Signaling Molecules

  1. Achondroplasia has a defective?
    Fibroblast Growth Factor (FGF) receptor
  2. TGF-beta has what effect on osteoblasts and osteoclasts?
    Increase osteoblast, decrease osteoclasts
  3. Insulin-like grwoth factor (IGF-1, IGF-II) has what effect on osteoblasts and osteoclasts?
    Increase osteoblast, none on osteoclast
  4. Matrix metalloproteinase-13 (MMP-13) is inhibited by?
  5. mediates viral immunity and pairs with beta2-microglobulin
    MHC I
  6. HLA-A3
  7. HLA-B27
    PAIR: Psoriasis, Ankylosing spondylitis, Inflammatory bowel disease, Reiter's syndrome
  8. HLA-B8
    Grave's Disease
  9. HLA-DR2
    MS, Hay fever, SLE, Goodpasture's
  10. HLA-DR3
    Diabetes Mellitus Type 1
  11. HLA-DR4
    Rheumatoid arthritis, Diabetes Mellitus Type 1
  12. HLA-DR5
    Pernicious anemia --> B12 deficiency, Hashimoto's thyroiditis
  13. HLA-DR7
    Steroid-responsive nephrotic syndrome
  14. Superantigens from S. pyogenes and S. aureus infect by
    cross-link the beta-region of the T-cell receptor to th eMHC Class II on APCs. Results in the uncoordinated relase of IFN-gamma from Th1 cells and subsequence release of IL-1, IL-6, TNF-alpha from macrophages
  15. Endotoxins/lipopolysaccharide from gram-negative bacteria exert their effect by:
    directly stimulating macrophages by bind to endotoxin receptor CD14. (CD14 are only on macrophages)
  16. Proinflammatory agent that induces insulin resistance by activation of serine kinase, which inhibits downstream signaling from macrophages
  17. Agent important for tissue regeneration & repair from T-cells, platelets, endothelial cells, smooth muscle cells, fibroblasts & keratinocytes
  18. stimulates macrophages and Th1 cells while inhibiting Th2 cells - secreted by antigen presenting cells
  19. Agent that mediates septic shock, causes leukocyte recruitment, vascular leak and secreted by macrophages
  20. Hereditary angioedema
    Deficiency of C1 esterase inhibitor
  21. Deficiency of C5-C8 often leads to?
    Neisseria bacteremia
  22. Deficiency of C3 leads to?
    recurrent pyogenic sinus and respiratory tract infections; increase susceptibility to type III hypersensitivity reactions
  23. These agents inhibit biral protein syntheis by inducing the production of ribonuclease and activating NK cells
    IFN-alpha & IFN-beta
  24. fever, hives, joint pain, proteinuria, lymphadenopahty 5-10 days after antigen exposure (usually a drug)
    Serum sickness
  25. Local subacute antibody-mediated hypersensitivy characterized by edema, necrosis and activation of complement
    Arthus reaction
  26. Antinuclear antibodies (ANA)
  27. Anti-dsDNA, anti-Smith
    Specific for SLE
  28. Antihistone
    Drug-induced lupus
  29. Anti-IgG
    Rheumatoid factor - rheumatoid arthritis
  30. anticentromere
    Scleroderma (CREST)
  31. anti-Scl-70 (anti-DNA topoisomerase I)
    Scleroderma (diffuse)
  32. Antimitochondrial
    primary biliary cirrhosis
  33. Anti-basement membrane
    Goodpasture's syndrome
  34. Anti-desmoglein
    Pemphigus vulgaris
  35. Antimicrosomal
    hashimoto's thyroiditis
  36. Antithyroglobulin
    Hashimoto's thyroiditis
  37. anti-Jo-1
    Polymyositis, dermatomyositis
  38. Anti-SSA, Anti-SSB
    Sjogren's syndrome
  39. Anti-U1 RNP
    Mixed connective tissue disease
  40. Anti-smooth muscle
    Autoimmune hepatitis
  41. Anti-glutamate decarboxylase
    Type 1 diabetes mellitus
  42. c-ANCA
    Wegener's granulomatosis
  43. p-ANCA
    other vasculitides
  44. B-cell disorders
    Bruton's agammaglobulinemia, Hyper-IgM syndrome, Selective Ig Deficiency, Common variable immunodeficiency
  45. Defection CD40L on helper T cells with the inability to class-switch B cells
    Hyper-IgM syndrome
  46. Defective BTK gene that blocks b-cell differentiation/maturation
    Bruton's agammaglobulinemia - decrease immunoglobulins in all classes
  47. Sinus and lung infections, milk allergies, and diarrhea
    Selective Ig deficiency, IgA most common
  48. Defective B cell maturation, many causes usually acquired in the 20s-30s, with decrease plasma cells and immunoglobulins even though there are normal B cells
  49. Tetany, recurrent viral/fungal infections, congenital heart and great vessel defects
    Thymic aplasia (DiGeorge Syndrome)
  50. Decrease Th1 response causing disseminated mycobacterial infections
    IL-12 receptor deficiency
  51. Th cells fail to produce IFN-gamma --> inability of neutrophils to respond to chemotactic stimuli
    Hyper-IgE syndrome
  52. FATED: coarse facies, cold staphylococcal abscesses, retained primary teeth, increased IgE, Dermatologic problems (eczema)
    Hyper-IgE syndrome (Job's syndrome)
  53. Chronic mucocutaenous candidiasis on the skin and mucous membranes
    T-cell dysfunction
  54. Cerebellar defects, spider angiomas, IgA deficiency
  55. Defect in DNA repair enzymes
  56. thrombocytopenic purpura, infections, eczema
    wiskott-aldrich syndrome
  57. X-linked recessive defect with progressive deletion of B and T cells, and decrease in IgM while increases in IgE and IgA
    Wiskott-Aldrich syndrome
  58. Neutrophilia with recurrent bacterial infections, absent pus formation, and delayed separation of umbilicus
    Leukocyte adhesion deficiency (type 1)
  59. Defect in LFA-1 integrin protein
    Leukocyte adhesion deficiency
  60. recurrent pygoenic infections by staphylococci and streptococcil partial albinism, and peripheral neuropathy
    Chediak-Higashi syndrome
  61. AR defect in microtubular function with decrease phagocytosis
    Chediak-Higashi syndrome
  62. Increase susceptibility to catalase-positive organisms (S. aureus, E.coli, Aspergillus)
    Chronic granulomatous Disease
  63. Negative Nitroblue tetrazolium dye reduction test indicates:
    Chronic granulomatous disease
  64. Which immunosuppressant binds to cyclophilins?
  65. Which immunosuppressant blocks the differentiation and activation of T cells by inhibiting calcineurin, thus preventing the production of IL-2 and its receptor?
  66. Which immunosuppressant binds to FK-binding protein and inhibits the secretion of IL-2
  67. Nephrotoxicity, peripheral neuropahty, hypertension, pleural effusion and hyperglycemia are all side effects of:
  68. What immunosuppressant is an antimetabolic precursor of 6-MP that interferes with the metabolism and synthesis of nucleic acids?
  69. Which immunosuppressants toxic effects can be increased by allopurinol?
  70. Which monoclonal antibody binds to CD3?
  71. Which binds to mTOR and inhibits response to IL2?
    Sirolimus (rapamycin)
  72. What inhibits de novo guanine synthesis and blocks lymphocyte production?
    Mycophenolate mofetil
  73. What monoclonal antibody has a high affinity for the IL2 receptor on activated T-cells?
  74. Aldesleukin (IL-2)
    • renal cell carcinoma
    • metastatic melanoma
  75. Gilgrastim (GCSF)
    recovery of bone marrow
  76. Sargramostim (GMCSF)
    recovery of bone marrow
  77. alpha interferon
    • hepatitis B and C
    • Kaposi's sarcoma
    • leukemias
    • malignant melanoma
  78. beta interferon
  79. gamma interferon
    Chronic granulomatous disease
  80. Oprelvekin (IL-11)
Card Set
Signaling Molecules