Results 71 to 80 of about 9,929 (238)

RNA toxicity is a component of ataxin-3 degeneration in Drosophila [PDF]

Nature, 2008
Polyglutamine (polyQ) diseases are a class of dominantly inherited neurodegenerative disorders caused by the expansion of a CAG repeat encoding glutamine within the coding region of the respective genes. The molecular and cellular pathways underlying polyQ-induced neurodegeneration are the focus of much research, and it is widely considered that toxic ...
Ling-Bo, Li, Zhenming, Yu, Xiuyin, Teng, Nancy M, Bonini   +3 more
openaire   +2 more sources

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

Striatal and nigral pathology in a lentiviral rat model of Machado-Joseph disease [PDF]

, 2017
Machado-Joseph disease (MJD) is a fatal, dominant neurodegenerative disorder. MJD results from polyglutamine repeat expansion in the MJD-1 gene, conferring a toxic gain of function to the ataxin-3 protein. In this study, we aimed at overexpressing ataxin-
Alves, Sandro, Brouillet, Emmanuel, Carvalho, Ana Luísa, de Almeida, Luís Pereira, de Lima, Maria C. Pedroso, Dufour, Noelle, Déglon, Nicole, Gould, Veronica Colomer, Hassig, Raymonde, Koeppen, Arnulf, Nascimento-Ferreira, Isabel, Régulier, Etienne, Simões, Sérgio   +12 more
core  

Combined therapy with m-TOR-dependent and -independent autophagy inducers causes neurotoxicity in a mouse model of Machado-Joseph disease [PDF]

, 2016
A major pathological hallmark in several neurodegenerative disorders, like polyglutamine disorders (polyQ), including Machado-Joseph disease (MJD), is the formation of protein aggregates.
Carvalho, Andreia Alexandra Neves, Castro, Andreia Cristiana Teixeira, Cunha, Carina Isabel Soares, Fernandes, Anabela Silva, Maciel, P., Silva, Sara Carina Duarte   +5 more
core   +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

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

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

Calpastatin-mediated inhibition of calpains in the mouse brain prevents mutant ataxin 3 proteolysis, nuclear localization and aggregation, relieving Machado-Joseph disease [PDF]

, 2017
Machado-Joseph disease is the most frequently found dominantly-inherited cerebellar ataxia. Over-repetition of a CAG trinucleotide in the MJD1 gene translates into a polyglutamine tract within the ataxin 3 protein, which upon proteolysis may trigger ...
Duarte, Carlos Bandeira, Déglon, Nicole, Gonçalves, Nélio, Koeppen, Arnulf, Kügler, Sebastian, Pereira de Almeida, Luís, Simões, Ana T.   +6 more
core  

Modulation of the Stress Granule Component Carhsp1 Mitigates Disease‐Associated Deficits in Spinocerebellar Ataxia Type 3 Mouse Models

Movement Disorders, EarlyView.
Abstract Background Spinocerebellar ataxia type 3 (SCA3) is a polyglutamine (polyQ) neurogenerative disorder that results from CAG trinucleotide repeat expansions in the ATXN3 gene, leading to toxic protein aggregate formation and cellular pathway dysfunction.
Tiago Moreira‐Gomes, Adriana Marcelo, Ana Teresa Rajado, Rafael G. Costa, Ricardo Afonso‐Reis, Cristiana R. Madeira, Inês T. Afonso, David V.C. Brito, Adriana A. Vaz, Clévio Nóbrega   +9 more
wiley   +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