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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...

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Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group 2016
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.
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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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