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    Utrophin Up-Regulation by an Artificial Transcription Factor in Transgenic Mice

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    Authors
    Mattei, Elisabetta
    Corbi, Nicoletta
    Di Certo, Maria Grazia
    Strimpakos, Georgios
    Severini, Cinzia
    Onori, Annalisa
    Desantis, Agata
    Libri, Valentina
    Buontempo, Serena
    Floridi, Aristide
    Fanciulli, Maurizio
    Baban, Dilair
    Davies, Kay E.
    Passananti, Claudio
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    Issue Date
    2007-08-22
    URI
    http://hdl.handle.net/10675.2/536
    
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    Abstract
    Duchenne Muscular Dystrophy (DMD) is a severe muscle degenerative disease, due to absence of dystrophin. There is currently no effective treatment for DMD. Our aim is to up-regulate the expression level of the dystrophin related gene utrophin in DMD, complementing in this way the lack of dystrophin functions. To this end we designed and engineered several synthetic zinc finger based transcription factors. In particular, we have previously shown that the artificial three zinc finger protein named Jazz, fused with the appropriate effector domain, is able to drive the transcription of a test gene from the utrophin promoter â Aâ . Here we report on the characterization of Vp16-Jazz-transgenic mice that specifically over-express the utrophin gene at the muscular level. A Chromatin Immunoprecipitation assay (ChIP) demonstrated the effective access/binding of the Jazz protein to active chromatin in mouse muscle and Vp16-Jazz was shown to be able to up-regulate endogenous utrophin gene expression by immunohistochemistry, western blot analyses and real-time PCR. To our knowledge, this is the first example of a transgenic mouse expressing an artificial gene coding for a zinc finger based transcription factor. The achievement of Vp16-Jazz transgenic mice validates the strategy of transcriptional targeting of endogenous genes and could represent an exclusive animal model for use in drug discovery and therapeutics.
    Citation
    PLoS ONE. 2007 Aug 22; 2(8):e774
    ae974a485f413a2113503eed53cd6c53
    10.1371/journal.pone.0000774
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    Department of Neurology: Faculty Research and Presentations

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