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: | Gatseva, Dr Anna and Sin, Dr Angie and Brezzo, Dr Gaia and Van Agtmael, Professor Tom |
| Authors: | Gatseva, A., Sin, Y. Y., Brezzo, G., and Van Agtmael, T. |
| College/School: | College of Medical Veterinary and Life Sciences > School of Cardiovascular & Metabolic Health |
| 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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