Translational behavioral phenotypes in DMSXL mice for CNS manifestations of DM1
This study defines a constellation of behavioral impairments in DMSXL mice that mirror CNS symptoms in DM1 patients and establishes a set of sensitive, age-dependent endpoints suitable for CNS-targeted therapeutic evaluation. The behavioral framework presented here offers valuable guidance for the design of future preclinical trials in DM1.
Authors
- Germana Falcone (ORCID: https://orcid.org/0000-0002-3508-7623)
- Silvia Mandillo (ORCID: https://orcid.org/0000-0002-4869-0974)
- Hélène Benyamine
- Elisabetta Golini
- Beatrice Cardinali (ORCID: https://orcid.org/0000-0002-3333-3384)
- Geneviève Gourdon (ORCID: https://orcid.org/0000-0002-3319-1347)
- Aline Huguet-Lachon
- Marcello Raspa
- Noëmy Forasté Gueriba
Institutions
- Inserm (FR)
- Sorbonne Université (FR)
- Institut de Myologie (FR)
- National Research Council (RO)
- Institute of Cell Biology and Neurobiology (IT)
- Institut des Maladies Génétiques Imagine (FR)
- Centre de Recherche en Myologie
Publication Details
- Journal
- Journal of Neuromuscular Diseases
- Published
- 2026-01-14
- DOI
- https://doi.org/10.1177/22143602251410998
- Primary Topic
- Genetic Neurodegenerative Diseases
- Type
- article
- Field-Weighted Citation Impact
- 0.00