Dysfunctional Quality Control in Gaucher DiseaseWith loss of gba, lysosome function is severely impaired, and autophagy does not occur. Dysfunctional organelles accumulate, including mitochondria. Due to loss of gba and the self-propagation of α-synuclein, the autophagic pathway is disrupted, compromising UPS function. The damaged mitochondrial respiratory chain cannot support the potential, which decreases to a level at which the ATPase reverses. A decreased ΔΨm limits their ability to re-enter the fission-fusion cycle; this low ΔΨm, maintained by the ATPase, appears to be sufficient in maintaining a potential above the critical level required to recruit Parkin, and thus mitochondria are not flagged for turnover, amplifying the defect. 