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

The type 1-transmembrane protein LMAN1 (ERGIC-53) forms a complex with the soluble protein MCFD2 and cycles between the endoplasmic reticulum (ER) and the ER-Golgi intermediate compartment (ERGIC). Mutations in either LMAN1 or MCFD2 cause the combined deficiency of factor V (FV) and factor VIII (FVI...

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Detalhes bibliográficos
Main Authors: Zhang, Bin, Zheng, Chunlei, Zhu, Min, Tao, Jiayi, Vasievich, Matthew P., Baines, Andrea, Kim, Jinoh, Schekman, Randy, Kaufman, Randal J., Ginsburg, David
Formato: Artigo
Idioma:Inglês
Publicado em: American Society of Hematology 2011
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3179404/
https://ncbi.nlm.nih.gov/pubmed/21795745
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1182/blood-2011-05-352815
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