Literature DB >> 20869586

PolyQ disease: too many Qs, too much function?

Ian H Kratter1, Steven Finkbeiner.   

Abstract

The nature of the gain-of-function toxicity found in polyglutamine (polyQ) diseases has been the subject of considerable debate. In this issue of Neuron, Duvick et al. and Nedelsky et al. show that, in two of these diseases, pathology is mediated by normal protein activity.
Copyright © 2010 Elsevier Inc. All rights reserved.

Entities:  

Year:  2010        PMID： 20869586      PMCID： PMC3989162          DOI： 10.1016/j.neuron.2010.09.012

Source DB:  PubMed          Journal:  Neuron        ISSN： 0896-6273            Impact factor:   17.173


  15 in total

1.  Altered transcriptional regulation in cells expressing the expanded polyglutamine androgen receptor.

Authors:  Andrew P Lieberman; George Harmison; Andrew D Strand; James M Olson; Kenneth H Fischbeck
Journal:  Hum Mol Genet       Date:  2002-08-15       Impact factor: 6.150

2.  Native functions of the androgen receptor are essential to pathogenesis in a Drosophila model of spinobulbar muscular atrophy.

Authors:  Natalia B Nedelsky; Maria Pennuto; Rebecca B Smith; Isabella Palazzolo; Jennifer Moore; Zhiping Nie; Geoffrey Neale; J Paul Taylor
Journal:  Neuron       Date:  2010-09-23       Impact factor: 17.173

Review 3.  Trinucleotide repeat disorders.

Authors:  Harry T Orr; Huda Y Zoghbi
Journal:  Annu Rev Neurosci       Date:  2007       Impact factor: 12.449

4.  Expanded polyglutamine peptides alone are intrinsically cytotoxic and cause neurodegeneration in Drosophila.

Authors:  J L Marsh; H Walker; H Theisen; Y Z Zhu; T Fielder; J Purcell; L M Thompson
Journal:  Hum Mol Genet       Date:  2000-01-01       Impact factor: 6.150

5.  Androgen-dependent neurodegeneration by polyglutamine-expanded human androgen receptor in Drosophila.

Authors:  Ken-ichi Takeyama; Saya Ito; Ayako Yamamoto; Hiromu Tanimoto; Takashi Furutani; Hirotaka Kanuka; Masayuki Miura; Tetsuya Tabata; Shigeaki Kato
Journal:  Neuron       Date:  2002-08-29       Impact factor: 17.173

6.  Efficacy and safety of leuprorelin in patients with spinal and bulbar muscular atrophy (JASMITT study): a multicentre, randomised, double-blind, placebo-controlled trial.

Authors:  Masahisa Katsuno; Haruhiko Banno; Keisuke Suzuki; Yu Takeuchi; Motoshi Kawashima; Ichiro Yabe; Hidenao Sasaki; Masashi Aoki; Mitsuya Morita; Imaharu Nakano; Kazuaki Kanai; Shoichi Ito; Kinya Ishikawa; Hidehiro Mizusawa; Tomotaka Yamamoto; Shoji Tsuji; Kazuko Hasegawa; Takayoshi Shimohata; Masatoyo Nishizawa; Hiroaki Miyajima; Fumio Kanda; Yasuhiro Watanabe; Kenji Nakashima; Akira Tsujino; Taro Yamashita; Makoto Uchino; Yasushi Fujimoto; Fumiaki Tanaka; Gen Sobue
Journal:  Lancet Neurol       Date:  2010-08-04       Impact factor: 44.182

Review 7.  Biological and chemical approaches to diseases of proteostasis deficiency.

Authors:  Evan T Powers; Richard I Morimoto; Andrew Dillin; Jeffery W Kelly; William E Balch
Journal:  Annu Rev Biochem       Date:  2009       Impact factor: 23.643

8.  Serine 776 of ataxin-1 is critical for polyglutamine-induced disease in SCA1 transgenic mice.

Authors:  Effat S Emamian; Michael D Kaytor; Lisa A Duvick; Tao Zu; Susan K Tousey; Huda Y Zoghbi; H Brent Clark; Harry T Orr
Journal:  Neuron       Date:  2003-05-08       Impact factor: 17.173

9.  Serines 13 and 16 are critical determinants of full-length human mutant huntingtin induced disease pathogenesis in HD mice.

Authors:  Xiaofeng Gu; Erin R Greiner; Rakesh Mishra; Ravindra Kodali; Alex Osmand; Steven Finkbeiner; Joan S Steffan; Leslie Michels Thompson; Ronald Wetzel; X William Yang
Journal:  Neuron       Date:  2009-12-24       Impact factor: 17.173

10.  Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1.

Authors:  Janghoo Lim; Juan Crespo-Barreto; Paymaan Jafar-Nejad; Aaron B Bowman; Ronald Richman; David E Hill; Harry T Orr; Huda Y Zoghbi
Journal:  Nature       Date:  2008-03-12       Impact factor: 49.962
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  20 in total

1.  Pathogenic polyglutamine proteins cause dendrite defects associated with specific actin cytoskeletal alterations in Drosophila.

Authors:  Sung Bae Lee; Joshua A Bagley; Hye Young Lee; Lily Yeh Jan; Yuh-Nung Jan
Journal:  Proc Natl Acad Sci U S A       Date:  2011-09-19       Impact factor: 11.205

2.  Modeling Huntington disease in Drosophila: Insights into axonal transport defects and modifiers of toxicity.

Authors:  Megan Krench; J Troy Littleton
Journal:  Fly (Austin)       Date:  2013-09-10       Impact factor: 2.160

Review 3.  Polyglutamine neurodegeneration: expanded glutamines enhance native functions.

Authors:  Harry T Orr
Journal:  Curr Opin Genet Dev       Date:  2012-01-25       Impact factor: 5.578

Review 4.  Huntington's Disease.

Authors:  Steven Finkbeiner
Journal:  Cold Spring Harb Perspect Biol       Date:  2011-06-01       Impact factor: 10.005

Review 5.  Proteins Containing Expanded Polyglutamine Tracts and Neurodegenerative Disease.

Authors:  Adewale Adegbuyiro; Faezeh Sedighi; Albert W Pilkington; Sharon Groover; Justin Legleiter
Journal:  Biochemistry       Date:  2017-02-21       Impact factor: 3.162

6.  Neurogenetics: advancing the "next-generation" of brain research.

Authors:  Huda Y Zoghbi; Stephen T Warren
Journal:  Neuron       Date:  2010-10-21       Impact factor: 17.173

Review 7.  Modeling Huntington's disease with induced pluripotent stem cells.

Authors:  Julia A Kaye; Steven Finkbeiner
Journal:  Mol Cell Neurosci       Date:  2013-02-28       Impact factor: 4.314

Review 8.  The unstable repeats--three evolving faces of neurological disease.

Authors:  David L Nelson; Harry T Orr; Stephen T Warren
Journal:  Neuron       Date:  2013-03-06       Impact factor: 17.173

Review 9.  Polyglutamine androgen receptor-mediated neuromuscular disease.

Authors:  Elisa Giorgetti; Andrew P Lieberman
Journal:  Cell Mol Life Sci       Date:  2016-05-17       Impact factor: 9.261

10.  Background-dependent effects of polyglutamine variation in the Arabidopsis thaliana gene ELF3.

Authors:  Soledad Francisca Undurraga; Maximilian Oliver Press; Matthieu Legendre; Nora Bujdoso; Jacob Bale; Hui Wang; Seth J Davis; Kevin J Verstrepen; Christine Queitsch
Journal:  Proc Natl Acad Sci U S A       Date:  2012-11-05       Impact factor: 11.205
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