Amyotrophic lateral sclerosis: a neurodegenerative disorder poised for successful therapeutic translation

RJ Mead, N Shan, HJ Reiser, F Marshall… - Nature Reviews Drug …, 2023 - nature.com
Amyotrophic lateral sclerosis (ALS) is a devastating disease caused by degeneration of
motor neurons. As with all major neurodegenerative disorders, development of disease …

TDP-43 pathology: from noxious assembly to therapeutic removal

SS Keating, R San Gil, MEV Swanson, EL Scotter… - Progress in …, 2022 - Elsevier
Our understanding of amyotrophic lateral sclerosis and frontotemporal dementia has
advanced dramatically since the discovery of cytoplasmic TAR DNA-binding protein 43 (TDP …

Synaptic dysfunction in ALS and FTD: anatomical and molecular changes provide insights into mechanisms of disease

PA Gelon, PA Dutchak, CF Sephton - Frontiers in Molecular …, 2022 - frontiersin.org
Synaptic loss is a pathological feature of all neurodegenerative diseases including
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). ALS is a disease of …

Sleep research in 2020: COVID-19-related sleep disorders

M Partinen - The Lancet. Neurology, 2020 - pmc.ncbi.nlm.nih.gov
2020 has been an unprecedented year because a modified coronavirus, severe acute
respiratory syndrome corona virus 2 (SARS-CoV-2), spread rapidly from China to all …

Pathologically mislocalised TDP-43 in upper motor neurons causes a die-forward spread of ALS-like pathogenic changes throughout the mouse corticomotor system

LA Reale, MS Dyer, SE Perry, KM Young… - Progress in …, 2023 - Elsevier
Alterations in upper motor neuron excitability are one of the earliest phenomena clinically
detected in ALS, and in 97% of cases, the RNA/DNA binding protein, TDP-43, is …

Molecular chaperones' potential against defective proteostasis of amyotrophic lateral sclerosis

S Kinger, AR Dubey, P Kumar, YA Jagtap… - Cells, 2023 - mdpi.com
Amyotrophic lateral sclerosis (ALS) is a neuronal degenerative condition identified via a
build-up of mutant aberrantly folded proteins. The native folding of polypeptides is mediated …

Aggregation-prone TDP-43 sequesters and drives pathological transitions of free nuclear TDP-43

SS Keating, AT Bademosi, R San Gil… - Cellular and Molecular …, 2023 - Springer
Aggregation of the RNA-binding protein, TDP-43, is the unifying hallmark of amyotrophic
lateral sclerosis and frontotemporal dementia. TDP-43-related neurodegeneration involves …

[HTML][HTML] Aberrant axon initial segment plasticity and intrinsic excitability of ALS hiPSC motor neurons

P Harley, C Kerins, A Gatt, G Neves, F Riccio… - Cell Reports, 2023 - cell.com
Dysregulated neuronal excitability is a hallmark of amyotrophic lateral sclerosis (ALS). We
sought to investigate how functional changes to the axon initial segment (AIS), the site of …

[HTML][HTML] Pathways to healing: Plants with therapeutic potential for neurodegenerative diseases

SEB Tyler, LDK Tyler - IBRO Neuroscience Reports, 2023 - Elsevier
Some of the greatest challenges in medicine are the neurodegenerative diseases (NDs),
which remain without a cure and mostly progress to death. A companion study employed a …

Familial ALS-associated SFPQ variants promote the formation of SFPQ cytoplasmic aggregates in primary neurons

J Widagdo, S Udagedara, N Bhembre… - Open …, 2022 - royalsocietypublishing.org
Splicing factor proline-and glutamine-rich (SFPQ) is a nuclear RNA-binding protein that is
involved in a wide range of physiological processes including neuronal development and …