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It can be inherited through autosomal dominant (AD), autosomal recessive (AR), or X\u2010linked genetic patterns. CMT2, one of the primary subtypes, is characterized by axonal degeneration and commonly presents with muscle weakness, atrophy, foot deformities, and sensory loss. Aminoacyl\u2010tRNA synthetases (aaRSs) play an important role in the genetic underpinnings of CMT2, with more than 60 disease\u2010causing alleles identified across eight different aaRSs, including alanyl\u2010, asparaginyl\u2010, histidyl\u2010, glycyl\u2010, methionyl\u2010, tryptophanyl\u2010, seryl\u2010, and tyrosyl\u2010tRNA synthetases. Mutations in aaRS genes can lead to destabilization of the enzyme, reduced aminoacylation, and aberrant protein complex formation. Yeast as a simple organism provides a robust model system to study the pathogenic effects of aaRS CMT mutations. In this review, we discuss the advantages and limitations of the yeast model systems for CMT2\u2010causative mutations in aaRS.<\/jats:p>","DOI":"10.1002\/iub.70017","type":"journal-article","created":{"date-parts":[[2025,3,31]],"date-time":"2025-03-31T03:56:23Z","timestamp":1743393383000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Yeast models for Charcot\u2010Marie\u2010Tooth disease\u2010causing aminoacyl\u2010\n                    <scp>tRNA<\/scp>\n                    synthetase alleles reveal the cellular basis of disease"],"prefix":"10.1002","volume":"77","author":[{"given":"Maria","family":"Mahmood","sequence":"first","affiliation":[{"name":"Department of Biochemistry The University of Western Ontario  London Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emma","family":"Little","sequence":"additional","affiliation":[{"name":"School of Integrated Science, Sustainability, 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