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