DMD Polyclonal Antibody

活动价:

¥680.00

市场价:¥880.00

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产品规格:
  • 50μL
  • 100μL
货号:

FAB2602249

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商品描述
  • 靶点-Target

    DMD

  • 反应性

    Human,Mouse,Rat

  • 应用

    IHC,IF

  • 宿主物种-Host Species

    Rabbit

  • 同种型-Isotype

    IgG

  • 修饰-Modification

    Unmodified

  • MW(Observed)

    405kD

  • 推荐稀释比

    IHC 1:50-300;IF 1:50-200

  • 组成

    PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA

  • 纯化工艺

    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

  • 储存

    -15°C to -25°C/1 year(Do not lower than -25°C)

  • 浓度

    1 mg/ml

  • 克隆性

    Polyclonal

  • 免疫原

    Synthesized peptide derived from part region of human protein

  • 特异性

    DMD Polyclonal Antibody detects endogenous levels of protein.

  • 基因名称

    DMD

  • 蛋白名称

    Dystrophin

  • Organism-1

    Human

  • 基因ID-1

    1756

  • SwissProt-1

    P11532

  • Organism-2

    Mouse

  • SwissProt-2

    P11531

  • Organism-3

    Rat

  • SwissProt-3

    P11530

  • 背景

    dystrophin(DMD) Homo sapiens 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 enc

  • 细胞定位

    Cell membrane, sarcolemma ; Peripheral membrane protein ; Cytoplasmic side . Cytoplasm, cytoskeleton . Cell junction, synapse, postsynaptic cell membrane . 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. .