Article
Crystal structure of the LMAN1-CRD/MCFD2 transport receptor complex provides insight into combined deficiency of factor V and factor VIII.
FEBS letters - 5 Mar 2010
Wigren Edvard, Bourhis Jean-Marie, Kursula Inari, Guy Jodie E, Lindqvist Ylva
Abstract excerpt
LMAN1 is a glycoprotein receptor, mediating transfer from the ER to the ER-Golgi intermediate compartment. Together with the co-receptor MCFD2, it transports coagulation factors V and VIII. Mutations in LMAN1 and MCFD2 can cause combined deficiency of factors V and VIII (F5F8D). We present the crystal structure of the LMAN1/MCFD2 complex and relate it to patient mutations. Circular dichroism data show that the...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.
