Characterization of a new B-ALL cell line with constitutional defect of the Notch signaling pathway
Notch signaling contribution to B-cell acute lymphoblastic leukemia (B-ALL) development is still under investigation.
Autori: Kamga PT, Dal Collo G, Bassi G, Midolo M, Delledonne M, Chilosi M, Bonifacio M, Krampera M.
Oncotarget. 2018 Apr 6;9(26):18341-18350.
The serendipitous onset of B-ALL in a patient affected by the germinal Notch mutation-dependent Alagille syndrome allowed us to establish a B-ALL cell line (VR-ALL) bearing a genetic loss of function in components of Notch signaling. Exome sequencing revealed deleterious mutations in some components of Notch signaling. In addition, VR-ALL is sensitive both in vitro and in vivo to ?-secretase inhibitors (GSIs) as well as conventional anti-leukemic drugs.