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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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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
132
Publications avec texte intégral
Open Access
52 %
Mots clés
Myotonic Dystrophy type 1
Astrocytes
Acetylcholinesterase deficiency
Autophagy
RNA splicing
Transgenic mouse model
Centronuclear myopathy
DM1
CTG repeats
CRISPRi
CTG repeat instability
Acute coronary syndrome
Glucocorticoids
Mice
Neuron
KNOCKOUT MICE
CTG repeat contractions
Muscular dystrophy
Gene editing
Myotonic Dystrophy
Cell model
Motoneuron
Cytoskeleton
Myotonic dystrophy
GSK3
MBNL
Male
Lc3
Desmin
Myostatin
RNA biology
Myelin
Cell penetrating peptide
Dystrophie Myotonique
Trinucleotide repeat expansion
Dynamin 2
Intermediate filament
Gene Therapy
Quantitative microdialysis
Glucocorticoid-receptor
Brain dysfunction
Fibrosis
Glutamate
Exercice
Cell culture model
Myotonic dystrophy type 1
Acetylcholinesterase knockout mouse
Dystrophin
Heart
DMPK
Maximal force
Thérapie génique
Exercise
Brain
Mouse model
Dilated cardiomyopathy
PacBio
In vivo
Glial cells
Therapy
PCR
Long read sequencing
DMSXL mice
Duchenne muscular dystrophy
Central nervous system
Genotype phenotype correlation
Hypoxia
Dystrophie myotonique
Astrocyte
Skeletal muscle
Transcriptomics
Diaphragm
GABA
BIOLOGIE MOLECULAIRE
Myotonic Dystrophy Type 1
RNA interference
Trinucleotide Repeat Expansion
Aging
CMS
Antisense oligonucleotide
Oligodendrocytes
Myotonic dystrophy mouse models
Cardiac muscle
Alternative splicing
Antisense oligonucleotides
Muscle
Knockout
ACETYLCHOLINESTERASE
CRISPR/Cas9
Expression
Oligodendrocyte
CONGENITAL MYATHENIC SYNDROME
Animals
ARN
Humans
AAV
Mouse models
Transgenic mouse
Heart failure
Gene therapy