概述

  • 产品名称Anti-NPHP3抗体
  • 描述
    兔多克隆抗体to NPHP3
  • 经测试应用适用于: ICC/IF, IHC-Pmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    antigen, corresponding to amino acids 256-360 of Human NPHP3.

  • 阳性对照
    • Human skin tissue

性能

  • 形式Liquid
  • 存放说明Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
  • 存储溶液pH: 7.20
    Preservative: 0.02% Sodium azide
    Constituents: 59% PBS, 40% Glycerol
  • Concentration information loading...
  • 纯度Immunogen affinity purified
  • 克隆多克隆
  • 同种型IgG

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应用

Our Abpromise guarantee covers the use of ab121305 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
ICC/IF Use a concentration of 1 - 4 µg/ml.

Recommend PFA Fixation and Triton X-100 treatment

IHC-P 1/50 - 1/200. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

靶标

  • 功能Required for normal ciliary development and function. Inhibits disheveled-1-induced canonical Wnt-signaling activity and may also play a role in the control of non-canonical Wnt signaling which regulates planar cell polarity. Probably acts as a molecular switch between different Wnt signaling pathways. Required for proper convergent extension cell movements.
  • 组织特异性Widely expressed at low level. Expressed in heart, placenta, liver, skeletal muscle, kidney and pancreas. Expressed at very low level in brain and lung.
  • 疾病相关Defects in NPHP3 are the cause of nephronophthisis type 3 (NPHP3) [MIM:604387]; also known as adolescent nephronophthisis. NPHP3 is a autosomal recessive disorder resulting in end-stage renal disease. It is characterized by polyuria, polydipsia, anemia. Onset of terminal renal failure occurr significantly later (median age, 19 years) than in juvenile nephronophthisis. Renal pathology is characterized by alterations of tubular basement membranes, tubular atrophy and dilation, sclerosing tubulointerstitial nephropathy, and renal cyst development predominantly at the corticomedullary junction.
    Defects in NPHP3 are a cause of renal-hepatic-pancreatic dysplasia (RHPD) [MIM:208540]. RHPD is an autosomal recessive disorder with variable expression, and patients surviving the neonatal period progress to renal and hepatic failure which can be treated successfully with combined liver-kidney transplantation.
    Defects in NPHP3 are the cause of Meckel syndrome type 7 (MKS7) [MIM:267010]. It is a form of Meckel syndrome, an autosomal recessive disorder. It is characterized by a combination of renal cysts and variably associated features including developmental anomalies of the central nervous system (typically encephalocele), hepatic ductal dysplasia and cysts, and polydactyly.
  • 序列相似性Contains 11 TPR repeats.
  • 细胞定位Cell projection > cilium.
  • Information by UniProt
  • 数据库链接
  • 别名
    • Meckel syndrome, type 7 antibody
    • MKS7 antibody
    • Nephrocystin-3 antibody
    • nephronophthisis 3 (adolescent) antibody
    • NPH3 antibody
    • Nphp3 antibody
    • NPHP3_HUMAN antibody
    • pcy antibody
    • RHPD antibody
    see all

Anti-NPHP3 antibody 图像

  • Immunofluorescent staining of Human cell line U-2 OS shows positivity in nucleoli. Recommended concentration of ab121305 1-4 µg/ml. Cells treated with PFA/Triton X-100.
  • ab121305 at 1/75 dilution staining NPHP3 in Paraffin-embedded Human skin tissue by Immunohistochemistry.

Anti-NPHP3 antibody (ab121305)参考文献

This product has been referenced in:
  • Hatzistergos KE  et al. S-Nitrosoglutathione Reductase Deficiency Enhances the Proliferative Expansion of Adult Heart Progenitors and Myocytes Post Myocardial Infarction. J Am Heart Assoc 4:N/A (2015). IHC-P ; Mouse . Read more (PubMed: 26178404) »

See 1 Publication for this product

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