Also, mutations in OCRL (OMIM 300535) may cause a severe tubular dysfunction called Lowe syndrome in the setting of Dent disease type 2 (Dent-2), characterized by ocular abnormalities, intellectual impairment, CKD, and rapid progression to KF, in which persistent proteinuria and FSGS have been described, as well [152]. Here, OCRL is linked to Dent disease.