To verify CER(d18:1/18:1)-LXRβ interaction in a human setting, we found that ACER3 knockdown significantly increased nuclear CER(d18:1/18:1) levels in LCA-treated HepG2 cells (Fig. S13a), confirming the nuclear colocalization of CER(d18:1/18:1) and LXRβ. Here, NR1H2 is linked to Leber congenital amaurosis.