tetragastrin and Spinocerebellar-Ataxias

Spinocerebellar ataxias (SCAs) are a group of genetic diseases characterized by progressive ataxia and neurodegeneration, often in cerebellar Purkinje neurons. A SCA1 mouse model, Pcp2-ATXN1[30Q]D776, has severe ataxia in absence of progressive Purkinje neuron degeneration and death. Previous RNA-seq analyses identify cerebellar upregulation of the peptide hormone cholecystokinin (Cck) in Pcp2-ATXN1[30Q]D776 mice. Importantly, absence of Cck1 receptor (Cck1R) in Pcp2-ATXN1[30Q]D776 mice confers a progressive disease with Purkinje neuron death. Administration of a Cck1R agonist, A71623, to Pcp2-ATXN1[30Q]D776;Cck

Topics: Animals; Ataxin-1; Atrophy; Behavior, Animal; Calbindins; Chemokines, CC; Cholecystokinin; Disease Models, Animal; Female; Genetic Predisposition to Disease; Guanine Nucleotide Exchange Factors; Male; Mechanistic Target of Rapamycin Complex 1; Mice, Inbred C57BL; Mice, Knockout; Motor Activity; Nerve Degeneration; Neuropeptides; Purkinje Cells; Signal Transduction; Spinocerebellar Ataxias; Tetragastrin