| Publication |
Sentence |
Publish Date |
Extraction Date |
Species |
| Martin L Duennwal. Cellular stress responses in protein misfolding diseases. Future science OA. vol 1. issue 2. 2020-10-01. PMID:28031871. |
huntington's disease is an inherited neurodegenerative disease caused by the misfolding of an abnormally expanded polyglutamine (polyq) region in the protein huntingtin (htt), polyqhtt. |
2020-10-01 |
2023-08-13 |
human |
| Dong-Kyu Kim, Kyu-Won Cho, Woo Jung Ahn, Dayana Perez-Acuña, Hyunsu Jeong, He-Jin Lee, Seung-Jae Le. Cell-to-cell Transmission of Polyglutamine Aggregates in Experimental neurobiology. vol 26. issue 6. 2020-10-01. PMID:29302199. |
cell-to-cell transmission of polyglutamine aggregates in huntington disease (hd) is an inherited neurodegenerative disorder characterized by motor and cognitive dysfunction caused by expansion of polyglutamine (polyq) repeat in exon 1 of huntingtin (htt). |
2020-10-01 |
2023-08-13 |
Not clear |
| Anne-Christine Plank, Fabio Canneva, Kerstin A Raber, Yvonne K Urbach, Julia Dobner, Maja Puchades, Jan G Bjaalie, Clarissa Gillmann, Tobias Bäuerle, Olaf Riess, Hoa H P Nguyen, Stephan von Hörste. Early Alterations in Operant Performance and Prominent Huntingtin Aggregation in a Congenic F344 Rat Line of the Classical CAG Frontiers in neuroscience. vol 12. 2020-10-01. PMID:29422836. |
early alterations in operant performance and prominent huntingtin aggregation in a congenic f344 rat line of the classical cag the transgenic rat model of huntington disease expressing a fragment of mutant htt (tghd rat) has been thoroughly characterized and reproduces hallmark symptoms of human adult-onset hd. |
2020-10-01 |
2023-08-13 |
human |
| Sonay Keskin, Cynthia C Brouwers, Marina Sogorb-Gonzalez, Raygene Martier, Josse A Depla, Astrid Vallès, Sander J van Deventer, Pavlina Konstantinova, Melvin M Ever. AAV5-miHTT Lowers Huntingtin mRNA and Protein without Off-Target Effects in Patient-Derived Neuronal Cultures and Astrocytes. Molecular therapy. Methods & clinical development. vol 15. 2020-10-01. PMID:31737741. |
huntington disease (hd) is a fatal neurodegenerative genetic disorder, thought to reflect a toxic gain of function in huntingtin (htt) protein. |
2020-10-01 |
2023-08-13 |
Not clear |
| Andonis Karachitos, Daria Grobys, Klaudia Kulczyńska, Adrian Sobusiak, Hanna Kmit. The Association of VDAC with Cell Viability of PC12 Model of Huntington's Disease. Frontiers in oncology. vol 6. 2020-09-30. PMID:27891320. |
therefore, we applied inducible pc12 cell model of hd to determine the relationship between the effect of expression of wild type and mutant huntingtin (htt and mhtt, respectively) on cell survival and mitochondria functioning in intact cells under conditions of undergoing cell divisions. |
2020-09-30 |
2023-08-13 |
Not clear |
| David M Smit. Could a Common Mechanism of Protein Degradation Impairment Underlie Many Neurodegenerative Diseases? Journal of experimental neuroscience. vol 12. 2020-09-30. PMID:30147359. |
prominent species that accumulate early and play fundamental roles in disease pathogenesis are amyloid β (aβ) and tau in alzheimer disease, α-synuclein (α-syn) in parkinson disease, and polyq-expanded huntingtin (htt) in huntington disease. |
2020-09-30 |
2023-08-13 |
Not clear |
| Magdalena Czeredys, Vladimir A Vigont, Vasilisa A Boeva, Katsuhiko Mikoshiba, Elena V Kaznacheyeva, Jacek Kuznick. Huntingtin-Associated Protein 1A Regulates Store-Operated Calcium Entry in Medium Spiny Neurons From Transgenic YAC128 Mice, a Model of Huntington's Disease. Frontiers in cellular neuroscience. vol 12. 2020-09-30. PMID:30455632. |
huntington's disease (hd) is a hereditary neurodegenerative disease that is caused by polyglutamine expansion within the huntingtin (htt) gene. |
2020-09-30 |
2023-08-13 |
mouse |
| Ryoma Morigaki, Satoshi Got. Striatal Vulnerability in Huntington's Disease: Neuroprotection Versus Neurotoxicity. Brain sciences. vol 7. issue 6. 2020-09-29. PMID:28590448. |
huntington's disease (hd) is an autosomal dominant neurodegenerative disease caused by the expansion of a cag trinucleotide repeat encoding an abnormally long polyglutamine tract (polyq) in the huntingtin (htt) protein. |
2020-09-29 |
2023-08-13 |
Not clear |
| Wojciech J Szlachcic, Kalina Wiatr, Marta Trzeciak, Marek Figlerowicz, Maciej Figie. The Generation of Mouse and Human Huntington Disease iPS Cells Suitable for Frontiers in molecular neuroscience. vol 10. 2020-09-29. PMID:28848389. |
the generation of mouse and human huntington disease ips cells suitable for huntington disease (hd) is an incurable neurodegenerative disorder caused by expansion of cag repeats in huntingtin (htt) gene, resulting in expanded polyglutamine tract in htt protein. |
2020-09-29 |
2023-08-13 |
mouse |
| Maxmore Chaibva, Xiang Gao, Pranav Jain, Warren A Campbell, Shelli L Frey, Justin Legleite. Sphingomyelin and GM1 Influence Huntingtin Binding to, Disruption of, and Aggregation on Lipid Membranes. ACS omega. vol 3. issue 1. 2020-09-29. PMID:29399649. |
huntington disease (hd) is an inherited neurodegenerative disease caused by the expansion beyond a critical threshold of a polyglutamine (polyq) tract near the n-terminus of the huntingtin (htt) protein. |
2020-09-29 |
2023-08-13 |
Not clear |
| Freja K Ekman, David S Ojala, Maroof M Adil, Paola A Lopez, David V Schaffer, Thomas Ga. CRISPR-Cas9-Mediated Genome Editing Increases Lifespan and Improves Motor Deficits in a Huntington's Disease Mouse Model. Molecular therapy. Nucleic acids. vol 17. 2020-09-28. PMID:31465962. |
huntington's disease (hd) is a currently incurable and, ultimately, fatal neurodegenerative disorder caused by a cag trinucleotide repeat expansion within exon 1 of the huntingtin (htt) gene, which results in the production of a mutant protein that forms inclusions and selectively destroys neurons in the striatum and other adjacent structures. |
2020-09-28 |
2023-08-13 |
mouse |
| Jordi Creus-Muncunill, Michelle E Ehrlic. Cell-Autonomous and Non-cell-Autonomous Pathogenic Mechanisms in Huntington's Disease: Insights from In Vitro and In Vivo Models. Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics. vol 16. issue 4. 2020-08-24. PMID:31529216. |
the huntingtin (htt) protein and its mutant form (mutant huntingtin, mhtt) are ubiquitously expressed but although multiple brain regions are affected, the most vulnerable brain region is the striatum. |
2020-08-24 |
2023-08-13 |
mouse |
| Daniel Savitt, Joseph Jankovi. Clinical phenotype in carriers of intermediate alleles in the huntingtin gene. Journal of the neurological sciences. vol 402. 2020-08-17. PMID:31103960. |
to describe the phenotype of individuals with intermediate allele (ia) cag repeat length in the huntingtin (htt) gene evaluated at the parkinson's disease center and movement disorders clinic (pdcmdc) at baylor college of medicine (bcm). |
2020-08-17 |
2023-08-13 |
Not clear |
| Joel D Federspiel, Todd M Greco, Krystal K Lum, Ileana M Criste. Hdac4 Interactions in Huntington's Disease Viewed Through the Prism of Multiomics. Molecular & cellular proteomics : MCP. vol 18. issue 8 suppl 1. 2020-06-15. PMID:31040226. |
huntington's disease (hd) is a monogenic disorder, driven by the expansion of a trinucleotide (cag) repeat within the huntingtin (htt) gene and culminating in neuronal degeneration in the brain, predominantly in the striatum and cortex. |
2020-06-15 |
2023-08-13 |
mouse |
| Marina R von Essen, Marie N N Hellem, Tua Vinther-Jensen, Cecilie Ammitzbøll, Rikke H Hansen, Lena E Hjermind, Troels T Nielsen, Jørgen E Nielsen, Finn Sellebjer. Early Intrathecal T Helper 17.1 Cell Activity in Huntington Disease. Annals of neurology. vol 87. issue 2. 2020-05-18. PMID:31725947. |
huntington disease (hd) is an autosomal dominantly inherited neurodegenerative disorder caused by a cag repeat expansion in the huntingtin (htt) gene. |
2020-05-18 |
2023-08-13 |
Not clear |
| Yin Xu, Sheng Zhang, Hui Zhen. The cargo receptor SQSTM1 ameliorates neurofibrillary tangle pathology and spreading through selective targeting of pathological MAPT (microtubule associated protein tau). Autophagy. vol 15. issue 4. 2020-05-11. PMID:30290707. |
abbreviations: aav: adeno-associated virus; ad: alzheimer disease; alp: autophagy-lysosomal pathway; als: amyotrophic lateral sclerosis; calcoco2/ndp52: calcium binding and coiled-coil domain 2; ftd: frontotemporal dementias; hd: huntington disease; htt: huntingtin; lir: lc3-interacting region; nbr1: autophagy cargo receptor; nfe2l2/nrf2: nuclear factor, erythroid derived 2, like 2; nfts: neurofibrillary tangles; mapt: microtubule associated protein tau; optn: optineurin; p-mapt: hyperphosphorylated mapt; pfa: paraformaldehyde; tardbp/tdp-43: tar dna binding protein; tax1bp1 tax1: binding protein 1; thios: thioflavin-s; uba: ubiquitin-associated. |
2020-05-11 |
2023-08-13 |
mouse |
| Shenglong Zou, Ujendra Kuma. Somatostatin and cannabinoid receptors crosstalk in protection of huntingtin knock-in striatal neuronal cells in response to quinolinic acid. Neurochemistry international. vol 129. 2020-05-11. PMID:31381945. |
in the present study, we describe the status of somatostatin receptor 2 and 5 (sstr2 and sstr5) as well as cannabinoid type 1 receptor (cb1r) in huntingtin (htt) knock-in striatal neuronal cells. |
2020-05-11 |
2023-08-13 |
Not clear |
| Zheng Wu, Matthew Parry, Xiao-Yi Hou, Min-Hui Liu, Hui Wang, Rachel Cain, Zi-Fei Pei, Yu-Chen Chen, Zi-Yuan Guo, Sambangi Abhijeet, Gong Che. Gene therapy conversion of striatal astrocytes into GABAergic neurons in mouse models of Huntington's disease. Nature communications. vol 11. issue 1. 2020-05-05. PMID:32107381. |
huntington's disease (hd) is caused by huntingtin (htt) gene mutation resulting in the loss of striatal gabaergic neurons and motor functional deficits. |
2020-05-05 |
2023-08-13 |
mouse |
| Zhihao Qi, Zhen Huang, Feng Xie, Linxi Che. Dynamin-related protein 1: A critical protein in the pathogenesis of neural system dysfunctions and neurodegenerative diseases. Journal of cellular physiology. vol 234. issue 7. 2020-05-04. PMID:30515821. |
here, we will give a detailed review about the recent developments in regards to dynamin-related protein 1 (drp1) induced unbalanced mitochondrial dynamics, excessive mitochondrial division, and neuronal injury in neural system dysfunctions and neurodegenerative diseases, including the drp1 knockout induced mice embryonic death, the dysfunction of the drp1-dependent mitochondrial division induced neuronal cell apoptosis and impaired neuronal axonal transportation, the abnormal interaction between drp1 and amyloid β (aβ) in alzheimer's disease (ad), the mutant huntingtin (htt) in huntington's disease (hd), and the drp1-associated pathogenesis of other neurodegenerative diseases such as parkinson's disease (pd) and amyotrophic lateral sclerosis (als). drp1 is required for mitochondrial division determining the size, shape, distribution, and remodeling as well as maintaining of mitochondrial integrity in mammalian cells. |
2020-05-04 |
2023-08-13 |
mouse |
| Lisa M Stanek, Jie Bu, Lamya S Shihabuddi. Astrocyte transduction is required for rescue of behavioral phenotypes in the YAC128 mouse model with AAV-RNAi mediated HTT lowering therapeutics. Neurobiology of disease. vol 129. 2020-03-24. PMID:31042572. |
huntington's disease (hd) is a fatal autosomal dominant neurodegenerative disease caused by a cag expansion, which translates into an elongated polyglutamine (polyq) repeat near the amino-terminus of the huntingtin protein (htt). |
2020-03-24 |
2023-08-13 |
mouse |