Dataset Information
Impaired nucleocytoplasmic transport in SOD1-mediated ALS.
Ontology highlight
ABSTRACT:
SUBMITTER: Argueti-Ostrovsky S
PROVIDER: S-EPMC12922372 | biostudies-literature | 2026 Feb
REPOSITORIES: biostudies-literature
Publications
Impaired nucleocytoplasmic transport in SOD1-mediated ALS.
Molecular neurodegeneration 20260214 1
BACKGROUND: Impaired nucleocytoplasmic transport (NCT) has emerged as a shared pathogenic mechanism in various neurodegenerative disorders, including amyotrophic lateral sclerosis (ALS). Although mutations in the gene encoding superoxide dismutase 1 (SOD1) account for approximately 20% of familial ALS cases, the impact of mutant SOD1 accumulation on the NCT remains unclear. METHODS: Utilizing in vitro and in vivo models, patient-derived fibroblasts, and postmortem spinal cord tissues from ALS pa ...[more]