In addition to the sensing mechanisms by cell surface receptors for extracellular and intercellular proteins, these results show a third mechanism of inflammation in the brain of neurodegenerative diseases by an intracellular receptor, which could be generalized to multiple diseases since interaction rather than co-aggregation between the disease protein and PQBP1 occurs in Huntington’s disease (HD) and spinocerebellar ataxia type 1 (SCA1)12,13,41. Here, PQBP1 is linked to spinocerebellar ataxia type 1.