Cysteamine treatment restores the in vitro ability to differentiate along the osteoblastic lineage of mesenchymal stromal cells isolated from bone marrow of a cystinotic patient

BACKGROUND: Cystinosis is a rare autosomal recessive disease caused by mutations of the CTNS gene, which encodes for a lysosomal cystine/H(+) symporter. In mice, inactivation of the CTNS gene causes intralysosomal cystine accumulation and progressive organ damage that can be reversed, at least in pa...

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Detalles Bibliográficos
Autores principales: Conforti, Antonella, Taranta, Anna, Biagini, Simone, Starc, Nadia, Pitisci, Angela, Bellomo, Francesco, Cirillo, Valentina, Locatelli, Franco, Bernardo, Maria Ester, Emma, Francesco
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2015
Materias:
Research
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4428230/
https://www.ncbi.nlm.nih.gov/pubmed/25947233
http://dx.doi.org/10.1186/s12967-015-0494-0

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