All Relations between Huntington Disease and huntingtin

Publication Sentence Publish Date Extraction Date Species
Manish Sharma, Srinivasa Subramania. Rhes travels from cell to cell and transports Huntington disease protein via TNT-like protrusion. The Journal of cell biology. vol 218. issue 6. 2020-04-09. PMID:31076452. here, we report, rhes, a brain-enriched gtpase/sumo e3-like protein, induces the biogenesis of tnt-like cellular protrusions, "rhes tunnels," through which rhes moves from cell to cell and transports huntington disease (hd) protein, the poly-q expanded mutant huntingtin (mhtt). 2020-04-09 2023-08-13 Not clear
Sara Sameni, Run Zhang, Michelle A Digma. The Phasor FLIM Method Reveals a Link Between a Change in Energy Metabolism and mHtt protein spread in Healthy Mammalian Cells when Co-cultured with Huntington Diseased Cells. Methods and applications in fluorescence. 2020-04-04. PMID:32235053. huntington disease (hd) is a late-onset autosomal neurodegenerative disease characterized by the aggregations of mutant huntingtin proteins (mhtt). 2020-04-04 2023-08-13 Not clear
Amy I Smith-Dijak, Marja D Sepers, Lynn A Raymon. Alterations in synaptic function and plasticity in Huntington disease. Journal of neurochemistry. vol 150. issue 4. 2020-03-18. PMID:31095731. huntington disease (hd) is an inherited neurodegenerative disorder caused by an expansion of the cag repeat region in the first exon of the huntingtin gene. 2020-03-18 2023-08-13 Not clear
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. potent and sustained huntingtin lowering via aav5 encoding mirna preserves striatal volume and cognitive function in a humanized mouse model of huntington disease. 2020-03-18 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
Raúl Orozco-Díaz, Angélica Sánchez-Álvarez, José Manuel Hernández-Hernández, José Tapia-Ramíre. The interaction between RE1-silencing transcription factor (REST) and heat shock protein 90 as new therapeutic target against Huntington's disease. PloS one. vol 14. issue 7. 2020-03-10. PMID:31361762. discovering mechanisms to reduce the cellular levels of mutant huntingtin and rest provide promising strategies for treating huntington disease. 2020-03-10 2023-08-13 Not clear
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. a human huntingtin snp alters post-translational modification and pathogenic proteolysis of the protein causing huntington disease. 2019-10-07 2023-08-13 human
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
Jun Wu, Daniel Ryskamp, Lutz Birnbaumer, Ilya Bezprozvann. Inhibition of TRPC1-Dependent Store-Operated Calcium Entry Improves Synaptic Stability and Motor Performance in a Mouse Model of Huntington's Disease. Journal of Huntington's disease. vol 7. issue 1. 2019-10-02. PMID:29480205. huntington disease (hd) is a dominantly inherited neurodegenerative disorder caused by a cag repeat expansion in the huntingtin gene. 2019-10-02 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
Elisabeth Singer, Carolin Walter, Jonasz J Weber, Ann-Christin Krahl, Ulrike A Mau-Holzmann, Nadine Rischert, Olaf Riess, Laura E Clemensson, Huu P Nguye. Reduced cell size, chromosomal aberration and altered proliferation rates are characteristics and confounding factors in the STHdh cell model of Huntington disease. Scientific reports. vol 7. issue 1. 2019-07-10. PMID:29203806. huntington disease is a fatal neurodegenerative disorder caused by a cag repeat expansion in the gene encoding the huntingtin protein. 2019-07-10 2023-08-13 mouse
Elisabeth Singer, Carolin Walter, Jonasz J Weber, Ann-Christin Krahl, Ulrike A Mau-Holzmann, Nadine Rischert, Olaf Riess, Laura E Clemensson, Huu P Nguye. Reduced cell size, chromosomal aberration and altered proliferation rates are characteristics and confounding factors in the STHdh cell model of Huntington disease. Scientific reports. vol 7. issue 1. 2019-07-10. PMID:29203806. a well-known and commonly used model to study molecular aspects of huntington disease are the striatum-derived sthdh cell lines generated from wild type and huntingtin knock-in mouse embryos. 2019-07-10 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
Willeke M C van Roon-Mom, Raymund A C Roos, Susanne T de Bo. Dose-Dependent Lowering of Mutant Huntingtin Using Antisense Oligonucleotides in Huntington Disease Patients. Nucleic acid therapeutics. vol 28. issue 2. 2019-07-05. PMID:29620999. dose-dependent lowering of mutant huntingtin using antisense oligonucleotides in huntington disease patients. 2019-07-05 2023-08-13 Not clear
Willeke M C van Roon-Mom, Raymund A C Roos, Susanne T de Bo. Dose-Dependent Lowering of Mutant Huntingtin Using Antisense Oligonucleotides in Huntington Disease Patients. Nucleic acid therapeutics. vol 28. issue 2. 2019-07-05. PMID:29620999. on december 11 of 2017, ionis pharmaceuticals published a press release announcing dose-dependent reductions of mutant huntingtin protein in their httrx phase 1/2a study in huntington disease (hd) patients. 2019-07-05 2023-08-13 Not clear
Mandi E Schmidt, Caodu Buren, James P Mackay, Daphne Cheung, Louisa Dal Cengio, Lynn A Raymond, Michael R Hayde. Altering cortical input unmasks synaptic phenotypes in the YAC128 cortico-striatal co-culture model of Huntington disease. BMC biology. vol 16. issue 1. 2019-05-13. PMID:29945611. huntington disease (hd) is a fatal neurodegenerative disorder caused by a cag expansion in the huntingtin (htt) gene, leading to selective and progressive neuronal death predominantly in the striatum. 2019-05-13 2023-08-13 mouse