Results 21 to 30 of about 9,929 (238)

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   +3 more sources

Ataxin-3 plays a role in mouse myogenic differentiation through regulation of integrin subunit levels. [PDF]

PLoS ONE, 2010
During myogenesis several transcription factors and regulators of protein synthesis and assembly are rapidly degraded by the ubiquitin-proteasome system (UPS).
Maria do Carmo Costa, Fernanda Bajanca, Ana-João Rodrigues, Ricardo J Tomé, Garry Corthals, Sandra Macedo-Ribeiro, Henry L Paulson, Elsa Logarinho, Patrícia Maciel   +8 more
doaj   +3 more sources

Mitochondrial Morphology, Function and Homeostasis Are Impaired by Expression of an N-terminal Calpain Cleavage Fragment of Ataxin-3 [PDF]

Frontiers in Molecular Neuroscience, 2018
Alterations in mitochondrial morphology and function have been linked to neurodegenerative diseases, including Parkinson disease, Alzheimer disease and Huntington disease.
Tina Harmuth, Tina Harmuth, Tina Harmuth, Caroline Prell-Schicker, Jonasz J. Weber, Jonasz J. Weber, Frank Gellerich, Claudia Funke, Stefan Drießen, Janine C. D. Magg, Janine C. D. Magg, Guido Krebiehl, Hartwig Wolburg, Stefanie N. Hayer, Stefanie N. Hayer, Stefan Hauser, Rejko Krüger, Rejko Krüger, Ludger Schöls, Ludger Schöls, Olaf Riess, Olaf Riess, Jeannette Hübener-Schmid, Jeannette Hübener-Schmid   +23 more
doaj   +2 more sources

Differential toxicity of ataxin-3 isoforms in Drosophila models of Spinocerebellar Ataxia Type 3 [PDF]

Neurobiology of Disease, 2019
The most commonly inherited dominant ataxia, Spinocerebellar Ataxia Type 3 (SCA3), is caused by a CAG repeat expansion that encodes an abnormally long polyglutamine (polyQ) repeat in the disease protein ataxin-3, a deubiquitinase.
Sean L. Johnson, Jessica R. Blount, Kozeta Libohova, Bedri Ranxhi, Henry L. Paulson, Wei-Ling Tsou, Sokol V. Todi   +6 more
doaj   +2 more sources

Identification and functional dissection of localization signals within ataxin-3

Neurobiology of Disease, 2009
Spinocerebellar ataxia type 3 (SCA3) or Machado–Joseph disease (MJD) belongs to a group of autosomal dominant neurodegenerative diseases, which are caused by the expansion of a polyglutamine repeat in the affected protein, in this case ataxin-3. Ataxin-3
Paul Michel Aloyse Antony, Simone Mäntele, Phillip Mollenkopf, Jana Boy, Ralph H. Kehlenbach, Olaf Riess, Thorsten Schmidt   +6 more
doaj   +4 more sources

The Deubiquitinating Enzyme Ataxin-3 Regulates Ciliogenesis and Phagocytosis in the Retina. [PDF]

Cell Rep, 2020
Toulis V, García-Monclús S, de la Peña-Ramírez C, Arenas-Galnares R, Abril JF, Todi SV, Khan N, Garanto A, Costa MDC, Marfany G.   +9 more
europepmc   +2 more sources

The Machado-Joseph Disease Deubiquitinase Ataxin-3 Regulates the Stability and Apoptotic Function of p53. [PDF]

PLoS Biol, 2016
Liu H, Li X, Ning G, Zhu S, Ma X, Liu X, Liu C, Huang M, Schmitt I, Wüllner U, Niu Y, Guo C, Wang Q, Tang TS.   +13 more
europepmc   +3 more sources

Gene Expression Profiling in Ataxin-3 Expressing Cell Lines Reveals Distinct Effects of Normal and Mutant Ataxin-3 [PDF]

Journal of Neuropathology & Experimental Neurology, 2003
Spinocerebellar ataxia type 3 (SCA3) is a late-onset neurodegenerative disorder caused by the expansion of a polyglutamine tract within the gene product, ataxin-3. We have previously shown that mutant ataxin-3 causes upregulation of inflammatory genes in transgenic SCA3 cell lines and human SCA3 pontine neurons.
Bernd O. Evert, Ina R. Vogt, Ana Vieira‐Saecker, Lucia Ozimek, Rob A. I. de Vos, E. R. Brunt, Thomas Klockgether, Ullrich Wüllner   +7 more
openalex   +4 more sources

Roles of orexinergic and noradrenergic neuronal activity in ketamine-induced analgesia: A study using an orexin/ataxin-3 transgenic rat model. [PDF]

J Anesth Transl Med
Tonosaki M, Kushikata T, Nikaido Y, Takekawa D, Kinoshita H, Saito J, Hirota K.   +6 more
europepmc   +2 more sources

Physiological and pathophysiological characteristics of ataxin-3 isoforms. [PDF]

J Biol Chem, 2019
Ataxin-3 is a deubiquitinating enzyme and the affected protein in the neurodegenerative disorder Machado-Joseph disease (MJD). The ATXN3 gene is alternatively spliced, resulting in protein isoforms that differ in the number of ubiquitin-interacting motifs.
Weishäupl D, Schneider J, Peixoto Pinheiro B, Ruess C, Dold SM, von Zweydorf F, Gloeckner CJ, Schmidt J, Riess O, Schmidt T.   +9 more
europepmc   +5 more sources