(A) Resting endothelium is protected against complement activation by complement regulators. (B) Normal serum deposited small amounts of C3b (and C3b degradation products) and C5b-9 on ADP-activated endothelial cells, but complement regulatory proteins presented in normal serum inhibited propagation of complement activation on the surface of endothelium. (C) In the presence of serum from a patient with acute aHUS, complement regulation on endothelial cells is ineffective, resulting in a larger number of C5b-9 complexes deposited on the surface of resting endothelium. (D) Serum samples from patients with acute aHUS, aHUS in remission, and healthy carriers of complement mutations deposited C5b-9 on the surface of ADP-activated endothelial cells. In this assay, HMEC-1 were used as the source of endothelial cells.

(A) Resting endothelium is protected against complement activation by complement regulators. (B) Normal serum deposited small amounts of C3b (and C3b degradation products) and C5b-9 on ADP-activated endothelial cells, but complement regulatory proteins presented in normal serum inhibited propagation of complement activation on the surface of endothelium. (C) In the presence of serum from a patient with acute aHUS, complement regulation on endothelial cells is ineffective, resulting in a larger number of C5b-9 complexes deposited on the surface of resting endothelium. (D) Serum samples from patients with acute aHUS, aHUS in remission, and healthy carriers of complement mutations deposited C5b-9 on the surface of ADP-activated endothelial cells. In this assay, HMEC-1 were used as the source of endothelial cells.

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