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Article

The proteostatic network chaperome is downregulated in F508del homozygote cystic fibrosis.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Mar 2021

Sala Marc A, Alexander Michael, Khuder Basil, Politanska Yuliya, Abdala-Valencia Hiam, Budinger G R Scott, Liu Jing, Jain Manu, Reyfman Paul A

Abstract excerpt

BACKGROUND: CF patients demonstrate clinical heterogeneity and much remains unknown about how to risk stratify individuals for disease progression. The most common cystic fibrosis mutation, F508del, is a protein folding mutation that has been shown in vitro to negatively affect proteostasis and CFTR transcription. Since CFTR is expressed in the nasal epithelium, we hypothesized that by using unbiased...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 33495079
DOI: 10.1016/j.jcf.2020.12.018
PMCID: PMC8900221

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