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Downregulation of PMP22 ameliorates myelin defects in iPSC-derived human organoid cultures of CMT1A
Charcot–Marie–Tooth disease is the most common inherited disorder of the PNS. CMT1A accounts for 40–50% of all cases and is caused by a duplication of the PMP22 gene on chromosome 17, leading to dysmyelination in the PNS. Patient-derived models to study such myelination defects are lacking as the in...
Autores principales: | , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Oxford University Press
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10316758/ https://www.ncbi.nlm.nih.gov/pubmed/36511878 http://dx.doi.org/10.1093/brain/awac475 |