Dissecting Pathogenetic Mechanisms and Therapeutic Strategies in Drosophila Models of Myotonic Dystrophy Type 1

Autor: Anissa Souidi, Monika Zmojdzian, Krzysztof Jagla
Jazyk: angličtina
Rok vydání: 2018
Předmět:
Zdroj: International Journal of Molecular Sciences, Vol 19, Iss 12, p 4104 (2018)
Druh dokumentu: article
ISSN: 1422-0067
19124104
DOI: 10.3390/ijms19124104
Popis: Myotonic dystrophy type 1 (DM1), the most common cause of adult-onset muscular dystrophy, is autosomal dominant, multisystemic disease with characteristic symptoms including myotonia, heart defects, cataracts and testicular atrophy. DM1 disease is being successfully modelled in Drosophila allowing to identify and validate new pathogenic mechanisms and potential therapeutic strategies. Here we provide an overview of insights gained from fruit fly DM1 models, either: (i) fundamental with particular focus on newly identified gene deregulations and their link with DM1 symptoms; or (ii) applied via genetic modifiers and drug screens to identify promising therapeutic targets.
Databáze: Directory of Open Access Journals
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