Affinage

PSTPIP1

Proline-serine-threonine phosphatase-interacting protein 1 · UniProt O43586

Length
416 aa
Mass
47.6 kDa
Annotated
2026-06-10
53 papers in source corpus 22 papers cited in narrative 22 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 6/6 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

PSTPIP1 is an F-BAR/coiled-coil cytoskeletal adaptor that organizes actin dynamics and inflammatory signaling by scaffolding PEST-type protein tyrosine phosphatases to specific substrates and receptors in hematopoietic cells (PMID:9265651, PMID:11711533). Its coiled-coil region binds PTP-PEST/PTP-HSCF, while its SH3 domain engages proline-rich partners including WASP, CD2, and FasL, and its phosphorylation state acts as a regulatory switch: c-Abl phosphorylates PSTPIP1 (principally at Y344), and PTP-PEST reverses this modification, with phosphorylation disrupting WASP binding (PMID:9488710, PMID:11163214, PMID:11711533). Through these interactions PSTPIP1 couples CD2/CD2AP engagement to WASp/Arp2/3-driven actin polymerization at the immunological synapse and balances podosome versus filopodia formation, acting upstream of WASP to restrain macrophage matrix degradation and, with PTPN6 and SHIP1/2, to control osteoclast podosome disassembly (PMID:12530983, PMID:24421327, PMID:27760174). PSTPIP1 self-assembles via its F-BAR domain into membrane-associated filaments and serves as a cytosolic ligand for pyrin: homotrimeric PSTPIP1 binds the pyrin B-box and unmasks the pyrin PYD, enabling ASC pyroptosome assembly and caspase-1/IL-1β activation (PMID:17964261, PMID:19584923). PSTPIP1 also functions in clathrin-independent endocytosis of L1CAM with endophilin-A3 and is required for macrophage differentiation (PMID:36843549, PMID:40997504). Gain-of-function mutations (A230T, E250Q, E250K, E257K) are hyperphosphorylated and bind pyrin with markedly increased affinity to drive constitutive inflammasome activation, the molecular basis of PAPA and PAMI syndromes, whereas these and other disease residues also disrupt PSTPIP1 binding to PTP-PEST and to LYP/PTPN22 (PMID:11971877, PMID:14595024, PMID:26025129, PMID:35152348).

Mechanistic history

Synthesis pass · year-by-year structured walk · 17 steps
  1. 1997 High

    Establishing that PSTPIP1 is a phosphatase-associated cytoskeletal protein defined its founding biochemical identity and linked it to actin and cell division.

    Evidence Yeast two-hybrid, dominant-negative overexpression and confocal localization identifying PTP-HSCF binding and actin/cleavage-furrow colocalization in COS and S. pombe

    PMID:9265651

    Open questions at the time
    • Y344 phosphorylation site not yet mapped
    • Physiological consequences of cytokinesis effect in mammalian cells unaddressed
  2. 1998 High

    Identifying SH3-mediated binding to WASP and CD2 established PSTPIP1 as an adaptor coupling actin effectors and surface receptors, with phosphorylation as a binding switch.

    Evidence In vitro binding, site-directed phosphomimetic mutagenesis and co-localization for WASP; interaction trap and adhesion assays for CD2

    PMID:9488710 PMID:9857189

    Open questions at the time
    • Kinase responsible for the regulatory phosphorylation not identified here
    • In vivo physiological readouts limited
  3. 2000 High

    Demonstrating that PSTPIP1 bridges c-Abl to PTP-PEST defined a ternary phosphoregulatory module and identified the kinase acting on PSTPIP1.

    Evidence Co-IP, PSTPIP1 mutants and phosphorylation assays in Abl-null and PTP-PEST-deficient cells with PDGF stimulation

    PMID:11163214

    Open questions at the time
    • Direct structural basis of the ternary complex not resolved
    • Quantitative kinetics of dephosphorylation not measured
  4. 2001 High

    Mapping Y344 as the PTP-PEST dephosphorylation site and showing PSTPIP1 scaffolds PTP-PEST onto WASP clarified how phosphatase activity is targeted to actin regulators.

    Evidence Tryptic phosphopeptide mapping, in vitro dephosphorylation and co-expression studies

    PMID:11711533

    Open questions at the time
    • Stoichiometry of scaffold assembly in vivo unclear
  5. 2002 Medium

    Linking PAPA-causing mutations to loss of PTP-PEST binding gave the first disease-mechanism connection for PSTPIP1.

    Evidence Yeast two-hybrid binding of A230T and E250Q mutants plus genetic sequencing in PAPA families

    PMID:11971877

    Open questions at the time
    • Mechanism connecting reduced phosphatase binding to inflammation not yet established
    • Single-method binding assay
  6. 2003 High

    Discovering the PSTPIP1-pyrin interaction and showing disease mutants are hyperphosphorylated with increased pyrin binding reframed PSTPIP1 as an inflammatory regulator, and defining the CD2/CD2AP-WASp synapse axis tied it to T cell actin dynamics.

    Evidence Co-IP from THP-1 cells, domain-deletion mapping, c-Abl phosphorylation assays and patient IL-1β measurement; co-IP and synapse assays in WASp-deficient T cells

    PMID:12530983 PMID:14595024

    Open questions at the time
    • Structural basis of mutant-enhanced pyrin binding unresolved at this stage
    • Direct link from pyrin binding to IL-1β processing mechanism not yet reconstituted
  7. 2005 Medium

    Showing PSTPIP1 sequesters FasL and recruits PTP-PEST extended its adaptor function to death-ligand availability.

    Evidence Co-IP, fluorescence co-localization and cytotoxicity assays

    PMID:16204241

    Open questions at the time
    • Single-lab finding without genetic loss-of-function
    • Physiological context of FasL regulation unconfirmed
  8. 2006 Medium

    Demonstrating that PSTPIP1 scaffolds PTP-PEST to the CD2 signalsome to dampen TCR-proximal signaling defined its negative-regulatory role in T cell activation.

    Evidence Primary T cell transduction with PLCγ1/ERK/p38 phosphorylation and cytokine readouts

    PMID:16670297

    Open questions at the time
    • Direct phosphatase substrates in CD2 signalsome not enumerated
    • Single lab
  9. 2007 High

    Reconstituting pyrin autoinhibition relief by PSTPIP1 ligation provided the mechanistic basis for inflammasome activation and for mutant gain-of-function.

    Evidence In vitro binding, pyroptosome assembly, caspase-1 activation assays and gel filtration for oligomeric state

    PMID:17964261

    Open questions at the time
    • Trigger controlling PSTPIP1-pyrin engagement in normal cells not defined
    • Atomic-resolution structure of the complex absent
  10. 2009 Medium

    Showing F-BAR-driven PSTPIP1 self-assembly into tubulin-dependent filaments and recruitment to ASC specks connected its membrane/cytoskeletal scaffolding to inflammasome aggregation.

    Evidence Deletion-construct transfection, immunofluorescence and ASC-speck co-localization with tubulin disruption

    PMID:19584923

    Open questions at the time
    • Functional role of filaments distinct from speck recruitment unclear
    • Single lab
  11. 2014 High

    Placing PSTPIP1 upstream of WASP in podosome/filopodia balance and showing the R405C SH3 mutant selectively loses WASP binding clarified genotype-to-cytoskeleton mechanism.

    Evidence Endogenous knockdown, R405C overexpression, WASP-inhibition epistasis, F-actin and matrix-degradation assays in macrophages

    PMID:24421327

    Open questions at the time
    • Relationship of cytoskeletal defect to inflammatory phenotype not bridged
  12. 2015 Medium

    Defining the PSTPIP1/PTPN6/SHIP1-2 complex in osteoclasts and PAMI-associated E250K/E257K enhanced pyrin binding broadened the substrate repertoire and the disease-mutation spectrum.

    Evidence Reciprocal co-IP, siRNA depletion and bone-resorption assays; immunoprecipitation and structural modeling of disease mutants; HL-60 leading-edge co-localization

    PMID:26025129 PMID:26179737 PMID:27760174

    Open questions at the time
    • Mechanism of dimer electrostatic change on binding modeled but not crystallographically resolved
    • HL-60 co-localization lacks direct interaction or perturbation evidence
  13. 2018 Medium

    Linking loss-of-function PSTPIP1 mutations to defective immunological synapse F-actin and altered T cell behavior established its requirement for synapse architecture in human T cells.

    Evidence Flow cytometry F-actin, IP, IS-formation and migration assays in Jurkat and primary patient T cells

    PMID:29432774

    Open questions at the time
    • Mechanistic link between F-actin preactivation and differentiation defect incomplete
    • Single lab
  14. 2022 High

    Solving the F-BAR/LYP co-structure revealed a novel proline-recognition mechanism and showed disease residues directly contact LYP, adding a structural basis for pathogenesis.

    Evidence X-ray crystallography of PSTPIP1 F-BAR alone and with LYP/PTPN22 C-terminal segment plus binding assays

    PMID:35152348

    Open questions at the time
    • Cellular consequence of disrupted PSTPIP1-LYP binding not directly tested in this study
  15. 2023 Medium

    Identifying PSTPIP1 as required for clathrin-independent L1CAM endocytosis with endophilin-A3 extended its F-BAR membrane-remodeling function to receptor trafficking.

    Evidence siRNA knockdown, fluorescence microscopy and endocytosis assays

    PMID:36843549

    Open questions at the time
    • Generality across cargoes beyond L1CAM unknown
    • Direct membrane-curvature contribution not isolated
  16. 2025 Medium

    A genome-wide screen placing PSTPIP1 and pyrin in the same macrophage differentiation pathway connected the adaptor's cytoskeletal and inflammatory roles to a developmental program.

    Evidence GeCKO CRISPR knockout screen with KO/KI validation, immunophenotyping, RNA-seq and functional adhesion/migration assays

    PMID:40997504

    Open questions at the time
    • Molecular steps linking PSTPIP1 loss to differentiation block undefined
    • Single lab
  17. 2026 Medium

    Defining a direct PTPN22-PSTPIP1 interaction controlling synapse actin nanoscale organization, and identifying a new pyrin-binding gain-of-function mutation (N236K), refined both the cytoskeletal-regulatory and autoinflammatory arms.

    Evidence Super-resolution DNA-PAINT with PTPN22 perturbation and calcium assays in Jurkat cells; co-IP and inflammasome assay with N236K patient characterization

    PMID:42007463 PMID:42263152

    Open questions at the time
    • N236K finding rests on single co-IP/inflammasome assay in case-report context
    • How PTPN22-PSTPIP1 binding integrates with phosphatase scaffolding not fully resolved

Open questions

Synthesis pass · forward-looking unresolved questions
  • How the phosphorylation switch, F-BAR membrane filaments, and pyrin ligation are coordinated under physiological triggers to toggle PSTPIP1 between actin-regulatory and inflammasome-activating states remains unresolved.
  • No atomic-resolution structure of the PSTPIP1-pyrin complex
  • Endogenous signals controlling PSTPIP1 phosphorylation in vivo undefined
  • Integration of endocytic and scaffolding functions unexplored

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0060090 molecular adaptor activity 4 GO:0008092 cytoskeletal protein binding 3 GO:0098772 molecular function regulator activity 3 GO:0008289 lipid binding 2
Localization
GO:0005829 cytosol 3 GO:0005856 cytoskeleton 3 GO:0005886 plasma membrane 3
Pathway
R-HSA-168256 Immune System 4 R-HSA-1266738 Developmental Biology 1 R-HSA-5357801 Programmed Cell Death 1 R-HSA-5653656 Vesicle-mediated transport 1
Partners
ABL1CD2CD2APMEFVPTPN12PTPN22PTPN6WAS
Complex memberships
ASC pyroptosome (via pyrin)PSTPIP1/PTP-PEST/WASP scaffold complexPSTPIP1/PTPN6/SHIP1-SHIP2 complex

Evidence

Reading pass · 22 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
1997 PSTPIP1 was identified as a substrate and binding partner of PEST-type protein tyrosine phosphatase PTP-HSCF; the interaction is mediated by the coiled-coil region of PSTPIP1 and the proline-rich C-terminus of the phosphatase. PSTPIP1 is tyrosine-phosphorylated endogenously and by v-Src, and dominant-negative PTP-HSCF causes PSTPIP1 hyperphosphorylation. PSTPIP1 colocalizes with cortical actin, lamellipodia, and the cytokinetic cleavage furrow; overexpression induces filopodia and inhibits cytokinesis in S. pombe. Yeast two-hybrid, co-transfection, dominant-negative overexpression, confocal microscopy, phosphorylation assays in COS cells and S. pombe The Journal of cell biology High 9265651
1998 PSTPIP1 SH3 domain directly binds two polyproline-rich regions of WASP; co-expression abolishes WASP-induced actin bundling. Tyrosine phosphorylation of PSTPIP1 (at a site within the SH3 domain poly-proline recognition site) disrupts WASP binding in vitro and co-localization in vivo, establishing phosphorylation as a regulatory switch for this interaction. In vitro binding assays, co-transfection co-localization, site-directed mutagenesis (Y→D/E phosphomimetics), pervanadate treatment The Journal of biological chemistry High 9488710
1998 CD2BP1/PSTPIP1 SH3 domain binds directly to the CD2 cytoplasmic sequence KGPPLPRPRV (aa 300-309); the N-terminal segment augments this interaction. Upon CD2 clustering, CD2BP1 redistributes from cytosol to the surface membrane co-localizing with CD2. CD2-stimulated adhesion is downregulated by CD2BP1, apparently through coupling of PTP-PEST to CD2. Interaction trap cloning, in vitro binding, immunofluorescence co-localization, adhesion assays The EMBO journal High 9857189
2000 PSTPIP1 acts as a scaffold bridging c-Abl kinase to PEST-type PTPs: c-Abl phosphorylates PSTPIP1, and PSTPIP1 brings PTP-PEST to dephosphorylate and negatively regulate c-Abl. c-Abl is hyperphosphorylated in PTP-PEST-deficient cells and PDGF-induced c-Abl activation is prolonged without PTP-PEST, confirming the ternary complex function. Co-immunoprecipitation, overexpression of PSTPIP1 mutants, phosphorylation assays in Abl-null fibroblasts and PTP-PEST-deficient cells, PDGF stimulation Molecular cell High 11163214
2001 PTP-PEST and PSTPIP1 form a complex in vivo via the CTH domain of PTP-PEST and the coiled-coil domain of PSTPIP1; PTP-PEST dephosphorylates PSTPIP1 at tyrosine 344 (the main phosphorylation site mapped by tryptic phosphopeptide mapping). PSTPIP1 serves as a scaffold between PTP-PEST and WASP, enabling PTP-PEST to dephosphorylate WASP and thereby modulate actin remodeling. Co-immunoprecipitation, tryptic phosphopeptide mapping, in vitro dephosphorylation assays, co-expression studies The Journal of biological chemistry High 11711533
2002 PAPA syndrome-causing mutations E250Q and A230T in PSTPIP1/CD2BP1 severely reduce binding to PTP-PEST in yeast two-hybrid assays, establishing that disrupted PSTPIP1-PTP-PEST interaction is the molecular basis of PAPA syndrome. Yeast two-hybrid, gene sequencing in PAPA families Human molecular genetics Medium 11971877
2003 PSTPIP1 interacts with pyrin (the FMF protein) in myeloid cells; the B-box of pyrin and both the SH3 and coiled-coil domains of PSTPIP1 are required for the interaction. PAPA-associated PSTPIP1 mutants (A230T and E250Q) are hyperphosphorylated when co-expressed with c-Abl and show markedly increased pyrin binding, linking PSTPIP1 phosphorylation state to pyrin interaction and IL-1β overproduction. Yeast two-hybrid, co-immunoprecipitation from THP-1 cells, domain-deletion mutants, c-Abl co-expression phosphorylation assays, IL-1β measurement in patient PBLs Proceedings of the National Academy of Sciences of the United States of America High 14595024
2003 PSTPIP1 acts downstream of CD2/CD2AP to link CD2 engagement to WASp-evoked actin polymerization required for immunological synapse formation; the PSTPIP1 SH3 domain interacts with WASp's proline-rich region, and the PSTPIP1 coiled-coil domain interacts with CD2 and CD2AP. Deletion of the coiled-coil domain disrupts induced co-localization of these proteins at the synapse. Co-IP, dominant-negative coiled-coil deletion, confocal co-localization, T cell:APC conjugate and synapse formation assays in WASp-deficient T cells Immunity High 12530983
2005 PSTPIP1 SH3 domain binds the proline-rich domain of FasL cytoplasmic tail; co-expression of PSTPIP1 increases intracellular localization of FasL, reducing its extracellular availability and cytotoxic activity, and recruits PTP-PEST into a FasL-PSTPIP1-PTP-PEST ternary complex. Co-immunoprecipitation, co-localization by fluorescence microscopy, cytotoxicity assays The Journal of biological chemistry Medium 16204241
2006 CD2BP1/PSTPIP1 negatively regulates T cell activation downstream of CD2 by scaffolding PTP-PEST to the CD2 signalsome; overexpression selectively attenuates PLCγ1, ERK1/2, and p38 phosphorylation. Disruption of PTP-PEST or CD2BP1 association with the CD2 complex rescues T cells from inhibition. Primary T cell transduction, cytokine expression assays (CD69, IL-2, IFN-γ), phosphorylation assays Journal of immunology Medium 16670297
2007 PSTPIP1 acts as a cytosolic receptor for pyrin; pyrin exists as an autoinhibited homotrimer with intramolecular PYD-B-box interactions. Ligation by PSTPIP1 (also a homotrimer) unmasks pyrin's PYD, enabling ASC recruitment and oligomerization into an active ASC pyroptosome that recruits and activates caspase-1. PAPA-associated PSTPIP1 mutants bind pyrin with higher affinity, causing constitutive pyrin activation and heightened caspase-1/IL-1β processing. In vitro binding assays, co-immunoprecipitation, pyroptosome assembly assays, caspase-1 activation assays, gel filtration for oligomeric state Molecular cell High 17964261
2009 PSTPIP1 forms homodimers and generates membrane-associated filaments in cells; the extended FCH (Fes-Cip4 homology / F-BAR) domain is necessary and sufficient for self-aggregation. The filament network depends on an intact tubulin cytoskeleton. Pyrin modulates PSTPIP1 filament distribution and can recruit PSTPIP1 into ASC specks (inflammasome aggregates); PAPA-associated PSTPIP1 mutants are recruited to ASC specks with particularly high efficiency. Transfection with deletion constructs, immunofluorescence microscopy, co-localization with ASC specks, tubulin disruption experiments PloS one Medium 19584923
2014 Endogenous PSTPIP1 negatively regulates macrophage podosome organization and extracellular matrix degradation. A disease-associated PSTPIP1-R405C mutation (in the SH3 domain) impairs WASP binding but not PTP-PEST binding, causing elevated F-actin, excessive filopodia formation, and increased matrix degradation. WASP inhibition reverses these phenotypes, placing PSTPIP1 upstream of WASP in podosome/filopodia balance. Endogenous knockdown, overexpression of R405C mutant, WASP inhibition, F-actin quantification, matrix degradation assays in macrophages Blood High 24421327
2015 PSTPIP1 E250K and E257K mutations (associated with PAMI/Hz-Hc syndrome) substantially increase PSTPIP1 interaction with pyrin through enhanced PSTPIP1 phosphorylation, as demonstrated by immunoprecipitation and Western blotting, and are predicted by structural modeling to alter the electrostatic potential of the PSTPIP1 dimer at a protein-protein interaction surface. Immunoprecipitation, Western blotting, structural modeling of PSTPIP1 dimer The Journal of allergy and clinical immunology Medium 26025129
2015 Pyrin co-localizes with PSTPIP1 and polymerized actin at the leading edge of migrating HL-60 cells, demonstrating that PSTPIP1 interacts with dynamic actin and pyrin at the site of cell polarization during migration. Immunofluorescence co-localization in differentiated HL-60 cells during scratch-wound migration assay Cell biology international Low 26179737
2016 In osteoclasts, PSTPIP1 F-BAR domain recruits the phosphatase PTPN6 (SHP-1), which dephosphorylates the phosphoinositide 5-phosphatases SHIP1/2 that are bound to PSTPIP1's SH3 domain. This PSTPIP1/PTPN6/SHIP1-SHIP2 complex negatively regulates podosome disassembly and sealing zone dynamics; depletion of any component prevents disassembly and increases osteoclast bone resorption activity. Co-immunoprecipitation, siRNA knockdown, confocal microscopy, bone resorption assays PloS one Medium 27760174
2018 PSTPIP1 controls immunological synapse (IS) formation and F-actin polymerization in human T cells; loss-of-function mutations R228C and T274M impair F-actin accumulation at the IS, disrupt CD2 membrane microdomain capping, and alter T cell migration and calcium flux. The T274M mutation causes a preactivated F-actin state particularly damaging to T cell differentiation. CD2-PSTPIP1 association was confirmed by immunoprecipitation. Flow cytometry (F-actin), immunoprecipitation, IS formation assay in Jurkat transfectants, 3D collagen migration assay, calcium flux measurement in primary patient T cells The Journal of allergy and clinical immunology Medium 29432774
2022 The crystal structure of the PSTPIP1 F-BAR domain alone and bound to the C-terminal homology segment of LYP (PTPN22) was solved, revealing that a single LYP molecule binds to the PSTPIP1 F-BAR dimer via a novel recognition mechanism for proline-rich motifs. Disease-associated residues R228, D246, E250, and E257 of PSTPIP1 directly contact LYP, linking disruption of PSTPIP1-LYP interaction to autoinflammatory pathogenesis. X-ray crystallography, structural analysis, binding assays Cellular and molecular life sciences : CMLS High 35152348
2023 PSTPIP1 (F-BAR domain protein) is required for clathrin-independent endocytosis of L1CAM; it acts together with the N-BAR protein endophilin-A3, and galectins serve as endocytic partners that negatively regulate this process. siRNA knockdown, fluorescence microscopy, endocytosis assays Traffic Medium 36843549
2025 A CRISPR/Cas9 knockout screen identified PSTPIP1 as a regulatory factor of macrophage differentiation; deletion of PSTPIP1 results in hampered differentiation, decreased inflammatory response, altered morphology, and impaired cell adhesion and migration. Deletion of pyrin similarly disrupts cellular dynamics, placing both proteins as crucial factors in the same macrophage differentiation pathway. Genome-wide CRISPR/Cas9 knockout screen (GeCKO), independent KO/KI validation, FACS immunophenotyping, fluorescence microscopy, ELISA, RNA-seq European journal of cell biology Medium 40997504
2026 PTPN22 modulates actin cytoskeletal dynamics at the T cell immunological synapse through a direct interaction with PSTPIP1; PTPN22 deficiency or inhibition causes aberrant Arp2/3-dependent actin remodeling, excessive central F-actin foci, PSTPIP1 mislocalization, and aberrant PSTPIP1-TCR nanoscale co-localization, leading to enhanced calcium signaling especially under low-affinity TCR stimulation. Super-resolution DNA-PAINT, live/fixed multi-color fluorescence imaging, PTPN22 knockdown/inhibition in Jurkat cells, calcium flux assays Science signaling Medium 42263152
2026 A novel gain-of-function PSTPIP1 mutation (p.N236K) shows increased binding to pyrin and leads to heightened inflammasome formation relative to WT PSTPIP1, causing PAMI syndrome with severe neutropenia. Co-immunoprecipitation (PSTPIP1-pyrin binding), inflammasome formation assay, patient genetic characterization Journal of human immunity Low 42007463

Source papers

Stage 0 corpus · 53 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2003 Pyrin binds the PSTPIP1/CD2BP1 protein, defining familial Mediterranean fever and PAPA syndrome as disorders in the same pathway. Proceedings of the National Academy of Sciences of the United States of America 356 14595024
2002 Mutations in CD2BP1 disrupt binding to PTP PEST and are responsible for PAPA syndrome, an autoinflammatory disorder. Human molecular genetics 341 11971877
2007 Pyrin activates the ASC pyroptosome in response to engagement by autoinflammatory PSTPIP1 mutants. Molecular cell 211 17964261
2003 The Wiskott-Aldrich syndrome protein acts downstream of CD2 and the CD2AP and PSTPIP1 adaptors to promote formation of the immunological synapse. Immunity 159 12530983
1998 Dual function of Cyk2, a cdc15/PSTPIP family protein, in regulating actomyosin ring dynamics and septin distribution. The Journal of cell biology 151 9864366
1997 PSTPIP: a tyrosine phosphorylated cleavage furrow-associated protein that is a substrate for a PEST tyrosine phosphatase. The Journal of cell biology 141 9265651
1998 Tyrosine phosphorylation regulates the SH3-mediated binding of the Wiskott-Aldrich syndrome protein to PSTPIP, a cytoskeletal-associated protein. The Journal of biological chemistry 108 9488710
2000 Cytoskeletal protein PSTPIP1 directs the PEST-type protein tyrosine phosphatase to the c-Abl kinase to mediate Abl dephosphorylation. Molecular cell 105 11163214
2001 PSTPIP is a substrate of PTP-PEST and serves as a scaffold guiding PTP-PEST toward a specific dephosphorylation of WASP. The Journal of biological chemistry 96 11711533
2015 Single amino acid charge switch defines clinically distinct proline-serine-threonine phosphatase-interacting protein 1 (PSTPIP1)-associated inflammatory diseases. The Journal of allergy and clinical immunology 88 26025129
1998 A cdc15-like adaptor protein (CD2BP1) interacts with the CD2 cytoplasmic domain and regulates CD2-triggered adhesion. The EMBO journal 79 9857189
2014 The F-BAR protein PSTPIP1 controls extracellular matrix degradation and filopodia formation in macrophages. Blood 75 24421327
1998 PSTPIP 2, a second tyrosine phosphorylated, cytoskeletal-associated protein that binds a PEST-type protein-tyrosine phosphatase. The Journal of biological chemistry 66 9804817
2013 Identification of a homozygous PSTPIP1 mutation in a patient with a PAPA-like syndrome responding to canakinumab treatment. JAMA dermatology 60 23426477
2005 Binding of the intracellular Fas ligand (FasL) domain to the adaptor protein PSTPIP results in a cytoplasmic localization of FasL. The Journal of biological chemistry 46 16204241
2015 Pyoderma gangrenosum, acne and ulcerative colitis in a patient with a novel mutation in the PSTPIP1 gene. Clinical and experimental dermatology 45 25683018
2006 CD2BP1 modulates CD2-dependent T cell activation via linkage to protein tyrosine phosphatase (PTP)-PEST. Journal of immunology (Baltimore, Md. : 1950) 45 16670297
2011 Mutations in the PSTPIP1 gene and aberrant splicing variants in patients with pyoderma gangrenosum. Clinical and experimental dermatology 41 21790734
2009 Pyrin Modulates the Intracellular Distribution of PSTPIP1. PloS one 41 19584923
2010 Longest form of CCTG microsatellite repeat in the promoter of the CD2BP1/PSTPIP1 gene is associated with aseptic abscesses and with Crohn disease in French patients. Digestive diseases and sciences 35 19731031
2015 Novel PSTPIP1 gene mutation in a patient with pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome. Seminars in arthritis and rheumatism 31 25845478
2022 Excess Serum Interleukin-18 Distinguishes Patients With Pathogenic Mutations in PSTPIP1. Arthritis & rheumatology (Hoboken, N.J.) 29 34492165
2013 Inflammation in mice ectopically expressing human Pyogenic Arthritis, Pyoderma Gangrenosum, and Acne (PAPA) Syndrome-associated PSTPIP1 A230T mutant proteins. The Journal of biological chemistry 28 23293022
2018 Proline-serine-threonine phosphatase interacting protein 1 (PSTPIP1) controls immune synapse stability in human T cells. The Journal of allergy and clinical immunology 22 29432774
2018 PSTPIP1-associated myeloid-related proteinemia inflammatory syndrome: A rare cause of childhood neutropenia associated with systemic inflammation and hyperzincemia. Pediatric blood & cancer 22 30198636
2016 BAR Proteins PSTPIP1/2 Regulate Podosome Dynamics and the Resorption Activity of Osteoclasts. PloS one 21 27760174
2020 Phenotypic Associations of PSTPIP1 Sequence Variants in PSTPIP1-Associated Autoinflammatory Diseases. The Journal of investigative dermatology 20 33218716
2020 HSCT is effective in patients with PSTPIP1-associated myeloid-related proteinemia inflammatory (PAMI) syndrome. The Journal of allergy and clinical immunology 20 33338535
2017 Haematological involvement associated with a mild autoinflammatory phenotype, in two patients carrying the E250K mutation of PSTPIP1. Clinical and experimental rheumatology 14 28628471
2015 Pyrin-PSTPIP1 colocalises at the leading edge during cell migration. Cell biology international 14 26179737
2024 Efficacy and safety of anakinra and canakinumab in PSTPIP1-associated inflammatory diseases: a comprehensive scoping review. Frontiers in immunology 12 38259483
2022 PSTPIP1-LYP phosphatase interaction: structural basis and implications for autoinflammatory disorders. Cellular and molecular life sciences : CMLS 10 35152348
2021 Kidney Involvement in PSTPIP1 Associated Inflammatory Diseases (PAID): A Case Report and Review of the Literature. Frontiers in medicine 10 34778321
2014 A novel de novo PSTPIP1 mutation in a boy with pyogenic arthritis, pyoderma gangrenosum, acne (PAPA) syndrome. Clinical and experimental rheumatology 10 24960411
2016 Identification of novel mutations in CD2BP1 gene in clinically proven rheumatoid arthritis patients of south India. European journal of medical genetics 9 27184502
2021 Clinical and genetic characteristics of PSTPIP1-associated myeloid-related proteinemia inflammatory syndrome. Pediatric rheumatology online journal 8 34620178
2001 Regulation of the association between PSTPIP and CD2 in murine T cells. Experimental and molecular pathology 7 11599917
2023 N-BAR and F-BAR proteins-endophilin-A3 and PSTPIP1-control clathrin-independent endocytosis of L1CAM. Traffic (Copenhagen, Denmark) 6 36843549
2023 PSTPIP1-Associated Myeloid-Related Proteinemia Inflammatory (PAMI) Syndrome: A Systematic Review. Genes 6 37628706
2023 Rare missense variants in the SH3 domain of PSTPIP1 are associated with hidradenitis suppurativa. HGG advances 5 37013170
2021 Rare cases of PAMI syndrome in both father and son with the same missense mutation in PSTPIP1 gene and literature review. The Journal of dermatology 5 33458872
2015 Imp2, the PSTPIP homolog in fission yeast, affects sensitivity to the immunosuppressant FK506 and membrane trafficking in fission yeast. Biochemical and biophysical research communications 4 25580011
2021 Immunological repertoire linked to PSTPIP1-associated myeloid-related inflammatory (PAMI) syndrome. Pediatric rheumatology online journal 3 34399798
2023 Detection of a rare variant in PSTPIP1 through three generations in a family with an initial diagnosis of FMF/MKD-overlapping phenotype. Rheumatology (Oxford, England) 2 36692132
2021 Mutation in the proline-serine-threonine phosphatase-interacting protein 1 (PSTPIP1) gene in a patient with acute lymphoblastic leukemia. Central-European journal of immunology 2 34764798
2022 Next-Generation Sequencing Analysis of MVK, NLRP3, TNFRSF1A, and PSTPIP1 Genes in Patients without MEFV Gene Variation and Genotype-Phenotype Correlation. European journal of rheumatology 1 35546330
2020 A child with recurrent pyogenic arthritis with the PSTPIP1 mutation. SAGE open medical case reports 1 32477554
2026 Autoinflammatory disease and severe neutropenia due to de novo variant of PSTPIP1 with increased binding to pyrin. Journal of human immunity 0 42007463
2026 PTPN22 regulates T cell synapse formation through PSTPIP1-dependent actin remodeling. Science signaling 0 42263152
2025 PSTPIP1 and pyrin, two key regulators of macrophage differentiation. European journal of cell biology 0 40997504
2025 Progressive increase of serum zinc level in a Pediatric patient with PSTPIP1- p.N236K mutation. Clinica chimica acta; international journal of clinical chemistry 0 41072568
2024 Stimulation of PSTPIP1 to trigger proinflammatory responses in asymptomatic SARS-CoV-2 infections. Heliyon 0 38463809
2023 Fas ligand intracellular signaling: does PSTPIP mediate T cell death? Apoptosis : an international journal on programmed cell death 0 37794219

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