Results 51 to 60 of about 9,929 (238)

Silencing ataxin-3 mitigates degeneration in a rat model of Machado–Joseph disease: no role for wild-type ataxin-3? [PDF]

Human Molecular Genetics, 2010
Machado-Joseph disease or spinocerebellar ataxia type 3 (MJD/SCA3) is a fatal, autosomal dominant disorder caused by a cytosine-adenine-guanine expansion in the coding region of the MJD1 gene. RNA interference has potential as a therapeutic approach but raises the issue of the role of wild-type ataxin-3 (WT ATX3) in MJD and of whether the expression of
Sandro, Alves, Isabel, Nascimento-Ferreira, Noëlle, Dufour, Raymonde, Hassig, Gwennaëlle, Auregan, Clévio, Nóbrega, Emmanuel, Brouillet, Philippe, Hantraye, Maria C, Pedroso de Lima, Nicole, Déglon, Luís Pereira, de Almeida   +10 more
openaire   +2 more sources

SUMO-1 modification on K166 of polyQ-expanded ataxin-3 strengthens its stability and increases its cytotoxicity. [PDF]

PLoS ONE, 2013
Post-translational modification by SUMO was proposed to modulate the pathogenesis of several neurodegenerative diseases. Spinocerebellar ataxia type 3/Machado-Joseph disease (SCA3/MJD) is an autosomal dominant neurodegenerative disease caused by polyQ ...
Ya-Fang Zhou, Shu-Sheng Liao, Ying-Ying Luo, Jian-Guang Tang, Jun-Ling Wang, Li-Fang Lei, Jing-Wei Chi, Juan Du, Hong Jiang, Kun Xia, Bei-Sha Tang, Lu Shen   +11 more
doaj   +1 more source

Mutant ataxin-3 promotes the autophagic degradation of parkin [PDF]

Autophagy, 2011
There is growing evidence that autophagy plays a key role in neurodegenerative diseases. For instance, stimulating autophagy is neuroprotective both in vitro and in vivo in models of trinucleotide-repeat diseases such as Machado-Joseph disease (MJD). Similarly, proteins associated with familial forms of Parkinson disease (PD) such as parkin and PINK1 ...
Thomas M, Durcan, Edward A, Fon
openaire   +2 more sources

Antibody Characterization Report for Ataxin-3

, 2021
Head-to-head comparison of available commercial antibodies against Ataxin-3 by immunoblot (Western blot), immunoprecipitation and immunofluorescence. The following study was funded in part by Genome Québec's Genomics Integration Program, awarded to the research laboratory of Peter S. McPherson.
Villegas, Lorenza, Alshafie, Walaa, Fotouhi, Maryam, You, Zhipeng, Durcan, Thomas M., McPherson, Peter S., Laflamme, Carl   +6 more
openaire   +2 more sources

Ataxin-3 is transported into the nucleus and associates with the nuclear matrix [PDF]

Human Molecular Genetics, 1998
It has been reported that the ataxin-3 protein containing a polyglutamine sequence in the pathological range (61-84Q) is localized within the nucleus of neuronal cells, whereas ataxin-3 with a normal repeat length (12-37Q) is predominantly a cytoplasmic protein.
D. Tait, RICCIO, Massimo, A. Sittler, E. Scherzinger, S. Santi, A. Ognibene, N. M. Maraldi, H. Lehrach, E. E. Wanker   +8 more
openaire   +2 more sources

Ataxin-3 promotes genome integrity by stabilizing Chk1. [PDF]

Nucleic Acids Res, 2017
The Chk1 protein is essential for genome integrity maintenance and cell survival in eukaryotic cells. After prolonged replication stress, Chk1 can be targeted for proteasomal degradation to terminate checkpoint signaling after DNA repair finishes. To ensure proper activation of DNA damage checkpoint and DNA repair signaling, a steady-state level of ...
Tu Y, Liu H, Zhu X, Shen H, Ma X, Wang F, Huang M, Gong J, Li X, Wang Y, Guo C, Tang TS.   +11 more
europepmc   +4 more sources

RBR E3 ubiquitin ligases: new structures, new insights, new questions [PDF]

, 2014
The RBR (RING-BetweenRING-RING) or TRIAD [two RING fingers and a DRIL (double RING finger linked)] E3 ubiquitin ligases comprise a group of 12 complex multidomain enzymes.
Shaw, Gary S., Spratt, Donald E., Walden, Helen   +2 more
core   +1 more source

Proteotoxic stress increases nuclear localization of ataxin-3 [PDF]

Human Molecular Genetics, 2009
Spinocerebellar ataxia type 3 (SCA3)/Machado Joseph disease results from expansion of the polyglutamine domain in ataxin-3 (Atx3). Atx3 is a transcriptional co-repressor, as well as a deubiquitinating enzyme that appears to function in cellular pathways involved in protein homeostasis.
Christopher P, Reina, Xiaoyan, Zhong, Randall N, Pittman   +2 more
openaire   +2 more sources

HDAC inhibitor sodium butyrate reverses transcriptional downregulation and ameliorates ataxic symptoms in a transgenic mouse model of SCA3

Neurobiology of Disease, 2011
Spinocerebellar ataxia type 3 (SCA3) is an autosomal dominant neurodegenerative disease caused by polyglutamine-expanded ataxin-3. Previously, we prepared a SCA3 animal model by generating transgenic mice expressing disease-causing ataxin-3-Q79.
An-Hsun Chou, Si-Ying Chen, Tu-Hsueh Yeh, Yi-Hsin Weng, Hung-Li Wang   +4 more
doaj   +1 more source

Impaired Nuclear Export of Polyglutamine-Expanded Androgen Receptor in Spinal and Bulbar Muscular Atrophy. [PDF]

, 2019
Spinal and bulbar muscular atrophy (SBMA) is a neuromuscular disease caused by polyglutamine (polyQ) expansion in the androgen receptor (AR). Prior studies have highlighted the importance of AR nuclear localization in SBMA pathogenesis; therefore, in ...
Arnold, Frederick J., Merry, Diane E., Pluciennik, Anna   +2 more
core   +3 more sources