Rescue of migratory defects of Ehlers-Danlos syndrome fibroblasts in vitro by type V collagen but not insulin-like binding protein-1
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Mutations in the genes encoding for type V collagen have been found in the classical type of Ehlers-Danlos syndrome (EDS); the most common mutations lead to a non-functional COL5A1 allele. We characterized three skin fibroblast strains derived from patients affected by classical EDS caused by COL5A1 haploinsufficiency. As a typical clinical hallmark of EDS is the impaired wound healing, we analyzed the repair capability of fibroblasts in a monolayer wounding assay. The mutant fibroblast strains were unable to move into the scraped area showing then a marked delay in wound repair. In all the EDS strains, type V collagen was absent in the extracellular space, also leading to the lack of fibronectin fibrillar network and impairing the expression of alpha(2)beta(1) and alpha(5)beta(1) integrins. The abnormal integrin pattern inhibited the positive effect of insulin-like growth factor-binding protein-1 on cell migration, whereas the migratory capability remarkably improved in the presence of exogenous type V collagen.
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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 [48]
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 [48]
High expression of IGFBP2 in cEDS cells might represent a transcriptional response to counteract, at least in vitro, their reduced migration capability.
“Its high expression in cEDS cells might represent a transcriptional response in the attempt to counteract, at least in vitro, their reduced migration capability [48,49].”
From Cellular and Molecular Mechanisms in the Pathogenesis of Classical, Vascular, and Hypermobile Ehlers‒Danlos Syndromes (2019) · cites this paper as [48]
Decreased expression of periostin encoded by POSTN in cEDS fibroblasts may contribute to the generalized ECM disarray and in vitro poor wound healing.
“In cEDS fibroblasts, the decreased expression of periostin encoded by POSTN may also contribute to the generalized ECM disarray and in vitro poor wound healing [44,48].”
From Cellular and Molecular Mechanisms in the Pathogenesis of Classical, Vascular, and Hypermobile Ehlers‒Danlos Syndromes (2019) · cites this paper as [48]
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 [48]
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- Authors
- Viglio, S., Zoppi, N., Sangalli, A., Gallanti, A., Barlati, S., Mottes, M., Colombi, M., & Valli, M.
- Affiliations
- University of Pavia (IT)
- University of Brescia (IT)
- University of Verona (IT)
- Corresponding author
- Valli, Maurizia
- Venue
- Journal of Investigative Dermatology
- Publisher
- Elsevier BV
- Published
- 2008-08
- Volume
- 128
- Issue
- 8
- Pages
- 1915-1919
- ISSN
- 0022-202X (ISSN-L)
- OpenAlex
- W2039306332
- Cited by
- 28
- References
- 23
- Citations by year
- 2025: 1 · 2024: 2 · 2023: 3 · 2022: 2 · 2021: 3 · 2020: 2 · 2019: 2 · 2017: 1 · 2016: 1 · 2015: 4 · 2014: 1 · 2012: 1
- FWCI
- 0.95
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Journal of Investigative Dermatology (publishedVersion)
- PubMed (publishedVersion)
- Institutional Research Information System (Università degli Studi di Brescia) (submittedVersion)
- INFM-OAR (INFN Catania) (submittedVersion, other-oa)
- Zenodo (CERN European Organization for Nuclear Research) (other-oa)
- Funders
- Università degli Studi di Brescia
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Health Sciences › Medicine › Rehabilitation › Wound Healing and Treatments
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Skin and Cellular Biology Research
- Keywords
- Ehlers-Danlos syndrome, type V collagen, fibroblast migration, wound healing, IGFBP-1, integrins, alpha2beta1 integrin, alpha5beta1 integrin, dermal fibroblasts
- MeSH terms
- Alleles, Cell Movement, Cells, Cultured, Child, Child, Preschool, Ehlers-Danlos Syndrome, Extracellular Matrix, Fibroblasts, Humans, Mutation, Wound Healing, Case-Control Studies, Insulin-Like Growth Factor Binding Protein 1, Collagen Type V, Integrin alpha2beta1, Integrin alpha5beta1
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