Type III and V collagens modulate the expression and assembly of EDA+fibronectin in the extracellular matrix of defective Ehlers-Danlos syndrome fibroblasts
Abstract
Background Alternative splicing of EDA fibronectin (FN) region is a cell type- and development-regulated mechanism controlled by pathological processes, growth factors and extracellular matrix (ECM). Classic and vascular Ehlers-Danlos syndrome (cEDS and vEDS) are connective tissue disorders caused by COL5A1/COL5A2 and COL3A1 gene mutations, leading to an in vivo abnormal collagen fibrillogenesis and to an in vitro defective organisation in the ECM of type V (COLLV) and type III collagen (COLLIII). These defects induce the FN-ECM disarray and the decrease of COLLs and FN receptors, the α2β1 and α5β1 integrins. Purified COLLV and COLLIII restore the COLL-FN-ECMs in both EDS cell strains.Methods Real-time PCR, immunofluorescence microscopy, and Western blotting were used to investigate the effects of COLLs on FN1 gene expression, EDA region alternative splicing, EDA(+)-FN-ECM assembly, α5β1 integrin and EDA(+)-FN-specific α9 integrin subunit organisation, α5β1 integrin and FAK co-regulation in EDS fibroblasts.Results COLLV-treated cEDS and COLLIII-treated vEDS fibroblasts up-regulate the FN1 gene expression, modulate the EDA(+) mRNA maturation and increase the EDA(+)-FN levels, thus restoring a control-like FN-ECM, which elicits the EDA(+)-FN-specific α9β1 integrin organisation, recruits the α5β1 integrin and switches on the FAK binding and phosphorylation.Conclusion COLLs regulate the EDA(+)-FN-ECM organisation at transcriptional and post-transcriptional level and activate the α5β1-FAK complexes. COLLs also recruit the α9β1 integrin involved in the assembly of the EDA(+)-FN-ECM in EDS cells.General significance The knowledge of the COLLs-ECM role in FN isotype expression and in EDA(+)-FN-ECM-mediated signal transduction adds insights in the ECM remodelling mechanisms in EDS cells.
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cEDS cells exhibit reduced in vitro migration capability.
“cEDS cells also exhibit a reduced in vitro migration capability, an abnormal wound healing response, and a crosstalk involving the αvβ3 integrin and epidermal growth factor (EGF) receptor that rescues them from anoikis [44,45,46,47,48,49,50].”
From Cellular and Molecular Mechanisms in the Pathogenesis of Classical, Vascular, and Hypermobile Ehlers‒Danlos Syndromes (2019) · cites this paper as [46]
cEDS patients' fibroblasts show disassembly of many ECM components, including collagen V and III, fibronectin, and fibrillins.
“Our in vitro findings demonstrated that cEDS patients’ fibroblasts show disassembly of many ECM components, including collagen V and III, fibronectin, and fibrillins, and disorganization of collagen- and fibronectin-specific α2β1 and α5β1 integrin receptors [44,45,46,47].”
From Cellular and Molecular Mechanisms in the Pathogenesis of Classical, Vascular, and Hypermobile Ehlers‒Danlos Syndromes (2019) · cites this paper as [46]
cEDS patients' fibroblasts show disorganization of collagen- and fibronectin-specific α2β1 and α5β1 integrin receptors.
“Our in vitro findings demonstrated that cEDS patients’ fibroblasts show disassembly of many ECM components, including collagen V and III, fibronectin, and fibrillins, and disorganization of collagen- and fibronectin-specific α2β1 and α5β1 integrin receptors [44,45,46,47].”
From Cellular and Molecular Mechanisms in the Pathogenesis of Classical, Vascular, and Hypermobile Ehlers‒Danlos Syndromes (2019) · cites this paper as [46]
cEDS cells exhibit an abnormal wound healing response.
“cEDS cells also exhibit a reduced in vitro migration capability, an abnormal wound healing response, and a crosstalk involving the αvβ3 integrin and epidermal growth factor (EGF) receptor that rescues them from anoikis [44,45,46,47,48,49,50].”
From Cellular and Molecular Mechanisms in the Pathogenesis of Classical, Vascular, and Hypermobile Ehlers‒Danlos Syndromes (2019) · cites this paper as [46]
cEDS cells exhibit a crosstalk involving the αvβ3 integrin and EGF receptor that rescues them from anoikis.
“cEDS cells also exhibit a reduced in vitro migration capability, an abnormal wound healing response, and a crosstalk involving the αvβ3 integrin and epidermal growth factor (EGF) receptor that rescues them from anoikis [44,45,46,47,48,49,50].”
From Cellular and Molecular Mechanisms in the Pathogenesis of Classical, Vascular, and Hypermobile Ehlers‒Danlos Syndromes (2019) · cites this paper as [46]
In the present study, the abnormal folding, secretion, and ECM organization of COLLIII cause an altered deposition and disassembly of other structural constituents, such as FN, FBNs, ELN, and EMILINs.
“In the present study, we demonstrated that the abnormal folding, secretion, and ECM organization of COLLIII cause an altered deposition and the disassembly of other structural constituents, such as FN, FBNs, ELN, and EMILINs [16,25], which provide a scaffold for elastogenesis and represent the major components of elastic fibers in elastic tissues including skin and arterial walls, and are essent”
Enhanced storages of FN and ELN in cytoplasmic compartments of vEDS fibroblasts were described.
“Indeed, enhanced storages of FN [25] and ELN (this work) in cytoplasmic compartments of vEDS fibroblasts were described, according to the notion that structural mutations in genes encoding for different COLLs, i.e., COLLI, COLLIII, and COLLV, affect not only the assembly but also the secretion pathways of other ECM components in EDS fibroblasts [16,17,33].”
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- Authors
- Zoppi, N., Ritelli, M., & Colombi, M.
- Affiliations
- University of Brescia (IT)
- Corresponding author
- Zoppi, Nicoletta
- Venue
- Biochimica et Biophysica Acta (BBA) - General Subjects
- Publisher
- Elsevier BV
- Published
- 2012-10
- Volume
- 1820
- Issue
- 10
- Pages
- 1576-1587
- ISSN
- 0304-4165 (ISSN-L)
- OpenAlex
- W1967974177
- Cited by
- 21
- References
- 57
- Citations by year
- 2025: 2 · 2023: 1 · 2019: 3 · 2018: 4 · 2016: 1 · 2015: 4 · 2014: 1 · 2013: 5
- FWCI
- 1.92
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Biochimica et Biophysica Acta (BBA) - General Subjects (publishedVersion)
- PubMed (publishedVersion)
- Institutional Research Information System (Università degli Studi di Brescia) (submittedVersion)
- Funders
- Ministero dell'Istruzione, dell'Università e della Ricerca, Fondo per gli Investimenti della Ricerca di Base 2009–2011
- Regione Lombardia (Network-Enabled Drug Design) 2011
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Health Sciences › Medicine › Immunology and Allergy › Cell Adhesion Molecules Research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Skin and Cellular Biology Research
- Keywords
- Ehlers-Danlos syndrome, type V collagen, type III collagen, ED-A fibronectin, extracellular matrix assembly, fibroblasts, integrin α9β1, alpha5beta1 integrin, focal adhesion kinase, collagen fibrillogenesis, connective tissue diseases, extracellular matrix remodeling
- MeSH terms
- Cells, Cultured, Ehlers-Danlos Syndrome, Extracellular Matrix, Female, Fibroblasts, Fibronectins, Gene Expression Regulation, Humans, Male, Case-Control Studies, Integrins, Protein Isoforms, Collagen Type III, Collagen Type V, Integrin alpha5beta1, Focal Adhesion Kinase 1, Protein Multimerization
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
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