Amyotrophic lateral sclerosis: translating genetic discoveries into therapies

F Akçimen, ER Lopez, JE Landers, A Nath… - Nature Reviews …, 2023 - nature.com
Recent advances in sequencing technologies and collaborative efforts have led to
substantial progress in identifying the genetic causes of amyotrophic lateral sclerosis (ALS) …

Molecular mechanisms underlying nucleotide repeat expansion disorders

I Malik, CP Kelley, ET Wang, PK Todd - Nature reviews Molecular cell …, 2021 - nature.com
The human genome contains over one million short tandem repeats. Expansion of a subset
of these repeat tracts underlies over fifty human disorders, including common genetic …

Integrative transcriptomic analysis of the amyotrophic lateral sclerosis spinal cord implicates glial activation and suggests new risk genes

J Humphrey, S Venkatesh, R Hasan, JT Herb… - Nature …, 2023 - nature.com
Amyotrophic lateral sclerosis (ALS) is a progressively fatal neurodegenerative disease
affecting motor neurons in the brain and spinal cord. In this study, we investigated gene …

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 …

Autosomal dominant cerebellar ataxias: new genes and progress towards treatments

G Coarelli, M Coutelier, A Durr - The Lancet Neurology, 2023 - thelancet.com
Dominantly inherited spinocerebellar ataxias (SCAs) are associated with phenotypes that
range from pure cerebellar to multisystemic. The list of implicated genes has lengthened in …

Genotype-associated cerebellar profiles in ALS: focal cerebellar pathology and cerebro-cerebellar connectivity alterations

P Bede, RH Chipika, F Christidi… - Journal of Neurology …, 2021 - jnnp.bmj.com
Objective Cerebellar disease burden and cerebro-cerebellar connectivity alterations are
poorly characterised in amyotrophic lateral sclerosis (ALS) despite the likely contribution of …

Short tandem repeat expansions in sporadic amyotrophic lateral sclerosis and frontotemporal dementia

L Henden, LG Fearnley, N Grima, EP McCann… - Science …, 2023 - science.org
Pathogenic short tandem repeat (STR) expansions cause over 20 neurodegenerative
diseases. To determine the contribution of STRs in sporadic amyotrophic lateral sclerosis …

[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 …

ATAXIN-2 intermediate-length polyglutamine expansions elicit ALS-associated metabolic and immune phenotypes

R Vieira de Sá, E Sudria-Lopez… - Nature …, 2024 - nature.com
Intermediate-length repeat expansions in ATAXIN-2 (ATXN2) are the strongest genetic risk
factor for amyotrophic lateral sclerosis (ALS). At the molecular level, ATXN2 intermediate …

Genome-wide meta-analysis finds the ACSL5-ZDHHC6 locus is associated with ALS and links weight loss to the disease genetics

A Iacoangeli, T Lin, A Al Khleifat, AR Jones… - Cell Reports, 2020 - cell.com
We meta-analyze amyotrophic lateral sclerosis (ALS) genome-wide association study
(GWAS) data of European and Chinese populations (84,694 individuals). We find an …