Amyotrophic lateral sclerosis

MA Van Es, O Hardiman, A Chio, A Al-Chalabi… - The Lancet, 2017 - thelancet.com
Amyotrophic lateral sclerosis is characterised by the progressive loss of motor neurons in
the brain and spinal cord. This neurodegenerative syndrome shares pathobiological …

[HTML][HTML] The role of TDP-43 propagation in neurodegenerative diseases: integrating insights from clinical and experimental studies

M Jo, S Lee, YM Jeon, S Kim, Y Kwon… - Experimental & molecular …, 2020 - nature.com
Abstract TAR DNA-binding protein 43 (TDP-43) is a highly conserved nuclear RNA/DNA-
binding protein involved in the regulation of RNA processing. The accumulation of TDP-43 …

[HTML][HTML] Neurodegenerative disorders and gut-brain interactions

A Singh, TM Dawson, S Kulkarni - The Journal of clinical …, 2021 - Am Soc Clin Investig
Neurodegenerative disorders (NDs) affect essential functions not only in the CNS, but also
cause persistent gut dysfunctions, suggesting that they have an impact on both CNS and gut …

[HTML][HTML] Passive immunotherapies targeting Aβ and tau in Alzheimer's disease

SS Plotkin, NR Cashman - Neurobiology of Disease, 2020 - Elsevier
Amyloid-β (Aβ) and tau proteins currently represent the two most promising targets to treat
Alzheimer's disease. The most extensively developed method to treat the pathologic forms of …

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 …

[HTML][HTML] Opinion: more mouse models and more translation needed for ALS

EMC Fisher, L Greensmith, A Malaspina… - Molecular …, 2023 - Springer
Amyotrophic lateral sclerosis is a complex disorder most of which is 'sporadic'of unknown
origin but approximately 10% is familial, arising from single mutations in any of more than 30 …

[HTML][HTML] Molecular mechanisms underlying TDP-43 pathology in cellular and animal models of ALS and FTLD

A Wood, Y Gurfinkel, N Polain, W Lamont… - International journal of …, 2021 - mdpi.com
Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are
neurodegenerative disorders that exist on a disease spectrum due to pathological, clinical …

Enrichment of SARM1 alleles encoding variants with constitutively hyperactive NADase in patients with ALS and other motor nerve disorders

J Gilley, O Jackson, M Pipis, MA Estiar, A Al-Chalabi… - Elife, 2021 - elifesciences.org
SARM1, a protein with critical NADase activity, is a central executioner in a conserved
programme of axon degeneration. We report seven rare missense or in-frame microdeletion …

[HTML][HTML] Employing nanoparticle tracking analysis of salivary neuronal exosomes for early detection of neurodegenerative diseases

V Sharma, F Nikolajeff, S Kumar - Translational Neurodegeneration, 2023 - Springer
Neurodegenerative diseases are a set of progressive and currently incurable diseases that
are primarily caused by neuron degeneration. Neurodegenerative diseases often lead to …

[HTML][HTML] Green tea epigallocatechin-3-gallate (EGCG) targeting protein misfolding in drug discovery for neurodegenerative diseases

PB Gonçalves, ACR Sodero, Y Cordeiro - Biomolecules, 2021 - mdpi.com
The potential to treat neurodegenerative diseases (NDs) of the major bioactive compound of
green tea, epigallocatechin-3-gallate (EGCG), is well documented. Numerous findings now …