The Monnat Lab | Publications

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Werner Syndrome | Homing Endoculeases | Human Cell Mutagenesis

Publications not available in pdf format may be obtained by contacting Ray Monnat

Werner Syndrome Publications

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 (submitted-in review)

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. (2009) Werner syndrome protein loss leads to divergent mouse and human cell
phenotypes. (submitted-in review)

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 (in press)

Sidorova, J.M. (2008) Roles of the Werner syndrome RecQ helicase in DNA replication. DNA Repair
7:1776-1786. [ pdf ]

Loeb, L.A. and Monnat, R.J., Jr. (2008) DNA polymerases and human disease. Nature Reviews Genetics
9:594-604 [ pdf ]

Sidorova, J.M., Li, N., Folch, A., Monnat, R.J., Jr., The RecQ helicase WRN is required for normal replication
fork progression after DNA damage or replication fork arrest. Cell Cycle. 2008 Jan 4;7(6). [ pdf ]

Sharma, A., Awasthi, S., Harrod, C.K., Matlock, E.F., Khan, S., Xu, L., Chan, S., Yang, H., Thammavaram,
C.K., Rasor, R.A., Burns, D.K., Skiest, D.J., Van Lint, C., Girard, A.M., McGee, M., Monnat, R.J. Jr.,
Harrod, R. (2007) The Werner syndrome helicase is a cofactor for HIV-1 long terminal repeat transactivation
and retroviral replication. J Biol Chem. 2007 Apr 20;282(16):12048-57. [ pdf ]

Dhillon, K., Sidorova, 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. [ pdf ]

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.
[ pdf ]

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

Awasthi, S., Sharma, A., Wong, K., Zhang, J., Matlock, E.F., Rogers, L., Motloch, P., Takemoto, S., Taguchi,
H., Cole, M.D., Luscher, B., Dittrich, O., Tagami, H., Nakatani, Y., McGee, M., Girard, A.M., Gaughan,
L., Robson, C.N., Monnat, R.J. 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. [ pdf ]

Monnat, R.J., Jr. (2005) ‘Werner Syndrome as a Model of Human Aging’, in Handbook of Models of Human
Aging, P. Michael Conn, ed. Elsevier Academic Press: chapter 80, pp.961-976.

Monnat, R.J., Jr. and Saintigny, Y. (2004) The Werner syndrome protein: in vivo function and disease
links. Science’s SAGE-KE (available at: http://sageke.sciencemag.org/cgi/content/full/2004/13/re3).
[ pdf ]

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. [ pdf ]

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.

Monnat, R.J., Jr. (2002) ‘Werner syndrome’, in 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.

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. [ pdf ]

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.

Monnat, R.J., Jr. (2001) Cancer pathogenesis in the human RecQ helicase deficiency syndromes, in ‘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.

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. [ pdf ]

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. [ PDF ]

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.
[ pdf ]

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 entitled ‘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. [ pdf ]

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. [ pdf ]

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.

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.

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

Monnat, R.J., Jr. (1992) Werner syndrome: molecular genetics and mechanistic hypotheses. Exp.
Gerontol. 27:447-453. (first prediction that that the WRN protein may have helicase activity)

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.

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). [ pdf ]

Homing Endonucleases Publications

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. 2009 Jan 19. [ pdf ]
[ supplemental data pdf ]

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 froms the I-AniI
homing LAGLIDADG endonuclease. Proc Natl Acad Sci USA (in press).

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. [ pdf ]

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. [ pdf ]

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(17):5839-50. [ pdf ]

Monnat, R.J. Jr. (2007) From broken to old: DNA damage, IGF1 endocrine suppression and aging. DNA
Repair 2007 Sep 1; 6(9): 1386-90. [ pdf ]

Berkovich, E., Monnat, R.J., 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. [ pdf ]

Eastberg, J., Eklund, J., Monnat, R.J. Jr. and Stoddard, B. (2007) Mutability of an HNH nuclease imidazole
general base and exchange of a deprotonation mechanism. Biochemistry 46:7215-7225. [ pdf ]

Volna, P., Jarjour, J. Baxter, S., Roffler, S., Monnat, R.J., 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. [ pdf ]

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. [ pdf ]

Chevalier, B., Monnat, R.J., Jr. and Stoddard, B.L. (2005) 'The LAGLIDADG Homing Endonuclease Family' In
Homing Endonucleases and Inteins, M. Belfort, B.L. Stoddard, D.W. Wood, V. Derbyshire (Eds). Springer,
Berlin. pp.33-47.

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. [ pdf ]

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. [ pdf ]

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. [ pdf ]

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. [ pdf ]

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. [ pdf ]

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. [ pdf ]

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. [ pdf ]

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. [ pdf ]

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. [ pdf ]

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. [ pdf ]

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. [ pdf ]

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. [ pdf ]

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.

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. [ pdf ]

Human Cell Mutagenesis Publications

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. [ pdf ]

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. [ pdf ]

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. [ pdf ]

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.

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.

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