Replication Protein A (RPA) deficiency activates the Fanconi anemia DNA repair pathway

References

• Wang W. Emergence of a DNA-damage response network consisting of Fanconi anaemia and BRCA proteins. Nat Rev Genet 2007; 8:735-48; PMID:17768402; http://dx.doi.org/10.1038/nrg2159
   PubMed Web of Science ®Google Scholar
• Kim H, D'Andrea AD. Regulation of DNA cross-link repair by the Fanconi anemia/BRCA pathway. Genes Dev 2012; 26:1393-408; PMID:22751496; http://dx.doi.org/10.1101/gad.195248.112
   PubMed Web of Science ®Google Scholar
• Joenje H, Patel KJ. The emerging genetic and molecular basis of Fanconi anaemia. Nat Rev Genet 2001; 2:446-57; PMID:11389461; http://dx.doi.org/10.1038/35076590
   PubMed Web of Science ®Google Scholar
• Bogliolo M, Schuster B, Stoepker C, Derkunt B, Su Y, Raams A, Trujillo JP, Minguillon J, Ramirez MJ, Pujol R, et al. Mutations in ERCC4, encoding the DNA-repair endonuclease XPF, cause Fanconi anemia. Am J Hum Genet 2013; 92:800-6; PMID:23623386; http://dx.doi.org/10.1016/j.ajhg.2013.04.002
   PubMed Web of Science ®Google Scholar
• Hira A, Yoshida K, Sato K, Okuno Y, Shiraishi Y, Chiba K, Tanaka H, Miyano S, Shimamoto A, Tahara H, et al. Mutations in the gene encoding the E2 conjugating enzyme UBE2T cause Fanconi anemia. Am J Hum Genet 2015; 96:1001-7; PMID:26046368; http://dx.doi.org/10.1016/j.ajhg.2015.04.022
   PubMed Web of Science ®Google Scholar
• Rickman KA, Lach FP, Abhyankar A, Donovan FX, Sanborn EM, Kennedy JA, Sougnez C, Gabriel SB, Elemento O, Chandrasekharappa SC, et al. Deficiency of UBE2T, the E2 Ubiquitin ligase necessary for FANCD2 and FANCI Ubiquitination, causes FA-T subtype of fanconi anemia. Cell Rep 2015; 12:35-41; PMID:26119737; http://dx.doi.org/10.1016/j.celrep.2015.06.014
   PubMed Web of Science ®Google Scholar
• Vaz F, Hanenberg H, Schuster B, Barker K, Wiek C, Erven V, Neveling K, Endt D, Kesterton I, Autore F, et al. Mutation of the RAD51C gene in a Fanconi anemia-like disorder. Nat Genet 2010; 42:406-9; PMID:20400963; http://dx.doi.org/10.1038/ng.570
   PubMed Web of Science ®Google Scholar
• Sawyer SL, Tian L, Kahkonen M, Schwartzentruber J, Kircher M, University of Washington Centre for Mendelian Genomics, FORGE Canada Consortium, Majewski J, Dyment DA, Innes AM, Boycott KM, Moreau LA, et al. Biallelic mutations in BRCA1 cause a new Fanconi anemia subtype. Cancer Discov 2015; 5:135-42; PMID:25472942; http://dx.doi.org/10.1158/2159-8290.CD-14-1156
   PubMed Web of Science ®Google Scholar
• Virts EL, Jankowska A, Mackay C, Glaas MF, Wiek C, Kelich SL, Lottmann N, Kennedy FM, Marchal C, Lehnert E, et al. AluY-mediated germline deletion, duplication and somatic stem cell reversion in UBE2T defines a new subtype of Fanconi anemia. Hum Mol Genetics 2015; 24:5093-108; http://dx.doi.org/10.1093/hmg/ddv227
   PubMed Web of Science ®Google Scholar
• Garcia-Higuera I, Taniguchi T, Ganesan S, Meyn MS, Timmers C, Hejna J, Grompe M, D'Andrea AD. Interaction of the Fanconi anemia proteins and BRCA1 in a common pathway. Mol Cell 2001; 7:249-62; PMID:11239454; http://dx.doi.org/10.1016/S1097-2765(01)00173-3
   PubMed Web of Science ®Google Scholar
• Sims AE, Spiteri E, Sims RJ, 3rd, Arita AG, Lach FP, Landers T, Wurm M, Freund M, Neveling K, Hanenberg H, et al. FANCI is a second monoubiquitinated member of the Fanconi anemia pathway. Nat Struct Mol Biol 2007; 14:564-7; PMID:17460694; http://dx.doi.org/10.1038/nsmb1252
   PubMed Web of Science ®Google Scholar
• Smogorzewska A, Matsuoka S, Vinciguerra P, McDonald ER, 3rd, Hurov KE, Luo J, Ballif BA, Gygi SP, Hofmann K, D'Andrea AD, et al. Identification of the FANCI Protein, a Monoubiquitinated FANCD2 Paralog Required for DNA Repair. Cell 2007; 129:289-301; PMID:17412408; http://dx.doi.org/10.1016/j.cell.2007.03.009
   PubMed Web of Science ®Google Scholar
• Iftode C, Daniely Y, Borowiec JA. Replication protein A (RPA): the eukaryotic SSB. Crit Rev Biochem Mol Biol 1999; 34:141-80; PMID:10473346; http://dx.doi.org/10.1080/10409239991209255
   PubMed Web of Science ®Google Scholar
• Wold MS. Replication protein A: a heterotrimeric, single-stranded DNA-binding protein required for eukaryotic DNA metabolism. Annu Rev Biochem 1997; 66:61-92; PMID:9242902; http://dx.doi.org/10.1146/annurev.biochem.66.1.61
   PubMed Web of Science ®Google Scholar
• Flynn RL, Zou L. ATR: a master conductor of cellular responses to DNA replication stress. Trends Biochem Sci 2011; 36:133-40; PMID:20947357; http://dx.doi.org/10.1016/j.tibs.2010.09.005
   PubMed Web of Science ®Google Scholar
• Cimprich KA, Cortez D. ATR: an essential regulator of genome integrity. Nat Rev Mol Cell Biol 2008; 9:616-27; PMID:18594563; http://dx.doi.org/10.1038/nrm2450
   PubMed Web of Science ®Google Scholar
• Zou L, Elledge SJ. Sensing DNA damage through ATRIP recognition of RPA-ssDNA complexes. Science 2003; 300:1542-8; PMID:12791985; http://dx.doi.org/10.1126/science.1083430
   PubMed Web of Science ®Google Scholar
• Ishiai M, Kitao H, Smogorzewska A, Tomida J, Kinomura A, Uchida E, Saberi A, Kinoshita E, Kinoshita-Kikuta E, Koike T, et al. FANCI phosphorylation functions as a molecular switch to turn on the Fanconi anemia pathway. Nat Struct Mol Biol 2008; 15:1138-46; PMID:18931676; http://dx.doi.org/10.1038/nsmb.1504
   PubMed Web of Science ®Google Scholar
• Wang X, Kennedy RD, Ray K, Stuckert P, Ellenberger T, D'Andrea AD. Chk1-mediated phosphorylation of FANCE is required for the Fanconi anemia/BRCA pathway. Mol Cell Biol 2007; 27:3098-108; PMID:17296736; http://dx.doi.org/10.1128/MCB.02357-06
   PubMed Web of Science ®Google Scholar
• Singh TR, Ali AM, Paramasivam M, Pradhan A, Wahengbam K, Seidman MM, Meetei AR. ATR-dependent phosphorylation of FANCM at serine 1045 is essential for FANCM functions. Cancer Res 2013; 73:4300-10; PMID:23698467; http://dx.doi.org/10.1158/0008-5472.CAN-12-3976
   PubMed Web of Science ®Google Scholar
• Taniguchi T, Garcia-Higuera I, Xu B, Andreassen PR, Gregory RC, Kim ST, Lane WS, Kastan MB, D'Andrea AD. Convergence of the fanconi anemia and ataxia telangiectasia signaling pathways. Cell 2002; 109:459-72; PMID:12086603; http://dx.doi.org/10.1016/S0092-8674(02)00747-X
   PubMed Web of Science ®Google Scholar
• Collins NB, Wilson JB, Bush T, Thomashevski A, Roberts KJ, Jones NJ, Kupfer GM. ATR-dependent phosphorylation of FANCA on serine 1449 after DNA damage is important for FA pathway function. Blood 2009; 113:2181-90; PMID:19109555; http://dx.doi.org/10.1182/blood-2008-05-154294
   PubMed Web of Science ®Google Scholar
• Andreassen PR, D'Andrea AD, Taniguchi T. ATR couples FANCD2 monoubiquitination to the DNA-damage response. Genes Dev 2004; 18:1958-63; PMID:15314022; http://dx.doi.org/10.1101/gad.1196104
   PubMed Web of Science ®Google Scholar
• D'Andrea AD, Grompe M. The Fanconi anaemia/BRCA pathway. Nat Rev Cancer 2003; 3:23-34; PMID:12509764; http://dx.doi.org/10.1038/nrc970
   PubMed Web of Science ®Google Scholar
• Paulsen RD, Soni DV, Wollman R, Hahn AT, Yee MC, Guan A, Hesley JA, Miller SC, Cromwell EF, Solow-Cordero DE, et al. A genome-wide siRNA screen reveals diverse cellular processes and pathways that mediate genome stability. Mol Cell 2009; 35:228-39; PMID:19647519; http://dx.doi.org/10.1016/j.molcel.2009.06.021
   PubMed Web of Science ®Google Scholar
• Chou DM, Elledge SJ. Tipin and Timeless form a mutually protective complex required for genotoxic stress resistance and checkpoint function. Proc Natl Acad Sci U S A 2006; 103:18143-7; PMID:17116885; http://dx.doi.org/10.1073/pnas.0609251103
   PubMed Web of Science ®Google Scholar
• Yoshizawa-Sugata N, Masai H. Human Tim/Timeless-interacting protein, Tipin, is required for efficient progression of S phase and DNA replication checkpoint. J Biol Chem 2007; 282:2729-40; PMID:17102137; http://dx.doi.org/10.1074/jbc.M605596200
   PubMed Web of Science ®Google Scholar
• Kim JM, Parmar K, Huang M, Weinstock DM, Ruit CA, Kutok JL, D'Andrea AD. Inactivation of murine Usp1 results in genomic instability and a Fanconi anemia phenotype. Dev Cell 2009; 16:314-20; PMID:19217432; http://dx.doi.org/10.1016/j.devcel.2009.01.001
   PubMed Web of Science ®Google Scholar
• Nijman SM, Huang TT, Dirac AM, Brummelkamp TR, Kerkhoven RM, D'Andrea AD, Bernards R. The deubiquitinating enzyme USP1 regulates the Fanconi anemia pathway. Mol Cell 2005; 17:331-9; PMID:15694335; http://dx.doi.org/10.1016/j.molcel.2005.01.008
   PubMed Web of Science ®Google Scholar
• Oestergaard VH, Langevin F, Kuiken HJ, Pace P, Niedzwiedz W, Simpson LJ, Ohzeki M, Takata M, Sale JE, Patel KJ. Deubiquitination of FANCD2 is required for DNA crosslink repair. Mol Cell 2007; 28:798-809; PMID:18082605; http://dx.doi.org/10.1016/j.molcel.2007.09.020
   PubMed Web of Science ®Google Scholar
• Huang TT, Nijman SM, Mirchandani K, Galardy PJ, Cohn MA, Haas W, Gygi SP, Ploegh HL, Bernards R, D'Andrea AD. Regulation of monoubiquitinated PCNA by DUB autocleavage. Nat Cell Biol 2006; 8:339-47; PMID:16531995
   PubMed Web of Science ®Google Scholar
• Kim JM, Kee Y, Gurtan A, D'Andrea AD. Cell cycle-dependent chromatin loading of the Fanconi anemia core complex by FANCM/FAAP24. Blood 2008; 111:5215-22; PMID:18174376; http://dx.doi.org/10.1182/blood-2007-09-113092
   PubMed Web of Science ®Google Scholar
• Longerich S, Li J, Xiong Y, Sung P, Kupfer GM. Stress and DNA repair biology of the Fanconi anemia pathway. Blood 2014; 124:2812-9; PMID:25237197; http://dx.doi.org/10.1182/blood-2014-04-526293
   PubMed Web of Science ®Google Scholar
• Yeo JE, Lee EH, Hendrickson EA, Sobeck A. CtIP mediates replication fork recovery in a FANCD2-regulated manner. Hum Mol Genetics 2014; 23:3695-705; http://dx.doi.org/10.1093/hmg/ddu078
   PubMed Web of Science ®Google Scholar
• Dodson GE, Shi Y, Tibbetts RS. DNA replication defects, spontaneous DNA damage, and ATM-dependent checkpoint activation in replication protein A-deficient cells. J Biol Chem 2004; 279:34010-4; PMID:15197179; http://dx.doi.org/10.1074/jbc.C400242200
   PubMed Web of Science ®Google Scholar
• Kar A, Kaur M, Ghosh T, Khan MM, Sharma A, Shekhar R, Varshney A, Saxena S. RPA70 depletion induces hSSB1/2-INTS3 complex to initiate ATR signaling. Nucleic Acids Res 2015; 43:4962-74; PMID:25916848; http://dx.doi.org/10.1093/nar/gkv369
   PubMed Web of Science ®Google Scholar
• Richard DJ, Bolderson E, Cubeddu L, Wadsworth RI, Savage K, Sharma GG, Nicolette ML, Tsvetanov S, McIlwraith MJ, Pandita RK, et al. Single-stranded DNA-binding protein hSSB1 is critical for genomic stability. Nature 2008; 453:677-81; PMID:18449195; http://dx.doi.org/10.1038/nature06883
   PubMed Web of Science ®Google Scholar
• Skaar JR, Richard DJ, Saraf A, Toschi A, Bolderson E, Florens L, Washburn MP, Khanna KK, Pagano M. INTS3 controls the hSSB1-mediated DNA damage response. J Cell Biol 2009; 187:25-32; PMID:19786574; http://dx.doi.org/10.1083/jcb.200907026
   PubMed Web of Science ®Google Scholar
• Jones MJ, Huang TT. The Fanconi anemia pathway in replication stress and DNA crosslink repair. Cell Mol Life Sci 2012; 69:3963-74; PMID:22744751; http://dx.doi.org/10.1007/s00018-012-1051-0
   PubMed Web of Science ®Google Scholar
• Raghunandan M, Chaudhury I, Kelich SL, Hanenberg H, Sobeck A. FANCD2, FANCJ and BRCA2 cooperate to promote replication fork recovery independently of the Fanconi Anemia core complex. Cell Cycle 2015; 14:342-53; PMID:25659033; http://dx.doi.org/10.4161/15384101.2014.987614
   PubMed Web of Science ®Google Scholar
• Wang LC, Stone S, Hoatlin ME, Gautier J. Fanconi anemia proteins stabilize replication forks. DNA Repair (Amst) 2008; 7:1973-81; PMID:18786657; http://dx.doi.org/10.1016/j.dnarep.2008.08.005
   PubMed Web of Science ®Google Scholar
• Wang Y, Putnam CD, Kane MF, Zhang W, Edelmann L, Russell R, Carrion DV, Chin L, Kucherlapati R, Kolodner RD, et al. Mutation in Rpa1 results in defective DNA double-strand break repair, chromosomal instability and cancer in mice. Nat Genet 2005; 37:750-5; PMID:15965476; http://dx.doi.org/10.1038/ng1587
   PubMed Web of Science ®Google Scholar
• Moldovan GL, D'Andrea AD. To the rescue: the Fanconi anemia genome stability pathway salvages replication forks. Cancer Cell 2012; 22:5-6; PMID:22789533; http://dx.doi.org/10.1016/j.ccr.2012.06.006
   PubMed Web of Science ®Google Scholar
• Lossaint G, Larroque M, Ribeyre C, Bec N, Larroque C, Decaillet C, Gari K, Constantinou A. FANCD2 binds MCM proteins and controls replisome function upon activation of s phase checkpoint signaling. Mol Cell 2013; 51:678-90; PMID:23993743; http://dx.doi.org/10.1016/j.molcel.2013.07.023
   PubMed Web of Science ®Google Scholar
• Chen YH, Jones MJ, Yin Y, Crist SB, Colnaghi L, Sims RJ, 3rd, Rothenberg E, Jallepalli PV, Huang TT. ATR-mediated phosphorylation of FANCI regulates dormant origin firing in response to replication stress. Mol Cell 2015; 58:323-38; PMID:25843623; http://dx.doi.org/10.1016/j.molcel.2015.02.031
   PubMed Web of Science ®Google Scholar
• Sobeck A, Stone S, Landais I, de Graaf B, Hoatlin ME. The Fanconi anemia protein FANCM is controlled by FANCD2 and the ATR/ATM pathways. J Biol Chem 2009; 284:25560-8; PMID:19633289; http://dx.doi.org/10.1074/jbc.M109.007690
   PubMed Web of Science ®Google Scholar
• Nagamani SC, Zhang F, Shchelochkov OA, Bi W, Ou Z, Scaglia F, Probst FJ, Shinawi M, Eng C, Hunter JV, et al. Microdeletions including YWHAE in the Miller-Dieker syndrome region on chromosome 17p13.3 result in facial dysmorphisms, growth restriction, and cognitive impairment. J Medical Genetics 2009; 46:825-33; http://dx.doi.org/10.1136/jmg.2009.067637
   PubMed Web of Science ®Google Scholar
• Neher TM, Bodenmiller D, Fitch RW, Jalal SI, Turchi JJ. Novel irreversible small molecule inhibitors of replication protein A display single-agent activity and synergize with cisplatin. Mol Cancer Therapeutics 2011; 10:1796-806; http://dx.doi.org/10.1158/1535-7163.MCT-11-0303
   PubMed Web of Science ®Google Scholar
• Shuck SC, Turchi JJ. Targeted inhibition of replication protein a reveals cytotoxic activity, synergy with chemotherapeutic DNA-damaging agents, and insight into cellular function. Cancer Res 2010; 70:3189-98; PMID:20395205; http://dx.doi.org/10.1158/0008-5472.CAN-09-3422
   PubMed Web of Science ®Google Scholar