WT1 (OMIM 607102) is an autosomal dominant gene associated with the development of isolated Wilms tumor, isolated nephrotic proteinuria, or in the setting of syndromic conditions like Denys–Drash syndrome (DDS) and Frasier syndromes (FS), both including FSGS in association with sexual abnormalities [107]. The gene discussed is WT1; the disease is focal segmental glomerulosclerosis.