[HTML][HTML] Combating deleterious phase transitions in neurodegenerative disease
AL Darling, J Shorter - Biochimica et Biophysica Acta (BBA)-Molecular Cell …, 2021 - Elsevier
Protein aggregation is a hallmark of neurodegenerative diseases. However, the mechanism
that induces pathogenic aggregation is not well understood. Recently, it has emerged that …
that induces pathogenic aggregation is not well understood. Recently, it has emerged that …
Post-translational modifications on RNA-binding proteins: accelerators, brakes, or passengers in neurodegeneration?
EL Sternburg, LAG da Silva, D Dormann - Trends in biochemical sciences, 2022 - cell.com
RNA-binding proteins (RBPs) are critical players in RNA expression and metabolism, thus,
the proper regulation of this class of proteins is critical for cellular health. Regulation of RBPs …
the proper regulation of this class of proteins is critical for cellular health. Regulation of RBPs …
Nuclear accumulation of CHMP7 initiates nuclear pore complex injury and subsequent TDP-43 dysfunction in sporadic and familial ALS
AN Coyne, V Baskerville, BL Zaepfel… - Science translational …, 2021 - science.org
Alterations in the components [nucleoporins (Nups)] and function of the nuclear pore
complex (NPC) have been implicated as contributors to the pathogenesis of genetic forms of …
complex (NPC) have been implicated as contributors to the pathogenesis of genetic forms of …
[HTML][HTML] ALS-associated KIF5A mutations abolish autoinhibition resulting in a toxic gain of function
DM Baron, AR Fenton, S Saez-Atienzar… - Cell reports, 2022 - cell.com
Understanding the pathogenic mechanisms of disease mutations is critical to advancing
treatments. ALS-associated mutations in the gene encoding the microtubule motor KIF5A …
treatments. ALS-associated mutations in the gene encoding the microtubule motor KIF5A …
[HTML][HTML] G4C2 repeat RNA initiates a POM121-mediated reduction in specific nucleoporins in C9orf72 ALS/FTD
Through mechanisms that remain poorly defined, defects in nucleocytoplasmic transport and
accumulations of specific nuclear-pore-complex-associated proteins have been reported in …
accumulations of specific nuclear-pore-complex-associated proteins have been reported in …
Interactions between ALS-linked FUS and nucleoporins are associated with defects in the nucleocytoplasmic transport pathway
YC Lin, MS Kumar, N Ramesh, EN Anderson… - Nature …, 2021 - nature.com
Nucleocytoplasmic transport (NCT) decline occurs with aging and neurodegeneration. Here,
we investigated the NCT pathway in models of amyotrophic lateral sclerosis–fused in …
we investigated the NCT pathway in models of amyotrophic lateral sclerosis–fused in …
[HTML][HTML] C9orf72 arginine-rich dipeptide repeat proteins disrupt karyopherin-mediated nuclear import
Disruption of nucleocytoplasmic transport is increasingly implicated in the pathogenesis of
neurodegenerative diseases, including ALS caused by a C9orf72 hexanucleotide repeat …
neurodegenerative diseases, including ALS caused by a C9orf72 hexanucleotide repeat …
[HTML][HTML] The nuclear SUMO-targeted ubiquitin quality control network regulates the dynamics of cytoplasmic stress granules
J Keiten-Schmitz, K Wagner, T Piller, M Kaulich… - Molecular cell, 2020 - cell.com
Exposure of cells to heat or oxidative stress causes misfolding of proteins. To avoid toxic
protein aggregation, cells have evolved nuclear and cytosolic protein quality control (PQC) …
protein aggregation, cells have evolved nuclear and cytosolic protein quality control (PQC) …
[HTML][HTML] Traffic jam at the nuclear pore: All roads lead to nucleocytoplasmic transport defects in ALS/FTD
Amyotrophic lateral sclerosis (ALS) is a fatal late-onset neurodegenerative disease that
specifically affects the function and survival of spinal and cortical motor neurons. ALS shares …
specifically affects the function and survival of spinal and cortical motor neurons. ALS shares …
The mechanistic role of alpha-synuclein in the nucleus: impaired nuclear function caused by familial Parkinson's disease SNCA mutations
V Chen, M Moncalvo, D Tringali… - Human molecular …, 2020 - academic.oup.com
Alpha-synuclein SNCA has been implicated in the etiology of Parkinson's disease (PD);
however, the normal function of alpha-synuclein protein and the pathway that mediates its …
however, the normal function of alpha-synuclein protein and the pathway that mediates its …