Article
Bone marrow transplantation improves proximal tubule dysfunction in a mouse model of Dent disease.
Kidney international - 1 Apr 2017
Gabriel Sarah S, Belge Hendrica, Gassama Alkaly, Debaix Huguette, Luciani Alessandro, Fehr Thomas, Devuyst Olivier
Abstract excerpt
Dent disease is a rare X-linked tubulopathy caused by mutations in the endosomal chloride-proton exchanger (ClC-5) resulting in defective receptor-mediated endocytosis and severe proximal tubule dysfunction. Bone marrow transplantation has recently been shown to preserve kidney function in cystinosis, a lysosomal storage disease causing proximal tubule dysfunction. Here we test the effects of bone marrow...
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