FUENTES BUSTOS, MARIA IGNACIA

Preferred name

Main Affiliation

Facultad de Medicina Clínica Alemana Universidad del Desarrollo

mifuentes@udd.cl

ORCID

0000-0002-7382-1956

Scopus Author ID

57193308856

Now showing 1 - 10 of 45

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Dataset - Antiviral drugs prolong survival in murine recessive dystrophic epidermolysis bullosa
(2024)
FUENTES BUSTOS, MARIA IGNACIA
1
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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
26Scopus© Citations 46
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Cells from discarded dressings differentiate chronic from acute wounds in patients with Epidermolysis Bullosa
(2020)
FUENTES BUSTOS, MARIA IGNACIA
;
Christina Guttmann-Gruber
;
Birgit Tockner
;
Anja Diem
;
Alfred Klausegger
<jats:title>Abstract</jats:title><jats:p>Impaired wound healing complicates a wide range of diseases and represents a major cost to healthcare systems. Here we describe the use of discarded wound dressings as a novel, cost effective, accessible, and non-invasive method of isolating viable human cells present at the site of skin wounds. By analyzing 133 discarded wound dressings from 51 patients with the inherited skin-blistering disease epidermolysis bullosa (EB), we show that large numbers of cells, often in excess of 100 million per day, continually infiltrate wound dressings. We show, that the method is able to differentiate chronic from acute wounds, identifying significant increases in granulocytes in chronic wounds, and we show that patients with the junctional form of EB have significantly more cells infiltrating their wounds compared with patients with recessive dystrophic EB. Finally, we identify subsets of granulocytes and T lymphocytes present in all wounds paving the way for single cell profiling of innate and adaptive immune cells with relevance to wound pathologies. In summary, our study delineates findings in EB that have potential relevance for all chronic wounds, and presents a method of cellular isolation that has wide reaching clinical application.</jats:p>
22Scopus© Citations 19
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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
19Scopus© Citations 7
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Patients suffering from dystrophic epidermolysis bullosa are prone to developing autoantibodies against skin proteins: A longitudinal confirmational study
(2024)
J. Bremer
;
H. H. Pas
;
G. F. H. Diercks
;
H. J. Meijer
;
S. M. van der Molen
<jats:title>Abstract</jats:title><jats:p>Epidermolysis bullosa (EB) is a heritable skin blistering disease caused by variants in genes coding for proteins that secure cell–cell adhesion and attachment of the epidermis to the dermis. Interestingly, several proteins involved in inherited EB are also associated with autoimmune blistering diseases (AIBD). In this study, we present a long‐term follow‐up of 15 patients suffering from recessive dystrophic or junctional EB. From these patients, 62 sera were analysed for the presence of autoantibodies associated with AIBD. We show that patients suffering from recessive dystrophic EB (RDEB) are more susceptible to developing autoantibodies against skin proteins than patients suffering from junctional EB (70% vs. 20%, respectively). Interestingly, no correlation with age was observed. Most patients showed reactivity to Type XVII collagen/linear IgA bullous dermatosis autoantigen (<jats:italic>n</jats:italic> = 5; 33%), followed by BP230 (<jats:italic>n</jats:italic> = 4; 27%), Type VII collagen (<jats:italic>n</jats:italic> = 4; 27%) and laminin‐332 (<jats:italic>n</jats:italic> = 1; 7%). The pathogenicity of these autoantibodies remains a subject for future experiments.</jats:p>
Scopus© Citations 3 1
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Antiviral drugs prolong survival in murine recessive dystrophic epidermolysis bullosa
(2024)
Grace Tartaglia
;
FUENTES BUSTOS, MARIA IGNACIA
;
Neil Patel
;
Abigail Varughese
;
Lauren E Israel
<jats:title>Abstract</jats:title><jats:p>Recessive dystrophic epidermolysis bullosa (RDEB) is a rare inherited skin disease characterized by defects in type VII collagen leading to a range of fibrotic pathologies resulting from skin fragility, aberrant wound healing, and altered dermal fibroblast physiology. Using a novel in vitro model of fibrosis based on endogenously produced extracellular matrix, we screened an FDA-approved compound library and identified antivirals as a class of drug not previously associated with anti-fibrotic action. Preclinical validation of our lead hit, daclatasvir, in a mouse model of RDEB demonstrated significant improvement in fibrosis as well as overall quality of life with increased survival, weight gain and activity, and a decrease in pruritus-induced hair loss. Immunohistochemical assessment of daclatasvir-treated RDEB mouse skin showed a reduction in fibrotic markers, which was supported by in vitro data demonstrating TGFβ pathway targeting and a reduction of total collagen retained in the extracellular matrix. Our data support the clinical development of antivirals for the treatment of patients with RDEB and potentially other fibrotic diseases.</jats:p>
Scopus© Citations 1 4
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Fibroblasts isolated from chronic wound dressings differentiate chronicity in recessive dystrophic epidermolysis bullosa
(2023)
DE GREGORIO CONCHA, CRISTIAN ALEJANDRO
;
EZQUER, EDUARDO MARCELO
;
F. Palisson
;
E. Catalán
;
A.P. South
5
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Epidermolysis bullosa simplex-generalized severe type due to keratin 5 p.Glu477Lys mutation: Genotype-phenotype correlation and in silico modeling analysis
(2019)
Leah Lalor
;
Matthias Titeux
;
FRANCIS PALISSON ETCHARREn
;
Ignacia Fuentes
;
María J. Yubero
Scopus© Citations 13 1
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IMMUNE CELL PROFILING OF WOUNDS FROM EPIDERMOLYSIS BULLOSA PATIENTS
(2020)
YUBERO GONCALVEZ, MARIA JOAO
;
FUENTES BUSTOS, MARIA IGNACIA
;
PALISSON ETCHARREN, FRANCIS
;
REBOLLEDO JARAMILLO, BORIS EDUARDO
;
Guttmann-Gruber, Christina
1
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Longitudinal study of wound healing status and bacterial colonisation of
<i>Staphylococcus aureus</i>
and
<i>Corynebacterium diphtheriae</i>
in epidermolysis bullosa patients
(2022)
FUENTES BUSTOS, MARIA IGNACIA
;
YUBERO GONCALVEZ, MARIA JOAO
;
Pilar Morandé
;
Carmen Varela
;
Karen Oróstica
Scopus© Citations 11 2

FUENTES BUSTOS, MARIA IGNACIA

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