The EXOSC3 antibody is a tool used to detect the EXOSC3 protein, a critical component of the human exosome complex. The exosome, a multi-subunit machinery conserved across eukaryotes, plays a central role in RNA processing and degradation. Specifically, it mediates 3'→5' degradation of various RNA substrates, including mRNAs, rRNAs, and non-coding RNAs, ensuring cellular RNA homeostasis. EXOSC3 (Exosome Component 3), also known as RRP40 or hRrp40. forms part of the exosome’s nine-core protein structure and is essential for its structural integrity and enzymatic activity. Mutations in the EXOSC3 gene are linked to pontocerebellar hypoplasia type 1B (PCH1B), a severe neurodegenerative disorder characterized by impaired brain development, highlighting its biological significance.
Antibodies targeting EXOSC3 are widely employed in research to study exosome assembly, RNA metabolism, and disease mechanisms. They enable the detection of EXOSC3 expression levels, subcellular localization (primarily nuclear/cytoplasmic), and interactions via techniques like Western blotting, immunohistochemistry, and immunofluorescence. Such studies have clarified EXOSC3’s role in ribosome biogenesis, mRNA surveillance, and its tissue-specific expression patterns. Additionally, EXOSC3 antibodies aid in exploring pathogenic mutations, as PCH1B-associated variants often destabilize the exosome, leading to RNA processing defects. These tools are vital for advancing understanding of RNA biology and developing therapeutic strategies for exosome-related disorders. Commercial EXOSC3 antibodies are typically validated for specificity using knockout controls or siRNA-based depletion.