RNA exosome complex (RNA exosome)

The RNA exosome complex is a multi-subunit ribonuclease assembly fundamental to RNA metabolism in all eukaryotes (UniProt). It is responsible for the 3' to 5' degradation and processing of a diverse range of RNA species, including messenger RNA (mRNA), ribosomal RNA (rRNA), and small nuclear RNAs (PubMed, PMID: 26373313). The complex is composed of a non-catalytic nine-subunit core (EXOSC1-9) that serves as a scaffold for the catalytic subunits DIS3 and EXOSC10 (Wikipedia). Mutations in the genes encoding exosome subunits are associated with severe neurodegenerative disorders, such as pontocerebellar hypoplasia and spinal muscular atrophy-like syndromes (NIH). Additionally, the complex is a major autoantigen in patients with polymyositis-scleroderma overlap syndrome, often referred to as the PM/Scl complex (StatPearls). In cancer therapy, the exosome is a target for certain antimetabolites like 5-fluorouracil, which inhibits its ability to process rRNA, leading to cell death (PubMed, PMID: 17507650). Given its essential role in cellular homeostasis, therapeutic targeting of the exosome requires careful consideration of potential systemic toxicity.

Other names

Exosome complexPM/Scl complexEXOSC complexRibonuclease complex

Inhibition of 3'-5' exoribonuclease activity and disruption of RNA processing (PubMed, PMID: 17507650)