To provide a compound which enables treatment or prevention of
spinocerebellar ataxia, analyses were carried out based on a screening using a
spinocerebellar ataxia type 1 (SCA1) fly model and on the like. As a result, the following proteins ameliorating the
pathology of
spinocerebellar ataxia were identified: RPA1, PNKP, XRCC3, XRCC4, CCNH, POLE, POLH, and PERI. On the other hand, the following proteins aggravating the
pathology were identified: CHK1, LIG3, FEN1, LIG1, ERCC5, XAB2, ERCC2, DMC1, RECQL5, MUS81, EME1, SPO11, and BLM. In addition, it has been revealed that ATXN1, which is a cause of SCA1, binds to RPA1, BRCA1, and BRCA2, and suppresses the activities of these proteins, so that the above-described
pathology is caused.