Results 141 to 150 of about 19,027,943 (182)

Molecular clearance of ataxin-3 is regulated by a mammalian E4 [PDF]

EMBO Journal, 2004
Insoluble aggregates of polyglutamine-containing proteins are usually conjugated with ubiquitin in neurons of individuals with polyglutamine diseases. We now show that ataxin-3, in which the abnormal expansion of a polyglutamine tract is responsible for spinocerebellar ataxia type 3 (SCA3), undergoes ubiquitylation and degradation by the proteasome ...
Shoji Tsuji, Teiichi Tanimura, Shigetsugu Hatakeyama   +2 more
exaly   +3 more sources

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Temperature profoundly affects ataxin-3 fibrillogenesis

Biochimie, 2012
Ataxin-3 (AT3) triggers spinocerebellar ataxia type 3 when it carries a polyglutamine stretch expanded beyond a critical threshold. By Fourier transform infrared spectroscopy and atomic force microscopy we previously showed that a normal (AT3Q24) and an expanded (AT3Q55) variant were capable of evolving into oligomers and protofibrils at 37 °C, whereas
A. Apicella, A. Natalello, A. M. Frana, A. Baserga, CASARI, CARLO SPARTACO, BOTTANI, CARLO ENRICO, S. M. Doglia, P. Tortora, M. E. Regonesi   +8 more
openaire   +3 more sources

Comment on: Polyglutamine‐Expanded Ataxin‐3: A Target Engagement Marker for Spinocerebellar Ataxia Type 3 in Peripheral Blood

Movement Disorders, 2022
Spinocerebellar ataxia 3 (SCA3) is a rare movement disorder for which there is no cure or effective treatment. In the November 2021 issue of Movement Disorders, HübenerSchmid et al published a brief report regarding the development of a single molecule ...
R. Hanna Al-Shaikh, Karen R. Jansen-West, L. Petrucelli, Z. Wszolek, M. Prudencio   +4 more
semanticscholar   +1 more source

Structural and functional analysis of ataxin‐2 and ataxin‐3

European Journal of Biochemistry, 2004
Spinocerebellar ataxia types 2 (SCA2) and 3 (SCA3) are autosomal‐dominantly inherited, neurodegenerative diseases caused by CAG repeat expansions in the coding regions of the genes encoding ataxin‐2 and ataxin‐3, respectively. To provide a rationale for further functional experiments, we explored the protein architectures of ataxin‐2 and ataxin‐3 ...
Albrecht, M., Golatta, M., Wüllner, U., Lengauer, T.   +3 more
openaire   +3 more sources

Deciphering deubiquitinating enzyme Ataxin-3 as an emerging target for cancer intervention.

International Journal of Biological Macromolecules
Ataxin-3 (ATXN3), encoded on chromosome 14q21, is a cysteine protease and deubiquitinating enzyme historically linked to the neurodegenerative Machado-Joseph disease (MJD). Emerging evidence now implicates ATXN3 in various aspects of cancer pathogenesis,
Adnan Khan, L. Bhatt
semanticscholar   +1 more source

Identification of the calpain‐generated toxic fragment of ataxin‐3 protein provides new avenues for therapy of Machado–Joseph disease| Spinocerebellar ataxia type 3

Neuropathology and Applied Neurobiology, 2021
Machado–Joseph disease (MJD) is the most frequent dominantly inherited cerebellar ataxia worldwide. Expansion of a CAG trinucleotide in the MJD1 gene translates into a polyglutamine tract within ataxin‐3, which upon proteolysis may lead to MJD.
A. Simões, V. Carmona, Joana Duarte-Neves, Janete Cunha-Santos, L. Pereira de Almeida   +4 more
semanticscholar   +1 more source

Aggregation of the polyglutamine protein ataxin-3

2021
This thesis was scanned from the print manuscript for digital preservation and is copyright the author. Researchers can access this thesis by asking their local university, institution or public library to make a request on their behalf. Monash staff and postgraduate students can use the link in the References field.
openaire   +1 more source

PML-II recruits ataxin-3 to PML-NBs and inhibits its deubiquitinating activity.

Biochemical and Biophysical Research Communications - BBRC, 2021
Promyelocytic leukemia protein (PML) nuclear bodies (NBs) are dynamic and multiprotein complexes implicated in a variety of important biochemical events.
Ying Zhang, Anwen Shao, Jun Tang, Yunyun Geng   +3 more
semanticscholar   +1 more source

Dynamic expression of Hsp27 in the presence of mutant ataxin-3

Biochemical and Biophysical Research Communications, 2005
Machado-Joseph disease (MJD)/spinocerebellar ataxia type 3 (SCA3) is an autosomal dominant spinocerebellar degeneration characterized by a wide range of clinical manifestations. The molecular mechanisms underlying the selective neuronal death typical of MJD/SCA3 are unknown.
Wei-Hsiu, Chang, Cemal K, Cemal, Yung-Hsiang, Hsu, Cheng-Liang, Kuo, Nobuyuki, Nukina, Ming-Hong, Chang, Hsiao-Tang, Hu, Chuan, Li, Mingli, Hsieh   +8 more
openaire   +2 more sources

Ataxin 1 and ataxin 3 in neuronal intranuclear inclusion disease

Annals of Neurology, 1999
Neuronal intranuclear inclusion disease (NIID) is a multisystem neurodegenerative disorder characterized by large intranuclear aggregates in neurons of the central and peripheral nervous system. These ubiquitinated intranuclear inclusions are morphologically similar to the intraneuronal aggregates that have been identified in the CAG/polyglutamine ...
A P, Lieberman, J Q, Trojanowski, D G, Leonard, K L, Chen, J L, Barnett, J B, Leverenz, T D, Bird, Y, Robitaille, A, Malandrini, K H, Fischbeck   +9 more
openaire   +3 more sources