Promoting the clearance of neurotoxic proteins in neurodegenerative disorders of ageing

B Boland, WH Yu, O Corti, B Mollereau… - Nature reviews Drug …, 2018 - nature.com
Neurodegenerative disorders of ageing (NDAs) such as Alzheimer disease, Parkinson
disease, frontotemporal dementia, Huntington disease and amyotrophic lateral sclerosis …

The debated toxic role of aggregated TDP-43 in amyotrophic lateral sclerosis: a resolution in sight?

RC Hergesheimer, AA Chami, DR De Assis, P Vourc'h… - Brain, 2019 - academic.oup.com
Transactive response DNA-binding protein-43 (TDP-43) is an RNA/DNA binding protein that
forms phosphorylated and ubiquitinated aggregates in the cytoplasm of motor neurons in …

[HTML][HTML] In situ structure of neuronal C9orf72 poly-GA aggregates reveals proteasome recruitment

Q Guo, C Lehmer, A Martínez-Sánchez, T Rudack… - Cell, 2018 - cell.com
Protein aggregation and dysfunction of the ubiquitin-proteasome system are hallmarks of
many neurodegenerative diseases. Here, we address the elusive link between these …

Structure, dynamics and function of the 26S proteasome

Y Mao - Macromolecular protein complexes III: structure and …, 2021 - Springer
The 26S proteasome is the most complex ATP-dependent protease machinery, of~ 2.5 MDa
mass, ubiquitously found in all eukaryotes. It selectively degrades ubiquitin-conjugated …

HDAC6 inhibition restores TDP‐43 pathology and axonal transport defects in human motor neurons with TARDBP mutations

R Fazal, S Boeynaems, A Swijsen, M De Decker… - The EMBO …, 2021 - embopress.org
TDP‐43 is the major component of pathological inclusions in most ALS patients and in up to
50% of patients with frontotemporal dementia (FTD). Heterozygous missense mutations in …

Causative genes in amyotrophic lateral sclerosis and protein degradation pathways: a link to neurodegeneration

C Maurel, A Dangoumau, S Marouillat, C Brulard… - Molecular …, 2018 - Springer
Amyotrophic lateral sclerosis (ALS) is a disease caused by the degeneration of motor
neurons (MNs) leading to progressive muscle weakness and atrophy. Several molecular …

TFEB/Mitf links impaired nuclear import to autophagolysosomal dysfunction in C9-ALS

KM Cunningham, K Maulding, K Ruan, M Senturk… - Elife, 2020 - elifesciences.org
Disrupted nucleocytoplasmic transport (NCT) has been implicated in neurodegenerative
disease pathogenesis; however, the mechanisms by which disrupted NCT causes …

Disrupted endoplasmic reticulum-mediated autophagosomal biogenesis in a Drosophila model of C9-ALS-FTD

H Sung, TE Lloyd - Autophagy, 2024 - Taylor & Francis
Macroautophagy/autophagy is a major pathway for the clearance of protein aggregates and
damaged organelles, and multiple intracellular organelles participate in the process of …

VAMP associated proteins are required for autophagic and lysosomal degradation by promoting a PtdIns4P-mediated endosomal pathway

D Mao, G Lin, B Tepe, Z Zuo, KL Tan, M Senturk… - Autophagy, 2019 - Taylor & Francis
Mutations in the ER-associated VAPB/ALS8 protein cause amyotrophic lateral sclerosis and
spinal muscular atrophy. Previous studies have argued that ER stress may underlie the …

Drug screening in Drosophila; why, when, and when not?

TT Su - Wiley Interdisciplinary Reviews: Developmental …, 2019 - Wiley Online Library
The best global seller among oncology drugs in 2018 is lenalidomide, an analog of
thalidomide. It took 53 years and a circuitous route from the discovery of thalidomide to …