CFTR trafficking mutations disrupt cotranslational protein folding by targeting biosynthetic intermediates

Cystic fibrosis (CF) is a lethal genetic disease that is primarily caused by misfolding of the cystic fibrosis transmembrane conductance regulator (CFTR). Here authors show that disease-causing mutations located within the first nucleotide binding domain of CFTR have distinct effects on nascent poly...

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Autores principales: Hideki Shishido, Jae Seok Yoon, Zhongying Yang, William R. Skach
Formato: article
Lenguaje:EN
Publicado: Nature Portfolio 2020
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Acceso en línea:https://doaj.org/article/da97e209777a41fe8119566b8058db29
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Sumario:Cystic fibrosis (CF) is a lethal genetic disease that is primarily caused by misfolding of the cystic fibrosis transmembrane conductance regulator (CFTR). Here authors show that disease-causing mutations located within the first nucleotide binding domain of CFTR have distinct effects on nascent polypeptides.