From CFTR to a CF signalling network: a systems biology approach to study Cystic Fibrosis.

ABSTRACT:

Background

Cystic Fibrosis (CF) is a monogenic disease caused by mutations in the gene coding the Cystic Fibrosis Transmembrane Regulator (CFTR) protein, but its overall physio-pathology cannot be solely explained by the loss of the CFTR chloride channel function. Indeed, CFTR belongs to a yet not fully deciphered network of proteins participating in various signalling pathways.

Methods

We propose a systems biology approach to study how the absence of the CFTR protein at the membrane leads to perturbation of these pathways, resulting in a panel of deleterious CF cellular phenotypes.

Results

Based on publicly available transcriptomic datasets, we built and analyzed a CF network that recapitulates signalling dysregulations. The CF network topology and its resulting phen

SUBMITTER: Najm M 

PROVIDER: S-EPMC11438383 | biostudies-literature | 2024 Sep

REPOSITORIES: biostudies-literature