ARIH1 Gene

Name	ariadne RBR E3 ubiquitin protein ligase 1
Description	Enables enzyme binding activity; ubiquitin-protein transferase activity; and zinc ion binding activity. Involved in protein ubiquitination. Located in several cellular components, including Lewy body; cullin-RING ubiquitin ligase complex; and nuclear body. [provided by Alliance of Genome Resources, Mar 2025]
Summary	{"type": "root", "children": [{"type": "p", "children": [{"type": "t", "text": "\n ARIH1 (also known as HHARI) is an RBR‐type E3 ubiquitin ligase that employs a RING/HECT hybrid mechanism to transfer ubiquitin onto substrates."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "1"}]}, {"type": "t", "text": " In cancer cells, ARIH1 has been shown to polyubiquitinate damaged mitochondria in a PINK1‐dependent manner, thereby triggering mitophagy and contributing to therapeutic resistance in tumors such as breast and lung adenocarcinomas."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "2"}]}, {"type": "t", "text": " Moreover, mutations in ARIH1 have been identified in chronic myelomonocytic leukemia, emphasizing its importance in maintaining hematopoietic cell homeostasis."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "3"}]}, {"type": "t", "text": ""}]}, {"type": "t", "text": "\n \n "}, {"type": "p", "children": [{"type": "t", "text": "\n ARIH1 activity can be stimulated through its association with neddylated Cullin–RING ligase complexes, which markedly enhances its ligase function."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "4"}]}, {"type": "t", "text": " In addition, ARIH1 plays a critical role in nuclear positioning by mono‐ubiquitinating components of the LINC complex – a function that, in Drosophila, can be functionally complemented by human ARIH1. Disruption of this process has been linked to smooth muscle cell dysfunction and vascular abnormalities, such as thoracic aortic aneurysms"}, {"type": "fg", "children": [{"type": "fg_f", "ref": "5"}]}, {"type": "t", "text": ";."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "6"}]}, {"type": "t", "text": ""}]}, {"type": "t", "text": "\n \n "}, {"type": "p", "children": [{"type": "t", "text": "\n In the context of protein ubiquitylation networks, ARIH1 has also been implicated in substrate-specific polyubiquitylation together with other E2 enzymes within SCF complexes, highlighting its versatility in regulating cellular protein turnover."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "7"}]}, {"type": "t", "text": " Its localization to both the nucleus—often within nuclear bodies—and the cytoplasm, as well as its impact on cell proliferation and survival, further underscore a broader role in cell cycle regulation that is particularly relevant to proliferative diseases."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "8"}]}, {"type": "t", "text": ""}]}, {"type": "rg", "children": [{"type": "r", "ref": 1, "children": [{"type": "t", "text": "Dawn M Wenzel, Alexei Lissounov, Peter S Brzovic, et al. "}, {"type": "b", "children": [{"type": "t", "text": "UBCH7 reactivity profile reveals parkin and HHARI to be RING/HECT hybrids."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Nature (2011)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1038/nature09966"}], "href": "https://doi.org/10.1038/nature09966"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "21532592"}], "href": "https://pubmed.ncbi.nlm.nih.gov/21532592"}]}, {"type": "r", "ref": 2, "children": [{"type": "t", "text": "Elodie Villa, Emma Proïcs, Camila Rubio-Patiño, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Parkin-Independent Mitophagy Controls Chemotherapeutic Response in Cancer Cells."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Cell Rep (2017)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.celrep.2017.08.087"}], "href": "https://doi.org/10.1016/j.celrep.2017.08.087"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "28930681"}], "href": "https://pubmed.ncbi.nlm.nih.gov/28930681"}]}, {"type": "r", "ref": 3, "children": [{"type": "t", "text": "C C Mason, J S Khorashad, S K Tantravahi, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Age-related mutations and chronic myelomonocytic leukemia."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Leukemia (2016)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1038/leu.2015.337"}], "href": "https://doi.org/10.1038/leu.2015.337"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "26648538"}], "href": "https://pubmed.ncbi.nlm.nih.gov/26648538"}]}, {"type": "r", "ref": 4, "children": [{"type": "t", "text": "Ian R Kelsall, David M Duda, Jennifer L Olszewski, et al. "}, {"type": "b", "children": [{"type": "t", "text": "TRIAD1 and HHARI bind to and are activated by distinct neddylated Cullin-RING ligase complexes."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "EMBO J (2013)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1038/emboj.2013.209"}], "href": "https://doi.org/10.1038/emboj.2013.209"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "24076655"}], "href": "https://pubmed.ncbi.nlm.nih.gov/24076655"}]}, {"type": "r", "ref": 5, "children": [{"type": "t", "text": "Kai Li Tan, Nele A Haelterman, Callie S Kwartler, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Ari-1 Regulates Myonuclear Organization Together with Parkin and Is Associated with Aortic Aneurysms."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Dev Cell (2018)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.devcel.2018.03.020"}], "href": "https://doi.org/10.1016/j.devcel.2018.03.020"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "29689197"}], "href": "https://pubmed.ncbi.nlm.nih.gov/29689197"}]}, {"type": "r", "ref": 6, "children": [{"type": "t", "text": "Sangram S Parelkar, Juan G Cadena, Chul Kim, et al. "}, {"type": "b", "children": [{"type": "t", "text": "The parkin-like human homolog of Drosophila ariadne-1 (HHARI) can induce aggresome formation in mammalian cells and is immunologically detectable in Lewy bodies."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Mol Neurosci (2012)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1007/s12031-011-9535-1"}], "href": "https://doi.org/10.1007/s12031-011-9535-1"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "21590270"}], "href": "https://pubmed.ncbi.nlm.nih.gov/21590270"}]}, {"type": "r", "ref": 7, "children": [{"type": "t", "text": "Spencer Hill, Kurt Reichermeier, Daniel C Scott, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Robust cullin-RING ligase function is established by a multiplicity of poly-ubiquitylation pathways."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Elife (2019)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.7554/eLife.51163"}], "href": "https://doi.org/10.7554/eLife.51163"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "31868589"}], "href": "https://pubmed.ncbi.nlm.nih.gov/31868589"}]}, {"type": "r", "ref": 8, "children": [{"type": "t", "text": "Fatima Elmehdawi, Gabrielle Wheway, Katarzyna Szymanska, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Human Homolog of Drosophila Ariadne (HHARI) is a marker of cellular proliferation associated with nuclear bodies."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Exp Cell Res (2013)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.yexcr.2012.10.002"}], "href": "https://doi.org/10.1016/j.yexcr.2012.10.002"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "23059369"}], "href": "https://pubmed.ncbi.nlm.nih.gov/23059369"}]}]}]}
Synonyms	ARI, HARI, HHARI, UBCH7BP
Proteins	ARI1_HUMAN
NCBI Gene ID	25820
API
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Predicted Functions
Co-expressed Genes
Expression in Tissues and Cell Lines

Functional Associations

ARIH1 has 9,885 functional associations with biological entities spanning 8 categories (molecular profile, organism, disease, phenotype or trait, functional term, phrase or reference, chemical, structural feature, cell line, cell type or tissue, gene, protein or microRNA) extracted from 113 datasets.

Click the + buttons to view associations for ARIH1 from the datasets below.

If available, associations are ranked by standardized value

Harmonizome 3.0

ARIH1 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 ARIH1 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 ARIH1 gene relative to other tissues from the Allen Brain Atlas Adult Human 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 ARIH1 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 ARIH1 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 ARIH1 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 ARIH1 gene relative to other tissues from the Allen Brain Atlas Prenatal Human Brain Tissue Gene Expression Profiles dataset.
	BioGPS Cell Line Gene Expression Profiles	cell lines with high or low expression of ARIH1 gene relative to other cell lines from the BioGPS Cell Line Gene Expression Profiles dataset.
	BioGPS Human Cell Type and Tissue Gene Expression Profiles	cell types and tissues with high or low expression of ARIH1 gene relative to other cell types and tissues from the BioGPS Human Cell Type and Tissue Gene Expression Profiles dataset.
	CCLE Cell Line Gene CNV Profiles	cell lines with high or low copy number of ARIH1 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 ARIH1 gene relative to other cell lines from the CCLE Cell Line Gene Expression Profiles dataset.
	CCLE Cell Line Proteomics	Cell lines associated with ARIH1 protein from the CCLE Cell Line Proteomics dataset.
	CellMarker Gene-Cell Type Associations	cell types associated with ARIH1 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 ARIH1 gene from the CHEA Transcription Factor Binding Site Profiles dataset.
	ChEA Transcription Factor Targets	transcription factors binding the promoter of ARIH1 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 ARIH1 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 ARIH1 gene from the curated ClinVar Gene-Phenotype Associations 2025 dataset.
	CM4AI U2OS Cell Map Protein Localization Assemblies	assemblies containing ARIH1 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 ARIH1 protein from the COMPARTMENTS Curated Protein Localization Evidence Scores dataset.
	COMPARTMENTS Curated Protein Localization Evidence Scores 2025	cellular components containing ARIH1 protein from the COMPARTMENTS Curated Protein Localization Evidence Scores 2025 dataset.
	COMPARTMENTS Text-mining Protein Localization Evidence Scores	cellular components co-occuring with ARIH1 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 ARIH1 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 ARIH1 gene relative to other cell lines from the COSMIC Cell Line Gene CNV Profiles dataset.
	COSMIC Cell Line Gene Mutation Profiles	cell lines with ARIH1 gene mutations from the COSMIC Cell Line Gene Mutation Profiles dataset.
	CTD Gene-Chemical Interactions	chemicals interacting with ARIH1 gene/protein from the curated CTD Gene-Chemical Interactions dataset.
	CTD Gene-Disease Associations	diseases associated with ARIH1 gene/protein from the curated CTD Gene-Disease Associations dataset.
	dbGAP Gene-Trait Associations	traits associated with ARIH1 gene in GWAS and other genetic association datasets from the dbGAP Gene-Trait Associations dataset.
	DeepCoverMOA Drug Mechanisms of Action	small molecule perturbations with high or low expression of ARIH1 protein relative to other small molecule perturbations from the DeepCoverMOA Drug Mechanisms of Action dataset.
	DISEASES Text-mining Gene-Disease Association Evidence Scores	diseases co-occuring with ARIH1 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 ARIH1 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 ARIH1 gene in GWAS and other genetic association datasets from the DisGeNET Gene-Disease Associations dataset.
	DisGeNET Gene-Phenotype Associations	phenotypes associated with ARIH1 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 ARIH1 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 ARIH1 gene from the ENCODE Transcription Factor Binding Site Profiles dataset.
	ENCODE Transcription Factor Targets	transcription factors binding the promoter of ARIH1 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 ARIH1 from the ESCAPE Omics Signatures of Genes and Proteins for Stem Cells dataset.
	GAD Gene-Disease Associations	diseases associated with ARIH1 gene in GWAS and other genetic association datasets from the GAD Gene-Disease Associations dataset.
	GAD High Level Gene-Disease Associations	diseases associated with ARIH1 gene in GWAS and other genetic association datasets from the GAD High Level Gene-Disease Associations dataset.
	GDSC Cell Line Gene Expression Profiles	cell lines with high or low expression of ARIH1 gene relative to other cell lines from the GDSC Cell Line Gene Expression Profiles dataset.
	GeneRIF Biological Term Annotations	biological terms co-occuring with ARIH1 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 ARIH1 from the GeneSigDB Published Gene Signatures dataset.