miR-19, miR-101 and miR-130 co-regulate ATXN1 levels to potentially modulate SCA1 pathogenesis

Y Lee, RC Samaco, JR Gatchel, C Thaller, HT Orr… - Nature …, 2008 - nature.com
Spinocerebellar ataxia type 1 is caused by expansion of a translated CAG repeat in ataxin1
(ATXN1). The level of the polyglutamine-expanded protein is one of the factors that …

Early activation of microglia and astrocytes in mouse models of spinocerebellar ataxia type 1

M Cvetanovic, M Ingram, H Orr, P Opal - Neuroscience, 2015 - Elsevier
Abstract Spinocerebellar ataxia type 1 (SCA1) is an incurable, dominantly inherited
neurodegenerative disease of the cerebellum caused by a polyglutamine-repeat expansion …

FOXO4-dependent upregulation of superoxide dismutase-2 in response to oxidative stress is impaired in spinocerebellar ataxia type 3

J Araujo, P Breuer, S Dieringer, S Krauss… - Human molecular …, 2011 - academic.oup.com
Abstract Ataxin-3 (ATXN3), the disease protein in spinocerebellar ataxia type 3 (SCA3),
binds to target gene promoters and modulates transcription by interaction with …

Duplication of Atxn1l suppresses SCA1 neuropathology by decreasing incorporation of polyglutamine-expanded ataxin-1 into native complexes

AB Bowman, YC Lam, P Jafar-Nejad, HK Chen… - Nature …, 2007 - nature.com
Abstract Spinocerebellar ataxia type 1 (SCA1) is a dominantly inherited neurodegenerative
disease caused by expansion of a glutamine tract in ataxin-1 (ATXN1). SCA1 pathogenesis …

Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence

U Bichelmeier, T Schmidt, J Hübener, J Boy… - Journal of …, 2007 - Soc Neuroscience
Spinocerebellar ataxia type 3 (SCA3) is an autosomal dominantly inherited
neurodegenerative disorder caused by the expansion of a CAG repeat in the MJD1 gene …

Gene expression profiling in ataxin-3 expressing cell lines reveals distinct effects of normal and mutant ataxin-3

BO Evert, IR Vogt, AM Vieira-Saecker… - … of Neuropathology & …, 2003 - academic.oup.com
Abstract Spinocerebellar ataxia type 3 (SCA3) is a late-onset neurodegenerative disorder
caused by the expansion of a polyglutamine tract within the gene product, ataxin-3. We have …

[HTML][HTML] Ataxin-3 is a histone-binding protein with two independent transcriptional corepressor activities

F Li, T Macfarlan, RN Pittman, D Chakravarti - Journal of Biological …, 2002 - ASBMB
The mechanisms of pathology for the family of polyglutamine disease proteins are unknown;
however, recently it was shown that several of these proteins inhibit transcription suggesting …

[HTML][HTML] RORα-mediated Purkinje cell development determines disease severity in adult SCA1 mice

HG Serra, L Duvick, T Zu, K Carlson, S Stevens… - Cell, 2006 - cell.com
Summary Spinocerebellar ataxia type 1 (SCA1) is one of nine inherited, typically adult onset,
polyglutamine neurodegenerative diseases. To examine whether development impacts …

Structural basis of protein complex formation and reconfiguration by polyglutamine disease protein Ataxin-1 and Capicua

E Kim, HC Lu, HY Zoghbi, JJ Song - Genes & development, 2013 - genesdev.cshlp.org
Spinocerebellar ataxia type 1 (SCA1) is a dominantly inherited neurodegenerative disease
caused by polyglutamine expansion in Ataxin-1 (ATXN1). ATXN1 binds to the transcriptional …

[HTML][HTML] The neurodegenerative disease protein ataxin-1 antagonizes the neuronal survival function of myocyte enhancer factor-2

TA Bolger, X Zhao, TJ Cohen, CC Tsai… - Journal of Biological …, 2007 - ASBMB
Ataxin-1 is a neurodegenerative disorder protein whose mutant form causes spinocerebellar
ataxia type-1 (SCA1). Evidence suggests that ataxin-1 may function as a transcription …