The role of TDP-43 mislocalization in amyotrophic lateral sclerosis

TR Suk, MWC Rousseaux - Molecular neurodegeneration, 2020 - Springer
Since its discovery as a primary component in cytoplasmic aggregates in post-mortem tissue
of patients with Amyotrophic Lateral Sclerosis (ALS), TAR DNA Binding Protein 43 kDa (TDP …

Molecular mechanisms of TDP-43 misfolding and pathology in amyotrophic lateral sclerosis

A Prasad, V Bharathi, V Sivalingam… - Frontiers in molecular …, 2019 - frontiersin.org
TAR DNA binding protein 43 (TDP-43) is a versatile RNA/DNA binding protein involved in
RNA-related metabolism. Hyper-phosphorylated and ubiquitinated TDP-43 deposits act as …

Heteromeric amyloid filaments of ANXA11 and TDP-43 in FTLD-TDP Type C

D Arseni, T Nonaka, MH Jacobsen, AG Murzin… - Nature, 2024 - nature.com
Neurodegenerative diseases are characterized by the abnormal filamentous assembly of
specific proteins in the central nervous system. Human genetic studies have established a …

[HTML][HTML] RNA binding antagonizes neurotoxic phase transitions of TDP-43

JR Mann, AM Gleixner, JC Mauna, E Gomes… - Neuron, 2019 - cell.com
TDP-43 proteinopathy is a pathological hallmark of amyotrophic lateral sclerosis and
frontotemporal dementia where cytoplasmic TDP-43 inclusions are observed within …

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 …

A single N‐terminal phosphomimic disrupts TDP‐43 polymerization, phase separation, and RNA splicing

A Wang, AE Conicella, HB Schmidt, EW Martin… - The EMBO …, 2018 - embopress.org
TDP‐43 is an RNA‐binding protein active in splicing that concentrates into membraneless
ribonucleoprotein granules and forms aggregates in amyotrophic lateral sclerosis (ALS) and …

The era of cryptic exons: implications for ALS-FTD

PR Mehta, AL Brown, ME Ward, P Fratta - Molecular Neurodegeneration, 2023 - Springer
TDP-43 is an RNA-binding protein with a crucial nuclear role in splicing, and mislocalises
from the nucleus to the cytoplasm in a range of neurodegenerative disorders. TDP-43 …

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 …

Heat-shock chaperone HSPB1 regulates cytoplasmic TDP-43 phase separation and liquid-to-gel transition

S Lu, J Hu, OA Arogundade, A Goginashvili… - Nature cell …, 2022 - nature.com
While acetylated, RNA-binding-deficient TDP-43 reversibly phase separates within nuclei
into complex droplets (anisosomes) comprised of TDP-43-containing liquid outer shells and …

Functional and dynamic polymerization of the ALS-linked protein TDP-43 antagonizes its pathologic aggregation

T Afroz, EM Hock, P Ernst, C Foglieni… - Nature …, 2017 - nature.com
TDP-43 is a primarily nuclear RNA-binding protein, whose abnormal phosphorylation and
cytoplasmic aggregation characterizes affected neurons in patients with amyotrophic lateral …