TDP-43 triggers mitochondrial DNA release via mPTP to activate cGAS/STING in ALS
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 …
lateral sclerosis (ALS), associated with a neuroinflammatory cytokine profile related to …
The ALS disease-associated mutant TDP-43 impairs mitochondrial dynamics and function in motor neurons
W Wang, L Li, WL Lin, DW Dickson… - Human molecular …, 2013 - academic.oup.com
Mutations in TDP-43 lead to familial ALS. Expanding evidence suggests that impaired
mitochondrial dynamics likely contribute to the selective degeneration of motor neurons in …
mitochondrial dynamics likely contribute to the selective degeneration of motor neurons in …
Mitochondrial dysregulation occurs early in ALS motor cortex with TDP-43 pathology and suggests maintaining NAD+ balance as a therapeutic strategy
Mitochondrial defects result in dysregulation of metabolomics and energy homeostasis that
are detected in upper motor neurons (UMNs) with TDP-43 pathology, a pathology that is …
are detected in upper motor neurons (UMNs) with TDP-43 pathology, a pathology that is …
TDP-43 induces mitochondrial damage and activates the mitochondrial unfolded protein response
Mutations in or dys-regulation of the TDP-43 gene have been associated with TDP-43
proteinopathy, a spectrum of neurodegenerative diseases including Frontotemporal Lobar …
proteinopathy, a spectrum of neurodegenerative diseases including Frontotemporal Lobar …
Axonal TDP-43 condensates drive neuromuscular junction disruption through inhibition of local synthesis of nuclear encoded mitochondrial proteins
Mislocalization of the predominantly nuclear RNA/DNA binding protein, TDP-43, occurs in
motor neurons of~ 95% of amyotrophic lateral sclerosis (ALS) patients, but the contribution …
motor neurons of~ 95% of amyotrophic lateral sclerosis (ALS) patients, but the contribution …
S-nitrosylated TDP-43 triggers aggregation, cell-to-cell spread, and neurotoxicity in hiPSCs and in vivo models of ALS/FTD
E Pirie, C Oh, X Zhang, X Han… - Proceedings of the …, 2021 - National Acad Sciences
Rare genetic mutations result in aggregation and spreading of cognate proteins in
neurodegenerative disorders; however, in the absence of mutation (ie, in the vast majority of …
neurodegenerative disorders; however, in the absence of mutation (ie, in the vast majority of …
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 …
heterogeneous nuclear ribonucleoprotein and is the major pathological protein in …
Full-length TDP-43 and its C-terminal fragments activate mitophagy in NSC34 cell line
K Hong, Y Li, W Duan, Y Guo, H Jiang, W Li, C Li - Neuroscience letters, 2012 - Elsevier
TAR DNA binding protein of 43kDa (TDP-43), which has been associated with amyotrophic
lateral sclerosis (ALS), plays an essential role in neurodegenerative disease pathogenesis …
lateral sclerosis (ALS), plays an essential role in neurodegenerative disease pathogenesis …
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 …
neurodegenerative disorder, and cytoplasmic inclusions containing transactive response …
TDP-43 proteinopathy and mitochondrial abnormalities in neurodegeneration
Genetic mutations in TAR DNA-binding protein 43 (TDP-43) cause amyotrophic lateral
sclerosis (ALS) and frontotemporal dementia (FTD). Importantly, TDP-43 proteinopathy …
sclerosis (ALS) and frontotemporal dementia (FTD). Importantly, TDP-43 proteinopathy …