Thus, LCA is an activator of Pregnane-X-Receptor (PXR; NR1I2); whereas the transcritpional activity of the Constitutive Androstan Receptor (CAR; NR1I3) is repressed by several bile acids, among which CA, tauro-CA and conjugated form of DCA16. This evidence concerns the gene NR1I3 and Leber congenital amaurosis.