The role of TDP-43 mislocalization in amyotrophic lateral sclerosis

TR Suk, MWC Rousseaux - Molecular neurodegeneration, 2020 - Springer
Since its discovery as a primary component in cytoplasmic aggregates in post-mortem tissue
of patients with Amyotrophic Lateral Sclerosis (ALS), TAR DNA Binding Protein 43 kDa (TDP …

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 …

TDP-43 pathology disrupts nuclear pore complexes and nucleocytoplasmic transport in ALS/FTD

CC Chou, YI Zhang, ME Umoh, SW Vaughan… - Nature …, 2018 - nature.com
The cytoplasmic mislocalization and aggregation of TAR DNA-binding protein-43 (TDP-43)
is a common histopathological hallmark of the amyotrophic lateral sclerosis and …

The mammalian ribo-interactome reveals ribosome functional diversity and heterogeneity

D Simsek, GC Tiu, RA Flynn, GW Byeon, K Leppek… - Cell, 2017 - cell.com
During eukaryotic evolution, ribosomes have considerably increased in size, forming a
surface-exposed ribosomal RNA (rRNA) shell of unknown function, which may create an …

PIKFYVE inhibition mitigates disease in models of diverse forms of ALS

ST Hung, GR Linares, WH Chang, Y Eoh, G Krishnan… - Cell, 2023 - cell.com
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that results from
many diverse genetic causes. Although therapeutics specifically targeting known causal …

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 …

Stability properties of neuronal microtubules

PW Baas, AN Rao, AJ Matamoros, L Leo - Cytoskeleton, 2016 - Wiley Online Library
Neurons are terminally differentiated cells that use their microtubule arrays not for cell
division but rather as architectural elements required for the elaboration of elongated axons …

[HTML][HTML] RNA dysregulation in amyotrophic lateral sclerosis

Z Butti, SA Patten - Frontiers in genetics, 2019 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is the most common adult-onset motor neuron disease
and is characterized by the degeneration of upper and lower motor neurons. It has become …

[HTML][HTML] Neurobiology of axonal transport defects in motor neuron diseases: Opportunities for translational research?

KJ De Vos, M Hafezparast - Neurobiology of disease, 2017 - Elsevier
Intracellular trafficking of cargoes is an essential process to maintain the structure and
function of all mammalian cell types, but especially of neurons because of their extreme …

[HTML][HTML] Stress granule mediated protein aggregation and underlying gene defects in the FTD-ALS spectrum

Y Baradaran-Heravi, C Van Broeckhoven… - Neurobiology of …, 2020 - Elsevier
Stress granules (SGs) are dynamic membraneless compartments composed out of RNA-
binding proteins (RBPs) and RNA molecules that assemble temporarily to allow the cell to …