Altered SOD1 maturation and post-translational modification in amyotrophic lateral sclerosis spinal cord.

ABSTRACT: Aberrant self-assembly and toxicity of wild-type and mutant superoxide dismutase 1 (SOD1) has been widely examined in silico, in vitro and in transgenic animal models of amyotrophic lateral sclerosis. Detailed examination of the protein in disease-affected tissues from amyotrophic lateral sclerosis patients, however, remains scarce. We used histological, biochemical and analytical techniques to profile alterations to SOD1 protein deposition, subcellular localization, maturation and post-translational modification in post-mortem spinal cord tissues from amyotrophic lateral sclerosis cases and controls. Tissues were dissected into ventral and dorsal spinal cord grey matter to assess the specificity of alterations within regions of motor neuron degeneration. We provide evidence of the misloca

SUBMITTER: Trist BG 

PROVIDER: S-EPMC9473357 | biostudies-literature | 2022 Sep

REPOSITORIES: biostudies-literature