In particular, the prominent cerebellar and spinal cord pathology in our KIN SCA2 mouse model closely resembles findings in patients with SCA2 in terms of atrophy and neuronal loss/disconnection, neuroinflammation with microgliosis and astrogliosis, TDP-43 mislocalization and aggregation, demyelination and early sensory axonal neuropathy9,25,28,32,76,96–101, stressing its relevance for translational research. This evidence concerns the gene ATXN2 and spinocerebellar ataxia type 2.