Notably, CAR has been shown to have a fundamental protective role in the response to LCA in vivo since CAR-KO mice have more severe defects in LCA detoxification compared to PXR-KO mice [15] and CAR activation in transgenic mice confers resistance to the hepatotoxicity of LCA [30]. The gene discussed is NR1I2; the disease is Leber congenital amaurosis.