| Publication |
Sentence |
Publish Date |
Extraction Date |
Species |
| Subrata Pradhan, Keegan Bush, Nan Zhang, Raj K Pandita, Chi-Lin Tsai, Charlene Smith, Devon F Pandlebury, Sagar Gaikwad, Francis Leonard, Linghui Nie, Annie Tao, William Russell, Subo Yuan, Sanjeev Choudhary, Kenneth S Ramos, Cornelis Elferink, Yogesh P Wairkar, John A Tainer, Leslie M Thompson, Tej K Pandita, Partha S Sarka. Chromatin remodeler BRG1 recruits huntingtin to repair DNA double-strand breaks in neurons. bioRxiv : the preprint server for biology. 2024-09-30. PMID:39345557. |
persistent dna double-strand breaks (dsbs) are enigmatically implicated in neurodegenerative diseases including huntington's disease (hd), the inherited late-onset disorder caused by cag repeat elongations in huntingtin (htt). |
2024-09-30 |
2024-10-02 |
Not clear |
| Anirban Chakraborty, Sravan Gopalkrishnashetty Sreenivasmurthy, Wyatt Miller, Weihan Huai, Tapan Biswas, Santi Mohan Mandal, Lisardo Boscá, Balaji Krishnan, Gourisankar Ghosh, Tapas Hazr. Fructose-2,6-bisphosphate restores DNA repair activity of PNKP and ameliorates neurodegenerative symptoms in Huntington's disease. Proceedings of the National Academy of Sciences of the United States of America. vol 121. issue 39. 2024-09-19. PMID:39298485. |
huntington's disease (hd) and spinocerebellar ataxia type 3 (sca3) are the two most prevalent polyglutamine (polyq) neurodegenerative diseases, caused by cag (encoding glutamine) repeat expansion in the coding region of the huntingtin (htt) and ataxin-3 (atxn3) proteins, respectively. |
2024-09-19 |
2024-09-22 |
mouse |
| Anna-Karin Roos, Erica Stenvall, Emmy Skelton Kockum, Kornelia Åman Grönlund, Helena Alstermark, Anna Wuolikainen, Peter M Andersen, Angelica Nordin, Karin M E Forsber. Small striatal huntingtin inclusions in patients with motor neuron disease with reduced penetrance and intermediate HTT gene expansions. Human molecular genetics. 2024-09-13. PMID:39270726. |
small striatal huntingtin inclusions in patients with motor neuron disease with reduced penetrance and intermediate htt gene expansions. |
2024-09-13 |
2024-09-16 |
human |
| Anna-Karin Roos, Erica Stenvall, Emmy Skelton Kockum, Kornelia Åman Grönlund, Helena Alstermark, Anna Wuolikainen, Peter M Andersen, Angelica Nordin, Karin M E Forsber. Small striatal huntingtin inclusions in patients with motor neuron disease with reduced penetrance and intermediate HTT gene expansions. Human molecular genetics. 2024-09-13. PMID:39270726. |
upon autopsy of three patients with intermediate or reduced penetrance htt alleles, huntingtin inclusions were observed in the caudate nucleus and frontal lobe, but no significant somatic mosaicism was detected in different parts of the nervous system. |
2024-09-13 |
2024-09-16 |
human |
| Ankita Deo, Rishita Ghosh, Snehal Ahire, Sayali Marathe, Amitabha Majumdar, Tania Bos. Two novel DnaJ chaperone proteins CG5001 and P58IPK regulate the pathogenicity of Huntington's disease related aggregates. Scientific reports. vol 14. issue 1. 2024-09-06. PMID:39242711. |
huntington's disease (hd) is a rare neurodegenerative disease caused due to aggregation of huntingtin (htt) protein. |
2024-09-06 |
2024-09-10 |
drosophila_melanogaster |
| Marina Sogorb-Gonzalez, Christian Landles, Nicholas S Caron, Anouk Stam, Georgina Osborne, Michael R Hayden, David Howland, Sander van Deventer, Gillian P Bates, Astrid Vallès, Melvin Ever. Exon 1-targeting miRNA reduces the pathogenic exon 1 HTT protein in Huntington disease models. Brain : a journal of neurology. 2024-08-18. PMID:39155061. |
huntington disease (hd) is a fatal neurodegenerative disease caused by a trinucleotide repeat expansion in exon 1 of the huntingtin gene (htt) resulting in toxic gain-of-function and cell death. |
2024-08-18 |
2024-08-21 |
mouse |
| Mohit Neema, Jordan L Schultz, Douglas R Langbehn, Amy L Conrad, Eric A Epping, Vincent A Magnotta, Peggy C Nopoulo. Mutant Huntingtin Drives Development of an Advantageous Brain Early in Life: Evidence in Support of Antagonistic Pleiotropy. Annals of neurology. 2024-08-08. PMID:39115048. |
huntington's disease (hd) is a neurodegenerative disease caused by a triplet repeat expansion within the gene huntingtin (htt). |
2024-08-08 |
2024-08-10 |
Not clear |
| Harman Sharma, Sushma Koirala, Yee Lian Chew, Anna Konopk. DNA Damage and Chromatin Rearrangement Work Together to Promote Neurodegeneration. Molecular neurobiology. 2024-07-08. PMID:38977621. |
this review focused on hallmark proteins involved in various neurodegenerative diseases, such as the microtubule-associated protein tau, tar dna/rna binding protein 43 (tdp-43), superoxide dismutase 1 (sod1), fused in sarcoma (fus), huntingtin (htt), α-synuclein, and β-amyloid precursor protein (app). |
2024-07-08 |
2024-07-12 |
Not clear |
| Ross Ferguson, Robert Goold, Lucy Coupland, Michael Flower, Sarah J Tabriz. Therapeutic validation of MMR-associated genetic modifiers in a human ex vivo model of Huntington disease. American journal of human genetics. 2024-05-15. PMID:38749429. |
the pathological huntingtin (htt) trinucleotide repeat underlying huntington disease (hd) continues to expand throughout life. |
2024-05-15 |
2024-05-27 |
human |
| Laiqiang Chen, Yiyang Qin, Tingting Guo, Wenzhen Zhu, Jingpan Lin, Tingting Xing, Xuezhi Duan, Yiran Zhang, Eshu Ruan, Xiang Li, Peng Yin, Shihua Li, Xiao-Jiang Li, Su Yan. HAP40 modulates mutant Huntingtin aggregation and toxicity in Huntington's disease mice. Cell death & disease. vol 15. issue 5. 2024-05-14. PMID:38744826. |
huntington's disease (hd) is a monogenic neurodegenerative disease, caused by the cag trinucleotide repeat expansion in exon 1 of the huntingtin (htt) gene. |
2024-05-14 |
2024-05-27 |
mouse |
| Javier Ojalvo-Pacheco, Sokhna M S Yakhine-Diop, José M Fuentes, Marta Paredes-Barquero, Mireia Niso-Santan. Role of TFEB in Huntington's Disease. Biology. vol 13. issue 4. 2024-04-26. PMID:38666850. |
huntington's disease (hd) is an autosomal dominant neurodegenerative disease caused by an expansion of the cag trinucleotide repeat in exon 1 of the huntingtin (htt) gene. |
2024-04-26 |
2024-04-28 |
Not clear |
| Adam Skeens, Chathuranga Siriwardhana, Sophia E Massinople, Michelle M Wunder, Zachary L Ellis, Kaitlyn M Keith, Tyler Girman, Shelli L Frey, Justin Legleite. The polyglutamine domain is the primary driver of seeding in huntingtin aggregation. PloS one. vol 19. issue 3. 2024-03-14. PMID:38483973. |
huntington's disease (hd) is a fatal, neurodegenerative disease caused by aggregation of the huntingtin protein (htt) with an expanded polyglutamine (polyq) domain into amyloid fibrils. |
2024-03-14 |
2024-03-17 |
caenorhabditis_elegans |
| Franziska Hoschek, Julia Natan, Maximilian Wagner, Kirupa Sathasivam, Alshaimaa Abdelmoez, Björn von Einem, Gillian P Bates, G Bernhard Landwehrmeyer, Andreas Neuede. Huntingtin HTT1a is generated in a CAG repeat-length-dependent manner in human tissues. Molecular medicine (Cambridge, Mass.). vol 30. issue 1. 2024-03-08. PMID:38459427. |
the disease-causing mutation in huntington disease (hd) is a cag trinucleotide expansion in the huntingtin (htt) gene. |
2024-03-08 |
2024-03-11 |
mouse |
| Karin Dalene Skarping, Larissa Arning, Åsa Petersén, Huu Phuc Nguyen, Samuel Gebre-Medhi. Attenuated huntingtin gene CAG nucleotide repeat size in individuals with Lynch syndrome. Scientific reports. vol 14. issue 1. 2024-02-21. PMID:38383663. |
dna mismatch repair (mmr) is thought to contribute to the onset and progression of huntington disease (hd) by promoting somatic expansion of the pathogenic cag nucleotide repeat in the huntingtin gene (htt). |
2024-02-21 |
2024-02-24 |
Not clear |
| Junjiao Wu, Jie Ren, Hongfei Cui, Yali Xie, Yu Tan. Rapid and high-purity differentiation of human medium spiny neurons reveals LMNB1 hypofunction and subtype necessity in modeling Huntington's disease. Inflammation and regeneration. vol 44. issue 1. 2024-02-15. PMID:38360694. |
the huntingtin (htt) mutation involves an expanded cag repeat, leading to insoluble polyq, which renders gaba |
2024-02-15 |
2024-02-18 |
human |
| Swamynathan Sowndharya, Koilmani Emmanuvel Raja. Environmental enrichment improves social isolation-induced memory impairment: The possible role of ITSN1-Reelin-AMPA receptor signaling pathway. PloS one. vol 19. issue 1. 2024-01-19. PMID:38241230. |
increased level of intersectin 1 (itsn1), huntingtin (htt), synaptotagmin -iv (syt4), variants of brain-derived neurotrophic factor (bdnf - iii), α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (ampa) receptor (glur1) expression, and decreased variants of bdnf (iv), bdnf, reelin, apolipoprotein e receptor 2 (apoer2), very low-density lipoprotein receptor (vldlr), src family tyrosine kinase (sfks), disabled protein (dab)-1, protein kinase b (pkb/akt), glur2, mitogen-activated protein kinase (mapk) and extracellular signal-regulated kinase (erk1/2) expression. |
2024-01-19 |
2024-01-22 |
mouse |
| Francesco D'Egidio, Vanessa Castelli, Giorgia Lombardozzi, Fabrizio Ammannito, Annamaria Cimini, Michele d'Angel. Therapeutic advances in neural regeneration for Huntington's disease. Neural regeneration research. vol 19. issue 9. 2024-01-16. PMID:38227527. |
huntington's disease is a neurodegenerative disease caused by the expansion mutation of a cytosine-adenine-guanine triplet in the exon 1 of the htt gene which is responsible for the production of the huntingtin (htt) protein. |
2024-01-16 |
2024-01-18 |
Not clear |
| Nicholas S Caron, Amirah E-E Aly, Hailey Findlay Black, Dale D O Martin, Mandi E Schmidt, Seunghyun Ko, Christine Anderson, Emily M Harvey, Lorenzo L Casal, Lisa M Anderson, Seyed M R Rahavi, Gregor S D Reid, Michael N Oda, Danica Stanimirovic, Abedelnasser Abulrob, Jodi L McBride, Blair R Leavitt, Michael R Hayde. Systemic delivery of mutant huntingtin lowering antisense oligonucleotides to the brain using apolipoprotein A-I nanodisks for Huntington disease. Journal of controlled release : official journal of the Controlled Release Society. 2024-01-12. PMID:38215984. |
huntington disease (hd) is a dominantly inherited neurodegenerative disorder caused by a cag trinucleotide expansion mutation in the htt gene which codes for a toxic mutant huntingtin (mhtt) protein. |
2024-01-12 |
2024-01-15 |
mouse |
| Elsa C Kuijper, Maurice Overzier, Ernst Suidgeest, Oleh Dzyubachyk, Cecile Maguin, Jean-Baptiste Pérot, Julien Flament, Yavuz Ariyurek, Hailiang Mei, Ronald A M Buijsen, Louise van der Weerd, Willeke van Roon-Mo. Antisense oligonucleotide-mediated disruption of HTT caspase-6 cleavage site ameliorates the phenotype of YAC128 Huntington disease mice. Neurobiology of disease. 2023-12-01. PMID:38040383. |
in huntington disease, cellular toxicity is particularly caused by toxic protein fragments generated from the mutant huntingtin (htt) protein. |
2023-12-01 |
2023-12-10 |
mouse |
| Himanshi Gupta, Shakti Sah. High-throughput virtual screening of potential inhibitors of GPR52 using docking and biased sampling method for Huntington's disease therapy. Molecular diversity. 2023-12-01. PMID:38038795. |
huntington's disease (hd) is a rare and progressive neurodegenerative disorder caused by polyglutamine (poly-q) mutations of the huntingtin (htt) gene resulting in chorea, cognitive, and psychiatric dysfunctions. |
2023-12-01 |
2023-12-10 |
Not clear |