EXOSC10
Exosome complex component 10
Also known as: EXOSX_HUMAN, p2, p3, p4, PM-Scl, PM/Scl-100, PMSCL2, RRP6, Rrp6p
Cross-references: UniProt · Ensembl · Human Protein Atlas · GeneCards · NCBI Gene
Protein identityUniProt · HPA
- UniProt accession
- Q01780
- Gene
- EXOSC10
- Ensembl
- ENSG00000171824
- Chromosome
- 1
- Canonical length
- 885 aa
- Protein class
- Predicted intracellular proteins
- Subcellular location
- Nucleoplasm,Nucleoli,Nucleoli rim,Cytosol
OverviewNCBI Gene
Enables 3'-5'-RNA exonuclease activity and telomerase RNA binding activity. Involved in several processes, including RNA metabolic process; negative regulation of telomere maintenance via telomerase; and regulation of telomerase RNA localization to Cajal body. Located in cytosol; euchromatin; and nuclear lumen. Part of nuclear exosome (RNase complex) and small-subunit processome. [provided by Alliance of Genome Resources, Jul 2025]
Canonical amino-acid sequenceUniProt
885 residues, UniProt reviewed canonical sequence.
>Q01780|EXOSC10
1 MAPPSTREPR VLSATSATKS DGEMVLPGFP DADSFVKFAL GSVVAVTKAS GGLPQFGDEY
61 DFYRSFPGFQ AFCETQGDRL LQCMSRVMQY HGCRSNIKDR SKVTELEDKF DLLVDANDVI
121 LERVGILLDE ASGVNKNQQP VLPAGLQVPK TVVSSWNRKA AEYGKKAKSE TFRLLHAKNI
181 IRPQLKFREK IDNSNTPFLP KIFIKPNAQK PLPQALSKER RERPQDRPED LDVPPALADF
241 IHQQRTQQVE QDMFAHPYQY ELNHFTPADA VLQKPQPQLY RPIEETPCHF ISSLDELVEL
301 NEKLLNCQEF AVDLEHHSYR SFLGLTCLMQ ISTRTEDFII DTLELRSDMY ILNESLTDPA
361 IVKVFHGADS DIEWLQKDFG LYVVNMFDTH QAARLLNLGR HSLDHLLKLY CNVDSNKQYQ
421 LADWRIRPLP EEMLSYARDD THYLLYIYDK MRLEMWERGN GQPVQLQVVW QRSRDICLKK
481 FIKPIFTDES YLELYRKQKK HLNTQQLTAF QLLFAWRDKT ARREDESYGY VLPNHMMLKI
541 AEELPKEPQG IIACCNPVPP LVRQQINEMH LLIQQAREMP LLKSEVAAGV KKSGPLPSAE
601 RLENVLFGPH DCSHAPPDGY PIIPTSGSVP VQKQASLFPD EKEDNLLGTT CLIATAVITL
661 FNEPSAEDSK KGPLTVAQKK AQNIMESFEN PFRMFLPSLG HRAPVSQAAK FDPSTKIYEI
721 SNRWKLAQVQ VQKDSKEAVK KKAAEQTAAR EQAKEACKAA AEQAISVRQQ VVLENAAKKR
781 ERATSDPRTT EQKQEKKRLK ISKKPKDPEP PEKEFTPYDY SQSDFKAFAG NSKSKVSSQF
841 DPNKQTPSGK KCIAAKKIKQ SVGNKSMSFP TGKSDRGFRY NWPQRLocalizationUniProt · AlphaFold · HPA
Whether an antibody against EXOSC10 can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.
- Antibody reachability
- Intracellular
- Secreted
- No
- Transmembrane segments
- 0
- Mean surface accessibility (rSASA)
- 0.44
- Highest tissue expression
- 44 nTPM
Expression across tissuesHPA
Tissue
- cerebellum: 44 nTPM
- bone marrow: 37 nTPM
- skin: 35 nTPM
- ovary: 34 nTPM
- retina: 33 nTPM
- tonsil: 32 nTPM
Single-cell type
- erythrocyte progenitors: 75 nCPM
- leydig cells: 66 nCPM
- oligodendrocyte progenitor cells: 56 nCPM
- breast myoepithelial cells: 54 nCPM
- myonuclei: 54 nCPM
- extravillous trophoblasts: 53 nCPM
Immune cell
- basophil: 58 nTPM
- non-classical monocyte: 52 nTPM
- NK-cell: 50 nTPM
- total PBMC: 43 nTPM
- MAIT T-cell: 43 nTPM
- intermediate monocyte: 41 nTPM
Brain region
- cerebellum: 45 nTPM
- cerebral cortex: 40 nTPM
- white matter: 37 nTPM
- amygdala: 35 nTPM
- thalamus: 34 nTPM
- medulla oblongata: 34 nTPM
DiseaseUniProt · ClinVar · IEDB · PubMed
Four sources answering four different questions about EXOSC10.
Disease | ImmuneIEDB
Conditions an epitope on EXOSC10 was assayed in.
Disease | AutoantibodyPubMed
Conditions in which antibodies against EXOSC10 are reported. Each links to that disease's full target list.
- Scleroderma, Systemic 82
- Myositis 34
- Polymyositis 33
- Dermatomyositis 25
- Lupus Erythematosus, Systemic 11
- Lung Diseases, Interstitial 10
- Scleroderma, Localized 5
- Muscle Weakness 4
- Arthritis 3
- Mixed Connective Tissue Disease 3
Showing 10 of 12 — disease pages carrying at least 10 antigens.
ReferencesPubMed · IEDB
Publications for EXOSC10 from three distinct lines of evidence, kept separate because they answer different questions: whether antibodies are directed at the protein, whether a B-cell epitope has been mapped on it, and whether a T-cell epitope has. Each is labelled with its source.
Reference: AutoantibodyPubMed
139 publications
- Pathological autoantibody internalisation in myositis.
2024 · Ann Rheum Dis · RCR 17.4 · 85 citations - Correlates between autoantibodies to nucleolar antigens and clinical features in patients with systemic sclerosis (scleroderma).
1988 · Arthritis Rheum · RCR 6 · 203 citations - Serum autoantibody to the nucleolar antigen PM-Scl. Clinical and immunogenetic associations.
1992 · Arthritis Rheum · RCR 5.5 · 165 citations - Muscular and extramuscular clinical features of patients with anti-PM/Scl autoantibodies.
2018 · Neurology · RCR 4.8 · 88 citations - The clinical phenotype of systemic sclerosis patients with anti-PM/Scl antibodies: results from the EUSTAR cohort.
2021 · Rheumatology (Oxford) · RCR 4.4 · 50 citations
Show 20 more of 139 total
- Autoantibody profiles in systemic sclerosis: predictive value for clinical evaluation and prognosis.
2010 · J Dermatol · RCR 4.4 · 146 citations - Frequency of disease-associated and other nuclear autoantibodies in patients of the German Network for Systemic Scleroderma: correlation with characteristic clinical features.
2011 · Arthritis Res Ther · RCR 4.4 · 124 citations - The clinical and immunogenetic features of patients with autoantibodies to the nucleolar antigen PM-Scl.
1992 · Medicine (Baltimore) · RCR 4.3 · 126 citations - Clinical significance of antibodies to Ro52/TRIM21 in systemic sclerosis.
2012 · Arthritis Res Ther · RCR 4.1 · 117 citations - Racial differences in the distribution of systemic sclerosis-related serum antinuclear antibodies.
1994 · Arthritis Rheum · RCR 3.9 · 118 citations - Immunogenetic risk and protective factors for the idiopathic inflammatory myopathies: distinct HLA-A, -B, -Cw, -DRB1, and -DQA1 allelic profiles distinguish European American patients with different myositis autoantibodies.
2006 · Medicine (Baltimore) · RCR 3.2 · 125 citations - Clinical and serologic correlates of anti-PM/Scl antibodies in systemic sclerosis: a multicenter study of 763 patients.
2014 · Arthritis Rheumatol · RCR 3.1 · 73 citations - Myositis-specific and myositis-associated antibodies in a series of eighty-eight Mediterranean patients with idiopathic inflammatory myopathy.
2006 · Arthritis Rheum · RCR 3 · 97 citations - Validation of potential classification criteria for systemic sclerosis.
2012 · Arthritis Care Res (Hoboken) · RCR 2.9 · 73 citations - Novel aspects of autoantibodies to the PM/Scl complex: clinical, genetic and diagnostic insights.
2007 · Autoimmun Rev · RCR 2.8 · 102 citations - Antibodies against PM/Scl-75 and PM/Scl-100 are independent markers for different subsets of systemic sclerosis patients.
2009 · Arthritis Res Ther · RCR 2.8 · 94 citations - Use of a commercial line blot assay as a screening test for autoantibodies in inflammatory myopathies.
2009 · Autoimmun Rev · RCR 2.8 · 95 citations - Long-term outcome of patients with polymyositis/ dermatomyositis and anti-PM-Scl antibody.
2010 · Br J Dermatol · RCR 2.7 · 72 citations - Spectrum of Muscle Histopathologic Findings in Forty-Two Scleroderma Patients With Weakness.
2015 · Arthritis Care Res (Hoboken) · RCR 2.6 · 56 citations - Immunolocalization and partial characterization of a nucleolar autoantigen (PM-Scl) associated with polymyositis/scleroderma overlap syndromes.
1986 · J Immunol · RCR 2.5 · 99 citations - Late-age onset systemic sclerosis-clinical and serological characteristics.
2024 · Clin Rheumatol · RCR 2.5 · 9 citations - The association of anti-Ro52 autoantibodies with myositis and scleroderma autoantibodies.
1999 · J Autoimmun · RCR 2.4 · 80 citations - Systemic sclerosis-associated myopathy.
2007 · Ann N Y Acad Sci · RCR 2.3 · 68 citations - Molecular characterization of an autoantigen of PM-Scl in the polymyositis/scleroderma overlap syndrome: a unique and complete human cDNA encoding an apparent 75-kD acidic protein of the nucleolar complex.
1991 · J Exp Med · RCR 2.3 · 103 citations - HLA class II haplotype and autoantibody associations in children with juvenile dermatomyositis and juvenile dermatomyositis-scleroderma overlap.
2007 · Rheumatology (Oxford) · RCR 2.3 · 76 citations
Reference: B cellIEDB
2 publications
- Computational analysis of high-density peptide microarray data with application from systemic sclerosis to multiple sclerosis.
2012 · Autoimmun Rev · RCR 1 · 35 citations - Identification of an alpha-helical epitope region on the PM/Scl-100 autoantigen with structural homology to a region on the heterochromatin p25beta autoantigen using immobilized overlapping synthetic peptides.
2000 · J Mol Med (Berl) · RCR 0.8 · 32 citations
Sources: PubMed — antigen-level antibody evidence from a custom retrieval. Records matching a controlled set of autoantibody terms (the MeSH descriptors Autoantibodies and Autoantigens, with title and abstract term variants) were obtained through NCBI E-utilities, and their titles and abstracts parsed for constructions that direct an antibody at a named protein rather than for co-occurrence. Captured names were resolved against UniProt nomenclature and each antigen adjudicated individually against the source text. IEDB — curated epitope assays from the Immune Epitope Database (Vita et al., Nucleic Acids Research 2019). Bibliographic records from PubMed and MeSH, U.S. National Library of Medicine; citation metrics from NIH iCite (Hutchins et al., PLoS Biology 2016). Titles link to PubMed; abstracts are not reproduced here. The NLM does not endorse this analysis.
Genetic constraint and essentialitygnomAD · DepMap
Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.
- gnomAD LOEUF (loss-of-function intolerance)
- 0.74
- gnomAD pLI
- 0
- gnomAD missense Z
- 0.87
- DepMap mean gene effect
- -0.58
- DepMap dependency class
- common
Cancer expressionTCGA
Across TCGA tumor cohorts, this protein is over-expressed in roughly 6% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).
OntologyGO
Biological processes
- CUT catabolic process
- DNA repair
- exonucleolytic trimming to generate mature 3'-end of 5.8S rRNA from tricistronic rRNA transcript (SSU-rRNA, 5.8S rRNA, LSU-rRNA)
- histone mRNA catabolic process
- maturation of 5.8S rRNA
- negative regulation of telomere maintenance via telomerase
- nuclear mRNA surveillance
- nuclear polyadenylation-dependent CUT catabolic process
- nuclear polyadenylation-dependent rRNA catabolic process
- nuclear polyadenylation-dependent snoRNA catabolic process
- nuclear polyadenylation-dependent snRNA catabolic process
- nuclear-transcribed mRNA catabolic process
- nuclear-transcribed mRNA catabolic process, nonsense-mediated decay
- poly(A)-dependent snoRNA 3'-end processing
- positive regulation of mRNA cis splicing, via spliceosome
- random inactivation of X chromosome
- regulation of telomerase RNA localization to Cajal body
- ribosomal small subunit biogenesis
- RNA catabolic process
- RNA processing
- rRNA processing
- TRAMP-dependent tRNA surveillance pathway
- nuclear polyadenylation-dependent antisense transcript catabolic process
Molecular functions
- 3'-5'-RNA exonuclease activity
- metal ion binding
- nucleotide binding
- RNA binding
- RNA exonuclease activity
- single-stranded RNA binding
- telomerase RNA binding
Cellular components
Protein domainsUniProt · Pfam · InterPro
- HRDC domain
- 3'-5' exonuclease domain
- HRDC-like superfamily
- Ribonuclease H-like superfamily
- Ribonuclease H superfamily
- HRDC domain superfamily
- HRDC domain
- 3'-5' exonuclease
- Exosome-associated factor Rrp6, N-terminal
- Exosome complex exonuclease Rrp6-like
- Rrp6-like, 3'-5' exonuclease domain
- PMC2NT (NUC016) domain
KeywordsUniProt
InteractionsUniProt · HPA
Protein binding partners of EXOSC10 in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.
Antibody and autoantibody relevanceSeroatlas analysis
Seroatlas reads EXOSC10 as an antibody target. Whether an autoantibody or antibody against EXOSC10 could matter depends on whether native EXOSC10 is physically reachable, whether the body needs it intact, and whether it acts in a disease-relevant tissue.
EXOSC10 is annotated as predominantly intracellular. Intracellular proteins are common autoantibody markers, becoming visible to the immune system after cell injury or altered processing, but are usually markers of disease rather than direct drivers.
Annotation status
The present source text does not explicitly label EXOSC10 as an autoantigen. Seroatlas presents hypothesis context only and does not manufacture a known-serology claim.
Loading the interactive Seroatlas protein explorer...