Interestingly, glucosylceramides, which are glycosphingolipid precursors, were found to be highly increased in the kidneys of cystic mouse models including in pcy/Nphp3 and Jck/Nek8/Nphp9 mice and pharmacological or genetic inhibition of glucosylceramide synthase activity efficiently reduced renal cysts in Jck/Nek8/Nphp9 mice (Natoli et al., 2010, 2012). The gene discussed is NPHP3; the disease is cystic kidney disease.