Disease-corrected haematopoietic progenitors from Fanconi anemia induced pluripotent stem cells

The generation of induced pluripotent stem (iPS) cells has enabled the derivation of patient-specific pluripotent cells and/nprovided valuable experimental platforms to model human disease. Patient-specific iPS cells are also thought to hold great/ntherapeutic potential, although direct evidence for...

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Detalhes bibliográficos
Autores: Rodríguez Pizà, Ignasi, Verma, Inder M., Veiga, Anna, Aasen, Trond, Izpisúa Belmonte, J. C., Bueren, Juan, Garreta Bahima, Elena, Tiscornia, Gustavo, Sleep Ronquillo, Eduard, Raya Chamorro, Ángel, Río, Paula, Consiglio, Antonella, Barrero Núñez, María José, Navarro, Susanna, Vassena, Rita, Guenechea, Guillermo
Tipo de documento: artigo
Estado:Versão publicada
Data de publicação:2009
País:España
Recursos:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)
Repositório:Recercat. Dipósit de la Recerca de Catalunya
OAI Identifier:oai:recercat.cat:10230/12426
Acesso em linha:http://hdl.handle.net/10230/12426
http://dx.doi.org/10.1038/nature08129
Access Level:Acceso aberto
Palavra-chave:Medicina regenerativa
Cél·lules mare embrionàries
Descrição
Resumo:The generation of induced pluripotent stem (iPS) cells has enabled the derivation of patient-specific pluripotent cells and/nprovided valuable experimental platforms to model human disease. Patient-specific iPS cells are also thought to hold great/ntherapeutic potential, although direct evidence for this is still lacking. Here we show that, on correction of the genetic defect,/nsomatic cells from Fanconi anaemia patients can be reprogrammed to pluripotency to generate patient-specific iPS cells. These cell lines appear indistinguishable from human embryonic stem cells and iPS cells from healthy individuals. Most importantly, we show that corrected Fanconi-anaemia-specific iPS cells can give rise to haematopoietic progenitors of the myeloid and erythroid lineages that are phenotypically normal, that is, disease-free. These data offer proof-of-concept that iPS cell technology can be used for the generation of disease-corrected, patient-specific cells with potential value for cell therapy applications.