Gene Symbol: PSTPIP1
Description: proline-serine-threonine phosphatase interacting protein 1
Alias: CD2BP1, CD2BP1L, CD2BP1S, H-PIP, PAPAS, PSTPIP, proline-serine-threonine phosphatase-interacting protein 1, CD2 antigen-binding protein 1, CD2 cytoplasmic tail-binding protein, CD2-binding protein 1, PEST phosphatase-interacting protein 1, truncated proline-serine-threonine phosphatase interacting protein 1
Species: human
Products:     PSTPIP1

Top Publications

  1. Yang H, Reinherz E. CD2BP1 modulates CD2-dependent T cell activation via linkage to protein tyrosine phosphatase (PTP)-PEST. J Immunol. 2006;176:5898-907 pubmed
    Human CD2 regulates T cell activation and adhesion via mechanisms yet to be fully understood. This study focuses on CD2BP1, a CD2 cytoplasmic tail-binding protein preferentially expressed in hematopoetic cells...
  2. Cooper K, Bennin D, Huttenlocher A. The PCH family member proline-serine-threonine phosphatase-interacting protein 1 targets to the leukocyte uropod and regulates directed cell migration. Mol Biol Cell. 2008;19:3180-91 pubmed publisher
    ..Here we show that PSTPIP1, a PCH family member expressed in hematopoietic cells, regulates the motility of neutrophil-like cells and is a ..
  3. Wu Y, Spencer S, Lasky L. Tyrosine phosphorylation regulates the SH3-mediated binding of the Wiskott-Aldrich syndrome protein to PSTPIP, a cytoskeletal-associated protein. J Biol Chem. 1998;273:5765-70 pubmed
    ..Here we report that WASP interacts with a recently described cytoskeletal-associated protein, PSTPIP, a molecule that is related to the Schizosaccharomyces pombe cleavage furrow regulatory protein, CDC15p...
  4. Wu Y, Dowbenko D, Lasky L. PSTPIP 2, a second tyrosine phosphorylated, cytoskeletal-associated protein that binds a PEST-type protein-tyrosine phosphatase. J Biol Chem. 1998;273:30487-96 pubmed
    ..the identification of a cytoskeletal-associated protein, proline-serine-threonine phosphatase-interacting protein (PSTPIP), whose level of tyrosine phosphorylation was controlled by PEST-type protein-tyrosine phosphatases (PTPs) bound ..
  5. Spencer S, Dowbenko D, Cheng J, Li W, Brush J, Utzig S, et al. PSTPIP: a tyrosine phosphorylated cleavage furrow-associated protein that is a substrate for a PEST tyrosine phosphatase. J Cell Biol. 1997;138:845-60 pubmed
    ..This resulted in the identification of proline, serine, threonine phosphatase interacting protein (PSTPIP), a novel member of the actin- associated protein family that is homologous to Schizosaccharomyces pombe CDC15p, a ..
  6. Cong F, Spencer S, Cote J, Wu Y, Tremblay M, Lasky L, et al. Cytoskeletal protein PSTPIP1 directs the PEST-type protein tyrosine phosphatase to the c-Abl kinase to mediate Abl dephosphorylation. Mol Cell. 2000;6:1413-23 pubmed
    A search for c-Abl interacting proteins resulted in the recovery of PSTPIP1, originally identified as a binding protein of the PEST-type protein tyrosine phosphatases (PTP)...
  7. Tsujita K, Suetsugu S, Sasaki N, Furutani M, Oikawa T, Takenawa T. Coordination between the actin cytoskeleton and membrane deformation by a novel membrane tubulation domain of PCH proteins is involved in endocytosis. J Cell Biol. 2006;172:269-79 pubmed
    ..Furthermore, knockdown of endogenous FBP17 and CIP4 impaired endocytosis. Our data indicate that PCH protein family members couple membrane deformation to actin cytoskeleton reorganization in various cellular processes. ..
  8. Cote J, Chung P, Theberge J, Halle M, Spencer S, Lasky L, et al. PSTPIP is a substrate of PTP-PEST and serves as a scaffold guiding PTP-PEST toward a specific dephosphorylation of WASP. J Biol Chem. 2002;277:2973-86 pubmed
    b>PSTPIP is a tyrosine-phosphorylated protein involved in the organization of the cytoskeleton. Its ectopic expression induces filipodial-like membrane extensions in NIH 3T3 cells...
  9. Badour K, Zhang J, Shi F, McGavin M, Rampersad V, Hardy L, et al. The Wiskott-Aldrich syndrome protein acts downstream of CD2 and the CD2AP and PSTPIP1 adaptors to promote formation of the immunological synapse. Immunity. 2003;18:141-54 pubmed
    ..evokes interactions between the proline-rich region required for WASp translocation to the synapse and the PSTPIP1 adaptor SH3 domain and between the PSTPIp1 coiled-coil domain and both CD2 and another CD2-binding adaptor, CD2AP...

More Information


  1. Yu J, Fernandes Alnemri T, Datta P, Wu J, Juliana C, Solorzano L, et al. Pyrin activates the ASC pyroptosome in response to engagement by autoinflammatory PSTPIP1 mutants. Mol Cell. 2007;28:214-27 pubmed
    The molecular mechanism by which mutations in the cytoskeleton-organizing protein PSTPIP1 cause the autoinflammatory PAPA syndrome is still elusive...
  2. Shoham N, Centola M, Mansfield E, Hull K, Wood G, Wise C, et al. Pyrin binds the PSTPIP1/CD2BP1 protein, defining familial Mediterranean fever and PAPA syndrome as disorders in the same pathway. Proc Natl Acad Sci U S A. 2003;100:13501-6 pubmed
    ..We now show that proline serine threonine phosphatase-interacting protein [PSTPIP1, or CD2-binding protein 1 (CD2BP1)], a tyrosine-phosphorylated protein involved in cytoskeletal organization, ..
  3. Wise C, Gillum J, Seidman C, Lindor N, Veile R, Bashiardes S, et al. Mutations in CD2BP1 disrupt binding to PTP PEST and are responsible for PAPA syndrome, an autoinflammatory disorder. Hum Mol Genet. 2002;11:961-9 pubmed
    ..CD2BP1 and its murine ortholog, proline-serine-threonine phosphatase interacting protein (PSTPIP1), are adaptor proteins known to interact with PEST-type protein tyrosine phosphatases (PTP)...
  4. Li J, Nishizawa K, An W, Hussey R, Lialios F, Salgia R, et al. A cdc15-like adaptor protein (CD2BP1) interacts with the CD2 cytoplasmic domain and regulates CD2-triggered adhesion. EMBO J. 1998;17:7320-36 pubmed
    ..of CD2BP1 is restricted to hematopoietic tissue, being prominent in T and natural killer (NK) cells, with long (CD2BP1L) and short (CD2BP1S) variants arising by alternative RNA splicing...
  5. Martinez Rios C, Jariwala M, Highmore K, Duffy K, Spiegel L, Laxer R, et al. Imaging findings of sterile pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome: differential diagnosis and review of the literature. Pediatr Radiol. 2019;49:23-36 pubmed publisher
    ..PAPA syndrome is the result of a mutation in the proline serine threonine phosphatase-interacting protein 1 (PSTPIP1/CD2BP1) gene located on chromosome 15, which results in an abnormal overproduction of the pro-inflammatory ..
  6. Soares A, Andriolo R, Atallah A, da Silva E. Botulinum toxin for myofascial pain syndromes in adults. Cochrane Database Syst Rev. 2012;:CD007533 pubmed publisher
    ..We searched the Cochrane Pain, Palliative and Supportive Care (PaPaS) Review Group's Specialised Register until December 2011, CENTRAL (Cochrane Database of Systematic Reviews 2011, ..
  7. Kita A, Higa M, Doi A, Satoh R, Sugiura R. Imp2, the PSTPIP homolog in fission yeast, affects sensitivity to the immunosuppressant FK506 and membrane trafficking in fission yeast. Biochem Biophys Res Commun. 2015;457:273-9 pubmed publisher
    ..Here, we isolated imp2-c3, a mutant allele of the imp2(+) gene, encoding a homolog of the mouse PSTPIP1 (proline-serine-threonine phosphatase interacting protein 1), using a genetic screen for mutations that are ..
  8. Amir O, Wolf M, Mick L, Levi O, Primov Fever A. Parents' Evaluations of Their Children's Dysphonia: The Mamas and the Papas. J Voice. 2015;29:469-75 pubmed publisher
    ..Mothers of dysphonic children evaluate their children's voice handicap more severely than fathers, whereas both parents of nondysphonic children perform this evaluation similarly. ..
  9. Brocklehurst P, Tickle M, Glenny A, Lewis M, Pemberton M, Taylor J, et al. Systemic interventions for recurrent aphthous stomatitis (mouth ulcers). Cochrane Database Syst Rev. 2012;:CD005411 pubmed publisher
    ..We undertook electronic searches of: Cochrane Oral Health Group and PaPaS Trials Registers (to 6 June 2012); CENTRAL via The Cochrane Library (to Issue 4, 2012); MEDLINE via OVID (1950 to ..
  10. O Connell N, Wand B, Marston L, Spencer S, DeSouza L. Non-invasive brain stimulation techniques for chronic pain. Cochrane Database Syst Rev. 2010;:CD008208 pubmed publisher
    ..We searched CENTRAL, MEDLINE, EMBASE, CINAHL, PsycINFO, LILACS, the Cochrane PaPaS Group Trials Register and clinical trials registers...
  11. Huang C, Oberbeck N, Hsiao Y, Liu P, Johnson A, Dixit V, et al. Crystal Structure of Ripk4 Reveals Dimerization-Dependent Kinase Activity. Structure. 2018;26:767-777.e5 pubmed publisher
    ..Mutations in human RIPK4 manifest as Bartsocas-Papas syndrome (BPS), a genetic disorder characterized by severe craniofacial and limb abnormalities...
  12. Kirino Y, Zhou Q, Ishigatsubo Y, Mizuki N, Tugal Tutkun I, Seyahi E, et al. Targeted resequencing implicates the familial Mediterranean fever gene MEFV and the toll-like receptor 4 gene TLR4 in Behçet disease. Proc Natl Acad Sci U S A. 2013;110:8134-9 pubmed publisher
    ..and ERAP1) and 11 genes selected for their role in innate immunity (IL1B, IL1R1, IL1RN, NLRP3, MEFV, TNFRSF1A, PSTPIP1, CASP1, PYCARD, NOD2, and TLR4) were evaluated for BD association...
  13. Kim K, Myers R, Lee J, Igartua C, Lee K, Kim Y, et al. Genome-wide association study of recalcitrant atopic dermatitis in Korean children. J Allergy Clin Immunol. 2015;136:678-684.e4 pubmed publisher
    ..quantitative trait loci for 2 nearby genes, ISL2 and proline-serine-threonine phosphatase interacting protein 1 (PSTPIP1), in immune cells...
  14. Kwa M, Huynh J, Aw J, Zhang L, Nguyen T, Reynolds E, et al. Receptor-interacting protein kinase 4 and interferon regulatory factor 6 function as a signaling axis to regulate keratinocyte differentiation. J Biol Chem. 2014;289:31077-87 pubmed publisher
    ..of keratinocyte differentiation, and their mutation causes the related developmental epidermal disorders Bartsocas-Papas syndrome and popliteal pterygium syndrome, respectively...
  15. Schäffler H, Blattmann T, Findeisen A, Meinel F, Meyer Bahlburg A, Lamprecht G, et al. [PAPA syndrome with Crohn's disease and primary sclerosing cholangitis/autoimmune hepatitis overlap syndrome]. Hautarzt. 2019;70:116-122 pubmed publisher
    ..pyoderma gangraenosum and acne, is an autosomal dominant hereditary disease which is caused by a mutation in the PSTPIP1 ("proline-serine-threonine phosphatase interacting protein 1") gene located on chromosome 15 and encodes the ..
  16. Lindwall E, Singla S, Davis W, Quinet R. Novel PSTPIP1 gene mutation in a patient with pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome. Semin Arthritis Rheum. 2015;45:91-3 pubmed publisher
    ..We report the case of a now 25-year-old male patient with PAPA syndrome with the E250K mutation in PSTPIP1. We also present a systematic literature review of other PAPA cases...
  17. Moghaddas F, Masters S. Monogenic autoinflammatory diseases: Cytokinopathies. Cytokine. 2015;74:237-46 pubmed publisher
    ..of IL-1 is increased by mutation of innate immune sensors such as NLRP3, upstream signalling molecules such as PSTPIP1 and receptors or downstream signalling molecules, such as IL-1Ra...
  18. Kwa M, Huynh J, Reynolds E, Hamilton J, Scholz G. Disease-associated mutations in IRF6 and RIPK4 dysregulate their signalling functions. Cell Signal. 2015;27:1509-16 pubmed publisher
    ..differentiation and their mutation cause the developmental syndromes Van der Woude syndrome (VWS) and Bartsocas-Papas syndrome (BPS), respectively...
  19. Soares A, Andriolo R, Atallah A, da Silva E. Botulinum toxin for myofascial pain syndromes in adults. Cochrane Database Syst Rev. 2014;:CD007533 pubmed publisher
    ..For the original review, we also searched the Cochrane Pain, Palliative and Supportive Care (PaPaS) Review Group Specialised Register until December 2011, PubMed (from 1966 to 2011) and LILACS (from 1982 to 2011)...
  20. Ginat D, Robson C. CT and MRI of congenital nasal lesions in syndromic conditions. Pediatr Radiol. 2015;45:1056-65 pubmed publisher
    ..incisor syndrome, CHARGE syndrome, Bosma syndrome, median cleft face syndrome, PHACES association, Bartsocas-Papas syndrome, Binder syndrome, duplication of the pituitary gland-plus syndrome and syndromic craniosynsotosis (e.g...
  21. Richardson R, Hammond N, Coulombe P, Saloranta C, Nousiainen H, Salonen R, et al. Periderm prevents pathological epithelial adhesions during embryogenesis. J Clin Invest. 2014;124:3891-900 pubmed publisher
    ..Furthermore, this study suggests that failure of periderm formation underlies a series of devastating birth defects, including popliteal pterygium syndrome, cocoon syndrome, and Bartsocas-Papas syndrome.
  22. Papaspyridakos P. Implant success rates for single crowns and fixed partial dentures in general dental practices may be lower than those achieved in well-controlled university or specialty settings. J Evid Based Dent Pract. 2015;15:30-2 pubmed publisher
    ..Da Silva JD, Kazimiroff J, Papas A, Curro FA, Thompson VP, Vena DA, Wu H, Collie D, Craig RG, Practitioners Engaged in Applied Research Learning ..
  23. Ryan J, Masters S, Booty M, Habal N, Alexander J, Barham B, et al. Clinical features and functional significance of the P369S/R408Q variant in pyrin, the familial Mediterranean fever protein. Ann Rheum Dis. 2010;69:1383-8 pubmed publisher
    ..Exon 3 encoding pyrin's B-box domain is necessary for interactions with proline serine threonine phosphatase interacting protein 1 (PSTPIP1)...
  24. Fuentes Valdes G, Del Valle Flores M, Vega Soto C. [Comparative analysis of the glycemic response and glycemic index of instant mashed potatoes in subjects undergoing laparoscopic sleeve gastrectomy and control subjects]. Nutr Hosp. 2014;30:1263-9 pubmed publisher
    ..Comparar la respuesta glicémica (RG) e IG del puré de papas instantáneo en sujetos sometidos a GVML versus sujetos controles...
  25. Ozel B, Poyrazoğlu O, Karaman A, Karaman H, Altinkaya E, Sevinç E, et al. The PAPAS index: a novel index for the prediction of hepatitis C-related fibrosis. Eur J Gastroenterol Hepatol. 2015;27:895-900 pubmed publisher
    ..This study aimed to determine the performance of the PAPAS (Platelet/Age/Phosphatase/AFP/AST) index in patients with CHC for the prediction of significant fibrosis and ..
  26. Hu J, Ye F, Cui M, Lee P, Wei C, Hao Y, et al. Protein Profiling of Bladder Urothelial Cell Carcinoma. PLoS ONE. 2016;11:e0161922 pubmed publisher
    ..Ten proteins (ß-catenin, HSP70, autotaxin, Notch4, PSTPIP1, DPYD, ODC, cyclinB1, calretinin and EPO) were able to classify muscle invasive BCa (MIBC) into 2 distinct groups,..
  27. Zhao Z, Dammert M, Hoppe S, Bierhoff H, Grummt I. Heat shock represses rRNA synthesis by inactivation of TIF-IA and lncRNA-dependent changes in nucleosome positioning. Nucleic Acids Res. 2016;44:8144-52 pubmed publisher
    ..Attenuation of pre-rRNA synthesis in response to heat stress is accompanied by upregulation of PAPAS, a long non-coding RNA (lncRNA) that is transcribed in antisense orientation to pre-rRNA...
  28. Campbell L, Raheem I, Malemud C, Askari A. The Relationship between NALP3 and Autoinflammatory Syndromes. Int J Mol Sci. 2016;17: pubmed publisher
    ..syndrome is caused by mutations in the genes encoding proline-serine-threonine phosphatase interacting protein 1 (PSTPIP1)...
  29. Gollasch B, Basmanav F, Nanda A, Fritz G, Mahmoudi H, Thiele H, et al. Identification of a novel mutation in RIPK4 in a kindred with phenotypic features of Bartsocas-Papas and CHAND syndromes. Am J Med Genet A. 2015;167A:2555-62 pubmed publisher
    ..Recently, mutations in RIPK4 have been reported in Bartsocas-Papas syndrome (OMIM 263650) that shows overlapping clinical symptoms with the phenotype observed in the affected ..
  30. Vural S, Gundogdu M, Kundakci N, Ruzicka T. Familial Mediterranean fever patients with hidradenitis suppurativa. Int J Dermatol. 2017;56:660-663 pubmed publisher
    ..associated with genetic alterations of gene encoding proline-serine-threonine phosphatase-interacting protein 1 (PSTPIP1), which interacts with the product of MEFV gene in the autoinflammatory pathway...
  31. Marzano A, Borghi A, Meroni P, Cugno M. Pyoderma gangrenosum and its syndromic forms: evidence for a link with autoinflammation. Br J Dermatol. 2016;175:882-891 pubmed publisher
    ..In PAPA syndrome, different mutations involving the PSTPIP1 gene, via an increased binding affinity to pyrin, induce the assembly of inflammasomes...
  32. Janssen W, Grobarova V, Leleux J, Jongeneel L, van Gijn M, van Montfrans J, et al. Proline-serine-threonine phosphatase interacting protein 1 (PSTPIP1) controls immune synapse stability in human T cells. J Allergy Clin Immunol. 2018;142:1947-1955 pubmed publisher
    Proline-serine-threonine phosphatase interacting protein 1 (PSTPIP1) is a cytosolic adaptor protein involved with T-cell activation, differentiation, and migration...
  33. Urwyler Rösselet C, Tanghe G, Leurs K, Gilbert B, De Rycke R, De Bruyne M, et al. Keratinocyte-Specific Ablation of RIPK4 Allows Epidermal Cornification but Impairs Skin Barrier Formation. J Invest Dermatol. 2018;138:1268-1278 pubmed publisher
    In humans, receptor-interacting protein kinase 4 (RIPK4) mutations can lead to the autosomal recessive Bartsocas-Papas and popliteal pterygium syndromes, which are characterized by severe skin defects, pterygia, as well as clefting...
  34. Sztacho M, Segeletz S, Sanchez Fernandez M, Czupalla C, Niehage C, Hoflack B. BAR Proteins PSTPIP1/2 Regulate Podosome Dynamics and the Resorption Activity of Osteoclasts. PLoS ONE. 2016;11:e0164829 pubmed publisher
    ..Whereas PSTPIP2 regulates podosome assembly, PSTPIP1 regulates their disassembly...
  35. Rusmini M, Federici S, Caroli F, Grossi A, Baldi M, Obici L, et al. Next-generation sequencing and its initial applications for molecular diagnosis of systemic auto-inflammatory diseases. Ann Rheum Dis. 2016;75:1550-7 pubmed publisher
    ..causative gene(s), were massively sequenced for the coding portions of MEFV, MVK, TNFRSF1A, NLRP3, NLRP12, NOD2, PSTPIP1, IL1RN, LPIN2 and PSMB8...
  36. Shi S, Zhao J, Wang J, Mi D, Ma Z. HPIP silencing inhibits TGF-?1-induced EMT in lung cancer cells. Int J Mol Med. 2017;39:479-483 pubmed publisher
    ..In conclusion, we demonstrated that HPIP silencing suppressed TGF-?1-induced EMT in lung cancer cells by inhibiting Smad2 activation. Therefore, HPIP may be a new therapeutic target for the treatment of lung cancer. ..
  37. Baum W, Kirkin V, Fernández S, Pick R, Lettau M, Janssen O, et al. Binding of the intracellular Fas ligand (FasL) domain to the adaptor protein PSTPIP results in a cytoplasmic localization of FasL. J Biol Chem. 2005;280:40012-24 pubmed
    ..Here we report the identification of the Src homology 3 domain-containing adaptor protein PSTPIP as a FasL-interacting partner, which binds to the proline-rich domain...
  38. Lu M, Zhang L, Sartippour M, Norris A, Brooks M. EG-1 interacts with c-Src and activates its signaling pathway. Int J Oncol. 2006;29:1013-8 pubmed
    ..Furthermore, EG-1 shows interaction with multiple other SH3- and WW-containing molecules involved in various signaling pathways. These observations suggest that EG-1 may be involved in signaling pathways including c-Src activation. ..
  39. Zhao Z, Dammert M, Grummt I, Bierhoff H. lncRNA-Induced Nucleosome Repositioning Reinforces Transcriptional Repression of rRNA Genes upon Hypotonic Stress. Cell Rep. 2016;14:1876-82 pubmed publisher has established that downregulation of rRNA synthesis in quiescent cells is accompanied by upregulation of PAPAS, a long noncoding RNA (lncRNA) that recruits the histone methyltransferase Suv4-20h2 to rDNA, thus triggering ..
  40. Ahlemeyer N, Streff E, Muthupandi P, Birman V. Dramatic Acceleration of an Acyl Transfer-Initiated Cascade by Using Electron-Rich Amidine-Based Catalysts. Org Lett. 2017;: pubmed publisher
    ..A highly diastereo- and enantioselective version of this process has been developed using H-PIP 1b, a chiral catalyst prepared over a decade ago, but never utilized since its disclosure...
  41. Belelli E, Passarelli C, Pardeo M, Holzinger D, De Benedetti F, Insalaco A. Haematological involvement associated with a mild autoinflammatory phenotype, in two patients carrying the E250K mutation of PSTPIP1. Clin Exp Rheumatol. 2017;35 Suppl 108:113-115 pubmed
    ..Hz/Hc) syndrome is a recently described condition caused by a specific de novo mutation (E250K) affecting PSTPIP1 gene...
  42. Hong J, Su Y, Chiu H. Pyogenic arthritis, pyoderma gangrenosum, and acne syndrome (PAPA syndrome): report of a sporadic case without an identifiable mutation in the CD2BP1 gene. J Am Acad Dermatol. 2009;61:533-5 pubmed publisher
  43. Holzinger D, Roth J. Alarming consequences - autoinflammatory disease spectrum due to mutations in proline-serine-threonine phosphatase-interacting protein 1. Curr Opin Rheumatol. 2016;28:550-9 pubmed publisher
    ..of autoinflammatory diseases due to mutations in proline-serine-threonine phosphatase-interacting protein 1 (PSTPIP1) and new insights into their pathogenesis...
  44. Hammond N, Dixon J, Dixon M. Periderm: Life-cycle and function during orofacial and epidermal development. Semin Cell Dev Biol. 2017;: pubmed publisher
    ..the autosomal dominant popliteal pterygium syndrome and the autosomal recessive cocoon syndrome and Bartsocas Papas syndrome...
  45. Bugide S, David D, Nair A, Kannan N, Samanthapudi V, Prabhakar J, et al. Hematopoietic PBX-interacting protein (HPIP) is over expressed in breast infiltrative ductal carcinoma and regulates cell adhesion and migration through modulation of focal adhesion dynamics. Oncogene. 2015;34:4601-12 pubmed publisher
    ..These molecular interactions between HPIP and FAK, and HPIP and calpain2 regulate cell adhesion and migration through modulation of FA dynamics. ..
  46. Marcos T, Ruiz Martín V, de la Puerta M, Trinidad A, Rodriguez M, de la Fuente M, et al. Proline-serine-threonine phosphatase interacting protein 1 inhibition of T-cell receptor signaling depends on its SH3 domain. FEBS J. 2014;281:3844-54 pubmed publisher
    Proline-serine-threonine phosphatase interacting protein 1 (PSTPIP1) is an adaptor protein associated with the cytoskeleton that is mainly expressed in hematopoietic cells...
  47. Dowling J, Becker C, Bourke N, Corr S, Connolly D, Quinn S, et al. Promyelocytic leukemia protein interacts with the apoptosis-associated speck-like protein to limit inflammasome activation. J Biol Chem. 2014;289:6429-37 pubmed publisher
    ..Collectively, our data indicate that PML limits ASC function, retaining ASC in the nucleus. ..
  48. Kone Paut I, Sanchez E, Le Quellec A, Manna R, Touitou I. Autoinflammatory gene mutations in Behçet's disease. Ann Rheum Dis. 2007;66:832-4 pubmed
    ..cold-induced autoinflammatory syndrome 1 (CIAS1) and proline/serine/threonine phosphatase-interacting protein 1 (PSTPIP1) genes, responsible for mevalonate kinase deficiency (MKD), cryopyrin associated periodic syndromes (CAPS) and ..
  49. Day T, Ramanan A, Hinks A, Lamb R, Packham J, Wise C, et al. Autoinflammatory genes and susceptibility to psoriatic juvenile idiopathic arthritis. Arthritis Rheum. 2008;58:2142-6 pubmed publisher
    To investigate the association of NLRP3, NOD2, MEFV, and PSTPIP1, genes that cause 4 of the autoinflammatory hereditary periodic fever syndromes (HPFS), with juvenile idiopathic arthritis (JIA)...
  50. Waite A, Schaner P, Richards N, Balci Peynircioglu B, Masters S, Brydges S, et al. Pyrin Modulates the Intracellular Distribution of PSTPIP1. PLoS ONE. 2009;4:e6147 pubmed publisher
    b>PSTPIP1 is a cytoskeleton-associated adaptor protein that links PEST-type phosphatases to their substrates...
  51. Badour K, Zhang J, Shi F, Leng Y, Collins M, Siminovitch K. Fyn and PTP-PEST-mediated regulation of Wiskott-Aldrich syndrome protein (WASp) tyrosine phosphorylation is required for coupling T cell antigen receptor engagement to WASp effector function and T cell activation. J Exp Med. 2004;199:99-112 pubmed
    ..Fyn enhanced WASp-mediated Arp2/3 activation and was required for synapse formation, PTP-PEST combined with PSTPIP1 inhibited WASp-driven actin polymerization and synapse formation...
  52. Dowbenko D, Spencer S, Quan C, Lasky L. Identification of a novel polyproline recognition site in the cytoskeletal associated protein, proline serine threonine phosphatase interacting protein. J Biol Chem. 1998;273:989-96 pubmed
    ..Thus, the interaction between PTP HSCF and PST PIP is mediated by a novel site in the cytoskeletal associated protein which interacts with residues within the proline-rich COOH terminus of the phosphatase. ..
  53. Akkaya Ulum Y, Balci Peynircioglu B, Purali N, Yilmaz E. Pyrin-PSTPIP1 colocalises at the leading edge during cell migration. Cell Biol Int. 2015;39:1384-94 pubmed publisher neutrophils, has been reported to interact with proline-serine-threonine phosphatase-interacting protein 1 (PSTPIP1) and actin proteins...
  54. Starnes T, Bennin D, Bing X, Eickhoff J, Grahf D, Bellak J, et al. The F-BAR protein PSTPIP1 controls extracellular matrix degradation and filopodia formation in macrophages. Blood. 2014;123:2703-14 pubmed publisher
    b>PSTPIP1 is a cytoskeletal adaptor and F-BAR protein that has been implicated in autoinflammatory disease, most notably in the PAPA syndrome: pyogenic sterile arthritis, pyoderma gangrenosum, and acne...
  55. Lin Z, Hegarty J, Lin T, Ostrov B, Wang Y, Yu W, et al. Failure of anakinra treatment of pyoderma gangrenosum in an IBD patient and relevance to the PSTPIP1 gene. Inflamm Bowel Dis. 2011;17:E41-2 pubmed publisher
  56. Chen Y, Zhao R, Zhao Q, Shao Y, Zhang S. Knockdown of HPIP Inhibits the Proliferation and Invasion of Head-and-Neck Squamous Cell Carcinoma Cells by Regulating PI3K/Akt Signaling Pathway. Oncol Res. 2016;24:153-60 pubmed publisher
    ..Therefore, HPIP may be a novel potential therapeutic target for the treatment of HNSCC. ..
  57. Kumar B, Kumar P, Sowgandhi N, Prajwal B, Mohan A, Sarma K, et al. Identification of novel mutations in CD2BP1 gene in clinically proven rheumatoid arthritis patients of south India. Eur J Med Genet. 2016;59:404-12 pubmed publisher
    ..The disease results due to mutations in the cluster of differentiation 2 binding protein 1 (CD2BP1) gene on chromosome 15q24.3...
  58. Bugide S, Gonugunta V, Penugurti V, Malisetty V, Vadlamudi R, Manavathi B. HPIP promotes epithelial-mesenchymal transition and cisplatin resistance in ovarian cancer cells through PI3K/AKT pathway activation. Cell Oncol (Dordr). 2017;40:133-144 pubmed publisher
    ..In addition, we conclude that HPIP may serve as a potential therapeutic target for cisplatin resistant ovarian tumors. ..
  59. Holzinger D, Fassl S, de Jager W, Lohse P, Röhrig U, Gattorno M, et al. Single amino acid charge switch defines clinically distinct proline-serine-threonine phosphatase-interacting protein 1 (PSTPIP1)-associated inflammatory diseases. J Allergy Clin Immunol. 2015;136:1337-45 pubmed publisher
    ..Proline-serine-threonine phosphatase-interacting protein 1 (PSTPIP1) gene sequencing was performed in 14 patients with Hz/Hc, and their clinical phenotype was compared with that of ..
  60. Qian J, Chen W, Lettau M, Podda G, Zörnig M, Kabelitz D, et al. Regulation of FasL expression: a SH3 domain containing protein family involved in the lysosomal association of FasL. Cell Signal. 2006;18:1327-37 pubmed
    ..Members of this protein family are characterized by a similar domain structure and include FBP17, PACSIN1-3, CD2BP1, CIP4, Rho-GAP C1 and several hypothetical proteins...
  61. Burillo Sanz S, Montes Cano M, García Lozano J, Ortiz Fernández L, Ortego Centeno N, García Hernández F, et al. Mutational profile of rare variants in inflammasome-related genes in Behçet disease: A Next Generation Sequencing approach. Sci Rep. 2017;7:8453 pubmed publisher
    ..was to investigate the role of rare variants in seven genes involved in AIDs: CECR1, MEFV, MVK, NLRP3, NOD2, PSTPIP1 and TNFRSF1A using a next generation sequencing (NGS) approach in 355 BD patients...
  62. Fathalla B, Al Wahadneh A, Al Mutawa M, Kambouris M, El Shanti H. A novel de novo PSTPIP1 mutation in a boy with pyogenic arthritis, pyoderma gangrenosum, acne (PAPA) syndrome. Clin Exp Rheumatol. 2014;32:956-8 pubmed
    ..The mutation analysis of the boy identified a novel de novo mutation in PSTPIP1, the gene responsible for PAPA syndrome...
  63. Nesterovitch A, Hoffman M, Simon M, Petukhov P, Tharp M, Glant T. Mutations in the PSTPIP1 gene and aberrant splicing variants in patients with pyoderma gangrenosum. Clin Exp Dermatol. 2011;36:889-95 pubmed publisher
    ..Two mutations (A230T and E250Q) in the PSTPIP1 gene, encoding proline-serine-threonine phosphatase-interacting protein (PSTPIP)1 have been identified in ..
  64. Oda A, Ochs H, Lasky L, Spencer S, Ozaki K, Fujihara M, et al. CrkL is an adapter for Wiskott-Aldrich syndrome protein and Syk. Blood. 2001;97:2633-9 pubmed
    ..The binding of GST-fusion SH3 domain of PSTPIP1 in vitro was also not affected by WASP tyrosine phosphorylation, suggesting that the binding of the SH3 domains ..
  65. André M, Aumaître O, Grateau G, Chamaillard M, Costedoat Chalumeau N, Cardoso M, et al. 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. Dig Dis Sci. 2010;55:1681-8 pubmed publisher
    ..dominant syndromic kind of pyoderma gangrenosum, the PAPA syndrome, is linked to mutations in the CD2BP1/PSTPIP1 gene. We systematically screened this gene in French AA patients...
  66. Klötgen H, Beltraminelli H, Yawalkar N, van Gijn M, Holzinger D, Borradori L. The expanding spectrum of clinical phenotypes associated with PSTPIP1 mutations: from PAPA to PAMI syndrome and beyond. Br J Dermatol. 2017;: pubmed publisher
    Mutations in the PSTPIP1 gene encoding proline-serine-threonine-phosphatase interactive protein 1 were first identified in an autosomal dominant syndrome called PAPA associated with pyogenic sterile arthritis, pyoderma gangrenosum (PG) ..