Journal articles on the topic 'GGGGCC repeats'
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Jiao, Bin, Mengli Wang, Hao Feng, Han Bao, Feiran Zhang, Hao Wu, Junling Wang, Beisha Tang, Peng Jin, and Lu Shen. "Downregulation of TOP2 modulates neurodegeneration caused by GGGGCC expanded repeats." Human Molecular Genetics 30, no. 10 (March 22, 2021): 893–901. http://dx.doi.org/10.1093/hmg/ddab079.
Full textLiu, Xiaole, Xinyue Zhao, Jinhan He, Sishi Wang, Xinfei Shen, Qingfeng Liu, and Shenlin Wang. "Advances in the Structure of GGGGCC Repeat RNA Sequence and Its Interaction with Small Molecules and Protein Partners." Molecules 28, no. 15 (August 1, 2023): 5801. http://dx.doi.org/10.3390/molecules28155801.
Full textvan ‘t Spijker, Heleen M., Emily E. Stackpole, Sandra Almeida, Olga Katsara, Botao Liu, Kuang Shen, Robert J. Schneider, Fen-Biao Gao, and Joel D. Richter. "Ribosome profiling reveals novel regulation of C9ORF72 GGGGCC repeat-containing RNA translation." RNA 28, no. 2 (November 30, 2021): 123–38. http://dx.doi.org/10.1261/rna.078963.121.
Full textBabić Leko, Mirjana, Vera Župunski, Jason Kirincich, Dinko Smilović, Tibor Hortobágyi, Patrick R. Hof, and Goran Šimić. "Molecular Mechanisms of Neurodegeneration Related to C9orf72 Hexanucleotide Repeat Expansion." Behavioural Neurology 2019 (January 15, 2019): 1–18. http://dx.doi.org/10.1155/2019/2909168.
Full textHatanaka, Yukari, Tomohiro Umeda, Keiko Shigemori, Toshihide Takeuchi, Yoshitaka Nagai, and Takami Tomiyama. "C9orf72 Hexanucleotide Repeat Expansion-Related Neuropathology Is Attenuated by Nasal Rifampicin in Mice." Biomedicines 10, no. 5 (May 6, 2022): 1080. http://dx.doi.org/10.3390/biomedicines10051080.
Full textZhang, Yong-Jie, Lin Guo, Patrick K. Gonzales, Tania F. Gendron, Yanwei Wu, Karen Jansen-West, Aliesha D. O’Raw, et al. "Heterochromatin anomalies and double-stranded RNA accumulation underlie C9orf72 poly(PR) toxicity." Science 363, no. 6428 (February 14, 2019): eaav2606. http://dx.doi.org/10.1126/science.aav2606.
Full textHaeusler, Aaron R. "Nucleotide Structural Polymorphisms Formed by GGGGCC Repeats Cause C9orf72 Abortive Transcription and Nucleolar Stress." Biophysical Journal 106, no. 2 (January 2014): 488a. http://dx.doi.org/10.1016/j.bpj.2013.11.4477.
Full textTeng, Ye, Ming Zhu, and Zhidong Qiu. "G-quadruplexes in Repeat Expansion Disorders." International Journal of Molecular Sciences 24, no. 3 (January 25, 2023): 2375. http://dx.doi.org/10.3390/ijms24032375.
Full textBalendra, Rubika, Igor Ruiz de los Mozos, Hana M. Odeh, Idoia Glaria, Carmelo Milioto, Katherine M. Wilson, Agnieszka M. Ule, et al. "Transcriptome-wide RNA binding analysis of C9orf72 poly(PR) dipeptides." Life Science Alliance 6, no. 9 (July 12, 2023): e202201824. http://dx.doi.org/10.26508/lsa.202201824.
Full textReddy, Kaalak, Monika H. M. Schmidt, Jaimie M. Geist, Neha P. Thakkar, Gagan B. Panigrahi, Yuh-Hwa Wang, and Christopher E. Pearson. "Processing of double-R-loops in (CAG)·(CTG) and C9orf72 (GGGGCC)·(GGCCCC) repeats causes instability." Nucleic Acids Research 42, no. 16 (August 21, 2014): 10473–87. http://dx.doi.org/10.1093/nar/gku658.
Full textTaki, Motahareh, Kushal J. Rohilla, Maria Barton, Madison Funneman, Najiyah Benzabeh, Swati Naphade, Lisa M. Ellerby, Keith T. Gagnon, and Mohtashim H. Shamsi. "Novel probes for label-free detection of neurodegenerative GGGGCC repeats associated with amyotrophic lateral sclerosis." Analytical and Bioanalytical Chemistry 411, no. 26 (August 21, 2019): 6995–7003. http://dx.doi.org/10.1007/s00216-019-02075-8.
Full textHalim, Dilara O., Gopinath Krishnan, Evan P. Hass, Soojin Lee, Mamta Verma, Sandra Almeida, Yuanzheng Gu, Deborah Y. Kwon, Thomas G. Fazzio, and Fen-Biao Gao. "The exocyst subunit EXOC2 regulates the toxicity of expanded GGGGCC repeats in C9ORF72-ALS/FTD." Cell Reports 43, no. 7 (July 2024): 114375. http://dx.doi.org/10.1016/j.celrep.2024.114375.
Full textDeJesus-Hernandez, Mariely, Ross A. Aleff, Jazmyne L. Jackson, NiCole A. Finch, Matthew C. Baker, Tania F. Gendron, Melissa E. Murray, et al. "Long-read targeted sequencing uncovers clinicopathological associations for C9orf72-linked diseases." Brain 144, no. 4 (April 1, 2021): 1082–88. http://dx.doi.org/10.1093/brain/awab006.
Full textvan der Ende, Emma L., Jazmyne L. Jackson, Adrianna White, Harro Seelaar, Marka van Blitterswijk, and John C. Van Swieten. "Unravelling the clinical spectrum and the role of repeat length in C9ORF72 repeat expansions." Journal of Neurology, Neurosurgery & Psychiatry 92, no. 5 (January 15, 2021): 502–9. http://dx.doi.org/10.1136/jnnp-2020-325377.
Full textSatoh, Jun-Ichi, Yoji Yamamoto, Shouta Kitano, Mika Takitani, Naohiro Asahina, and Yoshihiro Kino. "Molecular Network Analysis Suggests a Logical Hypothesis for the Pathological Role of C9orf72 in Amyotrophic Lateral Sclerosis/Frontotemporal Dementia." Journal of Central Nervous System Disease 6 (January 2014): JCNSD.S18103. http://dx.doi.org/10.4137/jcnsd.s18103.
Full textCooper-Knock, Johnathan, Joanna J. Bury, Paul R. Heath, Matthew Wyles, Adrian Higginbottom, Catherine Gelsthorpe, J. Robin Highley, et al. "C9ORF72 GGGGCC Expanded Repeats Produce Splicing Dysregulation which Correlates with Disease Severity in Amyotrophic Lateral Sclerosis." PLOS ONE 10, no. 5 (May 27, 2015): e0127376. http://dx.doi.org/10.1371/journal.pone.0127376.
Full textShpilyukova, Yu A., E. Yu Fedotova, T. V. Pogoda, N. Yu Abramycheva, A. S. Vetchinova, M. N. Zakharova, and S. N. Illarioshkin. "Evaluation of methylation status of the 5’-promoter region of C9orf72 gene in Russian patients with neurodegenerative diseases." Neuromuscular Diseases 8, no. 2 (July 20, 2018): 33–41. http://dx.doi.org/10.17650/2222-8721-2018-8-2-33-41.
Full textOrmandzhiev, S., T. Todorov, T. Angelov, T. Chamova, V. Mitev, A. Todorova, and I. Tournev. "Targeted Screening of the C9orf72 Gene in Bulgarian Amyotrophic Lateral Sclerosis Patients." Acta Medica Bulgarica 49, no. 1 (April 1, 2022): 12–16. http://dx.doi.org/10.2478/amb-2022-0002.
Full textShi, Kevin Y., Eiichiro Mori, Zehra F. Nizami, Yi Lin, Masato Kato, Siheng Xiang, Leeju C. Wu, et al. "Toxic PRn poly-dipeptides encoded by the C9orf72 repeat expansion block nuclear import and export." Proceedings of the National Academy of Sciences 114, no. 7 (January 9, 2017): E1111—E1117. http://dx.doi.org/10.1073/pnas.1620293114.
Full textZhang, Yuan, Christopher Roland, and Celeste Sagui. "Structural and Dynamical Characterization of DNA and RNA Quadruplexes Obtained from the GGGGCC and GGGCCT Hexanucleotide Repeats Associated with C9FTD/ALS and SCA36 Diseases." ACS Chemical Neuroscience 9, no. 5 (December 27, 2017): 1104–17. http://dx.doi.org/10.1021/acschemneuro.7b00476.
Full textKaur, Jaslovleen, Shaista Parveen, Uzma Shamim, Pooja Sharma, Varun Suroliya, Akhilesh Kumar Sonkar, Istaq Ahmad, et al. "Investigations of Huntington’s Disease and Huntington’s Disease-Like Syndromes in Indian Choreatic Patients." Journal of Huntington's Disease 9, no. 3 (October 8, 2020): 283–89. http://dx.doi.org/10.3233/jhd-200398.
Full textHu, Jiaxin, Jing Liu, Liande Li, Keith T. Gagnon, and David R. Corey. "Engineering Duplex RNAs for Challenging Targets: Recognition of GGGGCC/CCCCGG Repeats at the ALS/FTD C9orf72 Locus." Chemistry & Biology 22, no. 11 (November 2015): 1505–11. http://dx.doi.org/10.1016/j.chembiol.2015.09.016.
Full textKitano, Shouta, Yoshihiro Kino, Yoji Yamamoto, Mika Takitani, Junko Miyoshi, Tsuyoshi Ishida, Yuko Saito, Kunimasa Arima, and Jun-Ichi Satoh. "Bioinformatics Data Mining Approach Suggests Coexpression of AGTPBP1 with an ALS-linked Gene C9orf72." Journal of Central Nervous System Disease 7 (January 2015): JCNSD.S24317. http://dx.doi.org/10.4137/jcnsd.s24317.
Full textDunn, Ella, Joern R. Steinert, Aelfwin Stone, Virender Sahota, Robin S. B. Williams, Stuart Snowden, and Hrvoje Augustin. "Medium-Chain Fatty Acids Rescue Motor Function and Neuromuscular Junction Degeneration in a Drosophila Model of Amyotrophic Lateral Sclerosis." Cells 12, no. 17 (August 28, 2023): 2163. http://dx.doi.org/10.3390/cells12172163.
Full textBožič, Tim, Matja Zalar, Boris Rogelj, Janez Plavec, and Primož Šket. "Structural Diversity of Sense and Antisense RNA Hexanucleotide Repeats Associated with ALS and FTLD." Molecules 25, no. 3 (January 25, 2020): 525. http://dx.doi.org/10.3390/molecules25030525.
Full textLopez-Gonzalez, Rodrigo, Dejun Yang, Mochtar Pribadi, Tanya S. Kim, Gopinath Krishnan, So Yoen Choi, Soojin Lee, Giovanni Coppola, and Fen-Biao Gao. "Partial inhibition of the overactivated Ku80-dependent DNA repair pathway rescues neurodegeneration in C9ORF72-ALS/FTD." Proceedings of the National Academy of Sciences 116, no. 19 (April 24, 2019): 9628–33. http://dx.doi.org/10.1073/pnas.1901313116.
Full textBuchman, Vladimir L., Johnathan Cooper-Knock, Natalie Connor-Robson, Adrian Higginbottom, Janine Kirby, Olga D. Razinskaya, Natalia Ninkina, and Pamela J. Shaw. "Simultaneous and independent detection of C9ORF72 alleles with low and high number of GGGGCC repeats using an optimised protocol of Southern blot hybridisation." Molecular Neurodegeneration 8, no. 1 (2013): 12. http://dx.doi.org/10.1186/1750-1326-8-12.
Full textMori, Kohji, Sven Lammich, Ian R. A. Mackenzie, Ignasi Forné, Sonja Zilow, Hans Kretzschmar, Dieter Edbauer, et al. "hnRNP A3 binds to GGGGCC repeats and is a constituent of p62-positive/TDP43-negative inclusions in the hippocampus of patients with C9orf72 mutations." Acta Neuropathologica 125, no. 3 (February 5, 2013): 413–23. http://dx.doi.org/10.1007/s00401-013-1088-7.
Full textZhang, Yuan, Christopher Roland, and Celeste Sagui. "Structure and Dynamics of DNA and RNA Double Helices Obtained from the GGGGCC and CCCCGG Hexanucleotide Repeats That Are the Hallmark of C9FTD/ALS Diseases." ACS Chemical Neuroscience 8, no. 3 (December 19, 2016): 578–91. http://dx.doi.org/10.1021/acschemneuro.6b00348.
Full textFreibaum, Brian D., Yubing Lu, Rodrigo Lopez-Gonzalez, Nam Chul Kim, Sandra Almeida, Kyung-Ha Lee, Nisha Badders, et al. "GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport." Nature 525, no. 7567 (August 26, 2015): 129–33. http://dx.doi.org/10.1038/nature14974.
Full textThys, Ryan Griffin, and Yuh-Hwa Wang. "DNA Replication Dynamics of the GGGGCC Repeat of theC9orf72Gene." Journal of Biological Chemistry 290, no. 48 (October 13, 2015): 28953–62. http://dx.doi.org/10.1074/jbc.m115.660324.
Full textAkimoto, Chizuru, Lars Forsgren, Jan Linder, Anna Birve, Irene Backlund, Jörgen Andersson, Ann-Charloth Nilsson, Helena Alstermark, and Peter M. Andersen. "No GGGGCC-hexanucleotide repeat expansion inC9ORF72in parkinsonism patients in Sweden." Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration 14, no. 1 (September 17, 2012): 26–29. http://dx.doi.org/10.3109/17482968.2012.725415.
Full textFratta, Pietro, Mark Poulter, Tammaryn Lashley, Jonathan D. Rohrer, James M. Polke, Jon Beck, Natalie Ryan, et al. "Homozygosity for the C9orf72 GGGGCC repeat expansion in frontotemporal dementia." Acta Neuropathologica 126, no. 3 (July 2, 2013): 401–9. http://dx.doi.org/10.1007/s00401-013-1147-0.
Full textYe, Amanda J., W. John Haynes, and Daniel P. Romero. "Expression of Mutated Paramecium Telomerase RNAs In Vivo Leads to Templating Errors That Resemble Those Made by Retroviral Reverse Transcriptase." Molecular and Cellular Biology 19, no. 4 (April 1, 1999): 2887–94. http://dx.doi.org/10.1128/mcb.19.4.2887.
Full textMori, K., S. M. Weng, T. Arzberger, S. May, K. Rentzsch, E. Kremmer, B. Schmid, et al. "The C9orf72 GGGGCC Repeat Is Translated into Aggregating Dipeptide-Repeat Proteins in FTLD/ALS." Science 339, no. 6125 (February 7, 2013): 1335–38. http://dx.doi.org/10.1126/science.1232927.
Full textZhang, Yun, Junliu Huang, Kainan Yu, and Xiaojie Cui. "G-Quadruplexes Formation by the C9orf72 Nucleotide Repeat Expansion d(GGGGCC)n and Conformation Regulation by Fangchinoline." Molecules 28, no. 12 (June 9, 2023): 4671. http://dx.doi.org/10.3390/molecules28124671.
Full textTseng, Yi-Ju, Siara N. Sandwith, Katelyn M. Green, Antonio E. Chambers, Amy Krans, Heather M. Raimer, Meredith E. Sharlow, et al. "The RNA helicase DHX36–G4R1 modulates C9orf72 GGGGCC hexanucleotide repeat–associated translation." Journal of Biological Chemistry 297, no. 2 (August 2021): 100914. http://dx.doi.org/10.1016/j.jbc.2021.100914.
Full textKonno, T., A. Shiga, A. Tsujino, A. Sugai, T. Kato, K. Kanai, A. Yokoseki, et al. "Japanese amyotrophic lateral sclerosis patients with GGGGCC hexanucleotide repeat expansion in C9ORF72." Journal of Neurology, Neurosurgery & Psychiatry 84, no. 4 (September 25, 2012): 398–401. http://dx.doi.org/10.1136/jnnp-2012-302272.
Full textBrčić, Jasna, and Janez Plavec. "G-quadruplex formation of oligonucleotides containing ALS and FTD related GGGGCC repeat." Frontiers of Chemical Science and Engineering 10, no. 2 (February 1, 2016): 222–37. http://dx.doi.org/10.1007/s11705-016-1556-4.
Full textHe, Hua, Wen Huang, Ruoxi Wang, Yunting Lin, Yichen Guo, Jing Deng, Haitao Deng, et al. "Amyotrophic Lateral Sclerosis-associated GGGGCC repeat expansion promotes Tau phosphorylation and toxicity." Neurobiology of Disease 130 (October 2019): 104493. http://dx.doi.org/10.1016/j.nbd.2019.104493.
Full textLu, Yihuan, Chikara Dohno, and Kazuhiko Nakatani. "Recognition of expanded GGGGCC hexanucleotide repeat by synthetic ligand through interhelical binding." Biochemical and Biophysical Research Communications 531, no. 1 (October 2020): 56–61. http://dx.doi.org/10.1016/j.bbrc.2020.03.107.
Full textGoodman, Lindsey D., and Nancy M. Bonini. "Repeat-associated non-AUG (RAN) translation mechanisms are running into focus for GGGGCC-repeat associated ALS/FTD." Progress in Neurobiology 183 (December 2019): 101697. http://dx.doi.org/10.1016/j.pneurobio.2019.101697.
Full textRutherford, Nicola J., Michael G. Heckman, Mariely DeJesus-Hernandez, Matt C. Baker, Alexandra I. Soto-Ortolaza, Sruti Rayaprolu, Heather Stewart, et al. "Length of normal alleles of C9ORF72 GGGGCC repeat do not influence disease phenotype." Neurobiology of Aging 33, no. 12 (December 2012): 2950.e5–2950.e7. http://dx.doi.org/10.1016/j.neurobiolaging.2012.07.005.
Full textZamiri, Bita, Kaalak Reddy, Christopher E. Pearson, and Robert B. Macgregor. "The Structure of the Disease-Associated (GGGGCC)N Repeat from the C9ORF72 Gene." Biophysical Journal 106, no. 2 (January 2014): 283a. http://dx.doi.org/10.1016/j.bpj.2013.11.1655.
Full textROMERO, DANIEL P., and ELIZABETH H. BLACKBURN. "Circular rDNA Replicons Persist in Tetrahymena thermophila Transformants Synthesizing GGGGTC Telomeric Repeats." Journal of Eukaryotic Microbiology 42, no. 1 (January 1995): 32–43. http://dx.doi.org/10.1111/j.1550-7408.1995.tb01537.x.
Full textXu, Z., M. Poidevin, X. Li, Y. Li, L. Shu, D. L. Nelson, H. Li, et al. "Expanded GGGGCC repeat RNA associated with amyotrophic lateral sclerosis and frontotemporal dementia causes neurodegeneration." Proceedings of the National Academy of Sciences 110, no. 19 (April 3, 2013): 7778–83. http://dx.doi.org/10.1073/pnas.1219643110.
Full textGoodman, Lindsey D., Mercedes Prudencio, Nicholas J. Kramer, Luis F. Martinez-Ramirez, Ananth R. Srinivasan, Matthews Lan, Michael J. Parisi, et al. "Toxic expanded GGGGCC repeat transcription is mediated by the PAF1 complex in C9orf72-associated FTD." Nature Neuroscience 22, no. 6 (May 20, 2019): 863–74. http://dx.doi.org/10.1038/s41593-019-0396-1.
Full textShen, Jianying, Yu Zhang, Shi Zhao, Hong Mao, Zhongjing Wang, Honglian Li, and Zihui Xu. "Purα Repaired Expanded Hexanucleotide GGGGCC Repeat Noncoding RNA-Caused Neuronal Toxicity in Neuro-2a Cells." Neurotoxicity Research 33, no. 4 (October 3, 2017): 693–701. http://dx.doi.org/10.1007/s12640-017-9803-0.
Full textBrčić, Jasna, and Janez Plavec. "ALS and FTD linked GGGGCC-repeat containing DNA oligonucleotide folds into two distinct G-quadruplexes." Biochimica et Biophysica Acta (BBA) - General Subjects 1861, no. 5 (May 2017): 1237–45. http://dx.doi.org/10.1016/j.bbagen.2016.11.018.
Full textSouza, Paulo Victor Sgobbi de, Wladimir Bocca Vieira de Rezende Pinto, and Acary Souza Bulle Oliveira. "C9orf72-related disorders: expanding the clinical and genetic spectrum of neurodegenerative diseases." Arquivos de Neuro-Psiquiatria 73, no. 3 (March 2015): 246–56. http://dx.doi.org/10.1590/0004-282x20140229.
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