您好,欢迎来到半岛电竞官方网址 ! [ 登录] [ 免费注册]
半岛电竞官方网址
位置: 首页> 品牌> Sigma-Aldrich> Anti-Dystrophin Antibody, clone 2C6 (MANDYS106)

Anti-Dystrophin Antibody, clone 2C6 (MANDYS106)

品牌
Sigma-Aldrich
货号
MABT827
规格纯度
clone 2C6 (MANDYS106), from mouse
价格
5352.52 *本价格含增值税费
促销
服务
  • 原厂保证
  • 包邮
  • 增值税票
数量
- +
半岛bd体育手机客户端 名称:
Anti-Dystrophin Antibody, clone 2C6 (MANDYS106)
Dystrophin
半岛bd体育手机客户端 介绍:

半岛bd体育手机客户端 说明

一般描述

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.

特异性

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.

免疫原

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.

应用

Research Category
Cell Structure
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).
Research Sub Category
Adhesion (CAMs)

质量

Evaluated by Immunohistochemistry in human skeletal muscle myocytes.

Immunohistochemistry Analysis: A 1:50 dilution of this antibody detected Dystrophin in human skeletal muscle myocytes.

目标描述

~427 kDa observed

外形

Purified mouse monoclonal IgG2aκ antibody in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
Format: Purified
Protein G Purified

储存及稳定性

Stable for 1 year at 2-8°C from date of receipt.

其他说明

Concentration: Please refer to lot specific datasheet.

免责声明

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.

基本信息

eCl@ss 32160702
NACRES NA.41

半岛bd体育手机客户端 性质

质量水平 100
生物来源 mouse
抗体形式 purified antibody
antibody product type primary antibodies
克隆 2C6 (MANDYS106), monoclonal
species reactivity human
technique(s) immunofluorescence: suitable
immunohistochemistry: suitable
western blot: suitable
同位素/亚型 IgG2aκ
NCBI登记号 NP_000100
UniProt登记号 P11532-1
运输 wet ice
Gene Information human ... DMD(1756)

安全信息

储存分类代码 12 - Non Combustible Liquids
WGK WGK 1
闪点(F) Not applicable
闪点(C) Not applicable

Sigma-Aldrich

推荐半岛bd体育手机客户端
Baidu
map