Type III collagen affects dermal and vascular collagen fibrillogenesis and tissue integrity in a mutant Col3a1 transgenic mouse model
Abstract
Type III collagen is a major fibrillar collagen consisting of three identical α 1(III)-chains that is particularly present in tissues exhibiting elastic properties, such as the skin and the arterial wall. Heterozygous mutations in the COL3A1 gene result in vascular Ehlers-Danlos syndrome (vEDS), a severe, life-threatening disorder, characterized by thin, translucent skin and propensity to arterial, intestinal and uterine rupture. Most human vEDS cases result from a missense mutation substituting a crucial glycine residue in the triple helical domain of the α 1(III)-chains. The mechanisms by which these mutant type III collagen molecules cause dermal and vascular fragility are not well understood. We generated a transgenic mouse line expressing mutant type III collagen, containing a typical helical glycine substitution (p.(Gly182Ser)). This Col3a1 Tg-G182S mouse line displays a phenotype recapitulating characteristics of human vEDS patients with signs of dermal and vascular fragility. The Col3a1 Tg-G182S mice develop severe transdermal skin wounds, resulting in early demise at 13-14weeks of age. We found that this phenotype was associated with a reduced total collagen content and an abnormal collagen III:I ratio, leading to the production of severely malformed collagen fibrils in the extracellular matrix of dermal and arterial tissues. These results indicate that expression of the glycine substitution in the α 1(III)-chain disturbs formation of heterotypic type III:I collagen fibrils, and thereby demonstrate a key role for type III collagen in collagen fibrillogenesis in dermal and arterial tissues.
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In vEDS cells, enhanced expression of unfolded protein response-related genes was not identified.
“However, in vEDS cells an enhanced expression of unfolded protein response-related genes was not identified, consistent with the recent findings on cultured dermal fibroblasts from Col3a1 transgenic mice that did not show elevated levels of the unfolded protein response markers Bip and Chop [102].”
From Cellular and Molecular Mechanisms in the Pathogenesis of Classical, Vascular, and Hypermobile Ehlers‒Danlos Syndromes (2019) · cites this paper as [102]
Cultured dermal fibroblasts from Col3a1 transgenic mice did not show elevated levels of the unfolded protein response markers Bip and Chop.
“However, in vEDS cells an enhanced expression of unfolded protein response-related genes was not identified, consistent with the recent findings on cultured dermal fibroblasts from Col3a1 transgenic mice that did not show elevated levels of the unfolded protein response markers Bip and Chop [102].”
From Cellular and Molecular Mechanisms in the Pathogenesis of Classical, Vascular, and Hypermobile Ehlers‒Danlos Syndromes (2019) · cites this paper as [102]
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Pathogenesis of Abdominal Aortic Aneurysm
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- Authors
- D'hondt, S., Guillemyn, B., Syx, D., Symoens, S., De Rycke, R., Vanhoutte, L., Toussaint, W., Lambrecht, B. N., De Paepe, A., Keene, D. R., Ishikawa, Y., Bächinger, H. P., Janssens, S., Bertrand, M. J. M., & Malfait, F.
- Affiliations
- Ghent University Hospital (BE)
- VIB-UGent Center for Inflammation Research (BE)
- Shriners Hospitals for Children - Portland (US)
- Oregon Health & Science University (US)
- Corresponding author
- Malfait, Fransiska
- ORCID
- Venue
- Matrix Biology
- Publisher
- Elsevier BV
- Published
- 2018-09
- Volume
- 70
- Pages
- 72-83
- ISSN
- 0945-053X (ISSN-L)
- OpenAlex
- W2793754317
- Cited by
- 79
- References
- 33
- Citations by year
- 2026: 9 · 2025: 11 · 2024: 8 · 2023: 8 · 2022: 9 · 2021: 11 · 2020: 12 · 2019: 8 · 2018: 3
- FWCI
- 5.21
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Matrix Biology (publishedVersion)
- PubMed (publishedVersion)
- Ghent University Academic Bibliography (Ghent University) (submittedVersion)
- Funders
- Ghent University (BOFMET2015000401)
- Flanders Research Foundation (G17212N, G013715N)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Physical Sciences › Materials Science › Biomaterials › Collagen: Extraction and Characterization
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Dermatological and Skeletal Disorders
- Keywords
- type III collagen, COL3A1, vascular Ehlers-Danlos syndrome, collagen fibrillogenesis, transgenic mouse model, extracellular matrix, collagen fibril morphology, skin wound healing, arterial rupture
- MeSH terms
- Animals, Arteries, Disease Models, Animal, Ehlers-Danlos Syndrome, Female, Fibroblasts, Glycine, Heterozygote, Humans, Male, Mice, Transgenic, Mutation, Serine, Sex Factors, Skin, Gene Expression, Amino Acid Substitution, Collagen Type III, Tissue Culture Techniques, Mice
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