The exosome is a multisubunit complex composed of several highly conserved subunits, some of which are 3’ to 5’ exoribonucleases. The complex is involved in a variety of cellular processes and is responsible for degrading unstable mRNAs that contain AU-rich (ARE) elements in their untranslated 3’ region. EXOSC4 (exosome component 4), also known as SKI6, RRP41 or p12A, is a 245 amino acid protein that localizes to both the nucleus and the cytoplasm and shares 96% sequence identity with its mouse counterpart. Functioning as a component of the exosome complex, EXOSC4 exhibits 3'-5' exonuclease activity and is required for the 3'-processing of 7S pre-RNA to mature 5.8S rRNA. The gene encoding EXOSC4 maps to human chromosome 8, which consists of nearly 146 million base pairs, houses more than 800 genes and is associated with a variety of diseases and malignancies.