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Diseases of unstable repeat expansion: mechanisms and common principles

author

Jennifer R Gatchel

Huda Zoghbi

1954 -

published in: Nature Reviews Genetics

date of publication: 2005-10-01

Cites articles 142

Date

Title

Authors

2005-03-01

Pharmacological rescue of synaptic plasticity, courtship behavior, and mushroom body defects in a Drosophila model of fragile X syndrome
2005-04-01

Kissing complex RNAs mediate interaction between the Fragile-X mental retardation protein KH2 domain and brain polyribosomes

Sean Eddy, Giovanni Stefani, Robert B. Darnell
2005-02-01

Global impairment of the ubiquitin-proteasome system by nuclear or cytoplasmic protein aggregates precedes inclusion body formation.
2005-01-20

Neuronal dysfunction in a polyglutamine disease model occurs in the absence of ubiquitin-proteasome system impairment and inversely correlates with the degree of nuclear inclusion formation.

Aaron B. Bowman, Nico P. Dantuma, Huda Zoghbi
2005-08-02

Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions

Blair R Leavitt, Michael R. Hayden, Rona K Graham, Elizabeth J Slow, Nagat Bissada, Ronald Wetzel
2005-08-01

The AXH domain of Ataxin-1 mediates neurodegeneration through its interaction with Gfi-1/Senseless proteins

Hung-Kai Chen, Matthew F. Rose, Koen Venken, Harry T. Orr, Hugo J. Bellen, Huda Zoghbi
2005-06-02

Polyglutamine-expanded ataxin-7 inhibits STAGA histone acetyltransferase activity to produce retinal degeneration

Armin M Gamper
2005-06-02

Polyglutamine-expanded spinocerebellar ataxia-7 protein disrupts normal SAGA and SLIK histone acetyltransferase activity
2005-02-03

Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington's disease

Mutsuyuki Sugimori, Michael R. Hayden, Bruce S Kristal
2005-04-01

Ataxin-3 suppresses polyglutamine neurodegeneration in Drosophila by a ubiquitin-associated mechanism.

Nancy M. Bonini
2005-04-20

Transgenic mice expressing CUG-BP1 reproduce splicing mis-regulation observed in myotonic dystrophy

Thai Huu Ho
2005-10-01

Therapeutics development for triplet repeat expansion diseases
2005-07-01

Cognitive decline, neuromotor and behavioural disturbances in a mouse model for fragile-X-associated tremor/ataxia syndrome (FXTAS)

Frank Kooy, Ben A Oostra
2005-06-16

Colocalization of muscleblind with RNA foci is separable from mis-regulation of alternative splicing in myotonic dystrophy

Thai Huu Ho, Maurice S. Swanson
2004-07

The mGluR theory of fragile X mental retardation

Mark Bear
2004-06-01

The fragile X-related gene affects the crawling behavior of Drosophila larvae by regulating the mRNA level of the DEG/ENaC protein pickpocket1.
2004-02

Biochemical and genetic interaction between the fragile X mental retardation protein and the microRNA pathway

Stephanie Ceman, Stephen T. Warren, Mika Nakamoto
2004-11

RNA and microRNAs in fragile X mental retardation
2004-06-18

Frataxin-mediated iron delivery to ferrochelatase in the final step of heme biosynthesis
2004-12-22

Friedreich ataxia: the oxidative stress paradox.

Paweł Golik
2004-04-01

Eukaryotic proteasomes cannot digest polyglutamine sequences and release them during degradation of polyglutamine-containing proteins
2004-06-01

Proteasome degrades soluble expanded polyglutamine completely and efficiently.

Christine Van Broeckhoven
2004-05-01

Modulating huntingtin half-life alters polyglutamine-dependent aggregate formation and cell toxicity
2004-10-14

Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death
2004-06

Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease

Cahir J O'Kane, Douglas F. Easton
2004-07-01

Cellular toxicity of polyglutamine expansion proteins: mechanism of transcription factor deactivation

Manajit Hayer-Hartl, Franz-Ulrich Hartl, Katja Siegers
2004-03-23

Ataxin 1, a SCA1 neurodegenerative disorder protein, is functionally linked to the silencing mediator of retinoid and thyroid hormone receptors

Ronald M. Evans
2004-04-21

Sodium butyrate ameliorates phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy

Hiroaki Adachi, Gen Sobue
2004-04-02

SUMO modification of Huntingtin and Huntington's disease pathology

Pier Paolo Pandolfi, Elena Cattaneo
2004-10-01

Pathogenic RNA repeats: an expanding role in genetic disease
2004-10-20

Myotonic dystrophy type 1 is associated with nuclear foci of mutant RNA, sequestration of muscleblind proteins and deregulated alternative splicing in neurons

Maurice S. Swanson
2004-08-04

Muscleblind proteins regulate alternative splicing

Article - wd:Q36277192

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