Results 131 to 140 of about 9,929 (238)

Dominant negative effect of polyglutamine expansion perturbs normal function of ataxin-3 in neuronal cells [PDF]

, 2015
The physiological function of Ataxin-3 (ATXN3), a deubiquitylase (DUB) involved in Machado–Joseph Disease (MJD), remains elusive. In this study, we demonstrate that ATXN3 is required for neuronal differentiation and for normal cell morphology ...
Carvalho, Andreia Alexandra Neves, Costa, Maria do Carmo, Fernandes, Anabela Silva, Freitas, Ana, Heutink, Peter, Logarinho, Elsa, Lopes, André Teixeira, Maciel, P., Martins, Margarida Isabel Barros Coelho, Paulson, Henry L., Relvas, João B., Serra, Sofia Cravino, Silva, Sara Duarte   +12 more
core   +1 more source

Electroencephalogram paroxysmal theta characterizes cataplexy in mice and children [PDF]

, 2017
Astute control of brain activity states is critical for adaptive behaviours and survival. In mammals and birds, electroencephalographic recordings reveal alternating states of wakefulness, slow wave sleep and paradoxical sleep (or rapid eye movement ...
Dellepiane, Jose M., Emmenegger, Yann, Franken, Paul, Jimenez, Sonia, Plazzi, Giuseppe, Tafti, Mehdi, Vandi, Stefano, Vassalli, Anne   +7 more
core  

Overexpression of the autophagic beclin-1 protein clears mutant ataxin-3 and alleviates Machado–Joseph disease [PDF]

, 2011
Isabel Nascimento-Ferreira, Tiago Santos‐Ferreira, Lígia Sousa-Ferreira, Gwennaëlle Aurégan, Isabel Onofre, Sandro Alves, Noëlle Dufour, Veronica F. Colomer Gould, Arnulf H. Koeppen, Nicole Déglon, Luís Pereira de Almeida   +10 more
openalex   +1 more source

Inactivation of PNKP by mutant ATXN3 triggers apoptosis by activating the DNA damage-response pathway in SCA3. [PDF]

, 2015
Spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph disease (MJD), is an untreatable autosomal dominant neurodegenerative disease, and the most common such inherited ataxia worldwide.
A Basu, A Bharti, A Durr, A Silva-Fernandes, A-H Chou, A-H Chou, Adriana Paulucci-Holthauzen, AG Mason, AL Castel, Anabela Silva-Fernandes, Arnulf H. Koeppen, Arpita Chatterjee, B Halliwell, BJ Bae, BJ Winborn, C Culmsee, C Du, C Poulton, CA Matos, Christopher E. Pearson, DM Wilson, E Oda, E Rodriguez-Lebron, EU Kurz, F Coppede, FD Miller, H Date, H Takashima, Hang L. Zhang, HL Wang, HY Zoghbi, I Schmitt, I Weibrecht, J Shen, JE Chipuk, JE Chipuk, JJ Reynolds, JR Chang, JS Fridman, K Nakano, K Yoshida, K Yoshida, L Weissman, LJ Martin, LS Pacheco, M Weinfeld, MC Moreira, MC Polidori, MD Costa, ML Fishel, NA Di Prospero, Partha S. Sarkar, Patricia Maciel, R Alpatov, R Baskaran, RL Rolig, RN Rosenberg, Rui Gao, S Kharbanda, Sanjeev Choudhary, SK Muller, SM Mandal, SP Cregan, SP LeDoux, SV Todi, SW Ryter, T Klockgether, T Kobayashi, T Shafman, T Shibue, Tapas K. Hazra, Tetsuo Ashizawa, Tohru Matsuura, TS Adwan, W Liu, W Zhang, W-Y Wang, Xiang Fang, Y Kawaguchi, Y Lee, Y Takiyama, YJ Shyu, Yongping Liu, Z Guo, ZM Yuan   +84 more
core   +3 more sources

Allosteric regulation of deubiquitylase activity through ubiquitination

Frontiers in Molecular Biosciences, 2015
Ataxin-3, the protein responsible for spinocerebellar ataxia type-3, is a cysteine protease that specifically cleaves poly-ubiquitin chains and participates in the ubiquitin proteasome pathway.
Serena eFaggiano, Rajesh P. Menon, Geoff P. Kelly, Sokol V. Todi, K. Matthew Scaglione, Petr V. Konarev, Petr V. Konarev, Dmitri I. Svergun, Henry L. Paulson, Annalisa ePastore   +9 more
doaj   +1 more source

Mode of substrate recognition by the Josephin domain of ataxin‐3, which has an endo‐type deubiquitinase activity [PDF]

, 2014
Tadashi Satoh, Akira Sumiyoshi, Maho Yagi‐Utsumi, Eri Sakata, Hiroaki Sasakawa, Eiji Kurimoto, Yoshiki Yamaguchi, Wei Li, Claudio A.P. Joazeiro, Takatsugu Hirokawa, Koichi Kato   +10 more
openalex   +1 more source

Lysine 117 on ataxin-3 modulates toxicity in Drosophila models of Spinocerebellar Ataxia Type 3. [PDF]

J Neurol Sci, 2023
Blount JR, Patel NC, Libohova K, Harris AL, Tsou WL, Sujkowski A, Todi SV.   +6 more
europepmc   +1 more source

High throughput compound screening in neuronal cells identifies statins as activators of ataxin 3 expression. [PDF]

Sci Rep, 2023
Stahl F, Schmitt I, Denner P, de Boni L, Wüllner U, Breuer P.   +5 more
europepmc   +1 more source

High level expression of expanded full-length ataxin-3 in vitro causes cell death and formation of intranuclear inclusions in neuronal cells [PDF]

, 1999
Bernd O. Evert
openalex   +1 more source

Reply to: “Comment on: Polyglutamine‐Expanded Ataxin‐3: A Target Engagement Marker for Spinocerebellar Ataxia Type 3 in Peripheral Blood” [PDF]

, 2022
Jeannette Hübener‐Schmid, Kirsten Kuhlbrodt, Julien Peladan, European Spinocerebellar Ataxia Type 3/Machado‐Joseph Disease Initiative (ESMI) Study Group, Olaf Rieß   +4 more
openalex   +1 more source