Reactome: A Curated Pathway Database
THIS SITE IS USED FOR CURATION AND TESTING
IT IS NOT STABLE, IS LINKED TO AN INCOMPLETE DATA SET, AND IS NOT MONITORED FOR PERFORMANCE. WE STRONGLY RECOMMEND THE USE OF OUR PUBLIC SITE

Query author contributions in Reactome

Reactome depends on collaboration between our curation team and outside experts to assemble and peer-review its pathway modules. The integration of ORCID within Reactome enables us to meet a key challenge with authoring, curating and reviewing biological information by incentivizing and crediting the external experts that contribute their expertise and time to the Reactome curation process. More information is available at ORCID and Reactome.

If you have an ORCID ID that is not listed on this page, please forward this information to us and we will update your Reactome pathway records.

Name Email address

Details on Person UniProt:Q9Y2L1 DIS3

Class:IdReferenceGeneProduct:72245
_chainChangeLogchain:1-958 added on Sat February 7 2015
_displayNameUniProt:Q9Y2L1 DIS3
_timestamp2026-02-20 21:34:05
chainchain:1-958
checksum92336BA0D06247FE
commentFUNCTION Putative 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. DIS3 has both 3'-5' exonuclease and endonuclease activities.COFACTOR Component of the RNA exosome complex; within the complex interacts with EXOSC4, EXOSC7 and EXOSC9 of the exosome core complex (Exo-9) (PubMed:29906447, PubMed:30047866). The catalytically inactive RNA exosome core complex (Exo-9) associates with the catalytic subunit EXOSC10/RRP6 (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: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; DIS3 associates with the base ring of Exo-9 (PubMed:20531389, PubMed:30047866). The RNA exosome complex associates with cofactors C1D/RRP47, MPHOSPH6/MPP6 and MTREX/MTR4 (PubMed:30047866). Interacts with DHX34; the interaction is RNA-independent (PubMed:25220460).SUBCELLULAR LOCATION Predominantly located in the nucleus (PubMed:20531386). According to PubMed:12429849, found in the nucleolus (PubMed:12429849). According to PubMed:20531386, excluded from nucleolus supporting the existence of a nucleolar RNA exosome complex devoid of DIS3 (PubMed:20531386).ALTERNATIVE PRODUCTS Widely expressed.MISCELLANEOUS The association of DIS3 with the RNA exosome complex appears to be weak explaining its absence in some complex purifications.SIMILARITY Belongs to the RNR ribonuclease family.SEQUENCE CAUTION Extended N-terminus.
descriptionrecommendedName: Exosome complex exonuclease RRP44 ecNumber: 3.1.13.- ecNumber: 3.1.26.- alternativeName: Protein DIS3 homolog alternativeName: Ribosomal RNA-processing protein 44
geneNameDIS3
KIAA1008
RRP44
identifierQ9Y2L1
isSequenceChangedFALSE
keyword3D-structure
Acetylation
Alternative splicing
Cytoplasm
Endonuclease
Exonuclease
Exosome
Hydrolase
Magnesium
Manganese
Nuclease
Nucleus
Phosphoprotein
Proteomics identification
Reference proteome
RNA-binding
rRNA processing
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9939033] Weiser, Joel, 2025-02-21
[InstanceEdit:9983091] Weiser, Joel, 2026-02-20
nameDIS3
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8989196] ENSEMBL:ENSG00000083520 DIS3 [Homo sapiens]
secondaryIdentifierRRP44_HUMAN
A6NI21
B2RBL2
Q5W0P7
Q5W0P8
Q658Z7
Q7Z481
Q8WWI2
Q9UG36
sequenceLength958
species[Species:48887] Homo sapiens
(isoformParent)[ReferenceIsoform:246512] UniProt:Q9Y2L1-2 DIS3 [Homo sapiens]
[ReferenceIsoform:415959] UniProt:Q9Y2L1-1 DIS3 [Homo sapiens]
(referenceEntity)[EntityWithAccessionedSequence:429935] DIS3 [cytosol] [Homo sapiens]
[EntityWithAccessionedSequence:6791517] DIS3 [nucleoplasm] [Homo sapiens]
[EntityWithAccessionedSequence:9935841] DIS3 [nucleolus] [Homo sapiens]
[Change default viewing format]
No pathways have been reviewed or authored by UniProt:Q9Y2L1 DIS3 (72245)