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These mutations impair normal mitochondrial functioning via different mechanisms, disturbing the equilibrium between mitochondrial fusion and fission, of mitophagy and mitochondrial axonal transport. Although CMT2A disease causes a significant disability, no resolutive treatment for CMT2A patients to date. In this context, reliable experimental models are essential to precisely dissect the molecular mechanisms of disease and to devise effective therapeutic strategies. The most commonly used models are either in\u00a0vitro or in\u00a0vivo, and among the latter murine models are by far the most versatile and popular. Here, we critically revised the most relevant literature focused on the experimental models, providing an update on the mammalian models of CMT2A developed to date. We highlighted the different phenotypic, histopathological and molecular characteristics, and their use in translational studies for bringing potential therapies from the bench to the bedside. In addition, we discussed limitations of these models and perspectives for future improvement.<\/jats:p>","DOI":"10.1111\/jcmm.18293","type":"journal-article","created":{"date-parts":[[2024,5,9]],"date-time":"2024-05-09T14:39:19Z","timestamp":1715265559000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Charcot\u2013Marie\u2010Tooth type 2A in\u00a0vivo models: Current updates"],"prefix":"10.1111","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0797-8282","authenticated-orcid":false,"given":"Elena","family":"Abati","sequence":"first","affiliation":[{"name":"Neurology Unit, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico  Milan Italy"},{"name":"Department of Pathophysiology and Transplantation, Dino Ferrari Center Universit\u00e0 degli Studi di Milano  Milan 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