Affinage

VPS33B

Vacuolar protein sorting-associated protein 33B · UniProt Q9H267

Length
617 aa
Mass
70.6 kDa
Annotated
2026-04-28
53 papers in source corpus 26 papers cited in narrative 26 extracted findings

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

VPS33B is a Sec1/Munc18-family protein that functions as a master regulator of SNARE-dependent membrane fusion at endosomal compartments, orchestrating the trafficking of diverse cargoes including platelet alpha-granule proteins, ABC transporters, epidermal lamellar body contents, collagen-modifying enzyme LH3, and pattern recognition receptor-containing endosomes (PMID:15052268, PMID:16123220, PMID:28082148, PMID:27496733). VPS33B forms an obligate ~315 kDa complex with VIPAS39 (VPS16B) at 2:3 stoichiometry—distinct from CORVET/HOPS tethering complexes—that localizes to recycling endosomes and late endosomes, where it engages Rab11A, Rab25, SEC22B, and the SNARE Syntaxin 12, with transient handoff to the CCC complex (CCDC22) coordinating endosomal entry and exit (PMID:29778605, PMID:37062417, PMID:34905616, PMID:28017832). Beyond vesicle trafficking, VPS33B directly binds integrin β subunits to potentiate outside-in signaling through RhoA-ROCK and Rac1 pathways, and promotes late endosome–lysosome fusion to clear LYNUS complex-bearing endosomes, thereby suppressing mTORC1 activation (PMID:26399659, PMID:35705052). Loss-of-function mutations in VPS33B cause arthrogryposis–renal dysfunction–cholestasis (ARC) syndrome, a multisystem disorder reflecting the broad requirement for VPS33B-dependent trafficking in hepatocytes, megakaryocytes, keratinocytes, and other cell types (PMID:15052268, PMID:29409756).

Mechanistic history

Synthesis pass · year-by-year structured walk · 14 steps
  1. 2004 High

    Positional cloning of VPS33B as the ARC syndrome gene established that a human Sec1/Munc18 homolog is essential for multiorgan vesicle trafficking, resolving the molecular basis of this Mendelian disorder.

    Evidence Positional cloning and mutation analysis across 14 ARC kindreds

    PMID:15052268

    Open questions at the time
    • No binding partners or specific trafficking step identified
    • Sec1-like domain function not biochemically characterized
  2. 2005 High

    Demonstration that VPS33B localizes to alpha-granule compartments and late endosomes in megakaryocytes, and that ARC patient platelets lack alpha-granules, defined the first cell-biological function—granule biogenesis rather than secretion.

    Evidence Immunofluorescence, electron microscopy, and functional platelet assays in ARC patient and control megakaryocytes/platelets

    PMID:16123220

    Open questions at the time
    • Mechanism of granule biogenesis role unknown
    • Whether VPS33B acts at a specific endosomal sorting step undefined
  3. 2005 High

    Identification that zebrafish vps33b is transcriptionally regulated by hnf6/vhnf1 and required for biliary development provided the first developmental-genetic context for VPS33B function outside hematopoiesis.

    Evidence Morpholino knockdown, EMSA for promoter binding, epistasis analysis in zebrafish

    PMID:16284120

    Open questions at the time
    • Whether transcriptional regulation is conserved in mammals not tested
    • Downstream trafficking targets in bile duct cells unidentified
  4. 2012 High

    Discovery that VPS33B physically interacts with VPS16B (VIPAS39) and that both colocalize at trans-Golgi network, late endosomes, and alpha-granules revealed VPS33B operates as part of a dedicated heteromeric complex, paralleling but distinct from HOPS/CORVET.

    Evidence Yeast two-hybrid, mass spectrometry, co-immunoprecipitation, and immunofluorescence in megakaryocytes; EGF-stimulation and ubiquitination assays in COS-7 cells

    PMID:22677173 PMID:23002115

    Open questions at the time
    • Stoichiometry and structure of the complex unknown
    • Whether VPS33B-VPS16B complex functions independently of HOPS/CORVET not resolved
  5. 2015 High

    Functional studies in HeLa cells and conditional knockout mice established that VPS33B is required for late endosome–lysosome fusion and that its loss causes perinuclear accumulation of late endosomes, cargo degradation failure, and alpha/delta-granule defects in vivo.

    Evidence siRNA knockdown with BSA-gold cargo tracking in HeLa; tamoxifen-inducible KO mice with ultrastructural and functional platelet assays

    PMID:25947942 PMID:26403612

    Open questions at the time
    • SNARE partner mediating the fusion step not identified
    • Whether VPS33B acts catalytically or as a scaffold at the fusion step unclear
  6. 2015 High

    Direct binding of VPS33B to integrin β subunits and potentiation of αIIbβ3 outside-in signaling upstream of RhoA-ROCK and Rac1 pathways expanded VPS33B function beyond vesicle trafficking to include integrin signal transduction.

    Evidence Direct binding assay, CHO overexpression, megakaryocyte/platelet-specific conditional KO mice with spreading, clot retraction, and thrombosis assays

    PMID:26399659

    Open questions at the time
    • Structural basis of integrin β–VPS33B interaction unknown
    • Whether integrin signaling role is independent of vesicle trafficking not fully resolved
  7. 2016 High

    Studies in Drosophila and mammalian cells demonstrated cargo-selective trafficking: VPS33B is specifically required for maturation of PRR-containing endosomes after microbial recognition, with its loss amplifying inflammatory signaling while non-microbial cargo trafficking remains intact.

    Evidence Drosophila Vps33B mutants and mammalian knockdown, phagocytosis assays, inflammatory mediator quantification

    PMID:27496733

    Open questions at the time
    • How cargo selectivity is achieved mechanistically unknown
    • Whether selectivity is conferred by VPS33B itself or an adaptor unclear
  8. 2016 High

    Mapping of VPS33B interactions to Rab11a, Rab25, SEC22B, and alpha-tubulin, with domain-level resolution and functional validation in megakaryocytes, defined a recycling-endosome-centered interaction network and linked VPS33B to LH3 trafficking and collagen modification.

    Evidence Co-IP, mass spectrometry, pull-down with domain constructs, conditional KO mice, LH3 trafficking rescue, patient collagen analysis

    PMID:27319744 PMID:27797340 PMID:28017832

    Open questions at the time
    • Whether Rab11a/Rab25 binding is direct or via VIPAS39 not fully dissected
    • Structural basis of Sec1-domain interactions with SEC22B undefined
  9. 2017 High

    Liver-specific knockout established that VPS33B–Rab11a interaction is required for apical trafficking of ABC transporters and tight junction maintenance in hepatocytes, directly explaining the cholestasis phenotype in ARC syndrome.

    Evidence Liver-specific conditional KO mice with bile/plasma MS, immunostaining, EM, and AAV gene rescue

    PMID:28082148

    Open questions at the time
    • Specific SNARE or tethering factor mediating apical delivery not identified
    • Whether VPS33B acts at sorting, transport, or fusion at the apical membrane unclear
  10. 2018 High

    BioID proteomics definitively separated VPS33B from the CORVET/HOPS pathway, confirming it forms a distinct small complex with VIPAS39 that transiently contacts the CCC complex (CCDC22), establishing a unique identity for VPS33B-containing machinery.

    Evidence BioID proximity biotinylation, gel filtration chromatography, colocalization analysis in human cells

    PMID:29778605

    Open questions at the time
    • Function of transient CCC interaction unknown
    • Whether other transient interactors exist not exhaustively surveyed
  11. 2019 High

    Reconstitution of recombinant VPS33B–VPS16B heterodimer and CRISPR KO in megakaryocytes pinpointed the recycling endosome as the key compartment where VPS33B acts, with its loss causing alpha-granule cargo to be mis-sorted to lysosomes for degradation.

    Evidence Recombinant protein purification, SEC, CRISPR KO in imMKCLs, cargo pulse-chase, GFP-VPS33B rescue

    PMID:31501156

    Open questions at the time
    • Precise SNARE pairing at recycling endosome not determined
    • Whether VPS33B directly catalyzes SNARE assembly not tested
  12. 2022 High

    Identification of Syntaxin 12 as a VPS33B/VPS16B-binding SNARE, and demonstration that CCDC22 competes with Stx12 for complex binding, provided the first evidence of a hand-off mechanism coordinating CCC-mediated retrieval and SNARE-dependent fusion at endosomes.

    Evidence Co-IP, CRISPR KO of Stx12 and CCDC22 in megakaryocytes, competition binding assays, EM

    PMID:34905616

    Open questions at the time
    • Whether competition is direct or allosteric not resolved
    • Whether this hand-off operates in non-megakaryocyte cell types unknown
  13. 2022 High

    Discovery that VPS33B promotes clearance of LYNUS complex-bearing late endosomes via endolysosomal fusion, thereby suppressing mTORC1 activation, revealed a new signaling-regulatory role with metabolic consequences in Treg cells.

    Evidence Treg-specific conditional KO mice, mTORC1 activity assays, metabolic profiling, Co-IP

    PMID:35705052

    Open questions at the time
    • Whether LYNUS regulation is a general VPS33B function or Treg-specific not tested
    • Direct VPS33B–LYNUS binding interface uncharacterized
  14. 2023 High

    Structural characterization of the VPS33B–VPS16B complex revealed an unexpected 2:3 stoichiometry and bidirectional architecture with VPS33B at each lobe, providing the first low-resolution structural model and showing how ARC mutation L30P disrupts assembly.

    Evidence Recombinant expression in yeast, SEC-MALS, SAXS, negative-stain EM, CD, mutagenesis of ARC variants

    PMID:37062417

    Open questions at the time
    • High-resolution atomic structure not available
    • Functional significance of bidirectional orientation unknown
    • How the 2:3 complex engages SNAREs or Rabs structurally undefined

Open questions

Synthesis pass · forward-looking unresolved questions
  • Key open questions remain: the atomic structure of the VPS33B–VPS16B complex, how VPS33B engages SNAREs to catalyze membrane fusion, the molecular basis of cargo selectivity, and whether the integrin signaling and vesicle trafficking functions are mechanistically coupled.
  • No high-resolution structure of VPS33B–VPS16B complex
  • No reconstituted SNARE-dependent fusion assay with VPS33B
  • Mechanism of cargo-selective trafficking unresolved
  • Relationship between integrin signaling and trafficking roles unclear

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0098772 molecular function regulator activity 5 GO:0060090 molecular adaptor activity 4
Localization
GO:0005768 endosome 7 GO:0005764 lysosome 2 GO:0031410 cytoplasmic vesicle 2 GO:0005794 Golgi apparatus 1
Pathway
R-HSA-5653656 Vesicle-mediated transport 6 R-HSA-109582 Hemostasis 3 R-HSA-162582 Signal Transduction 2 R-HSA-168256 Immune System 2 R-HSA-1852241 Organelle biogenesis and maintenance 2 R-HSA-9612973 Autophagy 1
Partners
CCDC22GDI2ITGB3RAB11ARAB25SEC22BSTX12VIPAS39
Complex memberships
VPS33B–VIPAS39 complex

Evidence

Reading pass · 26 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2004 VPS33B encodes a homolog of class C yeast vacuolar protein sorting gene Vps33 containing a Sec1-like domain, identifying it as a regulator of SNARE-dependent vesicle-to-target membrane fusion. Positional cloning, sequence homology analysis, identification of germline mutations in ARC syndrome patients Nature genetics High 15052268
2005 VPS33B is essential for platelet alpha-granule biogenesis in megakaryocytes but not required for granule secretion; VPS33B colocalizes with alpha-granule markers and late endosomes/lysosomes in megakaryocytes, and is absent from platelets themselves. Immunofluorescence microscopy, immunoblotting of ARC patient platelets and megakaryocytes, electron microscopy, platelet aggregation assays Blood High 16123220
2005 Zebrafish vps33b acts downstream of the hnf6/vhnf1 transcription factor pathway to regulate biliary development; vhnf1 directly binds the vps33b promoter to increase its expression. Morpholino knockdown, epistasis analysis, electrophoretic mobility shift assay (EMSA), overexpression in embryos and mammalian liver cells Development (Cambridge, England) High 16284120
2008 VPS33B deficiency causes defective lamellar granule secretion in keratinocytes; ultrastructural analysis showed lamellar granules entombed in cornified cells rather than being secreted, indicating VPS33B is required for SNARE-mediated vesicle fusion in epidermal differentiation. VPS33B mutation sequencing, ultrastructural electron microscopy of ARC patient skin, mRNA splicing analysis Archives of dermatology Medium 18347289
2012 VPS33B interacts with VPS16B (encoded by C14orf133); both proteins colocalize at the trans-Golgi network, late endosomes, and alpha-granules in megakaryocytes, and VPS16B is required for platelet alpha-granule biogenesis. Yeast two-hybrid screen, mass spectrometry, coimmunoprecipitation, immunofluorescence microscopy, immunoblotting of ARC patient platelets Blood High 23002115
2012 SPE-39 (VIPAS39/VPS16B) undergoes tyrosine phosphorylation and ubiquitination upon EGF stimulation; VPS33B association with SPE-39 inhibits EGF-induced ubiquitination of SPE-39, stabilizing it; SPE-39 and VPS33B have opposing effects on EGF receptor downregulation. EGF stimulation assays, immunoprecipitation, ubiquitination assays, overexpression/knockdown in COS-7 cells FEBS letters Medium 22677173
2013 VPS33B pathogenic mutations alter subcellular localization of VPS33B to VIPAS39/SPE-39-positive endosomes and some mutants fragment VIPAS39-positive endosomes, implicating VPS33B in VIPAS39-dependent endosomal maturation or fusion. Yeast two-hybrid, immunoprecipitation, quantitative fluorescence microscopy Human molecular genetics Medium 23918659
2015 VPS33B depletion in HeLa cells causes accumulation of late endosomes in the perinuclear region, impairs cargo degradation, and blocks delivery of endocytosed BSA-gold from late endosomes to lysosomes, indicating VPS33B is required for late endosomal-lysosomal fusion. siRNA knockdown, fluorescence microscopy, electron microscopy with BSA-gold endocytosis assay Traffic (Copenhagen, Denmark) High 26403612
2015 VPS33B deficiency in a tamoxifen-inducible mouse model causes reduction in platelet alpha-granules, accumulation of large vacuoles in megakaryocytes, reduction in mature type-II multivesicular bodies (MVB II), and a defect in delta-granule secretion and stable aggregate formation under arteriolar shear. Tamoxifen-inducible conditional knockout, ultrastructural and immunoelectron microscopy, platelet aggregation assays, tail-bleeding assay, FeCl3 thrombosis model Blood High 25947942
2015 VPS33B binds directly to the integrin beta subunit; VPS33B overexpression potentiates αIIbβ3 outside-in signaling (but not inside-out signaling); megakaryocyte/platelet-specific VPS33B knockout mice show impaired spreading on fibrinogen, defective clot retraction, and reduced aggregation, placing VPS33B upstream of RhoA-ROCK-MLC and Rac1-dependent pathways. Direct binding assay, CHO cell overexpression, megakaryocyte/platelet-specific conditional knockout mice, platelet spreading, clot retraction, aggregation assays, FeCl3 thrombosis model Circulation High 26399659
2016 VPS33B interacts with the GDI2/RAB11A/RAB27A pathway to regulate trafficking of secretory proteins as exosomes in hematopoietic stem cells; VPS33B co-exists in exosomes with GDI2, VPS16B, FLOT1 and other exosome markers. VPS33B deletion in mouse and human HSCs, exosome purification and characterization, co-immunoprecipitation, rescue experiments with purified exosomes The Journal of clinical investigation High 27797340
2016 Drosophila and mammalian Vps33B are required for maturation of phagosomes and endosomes containing pattern recognition receptors (PRRs) following microbial recognition; loss of Vps33B results in enhanced inflammatory signaling due to failure to clear PRR-containing endosomes, while trafficking of non-microbial cargo is unaffected. Drosophila Vps33B mutants, mammalian cell knockdown, phagocytosis assays, inflammatory mediator quantification, endosomal trafficking assays Immunity High 27496733
2016 VPS33B associates with VIPAS39, alpha-tubulin, and SEC22B; VIPAS39 binds intact VPS33B while alpha-tubulin and SEC22B interact separately with the Sec1-like domains of VPS33B; Vps33b deficiency disrupts redistribution of Vipas39 and Sec22b to proplatelets and interrupts colocalization of Sec22b with Vwf-positive vesicles in megakaryocytes. Coimmunoprecipitation, mass spectrometry, immunoblotting, pull-down assays, conditional knockout mice, immunofluorescence microscopy The Journal of pathology High 27319744
2016 VPS33B interacts with Rab11a and Rab25; the p.Gly131Glu ARC/ARKID-causing mutation reduces coimmunoprecipitation and colocalization with Rab11a and Rab25 and fails to rescue trafficking of the collagen-modifying enzyme LH3, linking VPS33B to LH3 trafficking and collagen lysine modification. Mutagenesis, coimmunoprecipitation, colocalization microscopy, LH3 trafficking rescue assay, urine/fibroblast collagen modification analysis The Journal of investigative dermatology High 28017832
2017 VPS33B interacts with RAB11A at recycling endosomes and is required for trafficking of ABC transporters specifically trafficked via Rab11a-positive recycling endosomes to the hepatocyte apical membrane; Vps33b liver-specific knockout causes mislocalisation of these apical proteins, impaired tight junction integrity, and loss of functional bile secretion. Liver-specific conditional knockout mice, bile/plasma mass spectrometry, immunostaining of apical membrane and tight junction proteins, electron microscopy, adeno-associated virus gene rescue Journal of hepatology High 28082148
2018 VPS33B and VIPAR (VPS16B) are required for epidermal lamellar body biogenesis; Vps33b and Vipar knockout mice show abnormal lamellar body morphology, disrupted cargo localisation, impaired stratum corneum formation, and reduced corneocyte/cornified envelope integrity. Mouse knockouts (Vps33b and Vipar), histology, immunofluorescence, electron microscopy, primary cell isolation Biochimica et biophysica acta. Molecular basis of disease High 29409756
2018 VPS33B does not associate with CORVET or HOPS complex subunits but stably interacts with VIPAR; the VPS33B/VIPAR complex is considerably smaller than CORVET/HOPS; VPS33B transiently interacts with CCDC22, a CCC complex member, which does not stably co-fractionate with VPS33B/VIPAR. BioID proximity biotinylation proteomics, gel filtration of human cell lysates, co-localization analysis Journal of molecular biology High 29778605
2019 VPS33B and VPS16B form a distinct small complex with the same hydrodynamic radius as a recombinant VPS33B-VPS16B heterodimer purified from bacteria; this complex localizes to the recycling endosome in megakaryocytes, which is a key intermediate compartment for alpha-granule biogenesis; VPS33B deficiency causes alpha-granule cargo degradation in lysosomes. CRISPR/Cas9 VPS33B knockout in imMKCLs, recombinant protein purification, size-exclusion chromatography, cargo trafficking pulse-chase mapping, GFP-VPS33B rescue Blood advances High 31501156
2019 The VPS33B missense variant p.Cys576Arg abolishes interaction with VIPAS39 in vitro, demonstrating the functional importance of this region for VPS33B-VIPAS39 complex formation. In vitro binding/coimmunoprecipitation assay with mutant constructs Human mutation Medium 31479177
2020 Loss of p38b MAP kinase reduces enhanced inflammatory responses and prolongs survival of Vps33B-deficient Drosophila; p38 MAPK modulates endosomal trafficking of the PGRP-LC innate immune receptor and phagocytosis of bacteria, placing p38b downstream of Vps33B in the regulation of innate immune endosomal trafficking. Drosophila genetic epistasis (Vps33B mutant x p38b mutant), survival assays, constitutively active/dominant negative p38b expression, endosomal trafficking assays, phagocytosis assays Traffic (Copenhagen, Denmark) Medium 32677257
2022 VPS16B/VPS33B complex physically associates with Syntaxin 12 (Stx12), a SNARE protein mediating vesicle fusion at endosomes; Stx12 deficiency reduces alpha-granule numbers and alpha-granule protein levels in megakaryocytes; VPS16B/VPS33B also binds CCDC22 (CCC complex), and CCDC22 competes with Stx12 for binding to VPS16B/VPS33B, suggesting a hand-off mechanism at endosomes. Co-immunoprecipitation, CRISPR knockout of Stx12 and COMMD3/CCDC22 in megakaryocytes, immunofluorescence, flow cytometry, electron microscopy Blood High 34905616
2022 Vps33B binds with the lysosomal nutrient-sensing complex (LYNUS) and promotes late endosome-lysosome fusion and clearance of LYNUS-containing late endosomes, thereby suppressing mTORC1 activation; Vps33B deficiency in Treg cells leads to disordered endolysosomal fusion, LYNUS accumulation, elevated mTORC1 activation, and hyper-glycolytic metabolism. Treg-specific conditional knockout mice, mTORC1 activity assays, endosomal trafficking assays, metabolic profiling, co-immunoprecipitation Cell reports High 35705052
2022 Stable expression of VPS16B in platelets and megakaryocytes is dependent on VPS33B; loss of VPS33B results in loss of both VPS33B and VPS16B protein expression. Immunoblotting of platelets from ARC patients with VPS33B nonsense variant, protein expression analysis Journal of thrombosis and haemostasis : JTH Medium 35325493
2023 Human VPS33B-VPS16B forms a high molecular weight complex (~315 kDa) with a VPS33B:VPS16B stoichiometry of approximately 2:3; the complex has a two-lobed shape with one VPS33B molecule at each end oriented in opposite directions; truncated VPS16B (aa 143-316) is sufficient for complex formation; the ARC-causing L30P mutation disrupts complex formation while S243F and H344D do not. Yeast expression of recombinant complex, circular dichroism, size-exclusion chromatography-MALS, quantitative immunoblotting, small-angle X-ray scattering, negative-stain electron microscopy, avidin tagging, mutagenesis of ARC variants The Journal of biological chemistry High 37062417
2023 METTL16 methyltransferase induces m6A modification of VPS33B mRNA, impairing VPS33B transcript stability and thereby reducing VPS33B protein levels; this promotes osteosarcoma progression through the PI3K/AKT pathway. m6A profiling, METTL16 knockdown/overexpression, mRNA stability assays, VPS33B knockdown rescue experiments, in vivo tumor growth Journal of cellular physiology Medium 37357526
2026 VPS33B knockout in proximal tubular epithelial cells (RPTEC-TERT1) causes a 'peeling' phenotype with altered cell adhesion and cell-matrix attachment defects, accompanied by transcriptional changes in adhesion-related genes. CRISPR/Cas9 knockout, brightfield imaging, immunostaining, RNA sequencing, cell detachment assays PloS one Medium 41686830

Source papers

Stage 0 corpus · 53 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2004 Mutations in VPS33B, encoding a regulator of SNARE-dependent membrane fusion, cause arthrogryposis-renal dysfunction-cholestasis (ARC) syndrome. Nature genetics 243 15052268
2005 Requirement of VPS33B, a member of the Sec1/Munc18 protein family, in megakaryocyte and platelet alpha-granule biogenesis. Blood 118 16123220
2016 Sorting protein VPS33B regulates exosomal autocrine signaling to mediate hematopoiesis and leukemogenesis. The Journal of clinical investigation 76 27797340
2012 The VPS33B-binding protein VPS16B is required in megakaryocyte and platelet α-granule biogenesis. Blood 65 23002115
2019 VPS33B interacts with NESG1 to modulate EGFR/PI3K/AKT/c-Myc/P53/miR-133a-3p signaling and induce 5-fluorouracil sensitivity in nasopharyngeal carcinoma. Cell death & disease 57 30944308
2005 Zebrafish vps33b, an ortholog of the gene responsible for human arthrogryposis-renal dysfunction-cholestasis syndrome, regulates biliary development downstream of the onecut transcription factor hnf6. Development (Cambridge, England) 50 16284120
2015 VPS33B regulates protein sorting into and maturation of α-granule progenitor organelles in mouse megakaryocytes. Blood 44 25947942
2017 Vps33b is crucial for structural and functional hepatocyte polarity. Journal of hepatology 39 28082148
2008 Defective lamellar granule secretion in arthrogryposis, renal dysfunction, and cholestasis syndrome caused by a mutation in VPS33B. Archives of dermatology 37 18347289
2016 Autosomal Recessive Keratoderma-Ichthyosis-Deafness (ARKID) Syndrome Is Caused by VPS33B Mutations Affecting Rab Protein Interaction and Collagen Modification. The Journal of investigative dermatology 36 28017832
2015 Vps33B is required for delivery of endocytosed cargo to lysosomes. Traffic (Copenhagen, Denmark) 35 26403612
2015 Characterization of a Novel Integrin Binding Protein, VPS33B, Which Is Important for Platelet Activation and In Vivo Thrombosis and Hemostasis. Circulation 32 26399659
2009 Clinical characteristics and VPS33B mutations in patients with ARC syndrome. Journal of pediatric gastroenterology and nutrition 32 19274792
2006 VPS33B mutation with ichthyosis, cholestasis, and renal dysfunction but without arthrogryposis: incomplete ARC syndrome phenotype. The Journal of pediatrics 30 16492441
2018 VPS33B and VIPAR are essential for epidermal lamellar body biogenesis and function. Biochimica et biophysica acta. Molecular basis of disease 29 29409756
2016 Vps33b regulates Vwf-positive vesicular trafficking in megakaryocytes. The Journal of pathology 28 27319744
2016 ARC Syndrome-Linked Vps33B Protein Is Required for Inflammatory Endosomal Maturation and Signal Termination. Immunity 28 27496733
2020 VPS33B modulates c-Myc/p53/miR-192-3p to target CCNB1 suppressing the growth of non-small cell lung cancer. Molecular therapy. Nucleic acids 24 33425490
2013 Vps33b pathogenic mutations preferentially affect VIPAS39/SPE-39-positive endosomes. Human molecular genetics 23 23918659
2018 Proteomic and Biochemical Comparison of the Cellular Interaction Partners of Human VPS33A and VPS33B. Journal of molecular biology 22 29778605
2019 VPS33B negatively modulated by nicotine functions as a tumor suppressor in colorectal cancer. International journal of cancer 21 31125123
2000 Cloning, mapping and expression analysis of VPS33B, the human orthologue of rat Vps33b. Cytogenetics and cell genetics 21 10894945
2022 Syntaxin 12 and COMMD3 are new factors that function with VPS33B in the biogenesis of platelet α-granules. Blood 20 34905616
2019 Mechanism of platelet α-granule biogenesis: study of cargo transport and the VPS33B-VPS16B complex in a model system. Blood advances 20 31501156
2019 Metabolomics and Lipidomics Reveal the Effect of Hepatic Vps33b Deficiency on Bile Acids and Lipids Metabolism. Frontiers in pharmacology 17 30967781
2019 Novel missense mutation in VPS33B is associated with isolated low gamma-glutamyltransferase cholestasis: Attenuated, incomplete phenotype of arthrogryposis, renal dysfunction, and cholestasis syndrome. Human mutation 17 31479177
2023 METTL16 promotes osteosarcoma progression by downregulating VPS33B in an m6 A-dependent manner. Journal of cellular physiology 16 37357526
2022 Vps33B controls Treg cell suppressive function through inhibiting lysosomal nutrient sensing complex-mediated mTORC1 activation. Cell reports 15 35705052
2020 VPS33B suppresses lung adenocarcinoma metastasis and chemoresistance to cisplatin. Genes & diseases 14 33997178
2020 A Novel Mutation of VPS33B Gene Associated with Incomplete Arthrogryposis-Renal Dysfunction-Cholestasis Phenotype. Case reports in genetics 12 33029437
2019 A novel mutation in VPS33B gene causing a milder ARC syndrome phenotype with prolonged survival. JIMD reports 12 31240160
2014 Identification of novel mutations in the VPS33B gene involved in arthrogryposis, renal dysfunction, and cholestasis syndrome. Clinical genetics 12 24917129
2023 The Sec1-Munc18 protein VPS33B forms a uniquely bidirectional complex with VPS16B. The Journal of biological chemistry 11 37062417
2018 Novel VPS33B mutation in a patient with autosomal recessive keratoderma-ichthyosis-deafness syndrome. American journal of medical genetics. Part A 9 30561130
2014 ARC syndrome with high GGT cholestasis caused by VPS33B mutations. World journal of gastroenterology 9 24782640
2021 Hepatic Vps33b deficiency aggravates cholic acid-induced cholestatic liver injury in male mice. Acta pharmacologica Sinica 8 34253877
2014 Two novel VPS33B mutations in a patient with arthrogryposis, renal dysfunction and cholestasis syndrome in mainland China. World journal of gastroenterology 7 24415890
2022 Platelet VPS16B is dependent on VPS33B expression, as determined in two siblings with arthrogryposis, renal dysfunction, and cholestasis syndrome. Journal of thrombosis and haemostasis : JTH 6 35325493
2021 VPS33B interacts with NESG1 to suppress cell growth and cisplatin chemoresistance in ovarian cancer. Cancer science 6 33788346
2019 A Novel VPS33B Variant Identified by Exome Sequencing in a Patient with Arthrogryposis-Renal Dysfunction-Cholestasis Syndrome. Pediatric gastroenterology, hepatology & nutrition 6 31777725
2017 A Novel VPS33B Mutation in a Patient with Arthrogryposis-Renal Dysfunction-Cholestasis Syndrome. Pediatric dermatology 4 28544027
2012 Inhibitory effect of SPE-39 due to tyrosine phosphorylation and ubiquitination on the function of Vps33B in the EGF-stimulated cells. FEBS letters 4 22677173
2017 [Clinical features and VPS33B mutations in a family affected by arthrogryposis, renal dysfunction, and cholestasis syndrome]. Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics 3 29046204
2025 VPS33B-dependent exosomes modulate cellular senescence of mesenchymal stem cells via an autocrine signaling pathway. Experimental gerontology 2 40383211
2022 Two novel mutations in VPS33B gene cause a milder ARC syndrome with prolonged survival in a 12-year-old patient: Case report. Frontiers in pediatrics 2 36568436
2022 Mild Phenotype of Arthrogryposis, Renal Dysfunction, and Cholestasis Syndrome 1 Caused by a Novel VPS33B Variant. Frontiers in genetics 1 35281816
2021 A New Aberration in the VPS33B Gene Leads to Full-Symptom ARCS1. The American journal of case reports 1 34531360
2021 Vps33B in Dendritic Cells Regulates House Dust Mite-Induced Allergic Lung Inflammation. Journal of immunology (Baltimore, Md. : 1950) 1 34732466
2020 Hypersensitivity of Vps33B mutant flies to non-pathogenic infections is dictated by aberrant activation of p38b MAP kinase. Traffic (Copenhagen, Denmark) 1 32677257
2026 A VPS33B CRISPR knockout study: In vitro evidence of an adhesion defect. PloS one 0 41686830
2025 A novel homozygous splice-site variant in VPS33B identified as a cause of bleeding. Journal of thrombosis and haemostasis : JTH 0 41138802
2024 [Rare VPS33B gene mutation combined with GP1BA mutation causes severe decrease in plasma VWF levels: a case report and literature review]. Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi 0 39134495
2022 Two novel mutations in the VPS33B gene in a Chinese patient with arthrogryposis, renal dysfunction and cholestasis syndrome 1: A case report. World journal of clinical cases 0 36338198