PolyQ-expanded proteins impair cellular proteostasis of ataxin-3 through sequestering the co-chaperone HSJ1 into aggregates
HW Yue, JY Hong, SX Zhang, LL Jiang, HY Hu - Scientific Reports, 2021 - nature.com
… It is well-known that protein aggregation is a pathological hallmark shared among diverse …
5,6,7,8 . However, why amyloid-like aggregation of polyQ proteins is toxic to cells, especially to …
5,6,7,8 . However, why amyloid-like aggregation of polyQ proteins is toxic to cells, especially to …
A survey of protein interactions and posttranslational modifications that influence the polyglutamine diseases
… proteins—which causes protein misfolding and aggregation, … its toxicity, particularly with
proteins involved in intracellular … factor, Sp1, and sequesters it away from its normal function in …
proteins involved in intracellular … factor, Sp1, and sequesters it away from its normal function in …
[HTML][HTML] Polyglutamine expanded Ataxin-7 induces DNA damage and alters FUS localization and function
F Niss, W Zaidi, E Hallberg, AL Ström - Molecular and Cellular …, 2021 - Elsevier
… The sequestration of vital proteins into aggregates formed by polyQ proteins is believed to
… In this study, we show that FUS is sequestered into ATXN7 aggregates to a high degree, …
… In this study, we show that FUS is sequestered into ATXN7 aggregates to a high degree, …
Polyglutamine spinocerebellar ataxias: emerging therapeutic targets
A Neves-Carvalho, S Duarte-Silva… - Expert Opinion on …, 2020 - Taylor & Francis
… yet to be established, especially for aging-associated disorders. … aggregated species for
disease, protein aggregation is a … A small-molecule inhibitor (IU1) of ubiquitin-specific protease …
disease, protein aggregation is a … A small-molecule inhibitor (IU1) of ubiquitin-specific protease …
The molecular basis of spinocerebellar ataxia type 7
R Goswami, AI Bello, J Bean, KM Costanzo… - Frontiers in …, 2022 - frontiersin.org
… phenotypes associated with polyglutamine expanded ATXN7 that … that is affected by regulated
sequestration and release of the DUBm… SUMOylation at lysine 257 reduces aggregation of …
sequestration and release of the DUBm… SUMOylation at lysine 257 reduces aggregation of …
[HTML][HTML] Coaggregation of polyglutamine (polyQ) proteins is mediated by polyQ-tract interactions and impairs cellular proteostasis: Coaggregation of polyQ proteins
JY Hong, JY Wang, HW Yue, XL Zhang… - Acta Biochimica et …, 2023 - ncbi.nlm.nih.gov
… proteins and sequester them into amyloid-like aggregates. However, how these proteins
mutually coaggregate and sequester each other and how polyQ aggregates induce neuronal …
mutually coaggregate and sequester each other and how polyQ aggregates induce neuronal …
Deubiquitinase USP7 contributes to the pathogenicity of spinal and bulbar muscular atrophy
A Pluciennik, Y Liu, E Molotsky… - The Journal of …, 2021 - Am Soc Clin Investig
… species and that their formation may be important for cell survival due to their sequestration
… AR aggregation. USP7 is a ubiquitously expressed protein that possesses ubiquitin-specific …
… AR aggregation. USP7 is a ubiquitously expressed protein that possesses ubiquitin-specific …
Gene deregulation and underlying mechanisms in spinocerebellar ataxias with polyglutamine expansion
A Niewiadomska-Cimicka, A Hache… - Frontiers in …, 2020 - frontiersin.org
… proteins involved in RNA metabolism (Table 1). Moreover, misfolded … proteins progressively
accumulate in neuronal nuclei where they form aggregates that sequester nuclear proteins …
accumulate in neuronal nuclei where they form aggregates that sequester nuclear proteins …
Structural and dynamic studies reveal that the Ala-rich region of ataxin-7 initiates α-helix formation of the polyQ tract but suppresses its aggregation
JY Hong, DD Wang, W Xue, HW Yue, H Yang… - Scientific Reports, 2019 - nature.com
… PRR of Atx7 25 , and aggregation of polyQ-expanded Atx7 specifically sequesters
ubiquitin-specific protease 22 (USP22) and … Aggregation of Polyglutamine-expanded Ataxin 7 …
ubiquitin-specific protease 22 (USP22) and … Aggregation of Polyglutamine-expanded Ataxin 7 …
From quality control to neurodegeneration: regulation of autophagy and the DNA damage response by ubiquitin-modifying enzymes
LK Herzog Croona - 2020 - openarchive.ki.se
… evoked by expression of polyglutamine-expanded ataxin-3 in … While the ubiquitin-specific
metalloproteases are comprised by … sequestered in aggregates of polyglutamine-expanded …
metalloproteases are comprised by … sequestered in aggregates of polyglutamine-expanded …