[HTML][HTML] TDP-43 proteinopathy and ALS: insights into disease mechanisms and therapeutic targets

EL Scotter, HJ Chen, CE Shaw - Neurotherapeutics, 2015 - Elsevier
Therapeutic options for patients with amyotrophic lateral sclerosis (ALS) are currently
limited. However, recent studies show that almost all cases of ALS, as well as tau-negative …

The pathobiology of TDP-43 C-terminal fragments in ALS and FTLD

BA Berning, AK Walker - Frontiers in neuroscience, 2019 - frontiersin.org
During neurodegenerative disease, the multifunctional RNA-binding protein TDP-43
undergoes a vast array of post-translational modifications, including phosphorylation …

Prion-like propagation of protein misfolding and aggregation in amyotrophic lateral sclerosis

L McAlary, SS Plotkin, JJ Yerbury… - Frontiers in molecular …, 2019 - frontiersin.org
The discovery that prion protein can misfold into a pathological conformation that encodes
structural information capable of both propagation and inducing severe neuropathology has …

TDP-43 post-translational modifications in health and disease

E Buratti - Expert opinion on therapeutic targets, 2018 - Taylor & Francis
Introduction: Nuclear factor TDP-43 is a ubiquitously expressed RNA binding protein that
plays a key causative role in several neurodegenerative diseases, especially in the …

TDP-43 mutations link Amyotrophic Lateral Sclerosis with R-loop homeostasis and R loop-mediated DNA damage

M Giannini, A Bayona-Feliu, D Sproviero… - PLoS …, 2020 - journals.plos.org
TDP-43 is a DNA and RNA binding protein involved in RNA processing and with structural
resemblance to heterogeneous ribonucleoproteins (hnRNPs), whose depletion sensitizes …

[HTML][HTML] Exposure to ALS-FTD-CSF generates TDP-43 aggregates in glioblastoma cells through exosomes and TNTs-like structure

X Ding, M Ma, J Teng, RKF Teng, S Zhou, J Yin… - Oncotarget, 2015 - ncbi.nlm.nih.gov
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) represent a
continuum of devastating neurodegenerative diseases, characterized by transactive …

Distinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusions

NE Farrawell, IA Lambert-Smith, ST Warraich, IP Blair… - Scientific reports, 2015 - nature.com
Amyotrophic lateral sclerosis is a rapidly progressing neurodegenerative disease
associated with protein misfolding and aggregation. Most cases are characterized by TDP …

The N-terminal dimerization is required for TDP-43 splicing activity

LL Jiang, W Xue, JY Hong, JT Zhang, MJ Li, SN Yu… - Scientific reports, 2017 - nature.com
TDP-43 is a nuclear factor that functions in promoting pre-mRNA splicing. Deletion of the N-
terminal domain (NTD) and nuclear localization signal (NLS)(ie, TDP-35) results in …

Structural transformation of the amyloidogenic core region of TDP-43 protein initiates its aggregation and cytoplasmic inclusion

LL Jiang, MX Che, J Zhao, CJ Zhou, MY Xie… - Journal of Biological …, 2013 - ASBMB
TDP-43 (TAR DNA-binding protein of 43 kDa) is a major deposited protein in amyotrophic
lateral sclerosis and frontotemporal dementia with ubiquitin. A great number of genetic …

Metabolism and mis-metabolism of the neuropathological signature protein TDP-43

CC Huang, JK Bose, P Majumder… - Journal of cell …, 2014 - journals.biologists.com
ABSTRACT TDP-43 (also known as TARDBP) is a pathological signature protein of
neurodegenerative diseases, with TDP-43 proteinopathies including frontotemporal lobar …