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Ataxin-3

Known as: Spinocerebellar Ataxia 3 Protein, Ataxin 3, Ataxin 3 Protein 
A deubiquitinating enzyme of the ATAXINS family. It functions in protein homeostasis, GENETIC TRANSCRIPTION; CYTOSKELETON regulation, and MYOGENESIS… 
National Institutes of Health

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11 relations

Narrower (2)

ATXN3 protein, humanAtxn3 protein, rat
Ataxia, SpinocerebellarIn BloodProcess of secretionagonists

Papers overview

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Highly Cited
2011
Highly Cited
2011
HDAC inhibitor sodium butyrate reverses transcriptional downregulation and ameliorates ataxic symptoms in a transgenic mouse model of SCA3
Review
2010
Review
2010
Caring for Machado-Joseph disease: current understanding and how to help patients.
Highly Cited
2009
Highly Cited
2009
Ubiquitination directly enhances activity of the deubiquitinating enzyme ataxin‐3
Deubiquitinating enzymes (DUBs) control the ubiquitination status of proteins in various cellular pathways. Regulation of the… 
Highly Cited
2009
Highly Cited
2009
In vivo suppression of polyglutamine neurotoxicity by C-terminus of Hsp70-interacting protein (CHIP) supports an aggregation model of pathogenesis
Highly Cited
2006
Highly Cited
2006
The Two-stage Pathway of Ataxin-3 Fibrillogenesis Involves a Polyglutamine-independent Step*
The aggregation of ataxin-3 is associated with spinocerebellar ataxia type 3, which is characterized by the formation of… 
Highly Cited
2004
Highly Cited
2004
Structural and functional analysis of ataxin-2 and ataxin-3.
Spinocerebellar ataxia types 2 (SCA2) and 3 (SCA3) are autosomal-dominantly inherited, neurodegenerative diseases caused by CAG… 
Highly Cited
2004
Highly Cited
2004
Expression of a Poly-Glutamine-Ataxin-3 Transgene in Orexin Neurons Induces Narcolepsy–Cataplexy in the Rat
The sleep disorder narcolepsy has been linked to loss of hypothalamic neurons producing the orexin (hypocretin) neuropeptides… 
Highly Cited
2001
Highly Cited
2001
An expanded glutamine repeat destabilizes native ataxin-3 structure and mediates formation of parallel β-fibrils
The protein ataxin-3 contains a polyglutamine region; increasing the number of glutamines beyond 55 in this region gives rise to… 
Highly Cited
2001
Highly Cited
2001
Inflammatory Genes Are Upregulated in Expanded Ataxin-3-Expressing Cell Lines and Spinocerebellar Ataxia Type 3 Brains
Spinocerebellar ataxia type 3 (SCA3) is a polyglutamine disorder caused by a CAG repeat expansion in the coding region of a gene… 
Review
1997
Review
1997
Intranuclear Neuronal Inclusions: A Common Pathogenic Mechanism for Glutamine-Repeat Neurodegenerative Diseases?
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