[HTML][HTML] Liquid-liquid phase separation of TDP-43 and FUS in physiology and pathology of neurodegenerative diseases

JL Carey, L Guo - Frontiers in molecular biosciences, 2022 - frontiersin.org
Liquid-liquid phase separation of RNA-binding proteins mediates the formation of numerous
membraneless organelles with essential cellular function. However, aberrant phase …

The implications of physiological biomolecular condensates in amyotrophic lateral sclerosis

H Fakim, CV Velde - Seminars in Cell & Developmental Biology, 2023 - Elsevier
In recent years, there has been an emphasis on the role of phase-separated biomolecular
condensates, especially stress granules, in neurodegenerative diseases such as …

[HTML][HTML] TEX264 coordinates p97-and SPRTN-mediated resolution of topoisomerase 1-DNA adducts

J Fielden, K Wiseman, I Torrecilla, S Li, S Hume… - Nature …, 2020 - nature.com
Abstract Eukaryotic topoisomerase 1 (TOP1) regulates DNA topology to ensure efficient
DNA replication and transcription. TOP1 is also a major driver of endogenous genome …

[HTML][HTML] Depending on the stress, histone deacetylase inhibitors act as heat shock protein co-inducers in motor neurons and potentiate arimoclomol, exerting …

R Kuta, N Larochelle, M Fernandez, A Pal… - Cell Stress and …, 2020 - Elsevier
Upregulation of heat shock proteins (HSPs) is an approach to treatment of
neurodegenerative disorders with impaired proteostasis. Many neurons, including motor …

Changes to the TDP-43 and FUS Interactomes Induced by DNA Damage

T Kawaguchi, MG Rollins, M Moinpour… - Journal of proteome …, 2019 - ACS Publications
The RNA-binding proteins TDP-43 and FUS are tied as the third leading known genetic
cause for amyotrophic lateral sclerosis (ALS), and TDP-43 proteopathies are found in nearly …

[HTML][HTML] NUP98 and RAE1 sustain progenitor function through HDAC-dependent chromatin targeting to escape from nucleolar localization

AE Neely, LA Blumensaadt, PJ Ho, SM Lloyd… - Communications …, 2023 - nature.com
Self-renewing somatic tissues rely on progenitors to support the continuous tissue
regeneration. The gene regulatory network maintaining progenitor function remains …

Amyotrophic lateral sclerosis, FUS and protein synthesis defects

AF Assoni, F Foijer, M Zatz - Stem Cell Reviews and Reports, 2023 - Springer
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that mainly affects the
motor system. It is a very heterogeneous disorder, so far more than 40 genes have been …

snoRNAs in hematopoiesis and blood malignancies: A comprehensive review

MF Challakkara, R Chhabra - Journal of cellular physiology, 2023 - Wiley Online Library
Small nucleolar RNAs (snoRNAs) are noncoding RNA molecules of highly variable size,
usually ranging from 60 to 150 nucleotides. They are classified into H/ACA box snoRNAs …

[HTML][HTML] Binding of the nuclear ribonucleoprotein family member FUS to RNA prevents R-loop RNA: DNA hybrid structures

VF Thompson, DR Wieland, V Mendoza-Leon… - Journal of Biological …, 2023 - ASBMB
The protein FUS (FUSed in sarcoma) is a metazoan RNA-binding protein that influences
RNA production by all three nuclear polymerases. FUS also binds nascent transcripts, RNA …

[HTML][HTML] DNA damage stress-induced translocation of mutant FUS proteins into cytosolic granules and screening for translocation inhibitors

M Nogami, O Sano, K Adachi-Tominari… - Frontiers in Molecular …, 2022 - frontiersin.org
Fused in sarcoma/translated in liposarcoma (FUS) is an RNA-binding protein, and its
mutations are associated with neurodegenerative diseases, including amyotrophic lateral …