Journal article : Review
Collagen VI as a driver and disease biomarker in human fibrosis
- Abstract:
- Fibrosis of visceral organs such as the lungs, heart, kidneys and liver remains a major cause of morbidity and mortality and is also associated with many other disorders, including cancer and metabolic disease. In this review, we focus upon the microfibrillar collagen VI, which is present in the extracellular matrix (ECM) of most tissues. However, expression is elevated in numerous fibrotic conditions, such as idiopathic pulmonary disease (IPF), and chronic liver and kidney diseases. Collagen VI is composed of three subunits α1, α2 and α3, which can be replaced with alternate chains of α4, α5 or α6. The C-terminal globular domain (C5) of collagen VI α3 can be proteolytically cleaved to form a biologically active fragment termed endotrophin, which has been shown to actively drive fibrosis, inflammation and insulin resistance. Tissue biopsies have long been considered the gold standard for diagnosis and monitoring of progression of fibrotic disease. The identification of neoantigens from enzymatically processed collagen chains have revolutionised the biomarker field, allowing rapid diagnosis and evaluation of prognosis of numerous fibrotic conditions, as well as providing valuable clinical trial endpoint determinants. Collagen VI chain fragments such as endotrophin (PRO-C6), C6M and C6Mα3 are emerging as important biomarkers for fibrotic conditions.
- Publication status:
- Published
- Peer review status:
- Peer reviewed
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(Preview, Version of record, pdf, 2.2MB, Terms of use)
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- Publisher copy:
- 10.1111/febs.16039
Authors
- Publisher:
- FEBS Press
- Journal:
- The FEBS Journal More from this journal
- Volume:
- 289
- Issue:
- 13
- Pages:
- 3603-3629
- Place of publication:
- England
- Publication date:
- 2021-06-14
- Acceptance date:
- 2021-05-27
- DOI:
- EISSN:
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1742-4658
- ISSN:
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1742-464X
- Pmid:
-
34109754
- Language:
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English
- Keywords:
- Subtype:
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Review
- Pubs id:
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1182647
- Local pid:
-
pubs:1182647
- Deposit date:
-
2022-03-16
Terms of use
- Copyright holder:
- Williams et al.
- Copyright date:
- 2021
- Rights statement:
- ©2021 The Authors. The FEBS Journal published by John Wiley & Sons Ltd on behalf of Federation of European Biochemical Societies. This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
- Licence:
- CC Attribution (CC BY)
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