PEX14 Gene

Name	peroxisomal biogenesis factor 14
Description	This gene encodes an essential component of the peroxisomal import machinery. The protein is integrated into peroxisome membranes with its C-terminus exposed to the cytosol, and interacts with the cytosolic receptor for proteins containing a PTS1 peroxisomal targeting signal. The protein also functions as a transcriptional corepressor and interacts with a histone deacetylase. A mutation in this gene results in one form of Zellweger syndrome. [provided by RefSeq, Jul 2008]
Summary	{"type": "root", "children": [{"type": "p", "children": [{"type": "t", "text": "\nPEX14 is a central peroxisomal membrane protein that functions as the primary docking site for receptors such as PEX5, orchestrating the import of peroxisomal matrix proteins. Structural and kinetic studies show that its conserved N‐terminal domain binds to amphipathic, diaromatic motifs in PEX5 and PEX19, thereby facilitating the docking of receptor–cargo complexes at the peroxisomal membrane. In addition, PEX14 interactions help mediate cargo release by triggering conformational changes in the receptor—in one study, the N‑terminal domain of PEX14 disrupted a stable PEX5–catalase complex to promote cargo protein release. Low‐resolution structural models further support a scenario in which an extended PEX5 conformation intermingles with PEX14 to form a dynamic and ‘fuzzy’ docking/translocation machinery."}, {"type": "fg", "children": [{"type": "fg_fs", "start_ref": "1", "end_ref": "5"}]}, {"type": "t", "text": "\n"}]}, {"type": "t", "text": "\n\n"}, {"type": "p", "children": [{"type": "t", "text": "\nBeyond its role in protein import, PEX14 also contributes to the spatial organization and motility of peroxisomes. Purification studies have revealed that PEX14 forms multimolecular assemblies at the peroxisomal membrane that include nearly all peroxins necessary for matrix protein import. Remarkably, its conserved N‑terminal domain binds directly to microtubules by interacting with motifs in the C‑terminal region of β‑tubulin, and this microtubule tethering is modulated by competitive binding with PEX5. High‐resolution microscopy further documented that PEX14 is distributed in distinct subdomains in actively importing peroxisomes, underscoring its dual function both as a docking factor and as an anchor for peroxisome motility."}, {"type": "fg", "children": [{"type": "fg_fs", "start_ref": "6", "end_ref": "8"}]}, {"type": "t", "text": "\n"}]}, {"type": "t", "text": "\n\n"}, {"type": "p", "children": [{"type": "t", "text": "\nRegulation of PEX14 is integral not only to peroxisomal function but also to human health. Phosphorylation of PEX14—for example, at Ser‑232 during mitosis—modulates its conformation, leading to a selective reduction in catalase import and thereby contributing to cell cycle–regulated peroxisomal dynamics. Mutations in PEX14 have been directly associated with peroxisome biogenesis disorders such as Zellweger syndrome, as evidenced by patient fibroblast studies and genetic analyses. In an unexpected twist, PEX14 (also known as NAPP2) has been found to serve as a transcriptional corepressor by interacting with factors such as NF‑E2 and histone deacetylase, revealing a bifunctional role that links peroxisomal protein import with nuclear gene regulation. Moreover, mislocalization of PEX14 (for instance, to mitochondria in PEX3‑deficient cells or upon overexpression) underscores the importance of tightly regulated PEX14 levels for maintaining organellar integrity."}, {"type": "fg", "children": [{"type": "fg_fs", "start_ref": "9", "end_ref": "15"}]}, {"type": "t", "text": "\n"}]}, {"type": "rg", "children": [{"type": "r", "ref": 1, "children": [{"type": "t", "text": "Christian Neufeld, Fabian V Filipp, Bernd Simon, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Structural basis for competitive interactions of Pex14 with the import receptors Pex5 and Pex19."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "EMBO J (2009)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1038/emboj.2009.7"}], "href": "https://doi.org/10.1038/emboj.2009.7"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "19197237"}], "href": "https://pubmed.ncbi.nlm.nih.gov/19197237"}]}, {"type": "r", "ref": 2, "children": [{"type": "t", "text": "Kumiko Shiozawa, Petr V Konarev, Christian Neufeld, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Solution structure of human Pex5.Pex14.PTS1 protein complexes obtained by small angle X-ray scattering."}]}, {"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.M109.002311"}], "href": "https://doi.org/10.1074/jbc.M109.002311"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "19584060"}], "href": "https://pubmed.ncbi.nlm.nih.gov/19584060"}]}, {"type": "r", "ref": 3, "children": [{"type": "t", "text": "Marta O Freitas, Tânia Francisco, Tony A Rodrigues, et al. "}, {"type": "b", "children": [{"type": "t", "text": "PEX5 protein binds monomeric catalase blocking its tetramerization and releases it upon binding the N-terminal domain of PEX14."}]}, {"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.M111.287201"}], "href": "https://doi.org/10.1074/jbc.M111.287201"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "21976670"}], "href": "https://pubmed.ncbi.nlm.nih.gov/21976670"}]}, {"type": "r", "ref": 4, "children": [{"type": "t", "text": "Alexander Neuhaus, Hamed Kooshapur, Janina Wolf, et al. "}, {"type": "b", "children": [{"type": "t", "text": "A novel Pex14 protein-interacting site of human Pex5 is critical for matrix protein import into peroxisomes."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Biol Chem (2014)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1074/jbc.M113.499707"}], "href": "https://doi.org/10.1074/jbc.M113.499707"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "24235149"}], "href": "https://pubmed.ncbi.nlm.nih.gov/24235149"}]}, {"type": "r", "ref": 5, "children": [{"type": "t", "text": "Ana F Dias, Tony A Rodrigues, Ana G Pedrosa, et al. "}, {"type": "b", "children": [{"type": "t", "text": "The peroxisomal matrix protein translocon is a large cavity-forming protein assembly into which PEX5 protein enters to release its cargo."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Biol Chem (2017)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1074/jbc.M117.805044"}], "href": "https://doi.org/10.1074/jbc.M117.805044"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "28765278"}], "href": "https://pubmed.ncbi.nlm.nih.gov/28765278"}]}, {"type": "r", "ref": 6, "children": [{"type": "t", "text": "Pratima Bharti, Wolfgang Schliebs, Tanja Schievelbusch, et al. "}, {"type": "b", "children": [{"type": "t", "text": "PEX14 is required for microtubule-based peroxisome motility in human cells."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Cell Sci (2011)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1242/jcs.079368"}], "href": "https://doi.org/10.1242/jcs.079368"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "21525035"}], "href": "https://pubmed.ncbi.nlm.nih.gov/21525035"}]}, {"type": "r", "ref": 7, "children": [{"type": "t", "text": "Silvia Galiani, Dominic Waithe, Katharina Reglinski, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Super-resolution Microscopy Reveals Compartmentalization of Peroxisomal Membrane Proteins."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Biol Chem (2016)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1074/jbc.M116.734038"}], "href": "https://doi.org/10.1074/jbc.M116.734038"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "27311714"}], "href": "https://pubmed.ncbi.nlm.nih.gov/27311714"}]}, {"type": "r", "ref": 8, "children": [{"type": "t", "text": "Maren Reuter, Hamed Kooshapur, Jeff-Gordian Suda, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Competitive Microtubule Binding of PEX14 Coordinates Peroxisomal Protein Import and Motility."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Mol Biol (2021)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.jmb.2020.166765"}], "href": "https://doi.org/10.1016/j.jmb.2020.166765"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "33484719"}], "href": "https://pubmed.ncbi.nlm.nih.gov/33484719"}]}, {"type": "r", "ref": 9, "children": [{"type": "t", "text": "Narender R Gavva, Shau-Ching Wen, Pratibha Daftari, et al. "}, {"type": "b", "children": [{"type": "t", "text": "NAPP2, a peroxisomal membrane protein, is also a transcriptional corepressor."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Genomics (2002)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1006/geno.2002.6714"}], "href": "https://doi.org/10.1006/geno.2002.6714"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "11863372"}], "href": "https://pubmed.ncbi.nlm.nih.gov/11863372"}]}, {"type": "r", "ref": 10, "children": [{"type": "t", "text": "Marc Fransen, Ilse Vastiau, Chantal Brees, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Potential role for Pex19p in assembly of PTS-receptor docking complexes."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Biol Chem (2004)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1074/jbc.M304941200"}], "href": "https://doi.org/10.1074/jbc.M304941200"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "14715663"}], "href": "https://pubmed.ncbi.nlm.nih.gov/14715663"}]}, {"type": "r", "ref": 11, "children": [{"type": "t", "text": "Nobuyuki Shimozawa, Toshiro Tsukamoto, Tomoko Nagase, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Identification of a new complementation group of the peroxisome biogenesis disorders and PEX14 as the mutated gene."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Hum Mutat (2004)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1002/humu.20032"}], "href": "https://doi.org/10.1002/humu.20032"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "15146459"}], "href": "https://pubmed.ncbi.nlm.nih.gov/15146459"}]}, {"type": "r", "ref": 12, "children": [{"type": "t", "text": "Shoko Komatsuzaki, Eishin Ogawa, Nobuyuki Shimozawa, et al. "}, {"type": "b", "children": [{"type": "t", "text": "First Japanese case of Zellweger syndrome with a mutation in PEX14."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Pediatr Int (2015)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1111/ped.12713"}], "href": "https://doi.org/10.1111/ped.12713"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "26627464"}], "href": "https://pubmed.ncbi.nlm.nih.gov/26627464"}]}, {"type": "r", "ref": 13, "children": [{"type": "t", "text": "Koichiro Yamashita, Shigehiko Tamura, Masanori Honsho, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Mitotic phosphorylation of Pex14p regulates peroxisomal import machinery."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Cell Biol (2020)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1083/jcb.202001003"}], "href": "https://doi.org/10.1083/jcb.202001003"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "32854114"}], "href": "https://pubmed.ncbi.nlm.nih.gov/32854114"}]}, {"type": "r", "ref": 14, "children": [{"type": "t", "text": "Hans R Waterham, Janet Koster, Merel S Ebberink, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Autosomal dominant Zellweger spectrum disorder caused by de novo variants in PEX14 gene."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Genet Med (2023)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.gim.2023.100944"}], "href": "https://doi.org/10.1016/j.gim.2023.100944"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "37493040"}], "href": "https://pubmed.ncbi.nlm.nih.gov/37493040"}]}, {"type": "r", "ref": 15, "children": [{"type": "t", "text": "Renate L M Jansen, Rinse de Boer, Eline M F de Lange, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Overexpression of PEX14 results in mistargeting to mitochondria, accompanied by organelle fragmentation and clustering in human embryonic kidney cells."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Biochim Biophys Acta Mol Cell Res (2024)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.bbamcr.2024.119754"}], "href": "https://doi.org/10.1016/j.bbamcr.2024.119754"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "38762172"}], "href": "https://pubmed.ncbi.nlm.nih.gov/38762172"}]}]}]}
Synonyms	NAPP2, PBD13A, PEX14P, DJ734G22.2
Proteins	PEX14_HUMAN
NCBI Gene ID	5195
API
Download Associations
Predicted Functions
Co-expressed Genes
Expression in Tissues and Cell Lines

Functional Associations

PEX14 has 6,768 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 140 datasets.

Click the + buttons to view associations for PEX14 from the datasets below.

If available, associations are ranked by standardized value

Harmonizome 3.0

PEX14 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 PEX14 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 PEX14 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 PEX14 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 PEX14 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 PEX14 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 PEX14 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 PEX14 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 PEX14 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 PEX14 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 PEX14 gene from the Carcinogenome Chemical Perturbation Carcinogenicity Signatures dataset.
	CCLE Cell Line Gene CNV Profiles	cell lines with high or low copy number of PEX14 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 PEX14 gene relative to other cell lines from the CCLE Cell Line Gene Expression Profiles dataset.
	CCLE Cell Line Proteomics	Cell lines associated with PEX14 protein from the CCLE Cell Line Proteomics dataset.
	CellMarker Gene-Cell Type Associations	cell types associated with PEX14 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 PEX14 gene from the CHEA Transcription Factor Binding Site Profiles dataset.
	ChEA Transcription Factor Targets	transcription factors binding the promoter of PEX14 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 PEX14 gene in low- or high-throughput transcription factor functional studies from the CHEA Transcription Factor Targets 2022 dataset.
	ClinVar Gene-Phenotype Associations	phenotypes associated with PEX14 gene from the curated ClinVar Gene-Phenotype Associations dataset.
	ClinVar Gene-Phenotype Associations 2025	phenotypes associated with PEX14 gene from the curated ClinVar Gene-Phenotype Associations 2025 dataset.
	CM4AI KOLF21J CRISPRi Gene Perturbation Atlas	gene perturbations changing expression of PEX14 gene from the CM4AI KOLF21J CRISPRi Gene Perturbation Atlas dataset.
	CM4AI U2OS Cell Map Protein Localization Assemblies	assemblies containing PEX14 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 PEX14 gene from the CMAP Signatures of Differentially Expressed Genes for Small Molecules dataset.
	COMPARTMENTS Curated Protein Localization Evidence Scores	cellular components containing PEX14 protein from the COMPARTMENTS Curated Protein Localization Evidence Scores dataset.
	COMPARTMENTS Curated Protein Localization Evidence Scores 2025	cellular components containing PEX14 protein from the COMPARTMENTS Curated Protein Localization Evidence Scores 2025 dataset.
	COMPARTMENTS Experimental Protein Localization Evidence Scores	cellular components containing PEX14 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 PEX14 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 PEX14 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 PEX14 protein in abstracts of biomedical publications from the COMPARTMENTS Text-mining Protein Localization Evidence Scores 2025 dataset.
	CORUM Protein Complexes	protein complexs containing PEX14 protein from the CORUM Protein Complexes dataset.
	COSMIC Cell Line Gene CNV Profiles	cell lines with high or low copy number of PEX14 gene relative to other cell lines from the COSMIC Cell Line Gene CNV Profiles dataset.
	COSMIC Cell Line Gene Mutation Profiles	cell lines with PEX14 gene mutations from the COSMIC Cell Line Gene Mutation Profiles dataset.
	CTD Gene-Chemical Interactions	chemicals interacting with PEX14 gene/protein from the curated CTD Gene-Chemical Interactions dataset.
	CTD Gene-Disease Associations	diseases associated with PEX14 gene/protein from the curated CTD Gene-Disease Associations dataset.
	dbGAP Gene-Trait Associations	traits associated with PEX14 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 PEX14 protein relative to other small molecule perturbations from the DeepCoverMOA Drug Mechanisms of Action dataset.
	DepMap CRISPR Gene Dependency	cell lines with fitness changed by PEX14 gene knockdown relative to other cell lines from the DepMap CRISPR Gene Dependency dataset.
	DISEASES Curated Gene-Disease Association Evidence Scores	diseases involving PEX14 gene from the DISEASES Curated Gene-Disease Assocation Evidence Scores dataset.
	DISEASES Curated Gene-Disease Association Evidence Scores 2025	diseases involving PEX14 gene from the DISEASES Curated Gene-Disease Association Evidence Scores 2025 dataset.
	DISEASES Experimental Gene-Disease Association Evidence Scores	diseases associated with PEX14 gene in GWAS datasets from the DISEASES Experimental Gene-Disease Assocation Evidence Scores dataset.
	DISEASES Experimental Gene-Disease Association Evidence Scores 2025	diseases associated with PEX14 gene in GWAS datasets from the DISEASES Experimental Gene-Disease Assocation Evidence Scores 2025 dataset.