Cargo transport by the LMAN1-MCFD2 complex. (A) Factor V (FV), factor VIII (FVIII), cathepsin C (CatC), and cathepsin Z (CatZ) bind the LMAN1-MCFD2 complex and are packaged into COPII vesicles. (B) COPII vesicles bud from the ER membrane and fuse together forming the ER-Golgi intermediate compartment (ERGIC). (C) Anterograde transport of cargo protein to the Golgi apparatus occurs along microtubules. (D) The LMAN1-MCFD2 complex is recycled back to the ER through COPI vesicles. (E) Posttranslational modification of the cargo proteins occurs in the Golgi apparatus. (F) CatC and CatZ are transported to the lysosomes. (G) FV and FVIII are secreted outside of the cells. (H) In murine megakaryocytes, FV is transported and stored in α-granules. Reprinted from Baines and Zhang45 with permission.