Journal
SCIENCE
Volume 345, Issue 6195, Pages 455-459Publisher
AMER ASSOC ADVANCEMENT SCIENCE
DOI: 10.1126/science.1249749
Keywords
-
Categories
Funding
- CORE grant [CA34196]
- National Science Foundation [0850155]
Ask authors/readers for more resources
In higher eukaryotes, transfer RNAs (tRNAs) with the same anticodon are encoded by multiple nuclear genes, and little is known about how mutations in these genes affect translation and cellular homeostasis. Similarly, the surveillance systems that respond to such defects in higher eukaryotes are not clear. Here, we discover that loss of GTPBP2, a novel binding partner of the ribosome recycling protein Pelota, in mice with a mutation in a tRNA gene that is specifically expressed in the central nervous system causes ribosome stalling and 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.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available