The Fanconi anemia pathway in cancer

J Niraj, A Färkkilä, AD D'Andrea - Annual review of cancer …, 2019 - annualreviews.org
Fanconi anemia (FA) is a complex genetic disorder characterized by bone marrow failure
(BMF), congenital defects, inability to repair DNA interstrand cross-links (ICLs), and cancer
predisposition. FA presents two seemingly opposite characteristics:(a) massive cell death of
the hematopoietic stem and progenitor cell (HSPC) compartment due to extensive genomic
instability, leading to BMF, and (b) uncontrolled cell proliferation leading to FA-associated
malignancies. The canonical function of the FA proteins is to collaborate with several other …