PDI and ERp57 were identified as the two main proteins upregulated in the spinal cord of a symptomatic ALS transgenic mice [44], and in the spinal cord and CSF of human sporadic ALS patients [45, 46] where PDI also colocalizes with abnormal protein inclusions associated with sporadic ALS [47]. Here, PDIA2 is linked to amyotrophic lateral sclerosis.