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; Paul Gissen

doi:10.1038/ncomms12111

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-GrotSimon N Waddington, S J Howe, Francesco Ferraro, Asllan Gjinovci, Scott Lawrence, Mark Marsh, Mark Girolami, Laurent Bozec, Kevin Mills, Paul Gissen

Research output: Contribution to journal › Article › peer-review

27 Citations (Scopus)

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.

Original language	English
Pages (from-to)	12111
Journal	Nature Communications
Volume	7
DOIs	https://doi.org/10.1038/ncomms12111
Publication status	Published - 20 Jul 2016

Access to Document

10.1038/ncomms12111Licence: Creative Commons: Attribution (CC BY)

Cite this

Banushi, B., Forneris, F., Straatman-Iwanowska, A., Strange, A., Lyne, A-M., Rogerson, C., Burden, J. J., Heywood, W. E., Hanley, J., Doykov, I., Straatman, K. R., Smith, H., Bem, D., Kriston-Vizi, J., Ariceta, G., Risteli, M., Wang, C., Ardill, R. E., Zaniew, M., ... Gissen, P. (2016). Regulation of post-Golgi LH3 trafficking is essential for collagen homeostasis. Nature Communications, 7, 12111. https://doi.org/10.1038/ncomms12111

@article{b02e9cfc4bfe423db88397239a9667e5,

title = "Regulation of post-Golgi LH3 trafficking is essential for collagen homeostasis",

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.",

author = "Blerida Banushi and Federico Forneris and Anna Straatman-Iwanowska and Adam Strange and Anne-Marie Lyne and Clare Rogerson and Burden, {Jemima J} and Heywood, {Wendy E} and Joanna Hanley and Ivan Doykov and Straatman, {Kornelis R} and Holly Smith and Danai Bem and Janos Kriston-Vizi and Gema Ariceta and Maija Risteli and Chunguang Wang and Ardill, {Rosalyn E} and Marcin Zaniew and Julita Latka-Grot and Waddington, {Simon N} and Howe, {S J} and Francesco Ferraro and Asllan Gjinovci and Scott Lawrence and Mark Marsh and Mark Girolami and Laurent Bozec and Kevin Mills and Paul Gissen",

year = "2016",

month = jul,

day = "20",

doi = "10.1038/ncomms12111",

language = "English",

volume = "7",

pages = "12111",

journal = "Nature Communications",

issn = "2041-1723",

publisher = "Springer",

}

Banushi, B, Forneris, F, Straatman-Iwanowska, A, Strange, A, Lyne, A-M, Rogerson, C, Burden, JJ, Heywood, WE, Hanley, J, Doykov, I, Straatman, KR, Smith, H, Bem, D, Kriston-Vizi, J, Ariceta, G, Risteli, M, Wang, C, Ardill, RE, Zaniew, M, Latka-Grot, J, Waddington, SN, Howe, SJ, Ferraro, F, Gjinovci, A, Lawrence, S, Marsh, M, Girolami, M, Bozec, L, Mills, K & Gissen, P 2016, 'Regulation of post-Golgi LH3 trafficking is essential for collagen homeostasis', Nature Communications, vol. 7, pp. 12111. https://doi.org/10.1038/ncomms12111

TY - JOUR

T1 - Regulation of post-Golgi LH3 trafficking is essential for collagen homeostasis

AU - Banushi, Blerida

AU - Forneris, Federico

AU - Straatman-Iwanowska, Anna

AU - Strange, Adam

AU - Lyne, Anne-Marie

AU - Rogerson, Clare

AU - Burden, Jemima J

AU - Heywood, Wendy E

AU - Hanley, Joanna

AU - Doykov, Ivan

AU - Straatman, Kornelis R

AU - Smith, Holly

AU - Bem, Danai

AU - Kriston-Vizi, Janos

AU - Ariceta, Gema

AU - Risteli, Maija

AU - Wang, Chunguang

AU - Ardill, Rosalyn E

AU - Zaniew, Marcin

AU - Latka-Grot, Julita

AU - Waddington, Simon N

AU - Howe, S J

AU - Ferraro, Francesco

AU - Gjinovci, Asllan

AU - Lawrence, Scott

AU - Marsh, Mark

AU - Girolami, Mark

AU - Bozec, Laurent

AU - Mills, Kevin

AU - Gissen, Paul

PY - 2016/7/20

Y1 - 2016/7/20

N2 - 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.

AB - 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.

U2 - 10.1038/ncomms12111

DO - 10.1038/ncomms12111

M3 - Article

C2 - 27435297

SN - 2041-1723

VL - 7

SP - 12111

JO - Nature Communications

JF - Nature Communications

ER -

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

Abstract

Access to Document

Fingerprint

Cite this