BIOL 2050U Lecture Notes - Hyper Igm Syndrome, Cd154, Adenoid
Document Summary
**no b cells no plasma cells no antibodies** x-linked; heterozygous females are carriers and normal. Onset = 6 mos. (after passive immunity is gone) Infections = pneumococcus, influenza, staph (heavily encapsulated bacteria that can evade phagocytosis) enterovirus infection (echo, coxsackie, polio) fatal if untreated because virus is never cleared. Molecular basis structural ig chain inactive (found in x-chromosome) no preb prob transition. Btk point mutations that prevent substrate binding (involved in phos. cascade) precise function of btk is still unknown. Iv administration of monomeric gammaglobulin avoid live viral vaccines disadvantages: - no iga or igm. Not truly active immunity, but just the average of a population"s igg production (only recognizes common ag"s) Expensive: cd4 cells do not allow for class switching: x-linked hyper igm syndrome susceptibility to bacteria and other microorganisms (viruses, fungi, parasites) (b cell) (t cell) Cd40l defect cannot interact w/ b cells no class switching from igm cannot interact w/ apc"s no antigen processing .
