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From the ER to the golgi: insights from the study of combined factors V and VIII deficiency.

The unexpected identification of ERGIC-53 as the gene responsible for combined FV/FVIII deficiency clearly establishes ERGIC-53 as a component of the ER-Golgi transport machinery that is required for the efficient export of coagulation factors V and VIII. This is the first demonstration of a cargo-specific pathway for protein export from the ER in mammalian cells. However, the apparently normal levels observed in these patients for most other plasma proteins demonstrate that ERGIC-53 is not essential for the integrity of the intermediate compartment or the more general process of protein export. The identification of the genetic defect in the subset of patients with FV/VIII deficiency but with intact ERGIC-53 may unmask additional critical components of this unique transport pathway.