Peggy F Shelbourne, Christine Keller-McGandy, Wenya Linda Bi, Song-Ro Yoon, Louis Dubeau, Nicola J Veitch, Jean Paul Vonsattel, Nancy S Wexler, Norman Arnheim, Sarah J Augood. Hum Mol Genet 2007
Times Cited: 127
Times Cited: 127
Times Cited
Times Co-cited
Similarity
Dramatic tissue-specific mutation length increases are an early molecular event in Huntington disease pathogenesis.
Laura Kennedy, Elizabeth Evans, Chiung-Mei Chen, Lyndsey Craven, Peter J Detloff, Margaret Ennis, Peggy F Shelbourne. Hum Mol Genet 2003
Laura Kennedy, Elizabeth Evans, Chiung-Mei Chen, Lyndsey Craven, Peter J Detloff, Margaret Ennis, Peggy F Shelbourne. Hum Mol Genet 2003
62
Somatic expansion of the Huntington's disease CAG repeat in the brain is associated with an earlier age of disease onset.
Meera Swami, Audrey E Hendricks, Tammy Gillis, Tiffany Massood, Jayalakshmi Mysore, Richard H Myers, Vanessa C Wheeler. Hum Mol Genet 2009
Meera Swami, Audrey E Hendricks, Tammy Gillis, Tiffany Massood, Jayalakshmi Mysore, Richard H Myers, Vanessa C Wheeler. Hum Mol Genet 2009
48
Intergenerational and striatal CAG repeat instability in Huntington's disease knock-in mice involve different DNA repair genes.
Ella Dragileva, Audrey Hendricks, Allison Teed, Tammy Gillis, Edith T Lopez, Errol C Friedberg, Raju Kucherlapati, Winfried Edelmann, Kathryn L Lunetta, Marcy E MacDonald,[...]. Neurobiol Dis 2009
Ella Dragileva, Audrey Hendricks, Allison Teed, Tammy Gillis, Edith T Lopez, Errol C Friedberg, Raju Kucherlapati, Winfried Edelmann, Kathryn L Lunetta, Marcy E MacDonald,[...]. Neurobiol Dis 2009
40
Msh2 deficiency prevents in vivo somatic instability of the CAG repeat in Huntington disease transgenic mice.
K Manley, T L Shirley, L Flaherty, A Messer. Nat Genet 1999
K Manley, T L Shirley, L Flaherty, A Messer. Nat Genet 1999
40
39
DNA instability in postmitotic neurons.
Roman Gonitel, Hilary Moffitt, Kirupa Sathasivam, Ben Woodman, Peter J Detloff, Richard L M Faull, Gillian P Bates. Proc Natl Acad Sci U S A 2008
Roman Gonitel, Hilary Moffitt, Kirupa Sathasivam, Ben Woodman, Peter J Detloff, Richard L M Faull, Gillian P Bates. Proc Natl Acad Sci U S A 2008
38
Mismatch repair gene Msh2 modifies the timing of early disease in Hdh(Q111) striatum.
Vanessa C Wheeler, Lori-Anne Lebel, Vladimir Vrbanac, Allison Teed, Hein te Riele, Marcy E MacDonald. Hum Mol Genet 2003
Vanessa C Wheeler, Lori-Anne Lebel, Vladimir Vrbanac, Allison Teed, Hein te Riele, Marcy E MacDonald. Hum Mol Genet 2003
37
Somatic and gonadal mosaicism of the Huntington disease gene CAG repeat in brain and sperm.
H Telenius, B Kremer, Y P Goldberg, J Theilmann, S E Andrew, J Zeisler, S Adam, C Greenberg, E J Ives, L A Clarke. Nat Genet 1994
H Telenius, B Kremer, Y P Goldberg, J Theilmann, S E Andrew, J Zeisler, S Adam, C Greenberg, E J Ives, L A Clarke. Nat Genet 1994
36
Identification of Genetic Factors that Modify Clinical Onset of Huntington's Disease.
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34
Mismatch repair genes Mlh1 and Mlh3 modify CAG instability in Huntington's disease mice: genome-wide and candidate approaches.
Ricardo Mouro Pinto, Ella Dragileva, Andrew Kirby, Alejandro Lloret, Edith Lopez, Jason St Claire, Gagan B Panigrahi, Caixia Hou, Kim Holloway, Tammy Gillis,[...]. PLoS Genet 2013
Ricardo Mouro Pinto, Ella Dragileva, Andrew Kirby, Alejandro Lloret, Edith Lopez, Jason St Claire, Gagan B Panigrahi, Caixia Hou, Kim Holloway, Tammy Gillis,[...]. PLoS Genet 2013
31
OGG1 initiates age-dependent CAG trinucleotide expansion in somatic cells.
Irina V Kovtun, Yuan Liu, Magnar Bjoras, Arne Klungland, Samuel H Wilson, Cynthia T McMurray. Nature 2007
Irina V Kovtun, Yuan Liu, Magnar Bjoras, Arne Klungland, Samuel H Wilson, Cynthia T McMurray. Nature 2007
30
Instability of highly expanded CAG repeats in mice transgenic for the Huntington's disease mutation.
L Mangiarini, K Sathasivam, A Mahal, R Mott, M Seller, G P Bates. Nat Genet 1997
L Mangiarini, K Sathasivam, A Mahal, R Mott, M Seller, G P Bates. Nat Genet 1997
28
Somatic expansion behaviour of the (CTG)n repeat in myotonic dystrophy knock-in mice is differentially affected by Msh3 and Msh6 mismatch-repair proteins.
Walther J A A van den Broek, Marcel R Nelen, Derick G Wansink, Marga M Coerwinkel, Hein te Riele, Patricia J T A Groenen, Bé Wieringa. Hum Mol Genet 2002
Walther J A A van den Broek, Marcel R Nelen, Derick G Wansink, Marga M Coerwinkel, Hein te Riele, Patricia J T A Groenen, Bé Wieringa. Hum Mol Genet 2002
28
DNA repair pathways underlie a common genetic mechanism modulating onset in polyglutamine diseases.
Conceição Bettencourt, Davina Hensman-Moss, Michael Flower, Sarah Wiethoff, Alexis Brice, Cyril Goizet, Giovanni Stevanin, Georgios Koutsis, Georgia Karadima, Marios Panas,[...]. Ann Neurol 2016
Conceição Bettencourt, Davina Hensman-Moss, Michael Flower, Sarah Wiethoff, Alexis Brice, Cyril Goizet, Giovanni Stevanin, Georgios Koutsis, Georgia Karadima, Marios Panas,[...]. Ann Neurol 2016
28
Dramatic mutation instability in HD mouse striatum: does polyglutamine load contribute to cell-specific vulnerability in Huntington's disease?
L Kennedy, P F Shelbourne. Hum Mol Genet 2000
L Kennedy, P F Shelbourne. Hum Mol Genet 2000
27
Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice.
L Mangiarini, K Sathasivam, M Seller, B Cozens, A Harper, C Hetherington, M Lawton, Y Trottier, H Lehrach, S W Davies,[...]. Cell 1996
L Mangiarini, K Sathasivam, M Seller, B Cozens, A Harper, C Hetherington, M Lawton, Y Trottier, H Lehrach, S W Davies,[...]. Cell 1996
27
Identification of genetic variants associated with Huntington's disease progression: a genome-wide association study.
Davina J Hensman Moss, Antonio F Pardiñas, Douglas Langbehn, Kitty Lo, Blair R Leavitt, Raymund Roos, Alexandra Durr, Simon Mead, Peter Holmans, Lesley Jones,[...]. Lancet Neurol 2017
Davina J Hensman Moss, Antonio F Pardiñas, Douglas Langbehn, Kitty Lo, Blair R Leavitt, Raymund Roos, Alexandra Durr, Simon Mead, Peter Holmans, Lesley Jones,[...]. Lancet Neurol 2017
27
CAG Repeat Not Polyglutamine Length Determines Timing of Huntington's Disease Onset.
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27
Pms2 is a genetic enhancer of trinucleotide CAG.CTG repeat somatic mosaicism: implications for the mechanism of triplet repeat expansion.
Mário Gomes-Pereira, M Teresa Fortune, Laura Ingram, John P McAbney, Darren G Monckton. Hum Mol Genet 2004
Mário Gomes-Pereira, M Teresa Fortune, Laura Ingram, John P McAbney, Darren G Monckton. Hum Mol Genet 2004
26
MSH3 polymorphisms and protein levels affect CAG repeat instability in Huntington's disease mice.
Stéphanie Tomé, Kevin Manley, Jodie P Simard, Greg W Clark, Meghan M Slean, Meera Swami, Peggy F Shelbourne, Elisabeth R M Tillier, Darren G Monckton, Anne Messer,[...]. PLoS Genet 2013
Stéphanie Tomé, Kevin Manley, Jodie P Simard, Greg W Clark, Meghan M Slean, Meera Swami, Peggy F Shelbourne, Elisabeth R M Tillier, Darren G Monckton, Anne Messer,[...]. PLoS Genet 2013
31
Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse.
V C Wheeler, W Auerbach, J K White, J Srinidhi, A Auerbach, A Ryan, M P Duyao, V Vrbanac, M Weaver, J F Gusella,[...]. Hum Mol Genet 1999
V C Wheeler, W Auerbach, J K White, J Srinidhi, A Auerbach, A Ryan, M P Duyao, V Vrbanac, M Weaver, J F Gusella,[...]. Hum Mol Genet 1999
25
Repeat instability as the basis for human diseases and as a potential target for therapy.
Arturo López Castel, John D Cleary, Christopher E Pearson. Nat Rev Mol Cell Biol 2010
Arturo López Castel, John D Cleary, Christopher E Pearson. Nat Rev Mol Cell Biol 2010
25
Trinucleotide repeat length instability and age of onset in Huntington's disease.
M Duyao, C Ambrose, R Myers, A Novelletto, F Persichetti, M Frontali, S Folstein, C Ross, M Franz, M Abbott. Nat Genet 1993
M Duyao, C Ambrose, R Myers, A Novelletto, F Persichetti, M Frontali, S Folstein, C Ross, M Franz, M Abbott. Nat Genet 1993
21
A novel approach to investigate tissue-specific trinucleotide repeat instability.
Jong-Min Lee, Jie Zhang, Andrew I Su, John R Walker, Tim Wiltshire, Kihwa Kang, Ella Dragileva, Tammy Gillis, Edith T Lopez, Marie-Josee Boily,[...]. BMC Syst Biol 2010
Jong-Min Lee, Jie Zhang, Andrew I Su, John R Walker, Tim Wiltshire, Kihwa Kang, Ella Dragileva, Tammy Gillis, Edith T Lopez, Marie-Josee Boily,[...]. BMC Syst Biol 2010
28
Trinucleotide expansion in haploid germ cells by gap repair.
I V Kovtun, C T McMurray. Nat Genet 2001
I V Kovtun, C T McMurray. Nat Genet 2001
20
FAN1 modifies Huntington's disease progression by stabilizing the expanded HTT CAG repeat.
Robert Goold, Michael Flower, Davina Hensman Moss, Chris Medway, Alison Wood-Kaczmar, Ralph Andre, Pamela Farshim, Gill P Bates, Peter Holmans, Lesley Jones,[...]. Hum Mol Genet 2019
Robert Goold, Michael Flower, Davina Hensman Moss, Chris Medway, Alison Wood-Kaczmar, Ralph Andre, Pamela Farshim, Gill P Bates, Peter Holmans, Lesley Jones,[...]. Hum Mol Genet 2019
39
Stoichiometry of base excision repair proteins correlates with increased somatic CAG instability in striatum over cerebellum in Huntington's disease transgenic mice.
Agathi-Vassiliki Goula, Brian R Berquist, David M Wilson, Vanessa C Wheeler, Yvon Trottier, Karine Merienne. PLoS Genet 2009
Agathi-Vassiliki Goula, Brian R Berquist, David M Wilson, Vanessa C Wheeler, Yvon Trottier, Karine Merienne. PLoS Genet 2009
18
CTG repeat instability and size variation timing in DNA repair-deficient mice.
Cédric Savouret, Edith Brisson, Jeroen Essers, Roland Kanaar, Albert Pastink, Hein te Riele, Claudine Junien, Geneviève Gourdon. EMBO J 2003
Cédric Savouret, Edith Brisson, Jeroen Essers, Roland Kanaar, Albert Pastink, Hein te Riele, Claudine Junien, Geneviève Gourdon. EMBO J 2003
18
(CAG)(n)-hairpin DNA binds to Msh2-Msh3 and changes properties of mismatch recognition.
Barbara A L Owen, Zungyoon Yang, Maoyi Lai, Maciej Gajec, John D Badger, Jeffrey J Hayes, Winfried Edelmann, Raju Kucherlapati, Teresa M Wilson, Cynthia T McMurray. Nat Struct Mol Biol 2005
Barbara A L Owen, Zungyoon Yang, Maoyi Lai, Maciej Gajec, John D Badger, Jeffrey J Hayes, Winfried Edelmann, Raju Kucherlapati, Teresa M Wilson, Cynthia T McMurray. Nat Struct Mol Biol 2005
18
Msh2 acts in medium-spiny striatal neurons as an enhancer of CAG instability and mutant huntingtin phenotypes in Huntington's disease knock-in mice.
Marina Kovalenko, Ella Dragileva, Jason St Claire, Tammy Gillis, Jolene R Guide, Jaclyn New, Hualing Dong, Raju Kucherlapati, Melanie H Kucherlapati, Michelle E Ehrlich,[...]. PLoS One 2012
Marina Kovalenko, Ella Dragileva, Jason St Claire, Tammy Gillis, Jolene R Guide, Jaclyn New, Hualing Dong, Raju Kucherlapati, Melanie H Kucherlapati, Michelle E Ehrlich,[...]. PLoS One 2012
40
Suppression of Somatic Expansion Delays the Onset of Pathophysiology in a Mouse Model of Huntington's Disease.
Helen Budworth, Faye R Harris, Paul Williams, Do Yup Lee, Amy Holt, Jens Pahnke, Bartosz Szczesny, Karina Acevedo-Torres, Sylvette Ayala-Peña, Cynthia T McMurray. PLoS Genet 2015
Helen Budworth, Faye R Harris, Paul Williams, Do Yup Lee, Amy Holt, Jens Pahnke, Bartosz Szczesny, Karina Acevedo-Torres, Sylvette Ayala-Peña, Cynthia T McMurray. PLoS Genet 2015
28
A genetic association study of glutamine-encoding DNA sequence structures, somatic CAG expansion, and DNA repair gene variants, with Huntington disease clinical outcomes.
Marc Ciosi, Alastair Maxwell, Sarah A Cumming, Davina J Hensman Moss, Asma M Alshammari, Michael D Flower, Alexandra Durr, Blair R Leavitt, Raymund A C Roos, Peter Holmans,[...]. EBioMedicine 2019
Marc Ciosi, Alastair Maxwell, Sarah A Cumming, Davina J Hensman Moss, Asma M Alshammari, Michael D Flower, Alexandra Durr, Blair R Leavitt, Raymund A C Roos, Peter Holmans,[...]. EBioMedicine 2019
31
MSH3 modifies somatic instability and disease severity in Huntington's and myotonic dystrophy type 1.
Michael Flower, Vilija Lomeikaite, Marc Ciosi, Sarah Cumming, Fernando Morales, Kitty Lo, Davina Hensman Moss, Lesley Jones, Peter Holmans, Darren G Monckton,[...]. Brain 2019
Michael Flower, Vilija Lomeikaite, Marc Ciosi, Sarah Cumming, Fernando Morales, Kitty Lo, Davina Hensman Moss, Lesley Jones, Peter Holmans, Darren G Monckton,[...]. Brain 2019
32
Msh3 is a limiting factor in the formation of intergenerational CTG expansions in DM1 transgenic mice.
Laurent Foiry, Li Dong, Cédric Savouret, Laurence Hubert, Hein te Riele, Claudine Junien, Geneviève Gourdon. Hum Genet 2006
Laurent Foiry, Li Dong, Cédric Savouret, Laurence Hubert, Hein te Riele, Claudine Junien, Geneviève Gourdon. Hum Genet 2006
17
Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset.
Nancy S Wexler, Judith Lorimer, Julie Porter, Fidela Gomez, Carol Moskowitz, Edith Shackell, Karen Marder, Graciela Penchaszadeh, Simone A Roberts, Javier Gayán,[...]. Proc Natl Acad Sci U S A 2004
Nancy S Wexler, Judith Lorimer, Julie Porter, Fidela Gomez, Carol Moskowitz, Edith Shackell, Karen Marder, Graciela Penchaszadeh, Simone A Roberts, Javier Gayán,[...]. Proc Natl Acad Sci U S A 2004
17
Inherited CAG.CTG allele length is a major modifier of somatic mutation length variability in Huntington disease.
Nicola J Veitch, Margaret Ennis, John P McAbney, Peggy F Shelbourne, Darren G Monckton. DNA Repair (Amst) 2007
Nicola J Veitch, Margaret Ennis, John P McAbney, Peggy F Shelbourne, Darren G Monckton. DNA Repair (Amst) 2007
47
Huntington disease.
Gillian P Bates, Ray Dorsey, James F Gusella, Michael R Hayden, Chris Kay, Blair R Leavitt, Martha Nance, Christopher A Ross, Rachael I Scahill, Ronald Wetzel,[...]. Nat Rev Dis Primers 2015
Gillian P Bates, Ray Dorsey, James F Gusella, Michael R Hayden, Chris Kay, Blair R Leavitt, Martha Nance, Christopher A Ross, Rachael I Scahill, Ronald Wetzel,[...]. Nat Rev Dis Primers 2015
17
Xpa deficiency reduces CAG trinucleotide repeat instability in neuronal tissues in a mouse model of SCA1.
Leroy Hubert, Yunfu Lin, Vincent Dion, John H Wilson. Hum Mol Genet 2011
Leroy Hubert, Yunfu Lin, Vincent Dion, John H Wilson. Hum Mol Genet 2011
28
The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington's disease.
S E Andrew, Y P Goldberg, B Kremer, H Telenius, J Theilmann, S Adam, E Starr, F Squitieri, B Lin, M A Kalchman. Nat Genet 1993
S E Andrew, Y P Goldberg, B Kremer, H Telenius, J Theilmann, S Adam, E Starr, F Squitieri, B Lin, M A Kalchman. Nat Genet 1993
15
Repeat instability: mechanisms of dynamic mutations.
Christopher E Pearson, Kerrie Nichol Edamura, John D Cleary. Nat Rev Genet 2005
Christopher E Pearson, Kerrie Nichol Edamura, John D Cleary. Nat Rev Genet 2005
15
Mechanisms of trinucleotide repeat instability during human development.
Cynthia T McMurray. Nat Rev Genet 2010
Cynthia T McMurray. Nat Rev Genet 2010
15
Neuropathological classification of Huntington's disease.
J P Vonsattel, R H Myers, T J Stevens, R J Ferrante, E D Bird, E P Richardson. J Neuropathol Exp Neurol 1985
J P Vonsattel, R H Myers, T J Stevens, R J Ferrante, E D Bird, E P Richardson. J Neuropathol Exp Neurol 1985
15
Disease-associated repeat instability and mismatch repair.
Monika H M Schmidt, Christopher E Pearson. DNA Repair (Amst) 2016
Monika H M Schmidt, Christopher E Pearson. DNA Repair (Amst) 2016
15
Length of Uninterrupted CAG, Independent of Polyglutamine Size, Results in Increased Somatic Instability, Hastening Onset of Huntington Disease.
Galen E B Wright, Jennifer A Collins, Chris Kay, Cassandra McDonald, Egor Dolzhenko, Qingwen Xia, Kristina Bečanović, Britt I Drögemöller, Alicia Semaka, Charlotte M Nguyen,[...]. Am J Hum Genet 2019
Galen E B Wright, Jennifer A Collins, Chris Kay, Cassandra McDonald, Egor Dolzhenko, Qingwen Xia, Kristina Bečanović, Britt I Drögemöller, Alicia Semaka, Charlotte M Nguyen,[...]. Am J Hum Genet 2019
27
Transcription promotes contraction of CAG repeat tracts in human cells.
Yunfu Lin, Vincent Dion, John H Wilson. Nat Struct Mol Biol 2006
Yunfu Lin, Vincent Dion, John H Wilson. Nat Struct Mol Biol 2006
14
Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain.
M DiFiglia, E Sapp, K O Chase, S W Davies, G P Bates, J P Vonsattel, N Aronin. Science 1997
M DiFiglia, E Sapp, K O Chase, S W Davies, G P Bates, J P Vonsattel, N Aronin. Science 1997
14
MSH2 ATPase domain mutation affects CTG*CAG repeat instability in transgenic mice.
Stéphanie Tomé, Ian Holt, Winfried Edelmann, Glenn E Morris, Arnold Munnich, Christopher E Pearson, Geneviève Gourdon. PLoS Genet 2009
Stéphanie Tomé, Ian Holt, Winfried Edelmann, Glenn E Morris, Arnold Munnich, Christopher E Pearson, Geneviève Gourdon. PLoS Genet 2009
19
Isolated short CTG/CAG DNA slip-outs are repaired efficiently by hMutSbeta, but clustered slip-outs are poorly repaired.
Gagan B Panigrahi, Meghan M Slean, Jodie P Simard, Opher Gileadi, Christopher E Pearson. Proc Natl Acad Sci U S A 2010
Gagan B Panigrahi, Meghan M Slean, Jodie P Simard, Opher Gileadi, Christopher E Pearson. Proc Natl Acad Sci U S A 2010
19
Co-cited is the co-citation frequency, indicating how many articles cite the article together with the query article. Similarity is the co-citation as percentage of the times cited of the query article or the article in the search results, whichever is the lowest. These numbers are calculated for the last 100 citations when articles are cited more than 100 times.