Please use this identifier to cite or link to this item: https://doi.org/10.1016/j.cell.2011.07.021
DC FieldValue
dc.titleRescue of Δf508-CFTR trafficking via a GRASP-dependent unconventional secretion pathway
dc.contributor.authorGee, H.Y.
dc.contributor.authorNoh, S.H.
dc.contributor.authorTang, B.L.
dc.contributor.authorKim, K.H.
dc.contributor.authorLee, M.G.
dc.date.accessioned2014-11-26T07:47:42Z
dc.date.available2014-11-26T07:47:42Z
dc.date.issued2011-09-02
dc.identifier.citationGee, H.Y., Noh, S.H., Tang, B.L., Kim, K.H., Lee, M.G. (2011-09-02). Rescue of Δf508-CFTR trafficking via a GRASP-dependent unconventional secretion pathway. Cell 146 (5) : 746-760. ScholarBank@NUS Repository. https://doi.org/10.1016/j.cell.2011.07.021
dc.identifier.issn00928674
dc.identifier.urihttp://scholarbank.nus.edu.sg/handle/10635/109591
dc.description.abstractThe 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. © 2011 Elsevier Inc.
dc.description.urihttp://libproxy1.nus.edu.sg/login?url=http://dx.doi.org/10.1016/j.cell.2011.07.021
dc.sourceScopus
dc.typeArticle
dc.contributor.departmentBIOCHEMISTRY
dc.description.doi10.1016/j.cell.2011.07.021
dc.description.sourcetitleCell
dc.description.volume146
dc.description.issue5
dc.description.page746-760
dc.description.codenCELLB
dc.identifier.isiut000294477500015
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