STING1 and neurodegenerative disease: Interestingly, COPA dysfunction can be reduced by genetic or pharmacological interference with STING.121 Niemann-Pick disease type C1 is a rare inherited neurodegenerative disease with a mutation in NPC1, which leads to the accumulation of cholesterol and other lipids in the lysosome, resulting in low levels of cholesterol in the ER and activation of SREBP2-SCAP translocation from the ER to the Golgi.