Endoplasmic reticulum stress leads to accumulation of wild-type SOD1 aggregates associated with sporadic amyotrophic lateral sclerosis

• DOI: 10.1073/pnas.1801109115
• Publication Date: 2018-07-23T19:16:21Z
• Authors: Danilo B. Medinas; Pablo Rozas; Francisca Martíne...; Ute Woehlbier; Robert H. Brown; Daryl A. Bosco; Claudio Hetz
• Keywords: Adult; Male; 0301 basic medicine; Aging; Mice, Transgenic; protein aggregation; Cell Line; Mice; 03 medical and health sciences; Wild-type SOD1; Animals; Humans; Aged; Aged, 80 and over; Motor Neurons; aging; Amyotrophic Lateral Sclerosis; Brain; Middle Aged; Amyotrophic lateral sclerosis; Endoplasmic Reticulum Stress; Mice, Inbred C57BL; Disease Models, Animal; wild-type SOD1; Astrocytes; Mutation; Female; Protein aggregation; ER stress

Abnormal modifications to mutant superoxide dismutase 1 (SOD1) are linked familial amyotrophic lateral sclerosis (fALS). Misfolding of wild-type SOD1 (SOD1