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UniProtKB - Q06265 (EXOS9_HUMAN)

Entry version 200 (02 Jun 2021)
Sequence version 3 (15 May 2007)
Previous versions | rss
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Protein

Exosome complex component RRP45

Gene

EXOSC9

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score:

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the 'protein existence' evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

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. EXOSC9 binds to ARE-containing RNAs.

4 Publications

Caution

The six exosome core subunits containing a RNase PH-domain are not phosphorolytically active.Curated

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

Complete GO annotation on QuickGO ...

GO - Biological processi

Complete GO annotation on QuickGO ...

<p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

Molecular functionRNA-binding
Biological processrRNA processing

Enzyme and pathway databases

Pathway Commons web resource for biological pathway data

More...PathwayCommonsi		Q06265

Reactome - a knowledgebase of biological pathways and processes

More...Reactomei		R-HSA-380994, ATF4 activates genes in response to endoplasmic reticulum stress
R-HSA-429958, mRNA decay by 3' to 5' exoribonuclease
R-HSA-450385, Butyrate Response Factor 1 (BRF1) binds and destabilizes mRNA
R-HSA-450513, Tristetraprolin (TTP, ZFP36) binds and destabilizes mRNA
R-HSA-450604, KSRP (KHSRP) binds and destabilizes mRNA
R-HSA-6791226, Major pathway of rRNA processing in the nucleolus and cytosol

SIGNOR Signaling Network Open Resource

More...SIGNORi		Q06265

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>Protein namesi
Recommended name:
Exosome complex component RRP45
Alternative name(s):
Autoantigen PM/Scl 1
Exosome component 9
P75 polymyositis-scleroderma overlap syndrome-associated autoantigen
Polymyositis/scleroderma autoantigen 1
Polymyositis/scleroderma autoantigen 75 kDa
Short name:
PM/Scl-75
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: 'Name', 'Synonyms', 'Ordered locus names' and 'ORF names'.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
Name:EXOSC9
Synonyms:PMSCL1
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the 'taxonomic identifier' or 'taxid'.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
  • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes%5Fmanual">proteome</a> can consist of several components.<br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome 4

Organism-specific databases

Human Gene Nomenclature Database

More...HGNCi		HGNC:9137, EXOSC9

Online Mendelian Inheritance in Man (OMIM)

More...MIMi		606180, gene

neXtProt; the human protein knowledge platform

More...neXtProti		NX_Q06265

Eukaryotic Pathogen, Vector and Host Database Resources

More...VEuPathDBi		HostDB:ENSG00000123737.12

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Keywords - Cellular componenti

Cytoplasm, Exosome, Nucleus

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

<p>This subsection of the 'Pathology and Biotech' section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>Involvement in diseasei

Pontocerebellar hypoplasia 1D (PCH1D)1 Publication
The disease is caused by variants affecting the gene represented in this entry.
Disease descriptionAn autosomal recessive neurologic disorder with onset at birth or in infancy, and characterized by progressive axonal motor neuronopathy, severe generalized hypotonia, respiratory insufficiency, and cerebellar atrophy. Death in childhood may occur.
Related information in OMIM
Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'Sequence' section describes natural variant(s) of the protein sequence.<p><a href='/help/variant' target='_top'>More...</a></p>Natural variantiVAR_08105214L → P in PCH1D; reduced EXOSC9 and exosome levels in patient cells. 1 PublicationCorresponds to variant dbSNP:rs139632595EnsemblClinVar.1
Natural variantiVAR_081053161 – 439Missing in PCH1D; reduced EXOSC9 and exosome levels in patient cells. 1 PublicationAdd BLAST279

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/manual/pathology%5Fand%5Fbiotech%5Fsection">'Pathology and Biotech'</a> section describes the effect of the experimental mutation of one or more amino acid(s) on the biological properties of the protein.<p><a href='/help/mutagen' target='_top'>More...</a></p>Mutagenesisi388 – 391PIIL → EECP: Abolishes interaction with SETX. 1 Publication4
Mutagenesisi388 – 391Missing : Abolishes interaction with SETX. 1 Publication4
Mutagenesisi390 – 391Missing : Abolishes interaction with SETX. 1 Publication2
Mutagenesisi395 – 398EEEE → AAAA: Abolishes interaction with SETX. 1 Publication4
Mutagenesisi395 – 398Missing : Abolishes interaction with SETX. 1 Publication4

Keywords - Diseasei

Disease variant, Neurodegeneration

Organism-specific databases

DisGeNET

More...DisGeNETi		5393

MalaCards human disease database

More...MalaCardsi		EXOSC9
MIMi618065, phenotype

Open Targets

More...OpenTargetsi		ENSG00000123737

Orphanet; a database dedicated to information on rare diseases and orphan drugs

More...Orphaneti		2254, Pontocerebellar hypoplasia type 1

The Pharmacogenetics and Pharmacogenomics Knowledge Base

More...PharmGKBi		PA33463

Miscellaneous databases

Pharos NIH Druggable Genome Knowledgebase

More...Pharosi		Q06265, Tbio

Genetic variation databases

BioMuta curated single-nucleotide variation and disease association database

More...BioMutai		EXOSC9

Domain mapping of disease mutations (DMDM)

More...DMDMi		147744559

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'PTM / Processing' section describes the extent of a polypeptide chain in the mature protein following processing or proteolytic cleavage.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_00001399711 – 439Exosome complex component RRP45Add BLAST439

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'PTM / Processing' section specifies the position and type of each modified residue excluding <a href="http://www.uniprot.org/manual/lipid">lipids</a>, <a href="http://www.uniprot.org/manual/carbohyd">glycans</a> and <a href="http://www.uniprot.org/manual/crosslnk">protein cross-links</a>.<p><a href='/help/mod_res' target='_top'>More...</a></p>Modified residuei65PhosphoserineCombined sources1
Modified residuei297N6-acetyllysine; alternateCombined sources1
<p>This subsection of the <a href="http://www.uniprot.org/help/ptm%5Fprocessing%5Fsection">PTM / Processing</a> section describes <strong>covalent linkages</strong> of various types formed <strong>between two proteins (interchain cross-links)</strong> or <strong>between two parts of the same protein (intrachain cross-links)</strong>, except the disulfide bonds that are annotated in the <a href="http://www.uniprot.org/manual/disulfid">'Disulfide bond'</a> subsection.<p><a href='/help/crosslnk' target='_top'>More...</a></p>Cross-linki297Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO1); alternateCombined sources
Cross-linki297Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2); alternateCombined sources
Modified residuei306PhosphoserineCombined sources1
Modified residuei346PhosphoserineCombined sources1
Modified residuei392PhosphoserineCombined sources1
Modified residuei394PhosphoserineCombined sources1
Cross-linki419Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2)Combined sources
Isoform 4 (identifier: Q06265-4)
Modified residuei325PhosphoserineCombined sources1
Modified residuei327PhosphoserineCombined sources1
Isoform 2 (identifier: Q06265-2)
Modified residuei409PhosphoserineCombined sources1
Modified residuei411PhosphoserineCombined sources1

Keywords - PTMi

Acetylation, Isopeptide bond, Phosphoprotein, Ubl conjugation

Proteomic databases

Encyclopedia of Proteome Dynamics

More...EPDi		Q06265

jPOST - Japan Proteome Standard Repository/Database

More...jPOSTi		Q06265

MassIVE - Mass Spectrometry Interactive Virtual Environment

More...MassIVEi		Q06265

MaxQB - The MaxQuant DataBase

More...MaxQBi		Q06265

PaxDb, a database of protein abundance averages across all three domains of life

More...PaxDbi		Q06265

PeptideAtlas

More...PeptideAtlasi		Q06265

PRoteomics IDEntifications database

More...PRIDEi		Q06265

ProteomicsDB: a multi-organism proteome resource

More...ProteomicsDBi		58426 [Q06265-1]
58427 [Q06265-2]
58428 [Q06265-3]
58429 [Q06265-4]

PTM databases

iPTMnet integrated resource for PTMs in systems biology context

More...iPTMneti		Q06265

Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

More...PhosphoSitePlusi		Q06265

SwissPalm database of S-palmitoylation events

More...SwissPalmi		Q06265

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

Bgee dataBase for Gene Expression Evolution

More...Bgeei		ENSG00000123737, Expressed in female gonad and 234 other tissues

ExpressionAtlas, Differential and Baseline Expression

More...ExpressionAtlasi		Q06265, baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

More...Genevisiblei		Q06265, HS

Organism-specific databases

Human Protein Atlas

More...HPAi		ENSG00000123737, Low tissue specificity

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

<p>This subsection of the <a href="http://www.uniprot.org/help/interaction%5Fsection">'Interaction'</a> section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the <a href="http://www.uniprot.org/help/function%5Fsection">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

Component of the RNA exosome complex (PubMed:29906447). Specifically part of the catalytically inactive RNA exosome core (Exo-9) complex which is believed to associate with catalytic subunits EXOSC10, and DIS3 or DIS3L in cytoplasmic- and nuclear-specific RNA exosome complex forms. Exo-9 is formed by a hexameric ring of RNase PH domain-containing subunits specifically containing the heterodimers EXOSC4-EXOSC9, EXOSC5-EXOSC8 and EXOSC6-EXOSC7, and peripheral S1 domain-containing components EXOSC1, EXOSC2 and EXOSC3 located on the top of the ring structure (PubMed:11719186, PubMed:12788944, PubMed:20531389).

Interacts (via C-terminus region) with SETX (via N-terminus domain); the interaction enhances SETX sumoylation (PubMed:24105744).

5 Publications

<p>This subsection of the '<a href="http://www.uniprot.org/help/interaction%5Fsection">Interaction</a>' section provides information about binary protein-protein interactions. The data presented in this section are a quality-filtered subset of binary interactions automatically derived from the <a href="https://www.ebi.ac.uk/intact/">IntAct database</a>. It is updated at every <a href="http://www.uniprot.org/help/synchronization">UniProt release</a>.<p><a href='/help/binary_interactions' target='_top'>More...</a></p>Binary interactionsi

GO - Molecular functioni

Complete GO annotation on QuickGO ...

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGRID)

More...BioGRIDi		111402, 94 interactors

ComplexPortal: manually curated resource of macromolecular complexes

More...ComplexPortali		CPX-476, Nuclear exosome complex, DIS3-EXOSC10 variant
CPX-591, Nucleolar exosome complex, EXOSC10 variant
CPX-592, Cytoplasmic exosome complex, DIS3L variant
CPX-593, Exosome complex, DIS3 variant
CPX-600, Cytoplasmic exosome complex, DIS3L-EXOSC10 variant

CORUM comprehensive resource of mammalian protein complexes

More...CORUMi		Q06265

Database of interacting proteins

More...DIPi		DIP-31286N

Protein interaction database and analysis system

More...IntActi		Q06265, 51 interactors

Molecular INTeraction database

More...MINTi		Q06265

STRING: functional protein association networks

More...STRINGi		9606.ENSP00000368984

Miscellaneous databases

RNAct, Protein-RNA interaction predictions for model organisms.

More...RNActi		Q06265, protein

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

Secondary structure

1439
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

SWISS-MODEL Repository - a database of annotated 3D protein structure models

More...SMRi		Q06265

Database of comparative protein structure models

More...ModBasei		Search...

Protein Data Bank in Europe - Knowledge Base

More...PDBe-KBi		Search...

Miscellaneous databases

Relative evolutionary importance of amino acids within a protein sequence

More...EvolutionaryTracei		Q06265

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'Family and Domains' section describes a region of interest that cannot be described in other subsections.<p><a href='/help/region' target='_top'>More...</a></p>Regioni1 – 268ARE binding1 PublicationAdd BLAST268
Regioni335 – 363DisorderedSequence analysisAdd BLAST29
Regioni391 – 439DisorderedSequence analysisAdd BLAST49

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'Family and Domains' section describes the position of regions of compositional bias within the protein and the particular type of amino acids that are over-represented within those regions.<p><a href='/help/compbias' target='_top'>More...</a></p>Compositional biasi401 – 416Basic and acidic residuesSequence analysisAdd BLAST16
Compositional biasi425 – 439Basic residuesSequence analysisAdd BLAST15

<p>This subsection of the 'Family and domains' section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Belongs to the RNase PH family.Curated

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

More...eggNOGi		KOG1614, Eukaryota

Ensembl GeneTree

More...GeneTreei		ENSGT00950000183130

The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

More...HOGENOMi		CLU_038194_7_0_1

InParanoid: Eukaryotic Ortholog Groups

More...InParanoidi		Q06265

Identification of Orthologs from Complete Genome Data

More...OMAi		HGEICQI

Database of Orthologous Groups

More...OrthoDBi		996662at2759

Database for complete collections of gene phylogenies

More...PhylomeDBi		Q06265

TreeFam database of animal gene trees

More...TreeFami		TF300092

Family and domain databases

Conserved Domains Database

More...CDDi		cd11368, RNase_PH_RRP45, 1 hit

Gene3D Structural and Functional Annotation of Protein Families

More...Gene3Di		3.30.230.70, 1 hit

Integrated resource of protein families, domains and functional sites

More...InterProi		View protein in InterPro
IPR001247, ExoRNase_PH_dom1
IPR015847, ExoRNase_PH_dom2
IPR036345, ExoRNase_PH_dom2_sf
IPR027408, PNPase/RNase_PH_dom_sf
IPR020568, Ribosomal_S5_D2-typ_fold
IPR033100, Rrp45

Pfam protein domain database

More...Pfami		View protein in Pfam
PF01138, RNase_PH, 1 hit
PF03725, RNase_PH_C, 1 hit

Superfamily database of structural and functional annotation

More...SUPFAMi		SSF54211, SSF54211, 1 hit
SSF55666, SSF55666, 1 hit

<p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence%5Flength">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>. The information is filed in different subsections. The current subsections and their content are listed below:<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequences (4+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences%5Fsection">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

This entry describes 4 <p>This subsection of the 'Sequence' section lists the alternative protein sequences (isoforms) that can be generated from the same gene by a single or by the combination of up to four biological events (alternative promoter usage, alternative splicing, alternative initiation and ribosomal frameshifting). Additionally, this section gives relevant information on each alternative protein isoform.<p><a href='/help/alternative_products' target='_top'>More...</a></p> isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 4 described isoforms and 5 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: Q06265-1) [UniParc]FASTAAdd to basket
Also known as: PM/SCL-75c-alpha

This isoform has been chosen as the <div> <p><b>What is the canonical sequence?</b><p><a href='/help/canonical_and_isoforms' target='_top'>More...</a></p>canonicali 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
MKETPLSNCE RRFLLRAIEE KKRLDGRQTY DYRNIRISFG TDYGCCIVEL 
        60         70         80         90        100
GKTRVLGQVS CELVSPKLNR ATEGILFFNL ELSQMAAPAF EPGRQSDLLV 
       110        120        130        140        150
KLNRLMERCL RNSKCIDTES LCVVAGEKVW QIRVDLHLLN HDGNIIDAAS 
       160        170        180        190        200
IAAIVALCHF RRPDVSVQGD EVTLYTPEER DPVPLSIHHM PICVSFAFFQ 
       210        220        230        240        250
QGTYLLVDPN EREERVMDGL LVIAMNKHRE ICTIQSSGGI MLLKDQVLRC 
       260        270        280        290        300
SKIAGVKVAE ITELILKALE NDQKVRKEGG KFGFAESIAN QRITAFKMEK 
       310        320        330        340        350
APIDTSDVEE KAEEIIAEAE PPSEVVSTPV LWTPGTAQIG EGVENSWGDL 
       360        370        380        390        400
EDSEKEDDEG GGDQAIILDG IKMDTGVEVS DIGSQDAPII LSDSEEEEMI 
       410        420        430 
ILEPDKNPKK IRTQTTSAKQ EKAPSKKPVK RRKKKRAAN
Length:439
Mass (Da):48,949
Last modified:May 15, 2007 - v3
<p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> Checksum:i7E27322F094ED3F3
GO
Isoform 2 (identifier: Q06265-2) [UniParc]FASTAAdd to basket
Also known as: PM/SCL-75c-beta

The sequence of this isoform differs from the canonical sequence as follows:
     385-385: Q → QELGFHHVGQTGLEFLTS

Show »
Length:456
Mass (Da):50,803
Checksum:i693B31BE41C55545
GO
Isoform 3 (identifier: Q06265-3) [UniParc]FASTAAdd to basket
Also known as: PM/SCL-75a-alpha

The sequence of this isoform differs from the canonical sequence as follows:
     1-84: Missing.

Show »
Length:355
Mass (Da):39,235
Checksum:iDC37BB31767B6621
GO
Isoform 4 (identifier: Q06265-4) [UniParc]FASTAAdd to basket
Also known as: PM/SCL-75a-beta

The sequence of this isoform differs from the canonical sequence as follows:
     1-84: Missing.
     385-385: Q → QELGFHHVGQTGLEFLTS

Show »
Length:372
Mass (Da):41,089
Checksum:iDB666F47B5422E7E
GO

<p>In eukaryotic reference proteomes, unreviewed entries that are likely to belong to the same gene are computationally mapped, based on gene identifiers from Ensembl, EnsemblGenomes and model organism databases.<p><a href='/help/gene_centric_isoform_mapping' target='_top'>More...</a></p>Computationally mapped potential isoform sequencesi

There are 5 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
D6RIY6D6RIY6_HUMAN
Exosome complex component RRP45
EXOSC9 hCG_38830
423Annotation score:

Annotation score:2 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
D6R905D6R905_HUMAN
Exosome complex component RRP45
EXOSC9
226Annotation score:

Annotation score:2 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
D6RA17D6RA17_HUMAN
Exosome complex component RRP45
EXOSC9 hCG_38830
242Annotation score:

Annotation score:2 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
H0Y9L5H0Y9L5_HUMAN
Exosome complex component RRP45
EXOSC9
172Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
D6RAP4D6RAP4_HUMAN
Exosome complex component RRP45
EXOSC9
86Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_08105214L → P in PCH1D; reduced EXOSC9 and exosome levels in patient cells. 1 PublicationCorresponds to variant dbSNP:rs139632595EnsemblClinVar.1
Natural variantiVAR_081053161 – 439Missing in PCH1D; reduced EXOSC9 and exosome levels in patient cells. 1 PublicationAdd BLAST279
Natural variantiVAR_051867366I → V. Corresponds to variant dbSNP:rs1803183Ensembl.1
Natural variantiVAR_014924425S → T. Corresponds to variant dbSNP:rs1051881Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'Sequence' section describes the sequence of naturally occurring alternative protein isoform(s). The changes in the amino acid sequence may be due to alternative splicing, alternative promoter usage, alternative initiation, or ribosomal frameshifting.<p><a href='/help/var_seq' target='_top'>More...</a></p>Alternative sequenceiVSP_0255551 – 84Missing in isoform 3 and isoform 4. 2 PublicationsAdd BLAST84
Alternative sequenceiVSP_025556385Q → QELGFHHVGQTGLEFLTS in isoform 2 and isoform 4. 2 Publications1

Sequence databases

Select the link destinations:

EMBL nucleotide sequence database

More...EMBLi

GenBank nucleotide sequence database

More...GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

More...DDBJi
Links Updated
M58460 mRNA Translation: AAA58384.1
U09215 mRNA Translation: AAA18832.1
AJ505989 mRNA Translation: CAD44530.1
AJ517294 mRNA Translation: CAD56889.1
AC079341 Genomic DNA Translation: AAY40968.1

The Consensus CDS (CCDS) project

More...CCDSi		CCDS34057.1 [Q06265-2]
CCDS3722.2 [Q06265-1]

Protein sequence database of the Protein Information Resource

More...PIRi		G01425

NCBI Reference Sequences

More...RefSeqi		NP_001029366.1, NM_001034194.1 [Q06265-2]
NP_005024.2, NM_005033.2 [Q06265-1]

Genome annotation databases

Ensembl eukaryotic genome annotation project

More...Ensembli		ENST00000243498; ENSP00000243498; ENSG00000123737 [Q06265-1]
ENST00000379663; ENSP00000368984; ENSG00000123737 [Q06265-2]

Database of genes from NCBI RefSeq genomes

More...GeneIDi		5393

KEGG: Kyoto Encyclopedia of Genes and Genomes

More...KEGGi		hsa:5393

UCSC genome browser

More...UCSCi		uc003idz.4, human [Q06265-1]

Keywords - Coding sequence diversityi

Alternative splicing

<p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M58460 mRNA Translation: AAA58384.1
U09215 mRNA Translation: AAA18832.1
AJ505989 mRNA Translation: CAD44530.1
AJ517294 mRNA Translation: CAD56889.1
AC079341 Genomic DNA Translation: AAY40968.1
CCDSiCCDS34057.1 [Q06265-2]
CCDS3722.2 [Q06265-1]
PIRiG01425
RefSeqiNP_001029366.1, NM_001034194.1 [Q06265-2]
NP_005024.2, NM_005033.2 [Q06265-1]

3D structure databases

Select the link destinations:

Protein Data Bank Europe

More...PDBei

Protein Data Bank RCSB

More...RCSB PDBi

Protein Data Bank Japan

More...PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2NN6X-ray3.35A1-302[»]
6D6Qelectron microscopy3.45A1-439[»]
6D6Relectron microscopy3.45A1-439[»]
6H25electron microscopy3.80A1-439[»]
SMRiQ06265
ModBaseiSearch...
PDBe-KBiSearch...

Protein-protein interaction databases

BioGRIDi111402, 94 interactors
ComplexPortaliCPX-476, Nuclear exosome complex, DIS3-EXOSC10 variant
CPX-591, Nucleolar exosome complex, EXOSC10 variant
CPX-592, Cytoplasmic exosome complex, DIS3L variant
CPX-593, Exosome complex, DIS3 variant
CPX-600, Cytoplasmic exosome complex, DIS3L-EXOSC10 variant
CORUMiQ06265
DIPiDIP-31286N
IntActiQ06265, 51 interactors
MINTiQ06265
STRINGi9606.ENSP00000368984

PTM databases

iPTMnetiQ06265
PhosphoSitePlusiQ06265
SwissPalmiQ06265

Genetic variation databases

BioMutaiEXOSC9
DMDMi147744559

Proteomic databases

EPDiQ06265
jPOSTiQ06265
MassIVEiQ06265
MaxQBiQ06265
PaxDbiQ06265
PeptideAtlasiQ06265
PRIDEiQ06265
ProteomicsDBi58426 [Q06265-1]
58427 [Q06265-2]
58428 [Q06265-3]
58429 [Q06265-4]

Protocols and materials databases

Antibodypedia a portal for validated antibodies

More...Antibodypediai		26749, 144 antibodies

The DNASU plasmid repository

More...DNASUi		5393

Genome annotation databases

EnsembliENST00000243498; ENSP00000243498; ENSG00000123737 [Q06265-1]
ENST00000379663; ENSP00000368984; ENSG00000123737 [Q06265-2]
GeneIDi5393
KEGGihsa:5393
UCSCiuc003idz.4, human [Q06265-1]

Organism-specific databases

Comparative Toxicogenomics Database

More...CTDi		5393
DisGeNETi5393

GeneCards: human genes, protein and diseases

More...GeneCardsi		EXOSC9
HGNCiHGNC:9137, EXOSC9
HPAiENSG00000123737, Low tissue specificity
MalaCardsiEXOSC9
MIMi606180, gene
618065, phenotype
neXtProtiNX_Q06265
OpenTargetsiENSG00000123737
Orphaneti2254, Pontocerebellar hypoplasia type 1
PharmGKBiPA33463
VEuPathDBiHostDB:ENSG00000123737.12

GenAtlas: human gene database

More...GenAtlasi		Search...

Phylogenomic databases

eggNOGiKOG1614, Eukaryota
GeneTreeiENSGT00950000183130
HOGENOMiCLU_038194_7_0_1
InParanoidiQ06265
OMAiHGEICQI
OrthoDBi996662at2759
PhylomeDBiQ06265
TreeFamiTF300092

Enzyme and pathway databases

PathwayCommonsiQ06265
ReactomeiR-HSA-380994, ATF4 activates genes in response to endoplasmic reticulum stress
R-HSA-429958, mRNA decay by 3' to 5' exoribonuclease
R-HSA-450385, Butyrate Response Factor 1 (BRF1) binds and destabilizes mRNA
R-HSA-450513, Tristetraprolin (TTP, ZFP36) binds and destabilizes mRNA
R-HSA-450604, KSRP (KHSRP) binds and destabilizes mRNA
R-HSA-6791226, Major pathway of rRNA processing in the nucleolus and cytosol
SIGNORiQ06265

Miscellaneous databases

BioGRID ORCS database of CRISPR phenotype screens

More...BioGRID-ORCSi		5393, 645 hits in 1007 CRISPR screens

ChiTaRS: a database of human, mouse and fruit fly chimeric transcripts and RNA-sequencing data

More...ChiTaRSi		EXOSC9, human
EvolutionaryTraceiQ06265

The Gene Wiki collection of pages on human genes and proteins

More...GeneWikii		Exosome_component_9

Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens

More...GenomeRNAii		5393
PharosiQ06265, Tbio

Protein Ontology

More...PROi		PR:Q06265
RNActiQ06265, protein

The Stanford Online Universal Resource for Clones and ESTs

More...SOURCEi		Search...

Gene expression databases

BgeeiENSG00000123737, Expressed in female gonad and 234 other tissues
ExpressionAtlasiQ06265, baseline and differential
GenevisibleiQ06265, HS

Family and domain databases

CDDicd11368, RNase_PH_RRP45, 1 hit
Gene3Di3.30.230.70, 1 hit
InterProiView protein in InterPro
IPR001247, ExoRNase_PH_dom1
IPR015847, ExoRNase_PH_dom2
IPR036345, ExoRNase_PH_dom2_sf
IPR027408, PNPase/RNase_PH_dom_sf
IPR020568, Ribosomal_S5_D2-typ_fold
IPR033100, Rrp45
PfamiView protein in Pfam
PF01138, RNase_PH, 1 hit
PF03725, RNase_PH_C, 1 hit
SUPFAMiSSF54211, SSF54211, 1 hit
SSF55666, SSF55666, 1 hit

MobiDB: a database of protein disorder and mobility annotations

More...MobiDBi		Search...

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This subsection of the 'Entry information' section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiEXOS9_HUMAN
<p>This subsection of the 'Entry information' section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called 'Primary (citable) accession number'.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: Q06265
Secondary accession number(s): Q12883
, Q4W5P5, Q86Y41, Q86Y48
<p>This subsection of the 'Entry information' section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification ('Last modified'). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: June 1, 2001
Last sequence update: May 15, 2007
Last modified: June 2, 2021
This is version 200 of the entry and version 3 of the sequence. See complete history.
<p>This subsection of the 'Entry information' section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>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.

<p>This section contains any relevant information that doesn't fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

3D-structure, Reference proteome

Documents

  1. Human chromosome 4
    Human chromosome 4: entries, gene names and cross-references to MIM
  2. Human entries with genetic variants
    List of human entries with genetic variants
  3. Human variants curated from literature reports
    Index of human variants curated from literature reports
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. SIMILARITY comments
    Index of protein domains and families
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