ArticleBlood2011
Mice deficient in LMAN1 exhibit FV and FVIII deficiencies and liver accumulation of α1-antitrypsin.
Article in Blood, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 39 papers.
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Who cites it
39 citing papers in PubMed, 55 citations in OpenAlex.
- Zinc-mediated structural and functional regulation of ERp44 and ERGIC-53 in protein quality control.Journal of biochemistry · 2026Review
- Article
- Sex-dependent influence of LMAN1 on allergen-induced airway hyperresponsiveness.Journal of immunology (Baltimore, Md. : 1950) · 2025Article
- The intracellular chylomicron highway: novel insights into chylomicron biosynthesis, trafficking, and secretion.Current opinion in lipidology · 2025Review
- Clinical, Laboratory, Molecular, and Reproductive Aspects of Combined Deficiency of Factors V and VIII.Seminars in thrombosis and hemostasis · 2025Review
- LMAN1 serves as a cargo receptor for thrombopoietin.JCI insight · 2024Article
- HCMV US2 co-opts TRC8 to degrade the endoplasmic reticulum-resident protein LMAN2L.The Journal of general virology · 2024Article
- Structure of full-length ERGIC-53 in complex with MCFD2 for cargo transport.Nature communications · 2024Article
- Identification of LMAN1- and SURF4-Dependent Secretory Cargoes.Journal of proteome research · 2023Article
- Mammalian cargo receptors for endoplasmic reticulum-to-Golgi transport: mechanisms and interactions.Biochemical Society transactions · 2023Review
- Separate roles of LMAN1 and MCFD2 in ER-to-Golgi trafficking of FV and FVIII.Blood advances · 2023Article
- Cargo selection in endoplasmic reticulum-to-Golgi transport and relevant diseases.The Journal of clinical investigation · 2023Review
- Pancreatic beta cell ER export in health and diabetes.Frontiers in endocrinology · 2023Review
- The Vascular Endothelium and Coagulation: Homeostasis, Disease, and Treatment, with a Focus on the Von Willebrand Factor and Factors VIII and V.International journal of molecular sciences · 2022Review
- LMAN1-MCFD2 complex is a cargo receptor for the ER-Golgi transport of α1-antitrypsin.The Biochemical journal · 2022Article
- High Mutational Heterogeneity, and New Mutations in the Human Coagulation Factor V Gene. Future Perspectives for Factor V Deficiency Using Recombinant and Advanced Therapies.International journal of molecular sciences · 2021Article
- Cargo receptor-assisted endoplasmic reticulum export of pathogenic α1-antitrypsin polymers.Cell reports · 2021Article
- Altered phenotype in LMAN1-deficient mice with low levels of residual LMAN1 expression.Blood advances · 2020Article
- The Endoplasmic Reticulum Cargo Receptor SURF4 Facilitates Efficient Erythropoietin Secretion.Molecular and cellular biology · 2020Article
- Murine Surf4 is essential for early embryonic development.PloS one · 2020Article
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Authors and funding
10 authors at 4 institutions in 1 country.
Funding
Abstract
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 (FVIII; F5F8D), suggesting an ER-to-Golgi cargo receptor function for the LMAN1-MCFD2 complex. Here we report the analysis of LMAN1-deficient mice. Levels of plasma FV and FVIII, and platelet FV, are all reduced to ∼ 50% of wild-type in Lman1(-/-) mice, compared with the 5%-30% levels typically observed in human F5F8D patients. Despite previous reports identifying cathepsin C, cathepsin Z, and α1-antitrypsin as additional potential cargoes for LMAN1, no differences were observed between wild-type and Lman1(-/-) mice in the levels of cathepsin C and cathepsin Z in liver lysates or α1-antitrypsin levels in plasma. LMAN1 deficiency had no apparent effect on COPII-coated vesicle formation in an in vitro assay. However, the ER in Lman1(-/-) hepatocytes is slightly distended, with significant accumulation of α1-antitrypsin and GRP78. An unexpected, partially penetrant, perinatal lethality was observed for Lman1(-/-) mice, dependent on the specific inbred strain genetic background, suggesting a potential role for other, as yet unidentified LMAN1-dependent cargo proteins.
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