Introduction Cystic fibrosis (FC) is a disease caused by mutations that compromise the function of CFTR, a membrane protein with ion channel function permeable to chloride and other anions. FC mutations have been grouped into five different classes depending on the mechanism of action. In the last 15 years, the possibility of a pharmacological...
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May 20, 2020 (v1)PublicationUploaded on: April 14, 2023
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2013 (v1)Publication
A basal calpain activity promotes the limited proteolysis of wild type (WT) cystic fibrosis conductance regulator (CFTR), inducing the internalization of the split channel. This process contributes to the regulation in the level of the active CFTR at the plasma membranes. In peripheral blood mononuclear cells (PBMC) from 16 healthy donors, the...
Uploaded on: March 27, 2023 -
2011 (v1)Publication
We are here reporting that in peripheral blood mononuclear cells (PBMC) of patients homozygous for F508del-CFTR the calpainācalpastatin system undergoes a profound alteration. In fact, calpain basal activity, almost undetectable in control PBMC, becomes measurable at a significant extent in cells from cystic fibrosis (CF) patients, also due to...
Uploaded on: April 14, 2023 -
2016 (v1)Publication
Matrix metalloproteinase 9 (MMP9) is physiologically involved in remodeling the extracellular matrix components but its abnormal release has been observed in several human pathologies. We here report that peripheral blood mononuclear cells (PBMCs), isolated from cystic fibrosis (CF) patients homozygous for F508del-cystic fibrosis transmembrane...
Uploaded on: March 27, 2023