MABT827
Anti-Dystrophin Antibody
mouse monoclonal, 2C6 (MANDYS106)
Synonim(y):
Dystrophin
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About This Item
Kod UNSPSC:
12352203
eCl@ss:
32160702
NACRES:
NA.41
Polecane produkty
Nazwa produktu
Anti-Dystrophin Antibody, clone 2C6 (MANDYS106), clone 2C6 (MANDYS106), from mouse
pochodzenie biologiczne
mouse
Poziom jakości
forma przeciwciała
purified antibody
rodzaj przeciwciała
primary antibodies
klon
2C6 (MANDYS106), monoclonal
reaktywność gatunkowa
human
metody
immunofluorescence: suitable
immunohistochemistry: suitable
western blot: suitable
izotyp
IgG2aκ
numer dostępu NCBI
numer dostępu UniProt
Warunki transportu
wet ice
docelowa modyfikacja potranslacyjna
unmodified
informacje o genach
human ... DMD(1756)
Opis ogólny
Dystrophin (UniProt P11532) is encoded by the DMD (also known as BMD, CMD3B, DXS142, DXS164, DXS206, DXS230, DXS239, DXS268, DXS269, DXS270, DXS272, MRX85) gene (Gene ID 1756) in human. Dystrophin is localized to the inner part of the muscle fiber cell membrane (sarcolemma) and plays an important role in stabilizing the muscle fiber against the mechanical forces of muscle contraction by providing a shock-absorbing connection between the cytoskeleton and the extracellular matrix. Duchenne muscular dystrophy (DMD) is caused by gene mutations that disrupt the open reading frame (ORF) and prevent the full translation of dystrophin. ORF restoration by exon skipping using antisense oligonucleotides is designed to transform the DMD phenotype to that of the milder disorder, Becker muscular dystrophy (BMD), which is typically caused by in-frame dystrophin deletions that allow the production of an internally deleted, but partially functional dystrophin.
Specyficzność
Detects dystrophin spliced isoforms 1-4, but not isoforms 5-10, or utrophin. Positive muscle membrane staining of tissue samples from healthy donors, reduced staining of Becker muscular dystrophy (BMD) biopsies, and no staining is seen with Duchenne muscular dystrophy (DMD) biopsy samples.
Immunogen
Epitope: Exon 43-coded pectrin-like repeat 16 region
TrpE-tagged recombinant protein corresponding to the Exon 43-coded pectrin-like repeat 16 region of human Dystrophin.
Zastosowanie
Anti-Dystrophin Antibody, clone 2C6 (MANDYS106) is an antibody against Dystrophin for use in Immunohistochemistry, Immunofluorescence, Western Blotting.
Immunohistochemistry Analysis: A represenative lot stained sarcolemma of muscle fiber cells in tissue samples from healthy donors, while much reduced staining was observed in biopsy samples from patients with Becker muscular dystrophy (BMD) and no staining is seen with Duchenne muscular dystrophy (DMD) biopsy samples (Anthony, K., et al. (2011). Brain. 134(Pt 12):3547-3559).
Immunofluorescence Analysis: A represenative lot was employed together with a spectrin antibody in dual immunofluorescent sarcolemma staining for assessing dystrophin levels of muscle fiber cells in muscle biopsies from healthy donors and Becker muscular dystrophy (BMD) patients (Beekman, C., et al. (2014). PLoS One. 9(9):e107494).
Immunofluorescence Analysis: A represenative lot was employed together with a spectrin antibody in dual immunofluorescent sarcolemma staining for assessing dystrophin levels of muscle fiber cells in muscle biopsies from healthy donors and Becker muscular dystrophy (BMD) patients (Beekman, C., et al. (2014). PLoS One. 9(9):e107494).
Research Category
Cell Structure
Cell Structure
Research Sub Category
Adhesion (CAMs)
Adhesion (CAMs)
Jakość
Evaluated by Immunohistochemistry in human skeletal muscle myocytes.
Immunohistochemistry Analysis: A 1:50 dilution of this antibody detected Dystrophin in human skeletal muscle myocytes.
Immunohistochemistry Analysis: A 1:50 dilution of this antibody detected Dystrophin in human skeletal muscle myocytes.
Opis wartości docelowych
~427 kDa observed
Postać fizyczna
Format: Purified
Protein G Purified
Purified mouse monoclonal IgG2aκ antibody in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
Przechowywanie i stabilność
Stable for 1 year at 2-8°C from date of receipt.
Inne uwagi
Concentration: Please refer to lot specific datasheet.
Oświadczenie o zrzeczeniu się odpowiedzialności
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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polecane
Kod klasy składowania
12 - Non Combustible Liquids
Klasa zagrożenia wodnego (WGK)
WGK 1
Temperatura zapłonu (°F)
Not applicable
Temperatura zapłonu (°C)
Not applicable
Certyfikaty analizy (CoA)
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Menglong Chen et al.
Genome medicine, 13(1), 57-57 (2021-04-14)
Mutations in the DMD gene encoding dystrophin-a critical structural element in muscle cells-cause Duchenne muscular dystrophy (DMD), which is the most common fatal genetic disease. Clustered regularly interspaced short palindromic repeat (CRISPR)-mediated gene editing is a promising strategy for permanently
Diane E Frank et al.
Neurology, 94(21), e2270-e2282 (2020-03-07)
To report safety, pharmacokinetics, exon 53 skipping, and dystrophin expression in golodirsen-treated patients with Duchenne muscular dystrophy (DMD) amenable to exon 53 skipping. Part 1 was a randomized, double-blind, placebo-controlled, 12-week dose titration of once-weekly golodirsen; part 2 is an
Dystrophin quantification and clinical correlations in Becker muscular dystrophy: implications for clinical trials.
Anthony, K; Cirak, S; Torelli, S; Tasca, G; Feng, L; Arechavala-Gomeza, V; Armaroli et al.
Brain null
Valentina Sardone et al.
PloS one, 13(3), e0194540-e0194540 (2018-03-27)
Clinical trials using strategies aimed at inducing dystrophin expression in Duchenne muscular dystrophy (DMD) are underway or at advanced planning stage, including splice switching antisense oligonucleotides (AON), drugs to induce read-through of nonsense mutations and viral mediated gene therapy. In
Chantal Beekman et al.
PloS one, 9(9), e107494-e107494 (2014-09-23)
Duchenne muscular dystrophy (DMD) is characterized by the absence or reduced levels of dystrophin expression on the inner surface of the sarcolemmal membrane of muscle fibers. Clinical development of therapeutic approaches aiming to increase dystrophin levels requires sensitive and reproducible
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