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