Article
First-hit SETBP1 mutations cause a myeloproliferative disorder with bone marrow fibrosis.
Blood - 4 Apr 2024
Crespiatico Ilaria, Zaghi Mattia, Mastini Cristina, D'Aliberti Deborah, Mauri Mario, Mercado Carl Mirko, Fontana Diletta, Spinelli Silvia, Crippa Valentina, Inzoli Elena, Manghisi Beatrice, Civettini Ivan, Ramazzotti Daniele, Sangiorgio Valentina, Gengotti Michele, Brambilla Virginia, Aroldi Andrea, Banfi Federica, Barone Cristiana, Orsenigo Roberto, Riera Ludovica, Riminucci Mara, Corsi Alessandro, Breccia Massimo, Morotti Alessandro, Cilloni Daniela, Roccaro Aldo, Sacco Antonio, Stagno Fabio, Serafini Marta, Mottadelli Federica, Cazzaniga Giovanni, Pagni Fabio, Chiarle Roberto, Azzoni Emanuele, Sessa Alessandro, Gambacorti-Passerini Carlo, Elli Elena Maria, Mologni Luca, Piazza Rocco
Abstract excerpt
ABSTRACT: SETBP1 mutations are found in various clonal myeloid disorders. However, it is unclear whether they can initiate leukemia, because SETBP1 mutations typically appear as later events during oncogenesis. To answer this question, we generated a mouse model expressing mutated SETBP1 in hematopoietic tissue: this model showed profound alterations in the differentiation program of hematopoietic progenitors and...
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