Affinage

PEX3

Peroxisomal biogenesis factor 3 · UniProt P56589

Length
373 aa
Mass
42.1 kDa
Annotated
2026-04-29
58 papers in source corpus 25 papers cited in narrative 25 extracted findings

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

PEX3 is an integral peroxisomal membrane protein that serves as a central platform for peroxisome membrane biogenesis, organelle quality control, and inter-organelle communication. Its cytosolic helical bundle domain contains a hydrophobic groove that docks PEX19 with nanomolar affinity (engaging PEX19-Phe29), enabling PEX19-dependent import of class I peroxisomal membrane proteins and de novo peroxisome formation; the same groove region is also required for PMP insertion and preperoxisome maturation (PMID:20554521, PMID:15007061, PMID:22624858). PEX3 is co-translationally inserted into the ER via the SRP/Sec61 pathway and traffics to peroxisomes through PEX16-dependent and ATP-dependent budding vesicles, with overlapping binding sites on its cytosolic domain recruiting process-specific effectors—Atg36/Atg30 for pexophagy (where PEX3 actively promotes Hrr25-kinase-mediated phosphorylation and stabilization of these receptors), Inp1 for plasma-membrane tethering, and myosin for organelle inheritance (PMID:26572236, PMID:25002403, PMID:22643220, PMID:32958557, PMID:32970792, PMID:19822674, PMID:40847803). Loss-of-function mutations in human PEX3 cause Zellweger syndrome by abolishing peroxisome membrane assembly (PMID:10871277).

Mechanistic history

Synthesis pass · year-by-year structured walk · 14 steps
  1. 1999 High

    Establishing that PEX3 is an integral peroxisomal membrane protein with a defined topology (N-in/C-out) and identifying its N-terminal targeting signal and physical interaction with PEX19 laid the foundation for understanding its role in peroxisome biogenesis.

    Evidence GFP truncation analysis, immunofluorescence topology mapping, mammalian two-hybrid, and co-immunoprecipitation in human cells

    PMID:10430017

    Open questions at the time
    • No information yet on whether PEX3 is a receptor or merely a cargo of PEX19
    • PEX3 function in biogenesis not yet directly tested
  2. 2000 High

    Linking PEX3 loss-of-function to Zellweger syndrome and demonstrating that PEX3-mediated peroxisome biogenesis is independent of COPI/COPII trafficking defined PEX3 as essential for peroxisome membrane assembly through a non-classical vesicular route.

    Evidence Patient mutation analysis, PEX3 re-expression rescue, brefeldin A and dominant-negative SAR1 insensitivity in human fibroblasts

    PMID:10871277

    Open questions at the time
    • The actual membrane trafficking route for PEX3 remained unknown
    • How PEX3 absence leads to PMP mislocalization to mitochondria was not resolved
  3. 2004 High

    Demonstrating that PEX3 is the docking factor for PEX19 at the peroxisomal membrane—sufficient to dock PEX19 at heterologous organelles and required specifically for class I PMP import—resolved the directionality of the PEX3-PEX19 interaction.

    Evidence Co-immunoprecipitation, heterologous organelle docking assay, transient PEX3 inhibition with PMP import readout, domain mapping in human cells

    PMID:15007061

    Open questions at the time
    • Structural basis of the PEX3-PEX19 interaction unknown
    • Whether PEX3 has additional roles beyond PEX19 docking not explored
  4. 2006 Medium

    Observing that reintroduced Pex3 first localizes to the ER/nuclear envelope before peroxisomes form provided early evidence that the ER contributes membrane material for de novo peroxisome biogenesis.

    Evidence Inducible Pex3-GFP expression with live imaging and fractionation in Hansenula polymorpha

    PMID:16487342

    Open questions at the time
    • No direct demonstration of ER-to-peroxisome vesicular transport
    • Mechanism of Pex3 exit from the ER was undefined
  5. 2009 High

    Discovery that Pex3 directly binds the myosin V motor (Myo2p) to mediate peroxisome inheritance expanded PEX3's role from biogenesis factor to a multi-functional organelle scaffold.

    Evidence Direct interaction assay with myosin tail, genetic deletion/overexpression with inheritance readout in Yarrowia lipolytica

    PMID:19822674

    Open questions at the time
    • Whether Pex3-myosin interaction is conserved in mammals unknown
    • How multiple effector interactions are coordinated on one Pex3 molecule unclear
  6. 2010 High

    The crystal structure of the PEX3 cytosolic domain bound to a PEX19 peptide revealed a novel helical bundle fold with a conserved hydrophobic groove, and mutagenesis pinpointed PEX19-Phe29 as critical, providing the atomic-level mechanism of docking.

    Evidence X-ray crystallography, isothermal titration calorimetry, and site-directed mutagenesis of human proteins

    PMID:20554521

    Open questions at the time
    • Structure of full-length membrane-embedded PEX3 not determined
    • How PEX3 facilitates PMP insertion after docking remained unknown
  7. 2012 High

    Two parallel advances revealed that PEX3 operates as a dual-function scaffold: mutagenesis of surface regions showed the hydrophobic groove is required for PMP insertion distinct from PEX19 docking, while identification of pexophagy-specific Pex3 alleles demonstrated that Pex3 recruits the autophagy receptor Atg36 through a separable surface.

    Evidence Site-directed mutagenesis with PMP import and de novo biogenesis assays in human cells; pexophagy-specific mutant isolation and mitochondrial retargeting in S. cerevisiae

    PMID:22624858 PMID:22643220

    Open questions at the time
    • Structural details of Atg36 binding site on Pex3 not resolved
    • Whether PMP insertion and pexophagy receptor docking are mutually exclusive was unknown
  8. 2014 High

    Three studies collectively established the ER-to-peroxisome trafficking route of PEX3: PEX16 was shown to mediate ER-to-peroxisome transport of PEX3, HDX-MS revealed that PEX19 remains disordered upon PEX3 binding, and overexpressed PEX3 was found to trigger ubiquitin- and NBR1-dependent pexophagy in mammalian cells.

    Evidence ER-redirected PEX3 with PEX16 depletion and live-cell imaging; HDX-MS of purified PEX3-PEX19 complex; PEX3 overexpression with siRNA knockdown of NBR1/p62 in human cells

    PMID:25002403 PMID:25007327 PMID:25062251

    Open questions at the time
    • Identity of the ubiquitinated peroxisomal substrate in mammalian pexophagy not determined
    • Whether HDX-MS disorder of PEX19 reflects the cargo-loaded state unclear
  9. 2015 High

    Reconstitution of PEX3's co-translational insertion into the ER via SRP/Sec61 and demonstration that it exits in ATP-dependent budding vesicles defined the complete biogenetic itinerary of PEX3 from ribosome to peroxisome; concurrently, Pex3 was shown to actively promote Atg30 phosphorylation and Atg11 recruitment in P. pastoris pexophagy.

    Evidence Photocrosslinking and SRP binding assay with ribosome-nascent chains, ATP-dependent budding reconstitution; Atg30 phosphorylation and binding site mapping in P. pastoris

    PMID:25694426 PMID:26572236

    Open questions at the time
    • Whether ER exit vesicles require specific coat proteins remains unresolved
    • Kinase identity for Atg30 phosphorylation not fully confirmed in this study
  10. 2018 High

    Competition between Pex3 and Atg37 for overlapping binding sites on Atg30 was shown to control pexophagic receptor activation via Hrr25 kinase, revealing a regulatory switch on Pex3's cytosolic domain that balances biogenesis and degradation.

    Evidence Binding site mapping, phosphorylation assays, co-immunoprecipitation, and genetic epistasis in P. pastoris

    PMID:29260977

    Open questions at the time
    • Whether a similar competitive mechanism operates in S. cerevisiae or mammals is unknown
    • Structural basis of the Pex3-Atg30-Atg37 switch not resolved
  11. 2020 High

    In vitro reconstitution demonstrated that Pex3 directly promotes Hrr25-mediated phosphorylation of Atg36 and protects Atg36 from proteasomal degradation, while Pex3-Inp1 was established as a bona fide peroxisome–plasma membrane tether via PI(4,5)P2 binding, consolidating PEX3's role as a multi-effector scaffold.

    Evidence Reconstitution with recombinant Pex3/Atg36/Hrr25, proteasome inhibitor experiments in S. cerevisiae; Inp1 domain truncation, PI(4,5)P2 binding assay, and artificial tether rescue in S. cerevisiae

    PMID:32958557 PMID:32970792

    Open questions at the time
    • Structural mechanism by which Pex3 facilitates Hrr25 access to Atg36 is unknown
    • Whether Pex3-Inp1 tethering is regulated remains unexplored
  12. 2023 Medium

    Germ cell-specific Pex3 knockout in mice demonstrated that Pex3-dependent peroxisome biogenesis is essential for spermatid intercellular bridge integrity and male fertility, extending functional relevance beyond Zellweger syndrome.

    Evidence Conditional germ cell-specific Pex3 knockout mice, histology, proteomics

    PMID:38062668

    Open questions at the time
    • Specific peroxisomal metabolites underlying bridge destruction not identified
    • Whether PEX3 has non-peroxisomal roles in germ cells is unclear
  13. 2024 Medium

    Cardiomyocyte-specific PEX3 knockout revealed that PEX3-dependent plasmalogen metabolism activates AKT/GSK3β signaling through ITGB3 membrane localization, linking peroxisome biogenesis to cardiac regenerative repair.

    Evidence Conditional cardiomyocyte PEX3 knockout mice, lipid metabolomics, AKT/GSK3β pathway analysis, myocardial injury model

    PMID:38951640

    Open questions at the time
    • Whether AKT activation is a direct consequence of plasmalogen loss or secondary is unresolved
    • Mechanism of plasmalogen-dependent ITGB3 membrane retention not defined
  14. 2025 Medium

    Structural and competition studies confirmed that PEX19, Atg36/Atg30, and Inp1 bind overlapping sites on PEX3, creating a competitive regulatory network; additionally, PEX3 was shown to participate in constitutive Pex15 recycling and in homotypic peroxisome–peroxisome and peroxisome–lipid droplet contact site formation.

    Evidence Crystal structure of H. polymorpha Pex3-Pex19, AlphaFold2 predictions, overexpression competition assays in yeast; genetic epistasis of Msp1/Pex3/Pex19 for Pex15 recycling; Pex3 overexpression with GST-pulldown in yeast and Drosophila

    PMID:40344504 PMID:40628847 PMID:40847803

    Open questions at the time
    • Quantitative binding affinities for the competing partners not fully determined
    • Physiological relevance of Pex3-mediated peroxisome-lipid droplet contacts unclear
    • Whether constitutive Pex15 recycling occurs in mammals not tested

Open questions

Synthesis pass · forward-looking unresolved questions
  • How PEX3 mechanistically facilitates the membrane insertion step of PMPs after PEX19 docking, and whether the competitive binding of biogenesis versus quality-control effectors is regulated by post-translational modifications or signaling inputs, remain central unresolved questions.
  • No reconstituted PMP insertion assay with defined lipid bilayers exists
  • Post-translational regulation of PEX3 effector switching is unexplored
  • Full-length membrane-embedded PEX3 structure not available

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0060090 molecular adaptor activity 5 GO:0008289 lipid binding 1
Localization
GO:0005783 endoplasmic reticulum 4 GO:0043226 organelle 3 GO:0005886 plasma membrane 1
Pathway
R-HSA-1852241 Organelle biogenesis and maintenance 5 R-HSA-9609507 Protein localization 4 R-HSA-9612973 Autophagy 4 R-HSA-1430728 Metabolism 1
Partners
ATG30ATG36HRR25INP1MYO2NBR1PEX16PEX19
Complex memberships
PEX3-PEX19 import receptor complexPex3-Inp1 tethering complex

Evidence

Reading pass · 25 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2004 PEX3 functions as the docking factor for PEX19 at the peroxisomal membrane: PEX3 interacts specifically with the docking domain of PEX19, is required for PEX19 to dock at peroxisomes, and is sufficient to dock PEX19 at heterologous organelles. PEX3 binds PEX19 via a conserved motif essential for docking activity, and transient inhibition of PEX3 abrogates class I PMP import without affecting class II PMP or matrix protein import. Co-immunoprecipitation, heterologous organelle docking assay, transient inhibition/knockdown with PMP import readout, domain mapping The Journal of cell biology High 15007061
2010 Crystal structure of the cytosolic domain of human PEX3 in complex with a PEX19-derived peptide reveals that PEX3 adopts a novel helical bundle fold with a hydrophobic groove at its membrane-distal end that engages PEX19 with nanomolar affinity. Mutagenesis identifies phenylalanine 29 of PEX19 as critical for this interaction, and key PEX3 residues are highly conserved across species. X-ray crystallography, isothermal titration calorimetry, site-directed mutagenesis The Journal of biological chemistry High 20554521
1999 Human PEX3 is an integral peroxisomal membrane protein with its N-terminus inside the peroxisome and C-terminus facing the cytoplasm. The N-terminal 33 amino acids are necessary and sufficient to direct PEX3 to peroxisomes. PEX3 physically interacts with PEX19 as shown by mammalian two-hybrid assay and co-immunoprecipitation of in vitro translated proteins. Immunofluorescence microscopy with N/C-terminal tags, GFP fusion truncation analysis, mammalian two-hybrid, co-immunoprecipitation of in vitro translated proteins European journal of cell biology High 10430017
2000 Loss-of-function mutations in human PEX3 cause Zellweger syndrome by abrogating peroxisome membrane synthesis, resulting in reduced abundance and/or mislocalization of peroxisomal membrane proteins to mitochondria. PEX3-mediated peroxisome biogenesis is independent of COPI (brefeldin A-insensitive) and COPII (dominant-negative SAR1-insensitive) membrane trafficking pathways. Patient mutation analysis, PEX3 re-expression rescue, brefeldin A treatment, dominant-negative SAR1 expression, immunofluorescence The Journal of cell biology High 10871277
2003 FRET analysis demonstrates that the main intracellular site of PEX3-PEX19 interaction is at the peroxisomal membrane. PEX3 deletion constructs lacking either the N-terminal peroxisomal targeting sequence or the C-terminal PEX19-binding domain (residues 1-140) abolish both peroxisomal localization and PEX19 interaction. FRET (EYFP/ECFP fusion proteins), donor fluorescence photobleaching, PEX3 deletion mutant analysis in PEX3-deficient fibroblasts European journal of cell biology High 12924628
2012 In S. cerevisiae, Pex3 recruits the pexophagy receptor Atg36 to peroxisomes. Pex3 alleles blocked specifically in pexophagy fail to recruit Atg36. When Pex3 is redirected to mitochondria, Atg36 follows and restores mitophagy in atg32 mutants. Atg36 in turn binds Atg8 and adaptor Atg11 to link peroxisomes to the core autophagy machinery. Pex3 mutant isolation (pexophagy-specific alleles), co-localization studies, Pex3 mitochondrial retargeting experiment, genetic epistasis (atg32 mutant rescue) The EMBO journal High 22643220
2012 Mutagenesis of three conserved surface regions of PEX3 (PEX19-binding region, hydrophobic groove, acidic cluster) shows the PEX19-binding region is critical for PEX19 affinity and PEX3 stability. The hydrophobic groove near the base of PEX3 is required for PMP insertion and maturation of preperoxisomes, while the acidic cluster is not functionally relevant. The PEX3-PEX19 interaction has a dual function: PMP import and de novo peroxisome formation. Site-directed mutagenesis, biochemical binding assays, functional complementation assays in peroxisome-deficient cells Traffic (Copenhagen, Denmark) High 22624858
2014 High-level expression of PEX3 in mammalian cells induces pexophagy via a pathway requiring peroxisome ubiquitination and NBR1. Peroxisome targeting of PEX3 is essential for initiating this degradation. SQSTM1/p62 is required only for peroxisome clustering, not degradation. Ubiquitination of PEX3 itself is dispensable; an endogenous peroxisomal protein is the ubiquitination target. PEX3 overexpression, siRNA knockdown of NBR1/p62, autophagy inhibitors, PEX3 lysine/cysteine substitution mutant, immunofluorescence Autophagy High 25007327
2014 PEX16 mediates the peroxisomal trafficking of PEX3 (and PMP34) via the ER in mammalian cells. ER-redirected PEX3 (ssPEX3) is continuously imported into pre-existing peroxisomes, demonstrating that the ER constitutively supplies membrane proteins to peroxisomes. This was shown by depletion and overexpression of PEX16 combined with quantitative time-lapse live-cell fluorescence microscopy. ER signal sequence redirected PEX3, PEX16 siRNA depletion and overexpression, quantitative time-lapse fluorescence microscopy, biochemical fractionation Journal of cell science High 25002403
2015 In Pichia pastoris, Pex3 not only docks pexophagy receptor Atg30 at the peroxisomal membrane but actively promotes Atg30 phosphorylation and Atg11 recruitment. Specific Pex3 residues define the Atg30-binding site, and Pex3 interaction is required for activation of Atg30-dependent pexophagy signaling. Binding site mapping by mutagenesis, pexophagy assays, phosphorylation assays, Atg11 recruitment assays The Journal of biological chemistry High 25694426
2015 Human PEX3 inserts co-translationally into the mammalian ER via the Sec61 translocon. The N-terminal transmembrane segment of ribosome-bound PEX3 is recognized by the signal recognition particle (SRP), targeting the ribosome-nascent chain complex to the translocon. PEX3 then integrates into the ER membrane adjacent to Sec61α and TRAM, and subsequently exits the ER via ATP-dependent budding vesicles. Photocrosslinking, fluorescence spectroscopy, SRP binding assay, ATP-dependent budding assay, ribosome-nascent chain analysis Traffic (Copenhagen, Denmark) High 26572236
2009 In the yeast Yarrowia lipolytica, Pex3p and Pex3Bp function as peroxisomal receptors for class V myosin (Myo2p), directly interacting with the myosin globular tail domain to mediate peroxisome inheritance. Loss of Pex3Bp causes peroxisomes to be preferentially retained in the mother cell; overexpression of either Pex3Bp or Pex3p shifts peroxisomes to the bud. Direct interaction assay (myosin tail binding), genetic deletion and overexpression with peroxisome inheritance readout, fluorescence microscopy The Journal of cell biology High 19822674
2013 The N-terminal 17-amino acid segment of Pex3 contains two signals sufficient for sorting to the peroxisomal ER (pER) subdomain in S. cerevisiae. The Pex3 transmembrane segment is additionally required for subsequent transport from the pER to peroxisomes. The luminal domain mediates intra-ER sorting whereas the transmembrane segment mediates exit to peroxisomes. Pex3-Sec66 chimera analysis, GFP fusion truncations, expression in Drosophila S2R+ cells, fluorescence microscopy Biology open High 23951409
2009 The cytosolic domain of human PEX3 binds membrane lipids: purified recombinant cytosolic domain precipitates with mild detergents and induces flocculation or partial solubilization of liposomes in lipid-binding assays. Recombinant protein purification, detergent precipitation assay, liposome binding and flocculation assay Biochimica et biophysica acta Medium 19715730
2006 In Hansenula polymorpha, reintroduced Pex3-GFP initially localizes to the ER and nuclear envelope before peroxisome formation occurs, and fractionation experiments confirm a small ER/nuclear envelope-associated Pex3 fraction during early peroxisome reassembly, suggesting the ER/nuclear envelope contributes to peroxisome formation de novo. Inducible Pex3-GFP expression, live fluorescence microscopy, cell fractionation FEMS yeast research Medium 16487342
2018 In Pichia pastoris, Pex3 and Atg37 compete for overlapping binding sites in the middle domain of pexophagy receptor Atg30. Pex3 binding to Atg30 negatively regulates Hrr25 kinase-mediated phosphorylation of Atg30, while Atg37 binding positively regulates it. Atg37 localization at the peroxisomal membrane depends on Pex3. The competition between Pex3 and Atg37 for Atg30 controls assembly and activation of the pexophagic receptor protein complex. Binding site mapping, phosphorylation assays, co-immunoprecipitation, fluorescence microscopy, genetic epistasis Autophagy High 29260977
2020 In S. cerevisiae, Pex3 interaction with pexophagy receptor Atg36 is essential for Hrr25 kinase-mediated phosphorylation of Atg36: cells lacking Pex3 or expressing a Pex3 mutant defective in Atg36 binding show abolished Atg36 phosphorylation. In vitro reconstitution shows Pex3 directly promotes Atg36 phosphorylation by Hrr25. Co-immunoprecipitation reveals that Atg36-Hrr25 interaction depends on Pex3. Pex3 binding also protects Atg36 from proteasomal degradation. In vitro reconstitution with recombinant proteins, co-immunoprecipitation, Pex3 mutant analysis, proteasome inhibitor treatment The Journal of biological chemistry High 32958557
2020 In S. cerevisiae, the Pex3-Inp1 complex tethers peroxisomes to the plasma membrane. Inp1 bridges the peroxisomal membrane (via C-terminal Pex3-binding domain) and the plasma membrane (via N-terminal PI(4,5)P2-binding domain). Expression of artificial PM-PER tethers restores peroxisome retention in inp1Δ cells, and Inp1 meets criteria for a bona fide contact site tether. Genetic epistasis, domain truncation analysis, PI(4,5)P2 binding assay, artificial tether complementation, fluorescence microscopy The Journal of cell biology High 32970792
2018 In Hansenula polymorpha, Pex3 accumulates in patches at peroxisome-vacuole contact sites specifically during conditions of strong peroxisome expansion (methanol medium), and overproduction of Pex3 induces peroxisome-vacuole contact sites even under glucose conditions, indicating a direct role for Pex3 in forming this contact site. Electron and fluorescence microscopy, Pex3 overproduction, localization studies under different growth conditions Biochimica et biophysica acta. Molecular cell research Medium 30595161
2014 Hydrogen-deuterium exchange mass spectrometry of the PEX3-PEX19 complex shows PEX19 remains intrinsically disordered upon binding, with three specific regions becoming shielded: the N-terminus, a stretch F64-L74, and the C-terminus. PEX3 becomes more protected in its PEX19-binding groove with minor changes elsewhere. PEX3 stabilizes PEX19 and prevents PEX3 aggregation. Hydrogen-deuterium exchange mass spectrometry (HDX-MS) of purified proteins PloS one Medium 25062251
2025 In S. cerevisiae, the binding sites of Pex19, Atg30, and Inp1 on Pex3 overlap, and overexpression of any one partner causes competitive displacement affecting other Pex3-dependent peroxisomal processes (biogenesis, pexophagy, retention). Crystal structure of H. polymorpha Pex3-Pex19 complex and AlphaFold2 predictions confirm overlapping interaction regions. Overexpression competition assays, peroxisomal process readouts, crystal structure analysis, AlphaFold2 structural prediction The FEBS journal Medium 40847803
2025 High Pex3 levels in S. cerevisiae induce formation of peroxisome clusters surrounded by lipid droplets, mediated by peroxisome-peroxisome and peroxisome-lipid droplet contact sites. This clustering is independent of Pex19, Inp1, and Atg36. The cytosolic domain of Pex3 directly binds peroxisomes, suggesting a role in homotypic contact site formation. The lipid droplet-peroxisome contact sites require triacylglycerol lipase Tgl4. Similar effects are seen upon Pex3 overexpression in Drosophila. Pex3 overexpression, deletion of known Pex3 partners, GST-pulldown of cytosolic domain with peroxisomes, fluorescence microscopy in yeast and Drosophila Scientific reports Medium 40628847
2024 Cardiomyocyte-specific PEX3 knockout in mice impairs redox homeostasis and disrupts myocardial regenerative repair. Lipid metabolomics reveals that PEX3 promotes plasmalogen metabolism, and PEX3-regulated plasmalogens activate the AKT/GSK3β signaling pathway via plasma membrane localization of ITGB3. Cardiomyocyte-specific knockout mice, lipid metabolomics, AKT/GSK3β pathway analysis, ITGB3 plasma membrane localization assay, myocardial injury model Communications biology Medium 38951640
2025 In yeast, newly synthesized Pex15 (a tail-anchored peroxisomal membrane protein) is targeted to peroxisomes primarily via the Pex19- and Pex3-dependent pathway. Mistargeted Pex15 on mitochondria is extracted by Msp1 and re-routed to peroxisomes via Pex19-Pex3. Even endogenous peroxisomal Pex15 is extracted by peroxisomal Msp1 but returns via Pex19-Pex3, demonstrating a constitutive recycling role for the Pex3-Pex19 pathway. Genetic analysis (msp1, pex19, pex3 mutants), fluorescence microscopy, co-immunoprecipitation, in vivo targeting assays The FEBS journal Medium 40344504
2023 Germ cell-specific knockout of Pex3 in mice causes male sterility, with destruction of intercellular bridges between spermatids and formation of multinucleated giant cells. Sertoli cell-specific Pex3 deletion has no effect. Proteomics of Pex3-deleted spermatids reveals defective expression of peroxisomal and spermiogenesis-related proteins. Conditional germ cell-specific and Sertoli cell-specific Pex3 knockout mice, histology, proteomics Journal of biomedical research Medium 38062668

Source papers

Stage 0 corpus · 58 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2012 Pex3-anchored Atg36 tags peroxisomes for degradation in Saccharomyces cerevisiae. The EMBO journal 229 22643220
2004 PEX3 functions as a PEX19 docking factor in the import of class I peroxisomal membrane proteins. The Journal of cell biology 191 15007061
2000 Inhibitors of COPI and COPII do not block PEX3-mediated peroxisome synthesis. The Journal of cell biology 122 10871277
2014 The membrane peroxin PEX3 induces peroxisome-ubiquitination-linked pexophagy. Autophagy 103 25007327
1999 Identification and characterization of the human peroxin PEX3. European journal of cell biology 94 10430017
2000 Defective peroxisome membrane synthesis due to mutations in human PEX3 causes Zellweger syndrome, complementation group G. American journal of human genetics 80 10958759
2014 Preperoxisomal vesicles can form in the absence of Pex3. The Journal of cell biology 64 24590171
2000 PEX3 is the causal gene responsible for peroxisome membrane assembly-defective Zellweger syndrome of complementation group G. American journal of human genetics 62 10968777
2000 Identification of PEX3 as the gene mutated in a Zellweger syndrome patient lacking peroxisomal remnant structures. Human molecular genetics 59 10942428
2014 PEX16 contributes to peroxisome maintenance by constantly trafficking PEX3 via the ER. Journal of cell science 54 25002403
2019 The peroxisome biogenesis factors Pex3 and Pex19: multitasking proteins with disputed functions. FEBS letters 52 30776093
2010 Insights into peroxisome function from the structure of PEX3 in complex with a soluble fragment of PEX19. The Journal of biological chemistry 48 20554521
2018 WIPI1, BAG1, and PEX3 Autophagy-Related Genes Are Relevant Melanoma Markers. Oxidative medicine and cellular longevity 46 30622661
2006 Reassembly of peroxisomes in Hansenula polymorpha pex3 cells on reintroduction of Pex3p involves the nuclear envelope. FEMS yeast research 46 16487342
2015 Peroxisomal Pex3 activates selective autophagy of peroxisomes via interaction with the pexophagy receptor Atg30. The Journal of biological chemistry 42 25694426
2011 Drosophila carrying pex3 or pex16 mutations are models of Zellweger syndrome that reflect its symptoms associated with the absence of peroxisomes. PloS one 39 21826223
2012 The role of conserved PEX3 regions in PEX19-binding and peroxisome biogenesis. Traffic (Copenhagen, Denmark) 38 22624858
2003 The interaction between human PEX3 and PEX19 characterized by fluorescence resonance energy transfer (FRET) analysis. European journal of cell biology 38 12924628
2009 Pex3 peroxisome biogenesis proteins function in peroxisome inheritance as class V myosin receptors. The Journal of cell biology 34 19822674
2013 Intra-ER sorting of the peroxisomal membrane protein Pex3 relies on its luminal domain. Biology open 33 23951409
2018 Pex3 and Atg37 compete to regulate the interaction between the pexophagy receptor, Atg30, and the Hrr25 kinase. Autophagy 30 29260977
2018 Peroxisome development in yeast is associated with the formation of Pex3-dependent peroxisome-vacuole contact sites. Biochimica et biophysica acta. Molecular cell research 29 30595161
2017 Saccharomyces cerevisiae cells lacking Pex3 contain membrane vesicles that harbor a subset of peroxisomal membrane proteins. Biochimica et biophysica acta. Molecular cell research 26 28552664
2015 Human Peroxin PEX3 Is Co-translationally Integrated into the ER and Exits the ER in Budding Vesicles. Traffic (Copenhagen, Denmark) 23 26572236
2009 The cytosolic domain of PEX3, a protein involved in the biogenesis of peroxisomes, binds membrane lipids. Biochimica et biophysica acta 22 19715730
2012 Newly identified milder phenotype of peroxisome biogenesis disorder caused by mutated PEX3 gene. Brain & development 20 23245813
2017 New insights into the distribution, protein abundance and subcellular localisation of the endogenous peroxisomal biogenesis proteins PEX3 and PEX19 in different organs and cell types of the adult mouse. PloS one 18 28817674
2020 The Pex3-Inp1 complex tethers yeast peroxisomes to the plasma membrane. The Journal of cell biology 17 32970792
2019 The early-acting glycosome biogenic protein Pex3 is essential for trypanosome viability. Life science alliance 16 31341002
2019 Evolutionary divergent PEX3 is essential for glycosome biogenesis and survival of trypanosomatid parasites. Biochimica et biophysica acta. Molecular cell research 16 31369765
2020 Pex3 confines pexophagy receptor activity of Atg36 to peroxisomes by regulating Hrr25-mediated phosphorylation and proteasomal degradation. The Journal of biological chemistry 12 32958557
2007 Overproduction of translation elongation factor 1-alpha (eEF1A) suppresses the peroxisome biogenesis defect in a Hansenula polymorpha pex3 mutant via translational read-through. FEMS yeast research 12 17425673
2021 Quantitative Proteomics and Differential Protein Abundance Analysis after the Depletion of PEX3 from Human Cells Identifies Additional Aspects of Protein Targeting to the ER. International journal of molecular sciences 10 34884833
2017 Biochemical and genetic characterization of an unusual mild PEX3-related Zellweger spectrum disorder. Molecular genetics and metabolism 10 28673549
2022 rs9390123 and rs9399451 influence the DNA repair capacity of lung cancer by regulating PEX3 and PHACTR2‑AS1 expression instead of PHACTR2. Oncology reports 9 35059740
2022 The Pex3-mediated peroxisome biogenesis plays a critical role in metabolic biosynthesis, stress response, and pathogenicity in Alternaria alternata. Microbiological research 9 36334316
2021 A Small Molecule Inhibitor of Pex3-Pex19 Interaction Disrupts Glycosome Biogenesis and Causes Lethality in Trypanosoma brucei. Frontiers in cell and developmental biology 9 34350186
2016 Novel PEX3 Gene Mutations Resulting in a Moderate Zellweger Spectrum Disorder. JIMD reports 9 27557811
2024 PEX3 promotes regenerative repair after myocardial injury in mice through facilitating plasma membrane localization of ITGB3. Communications biology 8 38951640
2022 The Effect of a Pex3 Mutation on Hearing and Lipid Content of the Inner Ear. Cells 8 36291074
2014 Association between the intrinsically disordered protein PEX19 and PEX3. PloS one 8 25062251
2021 Novel Trypanocidal Inhibitors that Block Glycosome Biogenesis by Targeting PEX3-PEX19 Interaction. Frontiers in cell and developmental biology 6 34988071
2000 The human PEX3 gene encoding a peroxisomal assembly protein: genomic organization, positional mapping, and mutation analysis in candidate phenotypes. Biochemical and biophysical research communications 6 10679269
2020 Pex3 is involved in the genetic regulation of Nr3c2 expression in the amygdala of mice. Psychiatry research 5 32045820
1988 Purification of fusion proteins expressed by pEX3 and a truncated pEX3 derivative. FEBS letters 5 3136038
2025 Msp1 and Pex19-Pex3 cooperate to achieve correct localization of Pex15 to peroxisomes. The FEBS journal 4 40344504
2025 Pex3 promotes formation of peroxisome-peroxisome and peroxisome-lipid droplet contact sites. Scientific reports 4 40628847
2020 Genome sequencing identifies a rare case of moderate Zellweger spectrum disorder caused by a PEX3 defect: Case report and literature review. Molecular genetics and metabolism reports 4 33101983
2025 Integrative Omics reveals changes in the cellular landscape of peroxisome-deficient pex3 yeast cells. Microbial cell (Graz, Austria) 3 40012703
2024 STED super-resolution microscopy unveils the dynamics of Atg30 on yeast Pex3-labeled peroxisomes. iScience 3 39156652
2022 Hypomorphic mutation of PEX3 with peroxisomal mosaicism reveals the oscillating nature of peroxisome biogenesis coupled with differential metabolic activities. Molecular genetics and metabolism 3 35932552
2025 USF2 regulates the JAK2/STAT3 pathway through PEX3-mediated SLC25A17 upregulation to affect lipid metabolism and promote the progression of lung adenocarcinoma. Toxicology and applied pharmacology 2 40885408
2023 Germ cell-specific deletion of Pex3 reveals essential roles of PEX3-dependent peroxisomes in spermiogenesis. Journal of biomedical research 1 38062668
2026 Identification of a new frameshift homozygous variant of PEX3 gene in a preterm infant with profound global developmental delay and bilateral ptosis: a case report and updated literature review. BMC pediatrics 0 41495707
2025 PEX3 gene knockout influences recombinant xylanase expression by Komagataella phaffii. Synthetic and systems biotechnology 0 40248486
2025 Molecular interactions of Toxoplasma gondii dense granule 23 (GRA23) with host proteins PEX3 and TRAP1. Frontiers in veterinary science 0 40417354
2025 Competition between binding partners of yeast Pex3 affects peroxisome biology. The FEBS journal 0 40847603
2000 Genomic organization, expression analysis, and chromosomal localization of the mouse PEX3 gene encoding a peroxisomal assembly protein. Biological chemistry 0 10839463