ERGIC-53 protein; ER-Golgi intermediate compartment 53 kD protein; Gp58; intracellular mannose-specific lectin MR60; lectin mannose-binding 1 (LMAN1, ERGIC53, F5F8D)

Function: - mannose-specific lectin - may recognize sugars of glycoproteins, glycolipids, or glycosylphosphatidyl inositol anchors & play a role in the sorting or recycling of proteins, lipids, or both - interacts with MCFD2. (see LMAN1-MCFD2 complex) Structure: - forms disulfide-linked dimers & hexamers. Compartment: - endoplasmic reticulum-Golgi intermediate compartment (ERGIC) Expression: ubiquitous expression Pathology: - defects in ERGIC53 are a cause of multiple-coagulation-factor deficiency 1 (MCFD1)

General

Ca+2 binding protein glycoprotein membrane protein oligomerizing protein

Properties

SIZE: entity length = 510 aa MW = 58 kD COMPARTMENT: golgi endoplasmic reticulum MOTIF: signal sequence {1-30} L-type lectin-like {44-267} MOTIF: binding site SITE: 88-88 FOR-BINDING-OF: Carbohydrate binding site SITE: 121-121 FOR-BINDING-OF: Carbohydrate Ca+2-binding site SITE: 152-152 Ca+2-binding site SITE: 154-154 Ca+2-binding site SITE: 156-156 binding site SITE: 156-156 FOR-BINDING-OF: Carbohydrate binding site SITE: 178-178 FOR-BINDING-OF: Carbohydrate Ca+2-binding site SITE: 181-181 cysteine residue {C190} MODIFICATION: cysteine residue {C230} cysteine residue {C230} MODIFICATION: cysteine residue {C190} Carbohydrate binding {251-253} glycosylation site cysteine residue {C466} MODIFICATION: cysteine residue {CINTERCHAIN} cysteine residue {C475} MODIFICATION: cysteine residue {CINTERCHAIN} transmembrane domain {478-498} glutamine residue {501} ER export {509-510}

Database Correlations

OMIM correlations MORBIDMAP 601567 UniProt P49257 Pfam PF03388 Entrez Gene 3998 Kegg hsa:3998

References

UniProt :accession P49257