Extracellular heme:DNA complexes promote oxidative stress and inflammation during lupus-associated hemolysis

L Lubica Janovicova (Department of Surgery, Division of Surgical Sciences, Cancer Research Institute, Beth Israel Deaconess Medical Center, Harvard Medical School) M Monika Janikova (Department of Surgery, Division of Surgical Sciences, Cancer Research Institute, Beth Israel Deaconess Medical Center, Harvard Medical School) M Michal Pastorek (Department of Surgery, Division of Surgical Sciences, Cancer Research Institute, Beth Israel Deaconess Medical Center, Harvard Medical School) E Eva Csizmadia (Department of Surgery, Division of Surgical Sciences, Cancer Research Institute, Beth Israel Deaconess Medical Center, Harvard Medical School) C Cherith Yoder (Department of Surgery, Division of Surgical Sciences, Cancer Research Institute, Beth Israel Deaconess Medical Center, Harvard Medical School) A Alexandra Lesayova (Institute of Molecular Biomedicine, Faculty of Medicine, Comenius University) L Liam Geyer (Department of Surgery, Division of Surgical Sciences, Cancer Research Institute, Beth Israel Deaconess Medical Center, Harvard Medical School) V Vasileios C. Kyttaris G George C. Tsokos P Peter Celec (Institute of Molecular Biomedicine, Faculty of Medicine, Comenius University) B Barbara Wegiel (Department of Surgery, Division of Surgical Sciences, Cancer Research Institute, Beth Israel Deaconess Medical Center, Harvard Medical School)

Abstract

Hemolysis is associated with the release of damage-associated molecular patterns, including free heme and extracellular DNA (ecDNA). Using several mouse models of bleeding anemia and hemolysis, we demonstrate a significant increase in plasma ecDNA, independent of neutrophil extracellular trap formation. This ecDNA forms G-quadruplex (G4) structures, which we detected in both mice and patients with systemic lupus erythematosus. Catalytic complexes (DNAzymes) formed by G4 ecDNA and heme drive oxidative stress, tissue injury, and inflammation. In anemic mice lacking deoxyribonuclease 1L3 ( DNase1l3 −/− ), we found elevated polynucleosomal ecDNA in the plasma, reduced expression of the heme-degrading enzyme heme oxygenase-1 in macrophages, but also increased plasma creatinine, renal iron accumulation, and complement C3 deposition along elevated apoptosis and DNA damage. ecDNA isolated from these mice also triggered toll-like receptor 9-dependent inflammatory responses in vitro and in vivo. In summary, these findings suggest that concurrent release of heme and ecDNA during hemolysis promotes inflammation and tissue damage, contributing to lupus pathogenesis.

Article Details

Volume / Issue Vol. 123, Issue 10
Published March 10, 2026
ISSN 0027-8424
Publisher National Academy of Sciences

Authors (11)

L

Lubica Janovicova

Department of Surgery, Division of Surgical Sciences, Cancer Research Institute, Beth Israel Deaconess Medical Center, Harvard Medical School

M

Monika Janikova

Department of Surgery, Division of Surgical Sciences, Cancer Research Institute, Beth Israel Deaconess Medical Center, Harvard Medical School

M

Michal Pastorek

Department of Surgery, Division of Surgical Sciences, Cancer Research Institute, Beth Israel Deaconess Medical Center, Harvard Medical School

E

Eva Csizmadia

Department of Surgery, Division of Surgical Sciences, Cancer Research Institute, Beth Israel Deaconess Medical Center, Harvard Medical School

C

Cherith Yoder

Department of Surgery, Division of Surgical Sciences, Cancer Research Institute, Beth Israel Deaconess Medical Center, Harvard Medical School

A

Alexandra Lesayova

Institute of Molecular Biomedicine, Faculty of Medicine, Comenius University

L

Liam Geyer

Department of Surgery, Division of Surgical Sciences, Cancer Research Institute, Beth Israel Deaconess Medical Center, Harvard Medical School

V

Vasileios C. Kyttaris

G

George C. Tsokos

P

Peter Celec

Institute of Molecular Biomedicine, Faculty of Medicine, Comenius University

B

Barbara Wegiel

Department of Surgery, Division of Surgical Sciences, Cancer Research Institute, Beth Israel Deaconess Medical Center, Harvard Medical School