• Product name
  • Description
    Rabbit polyclonal to NPHP3
  • Host species
  • Tested applications
    Suitable for: ICC/IF, IHC-Pmore details
  • Species reactivity
    Reacts with: Human
  • Immunogen

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

  • Positive control
    • Human skin tissue


  • Form
  • Storage instructions
    Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
  • Storage buffer
    pH: 7.20
    Preservative: 0.02% Sodium azide
    Constituents: 59% PBS, 40% Glycerol
  • Concentration information loading...
  • Purity
    Immunogen affinity purified
  • Clonality
  • Isotype


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.

Application Abreviews Notes
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.


  • Function
    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.
  • Tissue specificity
    Widely expressed at low level. Expressed in heart, placenta, liver, skeletal muscle, kidney and pancreas. Expressed at very low level in brain and lung.
  • Involvement in disease
    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.
  • Sequence similarities
    Contains 11 TPR repeats.
  • Cellular localization
    Cell projection > cilium.
  • Information by UniProt
  • Database links
  • Alternative names
    • 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


  • 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.


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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