TP53RK Gene

Name	TP53 regulating kinase
Description	Enables p53 binding activity and protein serine/threonine kinase activity. Involved in protein phosphorylation. Located in cytoplasm and nucleus. Part of EKC/KEOPS complex. Implicated in Galloway-Mowat syndrome 4. [provided by Alliance of Genome Resources, Mar 2025]
Summary	{"type": "root", "children": [{"type": "p", "children": [{"type": "t", "text": "\nTP53RK, also known as PRPK, is an atypical protein kinase that directly phosphorylates p53 at serine 15, thereby enhancing its transcriptional activity and modulating cell survival and apoptosis. Studies have shown that over‐expressed PRPK localizes in the nucleus to boost p53‐driven transcription, while its activation is finely regulated by binding partners such as the small Ras‐like GTPase Rab1c, and by upstream kinases like Akt, which phosphorylates PRPK at serine 250 (enhancing its function). This intricate regulation links TP53RK to pathways governing DNA damage responses and apoptosis in cancer cells."}, {"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 role in p53 regulation, TP53RK phosphorylates alternative substrates that contribute to malignancy independent of the p53 axis. Notably, in colon cancer, TP53RK phosphorylates survivin (Birc5) at threonine 34, a modification that stabilizes survivin and promotes metastatic progression. Structural studies of the human PRPK–TPRKB complex have provided detailed insight into its active conformation and critical intermolecular interactions, while mutations in TP53RK (along with other KEOPS subunits) have been implicated in Galloway–Mowat syndrome, emphasizing its importance in normal cellular and developmental processes."}, {"type": "fg", "children": [{"type": "fg_fs", "start_ref": "4", "end_ref": "6"}]}, {"type": "t", "text": "\n"}]}, {"type": "t", "text": "\n\n"}, {"type": "p", "children": [{"type": "t", "text": "\nRecent research extends the functional impact of TP53RK to renal pathology, where its upregulation in fibrotic kidneys correlates with disease severity. In chronic kidney diseases, TP53RK is overexpressed in both tubular epithelial cells and fibroblasts, where it phosphorylates Birc5 and promotes its nuclear translocation. This event appears to trigger downstream PI3K/Akt and MAPK signaling pathways, fostering a profibrotic environment. Importantly, pharmacological inhibition of TP53RK (and its downstream effector Birc5) has shown promise in ameliorating kidney fibrosis, suggesting that targeting this kinase could represent a therapeutic strategy in conditions where aberrant TP53RK activity drives pathology."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "7"}]}, {"type": "t", "text": "\n"}]}, {"type": "rg", "children": [{"type": "r", "ref": 1, "children": [{"type": "t", "text": "Yasuhito Abe, Takashi Takeuchi, Yoshinori Imai, et al. "}, {"type": "b", "children": [{"type": "t", "text": "A Small Ras-like protein Ray/Rab1c modulates the p53-regulating activity of PRPK."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Biochem Biophys Res Commun (2006)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.bbrc.2006.03.071"}], "href": "https://doi.org/10.1016/j.bbrc.2006.03.071"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "16600182"}], "href": "https://pubmed.ncbi.nlm.nih.gov/16600182"}]}, {"type": "r", "ref": 2, "children": [{"type": "t", "text": "S Facchin, M Ruzzene, C Peggion, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Phosphorylation and activation of the atypical kinase p53-related protein kinase (PRPK) by Akt/PKB."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Cell Mol Life Sci (2007)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1007/s00018-007-7179-7"}], "href": "https://doi.org/10.1007/s00018-007-7179-7"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "17712528"}], "href": "https://pubmed.ncbi.nlm.nih.gov/17712528"}]}, {"type": "r", "ref": 3, "children": [{"type": "t", "text": "Teru Hideshima, Francesca Cottini, Yoshihisa Nozawa, et al. "}, {"type": "b", "children": [{"type": "t", "text": "p53-related protein kinase confers poor prognosis and represents a novel therapeutic target in multiple myeloma."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Blood (2017)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1182/blood-2016-09-738500"}], "href": "https://doi.org/10.1182/blood-2016-09-738500"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "28082445"}], "href": "https://pubmed.ncbi.nlm.nih.gov/28082445"}]}, {"type": "r", "ref": 4, "children": [{"type": "t", "text": "Tatyana Zykova, Feng Zhu, Lei Wang, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Targeting PRPK Function Blocks Colon Cancer Metastasis."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Mol Cancer Ther (2018)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1158/1535-7163.MCT-17-0628"}], "href": "https://doi.org/10.1158/1535-7163.MCT-17-0628"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "29483219"}], "href": "https://pubmed.ncbi.nlm.nih.gov/29483219"}]}, {"type": "r", "ref": 5, "children": [{"type": "t", "text": "Hye Sun Hyun, Seong Heon Kim, Eujin Park, et al. "}, {"type": "b", "children": [{"type": "t", "text": "A familial case of Galloway-Mowat syndrome due to a novel TP53RK mutation: a case report."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "BMC Med Genet (2018)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1186/s12881-018-0649-y"}], "href": "https://doi.org/10.1186/s12881-018-0649-y"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "30053862"}], "href": "https://pubmed.ncbi.nlm.nih.gov/30053862"}]}, {"type": "r", "ref": 6, "children": [{"type": "t", "text": "Jian Li, Xinli Ma, Surajit Banerjee, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Crystal structure of the human PRPK-TPRKB complex."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Commun Biol (2021)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1038/s42003-021-01683-4"}], "href": "https://doi.org/10.1038/s42003-021-01683-4"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "33547416"}], "href": "https://pubmed.ncbi.nlm.nih.gov/33547416"}]}, {"type": "r", "ref": 7, "children": [{"type": "t", "text": "Mengqiu Wu, Qianqian Jin, Xinyue Xu, et al. "}, {"type": "b", "children": [{"type": "t", "text": "TP53RK Drives the Progression of Chronic Kidney Disease by Phosphorylating Birc5."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Adv Sci (Weinh) (2023)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1002/advs.202301753"}], "href": "https://doi.org/10.1002/advs.202301753"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "37382161"}], "href": "https://pubmed.ncbi.nlm.nih.gov/37382161"}]}]}]}
Synonyms	C20ORF64, GAMOS4, PRPK, NORI-2P, NORI-2, BUD32, DJ101A2
Proteins	PRPK_HUMAN
NCBI Gene ID	112858
API
Download Associations
Predicted Functions
Co-expressed Genes
Expression in Tissues and Cell Lines

Functional Associations

TP53RK has 5,399 functional associations with biological entities spanning 9 categories (molecular profile, organism, chemical, disease, phenotype or trait, functional term, phrase or reference, 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 TP53RK from the datasets below.

If available, associations are ranked by standardized value

Harmonizome 3.0

TP53RK 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 TP53RK 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 TP53RK 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 TP53RK 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 TP53RK 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 TP53RK 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 TP53RK 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 TP53RK 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 TP53RK 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 TP53RK gene relative to other cell types and tissues from the BioGPS Mouse Cell Type and Tissue Gene Expression Profiles dataset.
	Carcinogenome Chemical Perturbation Carcinogenicity Signatures	small molecule perturbations changing expression of TP53RK gene from the Carcinogenome Chemical Perturbation Carcinogenicity Signatures dataset.
	CCLE Cell Line Gene CNV Profiles	cell lines with high or low copy number of TP53RK 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 TP53RK gene relative to other cell lines from the CCLE Cell Line Gene Expression Profiles dataset.
	CCLE Cell Line Gene Mutation Profiles	cell lines with TP53RK gene mutations from the CCLE Cell Line Gene Mutation Profiles dataset.
	CCLE Cell Line Proteomics	Cell lines associated with TP53RK protein from the CCLE Cell Line Proteomics dataset.
	ChEA Transcription Factor Binding Site Profiles	transcription factor binding site profiles with transcription factor binding evidence at the promoter of TP53RK gene from the CHEA Transcription Factor Binding Site Profiles dataset.
	ChEA Transcription Factor Targets	transcription factors binding the promoter of TP53RK 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 TP53RK gene in low- or high-throughput transcription factor functional studies from the CHEA Transcription Factor Targets 2022 dataset.
	ClinVar Gene-Phenotype Associations 2025	phenotypes associated with TP53RK gene from the curated ClinVar Gene-Phenotype Associations 2025 dataset.
	CM4AI KOLF21J CRISPRi Gene Perturbation Atlas	gene perturbations changing expression of TP53RK gene from the CM4AI KOLF21J CRISPRi Gene Perturbation Atlas dataset.
	CM4AI U2OS Cell Map Protein Localization Assemblies	assemblies containing TP53RK protein from integrated AP-MS and IF data from the CM4AI U2OS Cell Map Protein Localization Assemblies dataset.
	COMPARTMENTS Curated Protein Localization Evidence Scores	cellular components containing TP53RK protein from the COMPARTMENTS Curated Protein Localization Evidence Scores dataset.
	COMPARTMENTS Curated Protein Localization Evidence Scores 2025	cellular components containing TP53RK protein from the COMPARTMENTS Curated Protein Localization Evidence Scores 2025 dataset.
	COMPARTMENTS Text-mining Protein Localization Evidence Scores	cellular components co-occuring with TP53RK 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 TP53RK 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 TP53RK gene relative to other cell lines from the COSMIC Cell Line Gene CNV Profiles dataset.
	COSMIC Cell Line Gene Mutation Profiles	cell lines with TP53RK gene mutations from the COSMIC Cell Line Gene Mutation Profiles dataset.
	CTD Gene-Disease Associations	diseases associated with TP53RK gene/protein from the curated CTD Gene-Disease Associations dataset.
	DeepCoverMOA Drug Mechanisms of Action	small molecule perturbations with high or low expression of TP53RK protein relative to other small molecule perturbations from the DeepCoverMOA Drug Mechanisms of Action dataset.
	DISEASES Curated Gene-Disease Association Evidence Scores 2025	diseases involving TP53RK gene from the DISEASES Curated Gene-Disease Association Evidence Scores 2025 dataset.
	DISEASES Experimental Gene-Disease Association Evidence Scores 2025	diseases associated with TP53RK 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 TP53RK 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 TP53RK 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 TP53RK gene in GWAS and other genetic association datasets from the DisGeNET Gene-Disease Associations dataset.
	DisGeNET Gene-Phenotype Associations	phenotypes associated with TP53RK 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 TP53RK 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 TP53RK gene from the ENCODE Transcription Factor Binding Site Profiles dataset.
	ENCODE Transcription Factor Targets	transcription factors binding the promoter of TP53RK 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 TP53RK from the ESCAPE Omics Signatures of Genes and Proteins for Stem Cells dataset.
	GAD Gene-Disease Associations	diseases associated with TP53RK gene in GWAS and other genetic association datasets from the GAD Gene-Disease Associations dataset.
	GeneRIF Biological Term Annotations	biological terms co-occuring with TP53RK gene in literature-supported statements describing functions of genes from the GeneRIF Biological Term Annotations dataset.