The EXOSC9 antibody is a tool used to detect the EXOSC9 protein, a critical component of the RNA exosome complex. This evolutionarily conserved multi-subunit complex plays a central role in RNA processing, surveillance, and degradation. Specifically, EXOSC9 (Exosome Component 9), also known as PM/Scl-75 or Rrp45. functions as a structural and catalytic subunit within the exosome’s core. It contributes to 3'→5' exoribonuclease activity, essential for trimming or degrading various RNA substrates, including ribosomal RNA (rRNA), small nuclear RNA (snRNA), and damaged mRNAs.
Mutations or dysregulation of EXOSC9 are linked to human diseases. For example, it is associated with pontocerebellar hypoplasia type 1D (PCH1D), a severe neurodevelopmental disorder, and autoimmune conditions like the PM/Scl overlap syndrome. Autoantibodies against EXOSC9 are occasionally found in patients with systemic sclerosis or dermatomyositis, making it a biomarker of interest.
The EXOSC9 antibody is widely used in research to study RNA metabolism, gene expression regulation, and disease mechanisms. It enables detection of EXOSC9 expression levels via Western blotting, immunofluorescence, or immunoprecipitation. Commercially available antibodies are often validated for specificity in human, mouse, or rat samples. Researchers also employ it to investigate exosome assembly, substrate targeting, and interactions with cofactors like MTR4 or SKIV2L. Its applications extend to exploring therapeutic strategies for exosome-related disorders.