Defective removal of ribonucleotides from DNA promotes systemic autoimmunity

Constitutive low level DNA damage in RNASEH2 deficiency is linked to innate immune activation. Hierarchical clustering of over 16000 transcripts revealed remarkably similar profiles in patients with lupus erythematosus and patients with AGS with up-regulation of genes involved in DNA damage signaling and type I-IFN signaling. Comparison of transcriptional profiles of native RNASEH2-deficient patient fibroblasts with wild type cells.