Search Results - "Larcher, Fernando"

COL7A1 Editing via CRISPR/Cas9 in Recessive Dystrophic Epidermolysis Bullosa by Hainzl, Stefan, Peking, Patricia, Kocher, Thomas, Murauer, Eva M., Larcher, Fernando, Del Rio, Marcela, Duarte, Blanca, Steiner, Markus, Klausegger, Alfred, Bauer, Johann W., Reichelt, Julia, Koller, Ulrich

“…Designer nucleases allow specific and precise genomic modifications and represent versatile molecular tools for the correction of disease-associated mutations…”
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Human embryonic stem-cell derivatives for full reconstruction of the pluristratified epidermis: a preclinical study by Guenou, Hind, PhD, Nissan, Xavier, Larcher, Fernando, PhD, Feteira, Jessica, Lemaitre, Gilles, PhD, Saidani, Manoubia, Del Rio, Marcela, PhD, Barrault, Christine C, Bernard, François-Xavier, PhD, Peschanski, Marc, MD, Baldeschi, Christine, Dr, Waksman, Gilles, Prof

“…Summary Background Cell therapy for large burns is dependent upon autologous epidermis reconstructed in vitro. However, the effectiveness of current procedures…”
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In vitro and In vivo Wound Healing-Promoting Activities of Human Cathelicidin LL-37 by Carretero, Marta, Escámez, María J., García, Marta, Duarte, Blanca, Holguín, Almudena, Retamosa, Luisa, Jorcano, Jose L., Río, Marcela del, Larcher, Fernando

“…The human antimicrobial peptide LL-37 plays an important role in host defense against infection. In addition to its antimicrobial action, other activities have…”
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Induction of Scleroderma Fibrosis in Skin‐Humanized Mice by Administration of Anti−Platelet‐Derived Growth Factor Receptor Agonistic Autoantibodies by Luchetti, Michele M., Moroncini, Gianluca, Jose Escamez, Maria, Svegliati Baroni, Silvia, Spadoni, Tatiana, Grieco, Antonella, Paolini, Chiara, Funaro, Ada, Avvedimento, Enrico V., Larcher, Fernando, Del Rio, Marcela, Gabrielli, Armando

“…Objective To describe a skin–SCID mouse chimeric model of systemic sclerosis (SSc; scleroderma) fibrosis based on engraftment of ex vivo–bioengineered skin…”
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Topical Enzyme-Replacement Therapy Restores Transglutaminase 1 Activity and Corrects Architecture of Transglutaminase-1-Deficient Skin Grafts by Aufenvenne, Karin, Larcher, Fernando, Hausser, Ingrid, Duarte, Blanca, Oji, Vinzenz, Nikolenko, Heike, Del Rio, Marcela, Dathe, Margitta, Traupe, Heiko

“…Transglutaminase-1 (TG1)-deficient autosomal-recessive congenital ichthyosis (ARCI) is a rare and severe genetic skin disease caused by mutations in TGM1. It…”
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A non-viral gene therapy for treatment of recessive dystrophic epidermolysis bullosa by Cutlar, Lara, Zhou, Dezhong, Hu, Xuejun, Duarte, Blanca, Greiser, Udo, Larcher, Fernando, Wang, Wenxin

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Differential Features between Chronic Skin Inflammatory Diseases Revealed in Skin-Humanized Psoriasis and Atopic Dermatitis Mouse Models by Carretero, Marta, Guerrero-Aspizua, Sara, Illera, Nuria, Galvez, Victoria, Navarro, Manuel, García-García, Francisco, Dopazo, Joaquin, Jorcano, Jose Luis, Larcher, Fernando, del Rio, Marcela

“…Psoriasis and atopic dermatitis are chronic and relapsing inflammatory diseases of the skin affecting a large number of patients worldwide. Psoriasis is…”
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Correction of Recessive Dystrophic Epidermolysis Bullosa by Transposon-Mediated Integration of COL7A1 in Transplantable Patient-Derived Primary Keratinocytes by Latella, Maria Carmela, Cocchiarella, Fabienne, De Rosa, Laura, Turchiano, Giandomenico, Gonçalves, Manuel A.F.V., Larcher, Fernando, De Luca, Michele, Recchia, Alessandra

“…Recessive dystrophic epidermolysis bullosa (RDEB) is caused by defects in type-VII collagen (C7), a protein encoded by the COL7A1 gene and essential for…”
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Identification of novel small molecule-based strategies of COL7A1 upregulation and readthrough activity for the treatment of recessive dystrophic epidermolysis bullosa by Jover, Irene, Ramos, Maria C., Escámez, María José, Lozoya, Estrella, Tormo, José R., de Prado-Verdún, Diana, Mencía, Ángeles, Pont, Mercè, Puig, Carles, Larraufie, Marie-Helene, Gutiérrez-Caballero, Cristina, Reyes, Fernando, Trincado, Juan Luis, García-González, Vicente, Cerrato, Rosario, Andrés, Miriam, Crespo, Maribel, Vicente, Francisca, Godessart, Nuria, Genilloud, Olga, Larcher, Fernando, Nueda, Arsenio

“…Recessive dystrophic epidermolysis bullosa (RDEB) is a rare genetic disease caused by loss of function mutations in the gene coding for collagen VII (C7) due…”
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Deletion of a Pathogenic Mutation-Containing Exon of COL7A1 Allows Clonal Gene Editing Correction of RDEB Patient Epidermal Stem Cells by Mencía, Ángeles, Chamorro, Cristina, Bonafont, Jose, Duarte, Blanca, Holguin, Almudena, Illera, Nuria, Llames, Sara G., Escámez, Maria José, Hausser, Ingrid, Del Río, Marcela, Larcher, Fernando, Murillas, Rodolfo

“…Recessive dystrophic epidermolysis bullosa is a severe skin fragility disease caused by loss of functional type VII collagen at the dermal-epidermal junction…”
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Humanization of Tumor Stroma by Tissue Engineering as a Tool to Improve Squamous Cell Carcinoma Xenograft by Guerrero-Aspizua, Sara, González-Masa, Andrea, Conti, Claudio J, García, Marta, Chacón-Solano, Esteban, Larcher, Fernando, Del Río, Marcela

“…The role of stroma is fundamental in the development and behavior of epithelial tumors. In this regard, limited growth of squamous cell carcinomas (SCC) or…”
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Preclinical model for phenotypic correction of dystrophic epidermolysis bullosa by in vivo CRISPR-Cas9 delivery using adenoviral vectors by García, Marta, Bonafont, Jose, Martínez-Palacios, Jesús, Xu, Rudan, Turchiano, Giandomenico, Svensson, Stina, Thrasher, Adrian J., Larcher, Fernando, Del Rio, Marcela, Hernández-Alcoceba, Rubén, Garín, Marina I., Mencía, Ángeles, Murillas, Rodolfo

“…Recessive dystrophic epidermolysis bullosa, a devastating skin fragility disease characterized by recurrent skin blistering, scarring, and a high risk of…”
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Targeted Gene Addition in Human Epithelial Stem Cells by Zinc-finger Nuclease-mediated Homologous Recombination by Coluccio, Andrea, Miselli, Francesca, Lombardo, Angelo, Marconi, Alessandra, Malagoli Tagliazucchi, Guidantonio, Gonçalves, Manuel A, Pincelli, Carlo, Maruggi, Giulietta, Del Rio, Marcela, Naldini, Luigi, Larcher, Fernando, Mavilio, Fulvio, Recchia, Alessandra

“…Preclinical and clinical studies showed that autologous transplantation of epidermis derived from genetically modified epithelial stem cells (EpSCs) leads to…”
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Gene Editing for the Efficient Correction of a Recurrent COL7A1 Mutation in Recessive Dystrophic Epidermolysis Bullosa Keratinocytes by Chamorro, Cristina, Mencía, Angeles, Almarza, David, Duarte, Blanca, Büning, Hildegard, Sallach, Jessica, Hausser, Ingrid, Del Río, Marcela, Larcher, Fernando, Murillas, Rodolfo

“…Clonal gene therapy protocols based on the precise manipulation of epidermal stem cells require highly efficient gene-editing molecular tools. We have combined…”
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Early, Non-Invasive Sensing of Sustained Hyperglycemia in Mice Using Millimeter-Wave Spectroscopy by Moreno-Oyervides, Aldo, Martín-Mateos, Pedro, Aguilera-Morillo, M Carmen, Ulisse, Giacomo, Arriba, María C, Durban, María, Rio, Marcela Del, Larcher, Fernando, Krozer, Viktor, Acedo, Pablo

“…Diabetes is a very complex condition affecting millions of people around the world. Its occurrence, always accompanied by sustained hyperglycemia, leads to…”
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Classification of skin phenotypes caused by diabetes mellitus using complex scattering parameters in the millimeter-wave frequency range by Dornuf, Fabian, Martín-Mateos, Pedro, Duarte, Blanca, Hils, Bernhard, Bonilla-Manrique, Oscar Elias, Larcher, Fernando, Acedo, Pablo, Krozer, Viktor

“…The pathological skin phenotype caused by hyperglycemia is an important indicator for the progress of diabetes mellitus. An early detection of diabetes assures…”
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Efficient CRISPR-Cas9-Mediated Gene Ablation in Human Keratinocytes to Recapitulate Genodermatoses: Modeling of Netherton Syndrome by Gálvez, Victoria, Chacón-Solano, Esteban, Bonafont, Jose, Mencía, Ángeles, Di, Wei-Li, Murillas, Rodolfo, Llames, Sara, Vicente, Asunción, Del Rio, Marcela, Carretero, Marta, Larcher, Fernando

“…Current efforts to find specific genodermatoses treatments and define precise pathogenesis mechanisms require appropriate surrogate models with human cells…”
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Long-Term Survival of Type XVII Collagen Revertant Cells in an Animal Model of Revertant Cell Therapy by Gostyński, Antoni, Llames, Sara, García, Marta, Escamez, María J., Martinez-Santamaria, Lucía, Nijenhuis, Miranda, Meana, Alvaro, Pas, Hendri H., Larcher, Fernando, Pasmooij, Anna M.G., Jonkman, Marcel F., Del Rio, Marcela

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A humanized mouse model of HPV-associated pathology driven by E7 expression by Buitrago-Pérez, Águeda, Hachimi, Mariam, Dueñas, Marta, Lloveras, Belén, Santos, Almudena, Holguín, Almudena, Duarte, Blanca, Santiago, Juan Luis, Akgül, Baki, Rodríguez-Peralto, José L, Storey, Alan, Ribas, Catalina, Larcher, Fernando, del Rio, Marcela, Paramio, Jesús M, García-Escudero, Ramón

“…Human papillomavirus (HPV) is the causative agent of human cervical cancer and has been associated with oropharyngeal squamous cell carcinoma development…”
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Long-term Engraftment of Single Genetically Modified Human Epidermal Holoclones Enables Safety Pre-assessment of Cutaneous Gene Therapy by Larcher, Fernando, Dellambra, Elena, Rico, Laura, Bondanza, Sergio, Murillas, Rodolfo, Cattoglio, Claudia, Mavilio, Fulvio, Jorcano, José L, Zambruno, Giovanna, Del Rio, Marcela

“…Predicting the risks of permanent gene therapy approaches involving the use of integrative gene-targeting vectors has become a critical issue after the…”
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