Regulation of post-Golgi LH3 trafficking is essential for collagen homeostasis

Blerida Banushi, Federico Forneris (), Anna Straatman-Iwanowska, Adam Strange, Anne-Marie Lyne, Clare Rogerson, Jemima J. Burden, Wendy E. Heywood, Joanna Hanley, Ivan Doykov, Kornelis R. Straatman, Holly Smith, Danai Bem, Janos Kriston-Vizi, Gema Ariceta, Maija Risteli, Chunguang Wang, Rosalyn E. Ardill, Marcin Zaniew, Julita Latka-Grot, Simon N. Waddington, S. J. Howe, Francesco Ferraro, Asllan Gjinovci, Scott Lawrence, Mark Marsh, Mark Girolami, Laurent Bozec, Kevin Mills and Paul Gissen ()
Additional contact information
Blerida Banushi: MRC Laboratory for Molecular Cell Biology, University College London
Federico Forneris: The Armenise-Harvard Laboratory of Structural Biology, University of Pavia
Anna Straatman-Iwanowska: MRC Laboratory for Molecular Cell Biology, University College London
Adam Strange: Eastman Dental Institute, University College London
Anne-Marie Lyne: University College London
Clare Rogerson: MRC Laboratory for Molecular Cell Biology, University College London
Jemima J. Burden: MRC Laboratory for Molecular Cell Biology, University College London
Wendy E. Heywood: Institute of Child Health, University College London
Joanna Hanley: Institute of Child Health, University College London
Ivan Doykov: Institute of Child Health, University College London
Kornelis R. Straatman: Centre for Core Biotechnology Services, University of Leicester
Holly Smith: MRC Laboratory for Molecular Cell Biology, University College London
Danai Bem: Centre for Cardiovascular Sciences, School of Clinical and Experimental Medicine, College of Medical and Dental Sciences, University of Birmingham
Janos Kriston-Vizi: MRC Laboratory for Molecular Cell Biology, University College London
Gema Ariceta: University Hospital Vall d'Hebron, Universitat Autonoma Barcelona
Maija Risteli: Faculty of Biochemistry and Molecular Medicine, University of Oulu
Chunguang Wang: Medical Research Center Oulu, Oulu University Hospital, University of Oulu
Rosalyn E. Ardill: Royal Hospital for Sick Children
Marcin Zaniew: Children's Hospital
Julita Latka-Grot: Children's Memorial Health Institute
Simon N. Waddington: Institute for Women’s Health, University College London
S. J. Howe: Institute for Women’s Health, University College London
Francesco Ferraro: MRC Laboratory for Molecular Cell Biology, University College London
Asllan Gjinovci: MRC Laboratory for Molecular Cell Biology, University College London
Scott Lawrence: MRC Laboratory for Molecular Cell Biology, University College London
Mark Marsh: MRC Laboratory for Molecular Cell Biology, University College London
Mark Girolami: University of Warwick
Laurent Bozec: Eastman Dental Institute, University College London
Kevin Mills: Institute of Child Health, University College London
Paul Gissen: MRC Laboratory for Molecular Cell Biology, University College London

Nature Communications, 2016, vol. 7, issue 1, 1-14

Abstract: 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.

Date: 2016
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DOI: 10.1038/ncomms12111

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