Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

Exosome component 10

Gene

EXOSC10

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

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. EXOSC10 has 3'-5' exonuclease activity (By similarity). EXOSC10 is required for nucleolar localization of C1D and probably mediates the association of SKIV2L2, C1D and MPP6 wth the RNA exosome involved in the maturation of 5.8S rRNA.By similarity8 Publications

GO - Molecular functioni

  • 3'-5'-exoribonuclease activity Source: Reactome
  • exoribonuclease activity Source: UniProtKB
  • nucleotide binding Source: InterPro
  • poly(A) RNA binding Source: UniProtKB

GO - Biological processi

  • CUT catabolic process Source: UniProtKB
  • dosage compensation by inactivation of X chromosome Source: Ensembl
  • histone mRNA catabolic process Source: UniProtKB
  • maturation of 5.8S rRNA Source: UniProtKB
  • nuclear mRNA surveillance Source: UniProtKB
  • nuclear polyadenylation-dependent rRNA catabolic process Source: UniProtKB
  • nuclear retention of unspliced pre-mRNA at the site of transcription Source: UniProtKB
  • nuclear-transcribed mRNA catabolic process Source: UniProtKB
  • nuclear-transcribed mRNA catabolic process, nonsense-mediated decay Source: UniProtKB
  • rRNA processing Source: Reactome
Complete GO annotation...

Keywords - Molecular functioni

Exonuclease, Hydrolase, Nuclease

Keywords - Biological processi

Nonsense-mediated mRNA decay, rRNA processing

Keywords - Ligandi

RNA-binding

Enzyme and pathway databases

BioCyciZFISH:ENSG00000171824-MONOMER.
ReactomeiR-HSA-6791226. Major pathway of rRNA processing in the nucleolus and cytosol.

Names & Taxonomyi

Protein namesi
Recommended name:
Exosome component 10 (EC:3.1.13.-)
Alternative name(s):
Autoantigen PM/Scl 2
P100 polymyositis-scleroderma overlap syndrome-associated autoantigen
Polymyositis/scleroderma autoantigen 100 kDa
Short name:
PM/Scl-100
Polymyositis/scleroderma autoantigen 2
Gene namesi
Name:EXOSC10
Synonyms:PMSCL, PMSCL2, RRP6
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 1

Organism-specific databases

HGNCiHGNC:9138. EXOSC10.

Subcellular locationi

  • Cytoplasm
  • Nucleusnucleolus
  • Nucleus

  • Note: Strongly enriched in the nucleolus and a small amount has been found in cytoplasm supporting the existence of a nucleolar RNA exosome complex form.

GO - Cellular componenti

  • cytoplasm Source: UniProtKB
  • exosome (RNase complex) Source: UniProtKB
  • membrane Source: UniProtKB
  • nuclear exosome (RNase complex) Source: InterPro
  • nucleolus Source: UniProtKB
  • nucleoplasm Source: Reactome
  • nucleus Source: UniProtKB
  • transcriptionally active chromatin Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Cytoplasm, Exosome, Nucleus

Pathology & Biotechi

Organism-specific databases

DisGeNETi5394.
OpenTargetsiENSG00000171824.
PharmGKBiPA33464.

Polymorphism and mutation databases

BioMutaiEXOSC10.
DMDMi8928564.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000871331 – 885Exosome component 10Add BLAST885

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Cross-linki583Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO1)Combined sources
Cross-linki583Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2)Combined sources
Modified residuei821PhosphoserineCombined sources1
Cross-linki826Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2)Combined sources

Keywords - PTMi

Isopeptide bond, Phosphoprotein, Ubl conjugation

Proteomic databases

EPDiQ01780.
MaxQBiQ01780.
PaxDbiQ01780.
PeptideAtlasiQ01780.
PRIDEiQ01780.

2D gel databases

SWISS-2DPAGEQ01780.

PTM databases

iPTMnetiQ01780.
PhosphoSitePlusiQ01780.
SwissPalmiQ01780.

Expressioni

Gene expression databases

BgeeiENSG00000171824.
CleanExiHS_EXOSC10.
ExpressionAtlasiQ01780. baseline and differential.
GenevisibleiQ01780. HS.

Organism-specific databases

HPAiCAB037141.
HPA028470.
HPA028484.

Interactioni

Subunit structurei

Component of the RNA exosome complex. The catalytically inactive RNA exosome core (Exo-9) complex is believed to associate with catalytic subunits EXOSC10, and DIS3 or DIS3L in cytoplasmic- and nuclear-specific RNA exosome complex forms. Interacts with C1D and MPHOSPH6. Interacts with ALYREF/THOC4.3 Publications

Binary interactionsi

WithEntry#Exp.IntActNotes
C1DQ139015EBI-358236,EBI-3844053
DDX5P178443EBI-358236,EBI-351962
DIS3Q9Y2L14EBI-358236,EBI-373539
EXOSC4Q9NPD34EBI-358236,EBI-371823
EXOSC5Q9NQT43EBI-358236,EBI-371876
MPHOSPH6Q995474EBI-358236,EBI-373187

Protein-protein interaction databases

BioGridi111403. 75 interactors.
DIPiDIP-31249N.
IntActiQ01780. 54 interactors.
MINTiMINT-1143843.
STRINGi9606.ENSP00000366135.

Structurei

Secondary structure

1885
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Helixi183 – 186Combined sources4
Beta strandi194 – 196Combined sources3
Helixi214 – 216Combined sources3
Helixi218 – 220Combined sources3
Beta strandi253 – 255Combined sources3
Helixi259 – 263Combined sources5
Helixi269 – 272Combined sources4
Helixi283 – 285Combined sources3
Beta strandi288 – 291Combined sources4
Helixi294 – 304Combined sources11
Beta strandi308 – 317Combined sources10
Beta strandi325 – 332Combined sources8
Beta strandi337 – 341Combined sources5
Turni342 – 345Combined sources4
Helixi346 – 352Combined sources7
Helixi353 – 356Combined sources4
Beta strandi361 – 367Combined sources7
Helixi369 – 379Combined sources11
Beta strandi384 – 388Combined sources5
Helixi389 – 395Combined sources7
Helixi403 – 411Combined sources9
Turni418 – 421Combined sources4
Helixi431 – 442Combined sources12
Helixi444 – 458Combined sources15
Beta strandi461 – 463Combined sources3
Helixi464 – 476Combined sources13
Helixi490 – 492Combined sources3
Helixi493 – 496Combined sources4
Beta strandi497 – 500Combined sources4
Helixi504 – 524Combined sources21
Helixi528 – 531Combined sources4
Helixi534 – 543Combined sources10
Helixi548 – 552Combined sources5
Beta strandi555 – 557Combined sources3
Helixi560 – 564Combined sources5
Helixi566 – 577Combined sources12
Helixi583 – 586Combined sources4

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2CPRNMR-A483-593[»]
3SAFX-ray2.50A/B180-606[»]
3SAGX-ray2.70A/B180-606[»]
3SAHX-ray2.65A/B180-606[»]
ProteinModelPortaliQ01780.
SMRiQ01780.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiQ01780.

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini503 – 583HRDCPROSITE-ProRule annotationAdd BLAST81

Sequence similaritiesi

Contains 1 3'-5' exonuclease domain.Curated
Contains 1 HRDC domain.PROSITE-ProRule annotation

Phylogenomic databases

eggNOGiKOG2206. Eukaryota.
COG0349. LUCA.
GeneTreeiENSGT00390000015408.
HOGENOMiHOG000001579.
HOVERGENiHBG051524.
InParanoidiQ01780.
KOiK12591.
OMAiGNKSMSF.
OrthoDBiEOG091G03BH.
PhylomeDBiQ01780.
TreeFamiTF105991.

Family and domain databases

Gene3Di3.30.420.10. 1 hit.
InterProiIPR002562. 3'-5'_exonuclease_dom.
IPR012588. Exosome-assoc_fac_Rrp6_N.
IPR010997. HRDC-like.
IPR002121. HRDC_dom.
IPR012337. RNaseH-like_dom.
[Graphical view]
PfamiPF01612. DNA_pol_A_exo1. 1 hit.
PF00570. HRDC. 1 hit.
PF08066. PMC2NT. 1 hit.
[Graphical view]
SMARTiSM00474. 35EXOc. 1 hit.
SM00341. HRDC. 1 hit.
[Graphical view]
SUPFAMiSSF47819. SSF47819. 1 hit.
SSF53098. SSF53098. 1 hit.
PROSITEiPS50967. HRDC. 1 hit.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q01780-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MAPPSTREPR VLSATSATKS DGEMVLPGFP DADSFVKFAL GSVVAVTKAS
60 70 80 90 100
GGLPQFGDEY DFYRSFPGFQ AFCETQGDRL LQCMSRVMQY HGCRSNIKDR
110 120 130 140 150
SKVTELEDKF DLLVDANDVI LERVGILLDE ASGVNKNQQP VLPAGLQVPK
160 170 180 190 200
TVVSSWNRKA AEYGKKAKSE TFRLLHAKNI IRPQLKFREK IDNSNTPFLP
210 220 230 240 250
KIFIKPNAQK PLPQALSKER RERPQDRPED LDVPPALADF IHQQRTQQVE
260 270 280 290 300
QDMFAHPYQY ELNHFTPADA VLQKPQPQLY RPIEETPCHF ISSLDELVEL
310 320 330 340 350
NEKLLNCQEF AVDLEHHSYR SFLGLTCLMQ ISTRTEDFII DTLELRSDMY
360 370 380 390 400
ILNESLTDPA IVKVFHGADS DIEWLQKDFG LYVVNMFDTH QAARLLNLGR
410 420 430 440 450
HSLDHLLKLY CNVDSNKQYQ LADWRIRPLP EEMLSYARDD THYLLYIYDK
460 470 480 490 500
MRLEMWERGN GQPVQLQVVW QRSRDICLKK FIKPIFTDES YLELYRKQKK
510 520 530 540 550
HLNTQQLTAF QLLFAWRDKT ARREDESYGY VLPNHMMLKI AEELPKEPQG
560 570 580 590 600
IIACCNPVPP LVRQQINEMH LLIQQAREMP LLKSEVAAGV KKSGPLPSAE
610 620 630 640 650
RLENVLFGPH DCSHAPPDGY PIIPTSGSVP VQKQASLFPD EKEDNLLGTT
660 670 680 690 700
CLIATAVITL FNEPSAEDSK KGPLTVAQKK AQNIMESFEN PFRMFLPSLG
710 720 730 740 750
HRAPVSQAAK FDPSTKIYEI SNRWKLAQVQ VQKDSKEAVK KKAAEQTAAR
760 770 780 790 800
EQAKEACKAA AEQAISVRQQ VVLENAAKKR ERATSDPRTT EQKQEKKRLK
810 820 830 840 850
ISKKPKDPEP PEKEFTPYDY SQSDFKAFAG NSKSKVSSQF DPNKQTPSGK
860 870 880
KCIAAKKIKQ SVGNKSMSFP TGKSDRGFRY NWPQR
Length:885
Mass (Da):100,831
Last modified:December 1, 2000 - v2
Checksum:iA37BDC8F49BF2E57
GO
Isoform 2 (identifier: Q01780-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     695-719: Missing.

Note: No experimental confirmation available.
Show »
Length:860
Mass (Da):98,089
Checksum:iB7FBE8B15F28BFE2
GO

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_004362695 – 719Missing in isoform 2. 1 PublicationAdd BLAST25

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X66113 mRNA. Translation: CAA46904.1.
L01457 mRNA. Translation: AAB59352.1.
AJ300188 Genomic DNA. Translation: CAC15569.1.
AL109811 Genomic DNA. Translation: CAI22106.1.
AL109811 Genomic DNA. Translation: CAI22107.1.
CH471130 Genomic DNA. Translation: EAW71679.1.
BC039901 mRNA. Translation: AAH39901.1.
BC073788 mRNA. Translation: AAH73788.1.
CCDSiCCDS126.1. [Q01780-2]
CCDS30584.1. [Q01780-1]
PIRiA43920.
JH0796.
RefSeqiNP_001001998.1. NM_001001998.2. [Q01780-1]
NP_002676.1. NM_002685.3. [Q01780-2]
UniGeneiHs.632368.

Genome annotation databases

EnsembliENST00000304457; ENSP00000307307; ENSG00000171824. [Q01780-2]
ENST00000376936; ENSP00000366135; ENSG00000171824. [Q01780-1]
GeneIDi5394.
KEGGihsa:5394.
UCSCiuc001asa.4. human. [Q01780-1]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X66113 mRNA. Translation: CAA46904.1.
L01457 mRNA. Translation: AAB59352.1.
AJ300188 Genomic DNA. Translation: CAC15569.1.
AL109811 Genomic DNA. Translation: CAI22106.1.
AL109811 Genomic DNA. Translation: CAI22107.1.
CH471130 Genomic DNA. Translation: EAW71679.1.
BC039901 mRNA. Translation: AAH39901.1.
BC073788 mRNA. Translation: AAH73788.1.
CCDSiCCDS126.1. [Q01780-2]
CCDS30584.1. [Q01780-1]
PIRiA43920.
JH0796.
RefSeqiNP_001001998.1. NM_001001998.2. [Q01780-1]
NP_002676.1. NM_002685.3. [Q01780-2]
UniGeneiHs.632368.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2CPRNMR-A483-593[»]
3SAFX-ray2.50A/B180-606[»]
3SAGX-ray2.70A/B180-606[»]
3SAHX-ray2.65A/B180-606[»]
ProteinModelPortaliQ01780.
SMRiQ01780.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi111403. 75 interactors.
DIPiDIP-31249N.
IntActiQ01780. 54 interactors.
MINTiMINT-1143843.
STRINGi9606.ENSP00000366135.

PTM databases

iPTMnetiQ01780.
PhosphoSitePlusiQ01780.
SwissPalmiQ01780.

Polymorphism and mutation databases

BioMutaiEXOSC10.
DMDMi8928564.

2D gel databases

SWISS-2DPAGEQ01780.

Proteomic databases

EPDiQ01780.
MaxQBiQ01780.
PaxDbiQ01780.
PeptideAtlasiQ01780.
PRIDEiQ01780.

Protocols and materials databases

DNASUi5394.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000304457; ENSP00000307307; ENSG00000171824. [Q01780-2]
ENST00000376936; ENSP00000366135; ENSG00000171824. [Q01780-1]
GeneIDi5394.
KEGGihsa:5394.
UCSCiuc001asa.4. human. [Q01780-1]

Organism-specific databases

CTDi5394.
DisGeNETi5394.
GeneCardsiEXOSC10.
HGNCiHGNC:9138. EXOSC10.
HPAiCAB037141.
HPA028470.
HPA028484.
MIMi605960. gene.
neXtProtiNX_Q01780.
OpenTargetsiENSG00000171824.
PharmGKBiPA33464.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG2206. Eukaryota.
COG0349. LUCA.
GeneTreeiENSGT00390000015408.
HOGENOMiHOG000001579.
HOVERGENiHBG051524.
InParanoidiQ01780.
KOiK12591.
OMAiGNKSMSF.
OrthoDBiEOG091G03BH.
PhylomeDBiQ01780.
TreeFamiTF105991.

Enzyme and pathway databases

BioCyciZFISH:ENSG00000171824-MONOMER.
ReactomeiR-HSA-6791226. Major pathway of rRNA processing in the nucleolus and cytosol.

Miscellaneous databases

ChiTaRSiEXOSC10. human.
EvolutionaryTraceiQ01780.
GeneWikiiExosome_component_10.
GenomeRNAii5394.
PROiQ01780.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000171824.
CleanExiHS_EXOSC10.
ExpressionAtlasiQ01780. baseline and differential.
GenevisibleiQ01780. HS.

Family and domain databases

Gene3Di3.30.420.10. 1 hit.
InterProiIPR002562. 3'-5'_exonuclease_dom.
IPR012588. Exosome-assoc_fac_Rrp6_N.
IPR010997. HRDC-like.
IPR002121. HRDC_dom.
IPR012337. RNaseH-like_dom.
[Graphical view]
PfamiPF01612. DNA_pol_A_exo1. 1 hit.
PF00570. HRDC. 1 hit.
PF08066. PMC2NT. 1 hit.
[Graphical view]
SMARTiSM00474. 35EXOc. 1 hit.
SM00341. HRDC. 1 hit.
[Graphical view]
SUPFAMiSSF47819. SSF47819. 1 hit.
SSF53098. SSF53098. 1 hit.
PROSITEiPS50967. HRDC. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiEXOSX_HUMAN
AccessioniPrimary (citable) accession number: Q01780
Secondary accession number(s): B1AKQ0, B1AKQ1, Q15158
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 1, 1993
Last sequence update: December 1, 2000
Last modified: November 2, 2016
This is version 179 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. Human chromosome 1
    Human chromosome 1: entries, gene names and cross-references to MIM
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  4. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.