Although the elevated level of Beclin-1 has been shown in npc1-/- mice, fibroblasts of Niemann-Pick patients [27], and β-gal-/- mouse brain of GM1 gangliosidosis [12], the level of Beclin-1 has not changed in mice model of multiple sulphatase deficiency (MSD) and mucopolysaccharidosis type IIIA (MPS-IIIA) [6]. The gene discussed is BECN1; the disease is mucopolysaccharidosis type 3A.