This is a summary of the virtual presentation given at the 2021 meeting of the Society for Research on the Cerebellum and Ataxias,https://www.meetings.be/SRCA2021/, where the therapeutic potential of the CCK-CCK1R pathway for treating diseases involving Purkinje cell degeneration was presented. Spinocerebellar ataxia type 1 (SCA1) is one of a group of almost 50 genetic diseases characterized by the degeneration of cerebellar Purkinje cells. The SCA1Pcp2-ATXN1[30Q]D776mouse model displays ataxia, i.e. Purkinje cell dysfunction, but lacks progressive Purkinje cell degeneration. RNA-seq revealed increased expression of cholecystokinin (CCK) in cerebella ofPcp2-ATXN1[30Q]D776mice. Importantly, the absence of Cck1 receptor (CCK1R) inPcp2-ATXN1[30Q]D776mice conferred a progressive degenerative disease with Purkinje cell loss. Administration of a CCK1R agonist toPcp2-AXTN1[82Q]mice reduced Purkinje cell pathology and associated deficits in motor performance. In addition, administration of the CCK1R agonist improved motor performance ofPcp2-ATXN2[127Q]SCA2 mice. Furthermore, CCK1R activation corrected mTORC1 signaling and improved the expression of calbindin in the cerebella ofAXTN1[82Q]andATXN2[127Q]mice. These results support the Cck-Cck1R pathway is a potential therapeutic target for the treatment of diseases involving Purkinje neuron degeneration.