Your browser doesn't support javascript.
loading
Generation of induced Pluripotent Stem Cells (UNIBSi008-A, UNIBSi008-B, UNIBSi008-C) from an Ataxia-Telangiectasia (AT) patient carrying a novel homozygous deletion in ATM gene.
Masneri, S; Ferraro, R M; Lanzi, G; Piovani, G; Mori, L; Barisani, C; Moratto, D; Plebani, A; Badolato, R; Soresina, A; Giliani, S.
Afiliación
  • Masneri S; "Angelo Nocivelli" Institute for Molecular Medicine, Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy. Electronic address: stefania.masneri@libero.it.
  • Ferraro RM; "Angelo Nocivelli" Institute for Molecular Medicine, Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy.
  • Lanzi G; "Angelo Nocivelli" Institute for Molecular Medicine, Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy.
  • Piovani G; Biology and Genetics Division, Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy.
  • Mori L; Molecular Medicine Laboratory, Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy.
  • Barisani C; "Angelo Nocivelli" Institute for Molecular Medicine, Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy.
  • Moratto D; "Angelo Nocivelli" Institute for Molecular Medicine, Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy.
  • Plebani A; Pediatrics Clinic and "Angelo Nocivelli" Institute for Molecular Medicine, Department of Clinical and Experimental Sciences, ASST Spedali Civili of Brescia, University of Brescia, Brescia, Italy.
  • Badolato R; Pediatrics Clinic and "Angelo Nocivelli" Institute for Molecular Medicine, Department of Clinical and Experimental Sciences, ASST Spedali Civili of Brescia, University of Brescia, Brescia, Italy.
  • Soresina A; Unit of Pediatric Immunology, Department of Pediatrics, ASST Spedali Civili of Brescia, University of Brescia, Brescia, Italy.
  • Giliani S; "Angelo Nocivelli" Institute for Molecular Medicine, Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy.
Stem Cell Res ; 41: 101596, 2019 12.
Article en En | MEDLINE | ID: mdl-31669783
ABSTRACT
Using a Sendai Virus based vector delivering Yamanaka Factors, we generated induced Pluripotent Stem Cells (iPSCs) from peripheral blood mononuclear cells of a patient affected by Ataxia Telangiectasia (AT), caused by a novel homozygous deletion in ATM, spanning exons 5-7. Three clones were fully characterized for pluripotency and capability to differentiate. These clones preserved the causative mutation of parental cells and genomic stability over time (>100 passages). Furthermore, in AT derived iPSCs we confirmed the impaired DNA damage response after ionizing radiation. All these data underline potential usefulness of our clones as in vitro AT disease model.
Asunto(s)
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Ataxia Telangiectasia / Leucocitos Mononucleares / Diferenciación Celular / Células Madre Pluripotentes Inducidas / Proteínas de la Ataxia Telangiectasia Mutada / Mutación Tipo de estudio: Prognostic_studies Límite: Adult / Female / Humans Idioma: En Revista: Stem Cell Res Año: 2019 Tipo del documento: Article
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Ataxia Telangiectasia / Leucocitos Mononucleares / Diferenciación Celular / Células Madre Pluripotentes Inducidas / Proteínas de la Ataxia Telangiectasia Mutada / Mutación Tipo de estudio: Prognostic_studies Límite: Adult / Female / Humans Idioma: En Revista: Stem Cell Res Año: 2019 Tipo del documento: Article