PEX11B Gene

Name	peroxisomal biogenesis factor 11 beta
Description	The protein encoded by this gene facilitates peroxisomal proliferation and interacts with PEX19. The encoded protein is found in the peroxisomal membrane. Several transcript variants, some protein-coding and some not protein-coding, have been found for this gene. [provided by RefSeq, Dec 2012]
Summary	{"type": "root", "children": [{"type": "p", "children": [{"type": "t", "text": "\nPEX11B is a peroxisomal membrane protein that plays a pivotal role in modulating peroxisome dynamics, including membrane elongation, tubulation, and subsequent division. Mechanistically, it mediates these events through self‐interaction and homo-/heterodimerization, and by directly engaging with the peroxisomal fission machinery—most notably interacting with adaptor proteins such as FIS1 and the GTPase DRP1—to promote membrane deformation and subsequent fission. Structural analyses have further delineated critical domains, such as an amphipathic helix within its N-terminal region, which is essential for its membrane remodeling activity."}, {"type": "fg", "children": [{"type": "fg_fs", "start_ref": "1", "end_ref": "3"}]}, {"type": "t", "text": ""}]}, {"type": "t", "text": "\n\n"}, {"type": "p", "children": [{"type": "t", "text": "\nIn addition to its core role in peroxisome dynamics, defects in PEX11B have been directly linked to peroxisome biogenesis disorders in humans. Mutations in this gene lead to abnormal peroxisomal morphology—such as enlarged, undivided organelles—and are associated with a wide spectrum of clinical phenotypes ranging from neurological impairments to ocular and dermatological abnormalities. These findings underscore the clinical importance of PEX11B in cellular homeostasis and organismal development."}, {"type": "fg", "children": [{"type": "fg_fs", "start_ref": "4", "end_ref": "7"}]}, {"type": "t", "text": ""}]}, {"type": "t", "text": "\n\n"}, {"type": "p", "children": [{"type": "t", "text": "\nBeyond its established role in peroxisomal morphology, PEX11B also appears to intersect with broader cellular processes. Studies indicate that altered PEX11B function can impact peroxisomal membrane permeability and even influence mitochondrial dynamics, as evidenced by its capacity to induce mitochondrial division when mislocalized. Moreover, disruptions in PEX11B activity have been linked to significant changes in lipid compositions—including alterations in polyunsaturated fatty acid content—as well as to impaired neural differentiation pathways, potentially through interactions with regulatory proteins like SIRT1 and PPARγ. Collectively, these findings highlight PEX11B as a central coordinator of peroxisome maintenance that integrates membrane remodeling with metabolic and developmental signaling."}, {"type": "fg", "children": [{"type": "fg_fs", "start_ref": "8", "end_ref": "11"}]}, {"type": "t", "text": ""}]}, {"type": "rg", "children": [{"type": "r", "ref": 1, "children": [{"type": "t", "text": "Shinta Kobayashi, Atsushi Tanaka, Yukio Fujiki "}, {"type": "b", "children": [{"type": "t", "text": "Fis1, DLP1, and Pex11p coordinately regulate peroxisome morphogenesis."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Exp Cell Res (2007)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.yexcr.2007.02.028"}], "href": "https://doi.org/10.1016/j.yexcr.2007.02.028"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "17408615"}], "href": "https://pubmed.ncbi.nlm.nih.gov/17408615"}]}, {"type": "r", "ref": 2, "children": [{"type": "t", "text": "Johannes Koch, Kornelija Pranjic, Anja Huber, et al. "}, {"type": "b", "children": [{"type": "t", "text": "PEX11 family members are membrane elongation factors that coordinate peroxisome proliferation and maintenance."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Cell Sci (2010)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1242/jcs.064907"}], "href": "https://doi.org/10.1242/jcs.064907"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "20826455"}], "href": "https://pubmed.ncbi.nlm.nih.gov/20826455"}]}, {"type": "r", "ref": 3, "children": [{"type": "t", "text": "Nina A Bonekamp, Sandra Grille, Maria Joao Cardoso, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Self-interaction of human Pex11pβ during peroxisomal growth and division."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "PLoS One (2013)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1371/journal.pone.0053424"}], "href": "https://doi.org/10.1371/journal.pone.0053424"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "23308220"}], "href": "https://pubmed.ncbi.nlm.nih.gov/23308220"}]}, {"type": "r", "ref": 4, "children": [{"type": "t", "text": "Merel S Ebberink, Janet Koster, Gepke Visser, et al. "}, {"type": "b", "children": [{"type": "t", "text": "A novel defect of peroxisome division due to a homozygous non-sense mutation in the PEX11β gene."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Med Genet (2012)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1136/jmedgenet-2012-100778"}], "href": "https://doi.org/10.1136/jmedgenet-2012-100778"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "22581968"}], "href": "https://pubmed.ncbi.nlm.nih.gov/22581968"}]}, {"type": "r", "ref": 5, "children": [{"type": "t", "text": "S Thoms, Jutta Gärtner "}, {"type": "b", "children": [{"type": "t", "text": "First PEX11β patient extends spectrum of peroxisomal biogenesis disorder phenotypes."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Med Genet (2012)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1136/jmedgenet-2012-100899"}], "href": "https://doi.org/10.1136/jmedgenet-2012-100899"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "22581969"}], "href": "https://pubmed.ncbi.nlm.nih.gov/22581969"}]}, {"type": "r", "ref": 6, "children": [{"type": "t", "text": "Somayeh Khoddam, Neda Kamal, Amirmasoud Shiri, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Two siblings with PEX11B-related peroxisome biogenesis disorder."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Eur J Med Genet (2024)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.ejmg.2024.104928"}], "href": "https://doi.org/10.1016/j.ejmg.2024.104928"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "38423277"}], "href": "https://pubmed.ncbi.nlm.nih.gov/38423277"}]}, {"type": "r", "ref": 7, "children": [{"type": "t", "text": "Rachel L Taylor, Mark T Handley, Sarah Waller, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Novel PEX11B Mutations Extend the Peroxisome Biogenesis Disorder 14B Phenotypic Spectrum and Underscore Congenital Cataract as an Early Feature."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Invest Ophthalmol Vis Sci (2017)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1167/iovs.16-21026"}], "href": "https://doi.org/10.1167/iovs.16-21026"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "28129423"}], "href": "https://pubmed.ncbi.nlm.nih.gov/28129423"}]}, {"type": "r", "ref": 8, "children": [{"type": "t", "text": "Celien Lismont, Janet Koster, Sarah Provost, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Deciphering the potential involvement of PXMP2 and PEX11B in hydrogen peroxide permeation across the peroxisomal membrane reveals a role for PEX11B in protein sorting."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Biochim Biophys Acta Biomembr (2019)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.bbamem.2019.05.013"}], "href": "https://doi.org/10.1016/j.bbamem.2019.05.013"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "31129117"}], "href": "https://pubmed.ncbi.nlm.nih.gov/31129117"}]}, {"type": "r", "ref": 9, "children": [{"type": "t", "text": "Tina A Schrader, Ruth E Carmichael, Markus Islinger, et al. "}, {"type": "b", "children": [{"type": "t", "text": "PEX11β and FIS1 cooperate in peroxisome division independently of mitochondrial fission factor."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Cell Sci (2022)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1242/jcs.259924"}], "href": "https://doi.org/10.1242/jcs.259924"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "35678336"}], "href": "https://pubmed.ncbi.nlm.nih.gov/35678336"}]}, {"type": "r", "ref": 10, "children": [{"type": "t", "text": "Yuichi Abe, Ronald J A Wanders, Hans R Waterham, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Genetic defects in peroxisome morphogenesis (Pex11β, dynamin-like protein 1, and nucleoside diphosphate kinase 3) affect docosahexaenoic acid-phospholipid metabolism."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Inherit Metab Dis (2023)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1002/jimd.12582"}], "href": "https://doi.org/10.1002/jimd.12582"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "36522796"}], "href": "https://pubmed.ncbi.nlm.nih.gov/36522796"}]}, {"type": "r", "ref": 11, "children": [{"type": "t", "text": "Maryam Esmaeili, Mohammad Hossein Nasr-Esfahani, Alireza Shoaraye Nejati, et al. "}, {"type": "b", "children": [{"type": "t", "text": "PPARgamma dependent PEX11beta counteracts the suppressive role of SIRT1 on neural differentiation of HESCs."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "PLoS One (2024)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1371/journal.pone.0298274"}], "href": "https://doi.org/10.1371/journal.pone.0298274"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "38753762"}], "href": "https://pubmed.ncbi.nlm.nih.gov/38753762"}]}]}]}
Synonyms	PEX11-BETA, PEX14B
Proteins	PX11B_HUMAN
NCBI Gene ID	8799
API
Download Associations
Predicted Functions
Co-expressed Genes
Expression in Tissues and Cell Lines

Functional Associations

PEX11B has 5,677 functional associations with biological entities spanning 8 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) extracted from 115 datasets.

Click the + buttons to view associations for PEX11B from the datasets below.

If available, associations are ranked by standardized value

Harmonizome 3.0

PEX11B 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 PEX11B 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 PEX11B 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 PEX11B 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 PEX11B 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 PEX11B 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 PEX11B 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 PEX11B 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 PEX11B 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 PEX11B gene relative to other cell types and tissues from the BioGPS Human Cell Type and Tissue Gene Expression Profiles dataset.
	Carcinogenome Chemical Perturbation Carcinogenicity Signatures	small molecule perturbations changing expression of PEX11B gene from the Carcinogenome Chemical Perturbation Carcinogenicity Signatures dataset.
	CCLE Cell Line Gene CNV Profiles	cell lines with high or low copy number of PEX11B 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 PEX11B gene relative to other cell lines from the CCLE Cell Line Gene Expression Profiles dataset.
	CCLE Cell Line Proteomics	Cell lines associated with PEX11B 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 PEX11B gene from the CHEA Transcription Factor Binding Site Profiles dataset.
	ChEA Transcription Factor Targets	transcription factors binding the promoter of PEX11B 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 PEX11B 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 PEX11B gene from the curated ClinVar Gene-Phenotype Associations 2025 dataset.
	CM4AI U2OS Cell Map Protein Localization Assemblies	assemblies containing PEX11B 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 PEX11B gene from the CMAP Signatures of Differentially Expressed Genes for Small Molecules dataset.
	COMPARTMENTS Curated Protein Localization Evidence Scores	cellular components containing PEX11B protein from the COMPARTMENTS Curated Protein Localization Evidence Scores dataset.
	COMPARTMENTS Curated Protein Localization Evidence Scores 2025	cellular components containing PEX11B protein from the COMPARTMENTS Curated Protein Localization Evidence Scores 2025 dataset.
	COMPARTMENTS Text-mining Protein Localization Evidence Scores	cellular components co-occuring with PEX11B 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 PEX11B 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 PEX11B gene relative to other cell lines from the COSMIC Cell Line Gene CNV Profiles dataset.
	COSMIC Cell Line Gene Mutation Profiles	cell lines with PEX11B gene mutations from the COSMIC Cell Line Gene Mutation Profiles dataset.
	CTD Gene-Disease Associations	diseases associated with PEX11B gene/protein from the curated CTD Gene-Disease Associations dataset.
	DeepCoverMOA Drug Mechanisms of Action	small molecule perturbations with high or low expression of PEX11B 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 PEX11B gene knockdown relative to other cell lines from the DepMap CRISPR Gene Dependency dataset.
	DISEASES Curated Gene-Disease Association Evidence Scores	diseases involving PEX11B gene from the DISEASES Curated Gene-Disease Assocation Evidence Scores dataset.
	DISEASES Curated Gene-Disease Association Evidence Scores 2025	diseases involving PEX11B gene from the DISEASES Curated Gene-Disease Association Evidence Scores 2025 dataset.
	DISEASES Text-mining Gene-Disease Association Evidence Scores	diseases co-occuring with PEX11B 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 PEX11B 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 PEX11B gene in GWAS and other genetic association datasets from the DisGeNET Gene-Disease Associations dataset.
	DisGeNET Gene-Phenotype Associations	phenotypes associated with PEX11B 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 PEX11B 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 PEX11B gene from the ENCODE Transcription Factor Binding Site Profiles dataset.
	ENCODE Transcription Factor Targets	transcription factors binding the promoter of PEX11B 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 PEX11B from the ESCAPE Omics Signatures of Genes and Proteins for Stem Cells dataset.
	GAD High Level Gene-Disease Associations	diseases associated with PEX11B 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 PEX11B gene relative to other cell lines from the GDSC Cell Line Gene Expression Profiles dataset.