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    Quantitative Expression Analyses of Candidates for Alternative Anion Conductance in Cystic Fibrosis Mouse Models (2010)

    Art
    Zeitschriftenartikel / wissenschaftlicher Beitrag
    Autoren
    Braun, Josephine
    Mundhenk, Lars
    Range, Friederike
    Gruber, Achim D
    Quelle
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
    Bandzählung: 9
    Heftzählung: 5
    Seiten: 351 – 364
    ISSN: 1569-1993
    Sprache
    Englisch
    Verweise
    DOI: 10.1016/j.jcf.2010.06.003
    Pubmed: 20624691
    Kontakt
    Institut für Tierpathologie

    Robert-von-Ostertag-Str. 15
    14163 Berlin
    +49 30 838 62450
    pathologie@vetmed.fu-berlin.de

    Abstract / Zusammenfassung

    Diversity of cystic fibrosis (CF) phenotype in patients with the same CFTR-mutation raised the hypothesis that other factors modulate the phenotype including "alternative" calcium-activated anion currents (CaCC). This study compares the mRNA expression levels of candidate CaCC mediators in CF mouse models with wild type controls.

    mBEST1, mBEST2, mCLC-3B, mCLC-4, mTTYH3, mTMEM16A, mTMEM16F, mTMEM16K, mCLCA1 to -6 and SLC26A9 mRNA were quantified in CF-relevant tissues in cftr(tm1Cam) and cftr(TgH(neoim)Hgu) mice and controls using real-time RT-qPCR.

    No consistent differences were observed except for mTTYH3 which was significantly down-regulated throughout the intestinal tract of cftr(tm1Cam) mice.

    Down-regulation of mTTYH3 may point towards its involvement in the complex CF pathology. However, the markedly reduced expression argues against a direct compensatory action as an alternative anion conductance. If any of the other candidates plays a role as modulator, factors other than transcriptional regulation and mRNA stability may be involved.