Gene Symbol: MUPP1
Description: multiple PDZ domain crumbs cell polarity complex component
Alias: HYC2, MUPP1, multiple PDZ domain protein, multi-PDZ domain protein 1
Species: human
Products:     MUPP1

Top Publications

  1. Barritt D, Pearn M, Zisch A, Lee S, Javier R, Pasquale E, et al. The multi-PDZ domain protein MUPP1 is a cytoplasmic ligand for the membrane-spanning proteoglycan NG2. J Cell Biochem. 2000;79:213-24 pubmed
    ..sequence to a DNA segment coding for the most amino-terminal of the 13 PDZ domains found in the multi-PDZ-protein MUPP1. Antibodies made against recombinant polypeptides representing these two clones (NIP-2 and NIP-7) are reactive ..
  2. Hamazaki Y, Itoh M, Sasaki H, Furuse M, Tsukita S. Multi-PDZ domain protein 1 (MUPP1) is concentrated at tight junctions through its possible interaction with claudin-1 and junctional adhesion molecule. J Biol Chem. 2002;277:455-61 pubmed
    ..Using the yeast two-hybrid system, we identified ZO-1 and MUPP1 (multi-PDZ domain protein 1) as binding partners for the COOH terminus of claudin-1...
  3. Ullmer C, Schmuck K, Figge A, Lubbert H. Cloning and characterization of MUPP1, a novel PDZ domain protein. FEBS Lett. 1998;424:63-8 pubmed
    ..The protein, named MUPP1 (multi-PDZ-domain protein), contains thirteen PDZ domains and no obvious catalytic domain; it is related to hINADL ..
  4. Becamel C, Figge A, Poliak S, Dumuis A, Peles E, Bockaert J, et al. Interaction of serotonin 5-hydroxytryptamine type 2C receptors with PDZ10 of the multi-PDZ domain protein MUPP1. J Biol Chem. 2001;276:12974-82 pubmed
    ..system, we previously isolated a cDNA clone encoding a novel member of the multivalent PDZ protein family called MUPP1 containing 13 PDZ domains...
  5. Jansen F, Kalbe B, Scholz P, Fränzel B, Osterloh M, Wolters D, et al. Biochemical Large-Scale Interaction Analysis of Murine Olfactory Receptors and Associated Signaling Proteins with Post-Synaptic Density 95, Drosophila Discs Large, Zona-Occludens 1 (PDZ) Domains. Mol Cell Proteomics. 2015;14:2072-84 pubmed publisher
    ..This system includes the 13 individual PDZ domains of the multiple PDZ domain protein 1 (MUPP1; the largest PDZ protein), a broad range of murine olfactory receptors (a multifaceted gene ..
  6. Yang J, Simonneau C, Kilker R, Oakley L, Byrne M, Nichtova Z, et al. Murine MPDZ-linked hydrocephalus is caused by hyperpermeability of the choroid plexus. EMBO Mol Med. 2019;11: pubmed publisher
    ..These conclusions are supported by ultrastructural evidence, and by immunohistochemistry and cytology data. Our results provide a straightforward and concise explanation for the pathophysiology of Mpdz-linked hydrocephalus. ..
  7. Tanabe Y, Fujita Jimbo E, Momoi M, Momoi T. CASPR2 forms a complex with GPR37 via MUPP1 but not with GPR37(R558Q), an autism spectrum disorder-related mutation. J Neurochem. 2015;134:783-93 pubmed publisher
    ..CASPR2 forms a complex with receptors via interaction with multiple PDZ domain protein 1 (MUPP1)...
  8. Sommer B, Kormeier B, Demenkov P, Arrigo P, Hippe K, Ates O, et al. Subcellular localization charts: a new visual methodology for the semi-automatic localization of protein-related data sets. J Bioinform Comput Biol. 2013;11:1340005 pubmed publisher
    ..As an application case, a MUPP1-related protein-protein interaction network is localized and semi-automatically analyzed...
  9. Baliova M, Juhasova A, Jursky F. Using a collection of MUPP1 domains to investigate the similarities of neurotransmitter transporters C-terminal PDZ motifs. Biochem Biophys Res Commun. 2014;454:25-9 pubmed publisher
    ..compared their interactions with the natural collection of all 13 PDZ domains of the largest PDZ binding protein MUPP1. The GABA, glycine and serotonin transporters showed unique binding preferences scattered over one or several ..

More Information


  1. Zhu H, Liu Z, Huang Y, Zhang C, Li G, Liu W. Biochemical and structural characterization of MUPP1-PDZ4 domain from Mus musculus. Acta Biochim Biophys Sin (Shanghai). 2015;47:199-206 pubmed publisher
    ..Multi-PDZ-domain protein 1 (MUPP1), as a scaffold protein, contains 13 PDZ domains and plays an important role in cytoskeletal organization, cell ..
  2. Kruse L, Walter N, Buck K. Mpdz expression in the caudolateral substantia nigra pars reticulata is crucially involved in alcohol withdrawal. Genes Brain Behav. 2014;13:769-76 pubmed publisher
    Association studies implicate the multiple PDZ domain protein (MUPP1/MPDZ) gene in risk for alcoholism in humans and alcohol withdrawal in mice...
  3. MILNER L, Shirley R, Kozell L, Walter N, Kruse L, Komiyama N, et al. Novel MPDZ/MUPP1 transgenic and knockdown models confirm Mpdz's role in ethanol withdrawal and support its role in voluntary ethanol consumption. Addict Biol. 2015;20:143-7 pubmed publisher
    Association studies implicate multiple PDZ domain protein (MPDZ/MUPP1) sequence and/or expression in risk for alcoholism in humans and ethanol withdrawal (EW) in mice, but confirmation has been hindered by the dearth of targeted genetic ..
  4. Wang Y, Ma L, Lu S, Chan T, Yam J, Tang S, et al. Analysis of multiple genetic loci reveals MPDZ-NF1B rs1324183 as a putative genetic marker for keratoconus. Br J Ophthalmol. 2018;102:1736-1741 pubmed publisher
    ..It is thus a putative genetic marker for monitoring the progression of keratoconus to a severe form and facilitating early intervention. ..
  5. Levran O, Peles E, Randesi M, Correa Da Rosa J, Ott J, Rotrosen J, et al. Synaptic Plasticity and Signal Transduction Gene Polymorphisms and Vulnerability to Drug Addictions in Populations of European or African Ancestry. CNS Neurosci Ther. 2015;21:898-904 pubmed publisher
    ..rs3915568 in NTSR1, which encodes neurotensin receptor, and SNP rs1389752 in MPDZ, which encodes the multiple PDZ domain protein, were previously associated with heroin addiction or alcohol addiction, respectively...
  6. Abu Amero K, Helwa I, Al Muammar A, Strickland S, Hauser M, Allingham R, et al. Case-control association between CCT-associated variants and keratoconus in a Saudi Arabian population. J Negat Results Biomed. 2015;14:10 pubmed publisher
    ..This report aims at supporting the possible association between CCT-associated SNPs and KC susceptibility. ..
  7. Feldner A, Adam M, Tetzlaff F, Moll I, Komljenovic D, Sahm F, et al. Loss of Mpdz impairs ependymal cell integrity leading to perinatal-onset hydrocephalus in mice. EMBO Mol Med. 2017;9:890-905 pubmed publisher
    Hydrocephalus is a common congenital anomaly. LCAM1 and MPDZ (MUPP1) are the only known human gene loci associated with non-syndromic hydrocephalus...
  8. Yoo E, Park K, Won H, Park J, Park J, Lee S, et al. Genetic Characteristics of Polycythemia Vera and Essential Thrombocythemia in Korean Patients. J Clin Lab Anal. 2016;30:1061-1070 pubmed publisher
    ..014). This study was the first comprehensive investigation of the genetic characteristics of Korean patients with PV and ET. We found that somatic mutations and the 46 haplotype contribute to PV and ET pathogenesis in Korean patients. ..
  9. Al Jezawi N, Al Shamsi A, Suleiman J, Ben Salem S, John A, Vijayan R, et al. Compound heterozygous variants in the multiple PDZ domain protein (MPDZ) cause a case of mild non-progressive communicating hydrocephalus. BMC Med Genet. 2018;19:34 pubmed publisher
    ..The compound heterozygous variants identified in this study are the most likely cause of CH in the affected child. The study further confirms MPDZ as a gene underlying some CH cases. ..
  10. Saugier Veber P, Marguet F, Lecoquierre F, Adle Biassette H, Guimiot F, Cipriani S, et al. Hydrocephalus due to multiple ependymal malformations is caused by mutations in the MPDZ gene. Acta Neuropathol Commun. 2017;5:36 pubmed publisher
    ..MPDZ also named MUPP1 is an essential component of tight junctions which are expressed from early brain development in the choroid ..
  11. Donaldson R, Sun Y, Liang D, Zheng M, Sahbaie P, Dill D, et al. The multiple PDZ domain protein Mpdz/MUPP1 regulates opioid tolerance and opioid-induced hyperalgesia. BMC Genomics. 2016;17:313 pubmed publisher
    ..The strongest of these was the highly polymorphic Mpdz gene coding for the post-synaptic scaffolding protein Mpdz/MUPP1. Heterozygous Mpdz +/- mice displayed reduced opioid tolerance and OIH...
  12. van de Pavert S, Kantardzhieva A, Malysheva A, Meuleman J, Versteeg I, Levelt C, et al. Crumbs homologue 1 is required for maintenance of photoreceptor cell polarization and adhesion during light exposure. J Cell Sci. 2004;117:4169-77 pubmed
    ..We show that it is associated here with multiple PDZ protein 1 (Mupp1), protein associated with Lin-7 (Pals1 or Mpp5) and Mpp4...
  13. Zhang Y, Han Z, Sun Y. Structure-based identification of CaMKIIα-interacting MUPP1 PDZ domains and rational design of peptide ligands to target such interaction in human fertilization. Amino Acids. 2016;48:1509-21 pubmed publisher
    ..between Ca(2+)/calmodulin-activated protein kinase II-α (CaMKIIα) and multi-PDZ domain protein 1 (MUPP1) plays an important role in sperm acrosome reaction and human fertilization, which is mediated by the binding of ..
  14. Sindic A, Huang C, Chen A, Ding Y, Miller Little W, Che D, et al. MUPP1 complexes renal K+ channels to alter cell surface expression and whole cell currents. Am J Physiol Renal Physiol. 2009;297:F36-45 pubmed publisher
    ..We identified two independent partial cDNAs corresponding to the COOH-terminal 900 AA of MUPP1, a protein containing 13 PDZ binding domains that is expressed in the kidney in tight junctions and lateral ..
  15. Karpyak V, Kim J, Biernacka J, Wieben E, Mrazek D, Black J, et al. Sequence variations of the human MPDZ gene and association with alcoholism in subjects with European ancestry. Alcohol Clin Exp Res. 2009;33:712-21 pubmed publisher
    ..Further functional genomic analysis of MPDZ variants and investigation of their association with a broader array of alcoholism-related phenotypes could reveal additional genetic markers of alcoholism. ..
  16. Parker L, Backstrom J, Sanders Bush E, Shieh B. Agonist-induced phosphorylation of the serotonin 5-HT2C receptor regulates its interaction with multiple PDZ protein 1. J Biol Chem. 2003;278:21576-83 pubmed
    b>Multiple PDZ domain protein 1 (MUPP1), a putative scaffolding protein containing 13 PSD-95, Dlg, ZO-1 (PDZ) domains, was identified by a yeast two-hybrid screen as a serotonin2C receptor (5-HT2C R)-interacting protein (Ullmer, C...
  17. Vassilev A, Kaneko K, Shu H, Zhao Y, DePamphilis M. TEAD/TEF transcription factors utilize the activation domain of YAP65, a Src/Yes-associated protein localized in the cytoplasm. Genes Dev. 2001;15:1229-41 pubmed
    ..Because TEAD-dependent transcription was limited by YAP65, and YAP65 also binds Src/Yes protein tyrosine kinases, we propose that YAP65 regulates TEAD-dependent transcription in response to mitogenic signals. ..
  18. Hao X, Chen P, Chen Z, Li S, Wang Y. Evaluating the Association between Keratoconus and Reported Genetic Loci in a Han Chinese Population. Ophthalmic Genet. 2015;36:132-6 pubmed publisher
  19. Dooley R, Baumgart S, Rasche S, Hatt H, Neuhaus E. Olfactory receptor signaling is regulated by the post-synaptic density 95, Drosophila discs large, zona-occludens 1 (PDZ) scaffold multi-PDZ domain protein 1. FEBS J. 2009;276:7279-90 pubmed publisher
    ..In the present study, we show that multi-PDZ domain protein 1 (MUPP1) is expressed in the apical compartment of olfactory sensory neurons...
  20. Coffill C, Muller P, Oh H, Neo S, Hogue K, Cheok C, et al. Mutant p53 interactome identifies nardilysin as a p53R273H-specific binding partner that promotes invasion. EMBO Rep. 2012;13:638-44 pubmed publisher
    ..Advanced proteomics has thus allowed the detection of a new mechanism of p53-driven invasion. ..
  21. Al Dosari M, Al Owain M, Tulbah M, Kurdi W, Adly N, Al Hemidan A, et al. Mutation in MPDZ causes severe congenital hydrocephalus. J Med Genet. 2013;50:54-8 pubmed publisher
    ..Remarkably, we have also identified the same founder mutation in a stillbirth with massive congenital hydrocephalus from another family. Our data strongly support the candidacy of MPDZ as a novel congenital hydrocephalus disease gene. ..
  22. Assemat E, Crost E, Ponserre M, Wijnholds J, Le Bivic A, Massey Harroche D. The multi-PDZ domain protein-1 (MUPP-1) expression regulates cellular levels of the PALS-1/PATJ polarity complex. Exp Cell Res. 2013;319:2514-25 pubmed publisher
    ..All together these data indicate that a precise balance between MUPP-1 and PATJ is achieved in epithelial cells by regulating their association with PALS-1. ..
  23. Mancini A, Koch A, Stefan M, Niemann H, Tamura T. The direct association of the multiple PDZ domain containing proteins (MUPP-1) with the human c-Kit C-terminus is regulated by tyrosine kinase activity. FEBS Lett. 2000;482:54-8 pubmed
    ..Deletion of V967 of c-Kit abolished binding to MUPP-1 and drastically reduced its tyrosine kinase activity, suggesting that the structure of the C-terminal tail of c-Kit influences its enzymatic activity. ..
  24. Huang Y, Liu W, Han F, Yang J, Hao X, Chen H, et al. Copy number variations and expression of MPDZ are prognostic biomarkers for clear cell renal cell carcinoma. Oncotarget. 2017;8:78713-78725 pubmed publisher
    ..002). These results indicated that low MPDZ expression associated with CNV is a potential biomarker for the prognosis of ccRCC patients...
  25. Lee S, Glaunsinger B, Mantovani F, Banks L, Javier R. Multi-PDZ domain protein MUPP1 is a cellular target for both adenovirus E4-ORF1 and high-risk papillomavirus type 18 E6 oncoproteins. J Virol. 2000;74:9680-93 pubmed
    ..motifs of Ad9 E4-ORF1 and high-risk HPV-18 E6 also mediate binding to the widely expressed cellular factor MUPP1, a large multi-PDZ domain protein predicted to function as an adapter in signal transduction...
  26. Funk A, Rumbaugh G, Harotunian V, McCullumsmith R, Meador Woodruff J. Decreased expression of NMDA receptor-associated proteins in frontal cortex of elderly patients with schizophrenia. Neuroreport. 2009;20:1019-22 pubmed publisher
    ..Postsynaptic density protein 95 (PSD95), Synaptic GTPase-activating protein (SynGAP), and Multiple PDZ domain protein (MUPP1) are integral components of the NMDA receptor signaling complex, and help facilitate signaling, ..
  27. Griffon N, Jeanneteau F, Prieur F, Diaz J, Sokoloff P. CLIC6, a member of the intracellular chloride channel family, interacts with dopamine D(2)-like receptors. Brain Res Mol Brain Res. 2003;117:47-57 pubmed
    ..We isolated three partial cDNAs encoding, respectively, for the MUPP1 multi-PDZ protein, for the N-terminal region of radixin, for GIPC and for a 160-amino acid open reading frame ..
  28. Kimber W, Trinkle Mulcahy L, Cheung P, Deak M, Marsden L, Kieloch A, et al. Evidence that the tandem-pleckstrin-homology-domain-containing protein TAPP1 interacts with Ptd(3,4)P2 and the multi-PDZ-domain-containing protein MUPP1 in vivo. Biochem J. 2002;361:525-36 pubmed
    ..A search for proteins that interact with TAPP1 identified the multi-PDZ-containing protein termed 'MUPP1', a protein possessing 13 PDZ domains and no other known modular or catalytic domains [PDZ is postsynaptic density ..
  29. Karpyak V, Geske J, Colby C, Mrazek D, Biernacka J. Genetic variability in the NMDA-dependent AMPA trafficking cascade is associated with alcohol dependence. Addict Biol. 2012;17:798-806 pubmed publisher
    ..The statistically significant association of alcohol dependence with genetic variation in the NMDA-dependent AMPA receptor trafficking cascade indicates a need for further investigation of the role of this pathway in alcohol dependence. ..
  30. Heydecke D, Meyer D, Ackermann F, Wilhelm B, Gudermann T, Boekhoff I. The multi PDZ domain protein MUPP1 as a putative scaffolding protein for organizing signaling complexes in the acrosome of mammalian spermatozoa. J Androl. 2006;27:390-404 pubmed
    ..of the RT-PCR approaches were performed with specific primer pairs for the vertebrate INAD-like PDZ domain protein MUPP1. The results revealed that this scaffolding protein, which comprises 13 different PDZ domains, is expressed in ..
  31. Poliak S, Matlis S, Ullmer C, Scherer S, Peles E. Distinct claudins and associated PDZ proteins form different autotypic tight junctions in myelinating Schwann cells. J Cell Biol. 2002;159:361-72 pubmed
    ..PATJ is mainly found at the paranodal loops, where it colocalized with claudin-1. MUPP1 and claudin-5 colocalized in the incisures, and the COOH-terminal region of claudin-5 interacts with MUPP1 in a ..
  32. Wells C, Fawcett J, Traweger A, Yamanaka Y, Goudreault M, Elder K, et al. A Rich1/Amot complex regulates the Cdc42 GTPase and apical-polarity proteins in epithelial cells. Cell. 2006;125:535-48 pubmed
    ..We propose that Rich1 and Amot maintain TJ integrity by the coordinate regulation of Cdc42 and by linking specific components of the TJ to intracellular protein trafficking. ..
  33. Boulkour Touioui S, Zaraî Jaouadi N, Bouacem K, Ben Ayed R, Rekik H, Zenati B, et al. Biochemical and molecular characterization of a novel metalloprotease from Pseudomonas fluorescens strain TBS09. Int J Biol Macromol. 2018;107:2351-2363 pubmed publisher
    ..These data constitute an essential first step towards an understanding of the properties of MPDZ enzyme. ..
  34. Kimber W, Deak M, Prescott A, Alessi D. Interaction of the protein tyrosine phosphatase PTPL1 with the PtdIns(3,4)P2-binding adaptor protein TAPP1. Biochem J. 2003;376:525-35 pubmed
    ..Consistent with this notion we observed RNA-interference-mediated knock-down of TAPP1 in HEK-293 cells, enhanced activation and phosphorylation of PKB following IGF1 stimulation. ..
  35. Guillaume J, Daulat A, Maurice P, Levoye A, Migaud M, Brydon L, et al. The PDZ protein mupp1 promotes Gi coupling and signaling of the Mt1 melatonin receptor. J Biol Chem. 2008;283:16762-71 pubmed publisher
    ..The ubiquitously expressed multi-PDZ protein MUPP1, which is composed of 13 PDZ domains, has been shown to interact with multiple viral and cellular proteins and to ..
  36. Jiang X, An W, Yang X, Lin J, Ma S, Wang D, et al. Asymmetric distribution of CRUMBS polarity complex proteins from compacted 8-cell to blastocyst stage during mouse preimplantation development. Gene Expr Patterns. 2018;27:93-98 pubmed publisher
    ..The present study provides a foundation for further investigation on the functions of CRUMBS polarity complex in trophectoderm specification and blastocyst morphogenesis. ..
  37. Okuhira K, Fitzgerald M, Sarracino D, Manning J, Bell S, Goss J, et al. Purification of ATP-binding cassette transporter A1 and associated binding proteins reveals the importance of beta1-syntrophin in cholesterol efflux. J Biol Chem. 2005;280:39653-64 pubmed
    ..The approach used to identify beta1-syntrophin as an ABCA1-binding protein should prove useful in elucidating other protein interactions upon which ABCA1 function depends...
  38. Krapivinsky G, Medina I, Krapivinsky L, Gapon S, Clapham D. SynGAP-MUPP1-CaMKII synaptic complexes regulate p38 MAP kinase activity and NMDA receptor-dependent synaptic AMPA receptor potentiation. Neuron. 2004;43:563-74 pubmed
    ..hippocampal neurons, SynGAP and CaMKII are brought together by direct physical interaction with the PDZ domains of MUPP1, and in this complex, SynGAP is phosphorylated...
  39. Niggeler A, Tetens J, Stäuble A, Steiner A, Drogemuller C. A genome-wide significant association on chromosome 2 for footrot resistance/susceptibility in Swiss White Alpine sheep. Anim Genet. 2017;48:712-715 pubmed publisher
    ..the multiple PDZ domain crumbs cell polarity complex component protein, also known as multi-PDZ domain protein 1 (MUPP1). This protein is possibly involved in maintaining the barrier function and integrity of tight junctions...
  40. Bazellieres E, Aksenova V, Barthelemy Requin M, Massey Harroche D, Le Bivic A. Role of the Crumbs proteins in ciliogenesis, cell migration and actin organization. Semin Cell Dev Biol. 2018;81:13-20 pubmed publisher
    ..These are the Par complex including aPKC, PAR3 and PAR6 and the Crumbs complex including, CRUMBS, PALS1 and PATJ/MUPP1. These two complexes interact directly and in addition to their already well described functions, they play a role ..
  41. Coyne C, Voelker T, Pichla S, Bergelson J. The coxsackievirus and adenovirus receptor interacts with the multi-PDZ domain protein-1 (MUPP-1) within the tight junction. J Biol Chem. 2004;279:48079-84 pubmed
    ..In a yeast two-hybrid screen we identified the multi-PDZ domain protein MUPP1 as an interaction partner for the CAR cytoplasmic domain...
  42. Adachi M, Hamazaki Y, Kobayashi Y, Itoh M, Tsukita S, Furuse M, et al. Similar and distinct properties of MUPP1 and Patj, two homologous PDZ domain-containing tight-junction proteins. Mol Cell Biol. 2009;29:2372-89 pubmed publisher
    b>MUPP1 and Patj are both composed of an L27 domain and multiple PDZ domains (13 and 10 domains, respectively) and are localized to tight junctions (TJs) in epithelial cells...
  43. Sewduth R, Kovacic H, Jaspard Vinassa B, Jecko V, Wavasseur T, Fritsch N, et al. PDZRN3 destabilizes endothelial cell-cell junctions through a PKC?-containing polarity complex to increase vascular permeability. Sci Signal. 2017;10: pubmed publisher
    ..regulated Wnt signaling and associated with a complex containing PAR3, PKC?, and the multi-PDZ domain protein MUPP1 (Discs Lost-multi-PDZ domain protein 1) and targeted MUPP1 for proteasomal degradation in transfected cells...
  44. Liew C, Vockel M, Glassmeier G, Brandner J, Fernandez Ballester G, Schwarz J, et al. Interaction of the human somatostatin receptor 3 with the multiple PDZ domain protein MUPP1 enables somatostatin to control permeability of epithelial tight junctions. FEBS Lett. 2009;583:49-54 pubmed publisher
    ..We identify here an interaction between the human somatostatin receptor 3 (hSSTR3) and the multiple PDZ protein MUPP1. MUPP1 is a tight junction scaffold protein in epithelial cells, and as a result of the interaction with MUPP1 the ..
  45. Shaheen R, Sebai M, Patel N, Ewida N, Kurdi W, Altweijri I, et al. The genetic landscape of familial congenital hydrocephalus. Ann Neurol. 2017;81:890-897 pubmed publisher
    ..Our study highlights the importance of recessive mutations in familial congenital hydrocephalus and expands the locus heterogeneity of this condition. Ann Neurol 2017;81:890-897. ..