Brain-specific Pnpla6-deficient mice display striking defects in motor coordination and neuronal degeneration with loss of Purkinje cells, disruption of the ER, accumulation of reticular aggregates, and an increase of PC [66], resembling the features of PNPLA6-related disorders [12] or hereditary spastic paraplegia [67] in humans. Here, PNPLA6 is linked to hereditary spastic paraplegia.