“…While there are several known human hereditary diseases caused by cholesterol biosynthetic defects, they are all recessive and, thankfully, rare, and none of them are non-syndromic (i.e., all bodily tissues are affected, not specifically the retina) [ 2 , 84 ]. While pharmacological and dietary supplementation approaches have been tried as therapeutic interventions for clinical management of patients afflicted with such diseases, in general, those approaches have not proven to be widely effective [ 85 , 86 , 87 , 88 ]. With regard to human diseases that involve disruption of cholesterol homeostasis that involve structural and/or functional abnormalities in the retina, it is more often the case of having too much cholesterol (and its esters and oxidized by-products), i.e., deposition and failure to efficiently remove excess cholesterol-rich deposits, rather than local defective de novo synthesis of cholesterol [ 89 , 90 ].…”