Basement membrane collagens and disease mechanisms

Gatseva, A., Sin, Y. Y., Brezzo, G. and Van Agtmael, T. (2019) Basement membrane collagens and disease mechanisms. Essays in Biochemistry, 63(3), pp. 297-312. (doi: 10.1042/EBC20180071) (PMID:31387942) (PMCID:PMC6744580)

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Abstract

Basement membranes (BMs) are specialised extracellular matrix (ECM) structures and collagens are a key component required for BM function. While collagen IV is the major BM collagen, collagens VI, VII, XV, XVII and XVIII are also present. Mutations in these collagens cause rare multi-systemic diseases but these collagens have also been associated with major common diseases including stroke. Developing treatments for these conditions will require a collective effort to increase our fundamental understanding of the biology of these collagens and the mechanisms by which mutations therein cause disease. Novel insights into pathomolecular disease mechanisms and cellular responses to these mutations has been exploited to develop proof-of-concept treatment strategies in animal models. Combined, these studies have also highlighted the complexity of the disease mechanisms and the need to obtain a more complete understanding of these mechanisms. The identification of pathomolecular mechanisms of collagen mutations shared between different disorders represent an attractive prospect for treatments that may be effective across phenotypically distinct disorders.

Item Type:Articles
Status:Published
Refereed:Yes
Glasgow Author(s) Enlighten ID:Brezzo, Dr Gaia and Gatseva, Miss Anna and Sin, Dr Angie and Van Agtmael, Dr Tom
Authors: Gatseva, A., Sin, Y. Y., Brezzo, G., and Van Agtmael, T.
College/School:College of Medical Veterinary and Life Sciences > Institute of Cardiovascular and Medical Sciences
Journal Name:Essays in Biochemistry
Publisher:Portland Press
ISSN:0071-1365
ISSN (Online):1744-1358
Published Online:06 August 2019
Copyright Holders:Copyright © 2019 The Authors
First Published:First published in Essays in Biochemistry 63(3):297-312
Publisher Policy:Reproduced under a Creative Commons License

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Project CodeAward NoProject NamePrincipal InvestigatorFunder's NameFunder RefLead Dept
3021640Collagen IV variants and their role in intracerebral haemorrhage in the general populationTom Van AgtmaelMedical Research Council (MRC)MR/R005567/1CAMS - Cardiovascular Science
691851Targeting intracellular pathways to dissect mechanisms of cerebrovascular disease.Tom Van AgtmaelBritish Heart Foundation (BHF)PG/15/92/31813RI CARDIOVASCULAR & MEDICAL SCIENCES
3004780Elucidation of molecular pathways underlying cardiac disease caused by Col4a1 mutationsTom Van AgtmaelHeart Research UK (HEARTRES)RG 2664/17/20CAMS - Cardiovascular Science