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Dystrophin (DMD) (AA 3200-3684) antibody

Details for Product No. ABIN145213
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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
(29), (2), (1), (1), (1), (1), (1), (1)
Reactivity
Human
(75), (33), (32), (13), (12), (11), (10), (2), (1), (1)
Host
Mouse
(49), (43)
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), Immunohistochemistry (Frozen Sections) (IHC (fro)), Western Blotting (WB)
(49), (41), (17), (16), (12), (10), (9), (8), (6), (5), (2), (1)
Pubmed 7 references available
Quantity 0.1 mL
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Catalog No. ABIN145213
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Immunogen Fusion protein, corresponding to amino acids 3200-3684 of Human Dystrophin.
Clone MANDRA1
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).In Western blots, the antibody also stains the 70-75 kDa protein known as Apo-Dystrophin-1 or Dp71. This is detected in the brain as well as in lymphoblastoid cells, cultures of brain astroglial and neuronal cells, liver and Hep G2 cells (human hepatoma).
Cross-Reactivity Mouse (Murine), Fish, Rat (Rattus)
Characteristics Cellular Localization: Cell Membrane
Purification Ascites.
Alternative Name Dystrophin / DMD
Background The C-terminal domain of the human dystrophin molecule (a. A. residues 3558-3684) is present in normal muscle tissue. It is also present in nearly all Becker muscular dystrophies, but is absent in cases of Duchenne muscular dystrophies and in the dystrophic mouse (mdx) (ref 1-3).
Synonyms: Dystrophin, DXS269, DXS272, 1756, DXS268, DXS239, DXS270, DXS142, 300376, DXS206, P11532, DXS230, DMD, DXS164, BMD
Application Notes ELISA: Use at an assay dependant dilution. See Nyugen thi Man et al (1992) and Morris G.E et al (1993) IF: Use at a dilution of 1/100 for freshly dissected and frozen human or animal muscle tissue. IHC-Fr: Use at an assay dependant dilution. Immunohistochemical staining of muscle tissue results in a clear labeling confined to the periphery (plasma membrane) of normal striated muscle fibers. WB: Use at an assay dependant dilution. Not tested in other applications. Optimal dilutions/concentrations should be determined by the end user.
Restrictions For Research Use only
Format Liquid
Buffer Ascitic fluid with 15mM sodium azide
Preservative Sodium azide
Precaution of Use This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Handling Advice Avoid freeze-thaw cycles.
Storage 4 °C
Storage Comment Keep as concentrated solution. Store at 4°C short term. For extended storage aliquot and store at -20°C or below.
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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