92, 345, 346 and below). These patients suffer from marked developmental delays and die in early childhood (347, 348). Similarly, the mottled-brindled mice, an animal model of Menkes disease, do not survive beyond postnatal day 14, unless rescued by Cu supplementation using Cu-elesclomol (ES) (349) or the adeno-associated virus (AAV)-mediated Atp7a gene transfer together with Cu-histidine injection into the brain ventricles (350). Dietary Cu deficit during development has a particularly significant negative effect on the maturation of the dentate gyrus and hippocampus (351). This evidence concerns the gene ATP7A and Menkes disease.