We found a set of ribosomal proteins and some components of protein processing in the ER that similarly varied in ALS mouse motor neurons and in ALS patients’ skin fibroblasts: 40S ribosomal proteins S3, S14, and S23 and 60 S ribosomal proteins L8 and L11 were down-regulated, while 78 kDa glucose-regulated protein (HSP5A), dolichyl-diphosphooligosaccharide-protein glycosyltransferase 48 kDa subunit, protein disulfide-isomerase A3 and calreticulin were up-regulated in both models. Here, DDOST is linked to amyotrophic lateral sclerosis.