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Complement plays a role in the recognition, opsonization, and killing or clearance of invading microorganisms, immune complexes and altered host cells.
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There are three main pathways of complement activation: the classical pathway (C1 dependent), the lectin route (mannan-binding lectin/ficolin and MASP dependent), in which both C4 and C2 play a role, and the alternative pathway (Factor B, D, and properdin dependent).
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MBL deficiency occurs frequently and acts as a disease-modifying factor. All other complement deficiencies are rare and result in recurrent pyogenic infections or autoimmune disease reminiscent of systemic lupus erythematosus.
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Novel assays have made the diagnosis of complement deficiencies easier.
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C1INH (C1 inhibitor) replacement therapy is available for hereditary (or acquired) angioedema (HAE), and replacement therapy is being developed for MBL (mannan-binding lectin).
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Complement-targeted therapy (e.g., C1INH, soluble CR1, antibodies against C5, C5aR antagonists) will become important in the near future for adjuvant treatment in ischemia-reperfusion injury, transplantation medicine, and inflammatory disease.
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Keywords
- Paroxysmal Nocturnal Hemoglobinuria
- Classical Pathway
- Hereditary Angioedema
- Complement Deficiency
- C1INH Deficiency
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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Mahmoudi, M., Mollnes, T.E., Kuijpers, T.W., Roos, D. (2008). Complement Deficiencies. In: Rezaei, N., Aghamohammadi, A., Notarangelo, L.D. (eds) Primary Immunodeficiency Diseases. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-78936-9_8
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