Exosome component 9

Exosome component 9, also known as EXOSC9, is a human gene, the protein product of which (sometimes called RRP45 or PM/Scl-75) is part of the exosome complex and is an autoantigen is patients with certain auto immune diseases, most notably scleromyositis.

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; OMA:EXOSC9 - orthologs
Gene location (Human) Chr. Chromosome 4 (human) Band 4q27 Start 121,801,318 bp End 121,817,021 bp
Gene location (Mouse) Chr. Chromosome 3 (mouse) Band 3|3 B Start 36,606,755 bp End 36,619,876 bp
RNA expression pattern Bgee Human Mouse (ortholog) Top expressed in secondary oocyte Achilles tendon gonad testicle buccal mucosa cell left testis right testis ventricular zone ganglionic eminence lymph node Top expressed in otic vesicle otic placode saccule Paneth cell ventricular zone trigeminal ganglion spermatocyte tail of embryo endothelial cell of lymphatic vessel condyle More reference expression data BioGPS More reference expression data
Gene ontology Molecular function 3'-5'-exoribonuclease activity exoribonuclease activity protein 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 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 catabolism of deadenylated mRNA positive regulation of transcription by RNA polymerase II 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: 36.61 – 36.62 Mb PubMed search
Wikidata
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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. hdl:20.500.11820/a03680a4-cf3a-4827-805c-447483ff4b9f. 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. S2CID 14817671.
• 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. hdl:2066/261980. 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. hdl:2066/186665. 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. Bibcode:2002PNAS...9916899M. 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. hdl:2066/186428. 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. Bibcode:2005Natur.433...77A. doi:10.1038/nature03207. PMID 15635413. S2CID 4344740.
• 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. S2CID 7827573.
• 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.