D. Wiskott–aldrich syndrome (WaS), caused by WaS deficiency
E. Hyper-ige syndrome (also called Job’s syndrome), caused by Stat3 or DOCK8 mutations
F. Chronic granulomatous disease (CGD), caused by production of reactive oxygen species in phagocytes 13.7 Multiple Choice: Pyogenic bacteria are protected by polysaccharide capsules against recognition by receptors on macrophages and neutrophils. antibody-dependent opsonization is one of the mechanisms utilized by phagocytes to ingest and destroy these bacteria. Which of the following diseases or deficiencies directly affects a mechanism by which the immune system controls infection by these pathogens?
A. il-12 p40 deficiency
B. Defects in AIRE
C. WaSp deficiency
D. Defects in C3 13.8 Multiple Choice: Defects in which of the following genes have a phenotype similar to defects in ELA2, the gene that encodes neutrophil elastase?
A. GFI1
B. CD55 (encodes DaF) C. CD59
D. XIAP 13.9 Matching: Match each protein to the associated 13.10 Multiple Choice: Which of the following pathogens primarily evade(s) the immune system by antigenic variation?
A. influenza a virus
B. Herpes simplex virus-1
C. Cytomegalovirus
D. Trypanosoma brucei
E. Plasmodium falciparum
F. Hepatitis B virus 13.11 Human immunodeficiency virus (HiV) produces various immunoevasins. One of these, nef, is exceptionally pleiotropic and a major target of CD8+ t-cell responses. Which of the following is not one of the functions of nef?
A. inhibition of the restriction factor SaMHD1
B. MHC class i downregulation
General references.
Alcami, A., and Koszinowski, U.H.: Viral mechanisms of immune evasion. Immunol. Today 2000, 21:447–455.
De Cock, K.M., Mbori-Ngacha, D., and Marum, E.: Shadow on the continent: public health and HIV/AIDS in Africa in the 21st century. Lancet 2002, 360:67–72.
Finlay, B.B., and McFadden, G.: Anti-immunology: evasion of the host immune system by bacterial and viral pathogens. Cell 2006, 124:767–782.
Hill, A.V.: The immunogenetics of human infectious diseases. Annu. Rev. Immunol. 1998, 16:593–617.
Lederberg, J.: Infectious history. Science 2000, 288:287–293.
Notarangelo, L.D.: Primary immunodeficiencies. J. Allergy Clin. Immunol. 2010, 125:S182–S194.
Xu, X.N., Screaton, G.R., and McMichael, A.J.: Virus infections: escape, resistance, and counterattack. Immunity 2001, 15:867–870.
Section references.
13-1 A history of repeated infections suggests a diagnosis of immunodeficiency.
Carneiro-Sampaio, M., and Coutinho, A.: Immunity to microbes: lessons from primary immunodeficiencies. Infect. Immun. 2007, 75:1545–1555.
Cunningham-Rundles, C., and Ponda, P.P.: Molecular defects in Tand B-cell primary immunodeficiency diseases. Nat. Rev. Immunol. 2005, 5:880–892.