anti-DMD / Dystrophin antibody [MANDYS8]

Key features and details

  • 产品描述: Mouse Monoclonal antibody [MANDYS8] recognizes DMD / Dystrophin
  • 反应物种: Hu, Ms, Rat
  • 应用: IHC-P, WB
  • 宿主: Mouse
  • 克隆: Monoclonal
  • 克隆号: MANDYS8
  • 同位型: IgG2b
  • 靶点名称: DMD / Dystrophin
  • 抗原物种: Human
  • Brand:
CAT.NO. : ARG41559
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Product Details
概述
产品描述Mouse Monoclonal antibody [MANDYS8] recognizes DMD / Dystrophin
反应物种Hu, Ms, Rat
应用IHC-P, WB
宿主Mouse
克隆Monoclonal
克隆号MANDYS8
同位型IgG2b
靶点名称DMD / Dystrophin
抗原物种Human
抗原Recombinant Human DMD / Dystrophin fragment.
偶联标记Un-conjugated
別名BMD; CMD3B; DXS270; DXS272; MRX85; Dystrophin; DXS164; DXS239; DXS206; DXS142; DXS230; DXS269; DXS268
应用说明
应用建议
应用推荐稀释比
IHC-P1:25 - 1:50
WB1:50 - 1:100
应用说明* The dilutions indicate recommended starting dilutions and the optimal dilutions or concentrations should be determined by the scientist.
属性
形式Liquid
纯化Unpurified.
缓冲液Mouse ascites fluid, 1.2% Sodium acetate, 0.01% Sodium azide and 2% BSA.
抗菌剂0.01% Sodium azide
稳定剂2% BSA
浓度0.5 mg/ml
存放说明For continuous use, store undiluted antibody at 2-8°C for up to a week. For long-term storage, aliquot and store at -20°C or below. Storage in frost free freezers is not recommended. Avoid repeated freeze/thaw cycles. Suggest spin the vial prior to opening. The antibody solution should be gently mixed before use.
注意事项For laboratory research only, not for drug, diagnostic or other use.
生物信息
数据库连接

GeneID: 13405 Mouse DMD

GeneID: 1756 Human DMD

Swiss-port # P11531 Mouse Dystrophin

Swiss-port # P11532 Human Dystrophin

基因名称DMD
全名dystrophin
背景介绍The dystrophin gene is the largest gene found in nature, measuring 2.4 Mb. The gene was identified through a positional cloning approach, targeted at the isolation of the gene responsible for Duchenne (DMD) and Becker (BMD) Muscular Dystrophies. DMD is a recessive, fatal, X-linked disorder occurring at a frequency of about 1 in 3,500 new-born males. BMD is a milder allelic form. In general, DMD patients carry mutations which cause premature translation termination (nonsense or frame shift mutations), while in BMD patients dystrophin is reduced either in molecular weight (derived from in-frame deletions) or in expression level. The dystrophin gene is highly complex, containing at least eight independent, tissue-specific promoters and two polyA-addition sites. Furthermore, dystrophin RNA is differentially spliced, producing a range of different transcripts, encoding a large set of protein isoforms. Dystrophin (as encoded by the Dp427 transcripts) is a large, rod-like cytoskeletal protein which is found at the inner surface of muscle fibers. Dystrophin is part of the dystrophin-glycoprotein complex (DGC), which bridges the inner cytoskeleton (F-actin) and the extra-cellular matrix. [provided by RefSeq, Jul 2008]
生物功能Anchors the extracellular matrix to the cytoskeleton via F-actin. Ligand for dystroglycan. Component of the dystrophin-associated glycoprotein complex which accumulates at the neuromuscular junction (NMJ) and at a variety of synapses in the peripheral and central nervous systems and has a structural function in stabilizing the sarcolemma. Also implicated in signaling events and synaptic transmission. [UniProt]
细胞定位Cell membrane, sarcolemma; Peripheral membrane protein; Cytoplasmic side. Cytoplasm, cytoskeleton. Cell junction, synapse, postsynaptic cell membrane. Note=In muscle cells, sarcolemma localization requires the presence of ANK2, while localization to costameres requires the presence of ANK3. Localizes to neuromuscular junctions (NMJs). In adult muscle, NMJ localization depends upon ANK2 presence, but not in newborn animals. [UniProt]
预测分子量427 kDa
检测图片 (3)
  • ARG41559 anti-DMD / Dystrophin antibody [MANDYS8] WB image

    Western blot: Rat skeletal muscle stained with ARG41559 anti-DMD / Dystrophin antibody [MANDYS8] at 1 μg/mL dilution.

  • ARG41559 anti-DMD / Dystrophin antibody [MANDYS8] IHC-P image

    Immunohistochemistry: Mouse cardiac muscle stained with ARG41559 anti-DMD / Dystrophin antibody [MANDYS8] at 2 μg/mL dilution.

  • ARG41559 anti-DMD / Dystrophin antibody [MANDYS8] WB image

    Western blot: Mouse skeletal muscle stained with ARG41559 anti-DMD / Dystrophin antibody [MANDYS8] at 1 μg/mL dilution.

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