Triad of TDP43 control in neurodegeneration: autoregulation, localization and aggregation

P Tziortzouda, L Van Den Bosch, F Hirth - Nature Reviews …, 2021 - nature.com
Cytoplasmic aggregation of TAR DNA-binding protein 43 (TDP43; also known as TARDBP
or TDP-43) is a key pathological feature of several neurodegenerative diseases, including …

The role of TDP-43 propagation in neurodegenerative diseases: integrating insights from clinical and experimental studies

M Jo, S Lee, YM Jeon, S Kim, Y Kwon… - Experimental & molecular …, 2020 - nature.com
Abstract TAR DNA-binding protein 43 (TDP-43) is a highly conserved nuclear RNA/DNA-
binding protein involved in the regulation of RNA processing. The accumulation of TDP-43 …

TDP-43 condensation properties specify its RNA-binding and regulatory repertoire

M Hallegger, AM Chakrabarti, FCY Lee, BL Lee… - Cell, 2021 - cell.com
Mutations causing amyotrophic lateral sclerosis (ALS) often affect the condensation
properties of RNA-binding proteins (RBPs). However, the role of RBP condensation in the …

TDP-43 pathology disrupts nuclear pore complexes and nucleocytoplasmic transport in ALS/FTD

CC Chou, YI Zhang, ME Umoh, SW Vaughan… - Nature …, 2018 - nature.com
The cytoplasmic mislocalization and aggregation of TAR DNA-binding protein-43 (TDP-43)
is a common histopathological hallmark of the amyotrophic lateral sclerosis and …

Nuclear-import receptors reverse aberrant phase transitions of RNA-binding proteins with prion-like domains

L Guo, HJ Kim, H Wang, J Monaghan, F Freyermuth… - Cell, 2018 - cell.com
RNA-binding proteins (RBPs) with prion-like domains (PrLDs) phase transition to functional
liquids, which can mature into aberrant hydrogels composed of pathological fibrils that …

The C9orf72 repeat expansion disrupts nucleocytoplasmic transport

K Zhang, CJ Donnelly, AR Haeusler, JC Grima… - Nature, 2015 - nature.com
The hexanucleotide repeat expansion (HRE) GGGGCC (G4C2) in C9orf72 is the most
common cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) …

Monomerization of TDP-43 is a key determinant for inducing TDP-43 pathology in amyotrophic lateral sclerosis

K Oiwa, S Watanabe, K Onodera, Y Iguchi… - Science …, 2023 - science.org
The cytoplasmic aggregation of TAR DNA binding protein-43 (TDP-43), also known as TDP-
43 pathology, is the pathological hallmark of amyotrophic lateral sclerosis (ALS). However …

Nuclear pore complexes—a doorway to neural injury in neurodegeneration

AN Coyne, JD Rothstein - Nature Reviews Neurology, 2022 - nature.com
The genetic underpinnings and end-stage pathological hallmarks of neurodegenerative
diseases are increasingly well defined, but the cellular pathophysiology of disease initiation …

TDP-43 pathology in Alzheimer's disease

A Meneses, S Koga, J O'Leary, DW Dickson… - Molecular …, 2021 - Springer
Transactive response DNA binding protein of 43 kDa (TDP-43) is an intranuclear protein
encoded by the TARDBP gene that is involved in RNA splicing, trafficking, stabilization, and …

Physiological functions and pathobiology of TDP‐43 and FUS/TLS proteins

A Ratti, E Buratti - Journal of neurochemistry, 2016 - Wiley Online Library
The multiple roles played by RNA binding proteins in neurodegeneration have become
apparent following the discovery of TAR DNA binding protein 43 kDa (TDP‐43) and fused in …