Works matching DE "POLYGLUTAMINE"

Results: 446

Small Heat Shock Proteins Potentiate Amyloid Dissolution by Protein Disaggregases from Yeast and Humans.

Published in:

PLoS Biology, 2012, v. 10, n. 6, p. 1, doi. 10.1371/journal.pbio.1001346

By:

Duennwald, Martin L.;
Echeverria, AnaLisa;
Shorter, James

Publication type:

Article

Dissection and Design of Yeast Prions.

Published in:

PLoS Biology, 2004, v. 2, n. 3, p. 1, doi. 10.1371/journal.pbio.0020086

By:

Osherovich, Lev Z;
Cox, Brian S;
Tuite, Mick F;
Weissman, Jonathan S

Publication type:

Article

Combination of stem cell and gene therapy ameliorates symptoms in Huntington's disease mice.

Published in:

NPJ Regenerative Medicine, 2019, v. 4, n. 1, p. N.PAG, doi. 10.1038/s41536-019-0066-7

By:

Cho, In Ki;
Hunter, Carissa Emerson;
Ye, Sarah;
Pongos, Alvince Learnz;
Chan, Anthony Wing Sang

Publication type:

Article

Hyperbaric Oxygen Therapy Attenuated the Motor Coordination and Cognitive Impairment of Polyglutamine Spinocerebellar Ataxia SCA17 Mice.

Published in:

Cerebellum, 2024, v. 23, n. 2, p. 401, doi. 10.1007/s12311-023-01548-y

By:

Chiang, Meng-Ke;
Lin, Ta-Chun;
Lin, Keng-Hsuan;
Chang, Ya-Chin;
Hsieh-Li, Hsiu Mei;
Lai, Dar-Ming

Publication type:

Article

Targeting the <italic>CACNA1A</italic> IRES as a Treatment for Spinocerebellar Ataxia Type 6.

Published in:

Cerebellum, 2018, v. 17, n. 1, p. 72, doi. 10.1007/s12311-018-0917-6

By:

Pastor, Parviz Daniel Hejazi;
Du, Xiaofei;
Fazal, Sarah;
Davies, Andre N.;
Gomez, Christopher M.

Publication type:

Article

Mutant Ataxin-1 Inhibits Neural Progenitor Cell Proliferation in SCA1.

Published in:

Cerebellum, 2017, v. 16, n. 2, p. 340, doi. 10.1007/s12311-016-0794-9

By:

Cvetanovic, Marija;
Hu, Yuan-Shih;
Opal, Puneet

Publication type:

Article

Focused Cerebellar Laser Light Induced Hyperthermia Improves Symptoms and Pathology of Polyglutamine Disease SCA1 in a Mouse Model.

Published in:

Cerebellum, 2014, v. 13, n. 5, p. 596, doi. 10.1007/s12311-014-0576-1

By:

Hearst, Scoty;
Shao, Qingmei;
Lopez, Mariper;
Raucher, Drazen;
Vig, Parminder

Publication type:

Article

Mesenchymal Stem Cells Ameliorate Cerebellar Pathology in a Mouse Model of Spinocerebellar Ataxia Type 1.

Published in:

Cerebellum, 2014, v. 13, n. 3, p. 323, doi. 10.1007/s12311-013-0536-1

By:

Matsuura, Serina;
Shuvaev, Anton;
Iizuka, Akira;
Nakamura, Kazuhiro;
Hirai, Hirokazu

Publication type:

Article

Mutant Ataxin-3 with an Abnormally Expanded Polyglutamine Chain Disrupts Dendritic Development and Metabotropic Glutamate Receptor Signaling in Mouse Cerebellar Purkinje Cells.

Published in:

Cerebellum, 2014, v. 13, n. 1, p. 29, doi. 10.1007/s12311-013-0516-5

By:

Konno, Ayumu;
Shuvaev, Anton;
Miyake, Noriko;
Miyake, Koichi;
Iizuka, Akira;
Matsuura, Serina;
Huda, Fathul;
Nakamura, Kazuhiro;
Yanagi, Shigeru;
Shimada, Takashi;
Hirai, Hirokazu

Publication type:

Article

Lithium Chloride Alleviates Neurodegeneration Partly by Inhibiting Activity of GSK3β in a SCA3 Drosophila Model.

Published in:

Cerebellum, 2013, v. 12, n. 6, p. 892, doi. 10.1007/s12311-013-0498-3

By:

Jia, Dan-Dan;
Zhang, Li;
Chen, Zhao;
Wang, Chun-Rong;
Huang, Feng-Zhen;
Duan, Ran-Hui;
Xia, Kun;
Tang, Bei-Sha;
Jiang, Hong

Publication type:

Article

Altered Expression of Carbonic Anhydrase-Related Protein XI in Neuronal Cells Expressing Mutant Ataxin-3.

Published in:

Cerebellum, 2013, v. 12, n. 3, p. 338, doi. 10.1007/s12311-012-0430-2

By:

Hsieh, Mingli;
Chang, Wei-Hsiu;
Hsu, Chi-Fu;
Nishimori, Isao;
Kuo, Cheng-Liang;
Minakuchi, Tomoko

Publication type:

Article

Spinocerebellar Ataxia Type 7: Clinical Course, Phenotype-Genotype Correlations, and Neuropathology.

Published in:

Cerebellum, 2013, v. 12, n. 2, p. 176, doi. 10.1007/s12311-012-0412-4

By:

Horton, Laura;
Frosch, Matthew;
Vangel, Mark;
Weigel-DiFranco, Carol;
Berson, Eliot;
Schmahmann, Jeremy

Publication type:

Article

Deranged Calcium Signaling in Purkinje Cells and Pathogenesis in Spinocerebellar Ataxia 2 (SCA2) and Other Ataxias.

Published in:

Cerebellum, 2012, v. 11, n. 3, p. 630, doi. 10.1007/s12311-010-0182-9

By:

Kasumu, Adebimpe;
Bezprozvanny, Ilya

Publication type:

Article

Pathoanatomy of Cerebellar Degeneration in Spinocerebellar Ataxia Type 2 (SCA2) and Type 3 (SCA3).

Published in:

Cerebellum, 2012, v. 11, n. 3, p. 749, doi. 10.1007/s12311-011-0340-8

By:

Scherzed, W.;
Brunt, E.;
Heinsen, H.;
Vos, R.;
Seidel, K.;
Bürk, K.;
Schöls, L.;
Auburger, G.;
Turco, D.;
Deller, T.;
Korf, H.;
Dunnen, W.;
Rüb, U.

Publication type:

Article

Body Mass Index is Inversely Correlated with the Expanded CAG Repeat Length in SCA3/MJD Patients.

Published in:

Cerebellum, 2012, v. 11, n. 3, p. 771, doi. 10.1007/s12311-011-0326-6

By:

Saute, Jonas;
Silva, Andrew;
Souza, Gabriele;
Russo, Aline;
Donis, Karina;
Vedolin, Leonardo;
Saraiva-Pereira, Maria;
Portela, Luis;
Jardim, Laura

Publication type:

Article

Base editing strategies to convert CAG to CAA diminish the disease-causing mutation in Huntington’s disease.

Published in:

eLife, 2024, p. 1, doi. 10.7554/eLife.89782

By:

Doo Eun Choi;
Jun Wan Shin;
Zeng, Sophia;
Hong, Eun Pyo;
Jae-Hyun Jang;
Loupe, Jacob M.;
Wheeler, Vanessa C.;
Stutzman, Hannah E.;
Kleinstiver, Ben;
Jong-Min Lee

Publication type:

Article

Pathologic polyglutamine aggregation begins with a self-poisoning polymer crystal.

Published in:

eLife, 2023, p. 1, doi. 10.7554/eLife.86939

By:

Kandola, Tej;
Venkatesan, Shriram;
Jiahui Zhang;
Lerbakken, Brooklyn T.;
Von Schulze, Alex;
Blanck, Jillian F.;
Jianzheng Wu;
Unruh, Jay R.;
Berry, Paula;
Lange, Jeffrey J.;
Box, Andrew C.;
Cook, Malcolm;
Sagui, Celeste;
Halfmann, Randal

Publication type:

Article

Ubiquitin ligase ITCH recruitment suppresses the aggregation and cellular toxicity of cytoplasmic misfolded proteins.

Published in:

Scientific Reports, 2014, p. 1, doi. 10.1038/srep05077

By:

Chhangani, Deepak;
Upadhyay, Arun;
Amanullah, Ayeman;
Joshi, Vibhuti;
Mishra, Amit

Publication type:

Article

Functional genomics in Drosophila models of human disease.

Published in:

Briefings in Functional Genomics, 2012, v. 11, n. 5, p. 405, doi. 10.1093/bfgp/els038

By:

Chen, Ko-Fan;
Crowther, Damian C.

Publication type:

Article

Diagnosis of polyglutamine spinocerebellar ataxias by polymerase chain reaction amplification and Sanger sequencing.

Published in:

Molecular Medicine Reports, 2018, v. 18, n. 1, p. 1037, doi. 10.3892/mmr.2018.9043

By:

Chen, Changqiang;
Fang, Xuqian;
Sun, Shunchang

Publication type:

Article

Contactin-associated protein-like 2 expression in SH-SY5Y cells is upregulated by a FOXP2 mutant with a shortened poly-glutamine tract.

Published in:

Molecular Medicine Reports, 2015, v. 12, n. 6, p. 8162, doi. 10.3892/mmr.2015.4483

By:

YUNJING ZHAO;
XIAOLIANG LIU;
HONGWEI SUN;
YUEPING WANG;
WENZHU YANG;
HONGWEI MA

Publication type:

Article

Development of mAb-based polyglutamine-dependent and polyglutamine length-independent huntingtin quantification assays with cross-site validation.

Published in:

PLoS ONE, 2022, v. 17, n. 4, p. 1, doi. 10.1371/journal.pone.0266812

By:

Fischer, David F.;
Dijkstra, Sipke;
Lo, Kimberly;
Suijker, Johnny;
Correia, Ana C. P.;
Naud, Patricia;
Poirier, Martin;
Tessari, Michela A.;
Boogaard, Ivette;
Flynn, Geraldine;
Visser, Mijke;
Lamers, Marieke B. A. C.;
McAllister, George;
Munoz-Sanjuan, Ignacio;
Macdonald, Douglas

Publication type:

Article

Protein Aggregation Inhibitors as Disease-Modifying Therapies for Polyglutamine Diseases.

Published in:

Frontiers in Neuroscience, 2021, v. 14, p. N.PAG, doi. 10.3389/fnins.2021.621996

By:

Minakawa, Eiko N.;
Nagai, Yoshitaka

Publication type:

Article

Ophthalmic Manifestations and Genetics of the Polyglutamine Autosomal Dominant Spinocerebellar Ataxias: A Review.

Published in:

Frontiers in Neuroscience, 2020, v. 14, p. N.PAG, doi. 10.3389/fnins.2020.00892

By:

Park, Jun Young;
Joo, Kwangsic;
Woo, Se Joon

Publication type:

Article

Gene Deregulation and Underlying Mechanisms in Spinocerebellar Ataxias With Polyglutamine Expansion.

Published in:

Frontiers in Neuroscience, 2020, v. 14, p. 1, doi. 10.3389/fnins.2020.00571

By:

Niewiadomska-Cimicka, Anna;
Hache, Antoine;
Trottier, Yvon

Publication type:

Article

The Mechanisms of Nuclear Proteotoxicity in Polyglutamine Spinocerebellar Ataxias.

Published in:

Frontiers in Neuroscience, 2020, v. 14, p. 1, doi. 10.3389/fnins.2020.00489

By:

Lee, Davin;
Lee, Yun-Il;
Lee, Young-Sam;
Lee, Sung Bae

Publication type:

Article

Synergistic Toxicity of Polyglutamine-Expanded TATA-Binding Protein in Glia and Neuronal Cells: Therapeutic Implications for Spinocerebellar Ataxia 17.

Published in:

Journal of Neuroscience, 2017, v. 37, n. 38, p. 9101, doi. 10.1523/JNEUROSCI.0111-17.2017

By:

Yang Yang;
®Su Yang;
Jifeng Guo;
Yiting Cui;
Beisha Tang;
Xiao-Jiang Li;
Shihua Li

Publication type:

Article

Reduced Expression of Foxp1 as a Contributing Factor in Huntington's Disease.

Published in:

Journal of Neuroscience, 2017, v. 37, n. 27, p. 6575, doi. 10.1523/JNEUROSCI.3612-16.2017

By:

Titus, Anto Sam Crosslee Louis Sam;
Yusuff, Tanzeen;
Cassar, Marlène;
Thomas, Elizabeth;
Kretzschmar, Doris;
D'Mello, Santosh R.

Publication type:

Article

Pathogenic Huntington Alters BMP Signaling and Synaptic Growth through Local Disruptions of Endosomal Compartments.

Published in:

Journal of Neuroscience, 2017, v. 37, n. 12, p. 3425, doi. 10.1523/JNEUROSCI.2752-16.2017

By:

Akbergenova, Yulia;
Littleton, J. Troy

Publication type:

Article

Compartment-Dependent Degradation of Mutant Huntingtin Accounts for Its Preferential Accumulation in Neuronal Processes.

Published in:

Journal of Neuroscience, 2016, v. 36, n. 32, p. 8317, doi. 10.1523/JNEUROSCI.0806-16.2016

By:

Ting Zhao;
Yan Hong;
Shihua Li;
Xiao-Jiang Li

Publication type:

Article

Spinocerebellar Ataxia Type 6 Protein Aggregates Cause Deficits in Motor Learning and Cerebellar Plasticity.

Published in:

Journal of Neuroscience, 2015, v. 35, n. 23, p. 8882, doi. 10.1523/JNEUROSCI.0891-15.2015

By:

Mark, Melanie D.;
Krause, Martin;
Boele, Henk-Jan;
Kruse, Wolfgang;
Pollok, Stefan;
Kuner, Thomas;
Dalkara, Deniz;
Koekkoek, Sebastiaan;
De Zeeuw, Chris I.;
Herlitze, Stefan

Publication type:

Article

Differential Effects of Delayed Aging on Phenotype and Striatal Pathology in a Murine Model of Huntington Disease.

Published in:

Journal of Neuroscience, 2014, v. 34, n. 47, p. 15658, doi. 10.1523/JNEUROSCI.1830-14.2014

By:

Tallaksen-Greene, Sara J.;
Sadagurski, Marianna;
Zeng, Li;
Mauch, Roseanne;
Perkins, Matthew;
Banduseela, Varuna C.;
Lieberman, Andrew P.;
Miller, Richard A.;
Paulson, Henry L.;
Albin, Roger L.

Publication type:

Article

Mutant Huntingtin Affects Cortical Progenitor Cell Division and Development of the Mouse Neocortex.

Published in:

Journal of Neuroscience, 2014, v. 34, n. 30, p. 10034, doi. 10.1523/JNEUROSCI.0715-14.2014

By:

Molina-Calavita, Maria;
Barnat, Monia;
Elias, Salah;
Aparicio, Esther;
Piel, Matthieu;
Humbert, Sandrine

Publication type:

Article

HAP1 helps to regulate actin-based transport of insulin-containing granules in pancreatic β cells.

Published in:

Histochemistry & Cell Biology, 2015, v. 144, n. 1, p. 39, doi. 10.1007/s00418-015-1311-9

By:

Wang, Zhiyong;
Peng, Ting;
Wu, Hongnian;
He, Jun;
Li, He

Publication type:

Article

Molecular Pathogenesis in Huntington’s Disease.

Published in:

Biochemistry (00062979), 2018, v. 83, n. 9, p. 1030, doi. 10.1134/S0006297918090043

By:

Illarioshkin, S. N.;
Klyushnikov, S. A.;
Vigont, V. A.;
Seliverstov, Yu. A.;
Kaznacheyeva, E. V.

Publication type:

Article

Expansion of the polyQ repeats in THAP11 forms intranuclear aggregation and causes cell G0/G1 arrest.

Published in:

Cell Biology International, 2014, v. 38, n. 6, p. 757, doi. 10.1002/cbin.10255

By:

Yin, Rong‐Hua;
Li, Yang;
Yang, Fan;
Zhan, Yi‐Qun;
Yu, Miao;
Ge, Chang‐Hui;
Xu, Wang‐Xiang;
Tang, Liu‐Jun;
Wang, Xiao‐Hui;
Chen, Biao;
Yang, Yang;
Li, Jian‐Jie;
Li, Chang‐Yan;
Yang, Xiao‐Ming

Publication type:

Article

The composition of the polyglutamine-containing proteins influences their co-aggregation properties.

Published in:

Cell Biology International, 2010, v. 34, n. 9, p. 933, doi. 10.1042/CBI20090474

By:

Bąk, Daniel;
Milewski, Michał

Publication type:

Article

Peripheral markers of autophagy in polyglutamine diseases.

Published in:

2018

By:

Puorro, Giorgia;
Marsili, Angela;
Sapone, Francesca;
Pane, Chiara;
De Rosa, Anna;
Peluso, Silvio;
De Michele, Giuseppe;
Filla, Alessandro;
Saccà, Francesco

Publication type:

journal article

Selection and characterization of llama single domain antibodies against N-terminal huntingtin.

Published in:

Neurological Sciences, 2015, v. 36, n. 3, p. 429, doi. 10.1007/s10072-014-1971-6

By:

Schut, Menno;
Pepers, Barry;
Klooster, Rinse;
Maarel, Silvère;
Khatabi, Mohamed;
Verrips, Theo;
Dunnen, Johan;
Ommen, Gert-Jan;
Roon-Mom, Willeke

Publication type:

Article

Kynurenine 3-Monooxygenase Interacts with Huntingtin at the Outer Mitochondrial Membrane.

Published in:

Biomedicines, 2022, v. 10, n. 9, p. 2294, doi. 10.3390/biomedicines10092294

By:

Swaih, Aisha M.;
Breda, Carlo;
Sathyasaikumar, Korrapati V.;
Allcock, Natalie;
Collier, Mary E. W.;
Mason, Robert P.;
Feasby, Adam;
Herrera, Federico;
Outeiro, Tiago F.;
Schwarcz, Robert;
Repici, Mariaelena;
Giorgini, Flaviano

Publication type:

Article

Polyglutamine Ataxias: Our Current Molecular Understanding and What the Future Holds for Antisense Therapies.

Published in:

Biomedicines, 2021, v. 9, n. 11, p. 1499, doi. 10.3390/biomedicines9111499

By:

McIntosh, Craig S.;
Li, Dunhui;
Wilton, Steve D.;
Aung-Htut, May T.

Publication type:

Article

The neostriatum in polyglutamine diseases: preferential decreases in large neurons in dentatorubral‐pallidoluysian atrophy and Machado‐Joseph disease and in small neurons in Huntington disease.

Published in:

Neuropathology, 2022, v. 42, n. 4, p. 274, doi. 10.1111/neup.12811

By:

Oyanagi, Kiyomitsu;
Shimizu, Hiroshi;
Yamada, Mitsunori;
Kakita, Akiyoshi

Publication type:

Article

Expanded polyglutamine impairs normal nuclear distribution of fused in sarcoma and poly (rC)‐binding protein 1 in Huntington's disease.

Published in:

Neuropathology, 2019, v. 39, n. 5, p. 358, doi. 10.1111/neup.12600

By:

Mori, Shinichiro;
Honda, Hiroyuki;
Ishii, Takashi;
Yoshimura, Motoi;
Sasagasako, Naokazu;
Suzuki, Satoshi O.;
Taniwaki, Takayuki;
Iwaki, Toru

Publication type:

Article

Dentatorubral-pallidoluysian atrophy (DRPLA) with a small ganglioglioma component containing neurofibrillary tangles and polyglutamine aggregation.

Published in:

Neuropathology, 2017, v. 37, n. 4, p. 335, doi. 10.1111/neup.12365

By:

Yamada, Seiji;
Yamazaki, Tatsuya;
Nakata, Satoshi;
Nobusawa, Sumihito;
Ikota, Hayato;
Ide, Munenori;
Mizushima, Kazuyuki;
Harigaya, Yasuo;
Hirato, Junko;
Yokoo, Hideaki

Publication type:

Article

G protein-coupled receptor 26 immunoreactivity in intranuclear inclusions associated with polyglutamine and intranuclear inclusion body diseases.

Published in:

Neuropathology, 2016, v. 36, n. 1, p. 50, doi. 10.1111/neup.12237

By:

Mori, Fumiaki;
Tanji, Kunikazu;
Miki, Yasuo;
Toyoshima, Yasuko;
Yoshida, Mari;
Kakita, Akiyoshi;
Takahashi, Hitoshi;
Utsumi, Jun;
Sasaki, Hidenao;
Wakabayashi, Koichi

Publication type:

Article

Accumulation of the sigma-1 receptor is common to neuronal nuclear inclusions in various neurodegenerative diseases.

Published in:

Neuropathology, 2014, v. 34, n. 2, p. 148, doi. 10.1111/neup.12080

By:

Miki, Yasuo;
Mori, Fumiaki;
Kon, Tomoya;
Tanji, Kunikazu;
Toyoshima, Yasuko;
Yoshida, Mari;
Sasaki, Hidenao;
Kakita, Akiyoshi;
Takahashi, Hitoshi;
Wakabayashi, Koichi

Publication type:

Article

TDP-43 pathology in polyglutamine diseases: With reference to amyotrphic lateral sclerosis.

Published in:

Neuropathology, 2014, v. 34, n. 1, p. 77, doi. 10.1111/neup.12053

By:

Toyoshima, Yasuko;
Takahashi, Hitoshi

Publication type:

Article

ALS-associated protein FIG4 is localized in Pick and Lewy bodies, and also neuronal nuclear inclusions, in polyglutamine and intranuclear inclusion body diseases.

Published in:

Neuropathology, 2014, v. 34, n. 1, p. 19, doi. 10.1111/neup.12056

By:

Kon, Tomoya;
Mori, Fumiaki;
Tanji, Kunikazu;
Miki, Yasuo;
Toyoshima, Yasuko;
Yoshida, Mari;
Sasaki, Hidenao;
Kakita, Akiyoshi;
Takahashi, Hitoshi;
Wakabayashi, Koichi

Publication type:

Article

Valosin-containing protein immunoreactivity in tauopathies, synucleinopathies, polyglutamine diseases and intranuclear inclusion body disease.

Published in:

Neuropathology, 2013, v. 33, n. 6, p. 637, doi. 10.1111/neup.12050

By:

Mori, Fumiaki;
Tanji, Kunikazu;
Toyoshima, Yasuko;
Sasaki, Hidenao;
Yoshida, Mari;
Kakita, Akiyoshi;
Takahashi, Hitoshi;
Wakabayashi, Koichi

Publication type:

Article

Autophagy Function and Benefits of Autophagy Induction in Models of Spinocerebellar Ataxia Type 3.

Published in:

Cells (2073-4409), 2023, v. 12, n. 6, p. 893, doi. 10.3390/cells12060893

By:

Watchon, Maxinne;
Luu, Luan;
Plenderleith, Stuart K.;
Yuan, Kristy C.;
Laird, Angela S.

Publication type:

Article