In the meantime, mutations in the OCRL also can lead to a type 2 Dent disease (Dent-2 disease) that can present as isolated proximal renal tubulopathy, characterized by low-molecular-weight proteinuria (LMWP), hypercalciuria, and progressive renal insufficiency in the absence of extrarenal pathologies [2]. Here, OCRL is linked to Dent disease.