Results 31 to 40 of about 13,262,735 (197)

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

Ataxin-3 phosphorylation protects neurons [PDF]

J Cell Biol, 2016
![Figure][1] Expanded ataxin-3 causes an extensive loss of neurons (brown) in rat brains (left). But the amount of neurodegeneration is reduced when serine 12 is mutated to aspartate (right).
europepmc   +3 more sources

Flow cytometry allows rapid detection of protein aggregates in cellular and zebrafish models of spinocerebellar ataxia 3

Disease Models & Mechanisms, 2021
Spinocerebellar ataxia 3 (SCA3, also known as Machado–Joseph disease) is a neurodegenerative disease caused by inheritance of a CAG repeat expansion within the ATXN3 gene, resulting in polyglutamine (polyQ) repeat expansion within the ataxin-3 protein ...
Katherine J. Robinson, Madelaine C. Tym, Alison Hogan, Maxinne Watchon, Kristy C. Yuan, Stuart K. Plenderleith, Emily K. Don, Angela S. Laird   +7 more
doaj   +1 more source

Ataxin‐3 is subject to autolytic cleavage [PDF]

The FEBS Journal, 2006
The protein ataxin‐3 is responsible for spinocerebellar ataxia type 3, a neurodegenerative disease triggered when the length of a stretch of consecutive glutamines exceeds a critical threshold. Different physiologic roles have been suggested for this protein.
Pier Luigi Mauri, Matteo Riva, Daniela Ambu, Antonella De Palma, Francesco Secundo, Louise Benazzi, Marco Valtorta, Paolo Tortora, Paola Fusi   +8 more
openaire   +5 more sources

Neurodegenerative phosphoprotein signaling landscape in models of SCA3

Molecular Brain, 2021
Spinocerebellar ataxia type 3 (SCA3) is a rare neurodegenerative disorder resulting from an aberrant expansion of a polyglutamine stretch in the ataxin-3 protein and subsequent neuronal death. The underlying intracellular signaling pathways are currently
Anna S. Sowa, Taissia G. Popova, Tina Harmuth, Jonasz J. Weber, Priscila Pereira Sena, Jana Schmidt, Jeannette Hübener-Schmid, Thorsten Schmidt   +7 more
doaj   +1 more source

PolyQ-expanded ataxin-3 interacts with full-length ataxin-3 in a polyQ length-dependent manner [PDF]

Neuroscience Bulletin, 2008
Machado-Joseph disease (MJD), also known as spinocerebellar ataxia type 3 (SCA3), is a dominant neurodegenerative disorder caused by an expansion of the polyglutamine (polyQ) tract in MJD-1 gene product, ataxin-3 (AT3). This disease is characterized by the formation of intraneuronal inclusions, but the mechanism underlying their formation is still ...
Na-Li, Jia, Er-Kang, Fei, Zheng, Ying, Hong-Feng, Wang, Guang-Hui, Wang   +4 more
openaire   +2 more sources

Proteolytic cleavage of polyglutamine-expanded ataxin-3 is critical for aggregation and sequestration of non-expanded ataxin-3 [PDF]

Human Molecular Genetics, 2006
Spinocerebellar ataxia type 3 (SCA3), like other polyglutamine (polyQ) diseases, is characterized by the formation of intraneuronal inclusions, but the mechanism underlying their formation is poorly understood. Here, we tested the "toxic fragment hypothesis", which predicts that proteolytic production of polyQ-containing fragments from the full-length ...
Haacke, A., Broadley, S., Boteva, R., Tzvetkov, N., Hartl, F., Breuer, P.   +5 more
openaire   +3 more sources

Mutant Ataxin-2 Expression in Aged Animals Aggravates Neuropathological Features Associated with Spinocerebellar Ataxia Type 2 [PDF]

, 2022
Spinocerebellar ataxia type 2 (SCA2) is a rare autosomal, dominantly inherited disease, in which the affected individuals have a disease onset around their third life decade. The molecular mechanisms underlying SCA2 are not yet completely understood, for
André Conceição, Afonso, Inês T., Inês T. Afonso, Clévio Nóbrega, Carlos A. Matos, Matos, Carlos A., Matos, Carlos A, Patrícia Lima, Conceição, André, Lima, Patrícia, Nóbrega, Clévio   +10 more
core   +1 more source

Ataxin-1 fusion partners alter polyQ lethality and aggregation [PDF]

, 2007
Intranuclear inclusion bodies (IBs) are the histopathologic markers of multiple protein folding diseases. IB formation has been extensively studied using fluorescent fusion products of pathogenic polyglutamine (polyQ) expressing proteins.
Rich, T., Varadaraj, A., Archana Varadaraj, Varadaraj Archana, Tina Rich, Rich Tina   +5 more
core   +1 more source

Deranged calcium signaling and neurodegeneration in spinocerebellar ataxia type 3 [PDF]

, 2008
Spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph disease (MJD), is an autosomal-dominant neurodegenerative disorder caused by a polyglutamine expansion in ataxin-3 (SCA3, MJD1) protein.
X. Chen, Bezprozvanny, I, Nelson, O, Hammer, R, Tu, H, Nukina, N, O. Nelson, N. Nukina, Chen, X, Tang, TS, T.-S. Tang, Pook, M, H. Tu, R. Hammer, I. Bezprozvanny, M. Pook   +15 more
core   +1 more source