Results 121 to 130 of about 13,262,735 (197)

CDK5 protects from caspase-induced Ataxin-3 cleavage and neurodegeneration

, 2014
Spinocerebellar ataxia type 3 (SCA3) is one of at least nine inherited neurodegenerative diseases caused by an expansion of a polyglutamine tract within corresponding disease-specific proteins. In case of SCA3, mutation of Ataxin-3 results in aggregation
Karch, A., Schulz, J. B., Voßfeldt, Hannes, Deeg, Sebastian, Dohm, Christoph P., Weishaupt, Jochen, Kermer, Pawel, Liman, Jan, Deeg, S., Bähr, M., Bähr, Mathias, Schulz, Jörg B., Kermer, P., Karch, André, Voigt, Aaron, Voigt, A., Dohm, C. P., Voßfeldt, H.   +17 more
core   +1 more source

Label-free autofluorescence lifetime reveals the structural dynamics of ataxin-3 inside droplets formed via liquid-liquid phase separation. [PDF]

Sci Rep, 2023
Matsuura U, Tahara S, Kajimoto S, Nakabayashi T.   +3 more
europepmc   +1 more source

Stabilization and Degradation Mechanisms of Cytoplasmic Ataxin-1

, 2015
Aggregation-prone proteins in neurodegenerative disease disrupt cellular protein stabilization and degradation pathways. The neurodegenerative disease spinocerebellar ataxia type 1 (SCA1) is caused by a coding polyglutamine expansion in the Ataxin-1 gene
Mayumi F. Kohiyama, Sarita Lagalwar
core   +1 more source

KPNB1 modulates the Machado-Joseph disease protein ataxin-3 through activation of the mitochondrial protease CLPP. [PDF]

Cell Mol Life Sci, 2022
Abeditashi M, Weber JJ, Pereira Sena P, Velic A, Kalimeri M, Incebacak Eltemur RD, Schmidt J, Hübener-Schmid J, Hauser S, Macek B, Riess O, Schmidt T.   +11 more
europepmc   +1 more source

Pathophysiological interplay between O-GlcNAc transferase and the Machado-Joseph disease protein ataxin-3. [PDF]

Proc Natl Acad Sci U S A, 2021
Pereira Sena P, Weber JJ, Watchon M, Robinson KJ, Wassouf Z, Hauser S, Helm J, Abeditashi M, Schmidt J, Hübener-Schmid J, Schöls L, Laird AS, Riess O, Schmidt T.   +13 more
europepmc   +1 more source

Erinacine A-Enriched Hericium erinaceus Mycelium Ethanol Extract Lessens Cellular Damage in Cell and Drosophila Models of Spinocerebellar Ataxia Type 3 by Improvement of Nrf2 Activation

Antioxidants
Spinocerebellar ataxia type 3 (SCA3), caused by the abnormal expansion of polyglutamine (polyQ) in the ataxin-3 protein, is one of the inherited polyQ neurodegenerative diseases that share similar genetic and molecular features.
Yu-Ling Wu, Hai-Lun Sun, Jui-Chih Chang, Wei-Yong Lin, Pei-Yin Chen, Chin-Chu Chen, Li-Ya Lee, Chien-Chun Li, Mingli Hsieh, Haw-Wen Chen, Ya-Chen Yang, Chin-San Liu, Kai-Li Liu   +12 more
doaj   +1 more source

ePoster

European Journal of Neurology, Volume 33, Issue S1, June 2026.
wiley   +1 more source

Ubiquitin-binding site 1 of pathogenic ataxin-3 regulates its toxicity in <i>Drosophila</i> models of Spinocerebellar Ataxia Type 3. [PDF]

Front Neurosci, 2022
Prifti MV, Libohova K, Harris AL, Tsou WL, Todi SV.   +4 more
europepmc   +1 more source

ePosters Virtual

European Journal of Neurology, Volume 33, Issue S1, June 2026.
wiley   +1 more source

3UIM ataxin-3 is the predominant protein isoform in murine and human brain tissue.

, 2013
(A) Diagram of 2UIM (upper) and 3UIM (lower) ataxin-3 variants showing recognition sites for 1H9 mAb, which recognizes both isoforms, and α-ataxin-3C, which recognizes only 3UIM ataxin-3.
Katerina Dodelzon (360474), Ginny Marie Harris (360473), Pedro Gonzalez-Alegre (312771), Henry L. Paulson (136809), Lijie Gong (360475)   +4 more
core   +1 more source