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Phosphorylation of the Chaperone-Like HspB5 Rescues Trafficking and Function of F508del-CFTR

Cystic Fibrosis is a lethal monogenic autosomal recessive disease linked to mutations in Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) protein. The most frequent mutation is the deletion of phenylalanine at position 508 of the protein. This F508del-CFTR mutation leads to misfolded prote...

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Detalles Bibliográficos
Autores principales: Degrugillier, Fanny, Aissat, Abdel, Prulière-Escabasse, Virginie, Bizard, Lucie, Simonneau, Benjamin, Decrouy, Xavier, Jiang, Chong, Rotin, Daniela, Fanen, Pascale, Simon, Stéphanie
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7402320/
https://www.ncbi.nlm.nih.gov/pubmed/32650630
http://dx.doi.org/10.3390/ijms21144844