Immune System

Kathryn Clarkson, Chapters 6 & 7

Moby Thomas, Chapters 8 & 9

Chapter 6

Human Defense Mechanisms ( table p.176)

Innate resistance or Immunity (natural or native immunity)

·  1st line of defense at birth

Inflammatory response

·  2nd line of defense

Adaptive immunity

·  3rd line of defense

·  acquired or specific immunity

·  slow & specific

·  memory

First line of defense: Physical, Mechanical, & Biochemical Barriers

·  physical barrier (cellular roadblocks)

o  sloughing of dead skin cells

o  coughing or sneezing

o  vomiting or urination

·  mechanical barrier

o  hairlike cilia

o  low surface temp inhibits microorganisms

·  Biochemical Barriers (synthesize & secrete to trap & destroy)

o  Mucus, perspiration, saliva, tears & earwax

o  Use of proteins to destroy pathogens

§  Epithlial-Derived Chemical

·  Antimicrobial peptides

o  Cathelicdins (one….LL-37)

o  Defensins (eight)

§  ά require activation by proteolytic enzymes

§  rich in granules of neutrophils for killing bacteria

§  β are synthesized in active form

·  Collectins (in lungs….surfactant proteins A & D)

o  Promote phagocytosis

o  Interact with acquired immune system

§  Bacteria-Derived Chemicals

·  Normal bacteria flora

o  Prolonged antibiotic treatment alter normal flora leading to overgrowth Candida albicans or Clostridium difficile

o  Lactobacillus produce hydrogen peroxide, lactic acid, bacteriocins, ect. to prevent infection of vagina & urinary tract

Second Line of Defense: The Inflammatory Response (p.179)

·  2nd line of defense

·  prevent infection

·  depends on activity of cellular & chemical components

·  nonspecific to stimulus

·  Vascular response (redness, heat, swelling, & pain)

o  Blood vessel dilation

o  ↑ vascular permeability & leakage of fluid out of vessel

o  WBC adherence to inner vessel wall & migration to injury

o  Arterioles constrict briefly near injury

o  Vasodilation slows blood velocity & ↑ local blood flow to injured site

o  ↑ flow & capillary permeability result in leakage of plasma, causing edema

o  ↑ blood flow & concentration of RBCs at site of inflammation causes warmth & redness

o  Leukocytes adhere to vessel wall

o  Biochemical mediators (histamine, bradykinin, leukotrienes, substance P, & prostaglandins) stimulate endothelial cells that line capillaries & venules to retract allowing leukocytes and plasma to enter surrounding tissue

o  Once in tissue, cells, & chemicals associated with inflammatory response :

§  Drainage by lymphatics facilitates Limit & control process

§  Prevent infection & further damage

§  Interact with components

§  Prepare area for healing

o  acquired immunity through antigens activating B & T lympocytes

o  Inflammation & repair phases

§  Destroy & remove injurious agents from site

§  Wall off & confine agents

§  Stimulate adaptive immune response

§  promote healing

·  Acute inflammatory response

o  Begins immediately

o  Takes 8-10 days

o  Involves vascular response, activation of plasma protein systems, & activation of variety of cells.

·  Plasma Protein Systems

Complement system

§  Complement componets (10% circulating serum protein)

§  Important because collaborate with every other component

§  Proteins most potent defenders against bacterial infection.

§  Activated when 1st protein is proteolyically cleaved into active fragments

§  Complement activation before converge at third component (C3) is accomplished by

·  Classical pathway— activated by antibodies bound to antigens. Begins with C1 & forms antibody complex (immune complex). C1 complex consist of C1q & 2 CLr and Cls. After binding, C1q changes resulting in Clr coming into proximity with C1s activating to become enzyme (C1esterase) whose substrates are C4 & C2. The result uses C3 as substrate resulting in production of C3a & C3b.

o  C3 convertase—complex with C3

o  C13 has C5 as substrate, resulting in conversion of C5 to C5a & C6b.

o  C5 convertase—complex with C5

o  Can activate heparin, DNA or RNA, C-reactive protein (↑ in blood during inflammation)

o  C3b inactivated by protein factor H & I

·  Lectin pathway—activated by bacterial carbohydrates

o  Antibody-independent

o  Activated by plasma protein Mannose-binding lectin (MBL)

§  Activate C4 & C2 to create C3 convertase

·  Alternative pathway—activated by gram-negative bacterial & fungal cell wall polysaccharides

o  Initiated by lipopolysaccarides (endotoxin) on bacterial surface, yeast cell wall carbohydrates (zymosan) that bind C3b & protect it from factor H and destruction. C3b can then react factor B. Then recognized by enzyme, factor D, which activates factor B, producing factor Bb. Now have unstable C3b/Bb until bind with properdin (P). C3b/Bb/P produces C3b, resulting in C3b/Bb/P/C3b that is C5 convertase converging alternative pathway with classic pathway

§  Cascade continue to form membrane attack complex (MAC), creating pores in bacteria membrane, influx of water & ions, resulting in cell lysis and destruction of pathogen.

§  Most important Opsonin tag pathogenic organism for destruction

§  C4a are anaphylatoxins that induce rapid degranulation & histamine release from mast cells causing vasodilation & ↑capillary permeability

§  C5a is major chemotactic factor for neutrophils

§  Chemotactic factor attracts leukocytes to site of inflammation

§  Anaphylatoxic activity necessary in early inflammation close to site to induce mast cell degranulation & ↑ # of soluble mediators to enhance permeability & vasodilation.

§  Chemotactic activity require longer period & distal to inflammatory site to attract leukocytes from circulation

§  Caroxypeptidase—plasma enzyme removes terminal arginine on both peptides producing C3a desArg & C5a desArg which preserve healthy tissue

§  C3b can break down diminishing biologic activity.

§  Complement cascades can be activated by 3 different means and its products have 4 functions.

·  Opsonization

·  Anasphylatoxic activity resulting in mast cell degranlation

·  Leukocyte chemotaxis

·  Cell lysis

o  Clotting system—form fibrinous meshwork at injured site

§  Prevents spread of infection

§  Keeps microorganism & foreign bodies at site of greatest inflammatory activity

§  Forms clot

§  Provides framework for future repair & healing

§  Main substance is Fibrin, end product of coagulation cascade

§  Classic & alternative converge after activating C5. Extrinsic pathway & intrinsic pathway converge at factor x proceeding until fibrin is formed.

§  Activated also by collagen, proteinases, kallikrein, plasmin, & endotoxins (bacterial products)

§  Fibrinopeptides A & B cproduce fibrin & ↑ permeability by enhancing effects of bradykinin (formed in Kinin system)

o  Kinin system

§  Primary role to activate & assist inflammatory cells.

·  Primary kinin is Bradykinin, causes vasodilation, acts with prostaglandins to induce pain, smooth muscle contraction, ↑ vascular permeability & may ↑ leukocyte chemotaxis

o  May prolong phases ofinflammation

o  Probable cause endothelial cell retraction & ↑ permeability in later phase of inflammation

§  Activated by stimulation of plasma kinin cascade

·  Prekallikrein activator

·  Factor XIIa

o  Hageman factor

·  Kallikrein converts kiniogen to bradykinin

·  Lys-bradykinin—convert bradykinin

·  Kinins are controlled by kininases

o  Systems consist of inactive enzymes or proenzymes required for activation (complement cascade event)

o  Interactions Among the Plasma Protein Systems (p.184)

§  Plasmin degrades fibrin and fibrinogen & activates complement cascade through C1, C3, & C5, & Hageman factor (factor XII) and produce prekallikrein activator. This impacts all 3 plasma protein systems

·  Activates clotting cascade through factor XI

·  Control clotting

·  Activation of kinin system

·  Activation of C1

§  Plasma regulated by plasminogen

§  Tight control of inflammatory response are for:

§  Protection against infection

§  Potent biochemical mediators for strict control to injured site

§  C1 esterase inhibitor (C1inh) binds to C1r & C1s to inhibit further activation of classic complement cascade.

·  Cellular Mediators of Inflammation

o  Granulocytic or monocytic lines of leukocytes in blood & tissue

o  WBCs primary granulocytes (neutrophil, eosinophils, & basophils)

o  Platelets, agranular monocytes, & various lymphocytes

o  Mast cells

o  Lymphoid derived natural killer cells (NK cells)—recognize & destroy cells altered by viral infection or malignancy.

o  All remove debris for healing

o  Inflammation hard to control with drugs

o  Cellular Receptors (table p. 185 &186)

§  T-cell receptor or TCR, B-cell receptor or BCR

·  Develop during somatic rearrangement of DNA

·  Pattern recognition receptors (PRRs)

o  (Skin, respiratory tract, gastrointestinal tract, genitourinary tract)

o  monitor flaws in external barriers that result in cellular damage & infection

o  On surface, but some are secreted

·  Toll-like receptors (TLRs)

o  10 different receptors

o  expressed as homdimers or heterodimers

o  have early contact with pathogenic microorganisms

o  recognize PAMPs

o  recognize host factors produced by stressed or damage cells

o  interactions with PAMPs result in activation of cell & release of soluble products (cytokines) that ↑ resistance to pathogenic micro organism

o  bridges between innate resistance & acquired immune response through induction of cytokines that ↑ response of lymphocytes to foreign antigens on pathogens

§  Recognize molecular pattern on infectious agents or their products (pathogen-associatede molecular patterns (PAMPs)

§  Release soluble products (cytokines)

·  Complement receptors

o  Recognize fragments produced through activation of complement system

o  Include C3a, C4a, C5a and C1q

·  Scavenger receptors

o  Facilitate recognition and phagocytosis of bacterial pathogens, damaged cells, & altered soluble lipoproteins associated with vascular damage (HDl, acetylated LDL, oxidized LDL)

o  On macrophages to identify &remove old RBCs & cells undergoing apoptosis

o  CD14 recogniezs complex of LPS & LPS-binding protein (regulated during inflammation by cytokines IL-6 & IL-1 & helps remove endotoxin

o  Mast Cells

§  Initiate inflammatory response

§  Responsible for effects oninflammation by

§  Mast Cell Degranulation

·  Histamine cuases temporary, rapid constriction of large vessel walls & dilation of postcapillary venules resulting in ↑ blood flow to microcirculation

o  Cause ↑ vascular permeability

o  H1 (promotes inflammation) & H2 receptors ( bound with H1 result in anti-inflammation, suppression of leukocyte function)

o  H1 (bronchoconstriction) (augmentation of neitrophil chemotaxis)

o  H2 (inhibition)

§  Chemotactic factors, neutrophil chemotactic factor, & ECF-A are released

§  Chemotaxis—directional movement of cells along chemical gradient formed by chemotactic factor

·  Neutrophil chemotactic factor attract neutrophils

·  ECF-A attract eosinophils

§  Mast Cell Synthesis of Mediators

·  Leukotriens

o  Acidic, sulfur-containing lipids effect smooth muscle contraction, ↑ vascular permeability

o  Important in later stages of inflammatory response---stimulate slower & more prolonged responses

·  Prostaglandins

o  ↑ vascular permeability & neutrophil chemotaxis

o  induce pain

o  produced by action of the enzyme cyclooxygenase & classified in groups (E, D, A, F, & B)

o  smooth muscle contraction

o  suppress release of histamine & lysosomal enzymes (responsible for killing & digesting organisms)

o  Enhancement or suppression of inflammatory response may be related to concentration of prostaglandins.

o  Aspirin & NSAIDs block synthesis of prostaglandins of E series & inhibit inflammation

·  Platelet-activating factor (PAF)

o  Endothelial cell retraction to ↑ vascular permeability

o  Leukocyte adhesion to endothelial cells & platelet activation

o  Initiate allergic responses

o  Phagocytosis

§  Ingest & disposes foreign material & organisms

§  Biochemical products (histamine, TNF-ά, bradykinin, prostaglandins) produced early at inflammatory sites diffuse to the vessels & affect leukocytes & endothelial cells. Both respond by producing new adhesion molecules on their surfaces.

§  Initial change ↑ adhesion causing leukocytes to adhere to walls of capillaries & venules, process called margination or pavementing.

§  Adhesion moles expressed later lead to diapedsis

§  Endothelial cells release nitric oxide (NO)

·  Causing vasodilation

·  Suppress mast cell function

·  ↓ platelet adhesion & aggregation

o  Leukocytes attracted to inflammation site by chemotaxis

o  Phagocytosis involves 4steps:

§  Opsonization (recognition of target & adherence) (glue)

§  Engulfment (ingestion or endocytosis

§  Formation of phgosome

§  Fusion with lysosomal granules within phagocyte

§  Destruction of target

§  Engulfment (endocytosis) by pseudopods form intracellular phagocytic vacuole or phagosome

§  Phagosome fuse with lysosomes & discharge contents creating phagolysosome where destruction of bacteria takes place by oxygen dependent & oxygen-independent mechanisms

§  Phagocytosis is accompanied by burst of oxygen by phagocyte termed respiratory burst resulting from shift of cell’s glucose metabolism to hexose-monophosphate shunt

§  Destructive effects of enzymes released by dying phagocytes are minimized by natural inhibitors found in blood such as ά¹-antitrypsin, plasma protein produced by liver.

§  Inherited deficiency of ά¹-antititrypin results in chronic lung damage & emphysema

o  Neutrophils

§  Polymorphonuclear neutrophil (PMN)

§  Predominant phagocytes in early inflammation, 6 to 12 hours after injury

§  Macrophages & lymphocytes replace neutrophils 24 hours later

§  Mature cell incapable of dividion & sensitive to acidic environment

§  Becomes component of purulent exudates or pus & removed via lymphatic system

§  Primary role removal of debris in sterile lesions & phagocytosis of bacteria in nonsterile lesions

o  Monocytes & Macrophages

§  Largest normal blood cells, produced in bone marrow, develop into macrophages at inflammation site.

§  Kupffer cells in liver, alveolar macrophages in lungs, & microglia in brain.

§  May appear 24 hours at inflammatory site, but usually arrive 3 To 7 days

§  Chemotactic factors released by neutrophils attract them

§  Long term defense, survive & divide in acidic inflammation

§  Involved in activation of adaptive immune system

§  Macrophage Activation

·  Several bacteria are resistant to killing by granulocytes & can survive inside macrophages

·  Cyokines ↑ macrophage activity

o  Eosinophils

§  Primary defense against parasities

§  Regulate vascular mediators released from mast cells

o  Natural Killer Cells

§  Recognition & elimination of cells infected with viruses

o  Platelets

§  stop bleeding

§  degranulation

·  Cellular Products