EXOSC1 is a non-catalytic structural component of the nine-subunit RNA exosome complex, a ribonuclease responsible for RNA processing and degradation. As a peripheral component, EXOSC1 stabilizes the catalytic hexameric ring through interactions with EXOSC6 and EXOSC8 1. In the nucleus, the exosome complex processes stable RNAs (rRNA, snRNA, snoRNA), eliminates defective transcripts and non-coding RNAs, and may participate in immunoglobulin class switching 2. Cytoplasmic functions include mRNA turnover, degradation of AU-rich element-containing transcripts, and RNA surveillance pathways 2. Recent evidence demonstrates EXOSC1 also cleaves single-stranded DNA, promoting mutations in renal carcinoma cells and sensitizing them to PARP inhibitors 3. Disease relevance is significant: bi-allelic EXOSC1 variants cause pontocerebellar hypoplasia type 1F, characterized by developmental delay, microcephaly, hypotonia, and cerebellar hypoplasia 1, 4. Pathogenic variants reduce EXOSC1 protein levels and disrupt EXO9 complex stability 1. Additionally, EXOSC1 dysfunction impairs ribosome biogenesis and elevates p53 levels, linking exosome disorders to ribosomopathies 5. In cancer contexts, EXOSC1 appears to be a therapeutic target, with elevated expression correlating with poor prognosis in renal carcinoma and its inhibition identified as a potential strategy to overcome tumor immune evasion 6, 7.