EXOSC8 encodes a non-catalytic structural component of the RNA exosome complex, a critical multisubunit ribonuclease involved in RNA processing and degradation 1. As part of the exosome core, EXOSC8 participates in the degradation of AU-rich element (ARE)-containing mRNAs and plays essential roles in ribosome biogenesis, particularly in 18S rRNA processing and 40S ribosomal subunit maturation 23. The protein functions both in nuclear RNA surveillance pathways and cytoplasmic mRNA turnover 1. Homozygous mutations in EXOSC8 cause pontocerebellar hypoplasia type 1C (PCH1C), a rare autosomal recessive neurodegenerative disease characterized by cerebellar hypoplasia, spinal muscular atrophy, and abnormal myelination 42. Disease mechanisms involve disrupted mRNA metabolism, specifically affecting myelin protein-encoding transcripts, leading to imbalanced myelin protein supply and subsequent demyelination 2. Clinical presentation includes progressive neurological deterioration with cerebellar atrophy, motor neuron disease, and developmental delays 56. In cancer contexts, EXOSC8 upregulation promotes tumorigenesis through enhanced ribosome biogenesis and translation, suggesting potential therapeutic targeting opportunities 7. The protein's dual roles in RNA surveillance and ribosome maturation make it critical for cellular homeostasis and neurological development.