Results 111 to 120 of about 13,262,735 (197)

Data_Sheet_1_Implications of specific lysine residues within ataxin-3 for the molecular pathogenesis of Machado-Joseph disease.pdf

, 2023
Lysine residues are one of the main sites for posttranslational modifications of proteins, and lysine ubiquitination of the Machado-Joseph disease protein ataxin-3 is implicated in its cellular function and polyglutamine expansion-dependent toxicity ...
Xiaoling Li (129705), Jaqueline Jung (15649907), Rana Dilara Incebacak Eltemur (15649904), Jonasz Jeremiasz Weber (15649895), Ana Velic (79264), Rafael Saup (15649901), Olaf Riess (110686), Boris Macek (23897), Sercan Bayezit (15649898), Huu Phuc Nguyen (10979277), Priscila Pereira Sena (15649892), Thorsten Schmidt (5598)   +11 more
core   +1 more source

Ataxin-2 interacts with FUS and intermediate-length polyglutamine expansions enhance FUS-related pathology in amyotrophic lateral sclerosis

, 2013
Ataxin-2 interacts with FUS and intermediate-length polyglutamine expansions enhance FUS-related pathology in amyotrophic lateral ...
MA Farg (15824423), KY Soo (15824420), ST Warraich (14628641), JD Atkin (14472765), Vinod Sundaramoorthy (13096605), IP Blair (12507994)   +5 more
core  

Development of a SCA7 patient-derived lymphoblast cell model for testing RNAi knock-down of the disease-causing gene

, 2011
Includes bibliographical references (leaves 106-116).Spinocerebellar ataxia type 7 (SCA7) is an inherited neurodegenerative disease caused by the expansion of a CAG repeat within the ataxin-7 gene. The South African SCA7 population has been shown to have
Berkowitz, Danielle Claire
core  

Ataxin-3 phosphorylation decreases neuronal defects in spinocerebellar ataxia type 3 models [PDF]

, 2016
Different neurodegenerative diseases are caused by aberrant elongation of repeated glutamine sequences normally found in particular human proteins.
Sandra Macedo-Ribeiro, Almeida, L, Clévio Nóbrega, Nóbrega, C, Ana Luísa Carvalho, Luís Pereira de Almeida, Elisabete Ferreiro, Ferreiro, E, Bruno Almeida, Matos, C, Carlos A. Matos, Valero, J, Macedo-Ribeiro, S, Susana R. Louros, Louros, S, Carvalho, A, Jorge Valero, Almeida, B   +17 more
core   +1 more source

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

Allele-specific silencing of mutant ataxin-3 leads to diminution of the number of intranuclear aggregates.

, 2014
Confocal microscopy of ataxin-3, β-galactosidase, with nuclei counterstained in blue (DAPI). Mice injected with mutant ataxin-3 and short-hairpin against GFP (upper panel, MUT+ shGFP) exhibit aggregates of mutant ataxin-3 ((b,f), red, arrows) co ...
Nicole Déglon (46541), David Albuquerque (275749), Isabel Onofre (275748), Isabel Nascimento-Ferreira (275747), Clévio Nóbrega (275746), Luís Pereira de Almeida (221526)   +5 more
core   +1 more source

SUMO-1 modification did not affect ataxin-3 ubiquitination.

, 2013
(A) HEK293 cells were co-transfected with GFP-ataxin-3 and Flag-SUMO-1. The cells were treated with 10 µM MG132 for 12 h and subject to immunoprecipitation analysis using rabbit polyclonal antibodies against GFP.
Kun Xia (9853), Bei-Sha Tang (278458), Jian-Guang Tang (278454), Shu-Sheng Liao (278452), Hong Jiang (79543), Jun-Ling Wang (278455), Li-Fang Lei (278456), Ying-Ying Luo (278453), Juan Du (118107), Ya-Fang Zhou (278451), Jing-Wei Chi (278457), Lu Shen (161789)   +11 more
core   +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

Establishing a TR-FRET based immunoassay to detect soluble ataxin-3 levels.

, 2013
A) Schematic illustration of antibody binding sites in context of the ataxin-3 protein and the principal of the TR-FRET immunoassay. By using this antibody combination (1H9 and MW1) only ataxin-3 with more than 6 CAG repeats can be detected (no detection
Stephan Grueninger (338117), Jeannette Hübener (405961), Huu Phuc Nguyen (110694), Jonasz Jeremiasz Weber (405962), Olaf Riess (110686), Andreas Weiss (135692)   +5 more
core   +1 more source