[HTML][HTML] ALS genetics, mechanisms, and therapeutics: where are we now?

R Mejzini, LL Flynn, IL Pitout, S Fletcher… - Frontiers in …, 2019 - frontiersin.org
The scientific landscape surrounding amyotrophic lateral sclerosis (ALS) continues to shift
as the number of genes associated with the disease risk and pathogenesis, and the cellular …

[HTML][HTML] Improving clinical trial outcomes in amyotrophic lateral sclerosis

MC Kiernan, S Vucic, K Talbot, CJ McDermott… - Nature Reviews …, 2021 - nature.com
Individuals who are diagnosed with amyotrophic lateral sclerosis (ALS) today face the same
historically intransigent problem that has existed since the initial description of the disease in …

Amyotrophic lateral sclerosis: an update for 2018

B Oskarsson, TF Gendron, NP Staff - Mayo clinic proceedings, 2018 - Elsevier
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting motor neurons
and other neuronal cells, leading to severe disability and eventually death from ventilatory …

Pharmacotherapy for amyotrophic lateral sclerosis: a review of approved and upcoming agents

SA Johnson, T Fang, F De Marchi, D Neel… - Drugs, 2022 - Springer
Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disorder
involving loss of upper and lower motor neurons, with most cases ending in death within 3–5 …

[HTML][HTML] Glycogen synthase kinase-3 inhibitors: preclinical and clinical focus on CNS-A decade onward

SM Arciniegas Ruiz, H Eldar-Finkelman - Frontiers in molecular …, 2022 - frontiersin.org
The protein kinase, GSK-3, participates in diverse biological processes and is now
recognized a promising drug discovery target in treating multiple pathological conditions …

Hypermetabolism in ALS is associated with greater functional decline and shorter survival

FJ Steyn, ZA Ioannides, RPA Van Eijk… - Journal of Neurology …, 2018 - jnnp.bmj.com
Objective To determine the prevalence of hypermetabolism, relative to body composition, in
amyotrophic lateral sclerosis (ALS) and its relationship with clinical features of disease and …

[HTML][HTML] The SOD1-mediated ALS phenotype shows a decoupling between age of symptom onset and disease duration

S Opie-Martin, A Iacoangeli, SD Topp, O Abel… - Nature …, 2022 - nature.com
Superoxide dismutase (SOD1) gene variants may cause amyotrophic lateral sclerosis, some
of which are associated with a distinct phenotype. Most studies assess limited variants or …

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 …

Disease-modifying therapies in amyotrophic lateral sclerosis

A Chiò, L Mazzini, G Mora - Neuropharmacology, 2020 - Elsevier
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder of adult life, causing
weakness and wasting of voluntary muscles, associated in about 50% of cases with a …

The impact of age on genetic testing decisions in amyotrophic lateral sclerosis

PR Mehta, A Iacoangeli, S Opie-Martin, JJFA van Vugt… - Brain, 2022 - academic.oup.com
Amyotrophic lateral sclerosis (ALS) is a heterogeneous neurodegenerative syndrome. In up
to 20% of cases, a family history is observed. Although Mendelian disease gene variants are …