| http://purl.uniprot.org/citations/18056485 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/18056485 | http://www.w3.org/2000/01/rdf-schema#comment | "Combined deficiency of factors V and VIII (F5F8D) is a bleeding disorder caused by mutations in LMAN1 or MCFD2. LMAN1 encodes ERGIC-53, a cargo receptor with an L-type lectin domain, and MCFD2 is a EF-hand-containing protein. We prepared a biotinylated, soluble form of ERGIC-53, which we labeled with R-phycoerythrin conjugated streptavidin. By flow cytometry, sERGIC-53-SA bound to HeLaS3 cells in the presence of calcium but only after preincubation with MCFD2. Treating the cells with endo H or incubating them with high mannose-type oligosaccharides, especially M(8B), abrogated sERGIC-53-SA binding. Surface plasmon resonance experiments demonstrated that MCFD2 specifically bound to sERGIC-53 and 2 MCFD2 mutants found in F5F8D patients had a K(a) that was 3 or 4 orders of magnitude lower for sERGIC-53 than for wild-type MCFD2. The K(a) of sERGIC-53 and MCFD2 was measured at several pH values and calcium concentrations, and we found that at a calcium concentration less than 0.2 mM, this interaction became significantly weaker. These results demonstrate that the binding of ERGIC-53 to sugar is enhanced by its interaction with MCFD2, and defects in this interaction in F5F8D patients may be the cause for reduced secretion of factors V and VIII."xsd:string |
| http://purl.uniprot.org/citations/18056485 | http://purl.org/dc/terms/identifier | "doi:10.1182/blood-2007-06-097022"xsd:string |
| http://purl.uniprot.org/citations/18056485 | http://purl.uniprot.org/core/author | "Ichikawa Y."xsd:string |
| http://purl.uniprot.org/citations/18056485 | http://purl.uniprot.org/core/author | "Ito Y."xsd:string |
| http://purl.uniprot.org/citations/18056485 | http://purl.uniprot.org/core/author | "Matsumoto N."xsd:string |
| http://purl.uniprot.org/citations/18056485 | http://purl.uniprot.org/core/author | "Yamamoto K."xsd:string |
| http://purl.uniprot.org/citations/18056485 | http://purl.uniprot.org/core/author | "Matsuo I."xsd:string |
| http://purl.uniprot.org/citations/18056485 | http://purl.uniprot.org/core/author | "Totani K."xsd:string |
| http://purl.uniprot.org/citations/18056485 | http://purl.uniprot.org/core/author | "Kawasaki N."xsd:string |
| http://purl.uniprot.org/citations/18056485 | http://purl.uniprot.org/core/date | "2008"xsd:gYear |
| http://purl.uniprot.org/citations/18056485 | http://purl.uniprot.org/core/name | "Blood"xsd:string |
| http://purl.uniprot.org/citations/18056485 | http://purl.uniprot.org/core/pages | "1972-1979"xsd:string |
| http://purl.uniprot.org/citations/18056485 | http://purl.uniprot.org/core/title | "The sugar-binding ability of ERGIC-53 is enhanced by its interaction with MCFD2."xsd:string |
| http://purl.uniprot.org/citations/18056485 | http://purl.uniprot.org/core/volume | "111"xsd:string |
| http://purl.uniprot.org/citations/18056485 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/18056485 |
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