Article
Stem cell microvesicles transfer cystinosin to human cystinotic cells and reduce cystine accumulation in vitro.
PloS one - 1 Jan 2012
Iglesias Diana M, El-Kares Reyhan, Taranta Anna, Bellomo Francesco, Emma Francesco, Besouw Martine, Levtchenko Elena, Toelen Jaan, van den Heuvel Lambertus, Chu Leelee, Zhao Jing, Young Yoon Kow, Eliopoulos Nicoletta, Goodyer Paul
Abstract excerpt
Cystinosis is a rare disease caused by homozygous mutations of the CTNS gene, encoding a cystine efflux channel in the lysosomal membrane. In Ctns knockout mice, the pathologic intralysosomal accumulation of cystine that drives progressive organ damage can be reversed by infusion of wildtype bone marrow-derived stem cells, but the mechanism involved is unclear since the exogeneous stem cells are rarely integrated...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.
