Abstract |
C1q deficiency in both humans and mice is strongly associated with autoimmunity. We have previously shown that bone marrow transplantation (BMT) restored C1q levels in C1q-deficient (C1qa(-/-)) mice. Here, we studied the effect of BMT on autoimmunity in C1qa(-/-) mice. Following irradiation, young C1qa(-/-) or wild-type MRL/Mp mice received bone marrow cells (BMC) from strain-matched wild-type or C1qa(-/-) animals. C1q levels increased rapidly when C1qa(-/-) mice received BMC from wild-type mice. Conversely, they decreased slowly in wild-type mice transplanted with C1qa(-/-) BMC. C1qa(-/-) animals transplanted with C1qa(-/-) BMC demonstrated accelerated disease when compared with wild-type mice given wild-type BMC. In contrast, a significant delay in the development of autoantibodies and glomerulonephritis was observed in C1qa(-/-) mice reconstituted with wild-type BMC, and the impaired clearance of apoptotic cells, previously described in C1qa(-/-) mice, was rectified. Moreover, the autoimmune disease was accelerated in wild-type mice given C1qa(-/-) BMC compared to animals transplanted with wild-type cells. These results provide supporting evidence that BMT may be a therapeutic option in the treatment of autoimmunity associated with human C1q deficiency.
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Authors | Josefina Cortes-Hernandez, Liliane Fossati-Jimack, Franz Petry, Michael Loos, Shozo Izui, Mark J Walport, H Terence Cook, Marina Botto |
Journal | European journal of immunology
(Eur J Immunol)
Vol. 34
Issue 12
Pg. 3713-22
(Dec 2004)
ISSN: 0014-2980 [Print] Germany |
PMID | 15517607
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
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Topics |
- Animals
- Autoimmune Diseases
(immunology, therapy)
- Bone Marrow Transplantation
- Complement C1q
(deficiency, immunology, metabolism)
- Mice
- Phagocytosis
(immunology, physiology)
- Spleen
(metabolism)
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