MABT827
Anti-Dystrophin Antibody, clone 2C6 (MANDYS106)
clone 2C6 (MANDYS106), from mouse
Synonyma:
Dystrophin
Přihlásitk zobrazení cen stanovených pro organizaci a smluvních cen
About This Item
UNSPSC Code:
12352203
eCl@ss:
32160702
NACRES:
NA.41
Doporučené produkty
biological source
mouse
Quality Level
antibody form
purified antibody
antibody product type
primary antibodies
clone
2C6 (MANDYS106), monoclonal
species reactivity
human
technique(s)
immunofluorescence: suitable
immunohistochemistry: suitable
western blot: suitable
isotype
IgG2aκ
NCBI accession no.
UniProt accession no.
shipped in
wet ice
target post-translational modification
unmodified
Gene Information
human ... DMD(1756)
Související kategorie
General description
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.
Specificity
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.
Application
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)
Quality
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.
Target description
~427 kDa observed
Physical form
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.
Storage and Stability
Stable for 1 year at 2-8°C from date of receipt.
Other Notes
Concentration: Please refer to lot specific datasheet.
Disclaimer
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.
Ještě jste nenalezli správný produkt?
Vyzkoušejte náš produkt Nástroj pro výběr produktů.
recommended
Č. produktu
Popis
Stanovení ceny
Storage Class
12 - Non Combustible Liquids
wgk_germany
WGK 1
flash_point_f
Not applicable
flash_point_c
Not applicable
Osvědčení o analýze (COA)
Vyhledejte osvědčení Osvědčení o analýze (COA) zadáním čísla šarže/dávky těchto produktů. Čísla šarže a dávky lze nalézt na štítku produktu za slovy „Lot“ nebo „Batch“.
Již tento produkt vlastníte?
Dokumenty související s produkty, které jste v minulosti zakoupili, byly za účelem usnadnění shromážděny ve vaší Knihovně dokumentů.
Zákazníci si také prohlíželi
Increased Dystrophin Production With Golodirsen in Patients With Duchenne Muscular Dystrophy.
Neurology, 100(19), 936-936 (2023-05-09)
Jaemin Kim et al.
Stem cell research, 23, 87-94 (2017-07-22)
Currently, the most efficient and promising approach for generating large numbers of engraftable human skeletal myogenic progenitors from pluripotent stem cells requires the conditional in vitro overexpression of PAX7 using lentiviral vectors. Because a non-integrating approach would be preferable to
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
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
Náš tým vědeckých pracovníků má zkušenosti ve všech oblastech výzkumu, včetně přírodních věd, materiálových věd, chemické syntézy, chromatografie, analytiky a mnoha dalších..
Obraťte se na technický servis.