REXO2 is a 3’-to-5’ exonuclease that specifically targets small single-stranded RNA and DNA oligomers, primarily those that are five nucleotides or less in length . The enzyme degrades these small fragments, which is essential for maintaining cellular homeostasis. It has a higher affinity for oligonucleotides composed of only two nucleotides but can also bind and degrade longer oligonucleotides with lower affinity .
In mitochondria, REXO2 performs dual roles:
This activity is vital for the correct initiation of mitochondrial transcription, as it prevents RNA-primed transcription at non-canonical sites in the mitochondrial genome . Additionally, REXO2 is essential for embryonic development .
REXO2’s role in DNA repair is particularly significant. It helps in the resistance of human cells to UV-C-induced cell death through its involvement in the DNA repair process . This function underscores its importance in maintaining genomic stability and preventing mutations that could lead to diseases.
Mutations or dysregulation of the REXO2 gene have been associated with various diseases, including Pheochromocytoma and Geleophysic Dysplasia 1 . Understanding the function and regulation of REXO2 can provide insights into these conditions and potentially lead to the development of targeted therapies.
Recombinant human REXO2 is produced using Escherichia coli (E. coli) expression systems. The recombinant protein typically includes an N-terminal His-tag for purification purposes . This recombinant form is used in research to study the enzyme’s function, structure, and potential therapeutic applications.