© 2019 Monnat Lab University of Washington. Last Update: 20 Sep 2019. Contact Ray Monnat for website questions & comments.

Disclaimer: Information is provided here as a courtesy, and for educational purposes only. The information provided is accurate to the best of our knowledge. End users assume full responsibility for use, and will not hold the Monnat Lab or the UW Departments of Pathology and Genome Sciences responsible or liable for claims, loss or damage arising from use of the information presented here.

 
 
 
 
 

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. https://doi.org/10.1016/j.celrep.2018.03.076 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 https://doi.org/10.1101/396390 (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. http://www.els.net [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 (sageke.sciencemag.org/cgi/content/full/sageke;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: http://dx.doi.org/10.1016/S1097-2765(02)00690-1.  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: http://journals.wiley.com/1059-7794/pdf/mutation/246.pdf 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: http://www.pathology.washington.edu/werner/ (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: http://biomednet.com/hmsbeagle/14/cutedge/ 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:

1781355

 

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