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