The world's first wiki where authorship really matters (Nature Genetics, 2008). Due credit and reputation for authors. Imagine a global collaborative knowledge base for original thoughts. Search thousands of articles and collaborate with scientists around the globe.

wikigene or wiki gene protein drug chemical gene disease author authorship tracking collaborative publishing evolutionary knowledge reputation system wiki2.0 global collaboration genes proteins drugs chemicals diseases compound
Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)



Gene Review

EXOSC9  -  exosome component 9

Homo sapiens

Synonyms: Autoantigen PM/Scl 1, Exosome complex component RRP45, Exosome component 9, P75 polymyositis-scleroderma overlap syndrome-associated autoantigen, PM/Scl-75, ...
Welcome! If you are familiar with the subject of this article, you can contribute to this open access knowledge base by deleting incorrect information, restructuring or completely rewriting any text. Read more.

Disease relevance of EXOSC9


High impact information on EXOSC9


Chemical compound and disease context of EXOSC9


Biological context of EXOSC9

  • We have previously demonstrated that PM-Scl-75, a component of the human exosome complex involved in RNA maturation and mRNA decay, can specifically interact with RNAs containing an AU-rich instability element [9].
  • As such, it may be deduced that PMSCL1 sequences map to human chromosome 4q27 and the proximal portion of mouse chromosome (Chr) 3 where human and mouse cyclin A2 genes reside [10].
  • In the case of C8166 cells, both a K197R and a E49K mutation were identified within the capsid (CA) protein and the p6 protein of Gag, respectively, while the other point mutation (A423G) was found within the putative DIS loop [11].
  • The system required one of the p5 Rep proteins encoded by AAV (either Rep78 or Rep68) and a crude adenovirus (Ad)-infected HeLa cell cytoplasmic extract to catalyze origin of replication-dependent AAV DNA replication [12].
  • Here we show that mutation of a single tyrosine residue, p5, in the carboxyl terminus of the erbB oncogene product allows it to become sarcomagenic in vivo and to transform fibroblasts in vitro [13].

Anatomical context of EXOSC9

  • Approximately 30% of analogues at either Leu57 (p1), Ala60 (p4), or Asn62 (p6) residues exhibited TCR agonism to stimulate various magnitudes of proliferative responses in the T cell clone, and analogues exhibiting TCR antagonism are rare in these three residues [14].
  • The data show that during chronic exposure of HeLa cells to AZT, a compensatory response was induced at the earlier passages (p5-p37), and by p71 there was widespread mitochondrial morphological damage, severe mtDNA depletion, and a substantial loss of mitochondrial membrane potential [15].
  • METHODS: The trial was undertaken at a maternity teaching hospital in Adelaide, Australia, where 593 women less than 14 weeks' pregnant with symptoms of nausea or vomiting were randomized into 4 groups: traditional acupuncture, pericardium 6 (p6) acupuncture, sham acupuncture, or no acupuncture (control) [16].
  • Another gag gene product, p6, plays an integral role in successful viral budding from the plasma membrane and inclusion of the accessory protein Vpr within newly budding virions [17].

Associations of EXOSC9 with chemical compounds

  • RESULTS: IgE reactivities to new allergen components were not detected by CAP in treated patients after 15 weeks and a cumulative dose of approximately 65 microg of the major allergen Phl p 5 [18].
  • Rep78 and Rep68 mutants deleted for the methionine at residue 225 showed decreased abilities to down-regulate both p5 and p19 transcript levels, while mutants containing a substitution of glycine for the methionine resembled the wild-type Rep78 [19].
  • 9. Although there is evidence for a helix-forming tendency in the N-terminus of the protein, the experiments indicate that p6* has no overall stable secondary or tertiary structure with the single tryptophan exposed in aqueous solution [20].

Other interactions of EXOSC9

  • While looking for proteins that bind to E12 and E47 by the yeast interaction trap, we isolated the rat (r) homologue of the human (h) polymyositis-scleroderma autoantigen (PM-Scl), which has been localized to the granular layer of the nucleolus and to distinct nucleocytoplasmic foci [7].

Analytical, diagnostic and therapeutic context of EXOSC9

  • METHODS: Serum samples obtained from patients with the PM/scleroderma overlap syndrome and from patients with several other diseases were analyzed for the presence of autoantibodies reactive with recombinant PM-Scl-100 and PM-Scl-75 (both the original and the longer form) proteins, in an enzyme-linked immunosorbent assay (ELISA) [1].
  • Der p 5-specific immunological responses including changes to specific immunoglobulin G and E (IgE) levels, the presence of cells in the bronchoalveolar lavage fluid (BALF), and AHR were assessed following this inhalational challenge [21].
  • Women who received p6 acupuncture (p < 0.05) reported less nausea from the second week of the trial, and less dry retching (p < 0.001) from the third week compared with women in the no acupuncture control group [16].
  • CEA was not detected after passage 5 (p5) but the concentration of CA19-9 did not decrease further after the first few serial passages in protein-free medium [22].
  • Based on frequent co-sensitization patterns determined in 200 grass pollen-allergic patients, three recombinant hybrid molecules were developed by polymerase chain reaction-based mending of cDNAs coding for the major timothy grass pollen allergens (Phl p 1, Phl p 2, Phl p 5, Phl p 6) for vaccination against grass pollen allergy [23].


  1. PM-Scl-75 is the main autoantigen in patients with the polymyositis/scleroderma overlap syndrome. Raijmakers, R., Renz, M., Wiemann, C., Egberts, W.V., Seelig, H.P., van Venrooij, W.J., Pruijn, G.J. Arthritis Rheum. (2004) [Pubmed]
  2. IgE reactivity pattern to timothy and birch pollen allergens in Finnish and Russian Karelia. Movérare, R., Petays, T., Vartiainen, E., Haahtela, T. Int. Arch. Allergy Immunol. (2005) [Pubmed]
  3. Solution structure of the human immunodeficiency virus type 1 p6 protein. Fossen, T., Wray, V., Bruns, K., Rachmat, J., Henklein, P., Tessmer, U., Maczurek, A., Klinger, P., Schubert, U. J. Biol. Chem. (2005) [Pubmed]
  4. Novel Gag-Pol frameshift site in human immunodeficiency virus type 1 variants resistant to protease inhibitors. Doyon, L., Payant, C., Brakier-Gingras, L., Lamarre, D. J. Virol. (1998) [Pubmed]
  5. Interaction with the p6 domain of the gag precursor mediates incorporation into virions of Vpr and Vpx proteins from primate lentiviruses. Selig, L., Pages, J.C., Tanchou, V., Prévéral, S., Berlioz-Torrent, C., Liu, L.X., Erdtmann, L., Darlix, J., Benarous, R., Benichou, S. J. Virol. (1999) [Pubmed]
  6. Antisense-mediated silencing of a gene encoding a major ryegrass pollen allergen. Bhalla, P.L., Swoboda, I., Singh, M.B. Proc. Natl. Acad. Sci. U.S.A. (1999) [Pubmed]
  7. The polymyositis-scleroderma autoantigen interacts with the helix-loop-helix proteins E12 and E47. Kho, C.J., Huggins, G.S., Endege, W.O., Patterson, C., Jain, M.K., Lee, M.E., Haber, E. J. Biol. Chem. (1997) [Pubmed]
  8. Vaccination with gp120-depleted HIV-1 plus immunostimulatory CpG oligodeoxynucleotides in incomplete Freund's adjuvant stimulates cellular and humoral immunity in rhesus macaques. Silvera, P., Savary, J.R., Livingston, V., White, J., Manson, K.H., Wyand, M.H., Salk, P.L., Moss, R.B., Lewis, M.G. Vaccine (2004) [Pubmed]
  9. Sequence-specific RNA binding mediated by the RNase PH domain of components of the exosome. Anderson, J.R., Mukherjee, D., Muthukumaraswamy, K., Moraes, K.C., Wilusz, C.J., Wilusz, J. RNA (2006) [Pubmed]
  10. Structure and localization of mouse Pmscl1 and Pmscl2 genes. Bliskovski, V., Liddell, R., Ramsay, E.S., Miller, M.J., Mock, B.A. Genomics (2000) [Pubmed]
  11. Partial restoration of replication of simian immunodeficiency virus by point mutations in either the dimerization initiation site (DIS) or Gag region after deletion mutagenesis within the DIS. Guan, Y., Diallo, K., Detorio, M., Whitney, J.B., Liang, C., Wainberg, M.A. J. Virol. (2001) [Pubmed]
  12. Cellular proteins required for adeno-associated virus DNA replication in the absence of adenovirus coinfection. Ni, T.H., McDonald, W.F., Zolotukhin, I., Melendy, T., Waga, S., Stillman, B., Muzyczka, N. J. Virol. (1998) [Pubmed]
  13. A minor tyrosine phosphorylation site located within the CAIN domain plays a critical role in regulating tissue-specific transformation by erbB kinase. Chang, C.M., Shu, H.K., Ravi, L., Pelley, R.J., Shu, H., Kung, H.J. J. Virol. (1995) [Pubmed]
  14. Response of a human T cell clone to a large panel of altered peptide ligands carrying single residue substitutions in an antigenic peptide: characterization and frequencies of TCR agonism and TCR antagonism with or without partial activation. Chen, Y.Z., Matsushita, S., Nishimura, Y. J. Immunol. (1996) [Pubmed]
  15. Morphological and molecular course of mitochondrial pathology in cultured human cells exposed long-term to Zidovudine. Divi, R.L., Haverkos, K.J., Humsi, J.A., Shockley, M.E., Thamire, C., Nagashima, K., Olivero, O.A., Poirier, M.C. Environ. Mol. Mutagen. (2007) [Pubmed]
  16. Acupuncture to treat nausea and vomiting in early pregnancy: a randomized controlled trial. Smith, C., Crowther, C., Beilby, J. Birth (Berkeley, Calif.) (2002) [Pubmed]
  17. Molecular characterization of the HIV-1 gag nucleocapsid gene associated with vertical transmission. Wellensiek, B.P., Sundaravaradan, V., Ramakrishnan, R., Ahmad, N. Retrovirology (2006) [Pubmed]
  18. Evaluation of recombinant and native timothy pollen (rPhl p 1, 2, 5, 6, 7, 11, 12 and nPhl p 4)- specific IgG4 antibodies induced by subcutaneous immunotherapy with timothy pollen extract in allergic patients. Rossi, R.E., Monasterolo, G. Int. Arch. Allergy Immunol. (2004) [Pubmed]
  19. Analysis of adeno-associated virus (AAV) wild-type and mutant Rep proteins for their abilities to negatively regulate AAV p5 and p19 mRNA levels. Kyöstiö, S.R., Owens, R.A., Weitzman, M.D., Antoni, B.A., Chejanovsky, N., Carter, B.J. J. Virol. (1994) [Pubmed]
  20. Sequence-specific resonance assignments of the 1H-NMR spectra and structural characterization in solution of the HIV-1 transframe protein p6. Beissinger, M., Paulus, C., Bayer, P., Wolf, H., Rösch, P., Wagner, R. Eur. J. Biochem. (1996) [Pubmed]
  21. Inhibition of allergen-induced airway inflammation and hyperreactivity by recombinant lactic-acid bacteria. Charng, Y.C., Lin, C.C., Hsu, C.H. Vaccine (2006) [Pubmed]
  22. Protein-free culture of the human pancreatic cancer cell line, SUIT-2. Taniguchi, S., Iwamura, T., Kitamura, N., Yamanari, H., Kojima, A., Hidaka, K., Seguchi, K., Setoguchi, T. Hum. Cell (1994) [Pubmed]
  23. Combination vaccines for the treatment of grass pollen allergy consisting of genetically engineered hybrid molecules with increased immunogenicity. Linhart, B., Jahn-Schmid, B., Verdino, P., Keller, W., Ebner, C., Kraft, D., Valenta, R. FASEB J. (2002) [Pubmed]
WikiGenes - Universities