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Article

Molecular basis of LMAN1 in coordinating LMAN1-MCFD2 cargo receptor formation and ER-to-Golgi transport of FV/FVIII.

Blood - 16 Dec 2010

Zheng Chunlei, Liu Hui-Hui, Yuan Shuguang, Zhou Jiahai, Zhang Bin

Abstract excerpt

The LMAN1-MCFD2 (lectin, mannose binding 1/multiple coagulation factor deficiency protein 2) cargo receptor complex transports coagulation factors V (FV) and VIII (FVIII) from the endoplasmic reticulum (ER) to the ER-Golgi intermediate compartment (ERGIC). LMAN1 (ERGIC-53) is a hexameric transmembrane protein with a carbohydrate recognition domain (CRD) on the ER luminal side. Here, we show that mutations in the...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

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Identifiers

PMID: 20817851
DOI: 10.1182/blood-2010-04-278325
PMCID: PMC3031414

Genes

LMAN1
MCFD2

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Research trail→

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Molecular basis of LMAN1 in coordinating LMAN1-MCFD2 cargo receptor formation and ER-to-Golgi transport of FV/FVIII.PMID 20817851Read ↗

Structural characterization of carbohydrate binding by LMAN1 protein provides new insight into the endoplasmic reticulum export of factors V (FV) and VIII (FVIII).Semantic proximity Read ↗

Separate roles of LMAN1 and MCFD2 in ER-to-Golgi trafficking of factor V and factor VIIISemantic proximity Read ↗

Bleeding due to disruption of a cargo-specific ER-to-Golgi transport complex.Semantic proximity Read ↗

Recent developments in the understanding of the combined deficiency of FV and FVIII.Semantic proximity Read ↗

Combined deficiency of coagulation factors V and VIII: an update.Semantic proximity Read ↗

Crystal structure of the LMAN1-CRD/MCFD2 transport receptor complex provides insight into combined deficiency of factor V and factor VIII.Semantic proximity Read ↗

Mutations in the MCFD2 gene and a novel mutation in the LMAN1 gene in Indian families with combined deficiency of factor V and VIII.Semantic proximity Read ↗

New insights into multiple coagulation factor deficiency from the solution structure of human MCFD2.Semantic proximity Read ↗

Combined deficiency of factor V and factor VIII is due to mutations in either LMAN1 or MCFD2.Semantic proximity Read ↗

Mice deficient in LMAN1 exhibit FV and FVIII deficiencies and liver accumulation of α1-antitrypsin.Semantic proximity Read ↗

Regulation of Mac-2BP secretion is mediated by its N-glycan binding to ERGIC-53.Semantic proximity Read ↗

Altered phenotype in LMAN1-deficient mice with low levels of residual LMAN1 expression.Semantic proximity Read ↗

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