The same cannot be said in the case of accumulation of saccharopine, as a mutation in the SDH domain of AASS leads to hyperlysinemia type II or saccharopinuria, a mild symptomatic disease that, in some cases, can lead to detrimental neurological dysfunction, retardation, and brain malfunction (Houten et al., 2013; Carson et al., 1968). Here, AASS is linked to saccharopinuria.