SOD1opathy in sporadic amyotrophic lateral sclerosis with high plasma misfolded SOD1 protein level and specific electrophysiological pattern
Li-Kai Tsai, Hsing-Jung Lai, Yih-Chih Kuo, Chen-Hung Ting, I-Fan Wang · FENS Forum 2024
The clinical manifestations of sporadic amyotrophic lateral sclerosis (ALS) are highly variable, implying existence of subgroups in pathophysiology. We studied a group of patients with sporadic ALS and an ALS mouse model having superoxide dismutase 1 (SOD1)/G93A transgenes using chemical precipitation followed by enzyme-linked immunosorbent assay analysis for measurement of plasma misfolded protein levels and by a nerve excitability test (NET) to investigate axonal membrane properties. Six of 19 patients (31.6%) with sporadic ALS had elevated plasma misfolded SOD1 protein levels. In sporadic A
ePoster · Neuroscience