Exosome component 4 (EXOSC4)

Exosome component 4 (EXOSC4) is a structural subunit of the evolutionarily conserved RNA exosome complex, which is essential for the processing, maturation, and controlled degradation of multiple RNA species within the cell, including ribosomal RNA and aberrant transcripts. EXOSC4 (also known as RRP41) is one of the six core barrel-forming subunits of the exosome, interacting tightly with other subunits to maintain the integrity and function of the complex. Pathogenic mutations in EXOSC4 can destabilize the complex, reduce ribosome biogenesis, and are directly linked to rare neurodevelopmental disorders characterized by global developmental delay, cerebral calcifications, kidney dysfunction, and failure to thrive, indicating the critical requirement for intact exosome function for normal human development and homeostasis. EXOSC4 is not classically viewed as a primary drug target or receptor, but rather as a core molecular component of a fundamental RNA-processing machinery. Disruptions in EXOSC4 are increasingly recognized as genetic contributors to “RNA exosomopathies,” a set of human disorders with central nervous system and multi-organ manifestations. To date, drug interactions or targeted therapies against EXOSC4 have not been established, and there are no well-characterized biomarkers or mechanisms of action for pharmacologic targeting of this subunit.

Other names

Exosome complex component RRP41RRP41SKI6hRrp41pFLJ20591Rrp41pRRP41ASki6pp12AExosome component Rrp41ribosomal RNA-processing protein 41exosome complex exonuclease RRP41