MIP 342 Lecture Notes - Bruton'S Tyrosine Kinase, Thymus, Meningococcal Disease

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The big picture: lessons about the immune system from immunodeficiency diseases. Naturally occurring immunodeficiency diseases teach us about the role of different parts of the immune system in defense against pathogens. Immunodeficiency diseases also teach us about how the immune system controls autoimmunity. Presenting in early childhood, infections with many types of organisms (viruses, bacteria, fungal) Presenting later in life, infections with bacterial organisms. B and t cells (scid; severe combined immunodeficiencies) Susceptible to all pathogens and many microbes that are not a problem for people with normal immune systems. Can"t control c3 convertase red blood cell lysis, anemia. Lack of c3, other effector complement molecules uncontrolled bacterial infections. Results in failure to opsonize and control immune complex deposition. Opsonization probably most important (since deficiencies in the membrane attack complex do not predispose to immune complex deposition) Opsonization for phagocytosis important for other types of extracellular bacteria infections.

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