Human mtRF1 terminates COX1 translation and its ablation induces mitochondrial ribosome-associated quality control.

ABSTRACT: Translation termination requires release factors that read a STOP codon in the decoding center and subsequently facilitate the hydrolysis of the nascent peptide chain from the peptidyl tRNA within the ribosome. In human mitochondria eleven open reading frames terminate in the standard UAA or UAG STOP codon, which can be recognized by mtRF1a, the proposed major mitochondrial release factor. However, two transcripts encoding for COX1 and ND6 terminate in the non-conventional AGA or AGG codon, respectively. How translation termination is achieved in these two cases is not known. We address this long-standing open question by showing that the non-canonical release factor mtRF1 is a specialized release factor that triggers COX1 translation termination, while mtRF1a terminates the majority of ot

SUBMITTER: Nadler F 

PROVIDER: S-EPMC9613700 | biostudies-literature | 2022 Oct

REPOSITORIES: biostudies-literature