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Details on Person UniProt:Q9NQT4 EXOSC5

Class:IdReferenceGeneProduct:72247
_chainChangeLogchain:1-235 added on Fri February 6 2015
_displayNameUniProt:Q9NQT4 EXOSC5
_timestamp2025-02-21 19:47:39
chainchain:1-235
checksumC3C1FCB39D85B1EE
commentFUNCTION Non-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. The catalytic inactive RNA exosome core complex of 9 subunits (Exo-9) is proposed to play a pivotal role in the binding and presentation of RNA for ribonucleolysis, and to serve as a scaffold for the association with catalytic subunits and accessory proteins or complexes (PubMed:11782436, PubMed:21269460). In vitro, EXOSC5 does not bind or digest single-stranded RNA and binds to double-stranded DNA without detectable DNase activity (PubMed:20660080).SUBUNIT Homodimer (PubMed:20660080). Component of the RNA exosome core complex (Exo-9), composed of EXOSC1, EXOSC2, EXOSC3, EXOSC4, EXOSC5, EXOSC6, EXOSC7, EXOSC8 and EXOSC9; within the complex interacts with EXOSC3, EXOSC8, and EXOSC9 (PubMed:29906447, PubMed:30047866). The catalytically inactive RNA exosome core complex (Exo-9) associates with the catalytic subunit EXOSC10/RRP6 (PubMed:11719186, PubMed:20531389, PubMed:29906447). Exo-9 may associate with DIS3 to form the nucleolar exosome complex, or DIS3L to form the cytoplasmic exosome complex (PubMed:11719186, PubMed:20531389, PubMed:29906447). Exo-9 is formed by a hexameric base ring consisting of the heterodimers EXOSC4-EXOSC9, EXOSC5-EXOSC8 and EXOSC6-EXOSC7, and a cap ring consisting of EXOSC1, EXOSC2 and EXOSC3 (PubMed:11719186, PubMed:20531389, PubMed:30047866). The RNA exosome complex associates with cofactors C1D/RRP47, MPHOSPH6/MPP6 and MTREX/MTR4 (PubMed:30047866). Interacts with GTPBP1 (PubMed:21515746). Interacts with ZC3HAV1 (PubMed:17185417). Interacts with DDX17 only in the presence of ZC3HAV1 in an RNA-independent manner (PubMed:18334637).INTERACTION Highly expressed in a variety of hematopoietic and epithelial tumor cell lines, but not in normal hematopoietic tissues or other normal tissue, with the exception of testis.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the RNase PH family.CAUTION The six exosome core subunits containing a RNase PH-domain are not phosphorolytically active.SEQUENCE CAUTION Extended N-terminus.
descriptionrecommendedName: Exosome complex component RRP46 alternativeName: Chronic myelogenous leukemia tumor antigen 28 alternativeName: Exosome component 5 alternativeName: Ribosomal RNA-processing protein 46 alternativeName: p12B
geneNameEXOSC5
CML28
RRP46
identifierQ9NQT4
isSequenceChangedFALSE
keyword3D-structure
Cytoplasm
Disease variant
DNA-binding
Exosome
Intellectual disability
Nucleus
Phosphoprotein
Proteomics identification
Reference proteome
rRNA processing
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9862192] Weiser, Joel, 2024-02-26
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9939033] Weiser, Joel, 2025-02-21
nameEXOSC5
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8993605] ENSEMBL:ENSG00000077348 EXOSC5 [Homo sapiens]
secondaryIdentifierEXOS5_HUMAN
Q32Q81
Q8NG16
Q96I89
sequenceLength235
species[Species:48887] Homo sapiens
(referenceEntity)[EntityWithAccessionedSequence:429889] EXOSC5 [cytosol] [Homo sapiens]
[EntityWithAccessionedSequence:6791550] EXOSC5 [nucleoplasm] [Homo sapiens]
[EntityWithAccessionedSequence:9935829] EXOSC5 [nucleolus] [Homo sapiens]
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No pathways have been reviewed or authored by UniProt:Q9NQT4 EXOSC5 (72247)