Elife 2020 Nov 13; 9:e62731
Ribosome-associated quality control pathways respond to defects in translational elongation to recycle arrested ribosomes and degrade aberrant polypeptides and mRNAs. Loss of a tRNA gene leads to ribosomal pausing that is resolved by the translational GTPase GTPBP2, and in its absence causes neuron death. Here, we show that loss of the homologous protein GTPBP1 during tRNA deficiency in the mouse brain also leads to codon-specific ribosome pausing and neurodegeneration, suggesting that these non-redundant GTPases function in the same pathway to mitigate ribosome pausing. As observed in
Terrey, Markus; Adamson, Scott I; Gibson, Alana L; Deng, Tianda; Ishimura, Ryuta; Chuang, Jeffrey; and Ackerman, Susan L, "GTPBP1 resolves paused ribosomes to maintain neuronal homeostasis." (2020). Faculty Research 2020. 241.