Gain of function mutant of complement factor B K323E mimics pathogenic C3NeF autoantibodies in convertase assays

• Main Authors: Aleksandra Urban (Author), Anna Borowska (Author), Anna Felberg (Author), Lambertus van den Heuvel (Author), Grzegorz Stasiłojć (Author), Elena Volokhina (Author), Marcin Okrój (Author)
• Format: Book
• Published: Taylor & Francis Group, 2018-01-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

Complement convertases are enzymatic complexes, which play a critical role in propagation and amplification of the complement cascade. Under physiological conditions, convertases decay shortly after being formed in either spontaneous or inhibitor-driven process. Prolongation of their half-life by C3...