Gene Symbol: Exosc3
Description: exosome component 3
Alias: 2310005D06Rik, AI593501, Rrp40, exosome complex component RRP40, exosome complex exonuclease RRP40, ribosomal RNA-processing protein 40
Rudnik Schoneborn S, Senderek J, Jen J, Houge G, Seeman P, Puchmajerova A, et al
. Pontocerebellar hypoplasia type 1: clinical spectrum and relevance of EXOSC3 mutations. Neurology. 2013;80:438-46 pubmed publisher
..Gene defects had been discovered only in single patients until the recent identification of EXOSC3 mutations in several families with relatively mild course of PCH1...
Pefanis E, Wang J, Rothschild G, Lim J, Chao J, Rabadan R, et al
. Noncoding RNA transcription targets AID to divergently transcribed loci in B cells. Nature. 2014;514:389-93 pubmed publisher
..Here we generated a mouse model in which the essential subunit Exosc3 of the RNA exosome complex can be conditionally deleted...
Lim J, Giri P, Kazadi D, Laffleur B, Zhang W, Grinstein V, et al
. Nuclear Proximity of Mtr4 to RNA Exosome Restricts DNA Mutational Asymmetry. Cell. 2017;169:523-537.e15 pubmed publisher
..Our study suggests that strand-specific DNA mutagenesis-associated mechanisms will play major roles in other undiscovered aspects of organismic development. ..
Basu U, Meng F, Keim C, Grinstein V, Pefanis E, Eccleston J, et al
. The RNA exosome targets the AID cytidine deaminase to both strands of transcribed duplex DNA substrates. Cell. 2011;144:353-63 pubmed publisher
..Our findings reveal a role for noncoding RNA surveillance machinery in generating antibody diversity. ..
Sun J, Keim C, Wang J, Kazadi D, Oliver P, Rabadan R, et al
. E3-ubiquitin ligase Nedd4 determines the fate of AID-associated RNA polymerase II in B cells. Genes Dev. 2013;27:1821-33 pubmed publisher
..Our study also identifies Nedd4 as a regulator of noncoding RNAs that are generated by stalled RNA polII genome-wide. ..
Pefanis E, Wang J, Rothschild G, Lim J, Kazadi D, Sun J, et al
. RNA exosome-regulated long non-coding RNA transcription controls super-enhancer activity. Cell. 2015;161:774-89 pubmed publisher
..in differentiated B cells and pluripotent embryonic stem cells (ESCs) by conditional mutagenesis of core (Exosc3) and nuclear RNase (Exosc10) components of RNA exosome and identified a vast number of long non-coding RNAs (..
Rialdi A, Hultquist J, Jimenez Morales D, Peralta Z, Campisi L, Fenouil R, et al
. The RNA Exosome Syncs IAV-RNAPII Transcription to Promote Viral Ribogenesis and Infectivity. Cell. 2017;169:679-692.e14 pubmed publisher
..Our results suggest that evolutionary arms races have shaped the cellular RNA quality control machinery. ..