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Rio1 kinase is an atypical serine/threonine-specific protein kinase belonging to the RIO family. It is essential for the final steps of cytoplasmic maturation of the 40S ribosomal subunit in eukaryotes, specifically participating in processing 18S-E pre-rRNA into mature 18S rRNA. While it possesses weak serine/threonine kinase activity, its predominant function is ATPase activity, hydrolyzing ATP during late stages of 40S maturation. Rio1 acts as a checkpoint regulator during final quality control before functional ribosome assembly and interacts with PRMT5 methyltransferase and nucleolin (NCL), influencing post-translational modifications. It is an essential gene, and its loss leads to lethality due to failed ribosome synthesis.
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