1. What are the 3 effects of Interferon Alpha, Beta and Gamma have?
    • 1. Interferon Alpha and Beta--stop viral protein synthesis via upregulating ribonuclease
    • 2. Interferon gamma--upregulation of MHC I and II and increased Antigen presentation
    • 3. Activates NK cells to kill virus infected cells
  2. What are the different types of MHC class I?
    • A
    • B
    • C
  3. What are the different types of MHC class II?
    • DR
    • DP
    • DQ
  4. Thymus is embryologically derived from?
    3rd branchial arch
  5. Transmembrane and secreted IgM are both derived from the same mRNA thru what process?
    alternative splicing
  6. What is the enzyme involved in somatic hypermutation, gene conversion and class switching?
    activation induced cytidine deaminase (AID)
  7. Which MHC receptor has the beta 2 microglobulin?
    MHC I
  8. Leukocyte adhesion deficiency Type I is caused by what?
    deficiency of CD18 integrin on leukocyte membrane
  9. Leukocyte adhesion Deficiency Type II is caused by a defect in what?
    Fucosyl transferase---which synthesizes a ligand for a selectin molecule
  10. Chediak-Higashi syndrome is caused by a defect in what?
    defect in microtubular function
  11. Chronic Granulomatous Disease is caused by what?
    deficiency of NADPH
  12. Myeloperoxidase deficiency is caused by what?
    absent MPO-H202
  13. Gram positive or negative bacteria have LPS on their surface?
  14. Toll Like receptors (TLR) are located where in the cell?
    • - cell membrane
    • - endosomes
  15. Signaling thru TLR or NOD receptors induces the expression of what?
  16. Deficiency of C1 esterase inhibitor leads to what condition?
  17. Membrane attack complex defends against what type of bacteria?
    gram-negative bacteria
  18. C5B, C6,C7,C8,C9 make up what?
    membrane attack complex
  19. What are the derivates of common lymphoid progenitor?
    • B cells
    • T cells
    • NK cells
  20. Deficiency of C5-C8 causes what?
    Neisseria bacteremia
  21. Deficiency of DAF (Decay-accelelrating factor) leads to what?
    paroxysmal nocturnal hemoglobinuria
  22. An increased susceptibility to Type III hypersensitive rxn's and recurrent pyogenic sinus and respiratory tract infection are due to a deficiency of what complement factor?
  23. C1 thru C4 are involved in what ?
    viral neutralization
  24. C3b is involved in what?
    opsonization of bacteria
  25. C3a and C5a are involved in what?
    anaphylaxis and neutrophil chemotaxis
  26. What interleukin induces the liver to express acute phase proteins?
  27. Which portion of the Antibody molecule, Fab or Fc determines the isotype?
  28. Which part of the antibody determines the idotype?
  29. To which part of the antibody does the complement bind to?
  30. Which part of the antibody is the amino terminal?
  31. What are the 3 functions of antibodies?
    • -Opsonization
    • -Neutralization
    • -Complement activation
  32. Antibody diversity is generated in 4 ways, what are they?
    • 1. Random recombination of VJ (light chain) and VDJ (heavy chain)
    • 2. Random combination of heavy and light chains
    • 3. Somatic hypermutation after Ag stimulation
    • 4. Addition of nucleotides DNA during recombination by terminal deoxynucleotidyl transferase
  33. B cell is able to detect complement via CD21
  34. Co receptor on a B cell recognizes what?
  35. Cyclosporin is an inhibitor of what?
  36. Protein tyrosine kinases and other signalling molecules are located in what portion of the lipid membrane?
    lipid rafts
  37. In an immunological synapse, which part, the inner or the outer portion functions to anchor the Antigen presenting cell to the T cell?
    The outer portion via LFA-1 (integrin)
  38. What ion is often used to amplify the original signal?
    Ca 2+
  39. Upon binding of the antigen, what part of the T or BCR gets phosphorylated?
  40. Signal # 2 for B cell is what?
    Binding of CD 40 from a T helper cell
  41. Co receptors like CD 19, 20, 21 perform what function on a B cell??
    Complement component binding
  42. MAPK kinase cascade activates what in response to signals?
    Transcription factors
  43. ITIM attract ____________while ITAM attract ____________?
    • ITIM attract protein tyrosine Phosphatase
    • ITAM attract protein tyrosine kinases
  44. What type of receptors activate NFkappaB?
    Toll like receptors
  45. Deficiency of RAG1/2 causes what syndrome?
    Omenn Syndrome
  46. What does H. Pylori release that neutralizes the acidic environment in the stomach?
    ureaase---breaks down urea to NH4
  47. What are the 2 forms of Leprosy?
    • Tuberculoid
    • Lepromatous
  48. What enzymes are used for VDJ recombination?
    • RAG1
    • RAG2
    • Artemis
  49. Omenn Syndrome is caused by what?
    RAG1/2 missense mutation
  50. Bruton's agammaglobulemia is caused by a deficiency of what?
    BTK (Tyrosine kinase)
  51. Hyper IgM is caused by a deficiency of what?
    CD40L on a Helper T cell----> impaired class swithcing
  52. IgG, IgM, IgA are all different what?
  53. What determines the different idiotypes?
    antigenic binding site
  54. What determines the different isotypes?
    Heavy chain
  55. What causes Ataxia-telangiectasia?
    DNA enzyme repair defect
  56. What is the triad of Ataxia-telangiectasia?
    • ataxia
    • angiomas -spider
    • IgA deficiency
  57. B class switching requires two signals, what are they?
    • Signal 1--IL-4, IL-5, IL-6
    • Signal CD40L from a Th cell
  58. What are the 3 function of antibodies?
    • Opsonization
    • Neutralization
    • Complement activation---via IgG and IgM
  59. To what toxins are antibodies given to patients? (Passive immunity)
    • Tetanus
    • Botulism
    • HBV
    • Rabies
  60. Membrane attack complex is activated by what?
    • IgG and IgM
    • molecules on the surface of microbes
  61. What happens in Wiskott-Aldrich syndrome?
    progressive deletion of T and B cells
  62. What 2 antibodies are common in Hashimotos Thyroditis?
    • Thyroglobulin
    • Thyroid peroxidase enzyme
  63. What is amyloidosis?
    deposition of protein in tissues
  64. A condition in which fragments of immunoglobulins are deposited in the tissues is called?
  65. Presence of a large amount of protein in plasma cells is called?
    Russell Bodies
  66. What are the two most important action of the innate immune system?
    • inflammation--recruitment of leukocytes
    • anti-viral response--via dendritic and NK cells
  67. What are the Innate immunity receptors that recognize molecular patterns of microbes?
    • TLR's
    • NLR's
  68. TLRs and NLRs activate what common transcription factor?
    NF-kappa B
  69. What are defensis?
    anti-microbial molecules produced by the epithelial cells of the skin, respiratory and digestive tracts
  70. What 3 factors trigger activation of the complement system?
    • 1. Antigen-antibody complex
    • 2. Microbial surface antigens
    • 3. Mannose binding lectin
  71. What are the 2 ways of activating complement system in the innate immunity?
    • 1-Mannose binding lectin
    • 2- Microbial surface markers/antigens
  72. What are the 2 divisions of the adaptive immunity?
    • Humoral
    • Cellular immunity
  73. The enzyme which mediates the rearrangement of antigen receptor genes in developing lymphocytes is called?
    RAG 1 and 2
  74. What can be used as a T cell marker?
  75. How can lymphoid tumors be identified?
    by analyzing antigen receptor gene rearrangements
  76. Gamma and Sigma T cells which are specialized T cells are found where in the body?
    Epithelial surfaces
  77. What is the antigen binding component of the B cell receptor?
    IgM and IgD antibodies
  78. What receptor on the B cell is used by the Epstein Barr Virus for infection?
  79. What is the complement receptor on the B cells?
  80. What are the two types of dendritic cells?
    • Follicular Dendritic cells
    • Interdigitating dendritic cells
  81. Follicular dendritic cells are located in what two locations?
    • Spleen
    • Lymph Node
  82. Follicular dendritic cells in the spleen and L.node have what type of receptor?
    Fc receptor
  83. Fc receptor is for what two entities?
    • IgG
    • C3b
  84. Balance of what two signals determines whether a NK cell with kill a cell?
    activating and inhibitory signals
  85. What are the inhibitory cell signals that prevent NK cells from killing normal healthy cells?
    MHCI molecules
  86. Pathogens that penetrate the epithelium end up in lymph nodes how?
    Dendritic cells (langherans) cells ingest them and take them to l. nodes
  87. Th1 cells play a role in defense against what microbes?
    intracellular microbes
  88. Th2 cells play a role in defense against what ?
    • helminthic parasites
    • allergy
  89. Which Th cell is implicated in allergy?
  90. Th17 cells play a role in defense against what microbes?
    extracellular bacteria and fungi
  91. Which interleukin stimulates the proliferation of T cells?
  92. What molecules on a T helper cell activates macrophages and stimulates class switch on B cells?
  93. Which Ig is transported across the placental barrier?
  94. What receptor on the phagocytes binds to antibodies?
    Fc receptor--Fc for the Fc region of the antibody molecule
  95. Which Types of Hypersensitivity disorders are mediated by antibodies?
    Types I thru III
  96. Anti-Glutamate decarboxylase antibodies present in what disorder?
    DM I
  97. Antihistone antibodies present in what disorder?
    Drug induced SLE
  98. Anti-smooth muscle in what disorder?
    Autoimmune hepatitis
  99. Synovia cysts are associated with what disorder?
    Rheumatoid arthritis
  100. Anti-IgG antibodies are present in what disorder?
  101. What are epitheloid cells?
    epithelium like transformed macrophages
  102. What is a granuloma?
    aggregation of Epithelioid cells surrounded by lymphocytes
  103. What are the specific lysosomal enzymes?
    • histaminase
    • lysozyme
    • collagenase
    • gelatinase
    • lactoferrin
    • plasminogen activator
    • alkaline phosphatase
  104. What are the azurophilic enzymes?
    • acid hydrolase
    • myeloperoxidase
    • defensins
    • acid hydrolases
    • elastase
    • cathepsin G
    • non specific collagenase
    • proteinase=
  105. Which organism causes Lyme disease?
    borrelia burgorderi
  106. c-ANCA antibodies found in what condition?
    Wegener's granulomatosis
  107. p-ANCA in what disorder?
    other vasculitis
  108. M. Raphe
  109. N. Basalis
  110. N. Cerellus
  111. N. accumbens
  112. S. nigra
  113. Anti-smooth muscle
    immune hepatitis
  114. 3 drugs for Gout?
    • Allopurinol--xanthine oxidase inhibitor
    • Colchicine--microtubule
    • Propenecid--inhibits uric acid reabsorption in PCT and inhibits secretion of penacillin
  115. Self-reactive T cells that become nonreactive without co-stimulatory molecules are called?
  116. Stem cells express what marker?
  117. When does CD19 become expressed on the developing B cell?
    After heavy chain has been rearranged
  118. The heavy chain is rearranged by which stage in the developing B cell?
    Large Pre-B cell
  119. The light chain is rearranged by which stage in the developing B cell?
    Immature B cell
  120. By which stage in the developing B cell is the intracellular IgM expressed?
    Large Pre-B cell
  121. What are Reed-Sternberg cells?
    B cells without specific cell markers
  122. Positive selection of T cells occurs where in the thymus?
  123. Negative selection of T cells in the thymus occurs where?
  124. How is positive and negative selection of T cells different in the thymus?
    • Positive selection---T cells that recognize self antigen are selected for
    • Negative selection--T cells that recognize self antigens are selected against
  125. B cells are found in what area of the spleen?
    white pulp white for Bleach....B cells
  126. T cells are found in what area of the spleen?
    Red pulp
  127. A Superantigen binds to a site other than Ag binding site and elicits what type of syndrome?
    Toxic shock syndrome
  128. Clostridium Difficile causes diarhea usually secondary to an exposure to what agent?
    antibiotics --clindamyocin or ampicillin
  129. Polysaccharides or proteins are sufficient to activate B cells?
  130. Abnormal proliferation of plasma cells in the bone marrow leads to what kind of cancer?
    Multiple Myeloma
  131. What enzyme induces somatic hypermutation, gene conversion and isotype switch?
  132. Hyperacute organ rejection is mediated by what?
    B cell---antibody
  133. Acute, GVHD and chronic organ rejection is mediated by what?
    T cell
  134. What two superantigens cause toxis shock syndrome?
    • streptococcus pyogens
    • staphylococcus aureus
  135. What activates macrophages?
  136. What induces the Truncus Arteriousus to become ascending aorta and pulmonary trunk?
    Neural crest cell migration
  137. Humoral rejection of cell mediated rejection is characterized by vasculitis?
  138. Humoral or cell mediated rejection is characterized by monocyte infiltration?
    cell mediated rejection
  139. Primary immunodeficiencies are caused by what?
    genetic factors
  140. Secondary immunodeficiencies are caused ?
    • infections
    • immunosupression
    • malnutrition
  141. Neutrophilia, Omphalitis and recurrent bacterial infections are indicative of what?
    Leukocyte deficiency type I
  142. LAD
    Integrin CD18 defect beta chain
  143. What kinds of organisms cause recurrent infections in a person with Chronic Granulomatous Disease?
    • C
    • A
    • T
    • Aspergillus
    • L
    • Aureus
    • S
    • E. coli
  144. Infections with catalase positive organisms occur in people with what disease?
    Chronic Granulomatous disease
  145. Hyper Ig-E or Job syndrome is caused by what?
    deficient production of IFN-gamma from Th cell
  146. What Immunoglobulin is overproduced in Job syndrome?
  147. Hyper IgM syndrome is caused by what?
    • deficient CD40 on Th cell
    • failed class switching
  148. What is the only B cell immunodeficiency where there is no production of B cells?
    Bruton's agammaglobulinemia
  149. What is the etiology of rheumatic heart disease?
    antibodies produced to group A streptococcus cross react with heart
  150. What is the fluorescent pattern of glomeruli in good pasture?
    ribbon like--continuos
  151. What is the flourescent pattern of staining of glomeruli in SLE or Post streptococcal glomerulonephritis?
    Bumpy Lumpy--interrupted vs ribbon like in good pasture
  152. Cyclophosphamide and isofamide function how?
    covalently linking DNA at guanine N-7
  153. What are the VDRL false positives?
    • viruses (mono, hepatitis0
    • Drugs
    • Rheumatic fever
    • Lupus and Leprosy
  154. What CD markers are present on Reed-Sternberg cells?
    • CD15
    • CD30
  155. What condition is accompanied by secondary antiphospholipid syndrome?
    Systemic Lupus Erythromatouses
  156. Libman-Sacks endocarditis is a condition caused by what disorder?
    Systemic Lupus Erythromatosus
  157. Which syndrome is characterized by Xeropthalmia, Xerostomia and arthritis along with destruction of lacrimal and salivary glands?
    Sjogren syndrome
  158. What is the only lipid that is an antigen?
  159. What is a hapten?
    too small to start an immune response
  160. How does penicillin elicit an immune response/?
    penicillin is a hapten and binds to host proteins at which point is taken up by a B cell which is activated to produce antibodies by T helper 2 cells
  161. What is the function of an adjuvant?
    enhances immunogenicity of the antigen
  162. The TLRs located in the endosomes of the cells tend to detect what kind of foreign antigens?
    nucleic acids
  163. What is the best way of introducing antigens to induce immunity?
    subcutaneous because the antigen is take up by the Langerhan cells which migrates to the Lymph node and activatees T cells
  164. How are viruses attenuated?
    they are grown and selected for in non-human cells?
  165. Phosphatases are found where
  166. Kinases are found where?
  167. What is the function of Th17?
    activates neutrophils
  168. Defect in AIRE causes what?
    APECED--Autoimmune polyendcrinopathy candidiasis ectodermal dystrophy
  169. Defect in FAS causes what?
    • ALPS
    • Autoimmune lymphoproliferative syndrome
  170. What antigen is highly expressed in the anti-microsomal antigen in Hashimotos?
    Thyroid Peroxidase
  171. What is the mutated protein involved in the pathogenesis of Familial Mediterranean Fever?
  172. In all of the autoinflammatory conditions what protein is mutated?
    pyrin or cryopyrin
  173. Staphylocci cold absecesses and eczema with High IgE levels is indicative of what condition?
    Jobs, or Hyper IgE syndrome
  174. What is responsible for the bone resorption in RA?
    MMP --metaloproteinases secreted by fibroblasts activated by cytokines
  175. What is the only known environmental/behavioral trigger of RA?
  176. What condition produces sausage fingers?
    psoriatic arthritis
  177. How is RA treated?
    • Etanercept
    • Inflixamab
  178. Cant see, cant pee, cant climb describes what condition?
    • Reactive arthritis---Reiter's syndrome
    • acquired post GI or chlamydia infection
  179. Large vessels vasculitides have what pahological morphology?
    granulomatosis disease
  180. Medium vessel vasculitides have what patthological morphology?
    they are either immune complex fibrinoid inflammation or antibody mediated
  181. What does ANCA stand for?
    antineutrophil cytoplasmic antibody
  182. How does Large vessels vasculitis present typically?
    loss of pulse
  183. How does medium vessel vasculitis present typically?
  184. How does small vessel vasculitis present typically?
  185. Drug allergic reactions are what type of hypersensitivity?
  186. c-ANCA antibodies are produced against what protein?
  187. p-ANCA antibodies are produced against what protein?
  188. p- ANCA and c-ANCA antibodies bind to proteins found in what 3 types of cells?
    • neutrophils
    • macrophages
    • endothelial cells
  189. What vasculitis is not associated with ANCA?
    Polyarteritis nodosa
  190. Hemorrhagic mediastinitis is associated with what bacterial infection?
  191. Reynolds system is associated with what vasculitis?
  192. Which vasculitis has antigen antibody complexes of Hep B?
  193. What are 2 autoimmune disorders that affect the pulmonary and renal systems?
    • Good Pasture
    • Wegener's Granulomatosis
  194. What is the difference between Wegener's granulomatosis and Polyangitis?
    • Wegener's---c-ANCA
    • Polyangitis--p-ANCA
  195. What is the vasculitis that affects children?
    Henoch-Shonlein Purpura
  196. What malignancy is associated with cryglobuleniemai?
    multiple myeloma
  197. What cells amplify the response in SLE?
  198. The immune complexes deposit where in the kidney in a SLE pt?
    between endothelium and basement membrane
  199. What antibody is more specific for SLE?
    C1q and anti-dsDNA, Anti-SMith
  200. Cell mediated granulomas are responsible for crohn disease or ulcerative colitis?
  201. Hypersensitivity is responsible for crohn's disease or ulcerative colitis?
    ulcerative colitis
  202. Autoreactive T cells in the lamina propria cause what condition?
    Inflammatory bowel disease
  203. Which of the IBD involves the colon only
    Ulcerative colitis
  204. What is the gene mutated in Crohn's disease?
  205. Increased size of the crypts is characteristic of what condition?
    Celiac sprue disease
  206. Dermatatic Herpetiforms is associated with what disease?
    Celiac Disease
  207. Whats deposited in the dermis in Dermatatic Herpetiforms?
  208. BLS-Bare lymphocyte syndrome is due to deficiency of what MHC?
    MHC II
  209. Immunoelectrophoresis is used to demonstrate what imuunodeficiency?
    Bruton's agammaglobulinemia
  210. What do coreceptors on B cells bind?
    complement molecules
  211. BTK signalling deficiency prevents the the B cell from acquiring what?
    Pre-B cell receptor
  212. Wiskot-Aldrich Syndrome results in defective what?
  213. Autoimmune lymphoproliferative syndrome results from what?
    absent FAS killing
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