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 …

Spinocerebellar ataxia type 2: clinical presentation, molecular mechanisms, and therapeutic perspectives

JJ Magana, L Velázquez-Pérez, B Cisneros - Molecular neurobiology, 2013 - Springer
Abstract Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant genetic disease
characterized by cerebellar dysfunction associated with slow saccades, early hyporeflexia …

Comparison of spinocerebellar ataxia type 3 mouse models identifies early gain-of-function, cell-autonomous transcriptional changes in oligodendrocytes

B Ramani, B Panwar, LR Moore, B Wang… - Human molecular …, 2017 - academic.oup.com
Abstract Spinocerebellar ataxia type 3 (SCA3) is a neurodegenerative disorder caused by a
polyglutamine-encoding CAG repeat expansion in the ATXN3 gene. This expansion leads to …

[HTML][HTML] NEDD8: a new ataxin-3 interactor

A Ferro, AL Carvalho, A Teixeira-Castro… - … et Biophysica Acta (BBA …, 2007 - Elsevier
Machado–Joseph disease (MJD/SCA3) is an autosomal dominant neurodegenerative
disease caused by the expansion of a CAG tract in the coding portion of the ATXN3 gene …

Ataxin-10, the spinocerebellar ataxia type 10 neurodegenerative disorder protein, is essential for survival of cerebellar neurons

P März, A Probst, S Lang, M Schwager… - Journal of Biological …, 2004 - ASBMB
Spinocerebellar ataxia (SCA) type 10, an autosomal dominant disease characterized by
cerebellar ataxia, is caused by a novel pentanucleotide (ATTCT) repeat expansion in the …

USP7, a ubiquitin-specific protease, interacts with ataxin-1, the SCA1 gene product

S Hong, SJ Kim, S Ka, I Choi, S Kang - Molecular and Cellular …, 2002 - Elsevier
Spinocerebellar ataxia type 1 (SCA1) is an autosomal-dominant neurodegenerative
disorder characterized by ataxia and progressive motor deterioration. SCA1 has been …

Transcriptional profiling and biomarker identification reveal tissue specific effects of expanded ataxin-3 in a spinocerebellar ataxia type 3 mouse model

LJA Toonen, M Overzier, MM Evers, LG Leon… - Molecular …, 2018 - Springer
Background Spinocerebellar ataxia type 3 (SCA3) is a progressive neurodegenerative
disorder caused by expansion of the polyglutamine repeat in the ataxin-3 protein …

Ataxin-2 Regulates RGS8 Translation in a New BAC-SCA2 Transgenic Mouse Model

W Dansithong, S Paul, KP Figueroa, MD Rinehart… - PLoS …, 2015 - journals.plos.org
Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant disorder with progressive
degeneration of cerebellar Purkinje cells (PCs) and other neurons caused by expansion of a …

The Machado–Joseph disease deubiquitinase ataxin-3 regulates the stability and apoptotic function of p53

H Liu, X Li, G Ning, S Zhu, X Ma, X Liu, C Liu… - PLoS …, 2016 - journals.plos.org
As a deubiquitinating enzyme (DUB), the physiological substrates of ataxin-3 (ATX-3)
remain elusive, which limits our understanding of its normal cellular function and that of …

Deranged calcium signaling and neurodegeneration in spinocerebellar ataxia type 2

J Liu, TS Tang, H Tu, O Nelson, E Herndon… - Journal of …, 2009 - Soc Neuroscience
Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominantly inherited,
neurodegenerative disease caused by an expansion of polyglutamine tracts in the cytosolic …