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Dernières publications
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Medhi Hassani, Dylan Moutachi, Mégane Lemaitre, Alexis Boulinguiez, Denis Furling, et al.. 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, ⟨10.1371/journal.pone.0295700⟩. ⟨hal-04501283⟩
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Antonio Atalaia, Dagmar Wandrei, Nawel Lalout, Rachel Thompson, Adrian Tassoni, et al.. EURO-NMD registry: federated FAIR infrastructure, innovative technologies and concepts of a patient-centred registry for rare neuromuscular disorders. Orphanet Journal of Rare Diseases, 2024, 19 (1), pp.66. ⟨10.1186/s13023-024-03059-3⟩. ⟨hal-04460667⟩
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Florent Porquet, Lin Weidong, Kévin Jehasse, Hélène Gazon, Maria Kondili, et al.. Specific DMPK-promoter targeting by CRISPRi reverses myotonic dystrophy type 1-associated defects in patient muscle cells. Molecular Therapy - Nucleic Acids, 2023, 32, pp.857 - 871. ⟨10.1016/j.omtn.2023.05.007⟩. ⟨hal-04287597⟩
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Caroline Le Guiner, T Larcher, A Lafoux, G Toumaniantz, S Webb, et al.. Characterization of the muscular and cardiac diseases of the DMSXL mouse model, a transgenic mouse model for Myotonic Dystrophy type 1. American Society of Gene & Cell Therapy, May 2023, LOS ANGELES, United States. ⟨hal-04096181⟩
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Dylan Moutachi, Mégane Lemaitre, Clément Delacroix, Onnik Agbulut, Denis Furling, et al.. Valproic acid reduces muscle susceptibility to contraction‐induced functional loss but increases weakness in two murine models of Duchenne muscular dystrophy. Clinical and Experimental Pharmacology and Physiology, In press, ⟨10.1111/1440-1681.13804⟩. ⟨hal-04146953⟩
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Chiffres clés
134
Publications avec texte intégral
Open Access
52 %
Mots clés
CTG repeat contractions
Thérapie génique
Glucocorticoids
Oligodendrocytes
Dystrophie Myotonique
Quantitative microdialysis
Glucocorticoid-receptor
Cell model
Male
Exercise
ACETYLCHOLINESTERASE
Mice
Lc3
ARN
Myotonic dystrophy type 1
DM1
Central nervous system
DMSXL mice
Muscular dystrophy
Brain
Antisense oligonucleotide
RNA splicing
Myotonic Dystrophy type 1
Long read sequencing
Cardiac muscle
CONGENITAL MYATHENIC SYNDROME
Gene therapy
AAV
Transgenic mouse
CMS
Exercice
Mouse models
Mouse model
Cell culture model
PacBio
Glutamate
Autophagy
Therapy
Myotonic dystrophy mouse models
DMPK
Skeletal muscle
Cell penetrating peptide
Transcriptomics
Intermediate filament
Heart failure
Fibrosis
Humans
Knockout
Dystrophin
MBNL
Trinucleotide repeat expansion
Acetylcholinesterase knockout mouse
Expression
CTG repeat instability
RNA biology
Muscle
CRISPR/Cas9
GABA
Hypoxia
KNOCKOUT MICE
Centronuclear myopathy
Myelin
Aging
Heart
Astrocytes
Diaphragm
Dynamin 2
Gene editing
Duchenne muscular dystrophy
Glial cells
Oligodendrocyte
Cytoskeleton
Astrocyte
Myotonic Dystrophy Type 1
BIOLOGIE MOLECULAIRE
Trinucleotide Repeat Expansion
Dilated cardiomyopathy
Antisense oligonucleotides
CRISPRi
In vivo
Myotonic dystrophy
Genotype phenotype correlation
Animals
Acute coronary syndrome
Acetylcholinesterase deficiency
Myostatin
Gene Therapy
Desmin
RNA interference
CTG repeats
PCR
Transgenic mouse model
Myotonic Dystrophy
Motoneuron
Alternative splicing
Maximal force
Dystrophie myotonique
Brain dysfunction
GSK3
Neuron