IDEAS home Printed from https://ideas.repec.org/a/nat/natcom/v7y2016i1d10.1038_ncomms12111.html
   My bibliography  Save this article

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

Author

Listed:
  • Blerida Banushi

    (MRC Laboratory for Molecular Cell Biology, University College London)

  • Federico Forneris

    (The Armenise-Harvard Laboratory of Structural Biology, University of Pavia
    Bijvoet Center for Biomolecular Research, Faculty of Science, Utrecht University)

  • 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
    Unit of Cancer Research and Translational Medicine, Faculty of Medicine, University of Oulu
    Medical Research Center Oulu, Oulu University Hospital, University of Oulu)

  • Chunguang Wang

    (Medical Research Center Oulu, Oulu University Hospital, University of Oulu
    Medical Microbiology and Immunology, Unit of Biomedicine, Faculty of Medicine, 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
    Institute of Child Health, University College London
    Inherited Metabolic Diseases Unit, Great Ormond Street Hospital)

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.

Suggested Citation

  • 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. Straa, 2016. "Regulation of post-Golgi LH3 trafficking is essential for collagen homeostasis," Nature Communications, Nature, vol. 7(1), pages 1-14, November.
    • Handle: RePEc:nat:natcom:v:7:y:2016:i:1:d:10.1038_ncomms12111
    DOI: 10.1038/ncomms12111
    as

    Download full text from publisher

    File URL: https://www.nature.com/articles/ncomms12111
    File Function: Abstract
    Download Restriction: no
    File URL: https://libkey.io/10.1038/ncomms12111?utm_source=ideas
    LibKey link: if access is restricted and if your library uses this service, LibKey will redirect you to where you can use your library subscription to access this item
    ---><---

    More about this item

    Statistics

    Access and download statistics

    Corrections

    All material on this site has been provided by the respective publishers and authors. You can help correct errors and omissions. When requesting a correction, please mention this item's handle: RePEc:nat:natcom:v:7:y:2016:i:1:d:10.1038_ncomms12111. See general information about how to correct material in RePEc.

    If you have authored this item and are not yet registered with RePEc, we encourage you to do it here. This allows to link your profile to this item. It also allows you to accept potential citations to this item that we are uncertain about.

    We have no bibliographic references for this item. You can help adding them by using this form .

    If you know of missing items citing this one, you can help us creating those links by adding the relevant references in the same way as above, for each refering item. If you are a registered author of this item, you may also want to check the "citations" tab in your RePEc Author Service profile, as there may be some citations waiting for confirmation.

    For technical questions regarding this item, or to correct its authors, title, abstract, bibliographic or download information, contact: Sonal Shukla or Springer Nature Abstracting and Indexing (email available below). General contact details of provider: http://www.nature.com .

    Please note that corrections may take a couple of weeks to filter through the various RePEc services.

    IDEAS is a RePEc service. RePEc uses bibliographic data supplied by the respective publishers.