We then tested the functional role of PERK in PL trafficking in human fibroblasts derived from a healthy donor and a 2.5-mo-old girl presenting with Wolcott-Rallison Syndrome (WRS) clinically associated with neonatal diabetes and liver dysfunction, caused by a homozygous p.W681X/p.W681X nonsense mutation in EIF2AK3 (PERK). The gene discussed is EIF2AK3; the disease is Decreased liver function.