[HTML][HTML] LMAN1 and MCFD2 Form a Cargo Receptor Complex and Interact withCoagulation Factor VIII in the Early SecretoryPathway

B Zhang, RJ Kaufman, D Ginsburg - Journal of Biological Chemistry, 2005 - ASBMB
Mutations in LMAN1 (ERGIC-53) and MCFD2 are thecauses of a human genetic disorder,
combined deficiency of coagulation factorV and factor VIII. LMAN1 is a type 1
transmembrane protein with homology tomannose-binding lectins. MCFD2 is a soluble EF-
hand-containing protein that isretained in the endoplasmic reticulum through its interaction
with LMAN1. Weshowed that endogenous LMAN1 and MCFD2 are present primarily in
complex witheach other with a 1: 1 stoichiometry, although MCFD2 is not required …