DZIP1 Gene

HGNC Family	Zinc fingers
Name	DAZ interacting zinc finger protein 1
Description	Enables BBSome binding activity and molecular adaptor activity. Involved in cilium organization; heart development; and protein-containing complex localization to centriolar satellite. Located in cytoplasm; microtubule organizing center; and nucleoplasm. Implicated in mitral valve prolapse and spermatogenic failure 47. [provided by Alliance of Genome Resources, Mar 2025]
Summary	{"type": "root", "children": [{"type": "p", "children": [{"type": "t", "text": "\n DZIP1 (DAZ‐Interacting Protein 1) is a multifunctional protein that has emerged as an important regulator in several cellular processes. Initially identified based on its association with germ cell–specific RNA binding proteins, DZIP1 is predominantly expressed in embryonic stem cells and germ cells and appears to participate in ribonucleoprotein (RNP) complexes that regulate mRNA turnover and translation, including its relocalization to stress granules under oxidative stress conditions."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "1"}]}, {"type": "t", "text": " \n "}]}, {"type": "t", "text": "\n "}, {"type": "p", "children": [{"type": "t", "text": "\n In addition to its role in post‐transcriptional regulation, DZIP1 has also been implicated in the modulation of Hedgehog signaling. It performs a dual function in this pathway by both contributing to ciliogenesis and directly affecting Gli transcription factor activity. DZIP1 localizes to centrioles and basal bodies where it interacts with ciliogenic proteins such as CEP164 and IFT88, which is essential for proper primary cilium formation."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "3"}]}, {"type": "t", "text": " Moreover, DZIP1 is involved in controlling Gli protein turnover: it regulates the phosphorylation status and stability of Gli transcription factors via a cascade involving casein kinase 2 and B56‐containing protein phosphatase 2A, and it contributes to the maintenance of the Spop protein that mediates Gli degradation."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "4"}]}, {"type": "t", "text": " Additional studies in zebrafish have demonstrated that the DZIP1 ortholog shuttles between the cytoplasm and nucleus in a manner correlated with Hedgehog pathway activation."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "6"}]}, {"type": "t", "text": "\n "}]}, {"type": "t", "text": "\n "}, {"type": "p", "children": [{"type": "t", "text": "\n DZIP1’s influence on signaling pathways further extends into oncogenesis and cardiac development. In oral squamous carcinoma, for instance, DZIP1 overexpression promotes cell proliferation, migration, and invasion through an epithelial–mesenchymal transition mechanism that is dependent on GLI1/3 activity."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "8"}]}, {"type": "t", "text": " Conversely, epigenetic studies in breast cancer have identified methylation-associated silencing of DZIP1 as a marker of tumor suppression, suggesting that context‐dependent expression of DZIP1 may have opposite tumorigenic outcomes."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "9"}]}, {"type": "t", "text": " In addition, a mutation in a conserved region of DZIP1 that impairs its interaction with β‐catenin regulators has been linked to familial mitral valve prolapse, indicating a role in the modulation of Wnt/β‐catenin signaling during cardiac development."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "11"}]}, {"type": "t", "text": "\n "}]}, {"type": "t", "text": "\n "}, {"type": "p", "children": [{"type": "t", "text": "\n Finally, alterations in DZIP1 expression have also been connected to reproductive function. For example, decreased levels of DZIP1 have been observed in testicular tissue following exposure to toxic compounds, and such changes are associated with impaired germ cell development."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "12"}]}, {"type": "t", "text": "\n "}]}, {"type": "t", "text": "\n "}, {"type": "p", "children": [{"type": "t", "text": "\n In summary, DZIP1 acts as a molecular hub linking ciliary assembly, Hedgehog signaling, and mRNA regulation. Its context‐dependent activities impact key developmental processes and cellular signaling networks, thereby influencing oncogenic processes, cardiac development, and reproductive physiology (1,2,3,4,5,6,7,8,9,10,11,12).\n "}]}, {"type": "rg", "children": [{"type": "r", "ref": 1, "children": [{"type": "t", "text": "Frederick L Moore, Jadwiga Jaruzelska, David M Dorfman, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Identification of a novel gene, DZIP (DAZ-interacting protein), that encodes a protein that interacts with DAZ (deleted in azoospermia) and is expressed in embryonic stem cells and germ cells."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Genomics (2004)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.ygeno.2003.11.005"}], "href": "https://doi.org/10.1016/j.ygeno.2003.11.005"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "15081113"}], "href": "https://pubmed.ncbi.nlm.nih.gov/15081113"}]}, {"type": "r", "ref": 2, "children": [{"type": "t", "text": "Patrícia Shigunov, Jose Sotelo-Silveira, Marco Augusto Stimamiglio, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Ribonomic analysis of human DZIP1 reveals its involvement in ribonucleoprotein complexes and stress granules."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "BMC Mol Biol (2014)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1186/1471-2199-15-12"}], "href": "https://doi.org/10.1186/1471-2199-15-12"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "24993635"}], "href": "https://pubmed.ncbi.nlm.nih.gov/24993635"}]}, {"type": "r", "ref": 3, "children": [{"type": "t", "text": "Chengbing Wang, Wee-Chuang Low, Aimin Liu, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Centrosomal protein DZIP1 regulates Hedgehog signaling by promoting cytoplasmic retention of transcription factor GLI3 and affecting ciliogenesis."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Biol Chem (2013)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1074/jbc.M113.492066"}], "href": "https://doi.org/10.1074/jbc.M113.492066"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "23955340"}], "href": "https://pubmed.ncbi.nlm.nih.gov/23955340"}]}, {"type": "r", "ref": 4, "children": [{"type": "t", "text": "Zhigang Jin, Wenyan Mei, Stefan Strack, et al. "}, {"type": "b", "children": [{"type": "t", "text": "The antagonistic action of B56-containing protein phosphatase 2As and casein kinase 2 controls the phosphorylation and Gli turnover function of Daz interacting protein 1."}]}, {"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.M111.274761"}], "href": "https://doi.org/10.1074/jbc.M111.274761"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "21878643"}], "href": "https://pubmed.ncbi.nlm.nih.gov/21878643"}]}, {"type": "r", "ref": 5, "children": [{"type": "t", "text": "Tyler Schwend, Zhigang Jin, Kai Jiang, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Stabilization of speckle-type POZ protein (Spop) by Daz interacting protein 1 (Dzip1) is essential for Gli turnover and the proper output of Hedgehog signaling."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Biol Chem (2013)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1074/jbc.M113.512962"}], "href": "https://doi.org/10.1074/jbc.M113.512962"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "24072710"}], "href": "https://pubmed.ncbi.nlm.nih.gov/24072710"}]}, {"type": "r", "ref": 6, "children": [{"type": "t", "text": "Seema Agarwala, Galina V Aglyamova, Amanda K Marma, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Differential susceptibility of midbrain and spinal cord patterning to floor plate defects in the talpid2 mutant."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Dev Biol (2005)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.ydbio.2005.09.034"}], "href": "https://doi.org/10.1016/j.ydbio.2005.09.034"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "16246323"}], "href": "https://pubmed.ncbi.nlm.nih.gov/16246323"}]}, {"type": "r", "ref": 7, "children": [{"type": "t", "text": "Yuriko Katoh, Masaru Katoh "}, {"type": "b", "children": [{"type": "t", "text": "Hedgehog signaling pathway and gastric cancer."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Cancer Biol Ther (2005)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.4161/cbt.4.10.2184"}], "href": "https://doi.org/10.4161/cbt.4.10.2184"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "16258256"}], "href": "https://pubmed.ncbi.nlm.nih.gov/16258256"}]}, {"type": "r", "ref": 8, "children": [{"type": "t", "text": "Wangxiang Yan, Yun Deng, Yuhang Zhang, et al. "}, {"type": "b", "children": [{"type": "t", "text": "DZIP1 Promotes Proliferation, Migration, and Invasion of Oral Squamous Carcinoma Through the GLI1/3 Pathway."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Transl Oncol (2019)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.tranon.2019.07.005"}], "href": "https://doi.org/10.1016/j.tranon.2019.07.005"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "31450126"}], "href": "https://pubmed.ncbi.nlm.nih.gov/31450126"}]}, {"type": "r", "ref": 9, "children": [{"type": "t", "text": "Mizuho Kikuyama, Hideyuki Takeshima, Takayuki Kinoshita, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Development of a novel approach, the epigenome-based outlier approach, to identify tumor-suppressor genes silenced by aberrant DNA methylation."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Cancer Lett (2012)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.canlet.2012.03.016"}], "href": "https://doi.org/10.1016/j.canlet.2012.03.016"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "22433712"}], "href": "https://pubmed.ncbi.nlm.nih.gov/22433712"}]}, {"type": "r", "ref": 10, "children": [{"type": "t", "text": "Ye J Ha, Chan W Kim, Seon A Roh, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Epigenetic regulation of KLHL34 predictive of pathologic response to preoperative chemoradiation therapy in rectal cancer patients."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Int J Radiat Oncol Biol Phys (2015)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.ijrobp.2014.11.013"}], "href": "https://doi.org/10.1016/j.ijrobp.2014.11.013"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "25680604"}], "href": "https://pubmed.ncbi.nlm.nih.gov/25680604"}]}, {"type": "r", "ref": 11, "children": [{"type": "t", "text": "Lilong Guo, Tyler Beck, Diana Fulmer, et al. "}, {"type": "b", "children": [{"type": "t", "text": "DZIP1 regulates mammalian cardiac valve development through a Cby1-β-catenin mechanism."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Dev Dyn (2021)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1002/dvdy.342"}], "href": "https://doi.org/10.1002/dvdy.342"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "33811421"}], "href": "https://pubmed.ncbi.nlm.nih.gov/33811421"}]}, {"type": "r", "ref": 12, "children": [{"type": "t", "text": "Xin-Xian Zhang, Di Huang, Nan-Nan Liu, et al. "}, {"type": "b", "children": [{"type": "t", "text": "[GTW-induced abnormal expressions of testicular reproduction-related genes and intervention with kidney-tonifying Chinese herbs]."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Zhonghua Nan Ke Xue (2012)"}]}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "22741448"}], "href": "https://pubmed.ncbi.nlm.nih.gov/22741448"}]}]}]}
Synonyms	DZIP, DZIPT1
Proteins	DZIP1_HUMAN
NCBI Gene ID	22873
API
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Predicted Functions
Co-expressed Genes
Expression in Tissues and Cell Lines

Functional Associations

DZIP1 has 4,941 functional associations with biological entities spanning 9 categories (molecular profile, organism, chemical, functional term, phrase or reference, disease, phenotype or trait, structural feature, cell line, cell type or tissue, gene, protein or microRNA, sequence feature) extracted from 112 datasets.

Click the + buttons to view associations for DZIP1 from the datasets below.

If available, associations are ranked by standardized value

Harmonizome 3.0

DZIP1 Gene

Functional Associations

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

	Dataset	Summary
	Allen Brain Atlas Adult Human Brain Tissue Gene Expression Profiles	tissues with high or low expression of DZIP1 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 DZIP1 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 DZIP1 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 DZIP1 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 DZIP1 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 DZIP1 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 DZIP1 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 DZIP1 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 DZIP1 gene from the Carcinogenome Chemical Perturbation Carcinogenicity Signatures dataset.
	CCLE Cell Line Gene CNV Profiles	cell lines with high or low copy number of DZIP1 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 DZIP1 gene relative to other cell lines from the CCLE Cell Line Gene Expression Profiles dataset.
	CellMarker Gene-Cell Type Associations	cell types associated with DZIP1 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 DZIP1 gene from the CHEA Transcription Factor Binding Site Profiles dataset.
	ChEA Transcription Factor Targets	transcription factors binding the promoter of DZIP1 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 DZIP1 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 DZIP1 protein from integrated AP-MS and IF data from the CM4AI U2OS Cell Map Protein Localization Assemblies dataset.
	CMAP Signatures of Differentially Expressed Genes for Small Molecules	small molecule perturbations changing expression of DZIP1 gene from the CMAP Signatures of Differentially Expressed Genes for Small Molecules dataset.
	COMPARTMENTS Curated Protein Localization Evidence Scores	cellular components containing DZIP1 protein from the COMPARTMENTS Curated Protein Localization Evidence Scores dataset.
	COMPARTMENTS Curated Protein Localization Evidence Scores 2025	cellular components containing DZIP1 protein from the COMPARTMENTS Curated Protein Localization Evidence Scores 2025 dataset.
	COMPARTMENTS Experimental Protein Localization Evidence Scores 2025	cellular components containing DZIP1 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 DZIP1 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 DZIP1 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 DZIP1 gene relative to other cell lines from the COSMIC Cell Line Gene CNV Profiles dataset.
	COSMIC Cell Line Gene Mutation Profiles	cell lines with DZIP1 gene mutations from the COSMIC Cell Line Gene Mutation Profiles dataset.
	CTD Gene-Chemical Interactions	chemicals interacting with DZIP1 gene/protein from the curated CTD Gene-Chemical Interactions dataset.
	CTD Gene-Disease Associations	diseases associated with DZIP1 gene/protein from the curated CTD Gene-Disease Associations dataset.
	DepMap CRISPR Gene Dependency	cell lines with fitness changed by DZIP1 gene knockdown relative to other cell lines from the DepMap CRISPR Gene Dependency dataset.
	DISEASES Experimental Gene-Disease Association Evidence Scores 2025	diseases associated with DZIP1 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 DZIP1 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 DZIP1 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 DZIP1 gene in GWAS and other genetic association datasets from the DisGeNET Gene-Disease Associations dataset.
	ENCODE Histone Modification Site Profiles	histone modification site profiles with high histone modification abundance at DZIP1 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 DZIP1 gene from the ENCODE Transcription Factor Binding Site Profiles dataset.
	ENCODE Transcription Factor Targets	transcription factors binding the promoter of DZIP1 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 DZIP1 from the ESCAPE Omics Signatures of Genes and Proteins for Stem Cells dataset.
	GDSC Cell Line Gene Expression Profiles	cell lines with high or low expression of DZIP1 gene relative to other cell lines from the GDSC Cell Line Gene Expression Profiles dataset.
	GeneRIF Biological Term Annotations	biological terms co-occuring with DZIP1 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 DZIP1 from the GeneSigDB Published Gene Signatures dataset.
	GEO Signatures of Differentially Expressed Genes for Diseases	disease perturbations changing expression of DZIP1 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 DZIP1 gene from the GEO Signatures of Differentially Expressed Genes for Gene Perturbations dataset.