Exosome Component 10 (EXOSC10)

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PMSCL2; PM2; PM/Scl-100; Rrp6p; RRP6; p2; p3; p4; Polymyositis/Scleroderma Autoantigen 2; P100 polymyositis-scleroderma overlap syndrome-associated autoantigen

Exosome Component 10 (EXOSC10)

Antinucleolar antibodies are found in patients with scleroderma or polymyositis or in patients with overlap of these 2 syndromes. The 2 most prominent proteins in this PMSCL autoantigen complex, PMSCL1 and PMSCL2, are detected as 75- and 100-kD molecules, respectively. The deduced 860-amino acid protein contains 2 potential N-glycosylation sites and multiple potential phosphorylation sites. Northern blot analysis revealed expression of a 2.7-kb transcript. Immunoblot analysis confirmed the identity of native and recombinant 100-kD PMSCL2 proteins.

Sequence analysis predicted that the 885-amino acid protein contains a stretch of amino acids also found in serine/threonine kinases. Epitope mapping indicated a major epitope is localized between residues 153 and 324.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Exosome Component 10 (EXOSC10) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Exosome Component 10 (EXOSC10) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Exosome Component 10 (EXOSC10) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Exosome Component 10 (EXOSC10) CLIA Kit Customized Service Offer
n/a ELISA Kit for Exosome Component 10 (EXOSC10) ELISA Kit Customized Service Offer

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Exosome Component 10 (EXOSC10) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Exosome Component 10 (EXOSC10) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Exosome Component 10 (EXOSC10) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Exosome Component 10 (EXOSC10) CLIA Kit Customized Service Offer
n/a ELISA Kit for Exosome Component 10 (EXOSC10) ELISA Kit Customized Service Offer
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  2. "Cloning of a complementary DNA coding for the 100-kD antigenic protein of the PM-Scl autoantigen."J. Clin. Invest. 90:559-570(1992) [PubMed] [Europe PMC] [Abstract]
  3. "The human gene for mannan-binding lectin-associated serine protease-2 (MASP-2), the effector component of the lectin route of complement activation, is part of a tightly linked gene cluster on chromosome 1p36.2-3."Genes Immun. 2:119-127(2001) [PubMed] [Europe PMC] [Abstract]
  4. "The DNA sequence and biological annotation of human chromosome 1." Nature 441:315-321(2006) [PubMed] [Europe PMC] [Abstract]
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  7. "Functional proteomic analysis of human nucleolus."Mol. Biol. Cell 13:4100-4109(2002) [PubMed] [Europe PMC] [Abstract]
  8. "Nonsense-mediated mRNA decay in mammalian cells involves decapping, deadenylating, and exonucleolytic activities."Mol. Cell 12:675-687(2003) [PubMed] [Europe PMC] [Abstract]
  9. "A protein interaction framework for human mRNA degradation."Genome Res. 14:1315-1323(2004) [PubMed] [Europe PMC] [Abstract]
  10. "Adenylation and exosome-mediated degradation of cotranscriptionally cleaved pre-messenger RNA in human cells."Mol. Cell 21:437-443(2006) [PubMed] [Europe PMC] [Abstract]
  11. "The Spt6 SH2 domain binds Ser2-P RNAPII to direct Iws1-dependent mRNA splicing and export."Genes Dev. 21:160-174(2007) [PubMed] [Europe PMC] [Abstract]
  12. "C1D and hMtr4p associate with the human exosome subunit PM/Scl-100 and are involved in pre-rRNA processing."Nucleic Acids Res. 35:2564-2572(2007) [PubMed] [Europe PMC] [Abstract]
  13. "Human cell growth requires a functional cytoplasmic exosome, which is involved in various mRNA decay pathways."RNA 13:1027-1035(2007) [PubMed] [Europe PMC] [Abstract]
  14. "Degradation of histone mRNA requires oligouridylation followed by decapping and simultaneous degradation of the mRNA both 5' to 3' and 3' to 5'."Genes Dev. 22:50-65(2008) [PubMed] [Europe PMC] [Abstract]
  15. "RNA exosome depletion reveals transcription upstream of active human promoters."Science 322:1851-1854(2008) [PubMed] [Europe PMC] [Abstract]
  16. "The human core exosome interacts with differentially localized processive RNases: hDIS3 and hDIS3L."EMBO J. 29:2342-2357(2010) [PubMed] [Europe PMC] [Abstract]
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  18. "A link between nuclear RNA surveillance, the human exosome and RNA polymerase II transcriptional termination."Nucleic Acids Res. 38:8015-8026(2010) [PubMed] [Europe PMC] [Abstract]
  19. "Addition of poly(A) and poly(A)-rich tails during RNA degradation in the cytoplasm of human cells."Proc. Natl. Acad. Sci. U.S.A. 107:7407-7412(2010) [PubMed] [Europe PMC] [Abstract]
  20. "Initial characterization of the human central proteome."BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
  21. "Solution structure of the HRDC domain of human exosome component 10."Submitted (NOV-2005) to the PDB data bank