Amyotrophic lateral sclerosis

EL Feldman, SA Goutman, S Petri, L Mazzini… - The Lancet, 2022 - thelancet.com
Amyotrophic lateral sclerosis is a fatal CNS neurodegenerative disease. Despite intensive
research, current management of amyotrophic lateral sclerosis remains suboptimal from …

Emerging insights into the complex genetics and pathophysiology of amyotrophic lateral sclerosis

SA Goutman, O Hardiman, A Al-Chalabi… - The Lancet …, 2022 - thelancet.com
Amyotrophic lateral sclerosis is a fatal neurodegenerative disease. The discovery of genes
associated with amyotrophic lateral sclerosis, commencing with SOD1 in 1993, started fairly …

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 …

Recent advances in the diagnosis and prognosis of amyotrophic lateral sclerosis

SA Goutman, O Hardiman, A Al-Chalabi… - The Lancet …, 2022 - thelancet.com
The diagnosis of amyotrophic lateral sclerosis can be challenging due to its heterogeneity in
clinical presentation and overlap with other neurological disorders. Diagnosis early in the …

[HTML][HTML] The pathobiology of TDP-43 C-terminal fragments in ALS and FTLD

BA Berning, AK Walker - Frontiers in neuroscience, 2019 - frontiersin.org
During neurodegenerative disease, the multifunctional RNA-binding protein TDP-43
undergoes a vast array of post-translational modifications, including phosphorylation …

Cognitive impairment across ALS clinical stages in a population-based cohort

A Chiò, C Moglia, A Canosa, U Manera, R Vasta… - Neurology, 2019 - AAN Enterprises
Objective To assess the association of the degree of severity of motor impairment to that of
cognitive impairment in a large cohort of patients with amyotrophic lateral sclerosis (ALS) …

HDAC6 in Diseases of Cognition and of Neurons

P LoPresti - Cells, 2020 - mdpi.com
Central nervous system (CNS) neurodegenerative diseases are characterized by faulty
intracellular transport, cognition, and aggregate regulation. Traditionally, neuroprotection …

Clinical trials in amyotrophic lateral sclerosis: a systematic review and perspective

C Wong, M Stavrou, E Elliott, JM Gregory… - Brain …, 2021 - academic.oup.com
Amyotrophic lateral sclerosis is a progressive and devastating neurodegenerative disease.
Despite decades of clinical trials, effective disease-modifying drugs remain scarce. To …

Genetics of amyotrophic lateral sclerosis

JM Gregory, D Fagegaltier, H Phatnani… - Current Genetic Medicine …, 2020 - Springer
Abstract Purpose of Review Amyotrophic lateral sclerosis and frontotemporal dementia (ALS-
FTD) spectrum disorder is a rare fatal disease with strong genetic influences. The …

[HTML][HTML] The genetic architecture of ALS

A Shatunov, A Al-Chalabi - Neurobiology of Disease, 2021 - Elsevier
Amyotrophic lateral sclerosis (ALS) is a heterogeneous group of neurological conditions
which have in common the progressive degeneration of upper and lower motor neurons …