Works matching DE "POLYGLUTAMINE"

Results: 444

The number of glutamines in the N‐terminal of the canine androgen receptor affects signalling intensities.

Published in:

Veterinary & Comparative Oncology, 2021, v. 19, n. 2, p. 399, doi. 10.1111/vco.12663

By:

Ochiai, Kazuhiko;
Sutijarit, Samak;
Uemura, Mitsuki;
Morimatsu, Masami;
Michishita, Masaki;
Onozawa, Eri;
maeda, Marika;
Sasaki, Takanori;
Watanabe, Masami;
Tanaka, Yoshikazu;
Omi, Toshinori

Publication type:

Article

Proteotoxic stresses stimulate dissociation of UBL4A from the tail-anchored protein recognition complex.

Published in:

Biochemical Journal, 2023, v. 480, n. 19, p. 1583, doi. 10.1042/BCJ20230267

By:

Takumi Hagiwara;
Ryosuke Minami;
Chizuru Ushio;
Naoto Yokota;
Hiroyuki Kawahara

Publication type:

Article

Characterization of axonal transport defects in Drosophila Huntingtin mutants.

Published in:

Journal of Neurogenetics, 2016, v. 30, n. 3/4, p. 212, doi. 10.1080/01677063.2016.1202950

By:

Weiss, Kurt R.;
Littleton, J. Troy

Publication type:

Article

Allele-selective suppression of mutant genes in polyglutamine diseases.

Published in:

Journal of Neurogenetics, 2015, v. 29, n. 2/3, p. 41, doi. 10.3109/01677063.2015.1073275

By:

Liu, Chia-Rung;
Cheng, Tzu-Hao

Publication type:

Article

Infantile‐onset parkinsonism, dyskinesia, and developmental delay: do not forget polyglutamine defects!

Published in:

2023

By:

Baide‐Mairena, Heidy;
Coget, Arthur;
Leboucq, Nicolas;
Procaccio, Vincent;
Blanluet, Maud;
Meyer, Pierre;
Malinge, Marie‐Claire;
François‐Heude, Marie‐Céline;
Moreno, Mathis;
Geneviève, David;
Marelli, Cecilia;
Roubertie, Agathe

Publication type:

Case Study

Impaired muscle uptake of creatine in spinal and bulbar muscular atrophy.

Published in:

Annals of Clinical & Translational Neurology, 2016, v. 3, n. 7, p. 537, doi. 10.1002/acn3.324

By:

Hijikata, Yasuhiro;
Katsuno, Masahisa;
Suzuki, Keisuke;
Hashizume, Atsushi;
Araki, Amane;
Yamada, Shinichiro;
Inagaki, Tomonori;
Iida, Madoka;
Noda, Seiya;
Nakanishi, Hirotaka;
Banno, Haruhiko;
Mano, Tomoo;
Hirakawa, Akihiro;
Adachi, Hiroaki;
Watanabe, Hirohisa;
Yamamoto, Masahiko;
Sobue, Gen

Publication type:

Article

Altered Metabolic Signaling and Potential Therapies in Polyglutamine Diseases.

Published in:

Metabolites (2218-1989), 2024, v. 14, n. 6, p. 320, doi. 10.3390/metabo14060320

By:

Vohra, Alisha;
Keefe, Patrick;
Puthanveetil, Prasanth

Publication type:

Article

Motor neurone disease/amyotrophic lateral sclerosis associated with intermediate-length CAG repeat expansions in Ataxin-2 does not have 1 C2-positive polyglutamine inclusions.

Published in:

Neuropathology & Applied Neurobiology, 2016, v. 42, n. 4, p. 377, doi. 10.1111/nan.12254

By:

Highley, John Robin;
Lorente Pons, Alejandro;
Cooper‐Knock, Johnathan;
Wharton, Stephen B.;
Ince, Paul G.;
Shaw, Pamela J.;
Wood, Jon;
Kirby, Janine

Publication type:

Article

Polyglutamine aggregation in Huntington's disease and spinocerebellar ataxia type 3: similar mechanisms in aggregate formation.

Published in:

Neuropathology & Applied Neurobiology, 2016, v. 42, n. 2, p. 153, doi. 10.1111/nan.12253

By:

Seidel, K.;
Siswanto, S.;
Fredrich, M.;
Bouzrou, M.;
Brunt, E. R.;
Leeuwen, F. W.;
Kampinga, H. H.;
Korf, H. ‐W.;
Rüb, U.;
Dunnen, W. F. A.

Publication type:

Article

Schwann cell involvement in the peripheral neuropathy of spinocerebellar ataxia type 3.

Published in:

Neuropathology & Applied Neurobiology, 2014, v. 40, n. 5, p. 628, doi. 10.1111/nan.12055

By:

Suga, Noriaki;
Katsuno, Masahisa;
Koike, Haruki;
Banno, Haruhiko;
Suzuki, Keisuke;
Hashizume, Atsushi;
Mano, Tomoo;
Iijima, Masahiro;
Kawagashira, Yuichi;
Hirayama, Masaaki;
Nakamura, Tomohiko;
Watanabe, Hirohisa;
Tanaka, Fumiaki;
Sobue, Gen

Publication type:

Article

Involvement of the cholinergic basal forebrain nuclei in spinocerebellar ataxia type 2 ( SCA2).

Published in:

Neuropathology & Applied Neurobiology, 2013, v. 39, n. 6, p. 634, doi. 10.1111/nan.12025

By:

Rüb, U.;
Farrag, K.;
Seidel, K.;
Brunt, E. R.;
Heinsen, H.;
Bürk, K.;
Melegh, B.;
Gall, C.;
Auburger, G.;
Bohl, J.;
Korf, H. W.;
Hoche, F.;
Dunnen, W.

Publication type:

Article

Cellular protein quality control and the evolution of aggregates in spinocerebellar ataxia type 3 (SCA3).

Published in:

Neuropathology & Applied Neurobiology, 2012, v. 38, n. 6, p. 548, doi. 10.1111/j.1365-2990.2011.01220.x

By:

Seidel, K.;
Meister, M.;
Dugbartey, G. J.;
Zijlstra, M. P.;
Vinet, J.;
Brunt, E. R. P.;
van Leeuwen, F. W.;
Rüb, U.;
Kampinga, H. H.;
den Dunnen, W. F. A.

Publication type:

Article

Polyglutamine (polyQ) disorders.

Published in:

Nucleus (1949-1034), 2012, v. 3, n. 5, p. 1, doi. 10.4161/nucl.21481

By:

Cohen-Carmon, Dorit;
Meshorer, Eran

Publication type:

Article

Mouse polyQ database: a new online resource for research using mouse models of neurodegenerative diseases.

Published in:

Molecular Brain, 2015, v. 8, p. 1, doi. 10.1186/s13041-015-0160-8

By:

Szlachcic, Wojciech J.;
Switonski, Pawel M.;
Kurkowiak, Małgorzata;
Wiatr, Kalina;
Figiel, Maciej

Publication type:

Article

Changes of peripheral TGF-beta1 depend on monocytes-derived macrophages in Huntington disease.

Published in:

Molecular Brain, 2013, v. 6, n. 1, p. 3, doi. 10.1186/1756-6606-6-55

By:

Pardo, Alba Di;
Alberti, Silvia;
Maglione, Vittorio;
Amico, Enrico;
Cortes, Etty P.;
Elifani, Francesca;
Battaglia, Giuseppe;
Busceti, Carla L.;
Nicoletti, Ferdinando;
Vonsattel, Jean Paul;
Squitieri, Ferdinando

Publication type:

Article

Loss of the Spinocerebellar Ataxia type 3 disease protein ATXN3 alters transcription of multiple signal transduction pathways.

Published in:

PLoS ONE, 2018, v. 13, n. 9, p. 1, doi. 10.1371/journal.pone.0204438

By:

Zeng, Li;
Zhang, Dapeng;
McLoughlin, Hayley S.;
Zalon, Annie J.;
Aravind, L.;
Paulson, Henry L.

Publication type:

Article

Upregulation of miR-370 and miR-543 is associated with reduced expression of heat shock protein 40 in spinocerebellar ataxia type 3.

Published in:

PLoS ONE, 2018, v. 13, n. 8, p. 1, doi. 10.1371/journal.pone.0201794

By:

Evert, Bernd O.;
Nalavade, Rohit;
Jungverdorben, Johannes;
Matthes, Frank;
Weber, Stephanie;
Rajput, Ashish;
Bonn, Stefan;
Brüstle, Oliver;
Peitz, Michael;
Krauß, Sybille

Publication type:

Article

Potential Transfer of Polyglutamine and CAG-Repeat RNA in Extracellular Vesicles in Huntington's Disease: Background and Evaluation in Cell Culture.

Published in:

Cellular & Molecular Neurobiology, 2016, v. 36, n. 3, p. 459, doi. 10.1007/s10571-016-0350-7

By:

Zhang, Xuan;
Abels, Erik;
Redzic, Jasmina;
Margulis, Julia;
Finkbeiner, Steve;
Breakefield, Xandra

Publication type:

Article

Polyglutamine Expansion in Huntingtin and Mechanism of DNA Damage Repair Defects in Huntington's Disease.

Published in:

Frontiers in Cellular Neuroscience, 2022, v. 16, p. 1, doi. 10.3389/fncel.2022.837576

By:

Pradhan, Subrata;
Gao, Rui;
Bush, Keegan;
Zhang, Nan;
Wairkar, Yogesh P.;
Sarkar, Partha S.

Publication type:

Article

Detection of ubiquitinated huntingtin species in intracellular aggregates.

Published in:

Frontiers in Molecular Neuroscience, 2015, v. 8, p. 1, doi. 10.3389/fnmol.2015.00001

By:

Juenemann, Katrin;
Wiemhoefer, Anne;
Reits, Eric A.

Publication type:

Article

Ubiquitin-proteasome system involvement in Huntington's disease.

Published in:

Frontiers in Molecular Neuroscience, 2014, v. 7, p. 10, doi. 10.3389/fnmol.2014.00077

By:

Ortega, Zaira;
Lucas, Jose J.

Publication type:

Article

The ubiquitin-proteasome system in neurodegenerative diseases: precipitating factor, yet part of the solution.

Published in:

Frontiers in Molecular Neuroscience, 2014, p. 1, doi. 10.3389/fnmol.2014.00070

By:

Dantuma, Nico P.;
Bott, Laura C.

Publication type:

Article

Small non-coding RNAs add complexity to the RNA pathogenic mechanisms in trinucleotide repeat expansion diseases.

Published in:

Frontiers in Molecular Neuroscience, 2013, v. 6, p. 1, doi. 10.3389/fnmol.2013.00045

By:

Martí, Eulàlia;
Estivill, Xavier

Publication type:

Article

RNA toxicity induced by expanded CAG repeats in Huntington's disease.

Published in:

Brain Pathology, 2016, v. 26, n. 6, p. 779, doi. 10.1111/bpa.12427

By:

Martí, Eulàlia

Publication type:

Article

XVIII International Congress of Neuropathology September 17, 2014.

Published in:: 2014
Publication type:: Abstract

Huntington's Disease ( HD): Degeneration of Select Nuclei, Widespread Occurrence of Neuronal Nuclear and Axonal Inclusions in the Brainstem.

Published in:

Brain Pathology, 2014, v. 24, n. 3, p. 247, doi. 10.1111/bpa.12115

By:

Rüb, Udo;
Hentschel, Matthias;
Stratmann, Katharina;
Brunt, Ewout;
Heinsen, Helmut;
Seidel, Kay;
Bouzrou, Mohamed;
Auburger, Georg;
Paulson, Henry;
Vonsattel, Jean‐Paul;
Lange, Herwig;
Korf, Horst‐Werner;
Dunnen, Wilfred

Publication type:

Article

Naratriptan mitigates CGRP1-associated motor neuron degeneration caused by an expanded polyglutamine repeat tract.

Published in:

Nature Medicine, 2012, v. 18, n. 10, p. 1531, doi. 10.1038/nm.2932

By:

Minamiyama, Makoto;
Katsuno, Masahisa;
Adachi, Hiroaki;
Doi, Hideki;
Kondo, Naohide;
Iida, Madoka;
Ishigaki, Shinsuke;
Fujioka, Yusuke;
Matsumoto, Shinjiro;
Miyazaki, Yu;
Tanaka, Fumiaki;
Kurihara, Hiroki;
Sobue, Gen

Publication type:

Article

Sirt1 mediates neuroprotection from mutant huntingtin by activation of the TORC1 and CREB transcriptional pathway.

Published in:

Nature Medicine, 2012, v. 18, n. 1, p. 159, doi. 10.1038/nm.2559

By:

Jeong, Hyunkyung;
Cohen, Dena E;
Cui, Libin;
Supinski, Andrea;
Savas, Jeffrey N;
Mazzulli, Joseph R;
Yates, John R;
Bordone, Laura;
Guarente, Leonard;
Krainc, Dimitri

Publication type:

Article

Neuroprotective role of Sirt1 in mammalian models of Huntington's disease through activation of multiple Sirt1 targets.

Published in:

Nature Medicine, 2012, v. 18, n. 1, p. 153, doi. 10.1038/nm.2558

By:

Jiang, Mali;
Wang, Jiawei;
Fu, Jinrong;
Du, Lin;
Jeong, Hyunkyung;
West, Tim;
Xiang, Lan;
Peng, Qi;
Hou, Zhipeng;
Cai, Huan;
Seredenina, Tamara;
Arbez, Nicolas;
Zhu, Shanshan;
Sommers, Katherine;
Qian, Jennifer;
Zhang, Jiangyang;
Mori, Susumu;
Yang, X William;
Tamashiro, Kellie L K;
Aja, Susan

Publication type:

Article

Neuroprotective Potential of Eugenol in Polyglutamine-Mediated Neurodegenerative Disease Using Transgenic Drosophila Model.

Published in:

Dose-Response, 2024, v. 22, n. 4, p. 1, doi. 10.1177/15593258241291652

By:

Chongtham, Anjalika;
Agrawal, Namita

Publication type:

Article

Glutathione peroxidase activity is neuroprotective in models of Huntington's disease.

Published in:

Nature Genetics, 2013, v. 45, n. 10, p. 1249, doi. 10.1038/ng.2732

By:

Mason, Robert P;
Casu, Massimiliano;
Butler, Nicola;
Breda, Carlo;
Campesan, Susanna;
Clapp, Jannine;
Green, Edward W;
Dhulkhed, Devyani;
Kyriacou, Charalambos P;
Giorgini, Flaviano

Publication type:

Article

Large-scale functional RNAi screen in C. elegans identifies genes that regulate the dysfunction of mutant polyglutamine neurons.

Published in:: BMC Genomics, 2012, v. 13, n. 1, p. 91, doi. 10.1186/1471-2164-13-91
Publication type:: Article

Spinocerebellar ataxia type-7: Report of a family in Northwest Nigeria.

Published in:

Annals of African Medicine, 2016, v. 15, n. 2, p. 87, doi. 10.4103/1596-3519.176205

By:

Hamidu Alkali, Nura;
Bwala, Sunday A.;
Alimi, Saeed A.;
Oyakhire, Shyngle I.

Publication type:

Article

Dimerization of Polyglutamine within the PRIME20 Model using Stochastic Approximation Monte Carlo.

Published in:

Macromolecular Theory & Simulations, 2023, v. 32, n. 5, p. 1, doi. 10.1002/mats.202200075

By:

Lauer, Christian;
Paul, Wolfgang

Publication type:

Article

How Epigallocatechin-3-gallate and Tetracycline Interact with the Josephin Domain of Ataxin-3 and Alter Its Aggregation Mode.

Published in:

Chemistry - A European Journal, 2015, v. 21, n. 50, p. 18383, doi. 10.1002/chem.201503086

By:

Bonanomi, Marcella;
Visentin, Cristina;
Natalello, Antonino;
Spinelli, Michela;
Vanoni, Marco;
Airoldi, Cristina;
Regonesi, Maria E.;
Tortora, Paolo

Publication type:

Article

Computational investigation of molecular mechanism and neuropathological implications in Huntington disease.

Published in:

Molecular & Cellular Biochemistry, 2015, v. 409, n. 1/2, p. 1, doi. 10.1007/s11010-015-2462-7

By:

Gopalakrishnan, Chandrasekhar;
Kalsi, Namrata;
Jethi, Shraddha;
Purohit, Rituraj

Publication type:

Article

Genetik der spinozerebellären Ataxien.

Published in:

Der Nervenarzt, 2013, v. 84, n. 2, p. 137, doi. 10.1007/s00115-012-3637-z

By:

Jacobi, H.;
Minnerop, M.;
Klockgether, T.

Publication type:

Article

The autophagy/lysosome pathway is impaired in SCA7 patients and SCA7 knock-in mice.

Published in:

Acta Neuropathologica, 2014, v. 128, n. 5, p. 705, doi. 10.1007/s00401-014-1289-8

By:

Alves, Sandro;
Cormier-Dequaire, Florence;
Marinello, Martina;
Marais, Thibaut;
Muriel, Marie-Paule;
Beaumatin, Florian;
Charbonnier-Beaupel, Fanny;
Tahiri, Khadija;
Seilhean, Danielle;
Hachimi, Khalid;
Ruberg, Merle;
Stevanin, Giovanni;
Barkats, Martine;
Dunnen, Wilfred;
Priault, Muriel;
Brice, Alexis;
Durr, Alexandra;
Corvol, Jean-Christophe;
Sittler, Annie

Publication type:

Article

FTLD-ALS of TDP-43 type and SCA2 in a family with a full ataxin-2 polyglutamine expansion.

Published in:

2014

By:

Bäumer, Dirk;
East, Simon;
Tseu, Bing;
Zeman, Adam;
Hilton, David;
Talbot, Kevin;
Ansorge, Olaf

Publication type:

Report

Androgen-dependent impairment of myogenesis in spinal and bulbar muscular atrophy.

Published in:

Acta Neuropathologica, 2013, v. 126, n. 1, p. 109, doi. 10.1007/s00401-013-1122-9

By:

Malena, Adriana;
Pennuto, Maria;
Tezze, Caterina;
Querin, Giorgia;
D'Ascenzo, Carla;
Silani, Vincenzo;
Cenacchi, Giovanna;
Scaramozza, Annarita;
Romito, Silvia;
Morandi, Lucia;
Pegoraro, Elena;
Russell, Aaron;
Sorarù, Gianni;
Vergani, Lodovica

Publication type:

Article

Coexistence of Huntington's disease and amyotrophic lateral sclerosis: a clinicopathologic study.

Published in:

Acta Neuropathologica, 2012, v. 124, n. 5, p. 749, doi. 10.1007/s00401-012-1005-5

By:

Tada, Mari;
Coon, Elizabeth;
Osmand, Alexander;
Kirby, Patricia;
Martin, Wayne;
Wieler, Marguerite;
Shiga, Atsushi;
Shirasaki, Hiroe;
Tada, Masayoshi;
Makifuchi, Takao;
Yamada, Mitsunori;
Kakita, Akiyoshi;
Nishizawa, Masatoyo;
Takahashi, Hitoshi;
Paulson, Henry

Publication type:

Article

Distinct TDP-43 pathology in ALS patients with ataxin 2 intermediate-length polyQ expansions.

Published in:

Acta Neuropathologica, 2012, v. 124, n. 2, p. 221, doi. 10.1007/s00401-012-0985-5

By:

Hart, Michael;
Brettschneider, Johannes;
Lee, Virginia;
Trojanowski, John;
Gitler, Aaron

Publication type:

Article

Long-term proteasomal inhibition in transgenic mice by UBB expression results in dysfunction of central respiration control reminiscent of brainstem neuropathology in Alzheimer patients.

Published in:

Acta Neuropathologica, 2012, v. 124, n. 2, p. 187, doi. 10.1007/s00401-012-1003-7

By:

Irmler, Martin;
Gentier, Romina;
Dennissen, Frank;
Schulz, Holger;
Bolle, Ines;
Hölter, Sabine;
Kallnik, Magdalena;
Cheng, Jing;
Klingenspor, Martin;
Rozman, Jan;
Ehrhardt, Nicole;
Hermes, Denise;
Gailus-Durner, Valérie;
Fuchs, Helmut;
Hrabě de Angelis, Martin;
Meyer, Helmut;
Hopkins, David;
Leeuwen, Fred;
Beckers, Johannes

Publication type:

Article

Brain pathology of spinocerebellar ataxias.

Published in:

Acta Neuropathologica, 2012, v. 124, n. 1, p. 1, doi. 10.1007/s00401-012-1000-x

By:

Seidel, Kay;
Siswanto, Sonny;
Brunt, Ewout;
Dunnen, Wilfred;
Korf, Horst-Werner;
Rüb, Udo

Publication type:

Article

Ubiquilin immunoreactivity in cytoplasmic and nuclear inclusions in synucleinopathies, polyglutamine diseases and intranuclear inclusion body disease.

Published in:

2012

By:

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

Publication type:

Letter

Optineurin immunoreactivity in neuronal nuclear inclusions of polyglutamine diseases (Huntington's, DRPLA, SCA2, SCA3) and intranuclear inclusion body disease.

Published in:

2012

By:

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

Publication type:

Letter

Conserved Pbp1/Ataxin-2 regulates retrotransposon activity and connects polyglutamine expansion-driven protein aggregation to lifespan-controlling rDNA repeats.

Published in:

Communications Biology, 2018, v. 1, n. 1, p. N.PAG, doi. 10.1038/s42003-018-0187-3

By:

Ostrowski, Lauren A.;
Hall, Amanda C.;
Szafranski, Kirk J.;
Oshidari, Roxanne;
Abraham, Karan J.;
Chan, Janet N. Y.;
Krustev, Christian;
Zhang, Kevin;
Wang, Ashley;
Liu, Yupeng;
Guo, Ru;
Mekhail, Karim

Publication type:

Article

Cell biology of spinocerebellar ataxia.

Published in:

Journal of Cell Biology, 2012, v. 197, n. 2, p. 167, doi. 10.1083/jcb.201105092

By:

Orr, Harry T.

Publication type:

Article

Cognitive Deficits in the R6/2 mouse model of Huntington's disease and their Amelioration with Donepezil.

Published in:

International Journal of Comparative Psychology, 2014, v. 27, n. 3, p. 397, doi. 10.46867/ijcp.2014.27.03.08

By:

Murphy, Carol A.;
Paterson, Neil E.;
Chen, Angela;
Arias, Washington;
He, Dansha;
Alosio, William;
Oakeshott, Steven;
Farrar, Andrew;
Menalled, Liliana;
Ramboz, Sylvie;
Brunner, Dani;
Howland, David;
Park, Larry C.

Publication type:

Article

Exogenous polyserine and polyleucine are toxic to recipient cells.

Published in:

Scientific Reports, 2022, v. 12, n. 1, p. 1, doi. 10.1038/s41598-022-05720-y

By:

Owada, Ryuji;
Mitsui, Shinichi;
Nakamura, Kazuhiro

Publication type:

Article