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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
131
Publications avec texte intégral
Open Access
52 %
Mots clés
Expression
Cell model
RNA splicing
Dystrophin
Genotype phenotype correlation
Myotonic dystrophy
Cell penetrating peptide
PCR
Cardiac muscle
Thérapie génique
CONGENITAL MYATHENIC SYNDROME
Cytoskeleton
Gene therapy
Trinucleotide Repeat Expansion
Myotonic Dystrophy type 1
CTG repeat instability
Oligodendrocyte
ARN
RNA interference
Glial cells
Transgenic mouse model
Trinucleotide repeat expansion
Mouse model
MBNL
CTG repeat contractions
Lc3
Gene editing
Myotonic dystrophy mouse models
Mice
Acetylcholinesterase knockout mouse
Alternative splicing
Quantitative microdialysis
Brain
Animals
CRISPR/Cas9
Central nervous system
Intermediate filament
GABA
Myotonic Dystrophy
Cell culture model
Long read sequencing
Autophagy
Muscular dystrophy
CMS
Desmin
AAV
Duchenne muscular dystrophy
Mouse models
Antisense oligonucleotide
Exercise
Centronuclear myopathy
Therapy
Humans
Dilated cardiomyopathy
BIOLOGIE MOLECULAIRE
RNA biology
GSK3
Astrocytes
DM1
CTG repeats
Heart
Hypoxia
Dystrophie Myotonique
Antisense oligonucleotides
Muscle
Exercice
Glucocorticoid-receptor
Motoneuron
Acute coronary syndrome
Glutamate
Acetylcholinesterase deficiency
Glucocorticoids
CRISPRi
Dynamin 2
Oligodendrocytes
ACETYLCHOLINESTERASE
Skeletal muscle
Maximal force
Male
DMSXL mice
Aging
Heart failure
Dystrophie myotonique
Transgenic mouse
In vivo
Diaphragm
Myostatin
Knockout
Myotonic dystrophy type 1
Fibrosis
Gene Therapy
DMPK
Myelin
Neuron
KNOCKOUT MICE
Astrocyte
Brain dysfunction
Transcriptomics
PacBio
Myotonic Dystrophy Type 1