LMAN1 is a molecular chaperone for the secretion of coagulation factor VIII
Combined deficiency of both coagulation factors (F)V and VIII is a rare autosomal recessive bleeding disorder caused by null expression of LMAN1 (previously termed ERGIC-53) in a majority of affected individuals. Previously, a requirement for a functional LMAN1 cycling pathway between the ER and Gol...
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| Veröffentlicht in: | Journal of thrombosis and haemostasis 2003-11, Vol.1 (11), p.2360-2367 |
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| container_issue | 11 |
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| container_title | Journal of thrombosis and haemostasis |
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| creator | Cunningham, M A Pipe, S W Zhang, B Hauri, H-P Ginsburg, D Kaufman, R J |
| description | Combined deficiency of both coagulation factors (F)V and VIII is a rare autosomal recessive bleeding disorder caused by null expression of LMAN1 (previously termed ERGIC-53) in a majority of affected individuals. Previously, a requirement for a functional LMAN1 cycling pathway between the ER and Golgi was demonstrated for efficient secretion of FV and FVIII (Moussalli et al. J Biol Chem 1999; 274: 32569), however, the molecular nature of the interaction between LMAN1 and its cargo was not characterized. Using coimmunoprecipitation of LMAN1 and FVIII from transfected HeLa and COS-1 cells, we demonstrate an interaction between LMAN1 and FVIII in vivo. The interaction was mediated via high mannose-containing asparagine-linked oligosaccharides that are densely situated within the B domain of FVIII, as well as protein-protein interactions. These results are interpreted based on the recent determination of the crystal structure of the carbohydrate recognition domain of LMAN1. |
| doi_str_mv | 10.1046/j.1538-7836.2003.00415.x |
| format | Article |
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These results are interpreted based on the recent determination of the crystal structure of the carbohydrate recognition domain of LMAN1.</description><subject>Factor V Deficiency</subject><subject>Factor VIII - metabolism</subject><subject>Factor VIII - secretion</subject><subject>HeLa Cells</subject><subject>Hemophilia A</subject><subject>Humans</subject><subject>Mannose-Binding Lectins - deficiency</subject><subject>Mannose-Binding Lectins - metabolism</subject><subject>Mannose-Binding Lectins - physiology</subject><subject>Membrane Proteins - deficiency</subject><subject>Membrane Proteins - metabolism</subject><subject>Membrane Proteins - physiology</subject><subject>Molecular Chaperones - metabolism</subject><subject>Molecular Chaperones - physiology</subject><subject>Oligosaccharides</subject><subject>Precipitin Tests</subject><subject>Protein Binding</subject><subject>Protein Structure, Tertiary</subject><subject>Protein Transport</subject><subject>Transfection</subject><issn>1538-7933</issn><issn>1538-7836</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2003</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpFkLFOwzAQhj2AaCm8AvLElnC2EzseqwpoRIEFWC3HtWmqpC52IpW3J2krMZ1-3fffSR9CmEBKIOMP25TkrEhEwXhKAVgKkJE8PVyg6WkhGZug6xi3AETmFK7QhGScykzAFL2sXudvBNcRa9z6xpq-0QGbjd7b4HcWOx9wt7E4WhNsV_sd9g4br78H7hidNt3AfJVleYMunW6ivT3PGfp8evxYLJPV-3O5mK8Sw3LZJYwJ4AA5J5RqSTWjzkmASlBSCcsrbkVFdOFAinVhJadFlZk1ZUJnwHhu2Azdn-7ug__pbexUW0djm0bvrO-jEoRJybJiAIsTaIKPMVin9qFudfhVBNQoT23V6EiN8tQoTx3lqcNQvTv_6KvWrv-LZ3PsD2Sba4w</recordid><startdate>200311</startdate><enddate>200311</enddate><creator>Cunningham, M A</creator><creator>Pipe, S W</creator><creator>Zhang, B</creator><creator>Hauri, H-P</creator><creator>Ginsburg, D</creator><creator>Kaufman, R J</creator><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>200311</creationdate><title>LMAN1 is a molecular chaperone for the secretion of coagulation factor VIII</title><author>Cunningham, M A ; Pipe, S W ; Zhang, B ; Hauri, H-P ; Ginsburg, D ; Kaufman, R J</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c359t-337060056122a92a32ff900b721b7e6b6e7b1a8f097d8e9628b4cd237a40365c3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2003</creationdate><topic>Factor V Deficiency</topic><topic>Factor VIII - metabolism</topic><topic>Factor VIII - secretion</topic><topic>HeLa Cells</topic><topic>Hemophilia A</topic><topic>Humans</topic><topic>Mannose-Binding Lectins - deficiency</topic><topic>Mannose-Binding Lectins - metabolism</topic><topic>Mannose-Binding Lectins - physiology</topic><topic>Membrane Proteins - deficiency</topic><topic>Membrane Proteins - metabolism</topic><topic>Membrane Proteins - physiology</topic><topic>Molecular Chaperones - metabolism</topic><topic>Molecular Chaperones - physiology</topic><topic>Oligosaccharides</topic><topic>Precipitin Tests</topic><topic>Protein Binding</topic><topic>Protein Structure, Tertiary</topic><topic>Protein Transport</topic><topic>Transfection</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Cunningham, M A</creatorcontrib><creatorcontrib>Pipe, S W</creatorcontrib><creatorcontrib>Zhang, B</creatorcontrib><creatorcontrib>Hauri, H-P</creatorcontrib><creatorcontrib>Ginsburg, D</creatorcontrib><creatorcontrib>Kaufman, R J</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Journal of thrombosis and haemostasis</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Cunningham, M A</au><au>Pipe, S W</au><au>Zhang, B</au><au>Hauri, H-P</au><au>Ginsburg, D</au><au>Kaufman, R J</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>LMAN1 is a molecular chaperone for the secretion of coagulation factor VIII</atitle><jtitle>Journal of thrombosis and haemostasis</jtitle><addtitle>J Thromb Haemost</addtitle><date>2003-11</date><risdate>2003</risdate><volume>1</volume><issue>11</issue><spage>2360</spage><epage>2367</epage><pages>2360-2367</pages><issn>1538-7933</issn><issn>1538-7836</issn><abstract>Combined deficiency of both coagulation factors (F)V and VIII is a rare autosomal recessive bleeding disorder caused by null expression of LMAN1 (previously termed ERGIC-53) in a majority of affected individuals. 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| identifier | ISSN: 1538-7933 |
| ispartof | Journal of thrombosis and haemostasis, 2003-11, Vol.1 (11), p.2360-2367 |
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| source | MEDLINE; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals; Alma/SFX Local Collection |
| subjects | Factor V Deficiency Factor VIII - metabolism Factor VIII - secretion HeLa Cells Hemophilia A Humans Mannose-Binding Lectins - deficiency Mannose-Binding Lectins - metabolism Mannose-Binding Lectins - physiology Membrane Proteins - deficiency Membrane Proteins - metabolism Membrane Proteins - physiology Molecular Chaperones - metabolism Molecular Chaperones - physiology Oligosaccharides Precipitin Tests Protein Binding Protein Structure, Tertiary Protein Transport Transfection |
| title | LMAN1 is a molecular chaperone for the secretion of coagulation factor VIII |
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