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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⟩
Chiffres clés
134
Publications avec texte intégral
Open Access
52 %
Mots clés
Astrocyte
Myotonic dystrophy mouse models
Astrocytes
Brain
Intermediate filament
CTG repeat contractions
Gene editing
Acetylcholinesterase deficiency
Acute coronary syndrome
Central nervous system
Brain dysfunction
Maximal force
MBNL
ACETYLCHOLINESTERASE
Aging
RNA splicing
Mouse models
DM1
Hypoxia
Dystrophie Myotonique
Transcriptomics
Skeletal muscle
CRISPR/Cas9
RNA biology
Myotonic Dystrophy
Transgenic mouse
Centronuclear myopathy
Dystrophin
CMS
CONGENITAL MYATHENIC SYNDROME
Antisense oligonucleotides
Trinucleotide repeat expansion
Cardiac muscle
Gene therapy
KNOCKOUT MICE
Myotonic Dystrophy Type 1
Myotonic dystrophy
CRISPRi
Gene Therapy
Cell penetrating peptide
Expression
CTG repeat instability
Myotonic dystrophy type 1
GABA
Glutamate
CTG repeats
Therapy
Long read sequencing
Exercise
Neuron
Autophagy
GSK3
Diaphragm
Muscle
Cytoskeleton
Myotonic Dystrophy type 1
Acetylcholinesterase knockout mouse
Oligodendrocytes
Cell culture model
Dynamin 2
Lc3
Glucocorticoid-receptor
Humans
Desmin
Muscular dystrophy
Dystrophie myotonique
Myelin
Dilated cardiomyopathy
RNA interference
Mouse model
DMSXL mice
Glucocorticoids
Heart
In vivo
Antisense oligonucleotide
Trinucleotide Repeat Expansion
PacBio
Animals
Mice
Thérapie génique
Transgenic mouse model
DMPK
ARN
Glial cells
Cell model
Heart failure
Knockout
BIOLOGIE MOLECULAIRE
Fibrosis
PCR
AAV
Alternative splicing
Quantitative microdialysis
Oligodendrocyte
Genotype phenotype correlation
Male
Exercice
Motoneuron
Myostatin
Duchenne muscular dystrophy