Ribosome stalling induced by mutation of a CNS-specific tRNA causes neurodegeneration

ABSTRACT: In higher eukaryotes, the large numbers of nuclear-encoded tRNA genes partially ensure the robustness of cytoplasmic protein translation. Here we discover that a loss-of-function in n-Tr20, a member of the nuclear-encoded tRNA Arg UCU family that is expressed specifically in the central nervous systems leads to low but detectable levels of ribosome stalling. In the absence of GTPBP2, a novel binding partner of the ribosome recycling protein Pelota, ribosome stalling increases, leading to widespread neurodegeneration. Our results not only define GTPBP2 as a ribosome rescue factor, but also unmask the disease potential of mutations in nuclear-encoded tRNA genes. In this submission we provide ribosome footprinting data from the cerebella of four strains derived from the C57BL/6J strain with c

ORGANISM(S): Mus musculus

SUBMITTER: Jeffrey Chuang 

PROVIDER: E-GEOD-56127 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress