PRKAA1 and spinocerebellar ataxia type 17: AICAR, an AMPK activator, raised pAMPKα and diminished the effects of aggregation reducing and neurite outgrowth enhancing by treatment with LM-031, which suggests increased pAMPKα contributing to neurotoxicity in SCA17 and LM-031 provides beneficial effect by reducing pAMPKα.