Efficient CRISPR-Cas9-Mediated Gene Ablation in Human Keratinocytes to Recapitulate Genodermatoses: Modeling of Netherton Syndrome.

CRISPR/Cas9 Netherton syndrome disease modeling gene editing genodermatosis human keratinocytes skin equivalents.

Journal

Molecular therapy. Methods & clinical development
ISSN: 2329-0501
Titre abrégé: Mol Ther Methods Clin Dev
Pays: United States
ID NLM: 101624857

Informations de publication

Date de publication:
11 Sep 2020
Historique:
received: 07 02 2020
accepted: 27 05 2020
entrez: 9 7 2020
pubmed: 9 7 2020
medline: 9 7 2020
Statut: epublish

Résumé

Current efforts to find specific genodermatoses treatments and define precise pathogenesis mechanisms require appropriate surrogate models with human cells. Although transgenic and gene knockout mouse models for several of these disorders exist, they often fail to faithfully replicate the clinical and histopathological features of the human skin condition. We have established a highly efficient method for precise deletion of critical gene sequences in primary human keratinocytes, based on CRISPR-Cas9-mediated gene editing. Using this methodology, in the present study we generated a model of Netherton syndrome by disruption of

Identifiants

pubmed: 32637457
doi: 10.1016/j.omtm.2020.05.031
pii: S2329-0501(20)30118-2
pmc: PMC7329935
doi:

Types de publication

Journal Article

Langues

eng

Pagination

280-290

Informations de copyright

© 2020 The Author(s).

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Auteurs

Victoria Gálvez (V)

Epithelial Biomedicine Division, Centro de Investigaciones Energéticas Medioambientales y Tecnológicas (CIEMAT), 28040 Madrid, Spain.
Regenerative Medicine and Tissue Engineering Group, Instituto de Investigación Sanitaria de la Fundación Jiménez Díaz (IIS-FJD), 28040 Madrid, Spain.
Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) U714, Madrid, Spain.

Esteban Chacón-Solano (E)

Regenerative Medicine and Tissue Engineering Group, Instituto de Investigación Sanitaria de la Fundación Jiménez Díaz (IIS-FJD), 28040 Madrid, Spain.
Department of Biomedical Engineering, Carlos III University (UC3M), 28903 Madrid, Spain.
Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) U714, Madrid, Spain.

Jose Bonafont (J)

Regenerative Medicine and Tissue Engineering Group, Instituto de Investigación Sanitaria de la Fundación Jiménez Díaz (IIS-FJD), 28040 Madrid, Spain.
Department of Biomedical Engineering, Carlos III University (UC3M), 28903 Madrid, Spain.
Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) U714, Madrid, Spain.

Ángeles Mencía (Á)

Epithelial Biomedicine Division, Centro de Investigaciones Energéticas Medioambientales y Tecnológicas (CIEMAT), 28040 Madrid, Spain.
Regenerative Medicine and Tissue Engineering Group, Instituto de Investigación Sanitaria de la Fundación Jiménez Díaz (IIS-FJD), 28040 Madrid, Spain.
Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) U714, Madrid, Spain.

Wei-Li Di (WL)

UCL GOS Institute of Child Health, London WC1N 1EH, UK.

Rodolfo Murillas (R)

Epithelial Biomedicine Division, Centro de Investigaciones Energéticas Medioambientales y Tecnológicas (CIEMAT), 28040 Madrid, Spain.
Regenerative Medicine and Tissue Engineering Group, Instituto de Investigación Sanitaria de la Fundación Jiménez Díaz (IIS-FJD), 28040 Madrid, Spain.
Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) U714, Madrid, Spain.

Sara Llames (S)

Regenerative Medicine and Tissue Engineering Group, Instituto de Investigación Sanitaria de la Fundación Jiménez Díaz (IIS-FJD), 28040 Madrid, Spain.
Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) U714, Madrid, Spain.
Tissue Engineering Unit, Centro Comunitario Sangre y Tejidos (CCST), 33006 Oviedo, Spain.

Asunción Vicente (A)

Hospital Sant Joan de Deu, 08950 Barcelona, Spain.
Unidad de Dermatología, Hospital Materno-Infantil Sant Joan de Déu, 08950 Barcelona, Spain.

Marcela Del Rio (M)

Regenerative Medicine and Tissue Engineering Group, Instituto de Investigación Sanitaria de la Fundación Jiménez Díaz (IIS-FJD), 28040 Madrid, Spain.
Department of Biomedical Engineering, Carlos III University (UC3M), 28903 Madrid, Spain.
Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) U714, Madrid, Spain.

Marta Carretero (M)

Epithelial Biomedicine Division, Centro de Investigaciones Energéticas Medioambientales y Tecnológicas (CIEMAT), 28040 Madrid, Spain.
Regenerative Medicine and Tissue Engineering Group, Instituto de Investigación Sanitaria de la Fundación Jiménez Díaz (IIS-FJD), 28040 Madrid, Spain.
Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) U714, Madrid, Spain.

Fernando Larcher (F)

Epithelial Biomedicine Division, Centro de Investigaciones Energéticas Medioambientales y Tecnológicas (CIEMAT), 28040 Madrid, Spain.
Regenerative Medicine and Tissue Engineering Group, Instituto de Investigación Sanitaria de la Fundación Jiménez Díaz (IIS-FJD), 28040 Madrid, Spain.
Department of Biomedical Engineering, Carlos III University (UC3M), 28903 Madrid, Spain.
Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) U714, Madrid, Spain.

Classifications MeSH