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.

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Presentation transcript:

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 Blood Volume 118(12): September 22, 2011 ©2011 by American Society of Hematology

Characterization of mice carrying the Lman1 targeted allele. Bin Zhang et al. Blood 2011;118: ©2011 by American Society of Hematology

FV and FVIII levels in Lman1 WT (+/+), heterozygote (+/−), and homozygous (−/−) mice. Bin Zhang et al. Blood 2011;118: ©2011 by American Society of Hematology

Budding of COPII-coated vesicles is not affected by LMAN1 deficiency. Bin Zhang et al. Blood 2011;118: ©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: ©2011 by American Society of Hematology

Plasma and liver AAT levels. Bin Zhang et al. Blood 2011;118: ©2011 by American Society of Hematology