, MGC83347, DKFZp459C1629, dmd, MGC79631, DKFZp468A1620, Dp71, cb664, zfDYS, im:6911785, zgc:110165, CG17750, CG31175, CG34157, CG7240, CG7243, CG7344, DLP, DLP1, DLP186, DLP2, DLP3, DmDLP, Dm ... show more
DMD, Dmd, MGC83347, DKFZp459C1629, dmd, MGC79631, DKFZp468A1620, Dp71, cb664, zfDYS, im:6911785, zgc:110165, CG17750, CG31175, CG34157, CG7240, CG7243, CG7344, DLP, DLP1, DLP186, DLP2, DLP3, DmDLP, DmDYS, Dmel\\CG34157, Dp117, Dp186, Dp205, GI3046716, IDLP, det, dmDLP, dmDp186, dmDys, dys, BMD, CMD3B, DXS142, DXS164, DXS206, DXS230, DXS239, DXS268, DXS269, DXS270, DXS272, DNADMD1, RATDMD, DXSmh7, DXSmh9, Dp427, mdx, pke show less
Epitope
AA 3200-3684
(26), (2), (1), (1), (1), (1), (1), (1), (1)
Reactivity
Fish, Human, Mouse (Murine), Rat (Rattus)
(78), (35), (34), (13), (12), (11), (11), (2), (1), (1)
Host
Mouse
(51), (44)
Clonality (Clone)
Monoclonal ()
Conjugate
Un-conjugated
(1), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1)
Application
ELISA, Immunocytochemistry (ICC), Immunofluorescence (IF), Microarray (MA)
(49), (46), (18), (17), (14), (10), (9), (8), (7), (7), (6), (2), (1)
Pubmed 7 references available
Quantity 0.1 mL
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Catalog No. ABIN267904
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Immunogen Fusion protein, corresponding to amino acids 3200-3684 of Human Dystrophin.
Clone MANDRA1
Isotype IgG1
Specificity The antibody recognizes an epitope located on the 128 amino acids at the end of the C-terminal domain of the human dystrophin molecule (amino acid residues 3558-3684).
Cross-Reactivity (Details) Not yet tested in other species.
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 Symbol: DMD
Gene ID 1756, 13405, 24907
Application Notes ELISA: See Nyugen thi Man et al (1992) and Morris G.E et al (1993) IF: Use freshly dissected and frozen human or animal muscle tissue. Immunohistochemical staining of muscle tissue results in a clear labeling confined to the periphery (plasma membrane) of normal striated muscle fibers.
Recommended dilutions: ELISA 1:100-1:2000, Immunocytochemistry/Immunofluorescence 1:100, Microarray
Restrictions For Research Use only
Format Liquid
Preservative Sodium azide
Precaution of Use WARNING: Reagents contain sodium azide. Sodium azide is very toxic if ingested or inhaled. Avoid contact with skin, eyes, or clothing. Wear eye or face protection when handling. If skin or eye contact occurs, wash with copious amounts of water. If ingested or inhaled, contact a physician immediately. Sodium azide yields toxic hydrazoic acid under acidic conditions. Dilute azide-containing compounds in running water before discarding to avoid accumulation of potentially explosive deposits in lead or copper plumbing.
Handling Advice Avoid freeze-thaw cycles
Storage 4 °C
Storage Comment 4 °C short term. Aliquot and store at -20 °C long term.
General 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).

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