Mannose-dependent endoplasmic reticulum (ER)-Golgi intermediate compartment-53-mediated ER to Golgi trafficking of coagulation factors V and VIII
M Moussalli, SW Pipe, HP Hauri, WC Nichols… - Journal of Biological …, 1999 - ASBMB
The endoplasmic reticulum-Golgi intermediate compartment (ERGIC) is the site of
segregation of secretory proteins for anterograde transport, via packaging into COPII-coated
transport vesicles. ERGIC-53 is a homo-hexameric transmembrane lectin localized to the
ERGIC that exhibits mannose-selective properties in vitro. Null mutations in ERGIC-53 were
recently shown to be responsible for the autosomal recessive bleeding disorder, combined
deficiency of coagulation factors V and VIII. We have studied the effect of defective ER to …
segregation of secretory proteins for anterograde transport, via packaging into COPII-coated
transport vesicles. ERGIC-53 is a homo-hexameric transmembrane lectin localized to the
ERGIC that exhibits mannose-selective properties in vitro. Null mutations in ERGIC-53 were
recently shown to be responsible for the autosomal recessive bleeding disorder, combined
deficiency of coagulation factors V and VIII. We have studied the effect of defective ER to …