Protein ERGIC-53, also known as lectin mannose-binding 1 (LMAN1) is a cargo receptor responsible for the export of a subset of glycoproteins from the endoplasmic reticulum. LMAN1 binds to both glycoprotein cargo and its soluble coreceptor MCFD2 through its carbohydrate recognition domain (CRD) in a Ca(2+)-dependent manner []. LMAN1 is a lectin carrier protein that may mediate MMP-9 (matrix metalloproteinase-9) secretion [].Mutations in the LMAN1 and the MCFD2 genes cause combined deficiency of factor V (FV) and factor VIII (FVIII) (F5F8D, or FV+FVIII), an autosomal recessive bleeding disorder [].
