| Publication |
Sentence |
Publish Date |
Extraction Date |
Species |
| Nicholas S Caron, Raul Banos, Amirah E Aly, Yuanyun Xie, Seunghyun Ko, Nalini Potluri, Christine Anderson, Hailey Findlay Black, Lisa M Anderson, Benjamin Gordon, Amber L Southwell, Michael R Hayde. Cerebrospinal fluid mutant huntingtin is a biomarker for huntingtin lowering in the striatum of Huntington disease mice. Neurobiology of disease. 2022-02-10. PMID:35143966. |
huntington disease (hd) is a neurodegenerative disease caused by a trinucleotide repeat expansion in the htt gene encoding an elongated polyglutamine tract in the huntingtin (htt) protein. |
2022-02-10 |
2023-08-13 |
mouse |
| Li Zhang, Tengteng Wu, Yangyang Shan, Ge Li, Xue Ni, Xiaorui Chen, Xiuting Hu, Lishan Lin, Yongchao Li, Yalun Guan, Jinfeng Gao, Dingbang Chen, Yu Zhang, Zhong Pei, Xi Che. Therapeutic reversal of Huntington's disease by in vivo self-assembled siRNAs. Brain : a journal of neurology. vol 144. issue 11. 2021-12-17. PMID:34918046. |
huntington's disease is an autosomal-dominant neurodegenerative disease caused by cag expansion in exon 1 of the huntingtin (htt) gene. |
2021-12-17 |
2023-08-13 |
mouse |
| Adewale Adegbuyiro, Faezeh Sedighi, Pranav Jain, Mark V Pinti, Chathuranga Siriwardhana, John M Hollander, Justin Legleite. Mitochondrial membranes modify mutant huntingtin aggregation. Biochimica et biophysica acta. Biomembranes. vol 1863. issue 10. 2021-11-23. PMID:34089719. |
huntington's disease (hd) is a neurodegenerative disease caused by the expansion of a polyglutamine (polyq) tract near the n-terminus of the huntingtin (htt) protein. |
2021-11-23 |
2023-08-13 |
Not clear |
| b' Jordi Creus-Muncunill, Anna Guisado-Corcoll, Veronica Venturi, Lorena Pantano, Georgia Escaram\\xc3\\xads, Marta Garc\\xc3\\xada de Herreros, Maria Solaguren-Beascoa, Ana G\\xc3\\xa1mez-Valero, Cristina Navarrete, Merc\\xc3\\xa8 Masana, Franc Llorens, Daniela Diaz-Lucena, Esther P\\xc3\\xa9rez-Navarro, Eul\\xc3\\xa0lia Mart\\xc3\\xa. Huntington\'s disease brain-derived small RNAs recapitulate associated neuropathology in mice. Acta neuropathologica. vol 141. issue 4. 2021-11-15. PMID:33547932.' |
progressive motor alterations and selective death of striatal medium spiny neurons (msns) are key pathological hallmarks of huntington's disease (hd), a neurodegenerative condition caused by a cag trinucleotide repeat expansion in the coding region of the huntingtin (htt) gene. |
2021-11-15 |
2023-08-13 |
mouse |
| Bhagyashree S Joshi, Sameh A Youssef, Reinier Bron, Alain de Bruin, Harm H Kampinga, Inge S Zuhor. DNAJB6b-enriched small extracellular vesicles decrease polyglutamine aggregation in iScience. vol 24. issue 11. 2021-11-11. PMID:34755099. |
dnajb6b-enriched small extracellular vesicles decrease polyglutamine aggregation in huntington disease (hd) is a devastating neurodegenerative disorder characterized by aggregation of huntingtin (htt) protein containing expanded polyglutamine (polyq) tracts. |
2021-11-11 |
2023-08-13 |
Not clear |
| Wiebke Frank, Katrin S Lindenberg, Alzbeta Mühlbäck, Jan Lewerenz, G Bernhard Landwehrmeye. [Disease-modifying treatment approaches in Huntington disease : Past and future]. Der Nervenarzt. 2021-11-11. PMID:34762178. |
huntington disease (hd) is the most frequent monogenetic neurodegenerative disease and can be unequivocally diagnosed even in the preclinical stage, at least in all individuals in whom the cag expansion mutation in the huntingtin gene (htt) is in the range of full penetrance. |
2021-11-11 |
2023-08-13 |
Not clear |
| Mandi Gandelman, Warunee Dansithong, Karla P Figueroa, Sharan Paul, Daniel R Scoles, Stefan M Puls. Staufen 1 amplifies proapoptotic activation of the unfolded protein response. Cell death and differentiation. vol 27. issue 10. 2021-11-08. PMID:32415281. |
staufen-1 (stau1) is an rna-binding protein that becomes highly overabundant in numerous neurodegenerative disease models, including those carrying mutations in presenilin1 (psen1), microtubule-associated protein tau (mapt), huntingtin (htt), tar dna-binding protein-43 gene (tardbp), or c9orf72. |
2021-11-08 |
2023-08-13 |
Not clear |
| Sanaz Gabery, Jing Eugene Kwa, Rachel Y Cheong, Barbara Baldo, Costanza Ferrari Bardile, Brendan Tan, Catriona McLean, Nellie Georgiou-Karistianis, Govinda R Poudel, Glenda Halliday, Mahmoud A Pouladi, Åsa Petersé. Early white matter pathology in the fornix of the limbic system in Huntington disease. Acta neuropathologica. vol 142. issue 5. 2021-11-08. PMID:34448021. |
huntington disease (hd) is a fatal neurodegenerative disorder caused by an expanded cag repeat in the huntingtin (htt) gene. |
2021-11-08 |
2023-08-13 |
Not clear |
| R A Hickman, P L Faust, M K Rosenblum, K Marder, M F Mehler, J P Vonsatte. Developmental malformations in Huntington disease: neuropathologic evidence of focal neuronal migration defects in a subset of adult brains. Acta neuropathologica. vol 141. issue 3. 2021-11-05. PMID:33517535. |
neuropathologic hallmarks of huntington disease (hd) include the progressive neurodegeneration of the striatum and the presence of huntingtin (htt) aggregates that result from abnormal polyq expansion of the htt gene. |
2021-11-05 |
2023-08-13 |
human |
| Ashish Raj, Fon Powel. Network model of pathology spread recapitulates neurodegeneration and selective vulnerability in Huntington's Disease. NeuroImage. vol 235. 2021-10-25. PMID:33789134. |
huntington's disease (hd), an autosomal dominant genetic disorder caused by a mutation in the huntingtin gene (htt), displays a stereotyped topography in the human brain and a stereotyped progression, initially appearing in the striatum. |
2021-10-25 |
2023-08-13 |
human |
| Xiang Li, Jianbin Wan. Maintenance of chaperone-mediated autophagy activity in cultured cells expressing mutant huntingtin. Biomedical reports. vol 2. issue 4. 2021-10-21. PMID:24944802. |
huntington's disease (hd) is an autosomal dominant neurodegenerative disease caused by mutant huntingtin (htt) with an expanded polyglutamine tract. |
2021-10-21 |
2023-08-13 |
Not clear |
| Fanny L Lemarié, Nicholas S Caron, Shaun S Sanders, Mandi E Schmidt, Yen T N Nguyen, Seunghyun Ko, Xiaohong Xu, Mahmoud A Pouladi, Dale D O Martin, Michael R Hayde. Rescue of aberrant huntingtin palmitoylation ameliorates mutant huntingtin-induced toxicity. Neurobiology of disease. vol 158. 2021-10-14. PMID:34390831. |
huntington disease (hd) is a neurodegenerative disorder caused by a cag expansion in the htt gene that codes for an elongated polyglutamine tract in the huntingtin (htt) protein. |
2021-10-14 |
2023-08-13 |
mouse |
| Michael Axenhus, Bengt Winblad, Lars O Tjernberg, Sophia Schedin-Weis. Huntingtin Levels are Elevated in Hippocampal Post-Mortem Samples of Alzheimer's Disease Brain. Current Alzheimer research. vol 17. issue 9. 2021-10-11. PMID:33272184. |
we have recently identified huntingtin (htt), the pathogenic protein in huntington's disease, as a mediator of alzheimer's disease (ad) pathology in an amyloid precursor protein (app) knock-in mouse model of ad. |
2021-10-11 |
2023-08-13 |
mouse |
| X Zhao, A Chen, Z Wang, Xiao-Han Xu, Y Ta. Biological functions and potential therapeutic applications of huntingtin-associated protein 1: progress and prospects. Clinical & translational oncology : official publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of Mexico. 2021-09-26. PMID:34564830. |
huntington disease (hd) is a single-gene autosomal dominant inherited neurodegenerative disease caused by a polyglutamine expansion of the protein huntingtin (htt). |
2021-09-26 |
2023-08-13 |
Not clear |
| Assunta Ingannato, Silvia Bagnoli, Salvatore Mazzeo, Valentina Bessi, Sabrina Matà, Monica Del Mastio, Gemma Lombardi, Camilla Ferrari, Sandro Sorbi, Benedetta Nacmia. Neurofilament Light Chain and Intermediate HTT Alleles as Combined Biomarkers in Italian ALS Patients. Frontiers in neuroscience. vol 15. 2021-09-21. PMID:34539331. |
to study the possible implication of the two biomarkers, intermediate alleles (ias) of the huntingtin (htt) gene and neurofilament light chain (nfl) levels in plasma, in amyotrophic lateral sclerosis (als) patients. |
2021-09-21 |
2023-08-13 |
Not clear |
| Anna Pluciennik, Yuhong Liu, Elana Molotsky, Gregory B Marsh, Bedri Ranxhi, Frederick J Arnold, Sophie St-Cyr, Beverly Davidson, Naemeh Pourshafie, Andrew P Lieberman, Wei Gu, Sokol V Todi, Diane E Merr. Deubiquitinase USP7 contributes to the pathogenicity of spinal and bulbar muscular atrophy. The Journal of clinical investigation. vol 131. issue 1. 2021-09-08. PMID:33170804. |
finally, we found that usp7 also differentially interacts with mutant huntingtin (htt) protein in striatum and frontal cortex of a knockin mouse model of huntington's disease. |
2021-09-08 |
2023-08-13 |
mouse |
| Sharon E Groover, Adewale Adegbuyiro, Caleb K Fan, Breanna L Hodges, Maryssa Beasley, Katelyn Taylor, Alyssa R Stonebraker, Chathuranga Siriwardhana, Justin Legleite. Macromolecular crowding in solution alters huntingtin interaction and aggregation at interfaces. Colloids and surfaces. B, Biointerfaces. vol 206. 2021-09-07. PMID:34246856. |
huntington's disease (hd) is a fatal neurodegenerative disease caused by an extended polyglutamine (polyq) domain within the first exon of the huntingtin protein (htt). |
2021-09-07 |
2023-08-13 |
Not clear |
| Jo B Henningsen, Rana Soylu-Kucharz, Maria Björkqvist, Åsa Petersé. Effects of excitotoxicity in the hypothalamus in transgenic mouse models of Huntington disease. Heliyon. vol 7. issue 8. 2021-08-31. PMID:34458633. |
huntington disease (hd) is a fatal neurodegenerative movement disorder caused by an expanded cag repeat in the huntingtin gene (htt). |
2021-08-31 |
2023-08-13 |
mouse |
| Adelė Butėnaitė, Robertas Strumila, Aistė Lengvenytė, Indrė Kotryna Pakutkaitė, Aušra Morkūnienė, Aušra Matulevičienė, Edgaras Dlugauskas, Algirdas Utku. Significant Association Between Huntingtin Gene Mutation and Prevalence of Hopelessness, Depression and Anxiety Symptoms. Acta medica Lituanica. vol 28. issue 1. 2021-08-17. PMID:34393630. |
the aim of the present study was to assess and compare depression, anxiety and hopelessness rates in individuals with and without an abnormal expansion of cag repeats in the huntingtin (htt) gene and healthy controls. |
2021-08-17 |
2023-08-13 |
Not clear |
| Barbara J Bailus, Stephen M Scheeler, Jesse Simons, Maria A Sanchez, Kizito-Tshitoko Tshilenge, Jordi Creus-Muncunill, Swati Naphade, Alejandro Lopez-Ramirez, Ningzhe Zhang, Kuruwitage Lakshika Madushani, Stanislav Moroz, Ashley Loureiro, Katherine H Schreiber, Felix Hausch, Brian K Kennedy, Michelle E Ehrlich, Lisa M Ellerb. Modulating FKBP5/FKBP51 and autophagy lowers HTT (huntingtin) levels. Autophagy. 2021-07-11. PMID:34024231. |
current disease-modifying therapies for huntington disease (hd) focus on lowering mutant htt (huntingtin; mhtt) levels, and the immunosuppressant drug rapamycin is an intriguing therapeutic for aging and neurological disorders. |
2021-07-11 |
2023-08-13 |
mouse |