Results 61 to 70 of about 13,262,735 (197)

Basal and stress-induced Hsp70 are modulated by ataxin-3 [PDF]

Cell Stress and Chaperones, 2012
Regulation of basal and induced levels of hsp70 is critical for cellular homeostasis. Ataxin-3 is a deubiquitinase with several cellular functions including transcriptional regulation and maintenance of protein homeostasis. While investigating potential roles of ataxin-3 in response to cellular stress, it appeared that ataxin-3 regulated hsp70.
Reina, Christopher P., Nabet, Barzin Y., Young, Peter D., Pittman, Randall N.   +3 more
openaire   +2 more sources

Neurochemical Endpoints to Inform Early‐Stage Trials of Spinocerebellar Ataxia 2 and 3 in a Multisite Setting

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Neurochemical levels measured by brain MR spectroscopy (MRS) have been proposed as endpoints for clinical trials in early‐stage spinocerebellar ataxia (SCA) trials. We tested their trial‐readiness by quantifying neurochemicals in three affected brain regions in early‐stage cohorts of SCA2 and SCA3, examining their reproducibility in ...
James M. Joers, Yaqiao Wei, Dinesh K. Deelchand, Adam Berrington, Young Woo Park, Guita Banan, Michal Povazan, Jens T. Rosenberg, André J. W. van der Kouwe, Tetsuo Ashizawa, Khalaf O. Bushara, Chiadi U. Onyike, Sub H. Subramony, Jeremy D. Schmahmann, Brian J. Soher, Peter B. Barker, Thomas H. Mareci, Eva‐Maria Ratai, Lynn E. Eberly, Gülin Öz   +19 more
wiley   +1 more source

Ataxin-2 interacts with FUS and intermediate-length polyglutamine expansions enhance FUS-related pathology in amyotrophic lateral sclerosis

, 2022
Fused in sarcoma (FUS) is mutated in both sporadic amyotrophic lateral sclerosis (ALS) and familial ALS patients. The mechanisms underlying neurodegeneration are not fully understood, but FUS redistributes from the nucleus to the cytoplasm in affected ...
K Y Soo (13504855), I P Blair (13377654), J D Atkin (13433565), S T Warraich (13504861), Vinod Sundaramoorthy (13096605), M A Farg (13504867)   +5 more
core  

The deubiquitinase ataxin-3 requires Rad23 and DnaJ-1 for its neuroprotective role in Drosophila melanogaster

Neurobiology of Disease, 2015
Ataxin-3 is a deubiquitinase and polyglutamine (polyQ) disease protein with a protective role in Drosophila melanogaster models of neurodegeneration. In the fruit fly, wild-type ataxin-3 suppresses toxicity from several polyQ disease proteins, including ...
Wei-Ling Tsou, Michelle Ouyang, Ryan R. Hosking, Joanna R. Sutton, Jessica R. Blount, Aaron A. Burr, Sokol V. Todi   +6 more
doaj   +1 more source

RNA interference mitigates motor and neuropathological deficits in a cerebellar mouse model of Machado-Joseph disease. [PDF]

PLoS ONE, 2014
Machado-Joseph disease or Spinocerebellar ataxia type 3 is a progressive fatal neurodegenerative disorder caused by the polyglutamine-expanded protein ataxin-3. Recent studies demonstrate that RNA interference is a promising approach for the treatment of
Clévio Nóbrega, Isabel Nascimento-Ferreira, Isabel Onofre, David Albuquerque, Nicole Déglon, Luís Pereira de Almeida   +5 more
doaj   +1 more source

Redox‐Dependent Chaperoning of GBF1 Condensates Regulates Seed Germination in Arabidopsis

Advanced Science, EarlyView.
In dormant seeds (low ROS), GBF1 forms liquid condensates to repress the germination gene CathB3, and the chaperone GIP1 maintains condensate liquidity and repressive activity. Upon imbibition (high ROS), ROS oxidize GIP1 during germination, impairing its chaperone function.
Yunying Wang, Xiaofeng Fang
wiley   +1 more source

Modulators of spinocerebellar ataxia type 3-associated ataxin-3 aggregation

, 2016
Spinocerebellar ataxia type-3 (SCA3), also known as Machado-Joseph disease, is proposed to be the most common dominant autosomal ataxia. It is a progressive, fatal neurodegenerative disease with no known cure.
Leck, Su-Ling (3810796), Leck, Su-Ling
core   +1 more source

Polyglutamine-induced neurodegeneration in SCA3 is not mitigated by non-expanded ataxin-3: Conclusions from double-transgenic mouse models

Neurobiology of Disease, 2010
A crucial question in polyQ-induced neurodegeneration is the influence of wild type protein on the formation of aggregates and toxicity. Recently it was shown that non-expanded ataxin-3 protein mitigated neurodegeneration in a Drosophila and mouse model ...
Jeannette Hübener, Olaf Riess
doaj   +1 more source

Novel polyglutamine model uncouples proteotoxicity from aging. [PDF]

PLoS ONE, 2014
Polyglutamine expansions in certain proteins are the genetic determinants for nine distinct progressive neurodegenerative disorders and resultant age-related dementia.
Nakeirah T M Christie, Amy L Lee, Hannah G Fay, Amelia A Gray, Elise A Kikis   +4 more
doaj   +1 more source

HNRNPD Induces Radioresistance in Nasopharyngeal Carcinoma by Sequestering GRAMD4 mRNA in Stress Granules

Advanced Science, EarlyView.
HNRNPD promotes radioresistance in nasopharyngeal carcinoma by enhancing stress granule assembly and sequestering GRAMD4 mRNA. This suppresses GRAMD4 translation and inhibits mitochondrial apoptosis. Targeting the integrated stress response with ISRIB restores GRAMD4 expression and sensitizes tumors to radiotherapy, revealing a translational control ...
Yingzi Li, Tong Xiang, Yuanyuan Liu, Desheng Weng, Jingjing Zhao, Hao Chen, Yan Tang, Songzuo Xie, Hao Zhang, Jun Luo, Xinyi Yang, Qiuzhong Pan, Muping Di, Jianchuan Xia   +13 more
wiley   +1 more source