Literature DB >> 21906541

Modeling human trinucleotide repeat diseases in Drosophila.

Zhenming Yu1, Nancy M Bonini.   

Abstract

Drosophila is a powerful model system to study human trinucleotide repeat diseases. Findings in Drosophila models highlighted importance of host proteins, chaperons, and protein clearance pathways in polyglutamine diseases as well as that of RNA-binding proteins in noncoding repeat RNA toxicity diseases. Recent novel aspects revealed in Drosophila models include pleiotropic Ataxin 2 interactions, antisense transcription in trinucleotide repeat diseases, contribution of CAG RNA in polyglutamine diseases, and the role of RNA foci in CUG expansion diseases. Drosophila models have been also used for repeat stability studies.
Copyright © 2011 Elsevier Inc. All rights reserved.

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Year:  2011        PMID： 21906541     DOI： 10.1016/B978-0-12-387003-2.00008-2

Source DB:  PubMed          Journal:  Int Rev Neurobiol        ISSN： 0074-7742            Impact factor:   3.230


  8 in total

1.  The Social Amoeba Dictyostelium discoideum Is Highly Resistant to Polyglutamine Aggregation.

Authors:  Stephanie Santarriaga; Amber Petersen; Kelechi Ndukwe; Anthony Brandt; Nashaat Gerges; Jamie Bruns Scaglione; Kenneth Matthew Scaglione
Journal:  J Biol Chem       Date:  2015-09-01       Impact factor: 5.157

2.  A fly model for the CCUG-repeat expansion of myotonic dystrophy type 2 reveals a novel interaction with MBNL1.

Authors:  Zhenming Yu; Lindsey D Goodman; Shin-Yi Shieh; Michelle Min; Xiuyin Teng; Yongqing Zhu; Nancy M Bonini
Journal:  Hum Mol Genet       Date:  2014-10-09       Impact factor: 6.150

3.  An automated image analysis method to measure regularity in biological patterns: a case study in a Drosophila neurodegenerative model.

Authors:  Sergio Diez-Hermano; Jorge Valero; Cristina Rueda; Maria D Ganfornina; Diego Sanchez
Journal:  Mol Neurodegener       Date:  2015-03-12       Impact factor: 14.195

4.  Systematic analysis of fly models with multiple drivers reveals different effects of ataxin-1 and huntingtin in neuron subtype-specific expression.

Authors:  Risa Shiraishi; Takuya Tamura; Masaki Sone; Hitoshi Okazawa
Journal:  PLoS One       Date:  2014-12-31       Impact factor: 3.240

Review 5.  RNA-mediated pathogenic mechanisms in polyglutamine diseases and amyotrophic lateral sclerosis.

Authors:  Ho Yin Edwin Chan
Journal:  Front Cell Neurosci       Date:  2014-12-19       Impact factor: 5.505

Review 6.  Drosophila melanogaster As a Model Organism to Study RNA Toxicity of Repeat Expansion-Associated Neurodegenerative and Neuromuscular Diseases.

Authors:  Alex C Koon; Ho Yin Edwin Chan
Journal:  Front Cell Neurosci       Date:  2017-03-21       Impact factor: 5.505

7.  Genetic complexity in a Drosophila model of diabetes-associated misfolded human proinsulin.

Authors:  Soo-Young Park; Michael Z Ludwig; Natalia A Tamarina; Bin Z He; Sarah H Carl; Desiree A Dickerson; Levi Barse; Bharath Arun; Calvin L Williams; Cecelia M Miles; Louis H Philipson; Donald F Steiner; Graeme I Bell; Martin Kreitman
Journal:  Genetics       Date:  2013-11-26       Impact factor: 4.562

8.  Suppression of polyglutamine protein toxicity by co-expression of a heat-shock protein 40 and a heat-shock protein 110.

Authors:  Y Kuo; S Ren; U Lao; B A Edgar; T Wang
Journal:  Cell Death Dis       Date:  2013-10-03       Impact factor: 8.469
  8 in total

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