Deregulation of TDP-43 in amyotrophic lateral sclerosis triggers nuclear factor κB–mediated pathogenic pathways

V Swarup, D Phaneuf, N Dupré, S Petri… - Journal of Experimental …, 2011 - rupress.org
TDP-43 (TAR DNA-binding protein 43) inclusions are a hallmark of amyotrophic lateral
sclerosis (ALS). In this study, we report that TDP-43 and nuclear factor κB (NF-κB) p65 …

Inflammation induces TDP-43 mislocalization and aggregation

AS Correia, P Patel, K Dutta, JP Julien - PloS one, 2015 - journals.plos.org
TAR DNA-binding protein 43 (TDP-43) is a major component in aggregates of ubiquitinated
proteins in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration …

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 …

[HTML][HTML] Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice

LM Igaz, LK Kwong, EB Lee… - The Journal of …, 2011 - Am Soc Clin Investig
Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are
characterized by cytoplasmic protein aggregates in the brain and spinal cord that include …

Wild type human TDP-43 potentiates ALS-linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS

JC Mitchell, R Constable, E So, C Vance… - Acta neuropathologica …, 2015 - Springer
Introduction Amyotrophic lateral sclerosis (ALS) is a relentlessly progressive
neurodegenerative disorder, and cytoplasmic inclusions containing transactive response …

TDP-43 and inflammation: implications for amyotrophic lateral sclerosis and frontotemporal dementia

F Bright, G Chan, A van Hummel, LM Ittner… - International Journal of …, 2021 - mdpi.com
The abnormal mislocalisation and ubiquitinated protein aggregation of the TAR DNA
binding protein 43 (TDP-43) within the cytoplasm of neurons and glia in the central nervous …

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 …

Overexpression of ALS-associated p. M337V human TDP-43 in mice worsens disease features compared to wild-type human TDP-43 mice

J Janssens, H Wils, G Kleinberger, G Joris, I Cuijt… - Molecular …, 2013 - Springer
Mutations in TAR DNA-binding protein 43 (TDP-43) are associated with familial forms of
amyotrophic lateral sclerosis (ALS), while wild-type TDP-43 is a pathological hallmark of …

Activation of AMP-activated protein kinase α1 mediates mislocalization of TDP-43 in amyotrophic lateral sclerosis

YJ Liu, TC Ju, HM Chen, YS Jang… - Human molecular …, 2015 - academic.oup.com
TAR DNA-binding protein-43 (TDP-43) is a nuclear RNA-binding protein involved in many
cellular pathways. TDP-43-positive inclusions are a hallmark of amyotrophic lateral sclerosis …

Partial loss of TDP-43 function causes phenotypes of amyotrophic lateral sclerosis

C Yang, H Wang, T Qiao, B Yang… - Proceedings of the …, 2014 - National Acad Sciences
Amyotrophic lateral sclerosis (ALS) is a fatal neurological disease that causes motor neuron
degeneration, progressive motor dysfunction, paralysis, and death. Although multiple …