Entity Details

Primary name EXOS8_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionQ96B26
EntryNameEXOS8_HUMAN
FullNameExosome complex component RRP43
TaxID9606
Evidenceevidence at protein level
Length276
SequenceStatuscomplete
DateCreated2002-07-11
DateModified2021-06-02

Ontological Relatives

GenesEXOSC8

GO terms

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GOName
GO:0000176 nuclear exosome (RNase complex)
GO:0000177 cytoplasmic exosome (RNase complex)
GO:0000178 exosome (RNase complex)
GO:0000467 exonucleolytic trimming to generate mature 3'-end of 5.8S rRNA from tricistronic rRNA transcript (SSU-rRNA, 5.8S rRNA, LSU-rRNA)
GO:0001650 fibrillar center
GO:0005634 nucleus
GO:0005654 nucleoplasm
GO:0005694 chromosome
GO:0005737 cytoplasm
GO:0005829 cytosol
GO:0006364 rRNA processing
GO:0016075 rRNA catabolic process
GO:0034427 nuclear-transcribed mRNA catabolic process, exonucleolytic, 3'-5'
GO:0034473 U1 snRNA 3'-end processing
GO:0034475 U4 snRNA 3'-end processing
GO:0034476 U5 snRNA 3'-end processing
GO:0035925 mRNA 3'-UTR AU-rich region binding
GO:0042802 identical protein binding
GO:0043231 intracellular membrane-bounded organelle
GO:0043488 regulation of mRNA stability
GO:0043928 exonucleolytic catabolism of deadenylated mRNA
GO:0071028 nuclear mRNA surveillance
GO:0071035 nuclear polyadenylation-dependent rRNA catabolic process
GO:0071038 nuclear polyadenylation-dependent tRNA catabolic process
GO:0071042 nuclear polyadenylation-dependent mRNA catabolic process

Subcellular Location

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Subcellular Location
Cytoplasm
Nucleus

Domains

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DomainNameCategoryType
IPR001247 Exoribonuclease, phosphorolytic domain 1DomainDomain
IPR015847 Exoribonuclease, phosphorolytic domain 2DomainDomain
IPR020568 Ribosomal protein S5 domain 2-type foldFamilyHomologous superfamily
IPR027408 PNPase/RNase PH domain superfamilyFamilyHomologous superfamily
IPR033196 Exosome complex component Rrp43FamilyFamily
IPR036345 Exoribonuclease, PH domain 2 superfamilyFamilyHomologous superfamily

Diseases

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Disease IDSourceNameDescription
616081 OMIMPontocerebellar hypoplasia 1C (PCH1C)A severe autosomal recessive neurodegenerative disease characterized by cerebellar and corpus callosum hypoplasia, abnormal myelination of the central nervous system, and spinal motor neuron disease. Affected individuals manifest failure to thrive, severe muscle weakness, spasticity and psychomotor retardation. Vision and hearing are impaired. The disease is caused by variants affecting the gene represented in this entry. EXOSC8 dysfunction causes myelin disruption through an imbalanced supply of myelin proteins due to dysregulation of their ARE-containing mRNAs (PubMed:24989451).

Interactions

79 interactions

InteractorPartnerSourcesPublicationsLink
EXOS8_HUMANEXOS5_HUMANBioGRID, HPRD, IntAct, MINT11719186 12419256 15231747 21516116 24981860 25416956 26186194 26344197 26496610 28514442 31515488 32296183 details
EXOS8_HUMANEXOS8_HUMANBioGRID, HPRD, IntAct15231747 25416956 32296183 details
EXOS8_HUMANTTP_HUMANBioGRID, HPRD, IntAct15231747 details
EXOS8_HUMANEXOS3_HUMANBioGRID, IntAct, MINT15231747 20531389 21255825 24981860 26186194 28514442 details
EXOS8_HUMANEXOS1_HUMANBioGRID, DIP, IntAct15231747 24981860 25043052 26186194 26344197 26496610 28514442 32296183 details
EXOS8_HUMANEXOSX_HUMANBioGRID, HPRD, IntAct15231747 21255825 24981860 26186194 26344197 28514442 29395067 details
EXOS8_HUMANRRP44_HUMANBioGRID, HPRD, IntAct, MINT11719186 15231747 20531386 details
EXOS8_HUMANLSM1_HUMANBioGRID, HPRD, IntAct15231747 details
EXOS8_HUMANLSM7_HUMANBioGRID, HPRD, IntAct15231747 details
EXOS8_HUMANXRN2_HUMANBioGRID, HPRD, IntAct15231747 details
EXOS8_HUMANRENT2_HUMANBioGRID, HPRD, IntAct15231747 details
EXOS8_HUMANFHOD1_HUMANBioGRID, HPRD, IntAct15231747 details
EXOS8_HUMANRASF1_HUMANBioGRID, HPRD, IntAct15231747 details
EXOS8_HUMANMKRN1_HUMANBioGRID, HPRD, IntAct15231747 details
EXOS8_HUMANCWC22_HUMANBioGRID, HPRD, IntAct15231747 details
EXOS8_HUMANFOXN3_HUMANBioGRID, HPRD, IntAct15231747 details
EXOS8_HUMANMTMR3_HUMANIntAct12419256 details
EXOS8_HUMANRSMB_HUMANBioGRID, IntAct, MINT21516116 32296183 details
EXOS8_HUMANTXND9_HUMANBioGRID, IntAct25416956 32296183 details
EXOS8_HUMANATF2_HUMANBioGRID, IntAct25416956 details
EXOS8_HUMANCOX5A_HUMANBioGRID, IntAct25416956 details
EXOS8_HUMANTFAP4_HUMANBioGRID, IntAct25416956 32296183 details
EXOS8_HUMANOTUD4_HUMANBioGRID, IntAct25416956 32296183 details
EXOS8_HUMANFRG1_HUMANBioGRID, IntAct25416956 details
EXOS8_HUMANTCEA2_HUMANBioGRID, IntAct25416956 details
EXOS8_HUMANCONA1_HUMANBioGRID, IntAct25416956 details
EXOS8_HUMANF90A1_HUMANBioGRID, IntAct25416956 32296183 details
EXOS8_HUMANAEN_HUMANBioGRID, IntAct25416956 details
EXOS8_HUMANREL_HUMANBioGRID, IntAct25416956 details
EXOS8_HUMANTXD17_HUMANBioGRID, IntAct25416956 32296183 details
EXOS8_HUMANDUS23_HUMANBioGRID, IntAct25416956 25910212 32296183 details
EXOS8_HUMANDPYL1_HUMANBioGRID, IntAct24722188 details
EXOS8_HUMANEXOS9_HUMANBioGRID, HPRD, IntAct11719186 12788944 24981860 26186194 26496610 28514442 details
EXOS8_HUMANRXRB_HUMANBioGRID, IntAct26871637 details
EXOS8_HUMANSPC1L_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANRASD1_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANMORN4_HUMANBioGRID, IntAct25416956 32296183 details
EXOS8_HUMANLNX1_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANSOCS7_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANNTAQ1_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANGEM_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANHXB9_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANKCJ11_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANMYOZ1_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANLMO4_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANCASL_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANCNNM3_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANRUSC1_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANRFC5_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANLSM4_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANKANK2_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANPIAS2_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANSLAI1_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANSNAI1_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANRU1C_HUMANBioGRID, IntAct25416956 32296183 details
EXOS8_HUMANPF21A_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANRPP14_HUMANBioGRID, HPRD, IntAct11929972 32296183 details
EXOS8_HUMANFX4L1_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANF161B_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANUBP2_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANMACIR_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANCV039_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANHPLN2_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANINCA1_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANDDT4L_HUMANBioGRID, IntAct32296183 details
EXOS8_HUMANHBS1L_HUMANBioGRID, IntAct26186194 27345150 28514442 details
EXOS8_HUMANMPH6_HUMANBioGRID, IntAct24981860 26186194 26344197 28514442 28877463 details
EXOS8_HUMANEXOS6_HUMANBioGRID, HPRD11719186 12419256 15231747 24981860 details
EXOS8_HUMANUBC_HUMANBioGRID16196087 23314748 details
EXOS8_HUMANXRN1_HUMANBioGRID, HPRD15231747 details
EXOS8_HUMANUBP6_HUMANBioGRID25416956 32296183 details
EXOS8_HUMANPDIP3_HUMANBioGRID32296183 details
EXOS8_HUMANEXOS4_HUMANBioGRID, HPRD, IntAct, MINT11719186 20531386 22939629 24981860 26186194 26496610 28514442 details
EXOS8_HUMANEXOS7_HUMANBioGRID, HPRD, IntAct11719186 24981860 26186194 28514442 details
EXOS8_HUMANVHL_HUMANBioGRID7604013 details
EXOS8_HUMANMET22_HUMANBioGRID23349634 details
EXOS8_HUMANDAA10_HUMANBioGRID23844004 details
EXOS8_HUMANMTREX_HUMANBioGRID, HPRD11719186 24981860 26186194 details
EXOS8_HUMANPALS2_HUMANHPRD11719186 details