| Publication |
Sentence |
Publish Date |
Extraction Date |
Species |
| Chelsy R Eddings, Nicolas Arbez, Sergey Akimov, Michal Geva, Michael R Hayden, Christopher A Ros. Pridopidine protects neurons from mutant-huntingtin toxicity via the sigma-1 receptor. Neurobiology of disease. vol 129. 2020-03-24. PMID:31108174. |
huntington's disease (hd) is a neurodegenerative disease caused by a cag repeat expansion in the huntingtin gene (htt), translated into a huntingtin protein with a polyglutamine expansion. |
2020-03-24 |
2023-08-13 |
mouse |
| Andrea Comella Bolla, Tony Valente, Andres Miguez, Veronica Brito, Silvia Gines, Carme Solà, Marco Straccia, Josep M Canal. CD200 is up-regulated in R6/1 transgenic mouse model of Huntington's disease. PloS one. vol 14. issue 12. 2020-03-23. PMID:31790427. |
in huntington's disease (hd), striatal medium spiny neurons (msns) are particularly sensitive to the presence of a cag repeat in the huntingtin (htt) gene. |
2020-03-23 |
2023-08-13 |
mouse |
| Nicholas S Caron, Amber L Southwell, Cynthia C Brouwers, Louisa Dal Cengio, Yuanyun Xie, Hailey Findlay Black, Lisa M Anderson, Seunghyun Ko, Xiang Zhu, Sander J van Deventer, Melvin M Evers, Pavlina Konstantinova, Michael R Hayde. Potent and sustained huntingtin lowering via AAV5 encoding miRNA preserves striatal volume and cognitive function in a humanized mouse model of Huntington disease. Nucleic acids research. vol 48. issue 1. 2020-03-18. PMID:31745548. |
huntington disease (hd) is a fatal neurodegenerative disease caused by a pathogenic expansion of a cag repeat in the huntingtin (htt) gene. |
2020-03-18 |
2023-08-13 |
mouse |
| Hongsun Park, Haruko Miyazaki, Tomoyuki Yamanaka, Nobuyuki Nukin. Non-coding RNA Neat1 and Abhd11os expressions are dysregulated in medium spiny neurons of Huntington disease model mice. Neuroscience research. vol 147. 2020-03-17. PMID:30391555. |
huntington disease (hd) is a neurodegenerative disorder caused by expanded cag repeats in the exon1 of huntingtin gene (htt). |
2020-03-17 |
2023-08-13 |
mouse |
| 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, Brett Trost, Fiona Richards, Emilia K Bijlsma, Ferdinando Squitieri, Colin J D Ross, Stephen W Scherer, Michael A Eberle, Ryan K C Yuen, Michael R Hayde. Length of Uninterrupted CAG, Independent of Polyglutamine Size, Results in Increased Somatic Instability, Hastening Onset of Huntington Disease. American journal of human genetics. vol 104. issue 6. 2020-03-11. PMID:31104771. |
huntington disease (hd) is caused by a cag repeat expansion in the huntingtin (htt) gene. |
2020-03-11 |
2023-08-13 |
Not clear |
| Zixing Fu, Fang Liu, Chunyue Liu, Beifang Jin, Yueqing Jiang, Mingliang Tang, Xin Qi, Xing Gu. Mutant huntingtin inhibits the mitochondrial unfolded protein response by impairing ABCB10 mRNA stability. Biochimica et biophysica acta. Molecular basis of disease. vol 1865. issue 6. 2020-02-03. PMID:30802639. |
numerous studies have shown that mitochondrial dysfunction contributes to consequential phenotypes of huntington's disease (hd), a fatal and inherited neurodegenerative disease caused by the expanded cag repeats in the n-terminus of the huntingtin (htt) gene. |
2020-02-03 |
2023-08-13 |
Not clear |
| Ashley A Zurawel, Ruth Kabeche, Sonja E DiGregorio, Lin Deng, Kartikeya M Menon, Hannah Opalko, Martin L Duennwald, James B Moseley, Surachai Supattapon. CAG Expansions Are Genetically Stable and Form Nontoxic Aggregates in Cells Lacking Endogenous Polyglutamine Proteins. mBio. vol 7. issue 5. 2019-12-30. PMID:27677791. |
in humans, proteins such as huntingtin (htt) with abnormally expanded polyq regions cause neurodegenerative diseases such as huntington's disease (hd). |
2019-12-30 |
2023-08-13 |
Not clear |
| Chenglong Yu, Chun Hei Li, Sidong Chen, Hanna Yoo, Xianan Qin, Hyokeun Par. Decreased BDNF Release in Cortical Neurons of a Knock-in Mouse Model of Huntington's Disease. Scientific reports. vol 8. issue 1. 2019-12-13. PMID:30451892. |
huntington's disease (hd) is a dominantly inherited neurodegenerative disease caused by an increase in cag repeats in the huntingtin gene (htt). |
2019-12-13 |
2023-08-13 |
mouse |
| Shagun R Mehta, Colton M Tom, Yizhou Wang, Catherine Bresee, David Rushton, Pranav P Mathkar, Jie Tang, Virginia B Matti. Human Huntington's Disease iPSC-Derived Cortical Neurons Display Altered Transcriptomics, Morphology, and Maturation. Cell reports. vol 25. issue 4. 2019-11-14. PMID:30355486. |
huntington's disease (hd) is a neurodegenerative disease caused by an expanded cag repeat in the huntingtin (htt) gene. |
2019-11-14 |
2023-08-13 |
mouse |
| Arianna Novati, Thomas Hentrich, Zinah Wassouf, Jonasz J Weber, Libo Yu-Taeger, Nicole Déglon, Huu Phuc Nguyen, Julia M Schulze-Hentric. Environment-dependent striatal gene expression in the BACHD rat model for Huntington disease. Scientific reports. vol 8. issue 1. 2019-10-07. PMID:29643462. |
huntington disease (hd) is an autosomal dominant neurodegenerative disorder caused by a mutation in the huntingtin (htt) gene which results in progressive neurodegeneration in the striatum, cortex, and eventually most brain areas. |
2019-10-07 |
2023-08-13 |
mouse |
| D D O Martin, C Kay, J A Collins, Y T Nguyen, R A Slama, M R Hayde. A human huntingtin SNP alters post-translational modification and pathogenic proteolysis of the protein causing Huntington disease. Scientific reports. vol 8. issue 1. 2019-10-07. PMID:29802276. |
huntington disease (hd) is a dominantly inherited neurodegenerative disorder caused by an expanded cag trinucleotide repeat in the huntingtin (htt) gene. |
2019-10-07 |
2023-08-13 |
human |
| Priscila Aparecida Costa Valadão, Bruna da Silva Oliveira, Julliane V Joviano-Santos, Érica Leandro Marciano Vieira, Natalia Pessoa Rocha, Antônio Lúcio Teixeira, Cristina Guatimosim, Aline Silva de Mirand. Inflammatory changes in peripheral organs in the BACHD murine model of Huntington's disease. Life sciences. vol 232. 2019-10-07. PMID:31302194. |
huntington's disease (hd) is a neurodegenerative disease caused by a cag repeat expansion in the gene encoding the huntingtin protein (htt). |
2019-10-07 |
2023-08-13 |
mouse |
| Shu-Ying Liu, Xiao-Lin Yu, Jie Zhu, Xiang-Meng Liu, Yue Zhang, Quan-Xiu Dong, Shan Ma, Rui-Tian Li. Intravenous immunoglobulin ameliorates motor and cognitive deficits and neuropathology in R6/2 mouse model of Huntington's disease by decreasing mutant huntingtin protein level and normalizing NF-κB signaling pathway. Brain research. vol 1697. 2019-09-27. PMID:29902468. |
huntington's disease (hd) is a fatal neurodegenerative disorder characterized by progressive movement disorders and cognitive deficits, which is caused by a cag-repeat expansion encoding an extended polyglutamine (polyq) tract in the huntingtin protein (htt). |
2019-09-27 |
2023-08-13 |
mouse |
| Kenneth W Drombosky, Sascha Rode, Ravi Kodali, Tija C Jacob, Michael J Palladino, Ronald Wetze. Mutational analysis implicates the amyloid fibril as the toxic entity in Huntington's disease. Neurobiology of disease. vol 120. 2019-09-06. PMID:30171891. |
in huntington disease (hd), an expanded polyglutamine (polyq > 37) sequence within huntingtin (htt) exon1 leads to enhanced disease risk. |
2019-09-06 |
2023-08-13 |
rat |
| Ida Höijer, Yu-Chih Tsai, Tyson A Clark, Paul Kotturi, Niklas Dahl, Eva-Lena Stattin, Marie-Louise Bondeson, Lars Feuk, Ulf Gyllensten, Adam Ameu. Detailed analysis of HTT repeat elements in human blood using targeted amplification-free long-read sequencing. Human mutation. vol 39. issue 9. 2019-07-26. PMID:29932473. |
here, we used an amplification-free protocol for targeted enrichment utilizing the crispr/cas9 system (no-amp targeted sequencing) in combination with single molecule, real-time (smrt) sequencing for studying repeat elements in the huntingtin (htt) gene, where an expanded cag repeat is causative for huntington disease. |
2019-07-26 |
2023-08-13 |
human |
| Tara E Wood, Joshua Barry, Zhenquin Yang, Carlos Cepeda, Michael S Levine, Michelle Gra. Mutant huntingtin reduction in astrocytes slows disease progression in the BACHD conditional Huntington's disease mouse model. Human molecular genetics. vol 28. issue 3. 2019-07-15. PMID:30312396. |
neuronal and non-neuronal cells express the huntingtin (htt) protein, yet neurodegeneration in huntington's disease (hd) is largely selective, affecting most prominently striatal medium spiny neurons and cortical pyramidal neurons. |
2019-07-15 |
2023-08-13 |
mouse |
| Xiaoli Sun, Yuhua Fu, Yuyin Pan, Boxun L. Conformation-dependent recognition of mutant HTT (huntingtin) proteins by selective autophagy. Autophagy. vol 13. issue 12. 2019-07-05. PMID:28976800. |
protein misfolding is the common theme for neurodegenerative disorders including huntington disease (hd), which is mainly caused by cytotoxicity of the mutant htt (huntingtin) protein (mhtt). |
2019-07-05 |
2023-08-13 |
mouse |
| Robert Goold, Michael Flower, Davina Hensman Moss, Chris Medway, Alison Wood-Kaczmar, Ralph Andre, Pamela Farshim, Gill P Bates, Peter Holmans, Lesley Jones, Sarah J Tabriz. FAN1 modifies Huntington's disease progression by stabilizing the expanded HTT CAG repeat. Human molecular genetics. vol 28. issue 4. 2019-06-21. PMID:30358836. |
huntington's disease (hd) is an inherited neurodegenerative disease caused by an expanded cag repeat in the huntingtin (htt) gene. |
2019-06-21 |
2023-08-13 |
human |
| Edith L Pfister, Natalie DiNardo, Erica Mondo, Florie Borel, Faith Conroy, Cara Fraser, Gwladys Gernoux, Xin Han, Danjing Hu, Emily Johnson, Lori Kennington, PengPeng Liu, Suzanne J Reid, Ellen Sapp, Petr Vodicka, Tim Kuchel, A Jennifer Morton, David Howland, Richard Moser, Miguel Sena-Esteves, Guangping Gao, Christian Mueller, Marian DiFiglia, Neil Aroni. Artificial miRNAs Reduce Human Mutant Huntingtin Throughout the Striatum in a Transgenic Sheep Model of Huntington's Disease. Human gene therapy. vol 29. issue 6. 2019-06-07. PMID:29207890. |
huntington's disease (hd) is a fatal neurodegenerative disease caused by a genetic expansion of the cag repeat region in the huntingtin (htt) gene. |
2019-06-07 |
2023-08-13 |
mouse |
| Matheus B Victor, Michelle Richner, Hannah E Olsen, Seong Won Lee, Alejandro M Monteys, Chunyu Ma, Christine J Huh, Bo Zhang, Beverly L Davidson, X William Yang, Andrew S Yo. Striatal neurons directly converted from Huntington's disease patient fibroblasts recapitulate age-associated disease phenotypes. Nature neuroscience. vol 21. issue 3. 2019-05-15. PMID:29403030. |
in huntington's disease (hd), expansion of cag codons in the huntingtin gene (htt) leads to the aberrant formation of protein aggregates and the differential degeneration of striatal medium spiny neurons (msns). |
2019-05-15 |
2023-08-13 |
Not clear |