While the association of STING with neurodegenerative diseases has been poorly investigated in previous studies, the activation of the immune system is a prominent feature of several neurodegenerative diseases, including Alzheimer’s disease (AD), Parkinson’s disease (PD), Huntington’s disease (HD), frontotemporal dementia (FTD), multiple sclerosis (MS), and ALS. Here, STING1 is linked to Huntington disease.