Cargando…

Cerebellar Soluble Mutant Ataxin-3 Level Decreases during Disease Progression in Spinocerebellar Ataxia Type 3 Mice

Spinocerebellar Ataxia Type 3 (SCA3), also known as Machado-Joseph disease, is an autosomal dominantly inherited neurodegenerative disease caused by an expanded polyglutamine stretch in the ataxin-3 protein. A pathological hallmark of the disease is cerebellar and brainstem atrophy, which correlates...

Descripción completa

Detalles Bibliográficos
Autores principales:	Nguyen, Huu Phuc, Hübener, Jeannette, Weber, Jonasz Jeremiasz, Grueninger, Stephan, Riess, Olaf, Weiss, Andreas
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Public Library of Science 2013
Materias:	Research Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3633920/ https://www.ncbi.nlm.nih.gov/pubmed/23626768 http://dx.doi.org/10.1371/journal.pone.0062043

Ejemplares similares

Autophagy induction reduces mutant ataxin-3 levels and toxicity in a mouse model of spinocerebellar ataxia type 3
por: Menzies, Fiona M., et al.
Publicado: (2010)

Mitochondrial Dysfunction in Spinocerebellar Ataxia Type 3 Is Linked to VDAC1 Deubiquitination
por: Harmuth, Tina, et al.
Publicado: (2022)

Ataxin-3, The Spinocerebellar Ataxia Type 3 Neurodegenerative Disorder Protein, Affects Mast Cell Functions
por: Sowa, Anna S., et al.
Publicado: (2022)

Calpains as novel players in the molecular pathogenesis of spinocerebellar ataxia type 17
por: Weber, Jonasz Jeremiasz, et al.
Publicado: (2022)

TR-FRET-Based Immunoassay to Measure Ataxin-2 as a Target Engagement Marker in Spinocerebellar Ataxia Type 2
por: Bux, Jessica, et al.
Publicado: (2023)

KPNB1 modulates the Machado–Joseph disease protein ataxin-3 through activation of the mitochondrial protease CLPP
por: Abeditashi, Mahkameh, et al.
Publicado: (2022)

Implications of specific lysine residues within ataxin-3 for the molecular pathogenesis of Machado-Joseph disease
por: Pereira Sena, Priscila, et al.
Publicado: (2023)

Ataxin-3 phosphorylation decreases neuronal defects in spinocerebellar ataxia type 3 models
por: Matos, Carlos A., et al.
Publicado: (2016)

Urine levels of the polyglutamine ataxin-3 protein are elevated in patients with spinocerebellar ataxia type 3
por: Koike, Yuka, et al.
Publicado: (2021)

Mitochondrial Morphology, Function and Homeostasis Are Impaired by Expression of an N-terminal Calpain Cleavage Fragment of Ataxin-3
por: Harmuth, Tina, et al.
Publicado: (2018)

From Pathways to Targets: Understanding the Mechanisms behind Polyglutamine Disease
por: Weber, Jonasz Jeremiasz, et al.
Publicado: (2014)

Mutant Ataxin-2 Expression in Aged Animals Aggravates Neuropathological Features Associated with Spinocerebellar Ataxia Type 2
por: Afonso, Inês T., et al.
Publicado: (2022)

Ataxin-3 Protein and RNA Toxicity in Spinocerebellar Ataxia Type 3: Current Insights and Emerging Therapeutic Strategies
por: Evers, Melvin M., et al.
Publicado: (2013)

Targeting the VCP-binding motif of ataxin-3 improves phenotypes in Drosophila models of Spinocerebellar Ataxia Type 3
por: Johnson, Sean L., et al.
Publicado: (2021)

Allele-specific targeting of mutant ataxin-3 by antisense oligonucleotides in SCA3-iPSC-derived neurons
por: Hauser, Stefan, et al.
Publicado: (2021)

Aberrant Cerebellar Circuitry in the Spinocerebellar Ataxias
por: Robinson, Katherine J., et al.
Publicado: (2020)

Identification of the ataxin-1 interaction network and its impact on spinocerebellar ataxia type 1
por: Chen, Jiu-Ming, et al.
Publicado: (2022)

Histone deacetylase-3 interacts with ataxin-7 and is altered in a spinocerebellar ataxia type 7 mouse model
por: Duncan, Carlotta E, et al.
Publicado: (2013)

Ubiquitin-binding site 1 of pathogenic ataxin-3 regulates its toxicity in Drosophila models of Spinocerebellar Ataxia Type 3
por: Prifti, Matthew V., et al.
Publicado: (2023)

Calpain-mediated proteolysis as driver and modulator of polyglutamine toxicity
por: Incebacak Eltemur, Rana Dilara, et al.
Publicado: (2022)

Stage-dependent biomarker changes in spinocerebellar ataxia type 3
por: Faber, Jennifer, et al.
Publicado: (2023)

Indirect Negative Effect of Mutant Ataxin-1 on Short- and Long-Term Synaptic Plasticity in Mouse Models of Spinocerebellar Ataxia Type 1
por: Shuvaev, Anton N., et al.
Publicado: (2022)

Killing Two Angry Birds with One Stone: Autophagy Activation by Inhibiting Calpains in Neurodegenerative Diseases and Beyond
por: Weber, Jonasz Jeremiasz, et al.
Publicado: (2019)

Unique degeneration signatures in the cerebellar cortex for spinocerebellar ataxias 2, 3, and 7
por: Hernandez-Castillo, Carlos R., et al.
Publicado: (2018)

Neurocognitive and cerebellar function in ADHD, autism and spinocerebellar ataxia
por: Cundari, Maurizio, et al.
Publicado: (2023)

Partial Loss of Ataxin-1 Function Contributes to Transcriptional Dysregulation in Spinocerebellar Ataxia Type 1 Pathogenesis
por: Crespo-Barreto, Juan, et al.
Publicado: (2010)

Transcriptional profiling and biomarker identification reveal tissue specific effects of expanded ataxin-3 in a spinocerebellar ataxia type 3 mouse model
por: Toonen, Lodewijk J. A., et al.
Publicado: (2018)

Evidences for Mutant Huntingtin Inducing Musculoskeletal and Brain Growth Impairments via Disturbing Testosterone Biosynthesis in Male Huntington Disease Animals
por: Yu-Taeger, Libo, et al.
Publicado: (2022)

Cerebellar neuronal dysfunction accompanies early motor symptoms in spinocerebellar ataxia type 3
por: Mayoral-Palarz, Kristin, et al.
Publicado: (2022)

CAG repeat length does not associate with the rate of cerebellar degeneration in spinocerebellar ataxia type 3
por: Huang, Shang-Ran, et al.
Publicado: (2016)

Extra-Cerebellar Signs and Non-motor Features in Chinese Patients With Spinocerebellar Ataxia Type 3
por: Yuan, Xiaoqin, et al.
Publicado: (2019)

Effects of Repetitive Transcranial Magnetic Stimulation on Cerebellar Metabolism in Patients With Spinocerebellar Ataxia Type 3
por: Chen, Xin-Yuan, et al.
Publicado: (2022)

Tau and neurofilament light‐chain as fluid biomarkers in spinocerebellar ataxia type 3
por: Garcia‐Moreno, Hector, et al.
Publicado: (2022)

Cerebellar Development and Circuit Maturation: A Common Framework for Spinocerebellar Ataxias
por: Binda, Francesca, et al.
Publicado: (2020)

PolyQ-expanded ataxin-3 protein levels in peripheral blood mononuclear cells correlate with clinical parameters in SCA3: a pilot study
por: Gonsior, Kathrin, et al.
Publicado: (2020)

Recurrent divergence-insufficiency esotropia in Machado-Joseph disease (spinocerebellar ataxia type 3)
por: Stallworth, Jeannette Y., et al.
Publicado: (2022)

Axonal inclusions in spinocerebellar ataxia type 3
por: Seidel, Kay, et al.
Publicado: (2010)

Copy Number Variant Analysis of Spinocerebellar Ataxia Genes in a Cohort of Dutch Patients With Cerebellar Ataxia
por: Ghorbani, Fatemeh, et al.
Publicado: (2023)

Frequency of Spinocerebellar Ataxia type 1, 2, 3,6 and 7 and clinical profile of Spinocerebellar Ataxia type 3 in Malaysia
por: Mohamed Ibrahim, Norlinah, et al.
Publicado: (2020)

Editorial: The role of posttranslational modifications in polyglutamine diseases
por: Weber, Jonasz Jeremiasz, et al.
Publicado: (2023)

Cannot write session to /tmp/vufind_sessions/sess_baub70v8viuiskuab920i8nait