SACS Polyclonal Antibody

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¥680.00

市场价:¥880.00

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产品规格:
  • 50μL
  • 100μL
货号:

FAB2604635

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商品描述
  • 靶点-Target

    SACS

  • 反应性

    Human,Mouse

  • 应用

    IHC,IF

  • 宿主物种-Host Species

    Rabbit

  • 同种型-Isotype

    IgG

  • 修饰-Modification

    Unmodified

  • MW(Observed)

    503kD

  • 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 human protein . at AA range: 4291-4340

  • 特异性

    SACS Polyclonal Antibody detects endogenous levels of protein.

  • 基因名称

    SACS KIAA0730

  • 蛋白名称

    Sacsin (DnaJ homolog subfamily C member 29) (DNAJC29)

  • Organism-1

    Human

  • 基因ID-1

    26278

  • SwissProt-1

    Q9NZJ4

  • Organism-2

    Mouse

  • SwissProt-2

    Q9JLC8

  • 背景

    This gene encodes the sacsin protein, which includes a UbL domain at the N-terminus, a DnaJ domain, and a HEPN domain at the C-terminus. The gene is highly expressed in the central nervous system, also found in skin, skeletal muscles and at low levels in the pancreas. This gene includes a very large exon spanning more than 12.8 kb. Mutations in this gene result in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS), a neurodegenerative disorder characterized by early-onset cerebellar ataxia with spasticity and peripheral neuropathy. The authors of a publication on the effects of siRNA-mediated sacsin knockdown concluded that sacsin protects against mutant ataxin-1 and suggest that "the large multi-domain sacsin protein is able to recruit Hsp70 chaperone action and has the potential to regulate the effects of other ataxia proteins" (Parfitt et al., PubMed: 19208651).

  • 细胞定位

    Cytoplasm . Predominantly cytoplasmic, a small portion is present in the nucleus and also shows a partial mitochondrial overlap with the mitochondrial marker Hsp60.