Maintenance of chronicity signatures in fibroblasts isolated from recessive dystrophic epidermolysis bullosa chronic wound dressings under culture conditions

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dc.article.number23
dc.catalogadorcrc
dc.contributor.authorDe Gregorio, Cristian
dc.contributor.authorCatalán, Evelyng
dc.contributor.authorGarrido, Gabriel
dc.contributor.authorMorandé, Pilar
dc.contributor.authorBennett, Jimena C.
dc.contributor.authorMuñoz, Catalina
dc.contributor.authorCofré, Glenda
dc.contributor.authorHuang, Ya-Lin
dc.contributor.authorCuadra, Bárbara
dc.contributor.authorMurgas, Paola
dc.contributor.authorCalvo Bascuñan, Margarita
dc.contributor.authorAltermatt, Fernando
dc.contributor.authorYubero, María J.
dc.contributor.authorPalisson, Francis
dc.contributor.authorSouth, Andrew P.
dc.contributor.authorEzquer, Marcelo
dc.contributor.authorFuentes, Ignacia
dc.date.accessioned2023-05-17T17:14:44Z
dc.date.available2023-05-17T17:14:44Z
dc.date.issued2023
dc.date.updated2023-05-14T00:04:41Z
dc.description.abstractBackground Recessive Dystrophic Epidermolysis Bullosa (RDEB) is a rare inherited skin disease caused by variants in the COL7A1 gene, coding for type VII collagen (C7), an important component of anchoring fibrils in the basement membrane of the epidermis. RDEB patients suffer from skin fragility starting with blister formation and evolving into chronic wounds, inflammation and skin fibrosis, with a high risk of developing aggressive skin carcinomas. Restricted therapeutic options are limited by the lack of in vitro models of defective wound healing in RDEB patients. Results In order to explore a more efficient, non-invasive in vitro model for RDEB studies, we obtained patient fibroblasts derived from discarded dressings) and examined their phenotypic features compared with fibroblasts derived from non-injured skin of RDEB and healthy-donor skin biopsies. Our results demonstrate that fibroblasts derived from RDEB chronic wounds (RDEB-CW) displayed characteristics of senescent cells, increased myofibroblast differentiation, and augmented levels of TGF-β1 signaling components compared to fibroblasts derived from RDEB acute wounds and unaffected RDEB skin as well as skin from healthy-donors. Furthermore, RDEB-CW fibroblasts exhibited an increased pattern of inflammatory cytokine secretion (IL-1β and IL-6) when compared with RDEB and control fibroblasts. Interestingly, these aberrant patterns were found specifically in RDEB-CW fibroblasts independent of the culturing method, since fibroblasts obtained from dressing of acute wounds displayed a phenotype more similar to fibroblasts obtained from RDEB normal skin biopsies. Conclusions Our results show that in vitro cultured RDEB-CW fibroblasts maintain distinctive cellular and molecular characteristics resembling the inflammatory and fibrotic microenvironment observed in RDEB patients’ chronic wounds. This work describes a novel, non-invasive and painless strategy to obtain human fibroblasts chronically subjected to an inflammatory and fibrotic environment, supporting their use as an accessible model for in vitro studies of RDEB wound healing pathogenesis. As such, this approach is well suited to testing new therapeutic strategies under controlled laboratory conditions.
dc.fechaingreso.objetodigital2023-05-14
dc.fuente.origenAutoarchivo
dc.identifier.citationBiological Research. 2023 May 10;56(1):23
dc.identifier.doi10.1186/s40659-023-00437-2
dc.identifier.issn0717-6287
dc.identifier.urihttps://doi.org/10.1186/s40659-023-00437-2
dc.identifier.urihttps://repositorio.uc.cl/handle/11534/67008
dc.information.autorucEscuela de medicina; Calvo Bascuñán, Margarita ; 0000-0003-3349-9189 ; 3457
dc.information.autorucEscuela de medicina; Altermatt, Fernando; s/i; 7381
dc.information.autorucFacultad de Ciencias Biológicas; Fuentes, Ignacia; s/i; 127312
dc.issue.numero1
dc.language.isoen
dc.nota.accesoContenido completo
dc.pagina.final16
dc.pagina.inicio1
dc.revistaBiological Research
dc.rightsacceso abierto
dc.rights.holderThe Author(s)
dc.rights.licenseAttribution 4.0 International (CC BY 4.0)
dc.rights.urihttps://creativecommons.org/licenses/by/4.0/
dc.subjectRecessive Dystrophic Epidermolysis Bullosa
dc.subjectSkin fibroblast
dc.subjectChronic wounds
dc.subjectWound dressing
dc.subjectFibrosis
dc.subject.ddc610
dc.subject.deweyMedicina y saludes_ES
dc.titleMaintenance of chronicity signatures in fibroblasts isolated from recessive dystrophic epidermolysis bullosa chronic wound dressings under culture conditions
dc.typeartículo
dc.volumen56
sipa.codpersvinculados3457
sipa.codpersvinculados7381
sipa.codpersvinculados127312
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