FEATURES

DESCRIPTION

Also known as EXOSX_HUMAN, EXOSC10, PMSCL, PMSCL2, RRP6. 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 MTREX, C1D and MPHOSPH6 with the RNA exosome involved in the maturation of 5.8S rRNA. Component of the RNA exosome complex (PubMed:20531389, PubMed:26166824, PubMed:29906447). 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 (PubMed:20531389). Interacts with C1D and MPHOSPH6 (PubMed:17412707). Interacts with ALYREF/THOC4 (PubMed:17234882). Interacts with MTREX; the interaction mediates the association of MTREX with nuclear RNA exosomes (PubMed:26166824). Interacts with DHX36; this interaction occurs in a RNase-insensitive manner (PubMed:14731398). Interacts with NRDE2 (PubMed:30538148).

EXTERNAL LINKS

EXOSC10

Mutations

Mutation(s) distribution

Mutations table