FA is a rare disease and about 500 patients with FA are alive in the U.S.A. The 3 main clinical features of FA are birth defects (missing thumb/radius;microphthalmia;cafe-au-lait spots etc.), progressive marrow failure and cancer suscepfibility [1]. In FA. the life span Is severely shortened [2]. The main cause of death is bone marrow failure with the associated complications of anemia, bleeding and infecfion. Another cause of death is cancer, particulariy acute myelogenous leukemia, but also solid tumors, particulariy squamous cell carcinomas [3].

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Garbati, Michael R; Hays, Laura E; Rathbun, R Keaney et al. (2016) Cytokine overproduction and crosslinker hypersensitivity are unlinked in Fanconi anemia macrophages. J Leukoc Biol 99:455-65
Zhang, Haojian; Kozono, David E; O'Connor, Kevin W et al. (2016) TGF-β Inhibition Rescues Hematopoietic Stem Cell Defects and Bone Marrow Failure in Fanconi Anemia. Cell Stem Cell 18:668-81
Lombardi, Anne J; Hoskins, Elizabeth E; Foglesong, Grant D et al. (2015) Acquisition of Relative Interstrand Crosslinker Resistance and PARP Inhibitor Sensitivity in Fanconi Anemia Head and Neck Cancers. Clin Cancer Res 21:1962-72
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Zhang, Qing-Shuo; Deater, Matthew; Schubert, Kathryn et al. (2015) The Sirt1 activator SRT3025 expands hematopoietic stem and progenitor cells and improves hematopoiesis in Fanconi anemia mice. Stem Cell Res 15:130-40
Zhang, Qing-Shuo; Benedetti, Eric; Deater, Matthew et al. (2015) Oxymetholone therapy of fanconi anemia suppresses osteopontin transcription and induces hematopoietic stem cell cycling. Stem Cell Reports 4:90-102
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Kim, Hyungjin; Dejsuphong, Donniphat; Adelmant, Guillaume et al. (2014) Transcriptional repressor ZBTB1 promotes chromatin remodeling and translesion DNA synthesis. Mol Cell 54:107-18
Fargo, John H; Rochowski, Andrzej; Giri, Neelam et al. (2014) Comparison of chromosome breakage in non-mosaic and mosaic patients with Fanconi anemia, relatives, and patients with other inherited bone marrow failure syndromes. Cytogenet Genome Res 144:15-27
Owen, Nichole; Hejna, James; Rennie, Scott et al. (2014) Bloom syndrome radials are predominantly non-homologous and are suppressed by phosphorylated BLM. Cytogenet Genome Res 144:255-63

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