- Bone marrow transplantation improves proximal tubule dysfunction in a mouse model of Dent disease.
Bone marrow transplantation improves proximal tubule dysfunction in a mouse model of Dent disease.
Kidney international (2017-02-02)
Sarah S Gabriel, Hendrica Belge, Alkaly Gassama, Huguette Debaix, Alessandro Luciani, Thomas Fehr, Olivier Devuyst
PMID28143656
ABSTRACT
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 transplantation in Clcn5
MATERIALS
Product Number
Brand
Product Description
Sigma-Aldrich
Dulbecco′s Phosphate Buffered Saline, With calcium chloride and magnesium chloride, 10×, liquid, sterile-filtered, suitable for cell culture