Multifaced Roles of the αvβ3 Integrin in Ehlers–Danlos and Arterial Tortuosity Syndromes’ Dermal Fibroblasts

Zoppi, Nicoletta; Chiarelli, Nicola; Ritelli, Marco; Colombi, Marina

doi:10.3390/ijms19040982

Cited by 37Connective tissue disorders researchCell Adhesion Molecules ResearchCellular Mechanics and Interactions

Multifaced Roles of the αvβ3 Integrin in Ehlers–Danlos and Arterial Tortuosity Syndromes’ Dermal Fibroblasts

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Zoppi, Nicoletta, Chiarelli, Nicola, Ritelli, Marco, Colombi, Marina·2018·International Journal of Molecular Sciences

DOI 10.3390/ijms19040982PMC PMC5979373

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Abstract

The αvβ3 integrin, an endothelial cells’ receptor-binding fibronectin (FN) in the extracellular matrix (ECM) of blood vessels, regulates ECM remodeling during migration, invasion, angiogenesis, wound healing and inflammation, and is also involved in the epithelial mesenchymal transition. In vitro-grown human control fibroblasts organize a fibrillar network of FN, which is preferentially bound on the entire cell surface to its canonical α5β1 integrin receptor, whereas the αvβ3 integrin is present only in rare patches in focal contacts. We report on the preferential recruitment of the αvβ3 integrin, due to the lack of FN–ECM and its canonical integrin receptor, in dermal fibroblasts from Ehlers–Danlos syndromes (EDS) and arterial tortuosity syndrome (ATS), which are rare multisystem connective tissue disorders. We review our previous findings that unraveled different biological mechanisms elicited by the αvβ3 integrin in fibroblasts derived from patients affected with classical (cEDS), vascular (vEDS), hypermobile EDS (hEDS), hypermobility spectrum disorders (HSD), and ATS. In cEDS and vEDS, respectively, due to defective type V and type III collagens, αvβ3 rescues patients’ fibroblasts from anoikis through a paxillin-p60Src-mediated cross-talk with the EGF receptor. In hEDS and HSD, without a defined molecular basis, the αvβ3 integrin transduces to the ILK-Snail1-axis inducing a fibroblast-to-myofibroblast-transition. In ATS cells, the deficiency of the dehydroascorbic acid transporter GLUT10 leads to redox imbalance, ECM disarray together with the activation of a non-canonical αvβ3 integrin-TGFBRII signaling, involving p125FAK/p60Src/p38MAPK. The characterization of these different biological functions triggered by αvβ3 provides insights into the multifaced nature of this integrin, at least in cultured dermal fibroblasts, offering future perspectives for research in this field.

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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 [50]
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 [50]
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 [50]
Matrix perturbation may act as a key driving factor for EDS pathogenesis irrespective of underlying molecular defects.
“This ECM signature is in common with cEDS and vEDS cells, underlining that the matrix perturbation may act as a key driving factor for the EDS pathogenesis, irrespective of the underlying molecular defects [50].”
From Cellular and Molecular Mechanisms in the Pathogenesis of Classical, Vascular, and Hypermobile Ehlers‒Danlos Syndromes (2019) · cites this paper as [50]
vEDS fibroblasts show differential expression of several genes encoding structural constituents of the ECM.
“In line with cEDS and hEDS transcriptome profiling, vEDS fibroblasts show the differential expression of several genes encoding structural constituents of the ECM, further supporting the notion that abnormal ECM remodeling is a common denominator of these conditions [50,97].”
From Cellular and Molecular Mechanisms in the Pathogenesis of Classical, Vascular, and Hypermobile Ehlers‒Danlos Syndromes (2019) · cites this paper as [50]

Aberrant expression of collagen V and defective remodeling of ECM in cEDS cells might affect ER homeostasis and autophagy, and consequently activate a pro-survival mechanism mediated by a crosstalk between αvβ3 integrin and EGF receptor.
“In line with these observations, the aberrant expression of collagen V and defective remodeling of ECM in cEDS cells might affect ER homeostasis and autophagy, and consequently activate a pro-survival mechanism mediated by a crosstalk between αvβ3 integrin and EGF receptor [45,50].”
From Cellular and Molecular Mechanisms in the Pathogenesis of Classical, Vascular, and Hypermobile Ehlers‒Danlos Syndromes (2019) · cites this paper as [50]
Abnormal ECM remodeling is a common denominator of cEDS, vEDS, and hEDS.
“In line with cEDS and hEDS transcriptome profiling, vEDS fibroblasts show the differential expression of several genes encoding structural constituents of the ECM, further supporting the notion that abnormal ECM remodeling is a common denominator of these conditions [50,97].”
From Cellular and Molecular Mechanisms in the Pathogenesis of Classical, Vascular, and Hypermobile Ehlers‒Danlos Syndromes (2019) · cites this paper as [50]
hEDS/HSD, cEDS, and vEDS fibroblasts exhibit marked disorganization of collagen and fibronectin ECM and their specific α2β1 and α5β1 integrin receptors and show preferential expression of the αvβ3 integrin.
“In particular, hEDS/HSD, cEDS, and vEDS fibroblasts exhibit a marked disorganization of collagen and fibronectin ECM and their specific α2β1 and α5β1 integrin receptors and showed the preferential expression of the αvβ3 integrin [44,49,50,123].”
From Cellular and Molecular Mechanisms in the Pathogenesis of Classical, Vascular, and Hypermobile Ehlers‒Danlos Syndromes (2019) · cites this paper as [50]
vEDS fibroblasts are in a pre-apoptotic state due to downregulation of the Bcl-2 anti-apoptotic protein and increased levels of caspase enzymes, and activate a cell survival mechanism through an αvβ3-EGFR crosstalk.
“Consistently, we previously demonstrated that vEDS fibroblasts are in a pre-apoptotic state, due to downregulation of the Bcl-2 anti-apoptotic protein and increased levels of caspase enzymes, and activate a cell survival mechanism through an αvβ3-EGFR crosstalk [45,50].”
From Cellular and Molecular Mechanisms in the Pathogenesis of Classical, Vascular, and Hypermobile Ehlers‒Danlos Syndromes (2019) · cites this paper as [50]
This ECM signature is in common with cEDS and vEDS cells.
“This ECM signature is in common with cEDS and vEDS cells, underlining that the matrix perturbation may act as a key driving factor for the EDS pathogenesis, irrespective of the underlying molecular defects [50].”
From Cellular and Molecular Mechanisms in the Pathogenesis of Classical, Vascular, and Hypermobile Ehlers‒Danlos Syndromes (2019) · cites this paper as [50]
This phenotypic switch is elicited by a signal transduction pathway involving αvβ3 integrin signaling through ILK and transcription factor Snail1.
“This phenotypic switch is elicited by a signal transduction pathway involving the αvβ3 integrin that signals through the integrin linked kinase (ILK) and the transcription factor Snail1 [49,50].”
From Cellular and Molecular Mechanisms in the Pathogenesis of Classical, Vascular, and Hypermobile Ehlers‒Danlos Syndromes (2019) · cites this paper as [50]
ILK acts downstream of phosphatidylinositol 3-kinase signaling and negatively regulates GSK3β by phosphorylation of a specific serine residue.
“The ILK acts downstream of the phosphatidylinositol 3-kinase signaling pathway and negatively regulates the action of the glycogen synthase kinase 3β by phosphorylation of a specific serine residue, further strengthening our assumption that Wnt/β-catenin signaling is involved in hEDS/HSD pathogenesis [49,50].”
From Cellular and Molecular Mechanisms in the Pathogenesis of Classical, Vascular, and Hypermobile Ehlers‒Danlos Syndromes (2019) · cites this paper as [50]

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GLUT10 deficiency leads to oxidative stress and non-canonical αvβ3 integrin-mediated TGFβ signalling associated with extracellular matrix disarray in arterial tortuosity syndrome skin fibroblasts
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“The exact pathomechanisms by which GLUT10 deficiency leads to the generalized ECM disarray, which reflects not only the typical ATS vascular anomalies but also the multisystem involvement overlapping with EDSs [171], were debated for long time and remain incompletely explored [98,99,105,115,116,172].”
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Dermal fibroblast-to-myofibroblast transition sustained by αvß3 integrin-ILK-Snail1/Slug signaling is a common feature for hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders
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Authors: Zoppi, N., Chiarelli, N., Ritelli, M., & Colombi, M.
Affiliations: University of Brescia (IT)
Corresponding author: Colombi, Marina
ORCID: Zoppi, Nicoletta
Chiarelli, Nicola
Ritelli, Marco
Colombi, Marina
Venue: International Journal of Molecular Sciences
Publisher: MDPI AG
Published: 2018-03-26
Volume: 19
Issue: 4
Pages: 982
ISSN: 1422-0067 (ISSN-L)
DOI: 10.3390/ijms19040982
PMC: PMC5979373
OpenAlex: W2791421919
Cited by: 37
References: 185
Citations by year: 2026: 4 · 2025: 2 · 2024: 7 · 2023: 6 · 2022: 2 · 2021: 5 · 2020: 3 · 2019: 6 · 2018: 1
FWCI: 0.53
Type: journal article
Language: en
Open access: gold
doi.org
Licence: cc-by
creativecommons.org/licenses/by/4.0/ (vor)
Locations: International Journal of Molecular Sciences (publishedVersion, cc-by)
Europe PMC (PubMed Central) (publishedVersion, other-oa)
PubMed Central (publishedVersion, cc-by)
PubMed (publishedVersion)
DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
Institutional Research Information System (Università degli Studi di Brescia) (submittedVersion, cc-by-nc-nd)
Funders: Ministero dell’Istruzione, dell’Università e della Ricerca
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 › Cellular Mechanics and Interactions
Keywords: integrin αvβ3, Ehlers-Danlos syndrome, arterial tortuosity syndrome, dermal fibroblasts, fibronectin, extracellular matrix remodeling, anoikis, p60src, EGF receptor, p125FAK, myofibroblast differentiation, GLUT10, dehydroascorbic acid, redox imbalance, hypermobility spectrum disorders, connective tissue diseases
MeSH terms: Arteries, Ehlers-Danlos Syndrome, Humans, Joint Instability, Skin Diseases, Genetic, Signal Transduction, Collagen Type III, Collagen Type V, Integrin alphaVbeta3, Vascular Malformations

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Multifaced Roles of the αvβ3 Integrin in Ehlers–Danlos and Arterial Tortuosity Syndromes’ Dermal Fibroblasts

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