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dc.contributor.authorLee, Ming Y
dc.contributor.authorSumpter, Rhea
dc.contributor.authorZou, Zhongju
dc.contributor.authorSirasanagandla, Shyam
dc.contributor.authorWei, Yongjie
dc.contributor.authorMishra, Prashant
dc.contributor.authorRosewich, Hendrik
dc.contributor.authorCrane, Denis I
dc.contributor.authorLevine, Beth
dc.date.accessioned2017-11-23T23:27:35Z
dc.date.available2017-11-23T23:27:35Z
dc.date.issued2017
dc.identifier.issn1469-221X
dc.identifier.doi10.15252/embr.201642443
dc.identifier.urihttp://hdl.handle.net/10072/124090
dc.description.abstractPEX13 is an integral membrane protein on the peroxisome that regulates peroxisomal matrix protein import during peroxisome biogenesis. Mutations in PEX13 and other peroxin proteins are associated with Zellweger syndrome spectrum (ZSS) disorders, a subtype of peroxisome biogenesis disorder characterized by prominent neurological, hepatic, and renal abnormalities leading to neonatal death. The lack of functional peroxisomes in ZSS patients is widely accepted as the underlying cause of disease; however, our understanding of disease pathogenesis is still incomplete. Here, we demonstrate that PEX13 is required for selective autophagy of Sindbis virus (virophagy) and of damaged mitochondria (mitophagy) and that disease‐associated PEX13 mutants I326T and W313G are defective in mitophagy. The mitophagy function of PEX13 is shared with another peroxin family member PEX3, but not with two other peroxins, PEX14 and PEX19, which are required for general autophagy. Together, our results demonstrate that PEX13 is required for selective autophagy, and suggest that dysregulation of PEX13‐mediated mitophagy may contribute to ZSS pathogenesis.
dc.description.peerreviewedYes
dc.languageEnglish
dc.language.isoeng
dc.publisherWiley-Blackwell Publishing
dc.relation.ispartofpagefrom1
dc.relation.ispartofpageto13
dc.relation.ispartofjournalEMBO Reports
dc.subject.fieldofresearchBiochemistry and cell biology
dc.subject.fieldofresearchBiochemistry and cell biology not elsewhere classified
dc.subject.fieldofresearchcode3101
dc.subject.fieldofresearchcode310199
dc.titlePeroxisomal protein PEX13 functions in selective autophagy
dc.typeJournal article
dc.type.descriptionC1 - Articles
dc.type.codeC - Journal Articles
dcterms.licensehttp://creativecommons.org/licenses/by-nc-nd/4.0/
dc.description.versionVersion of Record (VoR)
gro.description.notepublicThis publication has been entered into Griffith Research Online as an Advanced Online Version.
gro.rights.copyright© The Author(s) 2016. This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International (CC BY-NC-ND 4.0) License, which permits unrestricted, non-commercial use, distribution and reproduction in any medium, providing that the work is properly cited.
gro.hasfulltextFull Text
gro.griffith.authorCrane, Denis I.


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