title: Expanded roles of the Fanconi anemia pathway in preserving genomic stability.
abstract: Studying rare human genetic diseases often leads to a better understanding of normal cellular functions. Fanconi anemia (FA), for example, has elucidated a novel DNA repair mechanism required for maintaining genomic stability and preventing cancer. The FA pathway, an essential tumor-suppressive pathway, is required for protecting the human genome from a specific type of DNA damage; namely, DNA interstrand cross-links (ICLs). In this review, we discuss the recent progress in the study of the FA pathway, such as the identification of new FANCM-binding partners and the identification of RAD51C and FAN1 (Fanconi-associated nuclease 1) as new FA pathway-related proteins. We also focus on the role of the FA pathway as a potential regulator of DNA repair choices in response to double-strand breaks, and its novel functions during the mitotic phase of the cell cycle.
authors: Department of Radiation Oncology and Pediatric Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA 02115, USA.
authors: Younghoon
authors: Y
authors: Kee
authors: Alan D
authors: AD
authors: D'Andrea
mesh_terms: D000818:Animals; D004249:DNA Damage / Q000235:genetics; D004260:DNA Repair / Q000235:genetics; D004268:DNA-Binding Proteins / Q000235:genetics / Q000378:metabolism; D004706:Endodeoxyribonucleases; D005090:Exodeoxyribonucleases / Q000378:metabolism; D005199:Fanconi Anemia / Q000235:genetics* / Q000503:physiopathology*; D042822:Genomic Instability / Q000235:genetics*; D006801:Humans; D064251:Multifunctional Enzymes; D009154:Mutation; D011485:Protein Binding; D025521:Tumor Suppressor Proteins / Q000378:metabolism
chemical_list: D004268:DNA-Binding Proteins; D064251:Multifunctional Enzymes; C448305:RAD51C protein, human; D025521:Tumor Suppressor Proteins; D004706:Endodeoxyribonucleases; D005090:Exodeoxyribonucleases; C551063:FAN1 protein, human