Ataxin-3 represses transcription via chromatin binding, interaction with histone deacetylase 3, and histone deacetylation

BO Evert, J Araujo, AM Vieira-Saecker… - Journal of …, 2006 - Soc Neuroscience
Ataxin-3 (AT3), the disease protein in spinocerebellar ataxia type 3 (SCA3), has been
associated with the ubiquitin–proteasome system and transcriptional regulation. Here we …

Partial loss of ataxin-1 function contributes to transcriptional dysregulation in spinocerebellar ataxia type 1 pathogenesis

J Crespo-Barreto, JD Fryer, CA Shaw, HT Orr… - PLoS …, 2010 - journals.plos.org
Spinocerebellar ataxia type 1 (SCA1) is a dominantly inherited neurodegenerative disease
caused by expansion of a CAG repeat that encodes a polyglutamine tract in ATAXIN1 …

Inactivation of the mouse Atxn3 (ataxin-3) gene increases protein ubiquitination

I Schmitt, M Linden, H Khazneh, BO Evert… - Biochemical and …, 2007 - Elsevier
Spinocerebellar ataxia type 3 is a neurodegenerative disease caused by expansion of a
polyglutamine domain in the protein ataxin-3 (ATXN3). Physiological functions of ATXN3 …

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

L Zeng, D Zhang, HS McLoughlin, AJ Zalon… - PLoS …, 2018 - journals.plos.org
Spinocerebellar ataxia type 3 (SCA3) is a dominantly inherited neurodegenerative disorder
caused by a polyglutamine-encoding CAG repeat expansion in the ATXN3 gene which …

The histone deacetylase HDAC3 is essential for Purkinje cell function, potentially complicating the use of HDAC inhibitors in SCA1

A Venkatraman, YS Hu, A Didonna… - Human molecular …, 2014 - academic.oup.com
Abstract Spinocerebellar ataxia type 1 (SCA1) is an incurable neurodegenerative disease
caused by a pathogenic glutamine repeat expansion in the protein ataxin-1 (ATXN1). One …

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

CC Tsai, HY Kao, A Mitzutani… - Proceedings of the …, 2004 - National Acad Sciences
Ataxin 1 (Atx1) is a foci-forming polyglutamine protein of unknown function, whose mutant
form causes type 1 spinocerebellar ataxia in humans and exerts neurotoxicity in transgenic …

Aggregation of polyglutamine-expanded ataxin 7 protein specifically sequesters ubiquitin-specific protease 22 and deteriorates its deubiquitinating function in the Spt …

H Yang, S Liu, WT He, J Zhao, LL Jiang… - Journal of Biological …, 2015 - ASBMB
Human ataxin 7 (Atx7) is a component of the deubiquitination module (DUBm) in the Spt-
Ada-Gcn5-acetyltransferase (SAGA) complex for transcriptional regulation, and expansion of …

Phosphorylation of ATXN1 at Ser776 in the cerebellum

ND Jorgensen, JM Andresen, S Lagalwar… - Journal of …, 2009 - Wiley Online Library
Abstract Spinocerebellar ataxia type 1 (SCA1) is one of nine inherited neurodegenerative
disorders caused by a mutant protein with an expanded polyglutamine tract …

Proteotoxic stress increases nuclear localization of ataxin-3

CP Reina, X Zhong, RN Pittman - Human molecular genetics, 2010 - academic.oup.com
Abstract Spinocerebellar ataxia type 3 (SCA3)/Machado Joseph disease results from
expansion of the polyglutamine domain in ataxin-3 (Atx3). Atx3 is a transcriptional co …

Functional genomics and biochemical characterization of the C. elegans orthologue of the Machado‐Joseph disease protein ataxin‐3

AJ Rodrigues, G Coppola, C Santos… - The FASEB …, 2007 - Wiley Online Library
Machado‐Joseph disease (MJD) is the most common dominant spinocerebellar ataxia. MJD
is caused by a CAG trinucleotide expansion in the ATXN3 gene, which encodes a protein …