| Publication |
Sentence |
Publish Date |
Extraction Date |
Species |
| Amber L Southwell, Jan Ko, Paul H Patterso. Intrabody gene therapy ameliorates motor, cognitive, and neuropathological symptoms in multiple mouse models of Huntington's disease. The Journal of neuroscience : the official journal of the Society for Neuroscience. vol 29. issue 43. 2009-12-02. PMID:19864571. |
huntington's disease (hd) is an autosomal dominant neurodegenerative disease resulting from the expansion of a glutamine repeat in the huntingtin (htt) protein. |
2009-12-02 |
2023-08-12 |
mouse |
| Ming-Chang Chiang, Hui-Mei Chen, Hsing-Lin Lai, Hsiao-Wen Chen, Szu-Yi Chou, Chiung-Mei Chen, Fuu-Jen Tsai, Yijuang Cher. The A2A adenosine receptor rescues the urea cycle deficiency of Huntington's disease by enhancing the activity of the ubiquitin-proteasome system. Human molecular genetics. vol 18. issue 16. 2009-10-27. PMID:19443488. |
huntington's disease (hd) is an autosomal dominant neurodegenerative disease caused by a cag trinucleotide expansion in the huntingtin (htt) gene. |
2009-10-27 |
2023-08-12 |
mouse |
| Justin Legleiter, Gregor P Lotz, Jason Miller, Jan Ko, Cheping Ng, Geneva L Williams, Steve Finkbeiner, Paul H Patterson, Paul J Muchowsk. Monoclonal antibodies recognize distinct conformational epitopes formed by polyglutamine in a mutant huntingtin fragment. The Journal of biological chemistry. vol 284. issue 32. 2009-09-28. PMID:19491400. |
huntington disease (hd) is a neurodegenerative disorder caused by an expansion of a polyglutamine (polyq) domain in the n-terminal region of huntingtin (htt). |
2009-09-28 |
2023-08-12 |
Not clear |
| Kimberly B Kegel, Vitali Schewkunow, Ellen Sapp, Nicholas Masso, Erich E Wanker, Marian DiFiglia, Wolfgang H Goldman. Polyglutamine expansion in huntingtin increases its insertion into lipid bilayers. Biochemical and biophysical research communications. vol 387. issue 3. 2009-09-01. PMID:19607813. |
an expanded polyglutamine (q) tract (>37q) in huntingtin (htt) causes huntington disease. |
2009-09-01 |
2023-08-12 |
Not clear |
| Ryan L Boudreau, Jodi L McBride, Inês Martins, Shihao Shen, Yi Xing, Barrie J Carter, Beverly L Davidso. Nonallele-specific silencing of mutant and wild-type huntingtin demonstrates therapeutic efficacy in Huntington's disease mice. Molecular therapy : the journal of the American Society of Gene Therapy. vol 17. issue 6. 2009-08-07. PMID:19240687. |
huntington's disease (hd) is a fatal neurodegenerative disease caused by mutant huntingtin (htt) protein, and there are currently no effective treatments. |
2009-08-07 |
2023-08-12 |
mouse |
| Peter O Bauer, Hon Kit Wong, Fumitaka Oyama, Anand Goswami, Misako Okuno, Yoshihiro Kino, Haruko Miyazaki, Nobuyuki Nukin. Inhibition of Rho kinases enhances the degradation of mutant huntingtin. The Journal of biological chemistry. vol 284. issue 19. 2009-07-21. PMID:19278999. |
huntington disease (hd) is a fatal hereditary neurodegenerative disease caused by an expansion of the polyglutamine (polyq) stretch in huntingtin (htt). |
2009-07-21 |
2023-08-12 |
Not clear |
| Maria Stella Lombardi, Leonie Jaspers, Christine Spronkmans, Cinzia Gellera, Franco Taroni, Emilio Di Maria, Stefano Di Donato, William F Kaemmere. A majority of Huntington's disease patients may be treatable by individualized allele-specific RNA interference. Experimental neurology. vol 217. issue 2. 2009-07-07. PMID:19289118. |
use of rna interference to reduce huntingtin protein (htt) expression in affected brain regions may provide an effective treatment for huntington disease (hd), but it remains uncertain whether suppression of both wild-type and mutant alleles in a heterozygous patient will provide more benefit than harm. |
2009-07-07 |
2023-08-12 |
Not clear |
| Jiaxin Hu, Masayuki Matsui, Keith T Gagnon, Jacob C Schwartz, Sylvie Gabillet, Khalil Arar, Jun Wu, Ilya Bezprozvanny, David R Core. Allele-specific silencing of mutant huntingtin and ataxin-3 genes by targeting expanded CAG repeats in mRNAs. Nature biotechnology. vol 27. issue 5. 2009-06-03. PMID:19412185. |
these include machado-joseph disease (mjd) and huntington's disease (hd), which are caused by expanded cag repeats within an allele of the ataxin-3 (atxn3) and huntingtin (htt) genes, respectively. |
2009-06-03 |
2023-08-12 |
Not clear |
| Rona K Graham, Mahmoud A Pouladi, Prasad Joshi, Ge Lu, Yu Deng, Nan-Ping Wu, Bryan E Figueroa, Martina Metzler, Véronique M André, Elizabeth J Slow, Lynn Raymond, Robert Friedlander, Michael S Levine, Blair R Leavitt, Michael R Hayde. Differential susceptibility to excitotoxic stress in YAC128 mouse models of Huntington disease between initiation and progression of disease. The Journal of neuroscience : the official journal of the Society for Neuroscience. vol 29. issue 7. 2009-04-16. PMID:19228972. |
polyglutamine expansion of huntingtin (htt) results in early, progressive loss of medium spiny striatal neurons, as well as cortical neurons that project to the striatum. |
2009-04-16 |
2023-08-12 |
mouse |
| Prasad R Joshi, Nan-Ping Wu, Véronique M André, Damian M Cummings, Carlos Cepeda, John A Joyce, Jeffrey B Carroll, Blair R Leavitt, Michael R Hayden, Michael S Levine, Nigel S Bamfor. Age-dependent alterations of corticostriatal activity in the YAC128 mouse model of Huntington disease. The Journal of neuroscience : the official journal of the Society for Neuroscience. vol 29. issue 8. 2009-03-16. PMID:19244517. |
huntington disease is a genetic neurodegenerative disorder that produces motor, neuropsychiatric, and cognitive deficits and is caused by an abnormal expansion of the cag tract in the huntingtin (htt) gene. |
2009-03-16 |
2023-08-12 |
mouse |
| Tie-Shan Tang, Caixia Guo, Hongyu Wang, Xi Chen, Ilya Bezprozvann. Neuroprotective effects of inositol 1,4,5-trisphosphate receptor C-terminal fragment in a Huntington's disease mouse model. The Journal of neuroscience : the official journal of the Society for Neuroscience. vol 29. issue 5. 2009-02-24. PMID:19193873. |
huntington's disease (hd) is a dominantly inherited, progressive neurodegenerative disease caused by an expanded polyglutamine tract in huntingtin protein (htt). |
2009-02-24 |
2023-08-12 |
mouse |
| Frank N Gellerich, Zemfira Gizatullina, Huu P Nguyen, Sonata Trumbeckaite, Stefan Vielhaber, Enn Seppet, Stephan Zierz, Bernhard Landwehrmeyer, Olaf Riess, Stephan von Hörsten, Frank Striggo. Impaired regulation of brain mitochondria by extramitochondrial Ca2+ in transgenic Huntington disease rats. The Journal of biological chemistry. vol 283. issue 45. 2008-12-23. PMID:18606820. |
huntington disease (hd) is characterized by polyglutamine expansions of huntingtin (htt), but the underlying pathomechanisms have remained unclear. |
2008-12-23 |
2023-08-12 |
rat |
| Reina Inagaki, Kazuhiko Tagawa, Mei-Ling Qi, Yasushi Enokido, Hikaru Ito, Takuya Tamura, Shigeomi Shimizu, Kityomitsu Oyanagi, Nobutaka Arai, Ichiro Kanazawa, Erich E Wanker, Hitoshi Okazaw. Omi / HtrA2 is relevant to the selective vulnerability of striatal neurons in Huntington's disease. The European journal of neuroscience. vol 28. issue 1. 2008-10-20. PMID:18662332. |
in microarray analysis, omi/htra2 was decreased under the expression of mutant huntingtin (htt) in striatal neurons but not in cortical or cerebellar neurons. |
2008-10-20 |
2023-08-12 |
mouse |
| Shweta Chandra, Jieya Shao, Jennifer X Li, Mei Li, Frank M Longo, Marc I Diamon. A common motif targets huntingtin and the androgen receptor to the proteasome. The Journal of biological chemistry. vol 283. issue 35. 2008-10-03. PMID:18586675. |
huntington disease derives from a critically expanded polyglutamine tract in the huntingtin (htt) protein; a similar polyglutamine expansion in the androgen receptor (ar) causes spinobulbar muscular atrophy. |
2008-10-03 |
2023-08-12 |
Not clear |
| Brinda Ravikumar, Sara Imarisio, Sovan Sarkar, Cahir J O'Kane, David C Rubinsztei. Rab5 modulates aggregation and toxicity of mutant huntingtin through macroautophagy in cell and fly models of Huntington disease. Journal of cell science. vol 121. issue Pt 10. 2008-09-05. PMID:18430781. |
huntington disease (hd) is caused by a polyglutamine-expansion mutation in huntingtin (htt) that makes the protein toxic and aggregate-prone. |
2008-09-05 |
2023-08-12 |
Not clear |
| Nicholas R Franich, Helen L Fitzsimons, Dahna M Fong, Matthias Klugmann, Matthew J During, Deborah Youn. AAV vector-mediated RNAi of mutant huntingtin expression is neuroprotective in a novel genetic rat model of Huntington's disease. Molecular therapy : the journal of the American Society of Gene Therapy. vol 16. issue 5. 2008-08-19. PMID:18388917. |
we report the characterization of a new rapid-onset model of huntington's disease (hd) generated by adeno-associated virus (aav) vector-mediated gene transfer of n-terminal huntingtin (htt) constructs into the rat striatum. |
2008-08-19 |
2023-08-12 |
mouse |
| Bin Huang, Johannes Schiefer, Christian Sass, Christoph M Kosinski, Stefan Kochane. Inducing huntingtin inclusion formation in primary neuronal cell culture and in vivo by high-capacity adenoviral vectors expressing truncated and full-length huntingtin with polyglutamine expansion. The journal of gene medicine. vol 10. issue 3. 2008-08-07. PMID:18067195. |
huntington's disease (hd) is an inherited autosomal dominant neurodegenerative disease caused by the expansion of a cag trinucleotide repeat in exon 1 of the huntingtin (htt) gene. |
2008-08-07 |
2023-08-12 |
rat |
| Jieya Shao, William J Welch, Marc I Diamon. ROCK and PRK-2 mediate the inhibitory effect of Y-27632 on polyglutamine aggregation. FEBS letters. vol 582. issue 12. 2008-07-24. PMID:18423405. |
polyglutamine expansion in huntingtin (htt) and the androgen receptor (ar) causes untreatable neurodegenerative diseases. |
2008-07-24 |
2023-08-12 |
drosophila_melanogaster |
| Adam L Orr, Shanshan Huang, Meredith A Roberts, John C Reed, Shihua Li, Xiao-Jiang L. Sex-dependent effect of BAG1 in ameliorating motor deficits of Huntington disease transgenic mice. The Journal of biological chemistry. vol 283. issue 23. 2008-07-15. PMID:18400759. |
the pathogenesis of huntington disease (hd) is attributed to the misfolding of huntingtin (htt) caused by an expanded polyglutamine (polyq) domain. |
2008-07-15 |
2023-08-12 |
mouse |
| Michelle Gray, Dyna I Shirasaki, Carlos Cepeda, Véronique M André, Brian Wilburn, Xiao-Hong Lu, Jifang Tao, Irene Yamazaki, Shi-Hua Li, Yi E Sun, Xiao-Jiang Li, Michael S Levine, X William Yan. Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice. The Journal of neuroscience : the official journal of the Society for Neuroscience. vol 28. issue 24. 2008-07-10. PMID:18550760. |
to elucidate the pathogenic mechanisms in huntington's disease (hd) elicited by expression of full-length human mutant huntingtin (fl-mhtt), a bacterial artificial chromosome (bac)-mediated transgenic mouse model (bachd) was developed expressing fl-mhtt with 97 glutamine repeats under the control of endogenous htt regulatory machinery on the bac. |
2008-07-10 |
2023-08-12 |
mouse |