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Reconstitution of the complement function in C1q-deficient (C1qa-/-) mice with wild-type bone marrow cells
F Petry1, M Botto, R Holtappels
1Institute of Medical Microbiology and Hygiene and Institute of Virology, Johannes Gutenberg-University, Augustusplatz/Hochhaus, D-55101 Mainz, Germany. fpetry@mail.uni-mainz.de
Journal of Immunology (Baltimore, Md. : 1950)
|September 21, 2001
Summary
Bone marrow transplantation effectively restored C1q levels in C1q-deficient mice, demonstrating that bone marrow cells are the primary source of serum C1q. This finding suggests stem cell therapy for C1q deficiency.
Area of Science:
- Immunology
- Hematology
- Genetics
Background:
- C1q is crucial for immune complex processing and apoptotic cell clearance.
- C1q deficiency in humans and mice causes systemic lupus erythematosus-like symptoms.
- Previous studies indicated monocyte-macrophage lineage cells produce C1q.
Purpose of the Study:
- To investigate if bone marrow cell transplantation can restore C1q levels in C1q-deficient mice.
- To confirm the role of bone marrow-derived cells as the source of serum C1q.
Main Methods:
- C1q-deficient (C1qa-/-) mice received bone marrow transplants from wild-type donors after irradiation.
- Engraftment was confirmed using Y chromosome-specific PCR and genotype differentiation PCR.
- Serum C1q antigen and function levels were measured post-transplantation.
Main Results:
- Serum C1q levels and function rapidly normalized within 6 weeks after bone marrow transplantation in C1qa-/- mice.
- Wild-type mice receiving C1q-deficient bone marrow showed a progressive decrease in serum C1q levels.
- These results confirm bone marrow-derived cells as the source of serum C1q.
Conclusions:
- Bone marrow-derived cells are the source of serum C1q.
- Stem cell transplantation is a viable strategy to reconstitute C1q levels in C1q-deficient individuals.
- This research offers a potential therapeutic approach for hereditary C1q deficiency and related autoimmune diseases.