Head: Denis furling
Our research, within the REDs team, focuses mainly on 2 complementary areas:
. Understanding the molecular and cellular mechanisms involved in the pathophysiology of DM1 induced by the expression of abnormally repeated triplet CTGs and, more specifically, in alterations in muscle function.
. The development of innovative therapeutic approaches to inhibit the toxicity induced by RNAs containing expansions of pathological triplets, and their evaluation using cell and mouse models of DM1.
In addition, the effects of muscular exercise, in combination with gene therapies, are also being evaluated in mouse models of neuromuscular diseases, under the expertise of Prof. Ferry.
Group members
Denis FURLING, PhD, DR, Team leader
Ludovic ARANDEL, PhD, IR
Arnaud FERRY, PhD, PU
Arnaud KLEIN, PhD, CR
Gilles MOULAY, PhD, IR
Frédérique RAU, PhD, CR
Alain SUREAU, PhD, CR
Aurélien CORDIER, Msc, IE
Julie FAGIOLI, PhD student
Xavière LORNAGE, PhD, Post-doctoral fellow
Florent PORQUET, PhD, Post-doctoral fellow
Krizia RONQUILLO, apprentice
Corentin ROUXEL, Msc, IE
Contact :Â denis.furling@sorbonne-universite.fr
Last publications
- Bogard B, Bonnet H, Boyarchuk E, Tellier G, Furling D, Mouly V, Francastel C, Hubé F. Small nucleolar RNAs promote the restoration of muscle differentiation defects in cells fom myotonic dystrophy type 1. Nucleic Acids Research (2025) 53(6):gkaf232. doi: 10.1093/nar/gkaf232.
-  Frison-Roche C, Demier CM, Cottin S, Lainé J, Arandel L, Halliez M, Lemaitre M, Lornage X, Strochlic L, Swanson MS, Martinat C, Messéant J, Furling D* & Rau F*. MBNL deficiency in motor neurons disrupts neuromuscular junction maintenance and gait coordination. Brain (2024) doi: 10.1093/brain/awae336
- Moutachi D, Hyzewicz J, Roy P, Lemaitre M, Bachasson D, Amthor H, Ritvos O, Li Z, Furling D, Agbulut O, and Ferry A. Treadmill running and mechanical overloading improved the strength of the plantaris muscle in the dystrophin-desmin double knockout (DKO) mouse. Journal of Physiology (2024) 602(15):3641-3660. doi: 10.1113/JP286425
- Hassani M, Moutachi D, Lemaitre M, Boulinguiez A, Furling D, Agbulut O, Ferry A. Beneficial effects of resistance training on both mild and severe mouse dystrophic muscle function as a preclinical option for Duchenne muscular dystrophy. PLoS One (2024) 19(3):e0295700. doi: 10.1371/journal.pone.0295700
- Â Moutachi D, Lemaitre M, Delacroix C, Agbulut O, Furling D, Ferry A. Valproic acid reduces muscle susceptibility to contraction-induced functional loss but increases weakness in two murine models of Duchenne muscular dystrophy. Clin Exp Pharmacol Physiol. 2023 Sep;50(9):749-756. doi: 10.1111/1440-1681.13804. Epub 2023 Jun 29. PMID: 37381823
- Arandel L, Matloka M, Klein AF, Rau F, Sureau A, Ney M, Cordier A, Kondili M, Polay-Espinoza M, Naouar N, Ferry A, Lemaitre M, Begard S, Colin M, Lamarre C, Tran H, Buée L, Marie J, Sergeant N, Furling D. Reversal of RNA toxicity in myotonic dystrophy via a decoy RNA-binding protein with high affinity for expanded CUG repeats.Nat Biomed Eng. 2022 Feb 10. doi: 10.1038/s41551-021-00838-2. Epub ahead of print. PMID: 35145256.
- Monceau A, Moutachi D, Lemaitre M, Garcia L, Trollet C, Furling D, Klein A, Ferry A. Dystrophin restoration after AAV-U7-mediated dmd exon-skipping is modulated by muscular exercise in the severe D2-mdx DMD murine model. The Amercan Journal of Pathology 2022 Sep 13:S0002-9440(22)00279-6 ; doi: 10.1016/j.ajpath.2022.07.016
- Tsai YC, de Pontual L, Heiner C, Stojkovic T, Furling D, Bassez G, Gourdon G, Tomé S. Identification of a CCG-Enriched Expanded Allele in Patients with Myotonic Dystrophy Type 1 Using Amplification-Free Long-Read Sequencing. The Journal Molecular of Diagnostics, 2022. 24: 1143-1154. doi: 10.1016/j.jmoldx.2022.08.003.
- Monceau A, Delacroix C, Lemaitre M, Revet G, Furling D, Agbulut O, Klein A, Ferry A. The beneficial effect of chronic muscular exercise on muscle fragility is increased by Prox1 gene transfer in dystrophic mdx muscle. PLoS One, 2022. 17(4):e0254274; doi: 10.1371/journal.pone.0254274.
- van Cruchten RTP, van As D, Glennon JC, van Engelen BGM, ‘t Hoen PAC; OPTIMISTIC consortium; ReCognitION consortium including D. Furling & G. Gourdon. Clinical improvement of DM1 patients reflected by reversal of disease-induced gene expression in blood. BMC Medicine. 2022 20(1):395. doi: 10.1186/s12916-022-02591-y.
Other publications
http://www.ncbi.nlm.nih.gov/pubmed/?term=Furling+D
