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MCMP 422 • Tony Hazbun • RHPH 406D • [email protected] – Include MCMP 422 in the subject line MCMP422 Immunology • Immunology is important personally and professionally! • Learn the language - use the glossary and index • RNR - Reading, Note taking, Reviewing • All materials in Chapters 1-5 are examinable (with exceptions) plus extra material from class What and why? • Immunology: Science of how the body responds to foreign agents • Immune system: the organs, cells and molecules that defend and respond to pathogens/allergens • Organ transplantation, cancer, immunodeficiency diseases, infectious diseases Immunology • How do we recognize foreign structures? • How do we recognize self vs non-self? • How do we stop and remove invading agents? Pharmacy and Therapy Perspective • How can we use the immune system as a therapeutic agent? • How do drugs affect the immune system? Chapter 1 Concepts What components make up the immune system? Cells, organs, cytokines and other molecules involved in the immune system What is the goal of the immune system? To clear pathogens in our body How do we classify immune responses? Innate and adaptive immune responses What are the side effects of the immune system? Autoimmune diseases, Allergies, Transplantation Rejection Machinery of the Immune system 1. Tissues/organs bone marrow, thymus, spleen, lymph nodes 2. Cells lymphocytes, dendritic cells, macrophages, natural killer cells, granulocytes (neutrophils, basophils, eosinophils), mast cells 3. Blood-borne proteins complement and mannose-binding proteins Origin of Immunology - individuals who survived a disease seemed to be untouched upon re-exposure Vaccination/Immunization - procedure where disease is prevented by deliberate exposure to infectious agent that cannot cause disease. Vaccinia - mild disease caused by cowpox Edward Jenner - first demonstration of vaccination Pathogen - any organism that can cause disease Figure 1-2 Diversity of Pathogens • Four Classes -Bacteria -Fungi -Viruses -Parasites • Opportunistic pathogens e.g. Pneumocystis carinii • Pathogen-Host relationship How Clean are You? Part of body Head (scalp) Surface of skin Saliva Nose mucus Faeces Bacteria 1,000,000 /cm2 1000 /cm2 100,000,000 /g 10,000,000 /g over 100,000,000 /g Defenses against Pathogens Physical Defenses 1. Skin - Tough water-proof Barrier - Pathogen Penetration is difficult - Breached by wounds/mosquito 2. Mucosal surfaces - line body cavities - epithelial cells covered with mucus - mucus thick fluid layer containing glycoproteins, proteoglycans and enzymes - e.g. mucus in lungs traps pathogens Immune Defenses 1. Innate - physical defenses are part of innate immunity 2. Adaptive Physical Barriers • Lungs: Mucus, cilia trap and move pathogens • Nose: Mucus traps pathogens which are then swallowed or blown out • Mouth: Friendly bacteria, Saliva • Eyes: lysozyme • Stomach: acid neutralization • Intestine: Friendly bacteria • Urogenital tract: Slightly acid conditions Immunity: Three Basic Parts Recognition (Binding event) Pathogen (Foreign) Immune disorders (Self) Signal Effector mechanisms Effector Cells Complement Two types of Immunity - Innate or Adaptive Innate Immunity • Ancient system - present in invertebrates • naïve, immediate, everyday immunity • Molecules recognize common features of pathogens – Lectin – Phagocytes, large lymphocytes (NK cells) – Complement Adaptive Immunity • Newer system - present in fish, birds, human • specialized, late, immunity • Molecules recognize specific features of pathogens – Antibodies – B and T cells - small lymphocytes – Immunological memory Example of Innate Immunity Figure 1-5 part 1 of 2 Complement - blood borne (serum) proteins that tag pathogens or attack them directly Effector cell - engulf bacteria, kill virus infected cells, attack pathogens Endocytosis - process by which extracellular material is taken up One type of effector cell is the phagocyte Innate Immunity Figure 1-6 Cytokines = signaling molecules --> inflammation/adaptive immunity Phagocytosis = “phagos” means to eat Inflammation is sometimes an unwanted by-product! Inflammatory cells = WBC’s contributing to inflammation Inflammation • • 1. Inflammation - local accumulation of fluid and cells involved in the immune response What happens when inflammation is induced Blood capillary dilation => heat (calor) & redness (rubor) - Local dilation of blood capillaries = increase of blood to the area (DOES NOT increase blood flow) 1. Vascular dilation (vasodilation) => swelling (tumor) & pain (dolor) 2. Extravasation - movement of cells/fluid into connective tissue. A) change in adhesiveness of the endothelial tissue allowing immune cells to attach and migrate into the connective tissue B) vascular dilation - gaps in endothelial cells Example of inflammation gone bad: Sepsis Systemic inflammatory response syndrome (SIRS) Results from the body's systemic over-response to infection Treatment: broad-spectrum antibiotics and supportive therapy Disturbance of innate immunity during sepsis and multiorgan dysfunction syndrome (MODS) probably linked to uncontrolled activation of the complement system Future Drug therapies could be used that modulate proinflammatory and anti-inflammatory factors Innate and Adaptive responses Innate Adaptive Pathogen independent Immediate (hours) Pathogen-dependent Slower (days) Neutrophils Macrophages Mast cells Eosinophils Basophils NK cells “Large Lymphocytes” = NK cells Dendritic cells B cells T cells (CD4 or CD8) “Small Lymphocytes” = B & T cells Both systems “talk” to each other to modulate response Both systems use leukocytes = white blood cells What if Innate Immunity is not Enough? • Innate immunity keeps us healthy most of the time • Some pathogens escape the innate immune process • Need a specific system to adapt to a specific pathogen - Hence vertebrates evolved the Adaptive immune response Principles of Adaptive Immunity 1. Lymphocytes each with different specificity generated by gene rearrangements 2. Small fraction of total pool of lymphocytes can recognize the pathogen 3. Pathogen recognizing lymphocyte is amplified - Clonal amplification 4. Pathogen recognizing lymphocyte can persist providing long-term immunological memory 5. Primary vs Secondary immune response eg. Influenza/Measles/Vaccination Characteristics of Innate vs Adaptive Immunity INNATE Figure 1-7 = genes are constant ADAPTIVE = genes are rearranged Leukocytes - white blood cells that increase the immune response to ongoing infection Innate vs Adaptive Molecular Recognition • Most important difference: Receptors used to recognize pathogens • Innate immunity: Receptors recognize conserved structures present in many pathogens (usually a repetitive pattern) Pathogen-associated Molecular Patterns (PAMPs): LPS, peptidoglycan, lipids, mannose, bacterial DNA and viral RNA e.g. Mannose-binding Lectin (MBL) • Adaptive immunity: Receptors recognize a specific structure unique to that pathogen e.g. Antibodies Flowchart of Hematopoiesis Pluripotent stem2 cell Figure 1-11 part 1 of Self-renewal Flowchart of Hematopoiesis Figure 1-11 Leukocytes Myeloid Lineage Granulocytes (Myeloid progenitor) Polymorphonuclear leukocytes (PMLs) Figure 1-9 Neutrophils: partMost 3 abundant of 6 Phagocyte Effector cells of Innate Immunity Short-lived - Pus Eosinophils: Worms/intestinal parasites Amplify inflammation Bind IgE Very Toxic - Pathogen and host Chronic asthma Basophils: Rare Unknown function Bind to IgE • Circulate in blood • Bigger than PMLs • Look similar • Immature form of macrophage Figure 1-9 part 5 of 6 • Scavengers • Phagocytose pathogens, cells, debris • Secrete cytokines Figure 1-13 Macrophages respond by two mechanisms - use 2 different receptors. 1) Phagocytosis - Phagosome fuses with lysosome - toxic small molecules and hydrolytic enzymes kill/degrade the bacteria 2) Signaling - bacterial component binds receptor - initiates transcription inflammatory cytokines synthesized and secreted • Star-shape • In tissue • Cellular messenger • Cargo cell • Connective tissue • Unknown progenitor • Granules • Degranulation major contributor to inflammation and allergies Lymphoid Lineage Cells Large lymphocytes NK cells Small lymphocytes B cells T cells Innate immunity Adaptive immunity Figure 1-9 part 2 of 6 Lymp • Large lymphocyte with granular cytoplasm • Effector cell of innate immunity 1) kill viral infected cells 2) secrete cytokines that interfere with virus infections • Adaptive IR • Small and immature • Activated by pathogen • Two types - B cell - T cell • B cells have B cell receptors and secrete Ab • T cells have T cell receptors Erythroid Lineage • Giant nucleus • Resident of bone marrow • Fusion of precursor cells • Fragments to make platelets Figure 1-9 part 6 of 6 • Gas transport • Infected by Plasmodium falciparum Lymphoid Myeloid Erythroid Neulasta (Amgen): Granulocyte Colony-Stimulating Factor (G-CSF) Recovery from Neutropenia & protect against Bacterial disease Leukine (Schering-Plough): Granulocyte-Macrophage ColonyStimulating Factor (GM-CSF), Recovery from Neutropenia and protection against Bacterial/fungal/parasitic disease Centrifuged blood sample Plasma White blood cells Red blood cells Figure 1-12 High WBC could be a sign of infection or leukemia Low WBC bone marrow diseases or HIV Polys = polymorphonucleocytes - mainly neutrophils High lymphocyte count indicates the bacterial or viral infection The lymph system and sites of lymphoid tissue Figure 1-15 Primary (Red) and Secondary (yellow) GALT, BALT, MALT Thoracic Duct Lymphpatic vessels - fluid collection Lymph nodes - junctions of vessels Recirculation Draining Lymph node Edema - is worse when patient is inactive Afferent (entry) Efferent (exit) Figure 1-17 part 1 of 2 Communications Center Afferent vessels bring in the lymph from infected tissue Efferent vessels place of exit for non-activated lymphocytes B-cell area (follicle) Lymphocytes Afferent lymph Pathogen Dendritic cells T helper cell (lymph node) Activate B cells Make Antibodies artery T-cell area Efferent lymph Lymphocyte not activated Activated by dendritic cell T helper cell (Infection site) Cytotoxic T cell (Infection site) Activate Macrophages Kills infected host cells Lymphocytes enter node through artery Tcells migrate to the T-cell area and if they meet a dendritic cell that is carrying pathogens from an infection site they get activated - to divide into functional effector cells. Some T-cells stay in the lymph node and become Thelper cells - secrete cytokines (soluble proteins) and have receptors that contact B-cells. This helps the Bcells differentiate into plasma cells. Plasma cells stay in the lymph or leave and pump out large amount of antibodies - a soluble form of their cell surface receptor A second type of activated T-cell is the T-helper cell that leaves the node to the infected area and interacts with macrophages and amplify inflammation Third type of T-cell is the cytotoxic T-Cell which kill cells infected with pathogen Remember 5 million lymphocytes are entering node every minute and only a few are activated in response to an infection. Anatomy of immune function in the Spleen Figure 1-19 • Blood filtering organ - remove old/damaged red cells (red pulp) • Blood-borne pathogens e.g. malaria • White pulp (Immune system) - similar to lymph node (except pathogens enter and leave by blood) Figure 1-20 M cells Activated lymphocytes M cells - specialized cells lining mucosal epithelium that deliver pathogen => activate lymphocytes Adaptive Immunity 1. Vertebrates only 2. Specificity - recognition modules - BCR, Ab and TCR - gene rearrangement is the source of diversity - clonal selection 3. Small lymphocytes - types and sub-types - functions Recognition concept Receptor or Antibody molecule Antigen - structure recognized by an Ab, BCR or TCR Epitope - particular sub-structure of the Ag that is bound Affinity - how much a molecule likes to bind to a structure Small lymphocyte sub-types B-cells BCR is Immunoglobulin (Ig) Plasma cells - effector cells that secrete Ab T-cells Tc = cytotoxic (CD8+) TH = helper T-cells (CD4+) Th1 (inflammation) Th2 (help B-cells make AB) Recognition modules of Adaptive immunity B-cell receptor (BCR) B cells Antibody is a secreted form of BCR T cell receptor (TCR) T cells TCR is membrane bound Native vs Denatured B-cells T-cell Antigen processing Major Histocomp -atibility (MHC) MHC APC - Antigen Presenting Cells Professional APC - macrophages - B cells -Dendritic cells MHC I - all nucleated cells - intracelluar pathogens e.g. virus Interact with cytotoxic T cells Interact with helper T cells MHC II - immune cells APC - extracellular MHC class I communicates with cytotoxic T cells (Tc cells) Cellular ribosomes are subverted into making more virus proteins Some of those proteins are degraded in the cytoplasm and transported to ER MHC1 bind to these peptides and help to display them on the cell surface Cytotoxic T cells = Tc cells, Cytotoxic T-lymphocytes (CTLs) MHC class II communicates with TH cells (TH1 or TH2) Also: Dendritic cells interact with naïve T-cells to initiate differentiation Antibodies Produced by B-cells Humoral Immunity - Humor = “body fluids” Passive immunity - serum transferred to another individual can confer passive reistance due to transfer antibodies Antibodies Parasitic infection Parasite + Mast cell Inflammation Mast cell activated Expel and/or destroy pathogen • • • Neutralization Opsonization Inflammation Principles of Adaptive Immunity Diversity Specificity Memory Self-tolerance Gene Rearrangement is the source of Diversity Germline configuration - the exact form of genes you inherit Somatic cells - all the cells of the body except germ cells Diversity 1. Alternative combinations 2. Imprecise joints 3. Different types of chains 4. B-cells - somatic hypermutation All this can happen in the absence of antigen Clonal Selection 1. Each cell = one receptor 2. Millions of lymphocytes are generated 3. Small subset will recognize a pathogen 4. Proliferation and differentiation 5. Acquired immunity - the adaptive immunity provided by immunological memory Antibodies are usually very specific Figure 1-22 Concept Behind Vaccination Many Few specific lymphocytes lympho cytes Some memory lymphocytes •Pre-industrialization infants built immunity naturally •Post-industrialization polio rate increased in adults hence a need for vaccination Polio Vaccine - Inactive vs Oral “live” version VDPV - vaccine derived polio virus, cheap and easy to administer - mutations can lead to polio at extremely low rate - immunocompromized individuals can be carriers of VDPV Principle of Self-tolerance B-cells with BCR that bind to self will undergo Apoptosis More complicated scheme of selection for T cells Selection of T cells 1. Thymocytes immature T-cells 2. Positive selection -Self MHC -cortex (epithelial cells) 3. Negative selection Mechanism of Self-tolerance Immunodeficiencies Inherited deficiencies e.g. Bubble boy disease Stress induced nutrition, emotional Pathogen caused deficiencies HIV - attacks CD4 T lymphocyte Cells and molecules involved in Hypersensitivity Diseases Figure 1-32 • IgE • IgG • CD4 TH1 • CD8 CTL Insulin-Dependent Diabetes Mellitus • Beta cells of the islets of Langerhans in the pancreas are attacked • Symptoms don’t show up for a long time • Infection by a specific virus has been correlated with higher rate of IDDM • Some of the activated CTL and Th1 cells will attack the healthy beta cells • IDDM also has been correlated with certain polymorphisms (types) of the MHC molecule Inflammatory Adaptive Immune Response Hygiene Hypothesis or Global Warming Hypothesis