The trafficking of ATP7A to melanosomes depends on the biogenesis of lysosome-related organelles complex-1 (BLOC-1), and defects in this pathway lead to mislocalization of ATP7A, impaired tyrosinase activity, and hypopigmentation, as observed in certain forms of Hermansky-Pudlak syndrome [48]. Here, ATP7A is linked to Hermansky-Pudlak syndrome.