In humans, Batten disease is characterised by the accumulate abnormal storage material in irregular granular deposits (GRODs) and autofluorescent inclusions in the brain, and the progressive death of neurons in the CNS. The Ppt1dsRNA.Scer\UAS, Ppt1 knockdown flies show abnormal storage material in highly laminar spherical deposits and increased autofluorescent inclusions.
Flies expressing Ppt1dsRNA.Scer\UAS under the control of Scer\GAL4Act5C.PI accumulate abnormal laminar deposits in their brains.
Used to study the phenotypic consequences of dsRNA interference (RNAi) of the Ppt1 gene.