NPHP1 Polyclonal Antibody

活动价:

¥680.00

市场价:¥880.00

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产品规格:
  • 50μL
  • 100μL
货号:

FAB2603113

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商品描述
  • 靶点-Target

    NPHP1

  • 反应性

    Human,Mouse

  • 应用

    WB,ELISA

  • 宿主物种-Host Species

    Rabbit

  • 同种型-Isotype

    IgG

  • 修饰-Modification

    Unmodified

  • MW(Observed)

    80kD

  • 推荐稀释比

    WB 1:500-2000;ELISA 1:5000-20000

  • 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: 510-590

  • 特异性

    NPHP1 Polyclonal Antibody detects endogenous levels of protein.

  • 基因名称

    NPHP1 NPH1

  • 蛋白名称

    Nephrocystin-1 (Juvenile nephronophthisis 1 protein)

  • Organism-1

    Human

  • 基因ID-1

    4867

  • SwissProt-1

    O15259

  • Organism-2

    Mouse

  • SwissProt-2

    Q9QY53

  • 背景

    This gene encodes a protein with src homology domain 3 (SH3) patterns. This protein interacts with Crk-associated substrate, and it appears to function in the control of cell division, as well as in cell-cell and cell-matrix adhesion signaling, likely as part of a multifunctional complex localized in actin- and microtubule-based structures. Mutations in this gene cause familial juvenile nephronophthisis type 1, a kidney disorder involving both tubules and glomeruli. Defects in this gene are also associated with Senior-Loken syndrome type 1, also referred to as juvenile nephronophthisis with Leber amaurosis, which is characterized by kidney and eye disease, and with Joubert syndrome type 4, which is characterized by cerebellar ataxia, oculomotor apraxia, psychomotor delay and neonatal breathing abnormalities, sometimes including retinal dystrophy and renal disease. Multiple transcript variants encoding diffe

  • 细胞定位

    Cell junction . Cell junction, adherens junction . Cell projection, cilium . Cytoplasm, cytoskeleton, cilium axoneme . Cell junction, tight junction. In the retinal photoreceptor cell layer, localizes at the connecting cilium (By similarity). Colocalizes with E-cadherin and BCAR1 at or near the cell-cell adherens junctions (By similarity). Localized to respiratory cilia axoneme (PubMed:16308564, PubMed:16885411). Localized to the transition zone of respiratory cilia (PubMed:16885411). Localized to the transition zone of photoreceptor-connecting cilia and renal monocilia (By similarity). In cultured renal cells, it localizes diffusely in the cytoplasm but, as cells approach confluence, it accumulates at basolateral tight junctions (By similarity). .