Mutations in the human nuclear hormone receptor gene NR2E3 (Haider et al., 2000) with two rather different conditions, a recessive one due to insufficient NR2E3, resulting in a disorder of photoreceptor cell fate known as the Enhanced S-Cone Syndrome (ESCS) in which rods are replaced by S-cones that remain preserved for extended periods of time (Roman et al., 2019; Iannaccone et al., 2021a), and in a dominant disorder in which abnormal gain of function mutations cause instead a form of RP without enhanced function or selective preservation of the S-cones (S. Li et al., 2021a). Here, NR2E3 is linked to retinitis pigmentosa 1.