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