C1r/C1s deficiency is insufficient to induce murine systemic lupus erythematosus

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C1s deficiency is strongly associated with the development of human systemic lupus erythematosus (SLE); however, the mechanisms by which C1s deficiency contributes to the development of SLE have not yet been elucidated in detail. Using ICR-derived-glomerulonephritis (ICGN) mouse strain that develops SLE and very weakly expresses C1s in the liver, we investigated the protective roles of C1s against SLE. A genetic sequence analysis revealed complete deletion of the C1s1 gene, a mouse homolog of the human C1s gene, with partial deletion of the C1ra and C1rb genes in the ICGN strain. This deletion led to the absence of C1r/C1s and a low level of C1q in the circulation. In order to investigate whether the C1r/C1s deficiency induces SLE, we produced a congenic mouse strain by introducing the deletion region of ICGN into the C57BL/6 strain. Congenic mice exhibited no C1r/C1s and a low level of C1q in the circulation, but did not have any autoimmune defects. These results suggest that C1r/C1s deficiency is not sufficient to drive murine SLE and also that other predisposing genes exist in ICGN mice.

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This study was supported by a Grant-in-Aid for Scientific Research from the Japan Society for the Promotion of Science. We thank Ms. Kyoko Sawada for her excellent technical assistance. We are also grateful to the late Dr. Yoshie Yamamoto for her helpful discussions.

Author information


  1. Laboratory of Animal Models for Human Diseases, National Institutes of Biomedical Innovation, Health and Nutrition, Ibaraki, Osaka, 567-0085, Japan

    • Kozue Uchio-Yamada
  2. Laboratory of Adjuvant Innovation, National Institutes of Biomedical Innovation, Health and Nutrition, Ibaraki, Osaka, 567-0085, Japan

    • Mayuri Tanaka
  3. Osaka International University, Moriguchi, Osaka, 570-8555, Japan

    • Noboru Manabe


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Conflict of interest

The authors declare that they have no conflict of interest.

Corresponding author

Correspondence to Kozue Uchio-Yamada.

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