FUENTES BUSTOS, MARIA IGNACIA
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FUENTES BUSTOS, MARIA IGNACIA
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mifuentes@udd.cl
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57193308856

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Immune cell profiling of wounds from Epidermolysis bullosa patients

2019 , FUENTES BUSTOS, MARIA IGNACIA , C. Guttmann-Gruber , J. Piñon Hofbauer , A. South

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Publication

Sephardic Ancestry in Recessive Dystrophic Epidermolysis Bullosa Individuals Carrying the Prevalent c.6527insC Mutation

2022 , FUENTES BUSTOS, MARIA IGNACIA , Warshauer, E. M. , Roop, D. , Maier, P. , Runfeldt, G. , Gene Gene, Houston, TX USA

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Epithelial HMGB1 Delays Skin Wound Healing and Drives Tumor Initiation by Priming Neutrophils for NET Formation

2019 , Esther Hoste , Christian Maueröder , Lisette van Hove , Leen Catrysse , Hanna-Kaisa Vikkula , Mozes Sze , Bastiaan Maes , Dyah Karjosukarso , Liesbet Martens , Amanda Gonçalves , Eef Parthoens , Ria Roelandt , Wim Declercq , FUENTES BUSTOS, MARIA IGNACIA , Francis Palisson , Sergio Gonzalez , Julio C. Salas-Alanis , Louis Boon , Peter Huebener , Klaas Willem Mulder , Kodi Ravichandran , Yvan Saeys , Robert Felix Schwabe , Geert van Loo

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

2023 , DE GREGORIO CONCHA, CRISTIAN ALEJANDRO , Evelyng Catalán , Gabriel Garrido , Pilar Morandé , CASTILLO BENNETT, JIMENA VICTORIA , Catalina Muñoz , Glenda Cofré , HUANG, YA LIN , Bárbara Cuadra , Paola Murgas , Margarita Calvo , Fernando Altermatt , Andrew P. South , YUBERO GONCALVEZ, MARIA JOAO , PALISSON ETCHARREN, FRANCIS , EZQUER, EDUARDO MARCELO , FUENTES BUSTOS, MARIA IGNACIA

Abstract Background 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.

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Recessive dystrophic epidermolysis bullosa results in painful small fibre neuropathy

2017 , Sofia von Bischhoffshausen , Dinka Ivulic , Paola Alvarez , Victor C. Schuffeneger , Juan Idiaquez , Constanza Fuentes , Pilar Morande , Ignacia Fuentes , FRANCIS PALISSON ETCHARREN , David L. H. Bennett , Margarita Calvo

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Characterization of the epidermal-dermal junction in hiPSC-derived skin organoids

2022 , Veronika Ramovs , Hans Janssen , FUENTES BUSTOS, MARIA IGNACIA , Amandine Pitaval , Walid Rachidi , Susana M. Chuva de Sousa Lopes , Christian Freund , Xavier Gidrol , Christine L. Mummery , Karine Raymond

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Deciphering wound chronicity with a novel non-invasive sampling technology in Epidermolysis bullosa patients

2021 , FUENTES BUSTOS, MARIA IGNACIA , South, A.

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Ancestral patterns of recessive dystrophic epidermolysis bullosa mutations in Hispanic populations suggest sephardic ancestry

2021 , Emily Mira Warshauer , Adam Brown , FUENTES BUSTOS, MARIA IGNACIA , Jonathan Shortt , Chris Gignoux , Francesco Montinaro , Mait Metspalu , Leila Youssefian , Hassan Vahidnezhad , Joanna Jacków , Angela M. Christiano , Jouni Uitto , Óscar R. Fajardo‐Ramírez , Julio C. Salas‐Alanis , John A. McGrath , Liliana Consuegra , Carolina Rivera , Paul A. Maier , Goran Runfeldt , Doron M. Behar , Karl Skorecki , Eli Sprecher , PALISSON ETCHARREN, FRANCIS , David A. Norris , Anna L. Bruckner , Igor Kogut , Ganna Bilousova , Dennis R. Roop

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Collagen VII maintains proteostasis in dermal fibroblasts by scaffolding TANGO1 cargo

2022 , Qingqing Cao , Grace Tartaglia , Michael Alexander , Pyung Hung Park , Shiv Poojan , Mehdi Farshchian , FUENTES BUSTOS, MARIA IGNACIA , Mei Chen , John A. McGrath , PALISSON ETCHARREN, FRANCIS , Julio Salas-Alanis , Andrew P. South

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Longitudinal study of wound healing status and bacterial colonisation of Staphylococcus aureus and Corynebacterium diphtheriae in epidermolysis bullosa patients

2022 , FUENTES BUSTOS, MARIA IGNACIA , YUBERO GONCALVEZ, MARIA JOAO , Pilar Morandé , Carmen Varela , Karen Oróstica , Francisco Acevedo , REBOLLEDO JARAMILLO, BORIS EDUARDO , Esteban Arancibia , PORTE TORRE, LORENA ISABEL , PALISSON ETCHARREN, FRANCIS

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