First, ER stress in neuroblastoma cells expressing mutant SOD1 results in SOD1- and ubiquitin-immunopositive LHIs, compatible with LBHI/Ast-HI, composed of granule-coated fibrils approximately 15–25 nm in diameter and granular materials (Figs. 5 and 6). Here, SOD1 is linked to neuroblastoma.