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Regulation of post-Golgi LH3 trafficking is essential for collagen homeostasis
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, w...
Autores principales: | , , , , , , , , , , , , , , , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Nature Publishing Group
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4961739/ https://www.ncbi.nlm.nih.gov/pubmed/27435297 http://dx.doi.org/10.1038/ncomms12111 |
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author | 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 |
author_facet | 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 |
author_sort | Banushi, Blerida |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-4961739 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | Nature Publishing Group |
record_format | MEDLINE/PubMed |
spelling | pubmed-49617392016-09-06 Regulation of post-Golgi LH3 trafficking is essential for collagen homeostasis 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 Nat Commun Article 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. Nature Publishing Group 2016-07-20 /pmc/articles/PMC4961739/ /pubmed/27435297 http://dx.doi.org/10.1038/ncomms12111 Text en Copyright © 2016, Nature Publishing Group, a division of Macmillan Publishers Limited. All Rights Reserved. http://creativecommons.org/licenses/by/4.0/ This work is licensed under a Creative Commons Attribution 4.0 International License. The images or other third party material in this article are included in the article's Creative Commons license, unless indicated otherwise in the credit line; if the material is not included under the Creative Commons license, users will need to obtain permission from the license holder to reproduce the material. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/ |
spellingShingle | Article 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 Regulation of post-Golgi LH3 trafficking is essential for collagen homeostasis |
title | Regulation of post-Golgi LH3 trafficking is essential for collagen homeostasis |
title_full | Regulation of post-Golgi LH3 trafficking is essential for collagen homeostasis |
title_fullStr | Regulation of post-Golgi LH3 trafficking is essential for collagen homeostasis |
title_full_unstemmed | Regulation of post-Golgi LH3 trafficking is essential for collagen homeostasis |
title_short | Regulation of post-Golgi LH3 trafficking is essential for collagen homeostasis |
title_sort | regulation of post-golgi lh3 trafficking is essential for collagen homeostasis |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4961739/ https://www.ncbi.nlm.nih.gov/pubmed/27435297 http://dx.doi.org/10.1038/ncomms12111 |
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