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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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Antonio Atalaia, Dagmar Wandrei, Nawel Lalout, Rachel Thompson, Adrian Tassoni, et al.. EURO-NMD registry: federated FAIR infrastructure, innovative technologies and concepts of a patient-centred registry for rare neuromuscular disorders. Orphanet Journal of Rare Diseases, 2024, 19 (1), pp.66. ⟨10.1186/s13023-024-03059-3⟩. ⟨hal-04460667⟩
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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
134
Publications avec texte intégral
Open Access
52 %
Mots clés
CONGENITAL MYATHENIC SYNDROME
Autophagy
ACETYLCHOLINESTERASE
Central nervous system
CTG repeat contractions
Maximal force
KNOCKOUT MICE
Expression
Heart failure
Antisense oligonucleotide
In vivo
BIOLOGIE MOLECULAIRE
Muscular dystrophy
Hypoxia
Long read sequencing
Motoneuron
Antisense oligonucleotides
Myotonic dystrophy mouse models
AAV
CRISPR/Cas9
MBNL
Genotype phenotype correlation
Mice
Desmin
CMS
RNA interference
Acetylcholinesterase knockout mouse
Intermediate filament
Glucocorticoid-receptor
Cell penetrating peptide
Cell culture model
RNA biology
Brain dysfunction
Myelin
Diaphragm
Exercice
Duchenne muscular dystrophy
Dilated cardiomyopathy
Oligodendrocytes
Heart
GABA
Transgenic mouse
Gene editing
Muscle
PCR
Centronuclear myopathy
Male
PacBio
Lc3
Dystrophin
Myotonic dystrophy
Myotonic Dystrophy Type 1
Oligodendrocyte
Myotonic dystrophy type 1
Cytoskeleton
Exercise
RNA splicing
ARN
Knockout
Gene therapy
CTG repeat instability
Fibrosis
Glutamate
Therapy
Acute coronary syndrome
Astrocytes
Glial cells
Trinucleotide repeat expansion
Brain
Astrocyte
Myotonic Dystrophy
Gene Therapy
Aging
Neuron
Alternative splicing
Transgenic mouse model
Glucocorticoids
Thérapie génique
Mouse models
Animals
Cardiac muscle
Trinucleotide Repeat Expansion
Dystrophie myotonique
Skeletal muscle
DMPK
Cell model
Transcriptomics
Humans
Mouse model
CTG repeats
DM1
Acetylcholinesterase deficiency
CRISPRi
Quantitative microdialysis
Dystrophie Myotonique
Dynamin 2
GSK3
DMSXL mice
Myostatin
Myotonic Dystrophy type 1