14 Questions
What can uncontrolled complement activation lead to?
Severe pathology
In paroxysmal nocturnal hemoglobinuria (PNH), what results in a deficiency of glycosylphosphatidylinositol-linked surface proteins?
CD59 on hematopoietic stem cells
What is the consequence of CD59 deficiency on PNH red blood cells?
Intravascular hemolysis
What is the main mechanism by which eculizumab acts?
Blocking the proinflammatory effects of terminal complement activation
What has the recent approval of eculizumab validated?
The concept of complement inhibition as an effective therapy
How does eculizumab prevent C5 entry into the C5 convertase?
By binding to C5 with high affinity
What is the primary challenge Rickettsia pose to the immune system?
They infect nonphagocytic cells
What is the main function of CD59 on hematopoietic stem cells?
Inhibiting terminal complement activation
What must work in conjunction with activated macrophages to kill infected endothelial cells in the early stages of infection?
Helper T cells
In the chronological order of the phases of T cell responses, what comes after the Recognition phase?
Clonal Expansion
What specifically results in the clonal expansion of T cells?
Interleukin 2 (IL-2)
What is the first signal when an extracellular antigen encounters a T cell?
Presentation by class II MHC molecules
To where must effector cytotoxic T lymphocytes (CTLs) migrate?
Peripheral tissues
Where do the complementary determining regions (CDRs) occur on the B cell receptor (BCR)?
VH and VL regions
Learn about the rare hemolytic disease Paroxysmal Nocturnal Hemoglobinuria (PNH) and its relationship with the complement system. Understand how somatic mutations in PNH lead to a deficiency of surface proteins, including CD59, and the resulting pathology.
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