Peptidyl-tRNA hydrolase 1 homolog (PTRH1)

Peptidyl-tRNA hydrolase 1 homolog (PTRH1) is an enzyme localized mainly in the mitochondria that plays a critical role in maintaining translation fidelity and cellular protein homeostasis by cleaving peptidyl-tRNAs which have dissociated prematurely from stalled ribosomes or which are not stably fixed in the ribosomal P-site[1][4]. This enzymatic activity helps to recycle tRNA molecules and prevent the accumulation of peptidyl-tRNA species that could otherwise be cytotoxic or interfere with further rounds of translation[1][3]. PTRH1 acts downstream of the ribosome-associated quality control (RQC) pathway and specifically hydrolyzes non-ubiquitinated nascent chains, thus acting as a safeguard to sustain efficient mitochondrial protein synthesis and cell viability[1][3]. Pathologically, mutations or dysregulation of PTRH1 have been associated with several diseases, including certain cancers and vascular skin diseases, underscoring its relevance in human health[1].

Other names

Peptidyl-tRNA hydrolaseC9orf115PTHPTH1probable peptidyl-tRNA hydrolase