Autophagy and ALS: mechanistic insights and therapeutic implications

JP Chua, H De Calbiac, E Kabashi, SJ Barmada - Autophagy, 2022 - Taylor & Francis
Mechanisms of protein homeostasis are crucial for overseeing the clearance of misfolded
and toxic proteins over the lifetime of an organism, thereby ensuring the health of neurons …

Glutamate and GABA in microglia-neuron cross-talk in Alzheimer's disease

GA Czapski, JB Strosznajder - International Journal of Molecular Sciences, 2021 - mdpi.com
The physiological balance between excitation and inhibition in the brain is significantly
affected in Alzheimer's disease (AD). Several neuroactive compounds and their signaling …

Protein aggregation in amyotrophic lateral sclerosis

AM Blokhuis, EJN Groen, M Koppers… - Acta …, 2013 - Springer
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the
aggregation of ubiquitinated proteins in affected motor neurons. Recent studies have …

TDP-43 is intercellularly transmitted across axon terminals

MS Feiler, B Strobel, A Freischmidt, AM Helferich… - Journal of Cell …, 2015 - rupress.org
Transactive response DNA-binding protein 43 kD (TDP-43) is an aggregation-prone prion-
like domain-containing protein and component of pathological intracellular aggregates …

Gains or losses: molecular mechanisms of TDP43-mediated neurodegeneration

EB Lee, VMY Lee, JQ Trojanowski - Nature Reviews Neuroscience, 2012 - nature.com
RNA-binding proteins, and in particular TAR DNA-binding protein 43 (TDP43), are central to
the pathogenesis of motor neuron diseases and related neurodegenerative disorders …

ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43

ES Arnold, SC Ling, SC Huelga… - Proceedings of the …, 2013 - National Acad Sciences
Transactivating response region DNA binding protein (TDP-43) is the major protein
component of ubiquitinated inclusions found in amyotrophic lateral sclerosis (ALS) and …

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 …

TDP‐43 regulates its mRNA levels through a negative feedback loop

YM Ayala, L De Conti, SE Avendaño‐Vázquez… - The EMBO …, 2011 - embopress.org
TAR DNA‐binding protein (TDP‐43) is an evolutionarily conserved heterogeneous nuclear
ribonucleoprotein (hnRNP) involved in RNA processing, whose abnormal cellular …

Loss of nuclear TDP-43 is associated with decondensation of LINE retrotransposons

EY Liu, J Russ, CP Cali, JM Phan, A Amlie-Wolf… - Cell reports, 2019 - cell.com
Loss of the nuclear RNA binding protein TAR DNA binding protein-43 (TDP-43) into
cytoplasmic aggregates is the strongest correlate to neurodegeneration in amyotrophic …

Functional recovery in new mouse models of ALS/FTLD after clearance of pathological cytoplasmic TDP-43

AK Walker, KJ Spiller, G Ge, A Zheng, Y Xu… - Acta …, 2015 - Springer
Accumulation of phosphorylated cytoplasmic TDP-43 inclusions accompanied by loss of
normal nuclear TDP-43 in neurons and glia of the brain and spinal cord are the molecular …