Overview

  • Product name
  • Description
    Rabbit polyclonal to PKHD1
  • Tested applications
    Suitable for: IHC-Pmore details
  • Species reactivity
    Reacts with: Human
  • Immunogen

    antigen sequence: KTTTVNYVRD TLSNPRGWMA LLLDQETYSL QSENLWINRS LQYSATFDNF APGNYLLLVH TDLPPYPDIL LRCGSRVGLS FPFLPSPGQN Q, corresponding to amino acids 2596-2686 of Human PKHD1.

  • Positive control
    • Human liver tissue.

Properties

Applications

Our Abpromise guarantee covers the use of ab122160 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
IHC-P 1/200 - 1/500. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

Target

  • Function
    May be a receptor protein that acts in collecting-duct and biliary differentiation.
  • Tissue specificity
    Predominantly expressed in fetal and adult kidney. Also present in the adult pancreas, but at much lower levels. Detectable in fetal and adult liver. Rather indistinct signal in fetal brain.
  • Involvement in disease
    Defects in PKHD1 are the cause of polycystic kidney disease autosomal recessive (ARPKD) [MIM:263200]. ARPKD is a severe form of polycystic kidney disease affecting the kidneys and the hepatic biliary tract. The clinical spectrum is widely variable, with most cases presenting during infancy. The fetal phenotypic features classically include enlarged and echogenic kidneys, as well as oligohydramnios secondary to a poor urine output. Up to 50% of the affected neonates die shortly after birth, as a result of severe pulmonary hypoplasia and secondary respiratory insufficiency. In the subset that survives the perinatal period, morbidity and mortality are mainly related to severe systemic hypertension, renal insufficiency, and portal hypertension due to portal-tract fibrosis.
  • Sequence similarities
    Contains 2 G8 domains.
    Contains 12 IPT/TIG domains.
    Contains 9 PbH1 repeats.
  • Cellular localization
    Membrane.
  • Information by UniProt
  • Database links
  • Alternative names
    • ARPKD antibody
    • FCYT antibody
    • Fibrocystin antibody
    • FPC antibody
    • PKHD1 antibody
    • PKHD1_HUMAN antibody
    • Polycystic kidney and hepatic disease 1 protein antibody
    • Polyductin antibody
    • TIGM1 antibody
    • Tigmin antibody
    see all

Images

  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) analysis of human kidney tissue labelling PKHD1 with ab122160. Staining shows strong cytoplasmic positivity in cells in tubules.

References

ab122160 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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