PDIA3 and amyotrophic lateral sclerosis: Global knockout of the PDIA3 gene in mice is embryonic lethal (Coe et al., 2010), however, targeted knockout of PDIA3 in the murine nervous system leads to severe motor dysfunction and growth retardation associated with a loss of neuromuscular synapses reminiscent of calnexin deficiency (Kraus et al., 2010), and more recently, of amyotrophic lateral sclerosis in humans (Woehlbier et al., 2016).