Mutant superoxide dismutase aggregates from human spinal cord transmit amyotrophic lateral sclerosis.

ABSTRACT: Motor neurons containing aggregates of superoxide dismutase 1 (SOD1) are hallmarks of amyotrophic lateral sclerosis (ALS) caused by mutations in the gene encoding SOD1. We have previously reported that two strains of mutant human (h) SOD1 aggregates (denoted A and B) can arise in hSOD1-transgenic models for ALS and that inoculation of such aggregates into the lumbar spinal cord of mice results in rostrally spreading, templated hSOD1 aggregation and premature fatal ALS-like disease. Here, we explored whether mutant hSOD1 aggregates with prion-like properties also exist in human ALS. Aggregate seeds were prepared from spinal cords from an ALS patient carrying the hSOD1^G127Gfs*7 truncation mutation and from mice transgenic for the same mutation. To separate from mono-, di- or any o

SUBMITTER: Ekhtiari Bidhendi E 

PROVIDER: S-EPMC6280858 | biostudies-literature | 2018 Dec

REPOSITORIES: biostudies-literature