EXOSC8 is a non-catalytic structural component of the RNA exosome, a multisubunit ribonuclease complex responsible for RNA processing and degradation. The exosome catalyzes 3'→5' exoribonuclease activity on diverse RNA substrates, including AU-rich element (ARE) containing mRNAs in the cytoplasm and precursor forms of stable RNAs (rRNA, snRNA, snoRNA) in the nucleus 1. EXOSC8 directly binds ARE-containing RNAs and facilitates their rapid degradation by recruiting ARE-binding proteins to the exosome. The protein also participates in elimination of aberrant transcripts and RNA surveillance pathways that prevent translation of defective mRNAs. Recessive mutations in EXOSC8 cause pontocerebellar hypoplasia type 1c (PCH1c), an autosomal recessive neurodegenerative disease characterized by cerebellar and pontine hypoplasia, spinal motor neuron disease, and abnormal CNS myelination 2 3. Experimental evidence shows that reduced EXOSC8 expression leads to accumulation of ARE-containing mRNAs encoding myelin proteins, causing disrupted myelin formation and explaining the neurodegenerative phenotype 2. Beyond inherited neurodegeneration, EXOSC8 is upregulated in colorectal cancer, where it promotes tumorigenesis by enhancing ribosome biogenesis and suppressing p53-mediated growth arrest 4. No approved therapeutics currently target EXOSC8, though its role in both neurodegeneration and cancer progression identifies it as a potential therapeutic target.