Examples of our recent findings

Novel Drosophila model of Myotonic dystrophy type 1 (DM1) 

  • DM1- a CTG repeats disease affecting splicing and gene activity
  • Leads to muscle weakness, cardiac arrythmia and heart failure

Generation, phenotyping and genome-wide transcriptomic analyses of the Drosophila  DM1 lines muscle>(iCTG)n and muscle>MblRNAi


Insights into myofibrilar myopathies: Functional analysis of sHsp dCryAB


Drosophila Hsp67Bc hot-spot variants alter muscle structure and function - Drosophila model of Charcot Marie Tooth disease

 Jablonska et al., Cell Mol Life Sci, 2018



Bruno-3 regulates sarcomere component expression and contributes to muscle phenotypes of myotonic dystrophy type 1


Picchio et al., Dis Mod Mech, 2018


Straightjacket/α2δ3 deregulation is associated with cardiac conduction defects in myotonic dystrophy type 1

 Auxerre-Plantié et al., eLife 2019




Current applied projects:
Exploiting generated DM1 and Myofibrilar myopathy models

Characterization of novel splice-independent Bru-3/CUGBP1 function in DM1

 L. Picchio, Y. Renaud in coll. V. Legagneux, CNRS Rennes 

Genome wide transcriptional profiling reveals that muscle-targeted gain of Bru-3 function leads to transcript depletion of genes encoding sarcomeric components

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Myonuclei positioning in Myofibrilar myopathy model

J. Jablonska, T. Jagla  Coll. S. Huelsman, G. Daczewska

Nuclei position in muscle cells play a key role in proper muscle development and function. We recently found that dCryAB, a small heat-shock protein  (sHsp) is specifically expressed in larval muscles around the nuclei and required for correct nuclei positioning (Wojtowicz et al., 2015).

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Cell-specific genome wide analyses of cardiac defects in DM1

E. Plantié, C. Girardin, Y. Renaud, L. Picchio  Coll. D. Furling and N. Charlet

This project is dedicated to identify transcriptomic alterations and related molecular mechanisms underlying cardiac symptom of DM1. Contrary to the vertebrate heart, the Drosophila heart is not essential for survival of fruit flies, making it a good model to study progression of cardiac diseases.  

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