Making a Dent in Dent Disease

Author:

Shipman Katherine E1,Weisz Ora A1

Affiliation:

1. Renal-Electrolyte Division, Department of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA

Abstract

Abstract Dent disease (DD) is a rare kidney disorder caused by mutations in the Cl−/H+ exchanger ClC-5. Extensive physiologic characterization of the transporter has begun to illuminate its role in endosomal ion homeostasis. Nevertheless, we have yet to understand how loss of ClC-5 function in the kidney proximal tubule impairs membrane traffic of megalin and cubilin receptors to cause the low molecular weight proteinuria characteristic of DD. This review identifies open questions that remain to be answered, evaluates the current literature addressing these questions, and suggests new testable models that may link loss of ClC-5 function to tubular proteinuria in DD.

Funder

NIH

Publisher

Oxford University Press (OUP)

Reference53 articles.

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3. Endocytosis in proximal tubule cells involves a two-phase membrane-recycling pathway;Nielsen;Am J Physiol,1993

4. Dent’s disease; a familial proximal renal tubular syndrome with low-molecular-weight proteinuria, hypercalciuria, nephrocalcinosis, metabolic bone disease, progressive renal failure and a marked male predominance;Wrong;QJM,1994

5. Idiopathic low molecular weight proteinuria associated with hypercalciuric nephrocalcinosis in Japanese children is due to mutations of the renal chloride channel (CLCN5);Lloyd;J Clin Invest,1997

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