Exosome component 9

Exosome component 9, also known as EXOSC9, is a human gene, the protein product of which is part of the exosome complex and is an autoantigen is patients with certain auto immune diseases, most notably scleromyositis.[5]

EXOSC9
Available structures PDB Ortholog search: PDBe RCSB List of PDB id codes 2NN6
Identifiers
Aliases	EXOSC9, PM/Scl-75, PMSCL1, RRP45, Rrp45p, p5, p6, Exosome component 9, PCH1D
External IDs	OMIM: 606180 MGI: 1355319 HomoloGene: 3693 GeneCards: EXOSC9
Gene location (Human) Chr. Chromosome 4 (human)[1] Band 4q27 Start 121,801,317 bp[1] End 121,817,021 bp[1]
Gene location (Mouse) Chr. Chromosome 3 (mouse)[2] Band 3|3 B Start 36,552,606 bp[2] End 36,565,727 bp[2]
RNA expression pattern More reference expression data
Gene ontology Molecular function • 3'-5'-exoribonuclease activity • exoribonuclease activity • AU-rich element binding • GO:0001948 protein binding • RNA polymerase II activating transcription factor binding • RNA binding • mRNA 3'-UTR AU-rich region binding Cellular component • cytoplasm • cytosol • exosome (RNase complex) • nuclear exosome (RNase complex) • nuclear chromosome • nucleolus • extracellular exosome • cell nucleus • nucleoplasm • cytoplasmic exosome (RNase complex) Biological process • RNA processing • regulation of mRNA stability • nuclear polyadenylation-dependent rRNA catabolic process • nuclear-transcribed mRNA catabolic process • positive regulation of cell growth • nuclear mRNA surveillance • immune response • exonucleolytic nuclear-transcribed mRNA catabolic process involved in deadenylation-dependent decay • positive regulation of transcription from RNA polymerase II promoter • rRNA processing • exonucleolytic trimming to generate mature 3'-end of 5.8S rRNA from tricistronic rRNA transcript (SSU-rRNA, 5.8S rRNA, LSU-rRNA) • nuclear-transcribed mRNA catabolic process, exonucleolytic, 3'-5' • U1 snRNA 3'-end processing • U4 snRNA 3'-end processing • U5 snRNA 3'-end processing • nuclear polyadenylation-dependent tRNA catabolic process • nuclear polyadenylation-dependent mRNA catabolic process • rRNA catabolic process Sources:Amigo / QuickGO
Orthologs
Species	Human	Mouse
Entrez	5393	50911
Ensembl	ENSG00000123737	ENSMUSG00000027714
UniProt	Q06265	Q9JHI7
RefSeq (mRNA)	NM_001034194 NM_005033	NM_019393
RefSeq (protein)	NP_001029366 NP_005024	NP_062266
Location (UCSC)	Chr 4: 121.8 – 121.82 Mb	Chr 3: 36.55 – 36.57 Mb
PubMed search	[3]	[4]
Wikidata
View/Edit Human View/Edit Mouse

References

1. GRCh38: Ensembl release 89: ENSG00000123737 - Ensembl, May 2017
2. GRCm38: Ensembl release 89: ENSMUSG00000027714 - Ensembl, May 2017
3. "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
4. "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
5. "Entrez Gene: EXOSC9 exosome component 9".

Further reading

• Alderuccio F, Chan EK, Tan EM (1991). "Molecular characterization of an autoantigen of PM-Scl in the polymyositis/scleroderma overlap syndrome: a unique and complete human cDNA encoding an apparent 75-kD acidic protein of the nucleolar complex". J. Exp. Med. 173 (4): 941–52. doi:10.1084/jem.173.4.941. PMC 2190817. PMID 2007859.
• Allmang C, Petfalski E, Podtelejnikov A, et al. (1999). "The yeast exosome and human PM–Scl are related complexes of 3′ → 5′ exonucleases". Genes Dev. 13 (16): 2148–58. doi:10.1101/gad.13.16.2148. PMC 316947. PMID 10465791.
• Brouwer R, Allmang C, Raijmakers R, et al. (2001). "Three novel components of the human exosome". J. Biol. Chem. 276 (9): 6177–84. doi:10.1074/jbc.M007603200. PMID 11110791.
• Chen CY, Gherzi R, Ong SE, et al. (2002). "AU binding proteins recruit the exosome to degrade ARE-containing mRNAs". Cell. 107 (4): 451–64. doi:10.1016/S0092-8674(01)00578-5. PMID 11719186.
• Mukherjee D, Gao M, O'Connor JP, et al. (2002). "The mammalian exosome mediates the efficient degradation of mRNAs that contain AU-rich elements". EMBO J. 21 (1–2): 165–74. doi:10.1093/emboj/21.1.165. PMC 125812. PMID 11782436.
• Raijmakers R, Noordman YE, van Venrooij WJ, Pruijn GJ (2002). "Protein-protein interactions of hCsl4p with other human exosome subunits". J. Mol. Biol. 315 (4): 809–18. doi:10.1006/jmbi.2001.5265. PMID 11812149.
• Brouwer R, Vree Egberts WT, Hengstman GJ, et al. (2002). "Autoantibodies directed to novel components of the PM/Scl complex, the human exosome". Arthritis Res. 4 (2): 134–8. doi:10.1186/ar389. PMC 83843. PMID 11879549.
• Raijmakers R, Egberts WV, van Venrooij WJ, Pruijn GJ (2002). "Protein-protein interactions between human exosome components support the assembly of RNase PH-type subunits into a six-membered PNPase-like ring". J. Mol. Biol. 323 (4): 653–63. doi:10.1016/S0022-2836(02)00947-6. PMID 12419256.
• Strausberg RL, Feingold EA, Grouse LH, et al. (2003). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences". Proc. Natl. Acad. Sci. U.S.A. 99 (26): 16899–903. doi:10.1073/pnas.242603899. PMC 139241. PMID 12477932.
• Raijmakers R, Egberts WV, van Venrooij WJ, Pruijn GJ (2003). "The association of the human PM/Scl-75 autoantigen with the exosome is dependent on a newly identified N terminus". J. Biol. Chem. 278 (33): 30698–704. doi:10.1074/jbc.M302488200. PMID 12788944.
• Lehner B, Semple JI, Brown SE, et al. (2004). "Analysis of a high-throughput yeast two-hybrid system and its use to predict the function of intracellular proteins encoded within the human MHC class III region". Genomics. 83 (1): 153–67. doi:10.1016/S0888-7543(03)00235-0. PMID 14667819.
• Raijmakers R, Renz M, Wiemann C, et al. (2004). "PM-Scl-75 is the main autoantigen in patients with the polymyositis/scleroderma overlap syndrome". Arthritis Rheum. 50 (2): 565–9. doi:10.1002/art.20056. PMID 14872500.
• Lehner B, Sanderson CM (2004). "A Protein Interaction Framework for Human mRNA Degradation". Genome Res. 14 (7): 1315–23. doi:10.1101/gr.2122004. PMC 442147. PMID 15231747.
• Andersen JS, Lam YW, Leung AK, et al. (2005). "Nucleolar proteome dynamics". Nature. 433 (7021): 77–83. doi:10.1038/nature03207. PMID 15635413.
• Tsang HT, Connell JW, Brown SE, et al. (2006). "A systematic analysis of human CHMP protein interactions: additional MIT domain-containing proteins bind to multiple components of the human ESCRT III complex". Genomics. 88 (3): 333–46. doi:10.1016/j.ygeno.2006.04.003. PMID 16730941.
• Olsen JV, Blagoev B, Gnad F, et al. (2006). "Global, in vivo, and site-specific phosphorylation dynamics in signaling networks". Cell. 127 (3): 635–48. doi:10.1016/j.cell.2006.09.026. PMID 17081983.
• van Dijk EL, Schilders G, Pruijn GJ (2007). "Human cell growth requires a functional cytoplasmic exosome, which is involved in various mRNA decay pathways". RNA. 13 (7): 1027–35. doi:10.1261/rna.575107. PMC 1894934. PMID 17545563.