Deoxyribonuclease-2-alpha

Definition:

Hydrolyzes DNA under acidic conditions with a preference for double-stranded DNA. Plays a major role in the clearance of nucleic acids generated through apoptosis, hence preventing autoinflammation. Necessary for proper fetal development and for definitive erythropoiesis in fetal liver and bone marrow, where it degrades nuclear DNA expelled from erythroid precursor cells.

References:

[1]. Mathieu P Rodero, et al. Type I interferon-mediated autoinflammation due to DNase II deficiency. Nat Commun. 2017 Dec 19;8(1):2176. [Content Brief]

[2]. Ying Hong, et al. Janus kinase inhibition for autoinflammation in patients with DNASE2 deficiency. J Allergy Clin Immunol. 2020 Feb;145(2):701-705.e8. [Content Brief]

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