[HTML][HTML] 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 …

Differential roles of M1 and M2 microglia in neurodegenerative diseases

Y Tang, W Le - Molecular neurobiology, 2016 - Springer
One of the most striking hallmarks shared by various neurodegenerative diseases, including
Parkinson's disease, Alzheimer's disease (AD), and amyotrophic lateral sclerosis, is …

[PDF][PDF] TDP-43 triggers mitochondrial DNA release via mPTP to activate cGAS/STING in ALS

CH Yu, S Davidson, CR Harapas, JB Hilton… - Cell, 2020 - cell.com
Cytoplasmic accumulation of TDP-43 is a disease hallmark for many cases of amyotrophic
lateral sclerosis (ALS), associated with a neuroinflammatory cytokine profile related to …

[HTML][HTML] Cellular specificity of NF-κB function in the nervous system

EC Dresselhaus, MK Meffert - Frontiers in immunology, 2019 - frontiersin.org
Nuclear Factor Kappa B (NF-κB) is a ubiquitously expressed transcription factor with key
functions in a wide array of biological systems. While the role of NF-κB in processes, such as …

The changing scene of amyotrophic lateral sclerosis

W Robberecht, T Philips - Nature Reviews Neuroscience, 2013 - nature.com
Several recent breakthroughs have provided notable insights into the pathogenesis of
amyotrophic lateral sclerosis (ALS), with some even shifting our thinking about this …

Motor neuron disease-associated loss of nuclear TDP-43 is linked to DNA double-strand break repair defects

J Mitra, EN Guerrero, PM Hegde… - Proceedings of the …, 2019 - National Acad Sciences
Genome damage and their defective repair have been etiologically linked to degenerating
neurons in many subtypes of amyotrophic lateral sclerosis (ALS) patients; however, the …

[PDF][PDF] Microglia induce motor neuron death via the classical NF-κB pathway in amyotrophic lateral sclerosis

AE Frakes, L Ferraiuolo, AM Haidet-Phillips… - Neuron, 2014 - cell.com
Neuroinflammation is one of the most striking hallmarks of amyotrophic lateral sclerosis
(ALS). Nuclear factor-kappa B (NF-κB), a master regulator of inflammation, is upregulated in …

STING trafficking as a new dimension of immune signaling

D Jeltema, K Abbott, N Yan - Journal of Experimental Medicine, 2023 - rupress.org
The cGAS–STING pathway is an evolutionarily conserved immune signaling pathway critical
for microbial defense. Unlike other innate immune pathways that largely rely on stationary …

Axonal transport deficits and neurodegenerative diseases

S Millecamps, JP Julien - Nature Reviews Neuroscience, 2013 - nature.com
The intracellular transport of organelles along an axon is crucial for the maintenance and
function of a neuron. Anterograde axonal transport has a role in supplying proteins and …

[HTML][HTML] 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 …