[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 …

RNA: nuclear glue for folding the genome

RS Nozawa, N Gilbert - Trends in cell biology, 2019 - cell.com
A significant amount of RNA is present in the nucleus of mammalian cells but only a small
proportion of it is destined for the cytoplasm and subsequent translation, leaving much RNA …

C2H2-zinc-finger transcription factors bind RNA and function in diverse post-transcriptional regulatory processes

S Nabeel-Shah, S Pu, JD Burns, U Braunschweig… - Molecular Cell, 2024 - cell.com
Summary Cys2-His2 zinc-finger proteins (C2H2-ZNFs) constitute the largest class of DNA-
binding transcription factors (TFs) yet remain largely uncharacterized. Although certain …

Analysis of RNA–protein networks with RNP-MaP defines functional hubs on RNA

CA Weidmann, AM Mustoe, PB Jariwala… - Nature …, 2021 - nature.com
RNA–protein interaction networks govern many biological processes but are difficult to
examine comprehensively. We devised ribonucleoprotein networks analyzed by mutational …

Inner nuclear protein Matrin-3 coordinates cell differentiation by stabilizing chromatin architecture

HJ Cha, Ö Uyan, Y Kai, T Liu, Q Zhu, Z Tothova… - Nature …, 2021 - nature.com
Precise control of gene expression during differentiation relies on the interplay of chromatin
and nuclear structure. Despite an established contribution of nuclear membrane proteins to …

Matrin 3-dependent neurotoxicity is modified by nucleic acid binding and nucleocytoplasmic localization

AM Malik, RA Miguez, X Li, YS Ho, EL Feldman… - elife, 2018 - elifesciences.org
Abnormalities in nucleic acid processing are associated with the development of
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Mutations in Matrin …

Splicing repression is a major function of TDP-43 in motor neurons

A Donde, M Sun, JP Ling, KE Braunstein, B Pang… - Acta …, 2019 - Springer
Nuclear depletion of TDP-43, an essential RNA binding protein, may underlie
neurodegeneration in amyotrophic lateral sclerosis (ALS). As several functions have been …

Nuclear RNA binding regulates TDP-43 nuclear localization and passive nuclear export

L Duan, BL Zaepfel, V Aksenova, M Dasso… - Cell reports, 2022 - cell.com
Nuclear clearance of the RNA-binding protein TDP-43 is a hallmark of neurodegeneration
and an important therapeutic target. Our current understanding of TDP-43 …

Selective neuronal degeneration in MATR3 S85C knock-in mouse model of early-stage ALS

CS Kao, R van Bruggen, JR Kim, XXL Chen… - Nature …, 2020 - nature.com
Abstract A missense mutation, S85C, in the MATR3 gene is a genetic cause for amyotrophic
lateral sclerosis (ALS). It is unclear how the S85C mutation affects MATR3 function and …

RNA-recognition motif in Matrin-3 mediates neurodegeneration through interaction with hnRNPM

N Ramesh, S Kour, EN Anderson… - Acta neuropathologica …, 2020 - Springer
Background Amyotrophic lateral sclerosis (ALS) is an adult-onset, fatal neurodegenerative
disease characterized by progressive loss of upper and lower motor neurons. While …