| Autor: |
Martini Paolo GV, Cook Lynette C, Alderucci Scott, Norton Angela W, Lundberg Dianna M, Fish Susan M, Langsetmo Knut, Jönsson Göran, Lood Christian, Gullstrand Birgitta, Zaleski Kate J, Savioli Nancy, Lottherand Jason, Bedard Charles, Gill John, Concino Michael F, Heartlein Michael W, Truedsson Lennart, Powell Jan L, Tzianabos Arthur O |
| Jazyk: |
angličtina |
| Rok vydání: |
2010 |
| Předmět: |
|
| Zdroj: |
BMC Immunology, Vol 11, Iss 1, p 43 (2010) |
| Druh dokumentu: |
article |
| ISSN: |
1471-2172 |
| DOI: |
10.1186/1471-2172-11-43 |
| Popis: |
Abstract Background Complement C2 deficiency is the most common genetically determined complete complement deficiency and is associated with a number of diseases. Most prominent are the associations with recurrent serious infections in young children and the development of systemic lupus erythematosus (SLE) in adults. The links with these diseases reflect the important role complement C2 plays in both innate immunity and immune tolerance. Infusions with normal fresh frozen plasma for the treatment of associated disease have demonstrated therapeutic effects but so far protein replacement therapy has not been evaluated. Results Human complement C2 was cloned and expressed in a mammalian cell line. The purity of recombinant human C2 (rhC2) was greater than 95% and it was characterized for stability and activity. It was sensitive to C1s cleavage and restored classical complement pathway activity in C2-deficient serum both in a complement activation ELISA and a hemolytic assay. Furthermore, rhC2 could increase C3 fragment deposition on the human pathogen Streptococcus pneumoniae in C2-deficient serum to levels equal to those with normal serum. Conclusions Taken together these data suggest that recombinant human C2 can restore classical complement pathway activity and may serve as a potential therapeutic for recurring bacterial infections or SLE in C2-deficient patients. |
| Databáze: |
Directory of Open Access Journals |
| Externí odkaz: |
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