| Autor: |
Lubka T, Roumenina, Roxane, Roquigny, Caroline, Blanc, Nelly, Poulain, Stéphanie, Ngo, Marie-Agnès, Dragon-Durey, Véronique, Frémeaux-Bacchi |
| Rok vydání: |
2013 |
| Předmět: |
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| Zdroj: |
Methods in molecular biology (Clifton, N.J.). 1100 |
| ISSN: |
1940-6029 |
| Popis: |
The atypical hemolytic uremic syndrome (aHUS) is a paradigm of a disease, caused by overactivation of the alternative complement pathway secondary to a not well-understood trigger event. About 60 % of the patients present genetic or acquired abnormalities in the proteins of the alternative complement pathway. In 40 % of the cases the affected protein is the complement regulator Factor H (FH)-30 % due to mutations and 10 % because of anti-FH autoantibodies. Here we describe the detailed protocol for a rapid test to analyse the functional defect associated with genetic or acquired FH-related abnormalities. It can be applied for the characterization of the underlying complement defect in aHUS, based on spontaneous lysis of non-sensitized sheep erythrocytes in contact with patients' plasma or serum. |
| Databáze: |
OpenAIRE |
| Externí odkaz: |
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