Article
Calpain digestion and HSP90-based chaperone protection modulate the level of plasma membrane F508del-CFTR.
Biochimica et biophysica acta - 1 Jan 2011
Averna Monica, Stifanese Roberto, Grosso Raffaella, Pedrazzi Marco, De Tullio Roberta, Salamino Franca, Sparatore Bianca, Pontremoli Sandro, Melloni Edon
Abstract excerpt
We are here showing that peripheral mononuclear blood cells (PBMC) from cystic fibrosis (CF) patients contain almost undetectable amounts of mature 170 kDa CF-transmembrane conductance regulator (CFTR) and a highly represented 100 kDa form. This CFTR protein, resembling the form produced by calpain digestion and present, although in lower amounts, also in normal PBMC, is localized in cytoplasmic internal...
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