In a recent study (Osellame et al. 2013), we showed that in neurons cultured from the CNS of GBA knockout mice, autophagy was down regulated, mitochondrial function was profoundly compromised and there was early accumulation of α-synuclein, the major component of Lewy bodies, the pathological signature of PD which reflects also a failure of UPS mediated protein degradation pathways. This evidence concerns the gene GBA1 and Parkinson disease.