Collagen IV-related diseases and therapies

Dean, A. and Van Agtmael, T. (2021) Collagen IV-related diseases and therapies. In: Ruggiero, F. (ed.) The Collagen Superfamily and Collagenopathies. Series: Biology of extracellular matrix (8). Springer: Cham, pp. 143-197. ISBN 9783030675912 (doi: 10.1007/978-3-030-67592-9_5)

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Abstract

While traditionally the function of the extracellular matrix and the basement membrane was considered to be providing structural support, it is now clear that this only covers one aspect of its multiple functions. This is also illustrated by our growing knowledge of the role of collagen IV, a major component of basement membranes, in development, health, and disease. With the extracellular matrix and collagen IV increasingly being recognised as key players in a growing number of diseases from stroke and vascular defects to kidney disease, deafness, and eye abnormalities, it is paramount that we increase our fundamental understanding of these complex molecules ranging from their biosynthesis to their role in human disease. Recently, exciting progress has been made in delineating the mechanisms by which mutations in collagen IV cause disease, and these are being exploited to develop mechanism-based treatments. Yet many important questions remain that need addressing to develop treatments for diseases associated with collagen IV.

Item Type:Book Sections
Status:Published
Glasgow Author(s) Enlighten ID:Dean, Dr Afshan and Van Agtmael, Professor Tom
Authors: Dean, A., and Van Agtmael, T.
College/School:College of Medical Veterinary and Life Sciences > School of Cardiovascular & Metabolic Health
Publisher:Springer
ISBN:9783030675912
Published Online:16 May 2021
Copyright Holders:Copyright © 2021 The Authors
First Published:First published in The Collagen Superfamily and Collagenopathies: 143-197
Publisher Policy:Reproduced in accordance with the publisher copyright policy

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Project CodeAward NoProject NamePrincipal InvestigatorFunder's NameFunder RefLead Dept
302164Collagen IV variants and their role in intracerebral haemorrhage in the general populationTom Van AgtmaelMedical Research Council (MRC)MR/R005567/1CAMS - Cardiovascular Science
172813Targeting intracellular pathways to dissect mechanisms of cerebrovascular disease.Tom Van AgtmaelBritish Heart Foundation (BHF)PG/15/92/31813Institute of Cardiovascular & Medical Sciences
300478Elucidation of molecular pathways underlying cardiac disease caused by Col4a1 mutationsTom Van AgtmaelHeart Research UK (HEARTRES)RG 2664/17/20CAMS - Cardiovascular Science