Charcot-Marie-Tooth disease 1A (CMT1A) is an inherited neuropathy caused by duplication of the gene encoding peripheral myelin protein 22 (PMP22). In this study, PMP22 duplication in rodents caused defects in Schwann cell differentiation in early postnatal development, which persisted into adulthood. This abnormal Schwann cell phenotype could be rescued by axonal overexpression of neuregulin-1 (NRG1) or by treatment with soluble NRG1 in early postnatal stages. These data suggest that there is a temporal window for correct Schwann cell differentiation that is vital to prevent the neuropathy observed in CMT1A.
References
Fledrich, R. et al. Soluble neuregulin-1 modulates disease pathogenesis in rodent models of Charcot-Marie-Tooth disease 1A. Nature Med. http://dx.doi.org/10.1038/nm.3664 (2014)
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Lewis, S. Schwann cells keep axons healthy. Nat Rev Neurosci 15, 632 (2014). https://doi.org/10.1038/nrn3831
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DOI: https://doi.org/10.1038/nrn3831
