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Details on Person UniProt:Q01780 EXOSC10

Class:Id	ReferenceGeneProduct:72237
_chainChangeLog	chain:1-885 added on Fri February 6 2015
_displayName	UniProt:Q01780 EXOSC10
_timestamp	2025-02-21 19:27:35
chain	chain:1-885
checksum	A37BDC8F49BF2E57
comment	FUNCTION 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. Part of the small subunit (SSU) processome, first precursor of the small eukaryotic ribosomal subunit. During the assembly of the SSU processome in the nucleolus, many ribosome biogenesis factors, an RNA chaperone and ribosomal proteins associate with the nascent pre-rRNA and work in concert to generate RNA folding, modifications, rearrangements and cleavage as well as targeted degradation of pre-ribosomal RNA by the RNA exosome (PubMed:34516797). 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 is required for nucleolar localization of C1D and probably mediates the association of MTREX, C1D and MPHOSPH6 with the RNA exosome involved in the maturation of 5.8S rRNA. Plays a role in the recruitment of replication protein A complex (RPA) and RAD51 to DNA double-strand breaks caused by irradiation, contributing to DNA repair by homologous recombination (PubMed:25632158, PubMed:31086179). Regulates levels of damage-induced RNAs in order to prevent DNA-RNA hybrid formation at DNA double-strand breaks and limit DNA end resection after damage (PubMed:31086179). Plays a role in oocyte development, maturation and survival (By similarity). Required for normal testis development and mitotic division of spermatogonia (By similarity). Plays a role in proper embryo development (By similarity). Required for global protein translation (PubMed:26857222, PubMed:36912080). Required for cell proliferation (PubMed:36912080). Regulates metabolism of C9orf72-derived repeat RNA that can be translated into toxic dipeptide repeat proteins (PubMed:32830871).COFACTOR Arginine-rich dipeptide repeat proteins expressed from C9orf72-derived repeat RNA interact with EXOSC10 and inhibit its ability to promote degradation of this RNA.SUBUNIT Component of the RNA exosome complex (PubMed:20531389, PubMed:26166824, PubMed:29906447). The catalytically inactive RNA exosome core complex (Exo-9) associates with the catalytic subunit EXOSC10/RRP6 (via its N-terminus) (PubMed:20531389, PubMed:29906447, PubMed:30047866). Exo-9 may associate with DIS3 to form the nucleolar exosome complex, or DIS3L to form the cytoplasmic exosome complex (PubMed:20531389, PubMed:29906447). The RNA exosome complex interacts with cofactors C1D/RRP47, MPHOSPH6/MPP6 and MTREX/MTR4 (PubMed:17412707, PubMed:26166824, PubMed:30047866). Interacts with MTREX; the interaction with MTREX mediates the association of MTREX with nuclear RNA exosomes (PubMed:26166824). Part of the small subunit (SSU) processome, composed of more than 70 proteins and the RNA chaperone small nucleolar RNA (snoRNA) U3 (PubMed:34516797). Interacts with ALYREF/THOC4 (PubMed:17234882). Interacts with DHX36; this interaction occurs in a RNase-insensitive manner (PubMed:14731398). Interacts with NRDE2 (PubMed:30538148). Interacts (via C-terminus) with USP36 (via C-terminus); the interaction is facilitated by the association with RNA and promotes sumoylation of EXOSC10 (PubMed:36912080).INTERACTION Strongly enriched in the nucleolus and a small amount has been found in cytoplasm supporting the existence of a nucleolar RNA exosome complex form (PubMed:20531386, PubMed:34516797). Arginine-rich dipeptide repeat proteins expressed from C9orf72-derived repeat RNA cause diffuse nuclear misdistribution of EXOSC10 (PubMed:32830871). Relocates to the DNA double-strand breaks in response to irradiation (PubMed:31086179).ALTERNATIVE PRODUCTS Down-regulated by mild hypothermia (at protein level).PTM Sumoylated by USP36; sumoylation does not significantly affect EXOSC10 nucleolar localization and association with core exosome and USP36, but regulates the nucleolar RNA exosome activity in rRNA processing by promoting binding of EXOSC10 to pre-rRNAs (PubMed:36912080). Effects of sumoylation on EXOSC10 levels vary between different studies (PubMed:26857222, PubMed:36912080). Sumoylation of EXOSC10 is required for the modulation of EXOSC10 effects on cellular protein translation and cell proliferation (PubMed:36912080). Sumoylation is promoted by mild hypothermia (PubMed:26857222).SIMILARITY Belongs to the exosome component 10/RRP6 family.
description	recommendedName: fullName evidence="30"Exosome complex component 10 ecNumber evidence="17"3.1.13.- alternativeName: Autoantigen PM/Scl 2 alternativeName: P100 polymyositis-scleroderma overlap syndrome-associated autoantigen alternativeName: Polymyositis/scleroderma autoantigen 100 kDa shortName: PM/Scl-100 alternativeName: Polymyositis/scleroderma autoantigen 2
geneName	EXOSC10 PMSCL PMSCL2 RRP6
identifier	Q01780
isSequenceChanged	FALSE
keyword	3D-structure Alternative splicing Cytoplasm DNA damage DNA repair Exonuclease Exosome Hydrolase Isopeptide bond Magnesium Metal-binding Nonsense-mediated mRNA decay Nuclease Nucleus Phosphoprotein Proteomics identification Reference proteome RNA-binding rRNA processing Ubl conjugation
modified	[InstanceEdit:9836292] Weiser, Joel, 2023-05-25 [InstanceEdit:9852000] Weiser, Joel, 2023-11-03 [InstanceEdit:9862192] Weiser, Joel, 2024-02-26 [InstanceEdit:9917590] Weiser, Joel, 2024-08-09 [InstanceEdit:9926675] Weiser, Joel, 2024-11-03 [InstanceEdit:9939033] Weiser, Joel, 2025-02-21
name	EXOSC10
referenceDatabase	[ReferenceDatabase:2] UniProt
referenceGene	[ReferenceDNASequence:9000036] ENSEMBL:ENSG00000171824 EXOSC10 [Homo sapiens]
secondaryIdentifier	EXOSX_HUMAN B1AKQ0 B1AKQ1 Q15158
sequenceLength	885
species	[Species:48887] Homo sapiens
(isoformParent)	[ReferenceIsoform:152259] UniProt:Q01780-2 EXOSC10 [Homo sapiens] [ReferenceIsoform:405042] UniProt:Q01780-1 EXOSC10 [Homo sapiens]
(referenceEntity)	[EntityWithAccessionedSequence:430026] EXOSC10 [cytosol] [Homo sapiens] [EntityWithAccessionedSequence:6791547] EXOSC10 [nucleoplasm] [Homo sapiens] [EntityWithAccessionedSequence:9935809] EXOSC10 [nucleolus] [Homo sapiens]
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No pathways have been reviewed or authored by UniProt:Q01780 EXOSC10 (72237)