As one example, mouse models of Niemann-Pick disease show deficits in cholesterol metabolism and 3α,5α-THP production in the brain, and such deficits could be reversed with administration of PXR ligands, one of them being 3α,5α-THP (Frye and Rhodes, 2006; Frye, 2009; Brinton, 2013). Here, NR1I2 is linked to Niemann-Pick disease.