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Publication : Leukemia and chromosomal instability in aged Fancc-/- mice.

First Author  Cerabona D Year  2016
Journal  Exp Hematol Volume  44
Issue  5 Pages  352-7
PubMed ID  26860989 Mgi Jnum  J:317004
Mgi Id  MGI:6843941 Doi  10.1016/j.exphem.2016.01.009
Citation  Cerabona D, et al. (2016) Leukemia and chromosomal instability in aged Fancc-/- mice. Exp Hematol 44(5):352-7
abstractText  Fanconi anemia (FA) is an inherited disorder of genomic instability associated with high risk of myelodysplasia and acute myeloid leukemia (AML). Young mice deficient in FA core complex genes do not naturally develop cancer, hampering preclinical studies on malignant hematopoiesis in FA. Here we describe that aging Fancc(-/-) mice are prone to genomically unstable AML and other hematologic neoplasms. We report that aneuploidy precedes malignant transformation during Fancc(-/-) hematopoiesis. Our observations reveal that Fancc(-/-) mice develop hematopoietic chromosomal instability followed by leukemia in an age-dependent manner, recapitulating the clinical phenotype of human FA and providing a proof of concept for future development of preclinical models of FA-associated leukemogenesis.
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