E3 ubiquitin-protein ligase ZNF598 (ZNF598)

E3 ubiquitin-protein ligase that plays a key role in the ribosome quality control (RQC), a pathway that happens when a ribosome has stalled during translation (PubMed:28065601, PubMed:28132843). Required for ribosomes to stall during translation of poly(A) sequences by mediating monoubiquitination of 40S ribosomal protein RPS10/eS10, RPS20/uS10 and RPS3/uS3 (PubMed:28065601, PubMed:28132843).

Stalling precludes synthesis of a long poly-lysine tail and initiates the RQC pathway to degrade the possibly harmful aberrant nascent polypeptide (PubMed:28065601, PubMed:28132843). Also acts as a component of the 4EHP-GYF2 complicated, a multiprotein complex that functions as a repressor of translation initiation (PubMed:22751931).