Please use this identifier to cite or link to this item: http://hdl.handle.net/2381/39806
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dc.contributor.authorBanushi, Blerida-
dc.contributor.authorForneris, Federico-
dc.contributor.authorStraatman-Iwanowska, Anna-
dc.contributor.authorStrange, Adam-
dc.contributor.authorLyne, Anne-Marie-
dc.contributor.authorRogerson, Clare-
dc.contributor.authorBurden, Jemima J.-
dc.contributor.authorHeywood, Wendy E.-
dc.contributor.authorHanley, Joanna-
dc.contributor.authorDoykov, Ivan-
dc.contributor.authorStraatman, Kornelis R.-
dc.contributor.authorSmith, Holly-
dc.contributor.authorBem, Danai-
dc.contributor.authorKriston-Vizi, Janos-
dc.contributor.authorAriceta, Gema-
dc.contributor.authorRisteli, Maija-
dc.contributor.authorWang, Chunguang-
dc.contributor.authorArdill, Rosalyn E.-
dc.contributor.authorZaniew, Marcin-
dc.contributor.authorLatka-Grot, Julita-
dc.contributor.authorWaddington, Simon N.-
dc.contributor.authorHowe, S. J.-
dc.contributor.authorFerraro, Francesco-
dc.contributor.authorGjinovci, Asllan-
dc.contributor.authorLawrence, Scott-
dc.contributor.authorMarsh, Mark-
dc.contributor.authorGirolami, Mark-
dc.contributor.authorBozec, Laurent-
dc.contributor.authorMills, Kevin-
dc.contributor.authorGissen, Paul-
dc.date.accessioned2017-05-17T14:06:37Z-
dc.date.available2017-05-17T14:06:37Z-
dc.date.issued2016-07-20-
dc.identifier.citationNature Communications, 2016, 7, Article number: 12111en
dc.identifier.urihttps://www.nature.com/articles/ncomms12111en
dc.identifier.urihttp://hdl.handle.net/2381/39806-
dc.description.abstractPost-translational modifications are necessary for collagen precursor molecules (procollagens) to acquire final shape and function. However, the mechanism and contribution of collagen modifications that occur outside the endoplasmic reticulum and Golgi are not understood. We discovered that VIPAR, with its partner proteins, regulate sorting of lysyl hydroxylase 3 (LH3, also known as PLOD3) into newly identified post-Golgi collagen IV carriers and that VIPAR-dependent sorting is essential for modification of lysines in multiple collagen types. Identification of structural and functional collagen abnormalities in cells and tissues from patients and murine models of the autosomal recessive multisystem disorder Arthrogryposis, Renal dysfunction and Cholestasis syndrome caused by VIPAR and VPS33B deficiencies confirmed our findings. Thus, regulation of post-Golgi LH3 trafficking is essential for collagen homeostasis and for the development and function of multiple organs and tissues.en
dc.language.isoenen
dc.publisherNature Publishing Groupen
dc.relation.urihttps://www.ncbi.nlm.nih.gov/pubmed/27435297-
dc.rightsCopyright © the authors, 2016. This is an open-access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.en
dc.titleRegulation of post-Golgi LH3 trafficking is essential for collagen homeostasisen
dc.typeJournal Articleen
dc.identifier.doi10.1038/ncomms12111-
dc.identifier.eissn2041-1723-
dc.identifier.piincomms12111-
dc.description.statusPeer-revieweden
dc.description.versionPublisher Versionen
dc.type.subtypeJournal Article-
pubs.organisational-group/Organisationen
pubs.organisational-group/Organisation/COLLEGE OF MEDICINE, BIOLOGICAL SCIENCES AND PSYCHOLOGYen
pubs.organisational-group/Organisation/COLLEGE OF MEDICINE, BIOLOGICAL SCIENCES AND PSYCHOLOGY/MBSP Non-Medical Departmentsen
pubs.organisational-group/Organisation/COLLEGE OF MEDICINE, BIOLOGICAL SCIENCES AND PSYCHOLOGY/MBSP Non-Medical Departments/Old Departments Pre 01 Aug 2015en
pubs.organisational-group/Organisation/COLLEGE OF MEDICINE, BIOLOGICAL SCIENCES AND PSYCHOLOGY/MBSP Non-Medical Departments/Old Departments Pre 01 Aug 2015/Department of Biochemistry (Pre 01 Aug 2015)en
dc.dateaccepted2016-06-02-
Appears in Collections:Published Articles, Dept. of Biochemistry

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