Article
The RNA exosome maintains cellular RNA homeostasis by controlling transcript abundance in the brain.
Cell reports - 27 Jan 2026
Higginson Lauryn A, Wang Xingjun, He Kevin, Torstrick Maggie, Kim Minhoo, Wijeratne H R Sagara, Runnebohm Avery M, Benayoun Bérénice A, MacLean Adam, Chanfreau Guillaume F, Morton Derrick J
Abstract excerpt
Intracellular ribonucleases (RNases) are essential for maintaining accurate RNA levels. Inherited mutations in genes encoding ubiquitous RNases are associated with human diseases, primarily affecting the nervous system. Recessive mutations in genes encoding the evolutionarily conserved RNA exosome, an RNase complex, cause syndromic neurodevelopmental disorders, such as pontocerebellar hypoplasia type 1b (PCH1b),...
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