Selected Publications | National Institute of Environmental Health Sciences Skip Navigation Selected Publications Close the left navigation Add Structural Cell Biology Group Full list of Publications Kuster A, Mozaffari NL, Wilkinson OJ, Wojtaszek JL, Zurfluh C, Przetocka S, Zyla D, von Aesch C, Dillingham MS, Williams RS, Sartori AA. A stapled peptide mimetic of the CtIP tetramerization motif interferes with double-strand break repair and replication fork protection. Sci Adv . 2021 Feb 19;7(8):eabc6381. doi: 10.1126/sciadv.abc6381. Print 2021 Feb. PMID: 33608267. [ Abstract Kuster A, Mozaffari NL, Wilkinson OJ, Wojtaszek JL, Zurfluh C, Przetocka S, Zyla D, von Aesch C, Dillingham MS, Williams RS, Sartori AA. A stapled peptide mimetic of the CtIP tetramerization motif interferes with double-strand break repair and replication fork protection. Sci Adv. 2021 Feb 19;7(8):eabc6381. doi: 10.1126/sciadv.abc6381. Print 2021 Feb. PMID: 33608267. ] [ Full Text Kuster A, Mozaffari NL, Wilkinson OJ, Wojtaszek JL, Zurfluh C, Przetocka S, Zyla D, von Aesch C, Dillingham MS, Williams RS, Sartori AA. A stapled peptide mimetic of the CtIP tetramerization motif interferes with double-strand break repair and replication fork protection. Sci Adv. 2021 Feb 19;7(8):eabc6381. doi: 10.1126/sciadv.abc6381. Print 2021 Feb. PMID: 33608267. Williams JS, Tumbale PP, Arana ME, Rana JA, Williams RS, Kunkel TA. 2021. High-fidelity DNA ligation enforces accurate Okazaki fragment maturation during DNA replication. Nature Communications 12:482. PMCID: PMC7817679. [ Abstract Williams JS, Tumbale PP, Arana ME, Rana JA, Williams RS, Kunkel TA. 2021. High-fidelity DNA ligation enforces accurate Okazaki fragment maturation during DNA replication. Nature Communications 12:482. PMCID: PMC7817679. ] [ Full Text Williams JS, Tumbale PP, Arana ME, Rana JA, Williams RS, Kunkel TA. 2021. High-fidelity DNA ligation enforces accurate Okazaki fragment maturation during DNA replication. Nature Communications 12:482. PMCID: PMC7817679. Jurkiw TJ, Tumbale PP, Schellenberg MJ, Cunningham-Rundles C, Williams RS, O'Brien PJ. LIG1 syndrome mutations remodel a cooperative network of ligand binding interactions to compromise ligation efficiency. Nucleic Acids Res . 2021. doi: 10.1093/nar/gkaa1297 [Online 14 January 2021]. [ Abstract Jurkiw TJ, Tumbale PP, Schellenberg MJ, Cunningham-Rundles C, Williams RS, O'Brien PJ. LIG1 syndrome mutations remodel a cooperative network of ligand binding interactions to compromise ligation efficiency. Nucleic Acids Res. 2021. doi: 10.1093/nar/gkaa1297 [Online 14 January 2021]. Rodriguez AA, Wojtaszek JL, Greer BH, Haldar T, Gates KS, Williams RS, Eichman BF. 2020. An autoinhibitory role for the GRF zinc finger domain of DNA glycosylase NEIL3. J Biol Chem . 2020. doi: 10.1074/jbc.RA120.015541 [Online 2 September 2020]. [ Abstract Rodriguez AA, Wojtaszek JL, Greer BH, Haldar T, Gates KS, Williams RS, Eichman BF. 2020. An autoinhibitory role for the GRF zinc finger domain of DNA glycosylase NEIL3. J Biol Chem. 2020. doi: 10.1074/jbc.RA120.015541 [Online 2 September 2020]. Olivieri M, Cho T, Alvarez-Quilon A, Li K, Schellenberg MJ, Zimmermann M, Hustedt N, Rossi SE, Adam S, Melo H, Heijink AM, Sastre-Moreno G, Moatti N, Szilard RK, McEwan A, Ling AK, Serrano-Benitez A, Ubhi T, Feng S, Pawling J, Delgado-Sainz I, Ferguson MW, Dennis JW, Brown GW, Cortes-Ledesma F, Williams RS, Martin A, Xu D, Durocher D. A Genetic Map of the Response to DNA Damage in Human Cells. Cell . 2020. doi: 10.1016/j.cell.2020.05.040 [Online 9 July 2020]. [ Abstract Olivieri M, Cho T, Alvarez-Quilon A, Li K, Schellenberg MJ, Zimmermann M, Hustedt N, Rossi SE, Adam S, Melo H, Heijink AM, Sastre-Moreno G, Moatti N, Szilard RK, McEwan A, Ling AK, Serrano-Benitez A, Ubhi T, Feng S, Pawling J, Delgado-Sainz I, Ferguson MW, Dennis JW, Brown GW, Cortes-Ledesma F, Williams RS, Martin A, Xu D, Durocher D. A Genetic Map of the Response to DNA Damage in Human Cells. Cell. 2020. doi: 10.1016/j.cell.2020.05.040 [Online 9 July 2020]. Álvarez-Quilón A, Wojtaszek JL, Mathieu MC, Patel T, Appel CD, Hustedt N, Rossi SE, Wallace BD, Setiaputra D, Adam S, Ohashi Y, Melo H, Cho T, Gervais C, Muñoz IM, Grazzini E, Young J, Rouse J, Zinda M, Williams RS, Durocher D. Endogenous DNA 3' Blocks Are Vulnerabilities for BRCA1 and BRCA2 Deficiency and Are Reversed by the APE2 Nuclease. Mol Cell. 2020;78(6):1152-1165.e8. [ Abstract Álvarez-Quilón A, Wojtaszek JL, Mathieu MC, Patel T, Appel CD, Hustedt N, Rossi SE, Wallace BD, Setiaputra D, Adam S, Ohashi Y, Melo H, Cho T, Gervais C, Muñoz IM, Grazzini E, Young J, Rouse J, Zinda M, Williams RS, Durocher D. Endogenous DNA 3' Blocks Are Vulnerabilities for BRCA1 and BRCA2 Deficiency and Are Reversed by the APE2 Nuclease. Mol Cell. 2020;78(6):1152-1165.e8. Schellenberg MJ, Appel CD, Riccio AA, Butler LR, Krahn JM, Liebermann JA, Cortés-Ledesma F, Williams RS. Ubiquitin stimulated reversal of topoisomerase 2 DNA-protein crosslinks by TDP2. Nucleic Acids Res ; 2020. doi: 10.1093/nar/gkaa318 [Online 1 May 2020]. [ Abstract Schellenberg MJ, Appel CD, Riccio AA, Butler LR, Krahn JM, Liebermann JA, Cortés-Ledesma F, Williams RS. Ubiquitin stimulated reversal of topoisomerase 2 DNA-protein crosslinks by TDP2. Nucleic Acids Res; 2020. doi: 10.1093/nar/gkaa318 [Online 1 May 2020]. Tumbale PP, Jurkiw TJ, Schellenberg MJ, Riccio AA, O'Brien PJ, Williams RS. Two-tiered enforcement of high-fidelity DNA ligation. Nature Communications . 2019 Nov 28;10(1):5431. doi: 10.1038/s41467-019-13478-7. [ Abstract Tumbale PP, Jurkiw TJ, Schellenberg MJ, Riccio AA, O'Brien PJ, Williams RS. Two-tiered enforcement of high-fidelity DNA ligation. Nature Communications. 2019 Nov 28;10(1):5431. doi: 10.1038/s41467-019-13478-7. Cannavo E, Johnson D, Andres SN, Kissling VM, Reinert JK, Garcia V, Erie DA, Hess D, Thomä NH, Enchev RI, Peter M, Williams RS, Neale MJ, Cejka P. Regulatory control of DNA end resection by Sae2 phosphorylation. Nature Communications 2018 Oct 1;9(1):4016. doi: 10.1038/s41467-018-06417-5. [ Abstract Cannavo E, Johnson D, Andres SN, Kissling VM, Reinert JK, Garcia V, Erie DA, Hess D, Thomä NH, Enchev RI, Peter M, Williams RS, Neale MJ, Cejka P. Regulatory control of DNA end resection by Sae2 phosphorylation. Nature Communications 2018 Oct 1;9(1):4016. doi: 10.1038/s41467-018-06417-5. ] [ Full Text Cannavo E, Johnson D, Andres SN, Kissling VM, Reinert JK, Garcia V, Erie DA, Hess D, Thomä NH, Enchev RI, Peter M, Williams RS, Neale MJ, Cejka P. Regulatory control of DNA end resection by Sae2 phosphorylation. Nature Communications 2018 Oct 1;9(1):4016. doi: 10.1038/s41467-018-06417-5. Kaminski AM, Tumbale PP, Schellenberg MJ, Williams RS, Williams JG, Kunkel TA, Pedersen LC, Bebenek K. Structures of DNA-bound human ligase IV catalytic core reveal insights into substrate binding and catalysis. Nat Commun. 2018 Jul 6;9(1):2642. doi: 10.1038/s41467-018-05024-8. [ Abstract Kaminski AM, Tumbale PP, Schellenberg MJ, Williams RS, Williams JG, Kunkel TA, Pedersen LC, Bebenek K. Structures of DNA-bound human ligase IV catalytic core reveal insights into substrate binding and catalysis. Nat Commun. 2018 Jul 6;9(1):2642. doi: 10.1038/s41467-018-05024-8. ] [ Full Text Kaminski AM, Tumbale PP, Schellenberg MJ, Williams RS, Williams JG, Kunkel TA, Pedersen LC, Bebenek K. Structures of DNA-bound human ligase IV catalytic core reveal insights into substrate binding and catalysis. Nat Commun. 2018 Jul 6;9(1):2642. doi: 10.1038/s41467-018-05024-8. Tumbale P, Schellenberg MJ, Mueller GA, Fairweather E, Watson M, Little JN, Krahn J1, Waddell I, London RE, Williams RS. Mechanism of APTX nicked DNA sensing and pleiotropic inactivation in neurodegenerative disease. EMBO J. 2018 Jun 22. pii: e98875. doi: 10.15252/embj.201798875. [Epub ahead of print] [ Abstract Tumbale P, Schellenberg MJ, Mueller GA, Fairweather E, Watson M, Little JN, Krahn J1, Waddell I, London RE, Williams RS. Mechanism of APTX nicked DNA sensing and pleiotropic inactivation in neurodegenerative disease. EMBO J. 2018 Jun 22. pii: e98875. doi: 10.15252/embj.201798875. [Epub ahead of print] ] [ Full Text Tumbale P, Schellenberg MJ, Mueller GA, Fairweather E, Watson M, Little JN, Krahn J1, Waddell I, London RE, Williams RS. Mechanism of APTX nicked DNA sensing and pleiotropic inactivation in neurodegenerative disease. EMBO J. 2018 Jun 22. pii: e98875. doi: 10.15252/embj.201798875. [Epub ahead of print] Schellenberg MJ, Lieberman JA, Herrero-Ruiz A, Butler LR, Williams JG, Muñoz-Cabello AM, Mueller GA, London RE, Cortés-Ledesma F, Williams RS. ZATT (ZNF451)-mediated resolution of topoisomerase 2 DNA-protein cross-links. Science 14 Sep 2017:eaam6468 doi: 10.1126/science.aam6468 [ Abstract Schellenberg MJ, Lieberman JA, Herrero-Ruiz A, Butler LR, Williams JG, Muñoz-Cabello AM, Mueller GA, London RE, Cortés-Ledesma F, Williams RS. ZATT (ZNF451)-mediated resolution of topoisomerase 2 DNA-protein cross-links. Science 14 Sep 2017:eaam6468 doi: 10.1126/science.aam6468 ] [ Full Text Schellenberg MJ, Lieberman JA, Herrero-Ruiz A, Butler LR, Williams JG, Muñoz-Cabello AM, Mueller GA, London RE, Cortés-Ledesma F, Williams RS. ZATT (ZNF451)-mediated resolution of topoisomerase 2 DNA-protein cross-links. Science 14 Sep 2017:eaam6468 doi: 10.1126/science.aam6468 ] [ Reprint Schellenberg MJ, Lieberman JA, Herrero-Ruiz A, Butler LR, Williams JG, Muñoz-Cabello AM, Mueller GA, London RE, Cortés-Ledesma F, Williams RS. ZATT (ZNF451)-mediated resolution of topoisomerase 2 DNA-protein cross-links. Science 14 Sep 2017:eaam6468 doi: 10.1126/science.aam6468 Wallace BD, Berman Z, Mueller GA, Lin Y, Chang T, Andres SN, Wojtaszek JL, DeRose EF, Appel CD, London RE, Yan S, Williams RS. APE2 Zf-GRF facilitates 3'-5' resection of DNA damage following oxidative stress. Proc Natl Acad Sci USA 2017 Jan 10;114(2):304-309. doi: 10.1073/pnas.1610011114. Epub 2016 Dec 27. PMID: 28028224 [ Abstract Wallace BD, Berman Z, Mueller GA, Lin Y, Chang T, Andres SN, Wojtaszek JL, DeRose EF, Appel CD, London RE, Yan S, Williams RS. APE2 Zf-GRF facilitates 3'-5' resection of DNA damage following oxidative stress. Proc Natl Acad Sci USA 2017 Jan 10;114(2):304-309. doi: 10.1073/pnas.1610011114. Epub 2016 Dec 27. PMID: 28028224 Andres SN, Appel CD, Westmoreland JW, Williams JS, Nguyen Y, Robertson PD, Resnick MA, Williams RS. Tetrameric Ctp1 coordinates DNA binding and DNA bridging in DNA double-strand-break repair. Nature structural & molecular biology 2015 22(2):158-166. [ Abstract Andres SN, Appel CD, Westmoreland JW, Williams JS, Nguyen Y, Robertson PD, Resnick MA, Williams RS. Tetrameric Ctp1 coordinates DNA binding and DNA bridging in DNA double-strand-break repair. Nature structural & molecular biology 2015 22(2):158-166. Deshpande RA, Williams GJ, Limbo O, Williams RS, Kuhnlein J, Lee JH, Classen S, Guenther G, Williams RS, Russell P , Tainer JA, Paull TT. ATP-driven Rad50 conformations regulate DNA tethering, end resection, and ATM checkpoint signaling. The EMBO journal 33(5):482-500 (2014). [ Abstract Deshpande RA, Williams GJ, Limbo O, Williams RS, Kuhnlein J, Lee JH, Classen S, Guenther G, Williams RS, Russell P , Tainer JA, Paull TT. ATP-driven Rad50 conformations regulate DNA tethering, end resection, and ATM checkpoint signaling. The EMBO journal. 33(5):482-500 (2014). Tumbale P, Williams JS, Schellenberg MJ, Kunkel TA, Williams RS. Aprataxin resolves adenylated RNA-DNA junctions to maintain genome integrity. Nature 506(7486):111-115 (2014). [ Abstract Tumbale P, Williams JS, Schellenberg MJ, Kunkel TA, Williams RS. Aprataxin resolves adenylated RNA-DNA junctions to maintain genome integrity. Nature 506(7486):111-115 (2014). Sharifi R, Morra R, Appel CD, Tallis , Chioza B , Jankevicius G , Simpson MA, Matic I , Ozkan E, Golia B , Schellenberg MJ, Weston R, Williams JG, Rossi MN, Galehdari H, Krahn J, Wan A , Trembath RC, Crosby AH, Ahel D, Hay R, Ladurner AG, Timinszky G, Williams RS, Ahel I. Deficiency of terminal ADP-ribose protein glycohydrolase TARG1/C6orf130 in neurodegenerative disease. The EMBO journal 32(9):1225-1237 (2013). [ Abstract Sharifi R, Morra R, Appel CD, Tallis , Chioza B , Jankevicius G , Simpson MA, Matic I , Ozkan E, Golia B , Schellenberg MJ, Weston R, Williams JG, Rossi MN, Galehdari H, Krahn J, Wan A , Trembath RC, Crosby AH, Ahel D, Hay R, Ladurner AG, Timinszky G, Williams RS, Ahel I. Deficiency of terminal ADP-ribose protein glycohydrolase TARG1/C6orf130 in neurodegenerative disease. The EMBO journal 32(9):1225-1237 (2013). Schellenberg MJ, Appel CD, Adhikari S, Robertson PD, Ramsden DA, Williams RS. Mechanism of repair of 5'-topoisomerase II-DNA adducts by mammalian tyrosyl-DNA phosphodiesterase 2. Nature structural & molecular biology 19(12):1363-71 (2012).  [ Abstract Schellenberg MJ, Appel CD, Adhikari S, Robertson PD, Ramsden DA, Williams RS. Mechanism of repair of 5'-topoisomerase II-DNA adducts by mammalian tyrosyl-DNA phosphodiesterase 2. Nature structural & molecular biology 19(12):1363-71 (2012). Schellenberg, M.J. and Williams, R.S. DNA end processing by polynucleotide kinase/phosphatase. Proc Natl Acad Sci USA 108(52):20855-20856 (2011). [ Abstract Schellenberg, M.J. and Williams, R.S. DNA end processing by polynucleotide kinase/phosphatase. Proc Natl Acad Sci USA 108(52):20855-20856 (2011). Tumbale, P., Appel, D.C., Kraehenbuehl, R., Robertson, P.D., Williams, J.S., Krahn, J., Ahel, I., and Williams, R.S. Structure of an Aprataxin-DNA complex with insights into AOA1 Neurodegenerative Disease. Nature Structural & Molecular Biology 18(11):1189-1195 (2011). [ Abstract Tumbale, P., Appel, D.C., Kraehenbuehl, R., Robertson, P.D., Williams, J.S., Krahn, J., Ahel, I., and Williams, R.S. Structure of an Aprataxin-DNA complex with insights into AOA1 Neurodegenerative Disease. Nature Structural & Molecular Biology 18(11):1189-1195 (2011). Williams, R.S., and Kunkel, T.A. Fen Nucleases: Bind, Bend, Fray, Cut. Cell 145(2):171-172 (2011). [ Abstract Williams, R.S., and Kunkel, T.A. Fen Nucleases: Bind, Bend, Fray, Cut. Cell 145(2):171-172 (2011). Williams, G.J., Williams, R.S., Williams, J.S., Moncalian, G., Arvai, A.S., Limbo, O., Guenther, G., SilDas, S., Hammel, M., Russell, P., and Tainer, J.A. ABC ATPase signature helices in Rad50 link nucleotide state to Mre11 interface for DNA repair. Nature Structural and Molecular Biology 18(4): 423-431 (2011). [ Abstract Williams, G.J., Williams, R.S., Williams, J.S., Moncalian, G., Arvai, A.S., Limbo, O., Guenther, G., SilDas, S., Hammel, M., Russell, P., and Tainer, J.A. ABC ATPase signature helices in Rad50 link nucleotide state to Mre11 interface for DNA repair. Nature Structural and Molecular Biology 18(4): 423-431 (2011). Williams, J.S., Williams, R.S., Dovey, C.L., Guenther, G., Tainer, J.A., and Russell, P. gammaH2A binds Brc1 to maintain genome integrity during S-phase. EMBO J. 29(6): 1136-48 (2010). [ Abstract Williams, J.S., Williams, R.S., Dovey, C.L., Guenther, G., Tainer, J.A., and Russell, P. gammaH2A binds Brc1 to maintain genome integrity during S-phase. EMBO J. 29(6): 1136-48 (2010). Williams, R.S., Dodson, G.E., Limbo, O., Yamada, Y., Williams, J.S., Guenther, G., Classen, S., Glover, J.N., Iwasaki, H., Russell, P., and Tainer, J.A. Nbs1 is an extended flexible arm Binding to Ctp1 and Mre11-Rad50 to coordinate dsDNA break processing. Cell 139(1): 87-99 (2009). [ Abstract Williams, R.S., Dodson, G.E., Limbo, O., Yamada, Y., Williams, J.S., Guenther, G., Classen, S., Glover, J.N., Iwasaki, H., Russell, P., and Tainer, J.A. Nbs1 is an extended flexible arm Binding to Ctp1 and Mre11-Rad50 to coordinate dsDNA break processing. Cell 139(1): 87-99 (2009). Williams, R.S., Williams, G.J., and Tainer, J.A. A charged performance by gp17 in viral packaging. Cell 135 (7):1169-1171 (2008). [ Abstract Williams, R.S., Williams, G.J., and Tainer, J.A. A charged performance by gp17 in viral packaging. Cell 135 (7):1169-1171 (2008). Williams, R.S., Moncalian, G., Williams, J.S., Yamada, Y, Limbo, O., Shin, D.S., Groocock, L.M., Cahill., D., Hitomi, C., Guenther, G., Moiani, D., Carney, J.P., Russell, P., and Tainer, J.A. Mre11 dimers coordinate DNA end bridging and nuclease processing in DNA double strand break repair. Cell 135 (1):97-109 (2008). [ Abstract Williams, R.S., Moncalian, G., Williams, J.S., Yamada, Y, Limbo, O., Shin, D.S., Groocock, L.M., Cahill., D., Hitomi, C., Guenther, G., Moiani, D., Carney, J.P., Russell, P., and Tainer, J.A. Mre11 dimers coordinate DNA end bridging and nuclease processing in DNA double strand break repair. Cell 135 (1):97-109 (2008). Williams, R.S., and Tainer, J.A. Learning our ABCs: Rad50 directs MRN repair functions via Adenylate Kinase activity from the conserved ATP Binding Cassette. Molecular Cell 25 (6):789-791 (2007). [ Abstract Williams, R.S., and Tainer, J.A. Learning our ABCs: Rad50 directs MRN repair functions via Adenylate Kinase activity from the conserved ATP Binding Cassette. Molecular Cell 25 (6):789-791 (2007). Williams, R.S., and Tainer, J.A. A Nanomachine for Making Ends Meet: MRN is a flexing scaffold for the repair of DNA double strand breaks. Molecular Cell 19(6):724-726 (2005). [ Abstract Williams, R.S., and Tainer, J.A. A Nanomachine for Making Ends Meet: MRN is a flexing scaffold for the repair of DNA double strand breaks. Molecular Cell 19(6):724-726 (2005). Bernstein, N.K., Williams, R.S., Rakovszky, M.L, Cui, D., Green, R., Karimi-Busheri, F., Mani, R.S, Galicia, S., Koch C.A., Cass, C.E, Durocher, D., Weinfeld, M., and Glover, J.N. The molecular architecture of the mammalian DNA repair enzyme, polynucleotide kinase. Molecular Cell 17(5):657-670 (2005). [ Abstract Bernstein, N.K., Williams, R.S., Rakovszky, M.L, Cui, D., Green, R., Karimi-Busheri, F., Mani, R.S, Galicia, S., Koch C.A., Cass, C.E, Durocher, D., Weinfeld, M., and Glover, J.N. The molecular architecture of the mammalian DNA repair enzyme, polynucleotide kinase. Molecular Cell 17(5):657-670 (2005). Glover, J.N.M, Williams, R.S., and Lee, MS. BRCT repeat-phosphoprotein interactions: tangled up in two. Trends in Biochemical Sciences 29(11):579-585 (2004). [ Abstract Glover, J.N.M, Williams, R.S., and Lee, MS. BRCT repeat-phosphoprotein interactions: tangled up in two. Trends in Biochemical Sciences 29(11):579-585 (2004). Williams, R.S., Lee, M.S., Hau., D.D., and Glover, J.N.M. Structural basis of phosphopeptide recognition by the BRCT domain of BRCA1. Nature Structural and Molecular Biology 11(6):519-525 (2004). [ Abstract Williams, R.S., Lee, M.S., Hau., D.D., and Glover, J.N.M. Structural basis of phosphopeptide recognition by the BRCT domain of BRCA1. Nature Structural and Molecular Biology 11(6):519-525 (2004). Williams, R.S., Green, R., and Glover, J.N.M. Crystal structure of the BRCT repeat region from the breast cancer-associated protein BRCA1. Nature Structural Biology 8(10):838-842 (2001). [ Abstract Williams, R.S., Green, R., and Glover, J.N.M. Crystal structure of the BRCT repeat region from the breast cancer-associated protein BRCA1. Nature Structural Biology 8(10):838-842 (2001). Contact Robert S. Williams, Ph.D. Acting Chief, Genome Integrity and Structural Biology Laboratory and Principal Investigator Tel 984-287-3542 [email protected] Back to Top Last Reviewed: April 10, 2026