This information has contributed to understand the mechanisms for acquired protein retention in humans as well but, unfortunately, the rationale is inadequate to explore the retention mechanism of mutant proteins occurring in Hepatic Endoplasmic Reticulum Storage Diseases (ERSD), i.e., Alpha-1-antitrypsin Deficiency (AATD) and Hereditary Hypofibrinogenemia with Hepatic Storage (HHHS) [9]. The gene discussed is SERPINA1; the disease is ornithine translocase deficiency.