Exosome component 10

Exosome component 10, also known as EXOSC10, 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]

EXOSC10
Available structures PDB Ortholog search: PDBe RCSB List of PDB id codes 2CPR, 3SAF, 3SAG, 3SAH
Identifiers
Aliases	EXOSC10, PM-Scl, PM/Scl-100, PMSCL, PMSCL2, RRP6, Rrp6p, p2, p3, p4, exosome component 10
External IDs	OMIM: 605960 MGI: 1355322 HomoloGene: 31105 GeneCards: EXOSC10
Gene location (Human) Chr. Chromosome 1 (human)[1] Band 1p36.22 Start 11,066,618 bp[1] End 11,099,881 bp[1]
Gene location (Mouse) Chr. Chromosome 4 (mouse)[2] Band 4|4 E2 Start 148,558,429 bp[2] End 148,582,401 bp[2]
RNA expression pattern More reference expression data
Gene ontology Molecular function • nucleotide binding • exoribonuclease activity • GO:0001948 protein binding • catalytic activity • 3'-5' exonuclease activity • nucleic acid binding • nuclease activity • exonuclease activity • hydrolase activity • RNA binding • 3'-5'-exoribonuclease activity • telomerase RNA binding • single-stranded RNA binding Cellular component • cytoplasm • exosome (RNase complex) • membrane • nuclear exosome (RNase complex) • intracellular • nucleolus • transcriptionally active chromatin • cell nucleus • nucleoplasm • cytosol Biological process • RNA processing • nuclear polyadenylation-dependent rRNA catabolic process • nucleic acid phosphodiester bond hydrolysis • nuclear retention of unspliced pre-mRNA at the site of transcription • CUT catabolic process • nuclear-transcribed mRNA catabolic process • nuclear mRNA surveillance • cell metabolism • maturation of 5.8S rRNA • histone mRNA catabolic process • dosage compensation by inactivation of X chromosome • nucleobase-containing compound metabolic process • rRNA processing • RNA phosphodiester bond hydrolysis, exonucleolytic • nuclear-transcribed mRNA catabolic process, nonsense-mediated decay • negative regulation of telomere maintenance via telomerase • regulation of telomerase RNA localization to Cajal body • exonucleolytic trimming to generate mature 3'-end of 5.8S rRNA from tricistronic rRNA transcript (SSU-rRNA, 5.8S rRNA, LSU-rRNA) • nuclear polyadenylation-dependent snoRNA catabolic process • nuclear polyadenylation-dependent snRNA catabolic process • nuclear polyadenylation-dependent tRNA catabolic process • nuclear polyadenylation-dependent CUT catabolic process • nuclear polyadenylation-dependent antisense transcript catabolic process • polyadenylation-dependent snoRNA 3'-end processing Sources:Amigo / QuickGO
Orthologs
Species	Human	Mouse
Entrez	5394	50912
Ensembl	ENSG00000171824	ENSMUSG00000017264
UniProt	Q01780	P56960
RefSeq (mRNA)	NM_001001998 NM_002685	NM_016699 NM_001355489 NM_001355490
RefSeq (protein)	NP_001001998 NP_002676	NP_057908 NP_001342418 NP_001342419
Location (UCSC)	Chr 1: 11.07 – 11.1 Mb	Chr 4: 148.56 – 148.58 Mb
PubMed search	[3]	[4]
Wikidata
View/Edit Human View/Edit Mouse

References

1. GRCh38: Ensembl release 89: ENSG00000171824 - Ensembl, May 2017
2. GRCm38: Ensembl release 89: ENSMUSG00000017264 - 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: EXOSC10 exosome component 10".

Further reading

• Blüthner M, Bautz FA (1992). "Cloning and characterization of the cDNA coding for a polymyositis-scleroderma overlap syndrome-related nucleolar 100-kD protein". J. Exp. Med. 176 (4): 973–80. doi:10.1084/jem.176.4.973. PMC 2119404. PMID 1383382.
• Ge Q, Frank MB, O'Brien C, Targoff IN (1992). "Cloning of a complementary DNA coding for the 100-kD antigenic protein of the PM-Scl autoantigen". J. Clin. Invest. 90 (2): 559–70. doi:10.1172/JCI115895. PMC 443135. PMID 1644924.
• 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.
• Stover C, Endo Y, Takahashi M, et al. (2001). "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 (3): 119–27. doi:10.1038/sj.gene.6363745. PMID 11426320.
• 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.
• 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.
• Scherl A, Couté Y, Déon C, et al. (2003). "Functional proteomic analysis of human nucleolus". Mol. Biol. Cell. 13 (11): 4100–9. doi:10.1091/mbc.E02-05-0271. PMC 133617. PMID 12429849.
• 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.
• Lejeune F, Li X, Maquat LE (2003). "Nonsense-mediated mRNA decay in mammalian cells involves decapping, deadenylating, and exonucleolytic activities". Mol. Cell. 12 (3): 675–87. doi:10.1016/S1097-2765(03)00349-6. PMID 14527413.
• Ota T, Suzuki Y, Nishikawa T, et al. (2004). "Complete sequencing and characterization of 21,243 full-length human cDNAs". Nat. Genet. 36 (1): 40–5. doi:10.1038/ng1285. PMID 14702039.
• Bouwmeester T, Bauch A, Ruffner H, et al. (2004). "A physical and functional map of the human TNF-alpha/NF-kappa B signal transduction pathway". Nat. Cell Biol. 6 (2): 97–105. doi:10.1038/ncb1086. PMID 14743216.
• 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.
• Gerhard DS, Wagner L, Feingold EA, et al. (2004). "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)". Genome Res. 14 (10B): 2121–7. doi:10.1101/gr.2596504. PMC 528928. PMID 15489334.
• 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.