Characterization of a new B-ALL cell line with constitutional defect of the Notch signaling pathway

Paul Takam Kamga, Giada Dal Collo, Giulio Bassi, Martina Midolo, Massimo Delledonne, Marco Chilosi, Massimiliano Bonifacio, Mauro Krampera

Research output: Contribution to journalArticleAcademicpeer-review

8 Citations (Scopus)
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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.

Original languageEnglish
Pages (from-to)18341-18350
Number of pages10
JournalOncotarget
Volume9
Issue number26
DOIs
Publication statusPublished - 6 Apr 2018
Externally publishedYes

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