Moreover, the characterization of the impact on spinal integrity, in which lipofuscin accumulation was already observed in an Atp13a2 KO mouse model77 as well as brain iron accumulation in KRS patients11, remains to be elucidated in this rodent model and can be a challenging point in better understanding the mechanisms induced by the ATP13A2 dysfunction in KRS. Here, ATP13A2 is linked to Kufor-Rakeb syndrome.