Ontology highlight
ABSTRACT:
SUBMITTER: Bidhendi EE
PROVIDER: S-EPMC4887173 | biostudies-literature | 2016 Jun
REPOSITORIES: biostudies-literature
Bidhendi Elaheh Ekhtiari EE Bergh Johan J Zetterström Per P Andersen Peter M PM Marklund Stefan L SL Brännström Thomas T
The Journal of clinical investigation 20160503 6
Amyotrophic lateral sclerosis (ALS) is an adult-onset degeneration of motor neurons that is commonly caused by mutations in the gene encoding superoxide dismutase 1 (SOD1). Both patients and Tg mice expressing mutant human SOD1 (hSOD1) develop aggregates of unknown importance. In Tg mice, 2 different strains of hSOD1 aggregates (denoted A and B) can arise; however, the role of these aggregates in disease pathogenesis has not been fully characterized. Here, minute amounts of strain A and B hSOD1 ...[more]