ALS genetics, mechanisms, and therapeutics: where are we now?

R Mejzini, LL Flynn, IL Pitout, S Fletcher… - Frontiers in …, 2019 - frontiersin.org
The scientific landscape surrounding amyotrophic lateral sclerosis (ALS) continues to shift
as the number of genes associated with the disease risk and pathogenesis, and the cellular …

A new phase of networking: the molecular composition and regulatory dynamics of mammalian stress granules

SR Millar, JQ Huang, KJ Schreiber, YC Tsai… - Chemical …, 2023 - ACS Publications
Stress granules (SGs) are cytosolic biomolecular condensates that form in response to
cellular stress. Weak, multivalent interactions between their protein and RNA constituents …

FUS-dependent liquid–liquid phase separation is important for DNA repair initiation

BR Levone, SC Lenzken, M Antonaci, A Maiser… - Journal of Cell …, 2021 - rupress.org
RNA-binding proteins (RBPs) are emerging as important effectors of the cellular DNA
damage response (DDR). The RBP FUS is implicated in RNA metabolism and DNA repair …

PARP-1 activation directs FUS to DNA damage sites to form PARG-reversible compartments enriched in damaged DNA

AS Singatulina, L Hamon, MV Sukhanova, B Desforges… - Cell reports, 2019 - cell.com
PARP-1 synthesizes long poly (ADP-ribose) chains (PAR) at DNA damage sites to recruit
DNA repair factors. Among proteins relocated on damaged DNA, the RNA-binding protein …

Functions of PARylation in DNA damage repair pathways

H Wei, X Yu - Genomics, Proteomics and Bioinformatics, 2016 - academic.oup.com
Protein poly ADP-ribosylation (PARylation) is a widespread post-translational modification at
DNA lesions, which is catalyzed by poly (ADP-ribose) polymerases (PARPs). This …

Readers of poly (ADP-ribose): designed to be fit for purpose

F Teloni, M Altmeyer - Nucleic acids research, 2015 - academic.oup.com
Post-translational modifications (PTMs) regulate many aspects of protein function and are
indispensable for the spatio-temporal regulation of cellular processes. The proteome-wide …

[HTML][HTML] PARP1-DNA co-condensation drives DNA repair site assembly to prevent disjunction of broken DNA ends

N Chappidi, T Quail, S Doll, LT Vogel, R Aleksandrov… - Cell, 2024 - cell.com
DNA double-strand breaks (DSBs) are repaired at DSB sites. How DSB sites assemble and
how broken DNA is prevented from separating is not understood. Here we uncover that the …

Ionizing radiation and complex DNA damage: from prediction to detection challenges and biological significance

IV Mavragani, Z Nikitaki, SA Kalospyros… - Cancers, 2019 - mdpi.com
Biological responses to ionizing radiation (IR) have been studied for many years, generally
showing the dependence of these responses on the quality of radiation, ie, the radiation …

Poly (ADP-ribose): a dynamic trigger for biomolecular condensate formation

AKL Leung - Trends in cell biology, 2020 - cell.com
Poly (ADP-ribose)(PAR) is a nucleic acid-like protein modification that can seed the
formation of microscopically visible cellular compartments that lack enveloping membranes …

[HTML][HTML] RNA-binding proteins in amyotrophic lateral sclerosis

M Zhao, JR Kim, R van Bruggen, J Park - Molecules and cells, 2018 - Elsevier
Significant research efforts are ongoing to elucidate the complex molecular mechanisms
underlying amyotrophic lateral sclerosis (ALS), which may in turn pinpoint potential …