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CFTR Rescue by Lumacaftor (VX-809) Induces an Extensive Reorganization of Mitochondria in the Cystic Fibrosis Bronchial Epithelium

Background: Cystic Fibrosis (CF) is a genetic disorder affecting around 1 in every 3000 newborns. In the most common mutation, F508del, the defective anion channel, CFTR, is prevented from reaching the plasma membrane (PM) by the quality check control of the cell. Little is known about how CFTR phar...

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Detalles Bibliográficos
Autores principales: Braccia, Clarissa, Christopher, Josie A., Crook, Oliver M., Breckels, Lisa M., Queiroz, Rayner M. L., Liessi, Nara, Tomati, Valeria, Capurro, Valeria, Bandiera, Tiziano, Baldassari, Simona, Pedemonte, Nicoletta, Lilley, Kathryn S., Armirotti, Andrea
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9222197/
https://www.ncbi.nlm.nih.gov/pubmed/35741067
http://dx.doi.org/10.3390/cells11121938