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