RIOK1 Gene

Name	RIO kinase 1
Description	The protein encoded by this gene competes with pICln for inclusion in the protein arginine methyltransferase 5 complex. This complex targets substrates for dimethylation. The encoded protein is essential for the last steps in the maturation of 40S subunits. [provided by RefSeq, Jan 2017]
Summary	{"type": "root", "children": [{"type": "p", "children": [{"type": "t", "text": "\nRIOK1, a member of the evolutionarily conserved atypical protein kinase family, plays a central role in ribosome biogenesis by orchestrating the final cytoplasmic maturation steps of pre‐40S ribosomal subunits. Functional studies have shown that loss or impairment of RIOK1’s ATPase/kinase activity disrupts 18S rRNA processing and interferes with the recycling of essential trans‐acting factors (e.g. hNob1 and hDim2), ultimately leading to defective formation of translation‐competent ribosomes. Structural and cryo‐electron microscopy analyses further reveal that ATP‐bound RIOK1, through interaction with ribosomal protein RPS26, displaces inhibitory factors to promote the final maturation cascade."}, {"type": "fg", "children": [{"type": "fg_fs", "start_ref": "1", "end_ref": "3"}]}, {"type": "t", "text": "\n"}]}, {"type": "t", "text": "\n\n"}, {"type": "p", "children": [{"type": "t", "text": "\nIn addition to its pivotal function in ribosome assembly, RIOK1 acts as an important regulatory component of protein arginine methylation. It has been identified as a novel, stoichiometric partner of the PRMT5 methyltransferase complex, where it competes with pICln for binding to PRMT5. This competitive interaction enables RIOK1 to function as an adaptor—recruiting substrates such as nucleolin for symmetrical arginine dimethylation—and connects the methylation state of cellular proteins with cellular metabolism. Moreover, in cancers with co-deletions involving MTAP and CDKN2A, the association of PRMT5 co‐complex proteins, including RIOK1, underlies a synthetic lethal vulnerability that may be therapeutically exploited."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "4"}]}, {"type": "t", "text": "\n"}]}, {"type": "t", "text": "\n\n"}, {"type": "p", "children": [{"type": "t", "text": "\nRIOK1 is also increasingly recognized for its role in malignant signaling and tumor progression across diverse cancer types. In glioblastoma, for example, constitutive Akt signaling upregulates RIOK1, which then partners with mTOR and mTOR-complex-2 components to reinforce an oncogenic feedforward loop. In RAS mutant cancers, RIOK1 drives NF-κB activation and cell cycle progression, while its dysregulation—modulated by posttranslational modifications such as SETD7-mediated methylation and CK2-dependent phosphorylation—affects its stability and downstream effects on metastasis and therapy resistance. Furthermore, pharmacological inhibitors (including toyocamycin and benzimidazole derivatives) targeting RIOK1 have demonstrated significant antitumor potential in colorectal, breast, and prostate cancer models."}, {"type": "fg", "children": [{"type": "fg_fs", "start_ref": "6", "end_ref": "12"}]}, {"type": "t", "text": "\n"}]}, {"type": "rg", "children": [{"type": "r", "ref": 1, "children": [{"type": "t", "text": "Barbara Widmann, Franziska Wandrey, Lukas Badertscher, et al. "}, {"type": "b", "children": [{"type": "t", "text": "The kinase activity of human Rio1 is required for final steps of cytoplasmic maturation of 40S subunits."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Mol Biol Cell (2012)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1091/mbc.E11-07-0639"}], "href": "https://doi.org/10.1091/mbc.E11-07-0639"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "22072790"}], "href": "https://pubmed.ncbi.nlm.nih.gov/22072790"}]}, {"type": "r", "ref": 2, "children": [{"type": "t", "text": "Sébastien Ferreira-Cerca, Irene Kiburu, Emma Thomson, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Dominant Rio1 kinase/ATPase catalytic mutant induces trapping of late pre-40S biogenesis factors in 80S-like ribosomes."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Nucleic Acids Res (2014)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1093/nar/gku542"}], "href": "https://doi.org/10.1093/nar/gku542"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "24948609"}], "href": "https://pubmed.ncbi.nlm.nih.gov/24948609"}]}, {"type": "r", "ref": 3, "children": [{"type": "t", "text": "Laura Plassart, Ramtin Shayan, Christian Montellese, et al. "}, {"type": "b", "children": [{"type": "t", "text": "The final step of 40S ribosomal subunit maturation is controlled by a dual key lock."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Elife (2021)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.7554/eLife.61254"}], "href": "https://doi.org/10.7554/eLife.61254"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "33908345"}], "href": "https://pubmed.ncbi.nlm.nih.gov/33908345"}]}, {"type": "r", "ref": 4, "children": [{"type": "t", "text": "Gernot Guderian, Christoph Peter, Julia Wiesner, et al. "}, {"type": "b", "children": [{"type": "t", "text": "RioK1, a new interactor of protein arginine methyltransferase 5 (PRMT5), competes with pICln for binding and modulates PRMT5 complex composition and substrate specificity."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Biol Chem (2011)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1074/jbc.M110.148486"}], "href": "https://doi.org/10.1074/jbc.M110.148486"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "21081503"}], "href": "https://pubmed.ncbi.nlm.nih.gov/21081503"}]}, {"type": "r", "ref": 5, "children": [{"type": "t", "text": "Katya Marjon, Michael J Cameron, Phong Quang, et al. "}, {"type": "b", "children": [{"type": "t", "text": "MTAP Deletions in Cancer Create Vulnerability to Targeting of the MAT2A/PRMT5/RIOK1 Axis."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Cell Rep (2016)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.celrep.2016.03.043"}], "href": "https://doi.org/10.1016/j.celrep.2016.03.043"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "27068473"}], "href": "https://pubmed.ncbi.nlm.nih.gov/27068473"}]}, {"type": "r", "ref": 6, "children": [{"type": "t", "text": "Renee D Read, Tim R Fenton, German G Gomez, et al. "}, {"type": "b", "children": [{"type": "t", "text": "A kinome-wide RNAi screen in Drosophila Glia reveals that the RIO kinases mediate cell proliferation and survival through TORC2-Akt signaling in glioblastoma."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "PLoS Genet (2013)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1371/journal.pgen.1003253"}], "href": "https://doi.org/10.1371/journal.pgen.1003253"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "23459592"}], "href": "https://pubmed.ncbi.nlm.nih.gov/23459592"}]}, {"type": "r", "ref": 7, "children": [{"type": "t", "text": "Konrad Kubiński, Maciej Masłyk, Andrzej Orzeszko "}, {"type": "b", "children": [{"type": "t", "text": "Benzimidazole inhibitors of protein kinase CK2 potently inhibit the activity of atypical protein kinase Rio1."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Mol Cell Biochem (2017)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1007/s11010-016-2892-x"}], "href": "https://doi.org/10.1007/s11010-016-2892-x"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "27909846"}], "href": "https://pubmed.ncbi.nlm.nih.gov/27909846"}]}, {"type": "r", "ref": 8, "children": [{"type": "t", "text": "Florian Weinberg, Nadine Reischmann, Lisa Fauth, et al. "}, {"type": "b", "children": [{"type": "t", "text": "The Atypical Kinase RIOK1 Promotes Tumor Growth and Invasive Behavior."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "EBioMedicine (2017)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.ebiom.2017.04.015"}], "href": "https://doi.org/10.1016/j.ebiom.2017.04.015"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "28499923"}], "href": "https://pubmed.ncbi.nlm.nih.gov/28499923"}]}, {"type": "r", "ref": 9, "children": [{"type": "t", "text": "Xuehui Hong, He Huang, Xingfeng Qiu, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Targeting posttranslational modifications of RIOK1 inhibits the progression of colorectal and gastric cancers."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Elife (2018)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.7554/eLife.29511"}], "href": "https://doi.org/10.7554/eLife.29511"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "29384474"}], "href": "https://pubmed.ncbi.nlm.nih.gov/29384474"}]}, {"type": "r", "ref": 10, "children": [{"type": "t", "text": "Zhiqi Huang, Xingyu Li, Tian Xie, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Elevated Expression of RIOK1 Is Correlated with Breast Cancer Hormone Receptor Status and Promotes Cancer Progression."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Cancer Res Treat (2020)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.4143/crt.2020.187"}], "href": "https://doi.org/10.4143/crt.2020.187"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "32599985"}], "href": "https://pubmed.ncbi.nlm.nih.gov/32599985"}]}, {"type": "r", "ref": 11, "children": [{"type": "t", "text": "Yaqi Chen, Sha Zhou, Kairui Wan, et al. "}, {"type": "b", "children": [{"type": "t", "text": "RIOK1 mediates p53 degradation and radioresistance in colorectal cancer through phosphorylation of G3BP2."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Oncogene (2022)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1038/s41388-022-02352-4"}], "href": "https://doi.org/10.1038/s41388-022-02352-4"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "35589951"}], "href": "https://pubmed.ncbi.nlm.nih.gov/35589951"}]}, {"type": "r", "ref": 12, "children": [{"type": "t", "text": "Florian Handle, Martin Puhr, Martina Gruber, et al. "}, {"type": "b", "children": [{"type": "t", "text": "The Oncogenic Protein Kinase/ATPase RIOK1 Is Up-Regulated via the c-myc/E2F Transcription Factor Axis in Prostate Cancer."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Am J Pathol (2023)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.ajpath.2023.05.013"}], "href": "https://doi.org/10.1016/j.ajpath.2023.05.013"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "37301535"}], "href": "https://pubmed.ncbi.nlm.nih.gov/37301535"}]}]}]}
Synonyms	AD034, RRP10, BA288G3.1, RIO1
Proteins	RIOK1_HUMAN
NCBI Gene ID	83732
API
Download Associations
Predicted Functions
Co-expressed Genes
Expression in Tissues and Cell Lines

Functional Associations

RIOK1 has 5,360 functional associations with biological entities spanning 9 categories (molecular profile, organism, functional term, phrase or reference, disease, phenotype or trait, chemical, structural feature, cell line, cell type or tissue, gene, protein or microRNA, sequence feature) extracted from 113 datasets.

Click the + buttons to view associations for RIOK1 from the datasets below.

If available, associations are ranked by standardized value

Harmonizome 3.0

RIOK1 Gene

Functional Associations

Please acknowledge the Harmonizome in your publications by citing the following reference(s):

	Dataset	Summary
	Achilles Cell Line Gene Essentiality Profiles	cell lines with fitness changed by RIOK1 gene knockdown relative to other cell lines from the Achilles Cell Line Gene Essentiality Profiles dataset.
	Allen Brain Atlas Adult Human Brain Tissue Gene Expression Profiles	tissues with high or low expression of RIOK1 gene relative to other tissues from the Allen Brain Atlas Adult Human Brain Tissue Gene Expression Profiles dataset.
	Allen Brain Atlas Adult Mouse Brain Tissue Gene Expression Profiles	tissues with high or low expression of RIOK1 gene relative to other tissues from the Allen Brain Atlas Adult Mouse Brain Tissue Gene Expression Profiles dataset.
	Allen Brain Atlas Aging Dementia and Traumatic Brain Injury Tissue Sample Gene Expression Profiles	tissue samples with high or low expression of RIOK1 gene relative to other tissue samples from the Allen Brain Atlas Aging Dementia and Traumatic Brain Injury Tissue Sample Gene Expression Profiles dataset.
	Allen Brain Atlas Developing Human Brain Tissue Gene Expression Profiles by Microarray	tissue samples with high or low expression of RIOK1 gene relative to other tissue samples from the Allen Brain Atlas Developing Human Brain Tissue Gene Expression Profiles by Microarray dataset.
	Allen Brain Atlas Developing Human Brain Tissue Gene Expression Profiles by RNA-seq	tissue samples with high or low expression of RIOK1 gene relative to other tissue samples from the Allen Brain Atlas Developing Human Brain Tissue Gene Expression Profiles by RNA-seq dataset.
	Allen Brain Atlas Prenatal Human Brain Tissue Gene Expression Profiles	tissues with high or low expression of RIOK1 gene relative to other tissues from the Allen Brain Atlas Prenatal Human Brain Tissue Gene Expression Profiles dataset.
	BioGPS Human Cell Type and Tissue Gene Expression Profiles	cell types and tissues with high or low expression of RIOK1 gene relative to other cell types and tissues from the BioGPS Human Cell Type and Tissue Gene Expression Profiles dataset.
	BioGPS Mouse Cell Type and Tissue Gene Expression Profiles	cell types and tissues with high or low expression of RIOK1 gene relative to other cell types and tissues from the BioGPS Mouse Cell Type and Tissue Gene Expression Profiles dataset.
	CCLE Cell Line Gene CNV Profiles	cell lines with high or low copy number of RIOK1 gene relative to other cell lines from the CCLE Cell Line Gene CNV Profiles dataset.
	CCLE Cell Line Gene Expression Profiles	cell lines with high or low expression of RIOK1 gene relative to other cell lines from the CCLE Cell Line Gene Expression Profiles dataset.
	CCLE Cell Line Gene Mutation Profiles	cell lines with RIOK1 gene mutations from the CCLE Cell Line Gene Mutation Profiles dataset.
	CCLE Cell Line Proteomics	Cell lines associated with RIOK1 protein from the CCLE Cell Line Proteomics dataset.
	CellMarker Gene-Cell Type Associations	cell types associated with RIOK1 gene from the CellMarker Gene-Cell Type Associations dataset.
	ChEA Transcription Factor Binding Site Profiles	transcription factor binding site profiles with transcription factor binding evidence at the promoter of RIOK1 gene from the CHEA Transcription Factor Binding Site Profiles dataset.
	ChEA Transcription Factor Targets	transcription factors binding the promoter of RIOK1 gene in low- or high-throughput transcription factor functional studies from the CHEA Transcription Factor Targets dataset.
	ChEA Transcription Factor Targets 2022	transcription factors binding the promoter of RIOK1 gene in low- or high-throughput transcription factor functional studies from the CHEA Transcription Factor Targets 2022 dataset.
	CM4AI U2OS Cell Map Protein Localization Assemblies	assemblies containing RIOK1 protein from integrated AP-MS and IF data from the CM4AI U2OS Cell Map Protein Localization Assemblies dataset.
	COMPARTMENTS Curated Protein Localization Evidence Scores 2025	cellular components containing RIOK1 protein from the COMPARTMENTS Curated Protein Localization Evidence Scores 2025 dataset.
	COMPARTMENTS Experimental Protein Localization Evidence Scores	cellular components containing RIOK1 protein in low- or high-throughput protein localization assays from the COMPARTMENTS Experimental Protein Localization Evidence Scores dataset.
	COMPARTMENTS Experimental Protein Localization Evidence Scores 2025	cellular components containing RIOK1 protein in low- or high-throughput protein localization assays from the COMPARTMENTS Experimental Protein Localization Evidence Scores 2025 dataset.
	COMPARTMENTS Text-mining Protein Localization Evidence Scores	cellular components co-occuring with RIOK1 protein in abstracts of biomedical publications from the COMPARTMENTS Text-mining Protein Localization Evidence Scores dataset.
	COMPARTMENTS Text-mining Protein Localization Evidence Scores 2025	cellular components co-occuring with RIOK1 protein in abstracts of biomedical publications from the COMPARTMENTS Text-mining Protein Localization Evidence Scores 2025 dataset.
	COSMIC Cell Line Gene CNV Profiles	cell lines with high or low copy number of RIOK1 gene relative to other cell lines from the COSMIC Cell Line Gene CNV Profiles dataset.
	COSMIC Cell Line Gene Mutation Profiles	cell lines with RIOK1 gene mutations from the COSMIC Cell Line Gene Mutation Profiles dataset.
	CTD Gene-Disease Associations	diseases associated with RIOK1 gene/protein from the curated CTD Gene-Disease Associations dataset.
	DISEASES Experimental Gene-Disease Association Evidence Scores 2025	diseases associated with RIOK1 gene in GWAS datasets from the DISEASES Experimental Gene-Disease Assocation Evidence Scores 2025 dataset.
	DISEASES Text-mining Gene-Disease Association Evidence Scores	diseases co-occuring with RIOK1 gene in abstracts of biomedical publications from the DISEASES Text-mining Gene-Disease Assocation Evidence Scores dataset.
	DISEASES Text-mining Gene-Disease Association Evidence Scores 2025	diseases co-occuring with RIOK1 gene in abstracts of biomedical publications from the DISEASES Text-mining Gene-Disease Assocation Evidence Scores 2025 dataset.
	DisGeNET Gene-Disease Associations	diseases associated with RIOK1 gene in GWAS and other genetic association datasets from the DisGeNET Gene-Disease Associations dataset.
	DisGeNET Gene-Phenotype Associations	phenotypes associated with RIOK1 gene in GWAS and other genetic association datasets from the DisGeNET Gene-Phenoptype Associations dataset.
	ENCODE Histone Modification Site Profiles	histone modification site profiles with high histone modification abundance at RIOK1 gene from the ENCODE Histone Modification Site Profiles dataset.
	ENCODE Transcription Factor Binding Site Profiles	transcription factor binding site profiles with transcription factor binding evidence at the promoter of RIOK1 gene from the ENCODE Transcription Factor Binding Site Profiles dataset.
	ENCODE Transcription Factor Targets	transcription factors binding the promoter of RIOK1 gene in ChIP-seq datasets from the ENCODE Transcription Factor Targets dataset.
	ESCAPE Omics Signatures of Genes and Proteins for Stem Cells	PubMedIDs of publications reporting gene signatures containing RIOK1 from the ESCAPE Omics Signatures of Genes and Proteins for Stem Cells dataset.
	GAD High Level Gene-Disease Associations	diseases associated with RIOK1 gene in GWAS and other genetic association datasets from the GAD High Level Gene-Disease Associations dataset.
	GeneRIF Biological Term Annotations	biological terms co-occuring with RIOK1 gene in literature-supported statements describing functions of genes from the GeneRIF Biological Term Annotations dataset.
	GeneSigDB Published Gene Signatures	PubMedIDs of publications reporting gene signatures containing RIOK1 from the GeneSigDB Published Gene Signatures dataset.
	GEO Signatures of Differentially Expressed Genes for Diseases	disease perturbations changing expression of RIOK1 gene from the GEO Signatures of Differentially Expressed Genes for Diseases dataset.
	GEO Signatures of Differentially Expressed Genes for Gene Perturbations	gene perturbations changing expression of RIOK1 gene from the GEO Signatures of Differentially Expressed Genes for Gene Perturbations dataset.