LMAN1
The protein encoded by this gene is a membrane mannose-specific lectin that cycles between the endoplasmic reticulum, endoplasmic reticulum-Golgi intermediate compartment, and cis-Golgi, functioning as a cargo receptor for glycoprotein transport.
Full Name
lectin, mannose-binding, 1
Function
Mannose-specific lectin. May recognize sugar residues of glycoproteins, glycolipids, or glycosylphosphatidyl inositol anchors and may be involved in the sorting or recycling of proteins, lipids, or both. The LMAN1-MCFD2 complex forms a specific cargo receptor for the ER-to-Golgi transport of selected proteins.
Biological Process
Blood coagulationManual Assertion Based On ExperimentTAS:ProtInc
Endoplasmic reticulum organizationManual Assertion Based On ExperimentIBA:GO_Central
Endoplasmic reticulum to Golgi vesicle-mediated transportManual Assertion Based On ExperimentIBA:GO_Central
Golgi organizationManual Assertion Based On ExperimentIMP:UniProtKB
Negative regulation of protein targeting to mitochondrionManual Assertion Based On ExperimentHMP:ParkinsonsUK-UCL
Positive regulation of organelle organizationManual Assertion Based On ExperimentIMP:UniProtKB
Protein foldingManual Assertion Based On ExperimentTAS:ProtInc
Protein transportIEA:UniProtKB-KW
Cellular Location
Endoplasmic reticulum-Golgi intermediate compartment membrane
Golgi apparatus membrane
Endoplasmic reticulum membrane
Involvement in disease
Factor V and factor VIII combined deficiency 1 (F5F8D1):
A blood coagulation disorder characterized by bleeding symptoms similar to those in hemophilia or parahemophilia, that are caused by single deficiency of FV or FVIII, respectively. The most common symptoms are epistaxis, menorrhagia, and excessive bleeding during or after trauma. Plasma levels of coagulation factors V and VIII are in the range of 5 to 30% of normal.
Topology
Lumenal: 31-477
Helical: 478-498
Cytoplasmic: 499-510
PTM
The N-terminal may be partly blocked.