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 …

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 …

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 …

HDAC6 inhibition reverses axonal transport defects in motor neurons derived from FUS-ALS patients

W Guo, M Naujock, L Fumagalli, T Vandoorne… - Nature …, 2017 - nature.com
Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disorder due
to selective loss of motor neurons (MNs). Mutations in the fused in sarcoma (FUS) gene can …

When cell death goes wrong: inflammatory outcomes of failed apoptosis and mitotic cell death

FJ Bock, JS Riley - Cell Death & Differentiation, 2023 - nature.com
Apoptosis is a regulated cellular pathway that ensures that a cell dies in a structured fashion
to prevent negative consequences for the tissue or the organism. Dysfunctional apoptosis is …

Pathomechanisms of TDP‐43 in neurodegeneration

J Gao, L Wang, ML Huntley, G Perry… - Journal of …, 2018 - Wiley Online Library
Neurodegeneration, a term that refers to the progressive loss of structure and function of
neurons, is a feature of many neurodegenerative diseases such as amyotrophic lateral …

Structural insights into TDP-43 and effects of post-translational modifications

L François-Moutal, S Perez-Miller, DD Scott… - Frontiers in molecular …, 2019 - frontiersin.org
Transactive response DNA binding protein (TDP-43) is a key player in neurodegenerative
diseases. In this review, we have gathered and presented structural information on the …

Molecular mechanisms underlying TDP-43 pathology in cellular and animal models of ALS and FTLD

A Wood, Y Gurfinkel, N Polain, W Lamont… - International journal of …, 2021 - mdpi.com
Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are
neurodegenerative disorders that exist on a disease spectrum due to pathological, clinical …

Limbic-predominant age-related TDP43 encephalopathy (LATE) neuropathological change in neurodegenerative diseases

S Nag, JA Schneider - Nature Reviews Neurology, 2023 - nature.com
Abstracts TAR DNA-binding protein 43 (TDP43) is a focus of research in late-onset
dementias. TDP43 pathology in the brain was initially identified in amyotrophic lateral …

TDP-43 interacts with mitochondrial proteins critical for mitophagy and mitochondrial dynamics

SA Davis, S Itaman, CM Khalid-Janney, JA Sherard… - Neuroscience …, 2018 - Elsevier
Transactive response DNA-binding protein of 43 kDa (TDP-43) functions as a
heterogeneous nuclear ribonucleoprotein and is the major pathological protein in …