Jessica Bordini, Chiara Lenzi, Michela Frenquelli, Alessia Morabito, Athanasios Pseftogas, Daniela Belloni, Larry Mansouri, George Tsiolas, Eleonora Perotta, Pamela Ranghetti, Francesca Gandini, Francesca Genova, Daniel Hägerstrand, Georgios Gavriilidis, Sofoklis Keisaris, Nikolaos Pechlivanis, Frederic Davi, Neil E Kay, Anton W Langerak, Sarka Pospisilova, Lydia Scarfò, Antonios Makris, Fotis E Psomopoulos, Kostas Stamatopoulos, Richard Rosenquist, Alessandro Campanella, Paolo Ghia
The NFKBIE gene, which encodes the NF-κB inhibitor IκBε, is mutated in 3-7% of patients with chronic lymphocytic leukemia (CLL). The most recurrent alteration is a 4-bp frameshift deletion associated with NF-κB activation in leukemic B cells and poor clinical outcome. To study the functional consequences of NFKBIE gene inactivation, both in vitro and in vivo, we engineered CLL B cells and CLL-prone mice to stably down-regulate NFKBIE expression and investigated its role in controlling NF-κB activity and disease expansion...
April 4, 2024: Leukemia