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Regulation of the Extracellular Matrix by Ciliary Machinery

The primary cilium is an organelle involved in cellular signalling. Mutations affecting proteins involved in cilia assembly or function result in diseases known as ciliopathies, which cause a wide variety of phenotypes across multiple tissues. These mutations disrupt various cellular processes, incl...

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Detalles Bibliográficos
Autores principales: I, Collins, A.K.T, Wann
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7072529/
https://www.ncbi.nlm.nih.gov/pubmed/31979260
http://dx.doi.org/10.3390/cells9020278
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author I, Collins
A.K.T, Wann
author_facet I, Collins
A.K.T, Wann
author_sort I, Collins
collection PubMed
description The primary cilium is an organelle involved in cellular signalling. Mutations affecting proteins involved in cilia assembly or function result in diseases known as ciliopathies, which cause a wide variety of phenotypes across multiple tissues. These mutations disrupt various cellular processes, including regulation of the extracellular matrix. The matrix is important for maintaining tissue homeostasis through influencing cell behaviour and providing structural support; therefore, the matrix changes observed in ciliopathies have been implicated in the pathogenesis of these diseases. Whilst many studies have associated the cilium with processes that regulate the matrix, exactly how these matrix changes arise is not well characterised. This review aims to bring together the direct and indirect evidence for ciliary regulation of matrix, in order to summarise the possible mechanisms by which the ciliary machinery could regulate the composition, secretion, remodelling and organisation of the matrix.
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spelling pubmed-70725292020-03-19 Regulation of the Extracellular Matrix by Ciliary Machinery I, Collins A.K.T, Wann Cells Review The primary cilium is an organelle involved in cellular signalling. Mutations affecting proteins involved in cilia assembly or function result in diseases known as ciliopathies, which cause a wide variety of phenotypes across multiple tissues. These mutations disrupt various cellular processes, including regulation of the extracellular matrix. The matrix is important for maintaining tissue homeostasis through influencing cell behaviour and providing structural support; therefore, the matrix changes observed in ciliopathies have been implicated in the pathogenesis of these diseases. Whilst many studies have associated the cilium with processes that regulate the matrix, exactly how these matrix changes arise is not well characterised. This review aims to bring together the direct and indirect evidence for ciliary regulation of matrix, in order to summarise the possible mechanisms by which the ciliary machinery could regulate the composition, secretion, remodelling and organisation of the matrix. MDPI 2020-01-23 /pmc/articles/PMC7072529/ /pubmed/31979260 http://dx.doi.org/10.3390/cells9020278 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
I, Collins
A.K.T, Wann
Regulation of the Extracellular Matrix by Ciliary Machinery
title Regulation of the Extracellular Matrix by Ciliary Machinery
title_full Regulation of the Extracellular Matrix by Ciliary Machinery
title_fullStr Regulation of the Extracellular Matrix by Ciliary Machinery
title_full_unstemmed Regulation of the Extracellular Matrix by Ciliary Machinery
title_short Regulation of the Extracellular Matrix by Ciliary Machinery
title_sort regulation of the extracellular matrix by ciliary machinery
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7072529/
https://www.ncbi.nlm.nih.gov/pubmed/31979260
http://dx.doi.org/10.3390/cells9020278
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