Mutations in the mouse gene n-Tr20, which encodes a tRNA specifically expressed in the central nervous system, can slow translation at AGA codons by increasing ribosome pausing, thereby promoting neuronal death, a new study in Science shows. However, analyses of multiple strains of mice revealed that the neurodegeneration only manifests when the n-Tr20 mutation co-occurs with a loss-of-function mutation in GTP-binding protein 2 (Gtpbp2). Co-immunoprecipitation and affinity capture experiments showed that GTPBP2 directly interacts with the ribosome recycling protein Pelota.