DNASE2 encodes a lysosomal deoxyribonuclease that functions optimally under acidic conditions and preferentially hydrolyzes double-stranded DNA 1. This enzyme plays a crucial role in the clearance of nucleic acids generated during apoptosis, serving as a key mechanism to prevent autoinflammation 1. DNASE2 is essential for proper fetal development and definitive erythropoiesis in fetal liver and bone marrow, where it degrades nuclear DNA expelled from erythroid precursor cells 1. The gene is located on chromosome 19.2-p13.1 2. DNASE2 also serves as a biomarker for differentiated epithelial cells in human meibomian glands, with expression increasing during cellular differentiation 3. Clinically, DNASE2 deficiency causes monogenic lupus, characterized by early-onset systemic lupus erythematosus with features including polyarthritis, erythematous rash, neurological involvement, and developmental delays 4. The enzyme has been investigated for potential therapeutic applications, including targeted cancer therapy through transgenic expression systems 5. Understanding DNASE2 function is critical as its elimination can lead to various diseases, representing an important area of emerging research 1.