NUP133 Gene

HGNC Family	Minor histocompatibility antigens
Name	nucleoporin 133kDa
Description	The nuclear envelope creates distinct nuclear and cytoplasmic compartments in eukaryotic cells. It consists of two concentric membranes perforated by nuclear pores, large protein complexes that form aqueous channels to regulate the flow of macromolecules between the nucleus and the cytoplasm. These complexes are composed of at least 100 different polypeptide subunits, many of which belong to the nucleoporin family. The nucleoporin protein encoded by this gene displays evolutionarily conserved interactions with other nucleoporins. This protein, which localizes to both sides of the nuclear pore complex at interphase, remains associated with the complex during mitosis and is targeted at early stages to the reforming nuclear envelope. This protein also localizes to kinetochores of mitotic cells. [provided by RefSeq, Jul 2008]
Summary	{"type": "root", "children": [{"type": "p", "children": [{"type": "t", "text": "\nNUP133 is a critical architectural element of the nuclear pore complex (NPC) that contributes to its assembly and overall stability. Detailed structural studies have revealed that NUP133 comprises two distinct domains: an NH₂‐terminal seven‐bladed β‐propeller domain whose surface conservation suggests a role in mediating multiple protein–protein interactions, and a COOH‐terminal domain that directly associates with Nup107 to integrate NUP133 into the Nup107–160 subcomplex—an essential module for NPC biogenesis. Furthermore, structural comparisons indicate that these domains share evolutionary origins with other nucleoporins and coatomer elements, underscoring a common and modular design underlying the large NPC scaffold."}, {"type": "fg", "children": [{"type": "fg_fs", "start_ref": "1", "end_ref": "4"}]}, {"type": "t", "text": "\n"}]}, {"type": "t", "text": "\n\n"}, {"type": "p", "children": [{"type": "t", "text": "\nBeyond its role in maintaining NPC architecture, NUP133 is indispensable during cell division. It not only facilitates the proper reassembly of the NPC at the end of mitosis—by promoting the recruitment of key components such as Nup107 and ELYS onto membrane fenestrations—but also contributes to centrosome positioning and spindle assembly during mitosis. These functions highlight the dual role of NUP133 in regulating both interphase nucleocytoplasmic transport and the dynamic remodeling events occurring during cell division."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "5"}]}, {"type": "t", "text": "\n"}]}, {"type": "t", "text": "\n\n"}, {"type": "p", "children": [{"type": "t", "text": "\nMutations in NUP133 have recently emerged as causative factors in human disease, notably in steroid‐resistant nephrotic syndrome (SRNS). In podocytes—the specialized epithelial cells integral to the glomerular filtration barrier—loss or partial loss of NUP133 function leads to disruption of the NPC, altered transcriptomic signatures, and impaired formation of cellular protrusions. Structural investigations of disease-associated variants, including an enlarged mutant form (p.Lys966Asn), further illuminate how defects in NUP133’s interactions within the NPC can contribute to the pathogenesis of these nucleoporopathies."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "7"}]}, {"type": "t", "text": "\n"}]}, {"type": "rg", "children": [{"type": "r", "ref": 1, "children": [{"type": "t", "text": "Ian C Berke, Thomas Boehmer, Günter Blobel, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Structural and functional analysis of Nup133 domains reveals modular building blocks of the nuclear pore complex."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Cell Biol (2004)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1083/jcb.200408109"}], "href": "https://doi.org/10.1083/jcb.200408109"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "15557116"}], "href": "https://pubmed.ncbi.nlm.nih.gov/15557116"}]}, {"type": "r", "ref": 2, "children": [{"type": "t", "text": "Thomas Boehmer, Thomas U Schwartz "}, {"type": "b", "children": [{"type": "t", "text": "Purification, crystallization and preliminary X-ray analysis of a Nup107-Nup133 heterodimeric nucleoporin complex."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Acta Crystallogr Sect F Struct Biol Cryst Commun (2007)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1107/S1744309107040523"}], "href": "https://doi.org/10.1107/S1744309107040523"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "17768364"}], "href": "https://pubmed.ncbi.nlm.nih.gov/17768364"}]}, {"type": "r", "ref": 3, "children": [{"type": "t", "text": "Thomas Boehmer, Sandra Jeudy, Ian C Berke, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Structural and functional studies of Nup107/Nup133 interaction and its implications for the architecture of the nuclear pore complex."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Mol Cell (2008)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.molcel.2008.04.022"}], "href": "https://doi.org/10.1016/j.molcel.2008.04.022"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "18570875"}], "href": "https://pubmed.ncbi.nlm.nih.gov/18570875"}]}, {"type": "r", "ref": 4, "children": [{"type": "t", "text": "James R R Whittle, Thomas U Schwartz "}, {"type": "b", "children": [{"type": "t", "text": "Architectural nucleoporins Nup157/170 and Nup133 are structurally related and descend from a second ancestral element."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Biol Chem (2009)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1074/jbc.M109.023580"}], "href": "https://doi.org/10.1074/jbc.M109.023580"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "19674973"}], "href": "https://pubmed.ncbi.nlm.nih.gov/19674973"}]}, {"type": "r", "ref": 5, "children": [{"type": "t", "text": "Atsushi Fujita, Hiroyasu Tsukaguchi, Eriko Koshimizu, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Homozygous splicing mutation in NUP133 causes Galloway-Mowat syndrome."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Ann Neurol (2018)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1002/ana.25370"}], "href": "https://doi.org/10.1002/ana.25370"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "30427554"}], "href": "https://pubmed.ncbi.nlm.nih.gov/30427554"}]}, {"type": "r", "ref": 6, "children": [{"type": "t", "text": "Şükriye Bilir, Tomoko Kojidani, Chie Mori, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Roles of Nup133, Nup153 and membrane fenestrations in assembly of the nuclear pore complex at the end of mitosis."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Genes Cells (2019)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1111/gtc.12677"}], "href": "https://doi.org/10.1111/gtc.12677"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "30821042"}], "href": "https://pubmed.ncbi.nlm.nih.gov/30821042"}]}, {"type": "r", "ref": 7, "children": [{"type": "t", "text": "Manuel Rogg, Jasmin I Maier, Markus Ehle, et al. "}, {"type": "b", "children": [{"type": "t", "text": "NUP133 Controls Nuclear Pore Assembly, Transcriptome Composition, and Cytoskeleton Regulation in Podocytes."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Cells (2022)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.3390/cells11081259"}], "href": "https://doi.org/10.3390/cells11081259"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "35455939"}], "href": "https://pubmed.ncbi.nlm.nih.gov/35455939"}]}, {"type": "r", "ref": 8, "children": [{"type": "t", "text": "Qin Wang, Rui Gu, Fu-Wei Li, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Steroid-resistant nephrotic syndrome caused by nuclear pore gene NUP133 variation."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Clin Genet (2023)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1111/cge.14339"}], "href": "https://doi.org/10.1111/cge.14339"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "37041680"}], "href": "https://pubmed.ncbi.nlm.nih.gov/37041680"}]}]}]}
Synonyms	HNUP133
Proteins	NU133_HUMAN
NCBI Gene ID	55746
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Predicted Functions
Co-expressed Genes
Expression in Tissues and Cell Lines

Functional Associations

NUP133 has 7,572 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 126 datasets.

Click the + buttons to view associations for NUP133 from the datasets below.

If available, associations are ranked by standardized value

Harmonizome 3.0

NUP133 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 NUP133 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 NUP133 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 NUP133 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 NUP133 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 NUP133 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 NUP133 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 NUP133 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 NUP133 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 NUP133 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 NUP133 gene from the Carcinogenome Chemical Perturbation Carcinogenicity Signatures dataset.
	CCLE Cell Line Gene CNV Profiles	cell lines with high or low copy number of NUP133 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 NUP133 gene relative to other cell lines from the CCLE Cell Line Gene Expression Profiles dataset.
	CCLE Cell Line Gene Mutation Profiles	cell lines with NUP133 gene mutations from the CCLE Cell Line Gene Mutation Profiles dataset.
	CCLE Cell Line Proteomics	Cell lines associated with NUP133 protein from the CCLE Cell Line Proteomics dataset.
	CellMarker Gene-Cell Type Associations	cell types associated with NUP133 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 NUP133 gene from the CHEA Transcription Factor Binding Site Profiles dataset.
	ChEA Transcription Factor Targets	transcription factors binding the promoter of NUP133 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 NUP133 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 NUP133 gene from the curated ClinVar Gene-Phenotype Associations 2025 dataset.
	CM4AI U2OS Cell Map Protein Localization Assemblies	assemblies containing NUP133 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 NUP133 gene from the CMAP Signatures of Differentially Expressed Genes for Small Molecules dataset.
	COMPARTMENTS Curated Protein Localization Evidence Scores	cellular components containing NUP133 protein from the COMPARTMENTS Curated Protein Localization Evidence Scores dataset.
	COMPARTMENTS Curated Protein Localization Evidence Scores 2025	cellular components containing NUP133 protein from the COMPARTMENTS Curated Protein Localization Evidence Scores 2025 dataset.
	COMPARTMENTS Experimental Protein Localization Evidence Scores 2025	cellular components containing NUP133 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 NUP133 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 NUP133 protein in abstracts of biomedical publications from the COMPARTMENTS Text-mining Protein Localization Evidence Scores 2025 dataset.
	CORUM Protein Complexes	protein complexs containing NUP133 protein from the CORUM Protein Complexes dataset.
	COSMIC Cell Line Gene CNV Profiles	cell lines with high or low copy number of NUP133 gene relative to other cell lines from the COSMIC Cell Line Gene CNV Profiles dataset.
	COSMIC Cell Line Gene Mutation Profiles	cell lines with NUP133 gene mutations from the COSMIC Cell Line Gene Mutation Profiles dataset.
	CTD Gene-Chemical Interactions	chemicals interacting with NUP133 gene/protein from the curated CTD Gene-Chemical Interactions dataset.
	CTD Gene-Disease Associations	diseases associated with NUP133 gene/protein from the curated CTD Gene-Disease Associations dataset.
	DISEASES Curated Gene-Disease Association Evidence Scores 2025	diseases involving NUP133 gene from the DISEASES Curated Gene-Disease Association Evidence Scores 2025 dataset.
	DISEASES Text-mining Gene-Disease Association Evidence Scores	diseases co-occuring with NUP133 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 NUP133 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 NUP133 gene in GWAS and other genetic association datasets from the DisGeNET Gene-Disease Associations dataset.
	DisGeNET Gene-Phenotype Associations	phenotypes associated with NUP133 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 NUP133 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 NUP133 gene from the ENCODE Transcription Factor Binding Site Profiles dataset.
	ENCODE Transcription Factor Targets	transcription factors binding the promoter of NUP133 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 NUP133 from the ESCAPE Omics Signatures of Genes and Proteins for Stem Cells dataset.