Furthermore, Ast-HI in spinal cord of L84V SOD1 transgenic mice at symptomatic stage also showed KDEL positive (Fig. 6G and H), meaning that the principle features of these inclusions in neuroblastoma cells and the LBHI/Ast-HI of FALS patients are the same and implying LHI and LBHI/Ast-HI might develop in similar procedure. This evidence concerns the gene SOD1 and neuroblastoma.