Overview

  • Product name

    Anti-PKHD1 antibody [8G12A1]
    See all PKHD1 primary antibodies
  • Description

    Rat monoclonal [8G12A1] to PKHD1
  • Host species

    Rat
  • Tested applications

    Suitable for: WB, ICC/IF, Flow Cyt, IHC-Pmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Mouse
  • Immunogen

    Recombinant fragment corresponding to Mouse PKHD1 aa 3878-4060.
    Sequence:

    TAW NAQKKHCELE RHLSTEQFLP NLGSTVPGEN YFDRTYQMLY LFLKGTTPVE VHTATVIFVS FHLPVMTADE FFSSHNLVRN LALFLKIPSD KIRVSRIIGA SLRKKRSTGH IMEFEIGAAP TQFLSNSTTG QMQLSELQEI TDSLGQAVVL GKISTILGFN ISSMSITSPI PQPTDSGWIK


    Database link: Q80ZA4

  • Positive control

    • Mouse recombinant protein, A431 cells, HeLa cells, esophageal cancer tissue.
  • General notes

    This product was changed from ascites to supernatant. Lot no’s high than GR291788-5 are from Tissue Culture Supernatant

Properties

Applications

Our Abpromise guarantee covers the use of ab204951 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
WB Use at an assay dependent concentration. Predicted molecular weight: 465 kDa.
ICC/IF 1/200 - 1/1000.
Flow Cyt 1/200 - 1/400.
IHC-P 1/200 - 1/1000.

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

  • Immunofluorescent analysis of A431 cells labeling PKHD1 with ab204951 at 1/200 (green). Blue: DRAQ5 fluorescent DNA dye. Red: Actin filaments have been labeled with Alexa Fluor- 555 phalloidin.

  • Flow cytometric analysis of HeLa cells labeling PKHD1 with ab204951 at 1/200 (green) or negative control (red)

  • Immunohistochemical analysis of paraffin-embedded esophageal cancer tissue labeling PKHD1 with ab204951 at 1/200 dilution followed by DAB staining

References

ab204951 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

Application
Western blot
Sample
Mouse Tissue lysate - other (Kidney)
Gel Running Conditions
Reduced Denaturing (3-8% Tris-Acetate)
Loading amount
50 µg
Specification
Kidney
Blocking step
BSA as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 21°C

Abcam user community

Verified customer

Submitted Sep 13 2018

Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
For licensing inquiries, please contact partnerships@abcam.com

Sign up