Please use this identifier to cite or link to this item: http://hdl.handle.net/2381/39806
Title: Regulation of post-Golgi LH3 trafficking is essential for collagen homeostasis
Authors: Banushi, Blerida
Forneris, Federico
Straatman-Iwanowska, Anna
Strange, Adam
Lyne, Anne-Marie
Rogerson, Clare
Burden, Jemima J.
Heywood, Wendy E.
Hanley, Joanna
Doykov, Ivan
Straatman, Kornelis R.
Smith, Holly
Bem, Danai
Kriston-Vizi, Janos
Ariceta, Gema
Risteli, Maija
Wang, Chunguang
Ardill, Rosalyn E.
Zaniew, Marcin
Latka-Grot, Julita
Waddington, Simon N.
Howe, S. J.
Ferraro, Francesco
Gjinovci, Asllan
Lawrence, Scott
Marsh, Mark
Girolami, Mark
Bozec, Laurent
Mills, Kevin
Gissen, Paul
First Published: 20-Jul-2016
Publisher: Nature Publishing Group
Citation: Nature Communications, 2016, 7, Article number: 12111
Abstract: Post-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.
DOI Link: 10.1038/ncomms12111
eISSN: 2041-1723
Links: https://www.nature.com/articles/ncomms12111
http://hdl.handle.net/2381/39806
Version: Publisher Version
Status: Peer-reviewed
Type: Journal Article
Rights: Copyright © 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.
Appears in Collections:Published Articles, Dept. of Biochemistry

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