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A Yeast-Based Model for Hereditary Motor and Sensory Neuropathies: A Simple System for Complex, Heterogeneous Diseases

1
Neuromuscular Unit, Mossakowski Medical Research Centre Polish Academy of Sciences, 02-106 Warsaw, Poland
2
Institute of Biochemistry and Biophysics Polish Academy of Sciences, 02-106 Warsaw, Poland
*
Author to whom correspondence should be addressed.
Int. J. Mol. Sci. 2020, 21(12), 4277; https://doi.org/10.3390/ijms21124277
Received: 19 May 2020 / Revised: 9 June 2020 / Accepted: 15 June 2020 / Published: 16 June 2020
(This article belongs to the Special Issue Yeast Models and Molecular Mechanisms of Neurodegenerative Diseases)
Charcot–Marie–Tooth (CMT) disease encompasses a group of rare disorders that are characterized by similar clinical manifestations and a high genetic heterogeneity. Such excessive diversity presents many problems. Firstly, it makes a proper genetic diagnosis much more difficult and, even when using the most advanced tools, does not guarantee that the cause of the disease will be revealed. Secondly, the molecular mechanisms underlying the observed symptoms are extremely diverse and are probably different for most of the disease subtypes. Finally, there is no possibility of finding one efficient cure for all, or even the majority of CMT diseases. Every subtype of CMT needs an individual approach backed up by its own research field. Thus, it is little surprise that our knowledge of CMT disease as a whole is selective and therapeutic approaches are limited. There is an urgent need to develop new CMT models to fill the gaps. In this review, we discuss the advantages and disadvantages of yeast as a model system in which to study CMT diseases. We show how this single-cell organism may be used to discriminate between pathogenic variants, to uncover the mechanism of pathogenesis, and to discover new therapies for CMT disease. View Full-Text
Keywords: Charcot-Marie-Tooth disease; neurodegenerative diseases; neuropathy; yeast model organism Charcot-Marie-Tooth disease; neurodegenerative diseases; neuropathy; yeast model organism
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MDPI and ACS Style

Rzepnikowska, W.; Kaminska, J.; Kabzińska, D.; Binięda, K.; Kochański, A. A Yeast-Based Model for Hereditary Motor and Sensory Neuropathies: A Simple System for Complex, Heterogeneous Diseases. Int. J. Mol. Sci. 2020, 21, 4277. https://doi.org/10.3390/ijms21124277

AMA Style

Rzepnikowska W, Kaminska J, Kabzińska D, Binięda K, Kochański A. A Yeast-Based Model for Hereditary Motor and Sensory Neuropathies: A Simple System for Complex, Heterogeneous Diseases. International Journal of Molecular Sciences. 2020; 21(12):4277. https://doi.org/10.3390/ijms21124277

Chicago/Turabian Style

Rzepnikowska, Weronika, Joanna Kaminska, Dagmara Kabzińska, Katarzyna Binięda, and Andrzej Kochański. 2020. "A Yeast-Based Model for Hereditary Motor and Sensory Neuropathies: A Simple System for Complex, Heterogeneous Diseases" International Journal of Molecular Sciences 21, no. 12: 4277. https://doi.org/10.3390/ijms21124277

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