Novel cell line to study rare kidney disease

Wilmer , Martijn

In a recent collaboration between the Department Pharmacology and Toxicology, the Department of Pediatrics and the Oxford University (UK), we recently demonstrated that Dent disease-causing chloride/proton antiporter chloride channel-5 (CLC-5) mutations have differing effects on endosomal acidification and receptor-mediated endocytosis that may not be coupled.

Our findings were published in the high ranking journal Proc Natl Acad Sci USA. Briefly, we established conditionally immortalized proximal-tubular epithelial cell lines (ciPTECs) from three patients with different CLC-5 mutations causing Dent's disease. These facilitate the study of receptor-mediated endocytosis and the role of the chloride/proton antiporter chloride channel-5 (CLC-5).

Receptor-mediated endocytosis and endosomal acidification is impaired in proximal tubule epithelial cells of Dent disease patients. Gorvin CM, Wilmer MJ, Piret SE, Harding B, van den Heuvel LP, Wrong O, Jat PS, Lippiat JD, Levtchenko EN, Thakker RV. Proc Natl Acad Sci U S A. 2013 Apr 9.

Photo: Martijn Wilmer, Department of Pharmacology and Toxicology


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