Mice deficient in LMAN1 exhibit FV and FVIII deficiencies and liver accumulation of α1-antitrypsin

by Bin Zhang, Chunlei Zheng, Min Zhu, Jiayi Tao, Matthew P. Vasievich, Andrea Baines, Jinoh Kim, Randy Schekman, Randal J. Kaufman, and David Ginsburg

BloodVolume 118(12):3384-3391

September 22, 2011

©2011 by American Society of Hematology

Characterization of mice carrying the Lman1 targeted allele.

Bin Zhang et al. Blood 2011;118:3384-3391

©2011 by American Society of Hematology

FV and FVIII levels in Lman1 WT (+/+), heterozygote (+/−), and homozygous (−/−) mice.

Bin Zhang et al. Blood 2011;118:3384-3391

©2011 by American Society of Hematology

Budding of COPII-coated vesicles is not affected by LMAN1 deficiency.

Bin Zhang et al. Blood 2011;118:3384-3391

©2011 by American Society of Hematology

ER morphology and expression of UPR markers in WT (+/+) and Lman1−/− cells.

Bin Zhang et al. Blood 2011;118:3384-3391

©2011 by American Society of Hematology

Plasma and liver AAT levels.

Bin Zhang et al. Blood 2011;118:3384-3391

©2011 by American Society of Hematology