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Exosome component 10

Known also as: Autoantigen PM/Scl 2, P100 polymyositis-scleroderma overlap syndrome-associated autoantigen, Polymyositis/scleroderma autoantigen 100 kDa, Polymyositis/scleroderma autoantigen 2

Known abbreviations: PM/Scl-100, PMSCL, PMSCL2, RRP6, EXOSC10


Putative catalytic component of the RNA exosome complex which has 3'->5' exoribonuclease activity and participates in a multitude of cellular RNA processing and degradation events. In the nucleus, the RNA exosome complex is involved in proper maturation of stable RNA species such as rRNA, snRNA and snoRNA, in the elimination of RNA processing by-products and non-coding 'pervasive' transcripts, such as antisense RNA species and promoter-upstream transcripts (PROMPTs), and of mRNAs with processing defects, thereby limiting or excluding their export to the cytoplasm. The RNA exosome may be involved in Ig class switch recombination (CSR) and/or Ig variable region somatic hypermutation (SHM) by targeting AICDA deamination activity to transcribed dsDNA substrates. In the cytoplasm, the RNA exosome complex is involved in general mRNA turnover and specifically degrades inherently unstable mRNAs containing AU-rich elements (AREs) within their 3' untranslated regions, and in RNA surveillance pathways, preventing translation of aberrant mRNAs. It seems to be involved in degradation of histone mRNA. EXOSC10 has 3'-5' exonuclease activity By similarity. EXOSC10 is required for nucleolar localization of C1D and probably mediates the association of SKIV2L2, C1D and MPP6 wth the RNA exosome involved in the maturation of 5.8S rRNA.


Component of the RNA exosome complex. The catalytically inactive RNA exosome core (Exo-9) complex is believed to associate with catalytic subunits EXOSC10, and DIS3 or DIS3L in cytoplasmic- and nuclear-specific RNA exosome complex forms. Interacts with C1D and MPHOSPH6.


Cytoplasm. Nucleus › nucleolus. Nucleus. Note: Strongly enriched in the nucleolus and a small amount has been found in cytoplasm supporting the existence of a nucleolar RNA exosome complex form.

This protein can be a part of a given complexes: Activities in which Exosome component 10 is involved: Pathways in which Exosome component 10 is involved:

NCBI GI number(s): 50301240
Species: Homo sapiens

Links to other databases:

Database ID Link
Uniprot Q01780 Q01780
KEGG hsa:5394 hsa:5394
PFAM: - Q01780 (Link - using uniprot id)
InterPro: - Q01780 (Link - using uniprot id)
CATH: - -
SCOP: - -
Solved crystal structures: 2CPR
[PDB] [details]
[PDB] [details]
[PDB] [details]
[PDB] [details]

Protein sequence:

Exosome component 10 (Homo sapiens) is product of expression of EXOSC10 exosome component 10 gene.


Title Authors Journal Publication date (Issue) PubMed ID
C1D and hMtr4p associate with the human exosome subunit PM/Scl-100 and are involved in pre-rRNA processing. Schilders G, van Dijk E, Pruijn GJ Nucleic Acids Res 2007-01-01 (35) 17412707
A protein interaction framework for human mRNA degradation. Lehner B, Sanderson CM Genome Res 2004-07-01 (14) 15231747
Adenylation and exosome-mediated degradation of cotranscriptionally cleaved pre-messenger RNA in human cells. West S, Gromak N, Norbury CJ, Proudfoot NJ Mol Cell 2006-01-03 (21) 16455498
The yeast exosome and human PM-Scl are related complexes of 3' --> 5' exonucleases. Allmang C, Petfalski E, Podtelejnikov A, Mann M, Tollervey D, Mitchell P Genes Dev 1999-08-15 (13) 10465791
The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, K Genome Res 2004-10-01 (14) 15489334
Degradation of histone mRNA requires oligouridylation followed by decapping and simultaneous degradation of the mRNA both 5' to 3' and 3' to 5'. Mullen TE, Marzluff WF Genes Dev 2008-02-01 (22) 18172165
Human cell growth requires a functional cytoplasmic exosome, which is involved in various mRNA decay pathways. van Dijk EL, Schilders G, Pruijn GJ RNA 2007-07-01 (13) 17545563
RNA exosome depletion reveals transcription upstream of active human promoters. Preker P, Nielsen J, Kammler S, Lykke-Andersen S, Christensen MS, Mapendano CK, Schierup MH, Jensen TH Science 2008-12-19 (322) 19056938
The human core exosome interacts with differentially localized processive RNases: hDIS3 and hDIS3L. Tomecki R, Kristiansen MS, Lykke-Andersen S, Chlebowski A, Larsen KM, Szczesny RJ, Drazkowska K, Pastula A, Andersen JS, Stepien PP, Dziembowski A, Jensen TH EMBO J 2010-07-21 (29) 20531386
Initial characterization of the human central proteome. Burkard TR, Planyavsky M, Kaupe I, Breitwieser FP, Burckstummer T, Bennett KL, Superti-Furga G, Colinge J BMC Syst Biol 2011-01-01 (5) 21269460
The DNA sequence and biological annotation of human chromosome 1. Gregory SG, Barlow KF, McLay KE, Kaul R, Swarbreck D, Dunham A, Scott CE, Howe KL, Woodfine K, Spencer CC Nature 2006-05-18 (441) 16710414
Nonsense-mediated mRNA decay in mammalian cells involves decapping, deadenylating, and exonucleolytic activities. Lejeune F, Li X, Maquat LE Mol Cell 2003-09-01 (12) 14527413
Functional proteomic analysis of human nucleolus. Scherl A, Coute Y, Deon C, Calle A, Kindbeiter K, Sanchez JC, Greco A, Hochstrasser D, Diaz JJ Mol Biol Cell 2002-11-01 (13) 12429849
Dis3-like 1: a novel exoribonuclease associated with the human exosome. Staals RH, Bronkhorst AW, Schilders G, Slomovic S, Schuster G, Heck AJ, Raijmakers R, Pruijn GJ EMBO J 2010-07-21 (29) 20531389
Cloning and characterization of the cDNA coding for a polymyositis-scleroderma overlap syndrome-related nucleolar 100-kD protein. Bluthner M, Bautz FA J Exp Med 1992-10-01 (176) 1383382
Cloning of a complementary DNA coding for the 100-kD antigenic protein of the PM-Scl autoantigen. Ge Q, Frank MB, O'Brien C, Targoff IN J Clin Invest 1992-08-01 (90) 1644924
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. Stover C, Endo Y, Takahashi M, Lynch NJ, Constantinescu C, Vorup-Jensen T, Thiel S, Friedl H, Hankeln T, Hall R, Gregory S, Fujita T, Schwaeble W Genes Immun 2001-05-01 (2) 11426320
A link between nuclear RNA surveillance, the human exosome and RNA polymerase II transcriptional termination. de Almeida SF, Garcia-Sacristan A, Custodio N, Carmo-Fonseca M Nucleic Acids Res 2010-12-01 (38) 20699273
Addition of poly(A) and poly(A)-rich tails during RNA degradation in the cytoplasm of human cells. Slomovic S, Fremder E, Staals RH, Pruijn GJ, Schuster G Proc Natl Acad Sci U S A 2010-04-20 (107) 20368444

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Last modification of this entry: Sept. 25, 2012.

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