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 …

Development of multifunctional molecules as potential therapeutic candidates for Alzheimer's disease, Parkinson's disease, and amyotrophic lateral sclerosis in the …

MG Savelieff, G Nam, J Kang, HJ Lee, M Lee… - Chemical …, 2018 - ACS Publications
Neurodegenerative diseases pose a substantial socioeconomic burden on society.
Unfortunately, the aging world population and lack of effective cures foreshadow a negative …

Physiological functions and pathobiology of TDP‐43 and FUS/TLS proteins

A Ratti, E Buratti - Journal of neurochemistry, 2016 - Wiley Online Library
The multiple roles played by RNA binding proteins in neurodegeneration have become
apparent following the discovery of TAR DNA binding protein 43 kDa (TDP‐43) and fused in …

TAR DNA-binding protein 43 (TDP-43) liquid–liquid phase separation is mediated by just a few aromatic residues

HR Li, WC Chiang, PC Chou, WJ Wang… - Journal of Biological …, 2018 - ASBMB
Eukaryotic cells contain distinct organelles, but not all of these compartments are enclosed
by membranes. Some intrinsically disordered proteins mediate membraneless organelle …

TDP-43 and RNA form amyloid-like myo-granules in regenerating muscle

TO Vogler, JR Wheeler, ED Nguyen, MP Hughes… - Nature, 2018 - nature.com
A dominant histopathological feature in neuromuscular diseases, including amyotrophic
lateral sclerosis and inclusion body myopathy, is cytoplasmic aggregation of the RNA …

The mutational landscape of a prion-like domain

B Bolognesi, AJ Faure, M Seuma, JM Schmiedel… - Nature …, 2019 - nature.com
Insoluble protein aggregates are the hallmarks of many neurodegenerative diseases. For
example, aggregates of TDP-43 occur in nearly all cases of amyotrophic lateral sclerosis …

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 …

Towards a TDP-43-based biomarker for ALS and FTLD

E Feneberg, E Gray, O Ansorge, K Talbot… - Molecular …, 2018 - Springer
TDP-43 accumulates in nerve cells of nearly all cases of amyotrophic lateral sclerosis (ALS;
the commonest form of motor neuron disease) and in the majority of Tau-negative …

Detection of TAR DNA-binding protein 43 (TDP-43) oligomers as initial intermediate species during aggregate formation

RL French, ZR Grese, H Aligireddy, DD Dhavale… - Journal of Biological …, 2019 - ASBMB
Aggregates of the RNA-binding protein TDP-43 (TAR DNA-binding protein) are a hallmark of
the overlapping neurodegenerative disorders amyotrophic lateral sclerosis (ALS) and …

RNA-binding proteins in neurodegeneration: mechanisms in aggregate

EG Conlon, JL Manley - Genes & development, 2017 - genesdev.cshlp.org
Neurodegeneration is a leading cause of death in the developed world and a natural, albeit
unfortunate, consequence of longer-lived populations. Despite great demand for therapeutic …