Tania D Gendron, PhD

  • 6212 Citations
  • 41 Scopus h-Index
20002020

Research output per year

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Research Output

  • 6212 Citations
  • 41 Scopus h-Index
  • 79 Article
  • 4 Review article
  • 3 Comment/debate
  • 2 Chapter
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Article
2020

Chimeric Peptide Species Contribute to Divergent Dipeptide Repeat Pathology in c9ALS/FTD and SCA36

Neuro–CEB Neuropathology Network, 2020, (Accepted/In press) In : Neuron.

Research output: Contribution to journalArticle

1 Scopus citations
Open Access
1 Scopus citations

Dipeptide repeat proteins inhibit homology-directed DNA double strand break repair in C9ORF72 ALS/FTD

Andrade, N. S., Ramic, M., Esanov, R., Liu, W., Rybin, M. J., Gaidosh, G., Abdallah, A., Del'olio, S., Huff, T. C., Chee, N. T., Anatha, S., Gendron, T. F., Wahlestedt, C., Zhang, Y., Benatar, M., Mueller, C. & Zeier, Z., Feb 24 2020, In : Molecular neurodegeneration. 15, 1, 13.

Research output: Contribution to journalArticle

Open Access

Hexanucleotide Repeat Expansions in c9FTD/ALS and SCA36 Confer Selective Patterns of Neurodegeneration In Vivo

Todd, T. W., McEachin, Z. T., Chew, J., Burch, A. R., Jansen-West, K., Tong, J., Yue, M., Song, Y., Castanedes-Casey, M., Kurti, A., Dunmore, J. H., Fryer, J. D., Zhang, Y. J., San Millan, B., Teijeira Bautista, S., Arias, M., Dickson, D., Gendron, T. F., Sobrido, M. J., Disney, M. D. & 3 others, Bassell, G. J., Rossoll, W. & Petrucelli, L., May 5 2020, In : Cell reports. 31, 5, 107616.

Research output: Contribution to journalArticle

Open Access
1 Scopus citations

Nucleocytoplasmic Proteomic Analysis Uncovers eRF1 and Nonsense-Mediated Decay as Modifiers of ALS/FTD C9orf72 Toxicity

Ortega, J. A., Daley, E. L., Kour, S., Samani, M., Tellez, L., Smith, H. S., Hall, E. A., Esengul, Y. T., Tsai, Y. H., Gendron, T. F., Donnelly, C. J., Siddique, T., Savas, J. N., Pandey, U. B. & Kiskinis, E., Apr 8 2020, In : Neuron. 106, 1, p. 90-107.e13

Research output: Contribution to journalArticle

3 Scopus citations

Reduced C9ORF72 function exacerbates gain of toxicity from ALS/FTD-causing repeat expansion in C9orf72

Zhu, Q., Jiang, J., Gendron, T. F., McAlonis-Downes, M., Jiang, L., Taylor, A., Diaz Garcia, S., Ghosh Dastidar, S., Rodriguez, M. J., King, P., Zhang, Y., La Spada, A. R., Xu, H., Petrucelli, L., Ravits, J., Da Cruz, S., Lagier-Tourenne, C. & Cleveland, D. W., May 1 2020, In : Nature Neuroscience. 23, 5, p. 615-624 10 p.

Research output: Contribution to journalArticle

5 Scopus citations
2019

Aberrant deposition of stress granule-resident proteins linked to C9orf72-associated TDP-43 proteinopathy

Chew, J., Cook, C., Gendron, T. F., Jansen-West, K., Del Rosso, G., Daughrity, L. M., Castanedes-Casey, M., Kurti, A., Stankowski, J. N., Disney, M. D., Rothstein, J. D., Dickson, D. W., Fryer, J. D., Zhang, Y. J. & Petrucelli, L., Feb 15 2019, In : Molecular neurodegeneration. 14, 1, 9.

Research output: Contribution to journalArticle

14 Scopus citations

Extensive transcriptomic study emphasizes importance of vesicular transport in C9orf72 expansion carriers

Dickson, D. W., Baker, M. C., Jackson, J. L., Dejesus-Hernandez, M., Finch, N. C. A., Tian, S., Heckman, M. G., Pottier, C., Gendron, T. F., Murray, M. E., Ren, Y., Reddy, J. S., Graff-Radford, N. R., Boeve, B. F., Petersen, R. C., Knopman, D. S., Josephs, K. A., Petrucelli, L., Oskarsson, B., Sheppard, J. W. & 3 others, Asmann, Y. W., Rademakers, R. & Van Blitterswijk, M., Oct 8 2019, In : Acta Neuropathologica Communications. 7, 1, 150.

Research output: Contribution to journalArticle

Open Access
1 Scopus citations

Heterochromatin anomalies and double-stranded RNA accumulation underlie C9orf72 poly(PR) toxicity

Zhang, Y. J., Guo, L., Gonzales, P. K., Gendron, T. F., Wu, Y., Jansen-West, K., O’Raw, A. D., Pickles, S. R., Prudencio, M., Carlomagno, Y., Gachechiladze, M. A., Ludwig, C., Tian, R., Chew, J., DeTure, M., Lin, W. L., Tong, J., Daughrity, L. M., Yue, M., Song, Y. & 15 others, Andersen, J. W., Castanedes-Casey, M., Kurti, A., Datta, A., Antognetti, G., McCampbell, A., Rademakers, R., Oskarsson, B., Dickson, D. W., Kampmann, M., Ward, M. E., Fryer, J. D., Link, C. D., Shorter, J. & Petrucelli, L., Feb 15 2019, In : Science. 363, 6428, eaav2606.

Research output: Contribution to journalArticle

31 Scopus citations

RPS25 is required for efficient RAN translation of C9orf72 and other neurodegenerative disease-associated nucleotide repeats

Yamada, S. B., Gendron, T. F., Niccoli, T., Genuth, N. R., Grosely, R., Shi, Y., Glaria, I., Kramer, N. J., Nakayama, L., Fang, S., Dinger, T. J. I., Thoeng, A., Rocha, G., Barna, M., Puglisi, J. D., Partridge, L., Ichida, J. K., Isaacs, A. M., Petrucelli, L. & Gitler, A. D., Sep 1 2019, In : Nature Neuroscience. 22, 9, p. 1383-1388 6 p.

Research output: Contribution to journalArticle

13 Scopus citations

The Hairpin Form of r(G 4 C 2 ) exp in c9ALS/FTD Is Repeat-Associated Non-ATG Translated and a Target for Bioactive Small Molecules

Wang, Z. F., Ursu, A., Childs-Disney, J. L., Guertler, R., Yang, W. Y., Bernat, V., Rzuczek, S. G., Fuerst, R., Zhang, Y., Gendron, T. D., Yildirim, I., Dwyer, B. G., Rice, J. E., Petrucelli, L. & Disney, M. D., Feb 21 2019, In : Cell Chemical Biology. 26, 2, p. 179-190.e12

Research output: Contribution to journalArticle

19 Scopus citations

Transcription elongation factor AFF2/FMR2 regulates expression of expanded GGGGCC repeat-containing C9ORF72 allele in ALS/FTD

Yuva-Aydemir, Y., Almeida, S., Krishnan, G., Gendron, T. F. & Gao, F. B., Dec 1 2019, In : Nature communications. 10, 1, 5466.

Research output: Contribution to journalArticle

Open Access
5 Scopus citations
2018

Amyotrophic Lateral Sclerosis: An Update for 2018

Oskarsson, B., Gendron, T. D. & Staff, N. P., Jan 1 2018, (Accepted/In press) In : Mayo Clinic Proceedings.

Research output: Contribution to journalArticle

45 Scopus citations

A zebrafish model for C9orf72 ALS reveals RNA toxicity as a pathogenic mechanism

Swinnen, B., Bento-Abreu, A., Gendron, T. F., Boeynaems, S., Bogaert, E., Nuyts, R., Timmers, M., Scheveneels, W., Hersmus, N., Wang, J., Mizielinska, S., Isaacs, A. M., Petrucelli, L., Lemmens, R., Van Damme, P., Van Den Bosch, L. & Robberecht, W., Mar 1 2018, In : Acta neuropathologica. 135, 3, p. 427-443 17 p.

Research output: Contribution to journalArticle

25 Scopus citations

CUG initiation and frameshifting enable production of dipeptide repeat proteins from ALS/FTD C9ORF72 transcripts

Tabet, R., Schaeffer, L., Freyermuth, F., Jambeau, M., Workman, M., Lee, C. Z., Lin, C. C., Jiang, J., Jansen-West, K., Abou-Hamdan, H., Désaubry, L., Gendron, T. D., Petrucelli, L., Martin, F. & Lagier-Tourenne, C., Dec 1 2018, In : Nature Communications. 9, 1, 152.

Research output: Contribution to journalArticle

44 Scopus citations

Disease mechanisms of c9orf72 repeat expansions

Gendron, T. F. & Petrucelli, L., Apr 2018, In : Cold Spring Harbor Perspectives in Medicine. 8, 4, a024224.

Research output: Contribution to journalArticle

23 Scopus citations

Long-read sequencing across the C9orf72 'GGGGCC' repeat expansion: Implications for clinical use and genetic discovery efforts in human disease

Ebbert, M. T. W., Farrugia, S. L., Sens, J. P., Jansen-West, K., Gendron, T. F., Prudencio, M., McLaughlin, I. J., Bowman, B., Seetin, M., Dejesus-Hernandez, M., Jackson, J., Brown, P. H., Dickson, D. W., Van Blitterswijk, M., Rademakers, R., Petrucelli, L. & Fryer, J. D., Aug 21 2018, In : Molecular neurodegeneration. 13, 1, 46.

Research output: Contribution to journalArticle

21 Scopus citations

Loss of Tmem106b is unable to ameliorate frontotemporal dementia-like phenotypes in an AAV mouse model of C9ORF72-repeat induced toxicity

Nicholson, A. M., Zhou, X., Perkerson, R. B., Parsons, T. M., Chew, J., Brooks, M., DeJesus-Hernandez, M., Finch, N. C. A., Matchett, B. J., Kurti, A., Jansen-West, K. R., Perkerson, E., Daughrity, L., Castanedes-Casey, M., Rousseau, L., Phillips, V., Hu, F., Gendron, T. F., Murray, M. E., Dickson, D. W. & 3 others, Fryer, J. D., Petrucelli, L. & Rademakers, R., May 31 2018, In : Acta Neuropathologica Communications. 6, 1, 1 p.

Research output: Contribution to journalArticle

Open Access
3 Scopus citations

Poly(GP), neurofilament and grey matter deficits in C9orf72 expansion carriers

Meeter, L. H. H., Gendron, T. F., Sias, A. C., Jiskoot, L. C., Russo, S. P., Donker Kaat, L., Papma, J. M., Panman, J. L., van der Ende, E. L., Dopper, E. G., Franzen, S., Graff, C., Boxer, A. L., Rosen, H. J., Sanchez-Valle, R., Galimberti, D., Pijnenburg, Y. A. L., Benussi, L., Ghidoni, R., Borroni, B. & 21 others, Laforce, R., del Campo, M., Teunissen, C. E., van Minkelen, R., Rojas, J. C., Coppola, G., Geschwind, D. H., Rademakers, R., Karydas, A. M., Öijerstedt, L., Scarpini, E., Binetti, G., Padovani, A., Cash, D. M., Dick, K. M., Bocchetta, M., Miller, B. L., Rohrer, J. D., Petrucelli, L., van Swieten, J. C. & Lee, S. E., May 2018, In : Annals of Clinical and Translational Neurology. 5, 5, p. 583-597 15 p.

Research output: Contribution to journalArticle

15 Scopus citations

Poly(GR) impairs protein translation and stress granule dynamics in C9orf72-associated frontotemporal dementia and amyotrophic lateral sclerosis

Zhang, Y. J., Gendron, T. F., Ebbert, M. T. W., O’Raw, A. D., Yue, M., Jansen-West, K., Zhang, X., Prudencio, M., Chew, J., Cook, C. N., Daughrity, L. M., Tong, J., Song, Y., Pickles, S. R., Castanedes-Casey, M., Kurti, A., Rademakers, R., Oskarsson, B., Dickson, D. W., Hu, W. & 3 others, Gitler, A. D., Fryer, J. D. & Petrucelli, L., Aug 1 2018, In : Nature Medicine. 24, 8, p. 1136-1142 7 p.

Research output: Contribution to journalArticle

59 Scopus citations
Open Access
23 Scopus citations
2017

Abnormal expression of homeobox genes and transthyretin in C9ORF72 expansion carriers

Finch, N. A., Wang, X., Baker, M. C., Heckman, M. G., Gendron, T. F., Bieniek, K. F., Wuu, J., Dejesus-Hernandez, M., Brown, P. H., Chew, J., Jansen-West, K. R., Daughrity, L. M., Nicholson, A. M., Murray, M. E., Josephs, K. A., Parisi, J. E., Knopman, D. S., Petersen, R. C., Petrucelli, L., Boeve, B. F. & 8 others, Graff-Radford, N. R., Asmann, Y. W., Dickson, D. W., Benatar, M., Bowser, R., Boylan, K. B., Rademakers, R. & Van Blitterswijk, M., Jan 1 2017, In : Neurology: Genetics. 3, 4

Research output: Contribution to journalArticle

6 Scopus citations

A C9ORF72 BAC mouse model recapitulates key epigenetic perturbations of ALS/FTD

Esanov, R., Cabrera, G. T., Andrade, N. S., Gendron, T. F., Brown, R. H., Benatar, M., Wahlestedt, C., Mueller, C. & Zeier, Z., Jun 12 2017, In : Molecular neurodegeneration. 12, 1, 46.

Research output: Contribution to journalArticle

8 Scopus citations

Clinical/Scientific Notes: ARHGEF28 P.LYS280METFS40TER in an amyotrophic lateral sclerosis family with a C9ORF72 expansion

Farhan, S. M. K., Gendron, T. D., Petrucelli, L., Hegele, R. A. & Strong, M. J., Oct 1 2017, In : Neurology: Genetics. 3, 5, e190.

Research output: Contribution to journalArticle

2 Scopus citations

In-depth clinico-pathological examination of RNA foci in a large cohort of C9ORF72 expansion carriers

DeJesus-Hernandez, M., Finch, N. C. A., Wang, X., Gendron, T. F., Bieniek, K. F., Heckman, M. G., Vasilevich, A., Murray, M. E., Rousseau, L., Weesner, R., Lucido, A., Parsons, M., Chew, J., Josephs, K. A., Parisi, J. E., Knopman, D. S., Petersen, R. C., Boeve, B. F., Graff-Radford, N. R., de Boer, J. & 6 others, Asmann, Y. W., Petrucelli, L., Boylan, K. B., Dickson, D. W., van Blitterswijk, M. & Rademakers, R., Aug 1 2017, In : Acta neuropathologica. 134, 2, p. 255-269 15 p.

Research output: Contribution to journalArticle

27 Scopus citations
4 Scopus citations

Phosphorylated neurofilament heavy chain: A biomarker of survival for C9ORF72-associated amyotrophic lateral sclerosis

C9ORF72 Neurofilament Study Group, Jul 2017, In : Annals of neurology. 82, 1, p. 139-146 8 p.

Research output: Contribution to journalArticle

32 Scopus citations

Poly(GP) proteins are a useful pharmacodynamic marker for C9ORF72-associated amyotrophic lateral sclerosis

Gendron, T. F., Chew, J., Stankowski, J. N., Hayes, L. R., Zhang, Y. J., Prudencio, M., Carlomagno, Y., Daughrity, L. M., Jansen-West, K., Perkerson, E. A., O'Raw, A., Cook, C., Pregent, L., Belzil, V., Van Blitterswijk, M., Tabassian, L. J., Lee, C. W., Yue, M., Tong, J., Song, Y. & 59 others, Castanedes-Casey, M., Rousseau, L., Phillips, V., Dickson, D. W., Rademakers, R., Fryer, J. D., Rush, B. K., Pedraza, O., Caputo, A. M., Desaro, P., Palmucci, C., Robertson, A., Heckman, M. G., Diehl, N. N., Wiggs, E., Tierney, M., Braun, L., Farren, J., Lacomis, D., Ladha, S., Fournier, C. N., McCluskey, L. F., Elman, L. B., Toledo, J. B., McBride, J. D., Tiloca, C., Morelli, C., Poletti, B., Solca, F., Prelle, A., Wuu, J., Jockel-Balsarotti, J., Rigo, F., Ambrose, C., Datta, A., Yang, W., Raitcheva, D., Antognetti, G., McCampbell, A., Van Swieten, J. C., Miller, B. L., Boxer, A. L., Brown, R. H., Bowser, R., Miller, T. M., Trojanowski, J. Q., Grossman, M., Berry, J. D., Hu, W. T., Ratti, A., Traynor, B. J., Disney, M. D., Benatar, M., Silani, V., Glass, J. D., Floeter, M. K., Rothstein, J. D., Boylan, K. B. & Petrucelli, L., Mar 29 2017, In : Science translational medicine. 9, 383, eaai7866.

Research output: Contribution to journalArticle

58 Scopus citations

Repetitive element transcripts are elevated in the brain of C9orf72 ALS/FTLD patients

Prudencio, M., Gonzales, P. K., Cook, C., Gendron, T. D., Daughrity, L. M., Song, Y., Ebbert, M. T. W., Van Blitterswijk, M., Zhang, Y., Jansen-West, K., Baker, M. C., Deture, M., Rademakers, R. V., Boylan, K. B., Dickson, D. W., Petrucelli, L. & Link, C. D., Sep 1 2017, In : Human Molecular Genetics. 26, 17, p. 3421-3431 11 p.

Research output: Contribution to journalArticle

28 Scopus citations

Spinal poly-GA inclusions in a C9orf72 mouse model trigger motor deficits and inflammation without neuron loss

Schludi, M. H., Becker, L., Garrett, L., Gendron, T. F., Zhou, Q., Schreiber, F., Popper, B., Dimou, L., Strom, T. M., Winkelmann, J., von Thaden, A., Rentzsch, K., May, S., Michaelsen, M., Schwenk, B. M., Tan, J., Schoser, B., Dieterich, M., Petrucelli, L., Hölter, S. M. & 7 others, Wurst, W., Fuchs, H., Gailus-Durner, V., de Angelis, M. H., Klopstock, T., Arzberger, T. & Edbauer, D., Aug 1 2017, In : Acta neuropathologica. 134, 2, p. 241-254 14 p.

Research output: Contribution to journalArticle

36 Scopus citations
2016

C9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteins

Zhang, Y., Gendron, T. D., Grima, J. C., Sasaguri, H., Jansen-West, K., Xu, Y. F., Katzman, R. B., Gass, J., Murray, M. E., Shinohara, M., Lin, W. L., Garrett, A., Stankowski, J. N., Daughrity, L., Tong, J., Perkerson, E. A., Yue, M., Chew, J., Castanedes-Casey, M., Kurti, A. & 16 others, Wang, Z. S., Liesinger, A. M., Baker, J. D., Jiang, J., Lagier-Tourenne, C., Edbauer, D., Cleveland, D. W., Rademakers, R. V., Boylan, K. B., Bu, G. D., Link, C. D., Dickey, C. A., Rothstein, J. D., Dickson, D. W., Fryer, J. D. & Petrucelli, L., May 1 2016, In : Nature Neuroscience. 19, 5, p. 668-677 10 p.

Research output: Contribution to journalArticle

124 Scopus citations

Gain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides Targeting GGGGCC-Containing RNAs

Jiang, J., Zhu, Q., Gendron, T. D., Saberi, S., McAlonis-Downes, M., Seelman, A., Stauffer, J. E., Jafar-nejad, P., Drenner, K., Schulte, D., Chun, S., Sun, S., Ling, S. C., Myers, B., Engelhardt, J., Katz, M., Baughn, M., Platoshyn, O., Marsala, M., Watt, A. & 21 others, Heyser, C. J., Ard, M. C., De Muynck, L., Daughrity, L. M., Swing, D. A., Tessarollo, L., Jung, C. J., Delpoux, A., Utzschneider, D. T., Hedrick, S. M., de Jong, P. J., Edbauer, D., Van Damme, P., Petrucelli, L., Shaw, C. E., Bennett, C. F., Da Cruz, S., Ravits, J., Rigo, F., Cleveland, D. W. & Lagier-Tourenne, C., May 4 2016, In : Neuron. 90, 3, p. 535-550 16 p.

Research output: Contribution to journalArticle

184 Scopus citations

Poly(GR) in C9ORF72-Related ALS/FTD Compromises Mitochondrial Function and Increases Oxidative Stress and DNA Damage in iPSC-Derived Motor Neurons

Lopez-Gonzalez, R., Lu, Y., Gendron, T. F., Karydas, A., Tran, H., Yang, D., Petrucelli, L., Miller, B. L., Almeida, S. & Gao, F. B., Oct 19 2016, In : Neuron. 92, 2, p. 383-391 9 p.

Research output: Contribution to journalArticle

111 Scopus citations

Spt4 selectively regulates the expression of C9orf72 sense and antisense mutant transcripts

Kramer, N. J., Carlomagno, Y., Zhang, Y., Almeida, S., Cook, C., Gendron, T. D., Prudencio, M., Van Blitterswijk, M., Belzil, V., Couthouis, J., Paul, J. W., Goodman, L. D., Daughrity, L., Chew, J., Garrett, A., Pregent, L., Jansen-West, K., Tabassian, L. J., Rademakers, R. V., Boylan, K. & 16 others, Graff Radford, N. R., Josephs, K. A., Parisi, J. E., Knopman, D. S., Petersen, R. C., Boeve, B. F., Deng, N., Feng, Y., Cheng, T. H., Dickson, D. W., Cohen, S. N., Bonini, N. M., Link, C. D., Gao, F. B., Petrucelli, L. & Gitler, A. D., Aug 12 2016, In : Science. 353, 6300, p. 708-712 5 p.

Research output: Contribution to journalArticle

53 Scopus citations

The extreme N-terminus of TDP-43 mediates the cytoplasmic aggregation of TDP-43 and associated toxicity in vivo

Sasaguri, H., Chew, J., Xu, Y. F., Gendron, T. D., Garrett, A., Lee, C. W., Jansen-West, K., Bauer, P. O., Perkerson, E. A., Tong, J., Stetler, C. & Zhang, Y., Feb 25 2016, (Accepted/In press) In : Brain Research.

Research output: Contribution to journalArticle

18 Scopus citations

Timing and significance of pathological features in C9orf72 expansion-associated frontotemporal dementia

Vatsavayai, S. C., Yoon, S. J., Gardner, R. C., Gendron, T. F., Vargas, J. N. S., Trujillo, A., Pribadi, M., Phillips, J. J., Gaus, S. E., Hixson, J. D., Garcia, P. A., Rabinovici, G. D., Coppola, G., Geschwind, D. H., Petrucelli, L., Miller, B. L. & Seeley, W. W., 2016, In : Brain. 139, 12, p. 3202-3216 15 p.

Research output: Contribution to journalArticle

48 Scopus citations
2015

C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD

O'Rourke, J. G., Bogdanik, L., Muhammad, A. K. M. G., Gendron, T. D., Kim, K. J., Austin, A., Cady, J., Liu, E. Y., Zarrow, J., Grant, S., Ho, R., Bell, S., Carmona, S., Simpkinson, M., Lall, D., Wu, K., Daughrity, L., Dickson, D. W., Harms, M. B., Petrucelli, L. & 3 others, Lee, E. B., Lutz, C. M. & Baloh, R. H., Dec 2 2015, In : Neuron. 88, 5, p. 892-901 10 p.

Research output: Contribution to journalArticle

124 Scopus citations

C9ORF72 repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits

Chew, J., Gendron, T. D., Prudencio, M., Sasaguri, H., Zhang, Y., Castanedes-Casey, M., Lee, C. W., Jansen-West, K., Kurti, A., Murray, M. E., Bieniek, K. F., Bauer, P. O., Whitelaw, E. C., Rousseau, L., Stankowski, J. N., Stetler, C., Daughrity, L. M., Perkerson, E. A., Desaro, P., Johnston, A. & 7 others, Overstreet, K., Edbauer, D., Rademakers, R. V., Boylan, K. B., Dickson, D. W., Fryer, J. D. & Petrucelli, L., Jun 5 2015, In : Science. 348, 6239, p. 1151-1154 4 p.

Research output: Contribution to journalArticle

185 Scopus citations

Cerebellar c9RAN proteins associate with clinical and neuropathological characteristics of C9ORF72 repeat expansion carriers

Gendron, T. F., van Blitterswijk, M., Bieniek, K. F., Daughrity, L. M., Jiang, J., Rush, B. K., Pedraza, O., Lucas, J. A., Murray, M. E., Desaro, P., Robertson, A., Overstreet, K., Thomas, C. S., Crook, J. E., Castanedes-Casey, M., Rousseau, L., Josephs, K. A., Parisi, J. E., Knopman, D. S., Petersen, R. C. & 9 others, Boeve, B. F., Graff-Radford, N. R., Rademakers, R., Lagier-Tourenne, C., Edbauer, D., Cleveland, D. W., Dickson, D. W., Petrucelli, L. & Boylan, K. B., Oct 26 2015, In : Acta neuropathologica. 130, 4, p. 559-573 15 p.

Research output: Contribution to journalArticle

52 Scopus citations

Differential Toxicity of Nuclear RNA Foci versus Dipeptide Repeat Proteins in a Drosophila Model of C9ORF72 FTD/ALS

Tran, H., Almeida, S., Moore, J., Gendron, T. D., Chalasani, U., Lu, Y., Du, X., Nickerson, J. A., Petrucelli, L., Weng, Z. & Gao, F. B., Sep 23 2015, In : Neuron. 87, 6, p. 1207-1214 8 p.

Research output: Contribution to journalArticle

102 Scopus citations

Distinct brain transcriptome profiles in C9orf72-associated and sporadic ALS

Prudencio, M., Belzil, V., Batra, R., Ross, C. A., Gendron, T. D., Pregent, L. J., Murray, M. E., Overstreet, K. K., Piazza-Johnston, A. E., Desaro, P., Bieniek, K. F., Deture, M., Lee, W. C., Biendarra, S. M., Davis, M. D., Baker, M. C., Perkerson, R. B., Van Blitterswijk, M., Stetler, C. T., Rademakers, R. V. & 5 others, Link, C. D., Dickson, D. W., Boylan, K. B., Li, H. & Petrucelli, L., Aug 30 2015, In : Nature Neuroscience. 18, 8, p. 1175-1182 8 p.

Research output: Contribution to journalArticle

153 Scopus citations

Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice

Peters, O. M., Cabrera, G. T., Tran, H., Gendron, T. D., McKeon, J. E., Metterville, J., Weiss, A., Wightman, N., Salameh, J., Kim, J., Sun, H., Boylan, K. B., Dickson, D. W., Kennedy, Z., Lin, Z., Zhang, Y., Daughrity, L., Jung, C., Gao, F. B., Sapp, P. C. & 7 others, Horvitz, H. R., Bosco, D. A., Brown, S. P., de Jong, P., Petrucelli, L., Mueller, C. & Brown, R. H., Dec 2 2015, In : Neuron. 88, 5, p. 902-909 8 p.

Research output: Contribution to journalArticle

118 Scopus citations

Novel clinical associations with specific C9ORF72 transcripts in patients with repeat expansions in C9ORF72

van Blitterswijk, M., Gendron, T. F., Baker, M. C., DeJesus-Hernandez, M., Finch, N. C. A., Brown, P. H., Daughrity, L. M., Murray, M. E., Heckman, M. G., Jiang, J., Lagier-Tourenne, C., Edbauer, D., Cleveland, D. W., Josephs, K. A., Parisi, J. E., Knopman, D. S., Petersen, R. C., Petrucelli, L., Boeve, B. F., Graff-Radford, N. R. & 3 others, Boylan, K. B., Dickson, D. W. & Rademakers, R., Dec 1 2015, In : Acta neuropathologica. 130, 6, p. 863-876 14 p.

Research output: Contribution to journalArticle

47 Scopus citations

Quantitative analysis and clinico-pathological correlations of different dipeptide repeat protein pathologies in C9ORF72 mutation carriers

Mackenzie, I. R. A., Frick, P., Grässer, F. A., Gendron, T. F., Petrucelli, L., Cashman, N. R., Edbauer, D., Kremmer, E., Prudlo, J., Troost, D. & Neumann, M., Dec 1 2015, In : Acta neuropathologica. 130, 6, p. 845-861 17 p.

Research output: Contribution to journalArticle

99 Scopus citations
2014

Acetylation of the KXGS motifs in tau is a critical determinant in modulation of tau aggregation and clearance

Cook, C., Carlomagno, Y., Gendron, T. F., Dunmore, J., Scheffel, K., Stetler, C., Davis, M., Dickson, D., Jarpe, M., DeTure, M. & Petrucelli, L., Jan 2014, In : Human molecular genetics. 23, 1, p. 104-116 13 p., ddt402.

Research output: Contribution to journalArticle

122 Scopus citations

Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress

Zhang, Y. J., Jansen-West, K., Xu, Y. F., Gendron, T. F., Bieniek, K. F., Lin, W. L., Sasaguri, H., Caulfield, T., Hubbard, J., Daughrity, L., Chew, J., Belzil, V. V., Prudencio, M., Stankowski, J. N., Castanedes-Casey, M., Whitelaw, E., Ash, P. E. A., DeTure, M., Rademakers, R., Boylan, K. B. & 2 others, Dickson, D. W. & Petrucelli, L., Jan 1 2014, In : Acta neuropathologica. 128, 4, p. 505-524 20 p.

Research output: Contribution to journalArticle

159 Scopus citations

Characterization of DNA hypermethylation in the cerebellum of c9FTD/ALS patients

Belzil, V. V., Bauer, P. O., Gendron, T. F., Murray, M. E., Dickson, D. & Petrucelli, L., Oct 10 2014, In : Brain Research. 1584, p. 15-21 7 p.

Research output: Contribution to journalArticle

50 Scopus citations

Dipeptide repeat proteins are present in the p62 positive inclusions in patients with frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9ORF72

Mann, D. M. A., Rollinson, S., Robinson, A., Bennion Callister, J., Thompson, J. C., Snowden, J. S., Gendron, T. D., Petrucelli, L., Masuda-Suzukake, M., Hasegawa, M., Davidson, Y. & Pickering-Brown, S., Jan 27 2014, In : Acta neuropathologica communications. 2, 1, 68.

Research output: Contribution to journalArticle

100 Scopus citations

Discovery of a Biomarker and Lead Small Molecules to Target r(GGGGCC)-Associated Defects in c9FTD/ALS

Su, Z., Zhang, Y., Gendron, T. F., Bauer, P. O., Chew, J., Yang, W. Y., Fostvedt, E., Jansen-West, K., Belzil, V. V., Desaro, P., Johnston, A., Overstreet, K., Oh, S. Y., Todd, P. K., Berry, J. D., Cudkowicz, M. E., Boeve, B. F., Dickson, D., Floeter, M. K., Traynor, B. J. & 9 others, Morelli, C., Ratti, A., Silani, V., Rademakers, R., Brown, R. H., Rothstein, J. D., Boylan, K. B., Petrucelli, L. & Disney, M. D., Sep 3 2014, In : Neuron. 83, 5, p. 1043-1050 8 p.

Research output: Contribution to journalArticle

181 Scopus citations

Targeted manipulation of the sortilin-progranulin axis rescues progranulin haploinsufficiency

Lee, W. C., Almeida, S., Prudencio, M., Caulfield, T. R., Zhang, Y. J., Tay, W. M., Bauer, P. O., Chew, J., Sasaguri, H., Jansen-west, K. R., Gendron, T. F., Stetler, C. T., Finch, N. C., Mackenzie, I. R., Rademakers, R., Gao, F. B. & Petrucelli, L., Mar 1 2014, In : Human molecular genetics. 23, 6, p. 1467-1478 12 p., ddt534.

Research output: Contribution to journalArticle

49 Scopus citations