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Publications: Recent
Publications: 2010-2006
Publications: 2005-2001
Publications: 2000-1996
Publications: 1995 and Prior

2018 Publications

Knijnenburg, T, Wang, L, Chambwe, N …Monnat, R.J., Jr., Xiao, Y. and Wang, C. on behalf of TCGA DNA Damage Repair Analysis Working Group (2018) Landscape of DNA damage repair deficiency across The Cancer Genome Atlas. Cell Rep. 2018 Apr; 23(1):239-254. PMID: 29617664

Oshima J., Fuki M. Hisama and Raymond J. Monnat, Jr. (2018) Werner Syndrome as a Model of Human Aging, chapter in Handbook of Models of Human Aging, 2nd Ed. P. Michael Conn, ed. Elsevier Academic Press (in press).

Lebel M, Monnat RJ Jr.. Werner syndrome (WRN) gene variants and their association with altered function and age-associated diseases. Ageing Res Rev. 2018 Jan;41:82-97. doi: 10.1016/j.arr.2017.11.003. Epub 2017 Nov 14. Review. PubMed PMID: 29146545.

2019 Publications

Pellenz S, Phelps MP, Tang W, Hovde BT, Sinit R, Fu W, Li H, Chen E, and Monnat RJ Jr. (2019) New human chromosomal sage harbor sites for genome engineering with CRISPER/Cas9, TAL effector and homing endonucleases.  Human Gene Therapy doi: 10.1089/hum.2018.169. [Epub ahead of print] and BiorXiv (posted on 20 August 2018).

Juarez E, Chambwe N, Tang W, Mitchell AD, Owen N, Kumari A, Monnat RJ Jr, and McCullough AK. (2018) An RNAi screen in human cell lines reveals conserved DNA damage repair pathways that mitigate formaldehyde sensitivity. DNA Repair 72: 1-9. doi: 10.1016/j.dnarep.2018.10.002

2017 Publications

Kamath-Loeb AS, Zavala-van Rankin DG, Flores-Morales J, Emond MJ, Sidorova JM, Carnevale A, Cárdenas-Cortés MD, Norwood TH, Monnat RJ, Loeb LA, Mercado-Celis GE. Homozygosity for the WRN Helicase-Inactivating Variant, R834C, does not confer a Werner syndrome clinical phenotype. Sci Rep. 2017 Mar 9;7:44081. doi: 10.1038/srep44081.  PMCID: PMC5343477.

Fu W, Ligabue A, Rogers KJ, Akey JM, Monnat RJ Jr. Human RECQ Helicase Pathogenic Variants, Population Variation and "Missing" Diseases. Hum Mutat. 2017 Feb;38(2):193-203. doi: 10.1002/humu.23148.  PMCID: PMC5518694.

Oshima J, Sidorova JM, Monnat RJ Jr. Werner syndrome: Clinical features, pathogenesis and potential therapeutic interventions. Ageing Res Rev. 2017 Jan; 33:105-114. doi: 10.1016/j.arr.2016.03.002.  PMCID: PMC5025328.

Sidorova, Julia M, and Monnat Jr, Raymond J(Feb 2018) DNA Helicase Deficiency Disorders. In: eLS. John Wiley & Sons Ltd, Chichester. [doi: 10.1002/9780470015902.a0006065.pub3]

2016 Publications


Scharenberg, A.M., Stoddard, B.L. and Monnat, RJ Jr. (2016) Retargeting: an unrecognized consideration in endonuclease-based gene drive biology. bioRxiv record 089946; DOI: 10.1101/089946 (posted November 27, 2016).


Tokita M, Kennedy SR, Risques RA, Chun SG, Pritchard C, Oshima J, Liu Y, Bryant-Greenwood PK, Welcsh P, and Monnat RJ Jr. (2016) Werner syndrome through the lens of tissue and tumour genomics. Scientific Reports Aug 25;6:32038. PMID: 27559010. PMC4997333. DOI: 10.1038/srep32038.


Bosch LJW, Luo Y, Lao VV, Snaebjornsson P, Trooskens G, Vlassenbroeck I, Mongera S, Tang W, Welcsh P, Herman JG, Koopman M, Nagtegaal I, Punt CJA, van Criekinge W, Meijer G, Monnat, RJ Jr., Carvalho B, and Grady WM. (2016) WRN promoter CpG island hypermethylation does not predict more favorable outcomes for metastatic colorectal cancer patients treated with irinotecan-based therapy. Clinical Cancer Research Apr 27. Epub ahead of print.  PMID: 27121793. PMC5026547. DOI: 10.1158/1078-0432.CCR-15-2703


Oshima J, Sidorova JM, and Monnat RJ Jr. (2016) Werner Syndrome. Aging Research Reviews Mar 15. pii: S1568-1637(16)30026-5. Epub ahead of print. Review. PMID: 26993153. PMC5025328 DOI: 10.1016/j.arr.2016.03.002


Zhang Q-S, Deater M, Phan N, Marcogliese AN, Tang W, Li H, Al-Dhalimy M, Major A, Monnat RJ Jr, Olson S, and Grompe M. (2016) Metformin alleviates hematopoietic defects and delays tumor formation in Fanconi anemia mice. Blood Oct 18. pii: Epub ahead of print. PMID:27756748. DOI:10.1182/blood-2015-11-683490  


Kehrli K, Phelps M, Lazarchuk P, Chen E, Monnat RJ Jr. and Sidorova, JM. (2016) Class I histone deacetylase HDAC1 and WRN RECQ helicase contribute additively to protection of replication forks from hydroxyurea-induced arrest. J Biol Chem 291(47):24487-24503. Epub 2016 Sep 26. DOI:10.1074/jbc.M115.708594


Fu W, Ligabue A, Rogers KJ, Akey JM, and Monnat RJ Jr. (2016) Human RECQ helicase

pathogenic variants, population variation and 'missing' diseases. Human Mutation. Nov 17. doi: 10.1002/humu.23148. Epub ahead of print. PMID:27859906


Oshima J, Hisama FM, and Monnat RJ Jr. (2016) Werner Syndrome as a Model of Human Aging, chapter in Handbook of Models of Human Aging, 2nd Ed. P. Michael Conn, ed. Elsevier Academic Press (in press 20 Nov 2016).


Kamath-Loeb AS, Zavala-van Rankin DG, Flores-Morales J, Sidorova JM, Carnevale A, Cárdenas-Cortés M, Norwood TH, Monnat RJ Jr, Loeb LA, and Mercado-Celis GE. (2016) Marked gender distortion in homozygous carriers of the common, catalytically inactivating Werner syndrome R834C -WRN polymorphism. Scientific Reports (in revision Nov 2016).


2015 Publications


Sidorova JM and Monnat RJ Jr. (2015) Human RECQ helicases: roles in cancer, aging, and inherited disease. Advances in Genomics and Genetics 5:19–33. DOI: 10.2147/AGG.S54078


Hovde BT, Deodato CR, Hunsperger HM, Ryken SA, Yost W, Jha RK, Patterson J, Monnat RJ Jr, Barlow SB, Starkenburg SR, Cattolico RA. (2015) Genome sequence and transcriptome analyses of Chrysochromulina tobin: metabolic tools for enhanced algal fitness in the prominent order Prymnesiales (Haptophyceae). PLoS Genetics 11(9):e1005469. PMID: 26397803. PMC4580454. DOI: 10.1371/journal.pgen.1005469.


Tang W, Robles AI, Beyer RP, Gray LT, Nguyen GH, Oshima J, Maizels N, Harris CC, and Monnat RJ Jr. (2015) Werner syndrome helicase modulates G4 DNA-dependent transcription and opposes mechanistically distinct senescence-associated gene expression programs. bioXriv Biology Preprint server, published as New Results on 4 August 2015 under bioXriv DOI: 10.1101/023739


Tang W, Robles AI, Beyer RP, Gray LT, Nguyen GH, Oshima J, Maizels N, Harris CC, and Monnat RJ Jr. (2015) Werner syndrome helicase targets G4 DNA in human cells to modulate transcription and suppress genomic instability. Human Molecular Genetics, Mar 16. Epub ahead of print. PMID:  26984941, PMC5062591 DOI: 10.1093/hmg/ddw079


Robinson R, Wonga L, Monnat RJ Jr., and Fu E. (2015) Development of a whole blood paper-based device for phenylalanine detection and PKU therapy monitoring. Micromachines 7(2), 28; DOI: 10.3390/mi7020028.

2014 Publications


Lauper JM, Monnat RJ Jr. (2014) Diabetes mellitus and cancer in Werner syndrome. Acta Diabetol. 51:159-61. doi: 10.1007/s00592-013-0456-z. PMCID: PMC3669637.


Friedman, J.I. Li, H. and Monnat, RJ Jr. (2014) Quantifying the information content of homing endonuclease target sites by single base pair profiling. Chapter 11 in David R. Edgell (ed.), Homing Endonucleases: Methods and Protocols, Methods in Molecular Biology, vol. 1123. DOI 10.1007/978-1-62703-968-0_11.


Li, H. and Monnat, RJ Jr. (2014) Homing endonuclease target site specificity defined by sequential enrichment and next-generation sequencing of highly complex target site libraries. Chapter 12 in David R. Edgell (ed.), Homing Endonucleases: Methods and Protocols, Methods in Molecular Biology, vol. 1123. DOI 10.1007/978-1-62703-968-0_12.


Pellenz, S. and Monnat, RJ Jr. (2014) Identification and analysis of genomic homing endonuclease target sites. Chapter 16 in David R. Edgell (ed.), Homing Endonucleases: Methods and Protocols, Methods in Molecular Biology, vol. 1123. DOI 10.1007/978-1-62703-968-0_16.


Shiovitz S, Bertagnolli MM, Renfro LA, Nam E, Foster NR, Dzieciatkowski S, Valinluck-Lao V, Monnat RJ Jr, Emond M, Maizels N, Niedzwiecki D, Saltz LB, Venook A, Warren RS, Grady WM. (2014) CpG island methylator phenotype predicts improved response to adjuvant therapy with irinotecan, fluorouracil, and leucovorin in stage III colon cancer: Cancer and Leukemia Group B Protocol 89803. Gastroenterology 147(3):637-45. doi: 10.1053/j.gastro.2014.05.009. PMCID: PMC4143495.  


Nguyen, G.H., Tang,W., Robles, A.I., Beyer, R.P., Gray, L.T., Welsh, J., Kumamoto, K. Wang, X.W., Hickson, I.D., Maizels, N., Monnat, R.J. Jr. and Harris, C.C. (2014) Regulation of gene expression by the BLM helicase correlates with the presence of G-quadruplex DNA motifs. Proc Natl Acad Sci U S A. 111: 9905-9910. doi: 10.1073/pnas.1404807111. PMCID: PMC4103369.


Hovde, B.T., Starkenburg, S.R., Hunsperger, H.M., Mercer, L.D., Deodato, C. Jha, R.K., Chertkov, O., Monnat, R.J., Jr. and Cattolico, R.A. (2014) The mitochondrial and chloroplast genomes of the haptophyte Chrysochromulina tobin contain unique repeat structures and gene profiles. BMC Genomics 15(1):604. doi: 10.1186/1471-2164-15-604. PMID: 25034814


Rogers, K.J., Fu, W., Akey, J.M. and Monnat, R.J., Jr. (2014) Global and disease-associated genetic variation in the human Fanconi anemia gene family. Human Molecular Genetics 23:6815-25. doi: 10.1093/hmg/ddu400 PMCID: PMC4245043.


Pavelitz T, Renfro L, Foster NR, Caracol A, Welsch P, Lao VL, Grady WM, Niedzwicki D, Saltz LB, Bertagnolli M, Goldberg RM, Rabinovitch PS, Emond M, Monnat RJ Jr, Maizels N. (2014) MRE11-Deficiency predicts improved long-term disease free survival and overall survival in a subset of stage III colon cancer patients treated with irinotecan: Cancer and Leukemia Group B Protocol 89803. PLoS ONE 9(10):e108483. doi: 10.1371/journal.pone.0108483. PMCID: PMC4195600.


Chang, TC, Mikheev, A.M., Huynh, W., Monnat, R.J., Jr., Rostomily, R.C. and Folch, A. (2014) Parallel microfluidic chemosensitivity testing on individual slice cultures. Lab Chip 14:4540-51. doi: 10.1039/c4lc00642a. PMCID: PMC4217250.


Thiessen G, De Los Reyes K, Monnat, R.J., Jr. and Fu E (2014) An equipment-free paper-based test for the detection of phenylketonuria in newborns in low-resource settings. The Analyst 140:609-15. doi: 10.1039/c4an01627k. PMID: 25427275 [PubMed - in process].


Chang, C., Tang, W., Koh, W.J.H., Emond, M.J., Monnat, R.J., Jr. and Folch, A. (2014) Microwell arrays reveal cellular heterogeneity during the clonal expansion of transformed human cells. Technology 3: 163-171. PMC4854201. doi: 10.1142/S2339547815200046.


2013 Publications


Sidorova JM, Kehrli K, Mao F, Monnat R Jr. (2013) Distinct functions of human RECQ helicases WRN and BLM in replication fork recovery and progression after hydroxyurea-induced stalling. DNA Repair 12:128-39. doi: 10.1016/j.dnarep.2012.11.005. PMCID: PMC3551992


Lauper JM, Monnat RJ Jr. (2013) Diabetes mellitus and cancer in Werner syndrome. Acta Diabetol. 51:159-61. doi: 10.1007/s00592-013-0456-z. PMCID: PMC3669637


Monnat, R.J., Jr. (2013) ‘Werner syndrome’, in WHO/IARC Monograph on Pathology and Genetics of Tumours of Soft Tissue and Bone, 4th Edition edited by C.D.M. Fletcher, J.A. Bridge, P.C.W. Hogendoorn and F.Mertens. IARC Press, Lyon. pp. 393-394.


Berti M, Chaudhuri AR, Thangavel S, Gomathinayagam S, Kenig S, Vujanovic M, Odreman F, Glatter T, Graziano S, Mendoza-Maldonado R, Marino F, Lucic B, Biasin V, Gstaiger M, Aebersold R, Sidorova JM, Monnat RJ Jr, Lopes M, Vindigni A. (2013) Human RECQ1 promotes restart of replication forks reversed by DNA topoisomerase I inhibition. Nature Structural Molec Biol. 20:347-54. doi: 10.1038/nsmb.2501. PMCID: PMC3897332  


Lauper, J.L. Krause, A., Vaughan, T.L. and Monnat, R.J.,Jr. (2013) Spectrum and risk of neoplasia in Werner syndrome: a systematic review. PLoS ONE 8(4):e59709. doi: 10.1371/journal.pone.0059709. PMCID: PMC3613408


Glukhova VA, Tomazela DM, Findlay GD, Monnat RJ Jr, Maccoss MJ. (2013) Rapid Assessment of RNAi-mediated Protein Depletion by Selected Reaction Monitoring Mass Spectrometry. J Proteome Res. 12(7):3246-54. DOI: 10.1021/pr400067k. PMCID: PMC3767422


Metzger MJ, Stoddard BL, Monnat RJ Jr. (2013) PARP-mediated repair, homologous recombination, and back-up non-homologous end joining-like repair of single-strand nicks. DNA Repair 12:529-34. doi: 10.1016/j.dnarep.2013.04.004. PMCID: PMC3683368


Hughes BT, Sidorova J, Swanger J, Monnat RJ Jr, Clurman BE. (2013) Essential role for Cdk2 inhibitory phosphorylation during replication stress revealed by a human Cdk2 knockin mutation. Proc Natl Acad Sci U S A. 110:8954-9. doi: 10.1073/pnas.1302927110. PMCID: PMC3670391


Lao VV, Welcsh P, Luo Y, Carter KT, Dzieciatkowski S, Dintzis S, Meza J, Sarvetnick NE, Monnat RJ Jr, Loeb LA and Grady WM (2013) Altered RECQ helicase expression in sporadic primary colorectal cancers. Transl Oncol. 6:458-69. doi:10.1593/tlo.13238. PMCID: PMC3730021.



2012 Publications


Duxin JP, Moore HR, Sidorova J. Karanja, K, Honaker Y, Dao B, Piwnica-Worms H, Campbell JL, Monnat RJ Jr, Stewart SA (2012) An Okazaki fragment processing-independent role for human Dna2 during DNA replication. J. Biol. Chem. 287:21980-91. doi: 10.1074/jbc.M112.359018. PMCID: PMC3381158


Li H, Ulge UY, Hovde BT, Doyle LA, Monnat RJ Jr. (2012) Comprehensive homing endonuclease target site specificity profiling reveals evolutionary constraints and enables genome engineering applications. Nucleic Acids Res. 40(6):2587-98. doi: 10.1093/nar/gkr1072. PMCID:    PMC3315327


2010 Publications


Dhillon,K.K., Sidorova,J.M., Albertson,T.M., Anderson,J.B., Ladiges,W.C., Rabinovitch,P.S., Preston, B.D. and Monnat, R.J., Jr. (2010) Werner syndrome protein loss leads to divergent mouse and human cell phenotypes. DNA Repair 9:11-22. doi: 10.1016/j.dnarep.2009.09.013. PMCID: PMC2818259


Bhavan-Thangavel, S., Mendoza-Maldonado, R. Tissino, E. Sidorova, J.M., Yin,J. Wang, W., Monnat, R.J., Jr., Falaschi, A. and Vindigni, A. (2010) The human RECQ1 and RECQ4 helicases play distinct roles in DNA replication initiation. Molec Cell Biol. 30:1382-1396 (on-line 11 Jan 2010; doi:10.1128/MCB.01290-09). PMCID: PMC2832491


Mao, F.J., Sidorova, J.M., Lauper, J.M., Emond, M.E. and Monnat, R.J., Jr. (2010) The human WRN and BLM RecQ helicases differentially regulate cell proliferation and survival after chemotherapeutic DNA damage. Cancer Research 70(16):6548-55. Epub 2010 Jul 27. doi: 10.1158/0008-5472.CAN-10-0475. PMCID: PMC2941797


Sidorova, J.M. and Monnat, R.J., Jr. (2010) DNA Helicase-deficiency Disorders. Encyclopedia of Life Sciences, Article # A6065. DOI: 10.1002/9780470015902.a0006065.pub2


Monnat, R.J., Jr. (2010) Human RECQ helicases: Roles in DNA metabolism, disease pathogenesis and disease therapy. Seminars Cancer Biol. 20(5):329-39. doi:   10.1016/j.semcancer.2010.10.002. PMCID: PMC3040982


Ulge, U.Y., Baker, D.A. and Monnat, R.J., Jr. (2010) Comprehensive computational design of mCreI homing endonuclease cleavage specificity for genome engineering. Nucleic Acids Research 39(10):4330-9. doi: 10.1093/nar/gkr022. PMCID: PMC3105429


Windbichler, N. Menichelli, M., Papathanos, P.A., Thyme, S.B., Li, H., Ulge, U.Y. Hovde, B.T., Baker, D., Monnat, R.J., Jr., Burt, A. and Crisanti, A. (2010) A synthetic, homing ednouclease- based genetic drive system in the human malaria mosquito. Nature 473(7346):212-5. doi:10.1038/nature09937. PMCID: PMC3093433


2009 Publications


McConnell-Smith, A., Pellenz, S., Davis, L.W., Maizels, N., Monnat, R.J., Jr. and Stoddard, B.L. (2009) Generation of a nicking enzyme that stimulates site-specific targeted gene correction from the I-AniI LAGLIDADG homing endonuclease. Proc Natl Acad Sci USA  106:5099-5104. doi: 10.1073/pnas.0810588106. PMCID: PMC2664052


Li, H. Pellenz, S., Ulge, U. Stoddard, B.L. and Monnat, R.J., Jr. (2009) Generation of single-chain LAGLIDADG homing endonucleases from native homodimeric precursor proteins. Nucleic Acids Res. 37:1650-1662. doi: 10.1093/nar/gkp004. PMCID: PMC2655683


Sidorova, J.M., Li, N., Schwartz, D.C., Folch, A. and Monnat, R.J., Jr. (2009) Microfluidic-assisted analysis of replicating DNA molecules. Nature Protocols 4:849-861. doi: 10.1038/nprot.2009.54. PMCID: PMC2762429


Rey, L., Sidorova, J.M., Puget, N., Boudsocq, F., Biard, D.S.F., Monnat, R.J., Jr., Cazaux, C. and Hoffman, J-S. (2009) Human DNA polymerase eta is required for common fragile site stability during unperturbed DNA replication. Molec Cell Biol 29:3344-3354. doi: 10.1128/MCB.00115-09. PMCID: PMC2698728

2008 Publications


Sidorova, J.M., Li, N. Folch, A. and Monnat, R.J., Jr. (2008) The Werner syndrome RecQ helicase is required for normal replication fork progression after DNA damage or replication arrest. Cell Cycle 7:796-807. PMCID: PMC4362724


Stoddard, B.L. Scharenberg, A.M. and Monnat, R.J., Jr. (2008) Advances in engineering homing endonucleases for gene targeting: Ten years after structures. Chapter 6 in Progress in Gene Therapy 3: Autologous and Cancer Stem Cell Gene Therapy, Eds. R. Bertolotti and K. Ozawa, World Scientific Press, Hackensack, NJ. pp. 135-167. DOI: 10.1142/9789812775870_0006


Berkovich, E., Monnat, R.J., Jr. and Kastan, M.B. (2008) Assessment of protein dynamics and DNA repair following generation of DNA double-strand breaks at defined genomic sites. Nature Protocols 3:915-922. doi: 10.1038/nprot.2008.54. PMID: 18451799


Loeb,L.A. and Monnat, R.J., Jr. (2008) DNA polymerases and human disease. Nature Reviews Genetics 9: 594-604. doi: 10.1038/nrg2345. PMID: 18626473

2007 Publications


Dhillon,K., Sirorova, J., Saintigny,Y., Poot,M., Gollahon,K., Rabinovitch, P.S. and Monnat, R.J.,Jr. (2007) Functional role of the Werner syndrome RecQ helicase in human fibroblasts. Aging Cell 6: 53-61. DOI: 10.1111/j.1474-9726.2006.00260.x. PMID: 17266675


Sharma A, Awasthi S, Harrod CK, Matlock EF, Khan S, Xu L, Chan S, Yang H, Thammavaram CK, Rasor RA, Burns DK, Skiest DJ, Van Lint C, Girard AM, McGee M, Monnat RJ Jr, Harrod R. (2007) The Werner syndrome helicase is a cofactor for HIV-1 long terminal repeat transactivation and retroviral replication. J Biol Chem. 282:12048-57. doi: 10.1074/jbc.M608104200. PMID: 17317667


Volna, P., Jarjour, J. Baxter, S., Roffler, S., Monnat, RJ Jr., Stoddard, B. and Scharenberg, A. (2007) Flow cytometric analysis of DNA binding and cleavage by cell surface-displayed homing endonucleases. Nucleic Acids Research 35: 2748-2758. doi: 10.1093/nar/gkm182. PMCID: PMC1885675


Berkovich, E., Monnat, RJ Jr. and Kastan, M. (2007) Roles of ATM and NBS1 in chromatin structure modulation and DNA double strand break repair. Nature Cell Biology 9: 683-690. doi:10.1038/ncb1599. PMID: 17486112


Monnat, RJ Jr. (2007) From broken to old: DNA damage, IGF1 endocrine suppression and aging. DNA Repair 6: 1386-1390 (invited commentary). doi: 10.1016/j.dnarep.2007.03.018. PMCID: PMC2704237


Eastberg, J., Eklund, J., Monnat, RJ Jr. and Stoddard, B. (2007) Mutability of an HNH nuclease imidazole general base and exchange of a deprotonation mechanism. Biochemistry 46: 7215- 7225. DOI: 10.1021/bi700418d. PMID: 17516660


Kudlow, B., Kennedy, B.K., and Monnat, RJ Jr. (2007) Werner and Hutchinson-Gilford progeria syndromes: mechanistic basis of human progeroid diseases. Nature Reviews Molecular and Cellular Biology 8: 394-404. doi:10.1038/nrm2161. PMID: 17450177


Eklund, J.L., Ulge, U.Y., Eastberg, J. and Monnat, R.J., Jr. (2007) Altered target site specificity variants of the I-PpoI His-Cys box homing endonuclease.  Nucleic Acids Research 35:5839- 5850. doi: 10.1093/nar/gkm624. PMCID: PMC2034468


2006 Publications


Monnat, R.J., Jr. (2006) Werner Syndrome as a Model of Human Aging. Handbook of Models of Human Aging, P. Michael Conn, ed. Elsevier Academic Press, Chapter 80, pp. 961-976.


Ashworth, J., Havranek, J.J., Duarte, C.M., Sussman, D., Monnat, R.J., Jr., Stoddard, B.L. and Baker, D. (2006) Computational redesign of endonuclease DNA binding and cleavage specificity. Nature 441:656-659. doi:10.1038/nature04818.

2005 Publications


Awasthi S, Sharma A, Wong K, Zhang J, Matlock EF, Rogers L, Motloch P,  Takemoto S, Taguchi, H, Cole MD, Luscher B, Dittrich O, Tagami H, Nakatani Y, McGee M, Girard AM, Gaughan L, Robson CN, Monnat RJ Jr, and Harrod R.(2005) A human T-cell lymphotropic virus type 1 enhancer of Myc transforming potential stabilizes Myc-TIP60 transcriptional interactions. Mol Cell Biol. 25:6178-98. doi: 10.1128/MCB.25.14.6178-6198.2005. PMCID: PMC1168837


Chevalier, B., Monnat, R.J., Jr. and Stoddard, B.L. (2005) The LAGLIDADG Homing Endonuclease Family. Homing Endonucleases and Inteins, M. Belfort, B.L. Stoddard, D.W. Wood, V. Derbyshire (Eds). Springer, Berlin. pp.33-47. Doi: 10.1007/3-540-29474-0_3


2004 Publications


Swanson, C., Saintigny, Y., Emond, M.J. and Monnat, R.J., Jr. (2004) The Werner syndrome protein has separable recombination and survival functions. DNA Repair 3:475-482. doi:10.1016/j.dnarep.2004.01.002. PMID: 15084309


Monnat, R.J., Jr. and Saintigny, Y. (2004) The Werner syndrome protein: in vivo function and disease links. Science SAGE-KE (;2004/13/re3). DOI: 10.1126/sageke.2004.13.re3.


Sussman, D., Chadsey, M., Fauce, S., Engel, A., Bruett, A., Monnat, R. Jr., Stoddard, B. L. and Seligman, L.M. (2004) Isolation and characterization of new homing endonuclease specificities at individual target site positions. J. Mol. Biol. 342:31-41. doi:10.1016/j.jmb.2004.07.031. PMID: 15313605


Chevalier, B.S., Sussman, J., Otis, C., Nöel, A-J., Turmel, M., Lemieux, C., Stephens, K., Monnat,R.J., Jr. and Stoddard, B.L.(2004) Metal-dependent DNA cleavage mechanism of the I- CreI LAGLIDADG homing endonuclease. Biochemistry 43:14015-14022. DOI: 10.1021/bi048970c. PMID: 15518550


2003 Publications


Chevalier, B.S., Turmel, M., Lemieux, C., Monnat, R.J., Jr. and Stoddard, B.L.(2003) Flexible DNA target site recognition by divergent homing endonuclease isoschizomers I-CreI and I-MsoI. J. Mol. Biol.  329:253-269. Doi: 10.1016/S0022-2836(03)00447-9. PMID: 12758074


Grandori, C., Wu, K-J., Fernandez, P., Ngouenet, C., Grim, J., Clurman, B.E., Moser, M.J., Oshima,J., Russell, D.W., Swisshelm, K., Frank, S., Amati, B., Dalla-Favera, R. and Monnat, R.J., Jr. (2003) Werner syndrome protein limits MYC-induced cellular senescence. Genes & Development 17:1569-1574. doi: 10.1101/gad.1100303.  PMCID: PMC196129


2002 Publications


Saintigny, Y. and Monnat, R.J., Jr. (2002) L’Hélicase RecQ, dont les altérations sont à l’origine du syndrome de Werner, participe à la réparation de l’ADN par recombinaison homologue. Médecine Sciences 18:79-85. doi: 10.1051/medsci/200218179.


Colgin, L.M., Hackmann, A.F.M., Emond, M.J. and Monnat, R.J., Jr. (2002) The unexpected landscape of in vivo somatic mutation in a human epithelial cell lineage. Proc. Natl. Acad. Sci. USA 99:1437-1442. doi: 10.1073/pnas.032655699. PMCID: PMC122209


Saintigny,Y., Makienko, K., Swanson, C., Emond, M.J. and Monnat, R.J., Jr. (2002) A homologous recombination resolution defect in Werner syndrome. Molec. Cell. Biol. 22:6971- 6978. doi: 10.1128/MCB.22.20.6971-6978.2002. PMCID: PMC139822


Chevalier, B.S., Kortemme,T., Chadsey, M.S., Baker, D., Monnat, R.J., Jr. and Stoddard, B.L. (2002) Design, activity and structure of E-DreI, a highly site-specific artificial endonuclease. Molecular Cell 10:895-905. DOI:  PMID: 12419232


Monnat, R.J., Jr. (2002) Werner syndrome. WHO/IARC Monograph on Pathology and Genetics of Tumours of Soft Tissue and Bone, edited by C. Fletcher, K. Unni and F.Mertens. IARC Press, Lyon. pp. 366-367.

2001 Publications


Chevalier, B.S., Monnat, R.J., Jr. and Stoddard, B.L. (2001) The LAGLIDADG homing endonuclease I-CreI shares three metals between two active sites. Nature Structural Biology 8:312-316. doi:10.1038/87656


Prince, P.R., Emond, M.J. and Monnat, R.J., Jr. (2001) Loss of Werner syndrome protein function promotes aberrant mitotic recombination. Genes & Development 15:933-938. doi: 10.1101/gad.877001. PMCID: PMC312674


Monnat, R.J., Jr. (2001) Cancer pathogenesis in the human RecQ helicase deficiency syndromes. From Premature Gray Hair to Helicase: Werner Syndrome Implications for Aging and Cancer. Edited by M. Goto and R.W. Miller. GANN Monograph on Cancer Research 49:83-94.

2000 Publications


Moser, M.J., Kamath-Loeb, A.S., Jacob, J.E., Bennett, S.E., Oshima, J. and Monnat, R.J., Jr. (2000) WRN helicase expression in Werner syndrome cell lines. Nucleic Acids Res. 28:648-654. doi: 10.1093/nar/28.2.648. PMCID: PMC102521


Moser, M.J., Bigbee, W.L., Grant, S.G., Emond, M.J., Langlois, R.G., Jensen, R.H., Oshima, J. and Monnat,R.J., Jr. (2000) Genetic instability and hematologic disease risk in Werner syndrome patients and heterozygotes. Cancer Research 60:2492-2496. PMID: 10811130


Flory, M.R., Moser, M.J., Monnat, R.J., Jr. and Davis, T.N. (2000) Identification of a human centrosomal calmodulin-binding protein that shares homology with pericentrin. Proc. Natl. Acad. Sci. USA 97:5919-5923. PMCID: PMC18534


Galburt, E.A., Chadsey, M.S., Jurica, M.S., Chevalier, B.S., Ehro, D., Tang, W., Monnat, R.J., Jr. and Stoddard, B.L. (2000) Conformational changes and cleavage by the homing endonuclease I-PpoI: a critical role for a leucine residue in the active site. J. Mol. Biol. 300:877-887. doi:10.1006/jmbi.2000.3874.  PMID: 10891275

1999 Publications


Bennett, S.E., Umar, A., Kodama, S., Barrett, J.C., Monnat, R.J., Jr. and Kunkel, T.A. (1999) Evidence against a role for the Werner syndrome gene product in DNA mismatch repair. Molecular biology of Aging, Alfred Benzon Symposium Proceedings, No. 44. Bohr, V.A., Clark, B.F.C. and Stevnsner, T. Munksgaard, Copenhagen. pp.214-224.


Moser, M.J., Oshima, J., and Monnat, R.J., Jr. (1999) Invited Mutation Update: WRN mutations in Werner syndrome. Human Mutation 13:271-279.


Monnat, R.J., Jr., Hackmann, A.F.M. and Cantrell, M.A. (1999) Generation of highly site-specific DNA double strand breaks in human cells by the homing endonucleases I-PpoI and I-CreI. Biochem. Biophys. Res. Comm. 255:88-93. Article id: bbrc.1999.0152 PMID: 10082660


Colgin, L.M., Hackmann, A.F.M., and Monnat, R.J., Jr. (1999) Different somatic and germline HPRT1 mutations promote use of a common, cryptic intron 1 splice site. Human Mutation 13:504-505 (also published electronically as Human Mutation Mutation in Brief #246 (1999) Online: DOI: 10.1002/(SICI)1098- 1004(1999)14:2<182::AID-HUMU12>3.0.CO;2-Y. PMID:10447274


Prince, P.R., Ogburn, C.E., Moser, M.J., Emond, M.J., Martin, G.M. and Monnat, R.J., Jr. (1999) Cell fusion corrects the 4-nitroquinoline 1-oxide sensitivity of Werner syndrome fibroblast cell lines. Human Genetics 105:132-138. PMID: 10480367


Monnat, R.J., Jr. (1999) Unusual features of thyroid carcinomas in Japanese patients with Werner syndrome and possible genotype-phenotype relations to cell type and race (originally ‘No association of specific WRN mutations with thyroid cancer risk or histologic subtype in Werner syndrome patients’ prior to editorial retitling). Cancer 86:728-729.


Galburt, E., Chevalier, B., Tang, W., Jurica, M.S., Flick, K.E., Monnat, R.J., Jr. and Stoddard,B.L. (1999) A novel endonuclease mechanism directly visualized for I-PpoI. Nature Structural Biology 6:1096-1099. doi:10.1038/70027. PMID: 10581547


1998 Publications


Argast, G., Stephens, K.M., Emond, M.J., and Monnat, R.J., Jr. (1998) I-PpoI and I- CreI homing site sequence degeneracy determined by random mutagenesis and sequential in vitro enrichment. J. Mol. Biol. 280:345-353. doi:10.1006/jmbi.1998.1886. PMID: 9665841


Flick, K.E., Jurica, M.S., Monnat, R.J., Jr. and Stoddard, B.L. (1998) DNA binding and cleavage by the nuclear intron-encoded homing endonuclease I-PpoI. Nature 394:96-101. doi:10.1038/27952. PMID: 9665136


Jurica, M.S., Monnat, R.J., Jr. and Stoddard, B.L. (1998) DNA recognition and cleavage by the LAGLIDADG homing endonuclease I-CreI. Molecular Cell 2:469-476.  doi:10.1016/S1097-2765(00)80146-X. PMID: 9809068


Moser, M.J., Oshima, J., Martin, G.M. and Monnat, R.J.,Jr. (1998) Werner syndrome mutational database and website. Available at: (1 November 1998).


1997 Publications


Brooks-Wilson, A.R., Emond, M. and Monnat, R.J., Jr. (1997) Paradoxically low loss of heterozygosity (LOH) in genetically unstable Werner syndrome cell lines. Genes, Chromosomes & Cancer 18:133-142.


Stephens, K.M., Monnat, R.J., Jr., Heath, P.D., and Stoddard, B.M. (1997) Crystallization and preliminary X-ray analysis of the Chlamydomonas reinhardtii I-CreI mobile intron endonuclease. Proteins: Structure, Function and Genetics 28:137-139. DOI: 10.1002/(SICI)1097-0134(199705)28:1<137::AID-PROT15>3.0.CO;2-K


Heath, P.D., Stephens, K.M., Monnat, R.J., Jr. and Stoddard, B.M. (1997) The structure of I-CreI: a Group I intron-encoded homing endonuclease. Nature Structural Biology 4:468-476. doi:10.1038/nsb0697-468.


Bennett, S., Umar, A., Oshima, J., Monnat, R.J., Jr. and Kunkel, T.M. (1997) Mismatch repair in extracts from Werner syndrome cell lines. Cancer Research 57: 256-2960. PMID: 9230208


Seligman, L.M., Stephens, K.M. and Monnat, R.J., Jr. (1997) Genetic analysis of the Chlamydomonas reinhardtii I-CreI mobile intron homing system in Escherichia coli. Genetics 147:1653-1664. PMCID: PMC1208338


Flick, K.E., McHugh, D., Heath, J.D., Stephens, K.M., Monnat, R.J., Jr., and Stoddard, B.L. (1997) Crystallization and preliminary X-ray studies of I-PpoI: a nuclear, intron-encoded homing endonuclease from P. polycephalum. Protein Science 6:1-4. DOI: 10.1002/pro.5560061226. PMCID: PMC2143617


Bridges, B.A., Drake, J.A., Fishel, R., Foster, P., Monnat, R.J., Jr., Ripley, L.S. and Taddei, F. (1997). “Optimum mutation rates in evolution and disease” (invited and moderated on-line discussion as part of the HMS Beagle Cutting Edge Debate series). HMS Beagle (13), 25 July 1997 (HMS Beagle web site: overview.htm).

1996 Publications


Martin, G.M., Ogburn, C.E., Colgin, L.M., Gown, A.M., Edland, S.D. and Monnat, R.J., Jr. (1996) Somatic mutations are frequent and increase with age in human kidney epithelial cells. Human Molec. Genetics 5:215-221. doi: 10.1093/hmg/5.2.215. PMID: 8824877

1993 Publications


Bendich,A.J., Loretz, C.J., and Monnat, R.J.,Jr. (1993) The structure of the plant mitochondrial genome. Plant Mitochondria: with emphasis on RNA editing and cytoplasmic male sterility, ed. A. Brennicke and U. Kück, pp.171-180. VCH Publishers, Inc, Weinheim.


Liu, P.K., Trujillo, J.M., and Monnat, R.J., Jr. (1993) Spectrum of spontaneous mutation in animal cells containing an aphidicolin-resistant DNA polymerase α. Mutation Research 288:229-236. doi:10.1016/0027-5107(93)90089-X. PMID: 7688082


Maresh, G.A. and Monnat, R.J., Jr. (1993) Novel fluoride-stimulated dichlorofluorescein dye oxidation pathway in human leukemia cell lines. Biochem Biophys Res. Comm. 194:869-875. doi:10.1006/bbrc.1993.1902. PMID: 8393671

1992 Publications


Chiaverotti, T.A, Battula, N., and Monnat, R.J., Jr. (1992) Rat hypoxanthine phosphoribo-syltransferase cDNA cloning and sequence analysis. Genomics 11:1158-1160. doi:10.1016/0888-7543(91)90046-H. PMID: 1783384


Monnat, R.J., Jr., Hackmann, A.F.M., and Chiaverotti, T.A. (1992) Nucleotide sequence analysis of human hypoxanthine phosphoribosyltransferase (HPRT) gene deletions. Genomics 11:777-787. doi:10.1016/0888-7543(92)90153-J. PMID: 1639404


Monnat, R.J., Jr., Chiaverotti, T.A., Hackmann, A.F.M., and Maresh, G.A. (1992) Molecular structure and genetic stability of human hypoxanthine phosphoribosyltransferase (HPRT) gene duplications. Genomics 11:788-796. doi:10.1016/0888-7543(92)90154-K. PMID: 1639405


Monnat, R.J., Jr. (1992) Werner syndrome: molecular genetics and mechanistic hypotheses. Exp. Gerontol. 27:447-453. PMID: 1459221

1991 Publications


Monnat, R.J., Jr., Hackmann, A.F.M., Chiaverotti, T.A., and Maresh, G.A. (1991) Molecular analysis of human hprt gene deletions and duplications. Adv. Exp. Biol. Med. 309B:113-116. PMID: 1781354


Chiaverotti, T.A., Battula, N. and Monnat, R.J., Jr. (1991) Rat hypoxanthine phosphoribosyltransferase cDNA cloning and sequence analysis. Adv. Exp. Biol. Med. 309B:117-120. PMID:



1990 Publications


Fukuchi, K.-i., Tanaka, K., Kumahara, Y., Marumo, K., Pride, M.B., Martin, G.M., and Monnat,R.J., Jr. (1990) Increased frequency of 6-thioguanine-resistant peripheral blood lymphocytes in Werner syndrome patients. Human Genetics 84:249-252. PMID:2303247

1989 Publications


Monnat, R.J., Jr. (1989) Molecular analysis of spontaneous hypoxanthine phosphoribosyl-transferase mutations in thioguanine-resistant HL-60 human leukemia cells. Cancer Research 49:81-87. PMID: 2908855


Fukuchi, K.-i., Martin, G.M., and Monnat, R.J., Jr. (1989) Mutator phenotype of Werner syndrome is characterized by extensive deletions. Proc. Natl. Acad. Sci. USA 86:5893-5897 (errata in Proc. Natl. Acad. Sci. USA 86:7994, 1989). PMCID: PMC297737


Monnat, R.J., Jr. (1989) General pathology teaching at the University of Washington. J. Pathol. 159:345-349. PMID: 2614580

1988 Publications


Mita, S., Monnat, R.J., Jr. and Loeb, L.A. (1988) Direct selection of mutations in the human mitochondrial tRNAThr gene: reversion of an "uncloneable" phenotype. Mutation Research 199:183-190. doi:10.1016/0027-5107(88)90244-8. PMID: 3362158


Turker, M.S., Monnat, R.J., Jr., Fukuchi, K., Johnston, P.A., Ogburn, C.E., Weller, R.E., Park, J.F. and Martin, G.M. (1988) A novel class of unstable 6-thioguanine resistant cells from dog and human kidneys. Cell Biol.Toxicol. 4:211-223. PMID: 3233532


Mita, S., Monnat, R.J., Jr. and Loeb, L.A. (1988) Resistance of HeLa cell mitochondrial DNA to mutagenesis by chemical carcinogens. Cancer Research 48:4578-4583. PMID: 3135115

1986 Publications


Monnat, R.J., Jr. and Reay, D.T. (1986) Nucleotide sequence identity of mitochondrial DNA from different human tissues. Gene 43:205-211. doi:10.1016/0378-1119(86)90208-8. PMID: 3744049

1985 Publications


Monnat, R.J., Jr. and Loeb, L.A. (1985) Nucleotide sequence preservation of human mitochondrial DNA. Proc. Natl. Acad. Sci. USA 82:2895-2899. PMID: 2986144


Monnat, R.J., Jr., Maxwell, C.L. and Loeb, L.A. (1985) Nucleotide sequence preservation of human leukemic mitochondrial DNA. Cancer Research 45:1809-1814. PMID: 3978642

1983 Publications


Monnat, R.J., Jr. and Loeb, L.A. (1983) Mechanisms of neoplastic transformation:"Molecular Biology and Malignancy" Series Introduction). Cancer Invest. 1:175-183.

1982 Publications


Rutledge, J.D., Haas, J.E., Monnat, R.J., Jr. and Milstein, J.M. (1982) Hypertrophic cardiomyopathy is a component of subacute necrotizing encephalomyelopathy. J.Pediatr. 101:706-710. PMID: 6890100

1980 Publications


Monnat, R.J., Jr. (1980) Gneuropeptides. New Scientist 87:54 (letter).


Monnat, R.J., Jr. (1980) Pulmonary calcification in a renal transplant recipient. Amer. Rev. Resp. Dis. 122:355 (letter).

1979 Publications


Martin, G.M., Ogburn, C.E., Colgin, L.M., Gown, A.M., Edland, S.D. and Monnat, R.J., Jr. (1996) Somatic mutations are frequent and increase with age in human kidney epithelial cells. Human Molec. Genetics 5:215-221. doi: 10.1093/hmg/5.2.215. PMID: 8824877

2020 Publications

Horowitz L. F., Rodriguez A.D., Dereli-Korkut Z., Lin R., Castro K., Mikheev A., Monnat R.J. Jr., Folch A. and Rostomily R.C. (2020) Multiplexed drug testing of tumor slices using a microfluidic platform NPJ Precision Oncology 19;4:12. PMID: 32435696 PMC7237421. doi: 10.1038/s41698-020-0117-y.

Rodriguez A.D., Horowitz L. F., Castro K., Kenerson H., Bhattacharjee N., Gandhe G., Raman A., Monnat R. J. Jr., Yueng R., Rostomily R.C.  and Folch A.  (2020) A microfluidic platform for fuctional testing of cancer drugs on intact tumor slices. Lab on a Chip 7;20(9):1658-1675. PMID: 32270149. doi: 10.1039/c9lc00811j

Hasle N, Cooke A, Srivatsan S, Huang H, Stephany JJ, Krieger Z, Jackson D, Tang Weilliang, Pendyala S, Monnat RJ Jr., Trapnell C, Hatch EM, and Fowler DM (2020). High-throughput microscope-based sorting to dissect cellular heterogeneity. Molecular Systems Biology 16(6):e9442. PMID: 32500953 PMCID: PMC7273721 DOI: 10.15252/msb.20209442.

Carbone M, Arron ST, Beutler B, Bononi A, Cavenee W, Cleaver JE, Croce CM, D'Andrea A, Foulkes WD, Gaudino G, Groden JL, Henske EP, Hickson ID, Hwang PM, Kolodner RD, Mak TW, Malkin D, Monnat RJ Jr, Novelli F, Pass HI, Petrini JH, Schmidt LS, Yang H. (2020) Tumour predisposition and cancer syndromes as models to study gene-environment interactions. Nature  Reviews Cancer PMID: 32472073 DOI: 10.1038/s41568-020-0265-y.

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