TDP-43 proteinopathies: a new wave of neurodegenerative diseases

EMJ De Boer, VK Orie, T Williams, MR Baker… - Journal of Neurology …, 2021 - jnnp.bmj.com
Inclusions of pathogenic deposits containing TAR DNA-binding protein 43 (TDP-43) are
evident in the brain and spinal cord of patients that present across a spectrum of …

There's something wrong with my MAM; the ER–mitochondria axis and neurodegenerative diseases

S Paillusson, R Stoica, P Gomez-Suaga… - Trends in …, 2016 - cell.com
Alzheimer's disease (AD), Parkinson's disease (PD), and amyotrophic lateral sclerosis with
associated frontotemporal dementia (ALS/FTD) are major neurodegenerative diseases for …

ER–mitochondria associations are regulated by the VAPB–PTPIP51 interaction and are disrupted by ALS/FTD-associated TDP-43

R Stoica, KJ De Vos, S Paillusson, S Mueller… - Nature …, 2014 - nature.com
Mitochondria and the endoplasmic reticulum (ER) form tight structural associations and
these facilitate a number of cellular functions. However, the mechanisms by which regions of …

Autophagy induction enhances TDP43 turnover and survival in neuronal ALS models

SJ Barmada, A Serio, A Arjun, B Bilican, A Daub… - Nature chemical …, 2014 - nature.com
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) have distinct clinical
features but a common pathology—cytoplasmic inclusions rich in transactive response …

Rodent models of amyotrophic lateral sclerosis

T Philips, JD Rothstein - Current protocols in pharmacology, 2015 - Wiley Online Library
Abstract Amyotrophic Lateral Sclerosis (ALS) is a motor neuron disease affecting upper and
lower motor neurons in the central nervous system. Patients with ALS develop extensive …

Genetics of amyotrophic lateral sclerosis

M Ghasemi, RH Brown - Cold Spring …, 2018 - perspectivesinmedicine.cshlp.org
Amyotrophic lateral sclerosis (ALS) is a devastating, uniformly lethal degenerative disorder
of motor neurons that overlaps clinically with frontotemporal dementia (FTD). Investigations …

New Insights on NOX Enzymes in the Central Nervous System

Z Nayernia, V Jaquet, KH Krause - Antioxidants & redox signaling, 2014 - liebertpub.com
Significance: There is increasing evidence that the generation of reactive oxygen species
(ROS) in the central nervous system (CNS) involves the NOX family of nicotinamide adenine …

ALS/FTD‐associated FUS activates GSK‐3β to disrupt the VAPB–PTPIP 51 interaction and ER–mitochondria associations

R Stoica, S Paillusson, P Gomez‐Suaga, JC Mitchell… - EMBO …, 2016 - embopress.org
Defective FUS metabolism is strongly associated with amyotrophic lateral sclerosis and
frontotemporal dementia (ALS/FTD), but the mechanisms linking FUS to disease are not …

A futile battle? Protein quality control and the stress of aging

R Higuchi-Sanabria, PA Frankino, JW Paul… - Developmental cell, 2018 - cell.com
There exists a phenomenon in aging research whereby early life stress can have positive
impacts on longevity. The mechanisms underlying these observations suggest a robust, long …

Genetics of amyotrophic lateral sclerosis: an update

S Chen, P Sayana, X Zhang, W Le - Molecular neurodegeneration, 2013 - Springer
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder involving
both upper motor neurons (UMN) and lower motor neurons (LMN). Enormous research has …