Overview

  • Product name

    Anti-Cirhin antibody
  • Description

    Rabbit polyclonal to Cirhin
  • Host species

    Rabbit
  • Tested applications

    Suitable for: ICC/IFmore details
  • Species reactivity

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

    Recombinant fragment corresponding to Human Cirhin aa 8-89.
    Sequence:

    RVRFFNYVPSGIRCVAYNNQSNRLAVSRTDGTVEIYNLSANYFQEKFFPG HESRATEALCWAEGQRLFSAGLNGEIMEYDLQ


    Database link: Q969X6

  • Positive control

    • U-2 OS cells.
  • General notes

    Gently mix before use. Optimal concentrations and conditions for each application should be determined by the user.

Properties

Applications

Our Abpromise guarantee covers the use of ab220326 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.

Fixation/Permeabilization: PFA/Triton X-100.

Target

  • Relevance

    Defects in Cirhin are the cause of North American Indian childhood cirrhosis. NAIC is a severe autosomal recessive intrahepatic cholestasis, originally described in Ojibway-Cree children from northwestern Quebec. NAIC typically presents with transient neonatal jaundice, in a child who is otherwise healthy, and progresses to biliary cirrhosis and portal hypertension. Biochemical and histopathological features suggest involvement of the bile ducts rather than of the bile canaliculi. They include elevated gamma glutamyltransferase and alkaline phosphatase levels, and, typically, marked fibrosis around bile ducts. Clinically, NAIC is distinct from other nonsyndromic familial cholestases because of its marked cholangiopathic features and severe degree of fibrosis on liver histology.
  • Cellular localization

    Nucleus; nucleolus
  • Database links

  • Alternative names

    • CIRH1A antibody
    • Cirhin antibody
    • Cirrhosis, autosomal recessive 1A (cirhin) antibody
    • FLJ17146 antibody
    • KIAA1988 antibody
    • NAIC antibody
    • testis expressed gene 292 antibody
    • TEX292 antibody
    • U3 small nucleolar RNA associated protein 4 homolog antibody
    • UTP4 antibody
    • UTP4 small subunit (SSU) processome component antibody
    • UTP4 small subunit (SSU) processome component homolog antibody
    • UTP4, small subunit processome component antibody
    see all

Images

  • Immunofluorescent analysis of PFA-fixed, Triton X-100 permeabilized U-2 OS cells labeling Cirhin with ab220326 at 4 µg/ml.  Antibody staining is shown in green.  

References

ab220326 has not yet been referenced specifically in any publications.

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