The debated toxic role of aggregated TDP-43 in amyotrophic lateral sclerosis: a resolution in sight?
RC Hergesheimer, AA Chami, DR De Assis, P Vourc'h… - Brain, 2019 - academic.oup.com
Transactive response DNA-binding protein-43 (TDP-43) is an RNA/DNA binding protein that
forms phosphorylated and ubiquitinated aggregates in the cytoplasm of motor neurons in …
forms phosphorylated and ubiquitinated aggregates in the cytoplasm of motor neurons in …
Mutant TDP-43 within motor neurons drives disease onset but not progression in amyotrophic lateral sclerosis
D Ditsworth, M Maldonado, M McAlonis-Downes… - Acta …, 2017 - Springer
Mutations in TDP-43 cause amyotrophic lateral sclerosis (ALS), a fatal paralytic disease
characterized by degeneration and premature death of motor neurons. The contribution of …
characterized by degeneration and premature death of motor neurons. The contribution of …
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 …
of patients with Amyotrophic Lateral Sclerosis (ALS), TAR DNA Binding Protein 43 kDa (TDP …
Partial loss of TDP-43 function causes phenotypes of amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) is a fatal neurological disease that causes motor neuron
degeneration, progressive motor dysfunction, paralysis, and death. Although multiple …
degeneration, progressive motor dysfunction, paralysis, and death. Although multiple …
TDP‐43: the relationship between protein aggregation and neurodegeneration in amyotrophic lateral sclerosis and frontotemporal lobar degeneration
RH Baloh - The FEBS journal, 2011 - Wiley Online Library
Accumulations of aggregated proteins are a key feature of the pathology of all of the major
neurodegenerative diseases. Amyotrophic lateral sclerosis (ALS) was brought into this fold …
neurodegenerative diseases. Amyotrophic lateral sclerosis (ALS) was brought into this fold …
Distinct responses of neurons and astrocytes to TDP-43 proteinopathy in amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) is a fatal and incurable neurodegenerative disease
caused by motor neuron loss, resulting in muscle wasting, paralysis and eventual death. A …
caused by motor neuron loss, resulting in muscle wasting, paralysis and eventual death. A …
Phosphorylated TDP-43 aggregates in peripheral motor nerves of patients with amyotrophic lateral sclerosis
N Riva, F Gentile, F Cerri, F Gallia, P Podini, G Dina… - Brain, 2022 - academic.oup.com
Abstract Phosphorylated TDP-43 (pTDP-43) aggregates in the cytoplasm of motor neurons
and neuroglia in the brain are one of the pathological hallmarks of amyotrophic lateral …
and neuroglia in the brain are one of the pathological hallmarks of amyotrophic lateral …
Seeding the aggregation of TDP-43 requires post-fibrillization proteolytic cleavage
Despite the strong evidence linking the transactive response DNA-binding protein 43 (TDP-
43) aggregation to the pathogenesis of frontotemporal lobar degeneration with TDP-43 …
43) aggregation to the pathogenesis of frontotemporal lobar degeneration with TDP-43 …
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 …
RNA-related metabolism. Hyper-phosphorylated and ubiquitinated TDP-43 deposits act as …
TDP-43 activates microglia through NF-κB and NLRP3 inflammasome
W Zhao, DR Beers, S Bell, J Wang, S Wen… - Experimental …, 2015 - Elsevier
Transactive response DNA-binding protein-43 (TDP-43) is a multifunctional nucleic acid
binding protein present in ubiquitinated inclusions in tissues of patients with amyotrophic …
binding protein present in ubiquitinated inclusions in tissues of patients with amyotrophic …