InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: EXOSC3

Summary

InnateDB Protein

IDBP-479331.4

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

EXOSC3

Protein Name

exosome component 3

Synonyms

Species

Homo sapiens

Ensembl Protein

ENSP00000418422

InnateDB Gene

IDBG-66000 (EXOSC3)

Protein Structure

UniProt Annotation

Function

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. EXOSC3 as peripheral part of the Exo-9 complex stabilizes the hexameric ring of RNase PH-domain subunits through contacts with EXOSC9 and EXOSC5. {ECO:0000269PubMed:11782436, ECO:0000269PubMed:17545563, ECO:0000269PubMed:19056938, ECO:0000269PubMed:21255825}.

Subcellular Localization

Cytoplasm. Nucleus, nucleolus. Nucleus.

Disease Associations

Pontocerebellar hypoplasia 1B (PCH1B) [MIM:614678]: A severe autosomal recessive neurologic disorder characterized by a combination of cerebellar and spinal motor neuron degeneration beginning at birth. There is diffuse muscle weakness, progressive microcephaly, global developmental delay, and brainstem involvement. {ECO:0000269PubMed:22544365}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 45 experimentally validated interaction(s) in this database.

Experimentally validated
Total	45 [view]
Protein-Protein	45 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0000175	3'-5'-exoribonuclease activity
GO:0003723	RNA binding
GO:0005515	protein binding

Biological Process

GO:0000288	nuclear-transcribed mRNA catabolic process, deadenylation-dependent decay
GO:0006364	rRNA processing
GO:0010467	gene expression
GO:0016070	RNA metabolic process
GO:0016071	mRNA metabolic process
GO:0043928	exonucleolytic nuclear-transcribed mRNA catabolic process involved in deadenylation-dependent decay
GO:0045006	DNA deamination
GO:0045190	isotype switching
GO:0071034	CUT catabolic process
GO:0090503	RNA phosphodiester bond hydrolysis, exonucleolytic

Cellular Component

GO:0000176	nuclear exosome (RNase complex)
GO:0000177	cytoplasmic exosome (RNase complex)
GO:0000178	exosome (RNase complex)
GO:0005634	nucleus
GO:0005730	nucleolus
GO:0005737	cytoplasm
GO:0005829	cytosol
GO:0035327	transcriptionally active chromatin