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