A mutation in CLCN5, which is expressed in podocytes [18], has also been identified in Dent-1 patients presenting with nephrotic range proteinuria and FSGS on renal biopsy [19–21], and in a recent review by van Berkel et al., glomerulosclerosis was detected in almost two thirds of patients with Dent disease [22]. Here, CLCN5 is linked to Dent disease.