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http://hdl.handle.net/10261/174536
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Campo DC | Valor | Lengua/Idioma |
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dc.contributor.author | García-Castellanos, Raquel | es_ES |
dc.contributor.author | Sukusu Nielsen, Nadia | es_ES |
dc.contributor.author | Runager, Kasper | es_ES |
dc.contributor.author | Thøgersen, Ida B. | es_ES |
dc.contributor.author | Lukassen, Marie V. | es_ES |
dc.contributor.author | Poulsen, Ebbe T. | es_ES |
dc.contributor.author | Goulas, Theodoros | es_ES |
dc.contributor.author | Enghild, Jan J. | es_ES |
dc.contributor.author | Gomis-Rüth, F. Xavier | es_ES |
dc.date.accessioned | 2019-01-22T13:19:21Z | - |
dc.date.available | 2019-01-22T13:19:21Z | - |
dc.date.issued | 2017-11-07 | - |
dc.identifier.citation | Structure 25(11): 1740-1750.e2 (2017) | es_ES |
dc.identifier.issn | 0969-2126 | - |
dc.identifier.uri | http://hdl.handle.net/10261/174536 | - |
dc.description.abstract | A major cause of visual impairment, corneal dystrophies result from accumulation of protein deposits in the cornea. One of the proteins involved is transforming growth factor β-induced protein (TGFBIp), an extracellular matrix component that interacts with integrins but also produces corneal deposits when mutated. Human TGFBIp is a multi-domain 683-residue protein, which contains one CROPT domain and four FAS1 domains. Its structure spans ∼120 Å and reveals that vicinal domains FAS1-1/FAS1-2 and FAS1-3/FAS1-4 tightly interact in an equivalent manner. The FAS1 domains are sandwiches of two orthogonal four-stranded β sheets decorated with two three-helix insertions. The N-terminal FAS1 dimer forms a compact moiety with the structurally novel CROPT domain, which is a five-stranded all-β cysteine-knot solely found in TGFBIp and periostin. The overall TGFBIp architecture discloses regions for integrin binding and that most dystrophic mutations cluster at both molecule ends, within domains FAS1-1 and FAS1-4 | es_ES |
dc.description.sponsorship | This study was funded in part by grants from Spanish (BFU2015-64487-R and MDM-2014-0435) and Catalan (2014SGR9) public agencies, as well as from the Danish Council for Independent Research, Medical Sciences (DFF-4004- 00471), the Lundbeck Foundation (R164-2013-15912), the Velux Foundation, and Fight for Sight, Denmark. T.G. acknowledges a ‘‘Juan de la Cierva’’ research contract (JCI-2012-13573) from the Spanish Ministry for Economy and Competitiveness. The Structural Biology Unit of IBMB is a ‘‘Marı´a de Maeztu’’ Unit of Excellence of the Spanish Ministry of Economy, Industry and Competitiveness. Funding for data collection was provided in part by ESRF | es_ES |
dc.language.iso | eng | es_ES |
dc.publisher | Elsevier | es_ES |
dc.relation | info:eu-repo/grantAgreement/MINECO/Plan Estatal de Investigación Científica y Técnica y de Innovación 2013-2016/BFU2015-64487-R | es_ES |
dc.relation | info:eu-repo/grantAgreement/MINECO/Plan Estatal de Investigación Científica y Técnica y de Innovación 2013-2016/MDM-2014-0435 | - |
dc.relation.isversionof | Postprint | - |
dc.rights | openAccess | es_ES |
dc.subject | Corneal dystrophy | es_ES |
dc.subject | Fasciclin FAS1 domain | es_ES |
dc.subject | Cysteine-rich CROPT domain | es_ES |
dc.subject | Crystal structures | es_ES |
dc.subject | Multi-domain protein | es_ES |
dc.subject | Pathological mutants | es_ES |
dc.subject | Extracellular matrix protein | es_ES |
dc.title | Structural and functional implications of human transforming growth factor b-Induced protein, TGFBIp, in corneal dystrophies | es_ES |
dc.type | artículo | es_ES |
dc.identifier.doi | 10.1016/j.str.2017.09.001 | - |
dc.description.peerreviewed | Peer reviewed | es_ES |
dc.relation.publisherversion | https://doi.org/10.1016/j.str.2017.09.001 | es_ES |
dc.identifier.e-issn | 1878-4186 | - |
dc.contributor.funder | Ministerio de Economía y Competitividad (España) | es_ES |
dc.contributor.funder | Generalitat de Catalunya | es_ES |
dc.contributor.funder | Danish Council for Independent Research | es_ES |
dc.contributor.funder | Lundbeck Foundation | es_ES |
dc.contributor.funder | Velux Foundation | es_ES |
dc.contributor.funder | Fight for Sight Denmark foundation | es_ES |
dc.contributor.funder | European Synchrotron Radiation Facility | es_ES |
dc.relation.csic | Sí | es_ES |
oprm.item.hasRevision | no ko 0 false | * |
dc.identifier.funder | http://dx.doi.org/10.13039/501100001671 | es_ES |
dc.identifier.funder | http://dx.doi.org/10.13039/501100002809 | es_ES |
dc.identifier.funder | http://dx.doi.org/10.13039/501100003554 | es_ES |
dc.identifier.funder | http://dx.doi.org/10.13039/100007214 | es_ES |
dc.identifier.funder | http://dx.doi.org/10.13039/501100003329 | es_ES |
dc.contributor.orcid | Gomis-Rüth, F. Xavier [0000-0002-6848-6874] | es_ES |
dc.type.coar | http://purl.org/coar/resource_type/c_6501 | es_ES |
item.openairetype | artículo | - |
item.grantfulltext | open | - |
item.cerifentitytype | Publications | - |
item.openairecristype | http://purl.org/coar/resource_type/c_18cf | - |
item.fulltext | With Fulltext | - |
item.languageiso639-1 | en | - |
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Structural and functional_GarciaCastellanos.pdf | 11,24 MB | Adobe PDF | Visualizar/Abrir |
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