Mechanisms of protein toxicity in neurodegenerative diseases
Protein toxicity can be defined as all the pathological changes that ensue from
accumulation, mis-localization, and/or multimerization of disease-specific proteins. Most …
accumulation, mis-localization, and/or multimerization of disease-specific proteins. Most …
Machado–Joseph disease/spinocerebellar ataxia type 3: lessons from disease pathogenesis and clues into therapy
CA Matos, LP de Almeida… - Journal of …, 2019 - Wiley Online Library
Abstract Machado–Joseph disease (MJD), also known as spinocerebellar ataxia type 3
(SCA 3), is an incurable disorder, widely regarded as the most common form of …
(SCA 3), is an incurable disorder, widely regarded as the most common form of …
VCP recruitment to mitochondria causes mitophagy impairment and neurodegeneration in models of Huntington's disease
Mutant Huntingtin (mtHtt) causes neurodegeneration in Huntington's disease (HD) by
evoking defects in the mitochondria, but the underlying mechanisms remains elusive. Our …
evoking defects in the mitochondria, but the underlying mechanisms remains elusive. Our …
PERK pathway and neurodegenerative disease: to inhibit or to activate?
T Shacham, C Patel, GZ Lederkremer - Biomolecules, 2021 - mdpi.com
With the extension of life span in recent decades, there is an increasing burden of late-onset
neurodegenerative diseases, for which effective treatments are lacking. Neurodegenerative …
neurodegenerative diseases, for which effective treatments are lacking. Neurodegenerative …
Sequestration of cellular interacting partners by protein aggregates: implication in a loss‐of‐function pathology
H Yang, HY Hu - The FEBS journal, 2016 - Wiley Online Library
Protein misfolding and aggregation are a hallmark of several neurodegenerative diseases
(ND s). However, how protein aggregation leads to cytotoxicity and neurodegeneration is …
(ND s). However, how protein aggregation leads to cytotoxicity and neurodegeneration is …
[HTML][HTML] From pathogenesis to novel therapeutics for spinocerebellar ataxia type 3: evading potholes on the way to translation
Abstract Spinocerebellar ataxia type 3 (SCA3), also known as Machado–Joseph disease
(MJD), is a neurodegenerative disorder caused by a polyglutamine expansion in the ATXN3 …
(MJD), is a neurodegenerative disorder caused by a polyglutamine expansion in the ATXN3 …
ATXN3 controls DNA replication and transcription by regulating chromatin structure
E Hernández‐Carralero, E Cabrera… - Nucleic Acids …, 2023 - academic.oup.com
The deubiquitinating enzyme Ataxin-3 (ATXN3) contains a polyglutamine (PolyQ) region, the
expansion of which causes spinocerebellar ataxia type-3 (SCA3). ATXN3 has multiple …
expansion of which causes spinocerebellar ataxia type-3 (SCA3). ATXN3 has multiple …
Protein aggregation in cell biology: An aggregomics perspective of health and disease
Maintaining protein homeostasis (proteostasis) is essential for cellular health and is
governed by a network of quality control machinery comprising over 800 genes. When …
governed by a network of quality control machinery comprising over 800 genes. When …
The truncated C-terminal fragment of mutant ATXN3 disrupts mitochondria dynamics in spinocerebellar ataxia type 3 models
JY Hsu, YL Jhang, PH Cheng, YF Chang… - Frontiers in molecular …, 2017 - frontiersin.org
Spinocerebellar ataxia type 3 (SCA3), known as Machado-Joseph disease, is an autosomal
dominant disease caused by an abnormal expansion of polyglutamine in ATXN3 gene …
dominant disease caused by an abnormal expansion of polyglutamine in ATXN3 gene …
ATXN3: a multifunctional protein involved in the polyglutamine disease spinocerebellar ataxia type 3
E Hernández-Carralero, G Quinet… - Expert Reviews in …, 2024 - cambridge.org
ATXN3 is a ubiquitin hydrolase (or deubiquitinase, DUB), product of the ATXN3 gene,
ubiquitously expressed in various cell types including peripheral and neuronal tissues and …
ubiquitously expressed in various cell types including peripheral and neuronal tissues and …