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Selected Publications

Structural Cell Biology Group

Publications (2009-Present)
*Correspondence: williamsrs@niehs.nih.gov

  1. 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] [Full Text] [Reprint] 
  2. Andres SN, Williams RS. CtIP/Ctp1/Sae2, molecular form fit for function. DNA Repair 2017 Jun 9. pii: S1568-7864(17)30213-6. doi: 10.1016/j.dnarep.2017.06.013. PMID: 28623092 [Abstract] 
  3. 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]
  4. Appel CD, Feld GK, Wallace BD, Williams RS.Structure of the sirtuin-linked macrodomain SAV0325 from Staphylococcus aureus. Protein Sci. 2016 Sep;25(9):1682-91. doi: 10.1002/pro.2974. Epub 2016 Jul 7. PMID: 27345688 [Abstract]
  5. Schellenberg MJ, Perera L, Strom CN, Waters CA, Monian B, Appel CD, Vilas CK, Williams JG, Ramsden DA, Williams RS. Reversal of DNA damage induced Topoisomerase 2 DNA-protein crosslinks by Tdp2. Nucleic Acids Res. 2016 May 5;44(8):3829-44. doi: 10.1093/nar/gkw228. Epub 2016 Apr 8. PMID: 27060144 [Abstract]
  6. Schellenberg MJ, Tumbale PP, Williams RS. Molecular underpinnings of Aprataxin RNA/DNA deadenylase function and dysfunction in neurological disease. Progress in biophysics and molecular biology 2015 117(2-3):157-165. [Abstract]
  7. Andres SN, Schellenberg MJ, Wallace BD, Tumbale P, Williams RS. Recognition and repair of chemically heterogeneous structures at DNA ends. Environmental and molecular mutagenesis 2015 56(1):1-21. [Abstract]
  8. 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]
  9. Wallace BD, Williams RS. Ribonucleotide triggered DNA damage and RNA-DNA damage responses. RNA biology 2014 11(11):1340-1346. [Abstract]
  10. Gao R, Schellenberg MJ , Huang SY, Abdelmalak M, Marchand C, Nitiss KC, Nitiss JL, Williams RS, Pommier Y. Proteolytic Degradation of Topoisomerase II (Top2) Enables the Processing of Top2┬ĚDNA and Top2┬ĚRNA Covalent Complexes by Tyrosyl-DNA-Phosphodiesterase 2 (TDP2). The Journal of biological chemistry 2014 289(26):17960-17969. [Abstract]
  11. 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]
  12. 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]
  13. Crown NK, Savytskyy OP, Malik SB, Logsdon J , Williams RS, Tainer JA, Zolan ME. A Mutation in the FHA Domain of Coprinus cinereus Nbs1 Leads to Spo11-Independent Meiotic Recombination and Chromosome Segregation. G3 (Bethesda, Md.) 2013 3(11):1927-1943. [Abstract]
  14. 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]
  15. 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]
  16. 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]
  17. Adhikari, S., Karmahapatra, S.K., Elias, H., Dhopeschwarkar, P., Williams, R.S., Byers, S., Uren, A., Roy, R. Development of a novel assay for human tyrosyl DNA phosphodiesterase 2. Anal Biochem 416(1):112-116 (2011). [Abstract]
  18. 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]
  19. Williams, R.S., and Kunkel, T.A. Fen Nucleases: Bind, Bend, Fray, Cut. Cell 145(2):171-172 (2011). [Abstract]
  20. 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]
  21. Rahal, E.A., Henricksen, L.A., Li, Y., Williams, R.S., Tainer, J.A. and Dixon, K. ATM regulates Mre11-dependent DNA end-degradation and microhomology-mediated end joining. Cell Cycle 12(9): 14 (2010).   [Abstract]
  22. Lee, M.S., Green, R., Marsillac. S.M, Williams, R.S., Coquelle, N. Yeung, T., Foo, D., Hau,D.D, Hui, B., Monteiro, A.N.A., and Glover, J.N.M. Comprehensive analysis of missense variations in the BRCT domain of BRCA1 by structural and functional assays. Cancer Res. 70(12): 4880-4890 (2010). [Abstract]
  23. 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]
  24. 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]

    Publications (2001-2008)

  25. 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]
  26. Acharya, S., Many, A.M., Schroeder, A., Kennedy, F., Savytskyy, O.P., Grubb, J., Vincent, J., Friedle, E., Celerin, M., Maillet, D., Palmerini, H.J., Greischar, M.A., Moncalian, G., Williams, R.S., Tainer, J.A., and Zolan, M.E. Coprinus cinereus Rad50 mutants reveal an essential structural role for Rad50 in axial element and synaptonemal complex formation, homolog pairing, and meiotic recombination. Genetics 180(4):1889-1907 (2008). [Abstract]
  27. Edwards, R.A., Lee, M.S., Tsutakawa, S.E., Williams, R.S., Nazeer, I., Keliman, F.E., Tainer, J.A., and Glover, J.N. The Bard1 C-terminal domain structure and interactions with polyadenylation factor, CstF-50. Biochemistry 47(44):11446-11456 (2008). [Abstract]
  28. 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]
  29. Williams, R.S., Williams, J.S., and Tainer, J.A. Mre11/Rad50/Nbs1 is a keystone complex connecting DNA repair machinery, double strand break signaling, and the chromatin template. Biochemistry and Cell Biology 85 (4):509-520 (2007). [Abstract]
  30. 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]
  31. Simons, A.M., Horwitz, A.A., Starita, L.M., Griffin, K., Williams, R.S., Glover, J.N., and Parvin, J.D. BRCA1 DNA-binding activity is stimulated by BARD1. Cancer Research 66(4):2012-2018 (2006). [Abstract]
  32. Williams, R.S, Bernstein, N., Lee, M.S., Rakovszky, M.L., Cui, D., Green, R., Weinfeld, M., and Glover, J.N. Structural basis for phosphorylation-dependent signaling in the DNA damage response. Biochemistry and Cell Biology 83(6):721-727 (2005). [Abstract]
  33. 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]
  34. 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]
  35. 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]
  36. 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]
  37. Dubin M.J., Stokes, P., Sum, E.Y.M, Williams, R.S., Valova, V., Robinson, P.,Lindeman, G.J., Glover, J.N., Visvader, J.E., and Matthews, J.M. Dimerization of CtIP, a BRCA1- and CtBP-interacting protein, is mediated by an N-terminal coiled-coil motif. Journal of Biological Chemistry 279(26):26932-26938 (2004). [Abstract]
  38. Williams, R.S., Hau, D, Chasman, D., Hui, B., and Glover, J.N.M. Detection of protein folding defects caused by BRCA1-BRCT truncation and missense mutations. Journal of Biological Chemistry 278(52):53007-53016 (2003). [Abstract]
  39. Williams, R.S., and Glover, J.N.M. Structural consequences of a cancer-causing BRCA1-BRCT missense mutation. Journal cover feature. Journal of Biological Chemistry 278(4):2630-2635 (2003). [Abstract]
  40. Hamilton, K.S., Ellison, M.J., Barber, K.R., Williams, R.S., Huzil, J.T., McKenna, S., Ptak, C., Glover, M., and Shaw, G.S. Structure of a conjugating enzyme-ubiquitin thiolester intermediate reveals a novel role for the ubiquitin tail. Structure 9:897-904 (2001). [Abstract]
  41. 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]