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Analysis of MCFD2- and LMAN1-deficient mice demonstrates distinct functions in vivo

The LMAN1–MCFD2 complex serves as a cargo receptor for efficient transport of factor V (FV) and FVIII from the endoplasmic reticulum (ER) to the Golgi. Genetic deficiency of LMAN1 or MCFD2 in humans results in the moderate bleeding disorder combined FV and FVIII deficiency, with a similar phenotype...

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Pubblicato in:Blood Adv
Autori principali: Zhu, Min, Zheng, Chunlei, Wei, Wei, Everett, Lesley, Ginsburg, David, Zhang, Bin
Natura: Artigo
Lingua:Inglês
Pubblicazione: American Society of Hematology 2018
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC5942004/
https://ncbi.nlm.nih.gov/pubmed/29735583
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1182/bloodadvances.2018018317
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