Cited 241 times in
Rescue of ΔF508-CFTR trafficking via a GRASP-dependent unconventional secretion pathway.
DC Field | Value | Language |
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dc.contributor.author | 김경환 | - |
dc.contributor.author | 이민구 | - |
dc.contributor.author | 노신혜 | - |
dc.date.accessioned | 2014-12-20T16:53:13Z | - |
dc.date.available | 2014-12-20T16:53:13Z | - |
dc.date.issued | 2011 | - |
dc.identifier.issn | 0092-8674 | - |
dc.identifier.uri | https://ir.ymlib.yonsei.ac.kr/handle/22282913/93566 | - |
dc.description.abstract | The most prevalent disease-causing mutation of CFTR is the deletion of Phe508 (ΔF508), which leads to defects in conventional Golgi-mediated exocytosis and cell surface expression. We report that ΔF508-CFTR surface expression can be rescued in vitro and in vivo by directing it to an unconventional GRASP-dependent secretion pathway. An integrated molecular and physiological analysis indicates that mechanisms associated with ER stress induce cell surface trafficking of the ER core-glycosylated wild-type and ΔF508-CFTR via the GRASP-dependent pathway. Phosphorylation of a specific site of GRASP and the PDZ-based interaction between GRASP and CFTR are critical for this unconventional surface trafficking. Remarkably, transgenic expression of GRASP in ΔF508-CFTR mice restores CFTR function and rescues mouse survival without apparent toxicity. These findings provide insight into how unconventional protein secretion is activated, and offer a potential therapeutic strategy for the treatment of cystic fibrosis and perhaps diseases stemming from other misfolded proteins | - |
dc.description.statementOfResponsibility | open | - |
dc.relation.isPartOf | CELL | - |
dc.rights | CC BY-NC-ND 2.0 KR | - |
dc.rights.uri | https://creativecommons.org/licenses/by-nc-nd/2.0/kr/ | - |
dc.subject.MESH | Animals | - |
dc.subject.MESH | Carrier Proteins/metabolism* | - |
dc.subject.MESH | Cell Membrane/metabolism | - |
dc.subject.MESH | Cystic Fibrosis Transmembrane Conductance Regulator/metabolism* | - |
dc.subject.MESH | Endoplasmic Reticulum/metabolism | - |
dc.subject.MESH | Membrane Proteins/metabolism* | - |
dc.subject.MESH | Mice | - |
dc.subject.MESH | Protein Transport | - |
dc.subject.MESH | Secretory Pathway* | - |
dc.title | Rescue of ΔF508-CFTR trafficking via a GRASP-dependent unconventional secretion pathway. | - |
dc.type | Article | - |
dc.contributor.college | College of Medicine (의과대학) | - |
dc.contributor.department | Dept. of Pharmacology (약리학) | - |
dc.contributor.googleauthor | Heon Yung Gee | - |
dc.contributor.googleauthor | Shin Hye Noh | - |
dc.contributor.googleauthor | Bor Luen Tang | - |
dc.contributor.googleauthor | Kyung Hwan Kim | - |
dc.contributor.googleauthor | Min Goo Lee | - |
dc.identifier.doi | 10.1016/j.cell.2011.07.021 | - |
dc.admin.author | false | - |
dc.admin.mapping | false | - |
dc.contributor.localId | A00311 | - |
dc.contributor.localId | A02781 | - |
dc.relation.journalcode | J00472 | - |
dc.identifier.eissn | 1097-4172 | - |
dc.identifier.pmid | 21884936 | - |
dc.identifier.url | http://www.sciencedirect.com/science/article/pii/S0092867411008191 | - |
dc.contributor.alternativeName | Kim, Kyung Hwan | - |
dc.contributor.alternativeName | Lee, Min Goo | - |
dc.contributor.affiliatedAuthor | Kim, Kyung Hwan | - |
dc.contributor.affiliatedAuthor | Lee, Min Goo | - |
dc.rights.accessRights | not free | - |
dc.citation.volume | 146 | - |
dc.citation.number | 5 | - |
dc.citation.startPage | 746 | - |
dc.citation.endPage | 760 | - |
dc.identifier.bibliographicCitation | CELL, Vol.146(5) : 746-760, 2011 | - |
dc.identifier.rimsid | 28314 | - |
dc.type.rims | ART | - |
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