Research Papers:
Characterization of a new B-ALL cell line with constitutional defect of the Notch signaling pathway
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Abstract
Paul Takam Kamga1, Giada Dal Collo1, Giulio Bassi1, Martina Midolo1, Massimo Delledonne2,3, Marco Chilosi4, Massimiliano Bonifacio1 and Mauro Krampera1
1Stem Cell Research Laboratory, Section of Hematology, Department of Medicine, University of Verona, Verona, Italy
2Department of Biotechnology, University of Verona, Verona, Italy
3Personal Genomics S.R.L., Verona, Italy
4Section of Pathology, Department of Diagnostics and Public Health, University of Verona, Verona, Italy
Correspondence to:
Mauro Krampera, email: [email protected]
Keywords: Notch signaling; B-acute lymphoblastic leukemia; B-ALL; Alagille syndrome; ALGS
Received: January 02, 2018 Accepted: March 11, 2018 Published: April 06, 2018
ABSTRACT
Notch signaling contribution to B-cell acute lymphoblastic leukemia (B-ALL) development is still under investigation. 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. VR-ALL is a common-type B-ALL cell line, grows in conventional culture medium supplemented with 10% serum, and gives rise, once injected into immunodeficient NOG mice, to a mouse xenograft model of B-ALL. Exome sequencing revealed deleterious mutations in some components of Notch signaling, including Jagged1, Notch1, and Notch2. In addition, VR-ALL is sensitive both in vitro and in vivo to γ-secretase inhibitors (GSIs) as well as conventional anti-leukemic drugs. For all these reasons, VR-ALL may help to gain more insights into the role of Notch signaling in B-ALL.
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