Immunology

blood

Blood is a highly specialized tissue produced in the bone marrow in a process called hematopoiesis. Blood contains red blood cells (erythrocytes) and white cells (leukocytes) circulating in plasma accompanied by platelets, plasma proteins, and other dissolved substances.

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hematopoiesis

Hematopoiesis is the production of blood cells, a developmental process located in the (red) bone marrow, though some cells mature elsewhere. For example, T lymphocytes are so named because they mature in the thymus, and antigenic stimulation of B lymphocytes to become plasma cells typically takes place in the periphery.

B lymphocyte development : common lymphoid progenitor : common myeloid progenitor : E2A : EBF : early B lineage : erythropoiesis : granulopoiesis : hematopoietic growth factors : lymphopoiesis : monocytopoiesis : Pax-5 : pluripotential stem cell : precursors : progenitors : regulatory transcription factors : stages : stem cells : thrombopoiesis : transcriptional regulatory proteins

The process of haematopoiesis occurs in several stages, and is controlled by at least 11 hematopoietic growth factors (including the colony-stimulating factors, IL-2 through IL-7, G-CSF, GM-CSF, and M-CSF). The first stage involves the differentiation of a pluripotential stem cell into a committed progenitor, which is followed by maturation of committed progenitors in distinct pathways, in which precursors are partially developed, 'adolescent' cells en route to maturity.

stem → progenitor → precursor → adult → mature

[] labeled photomicrograph of bone marrow, diagram of adult stem cell plasticity, diagram of stem cell versus progenitor cell

Stem cell stage:
pluripotential hematopoietic stem cell
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The common myeloid progenitor can generate:

● proerythroblasts (pronormoblasts) → erythropoiesis

● myeloblasts → granulopoiesis

● monoblasts → monocytopoiesis

● megakaryoblasts → thrombopoiesis

The common lympoid progenitor can generate:

● lymphoblasts → lymphopoiesis

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Committed progenitor stage to mature cell : granulopoiesis

common myeloid progenitor

myeloblast

B/E/N promyelocyte

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B/E/N myelocyte

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B/E/N metamyelocyte

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B/E/N band

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basophil, eosinophil, neutrophil

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mast cell -----------------------

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Committed progenitor stage to mature cell : lymphopoiesis

common lymphoid progenitor

lymphoblast

----------------------------------------↓ rearrangements H: D-J → H: V-DJ

prolymphocyte

-----------------↓ rearrangements L: V-J --------------------------

small lymphocyte------or----- natural killer cell (large granular lymphocyte)

------------↓ IgM→IgD ---------------------------------------------

------B lymphocyte--or-- T lymphocyte

-----------------------------------------------------------------------

--------plasma cell------------------------------------------lymphoid dendritic cell

Development of mature B lymphocytes from multipotent progenitors requires the coordinated activities of a number of transcriptional regulatory proteins, including EBF, Pax-5, and E2A.

During B cell-development from the precursor stage, differentiation involves rearrangement of the heavy chain gene segments. The functional integrity of the rearranged gene is tested: Precursor-B cells express two single domain Ig-like proteins of invariant sequence that substitute for the light chain. Formation of a complex comprising the μ (mu) heavy chain with the surrogate light chains instructs the cell to discontinue rearrangement of the heavy chain locus and to commence rearrangement of the k (kappa) locus. If successful light chain rearrangement is achieved such that the light and heavy chains form a complete antibody, then this complex instructs the cell to discontinue rearrangement of light chains, ensuring that only a single specificity is produced (allelic exclusion). Џ B cell maturation - animation Џ

Those developing B cell clones that fail to generate a productive rearrangement at both one of their heavy chain alleles and a light chain locus will undergo apoptosis. Immune tolerance mechanisms also exist to ensure the death of any newly produced B cells that express an antibody that reacts strongly with self proteins on the surface of host cells.

E2A proteins function in early B lineage development to regulate B lineage-specific gene expression as well as B cell survival. E2A-encoded proteins are involved in the differentiation of a number of cell types, and they are especially important in lymphocyte development.

The E2A gene encodes E47 and E12, which are basic-helix-loop-helix (bHLH) transcription factors that bind DNA either as homodimers or as heterodimers with other bHLH proteins. Such bHLH DNA binding activity in the B-lineage comprises E47 homodimers. Development of thymocytes mainly involves heterodimers of E47 and a related bHLH protein, HEB. Thymocytic E2A protein expression is required to initiate T-cell differentiation. During the development of thymoctyes, E-proteins and their antagonists, Id2 and Id3, regulate T-lineage specific gene expression and TCR rearrangement. E2A and Id proteins block thymocytic maturation in the absence of pre-TCR expression, and pre-TCR signaling acts to promote development in part by inhibiting E2A activity. [l]

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Committed progenitor stage to mature cell : monocytopoiesis

common myeloid progenitor

monoblast

promonocyte

monocyte

----

macrophage or myeloid dendritic cell


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Committed progenitor stage to mature cell : erythropoiesis

common myeloid progenitor

proerythroblast

basophilic erythroblast

polychromatic erythroblast

polychromatic erythrocyte (reticulocyte)

erythrocyte (RBC)

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Committed progenitor stage to mature cell : thrombopoiesis

megakaryoblast
promegakaryocyte
megakaryocyte
thrombocytes (platelets)

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immune cytokines

Cytokines are small proteins that regulate and mediate immunity, inflammation, and hematopoiesis. They are secreted de novo in response to immune stimuli, and usually act briefly, locally, at very low concentrations (the exception being endocrine action at distant cells). All cytokines possess four conserved cysteine residues, and are divided into two families based upon the contiguity (CC) or separation of cysteine residues (CXC).

The orchestration and regulation of cytokine production during inflammatory responses constitutes a key determinant of both the resolution of challenge and the limitation of host tissue damage. Cytokines bind to specific membrane receptors, which then signal the cell via second messengers, often tyrosine kinases, to alter cellular activity (gene expression). Cytokines are often produced in cascades.

Tables  Immune Cytokines  Fc receptors  Immunoglobulins  Interferons

actions : chemokine : chemokine family receptors : classification of cytokines : colony-stimulating factors (CSF) : cytokine cascade : cytokine effects : cytokine receptors : cytokine secreting cells : distance of cytokine action : granulocyte colony-stimulating factor (G-CSF) : granulocyte-macrophage colony-stimulating factor (GM-CSF) : hematopoietin family receptors : interleukins, interleukins as largest group : interferon family receptors (IFNR) : largest group of cytokines : lymphokine : lymphotoxins : macrophage colony-stimulating factor (M-CSF) : mechanism of cytokine action : monokine : receptors : search for novel cytokines : target cells : tumor necrosis family receptors
(TNFR) ▼

Actions of cytokines include:
a) up- or down-regulation of the expression of membrane proteins (including cytokine receptors),
b) secretion of effector molecules: histamine release; antibody secretion - IgA, IgG1, and IgE synthesis; IL-1 synthesis; cytokine production; MHC Class II; and CAM expression
c) cellular proliferation
d) chemotaxis of neutrophils, monocytes, and T cells
e) cellular differentiation
f) inflammation
g) phagocytosis
h) death of tumor cells
i) elimination of pathogens

Cytokines are classified according to the cells that produce them:
a) Lymphokines - produced by lymphocytes
b) Monokines - produced by monocytes,
c) Chemokines - chemotactic activities
d) Interleukins - manufactured by one leukocyte to act on other leukocytes.

The cells that produce cytokines include B cells, T cells, dendritic cells, NK, Tc, Th, Th1, Th2, endothelial cells, mast cells, plasma cells, progenitor, marrow stroma, thymus stroma, and tumor cells, along with fibroblasts, leukocytes, monocytes, and macrophages.

Cytokine function may be targetted at:
a) The cells that secrete them (autocrine action),
b) Local cells (paracrine action),
c) Distant cells (endocrine action).

Target cells include: B cells, T cells, NK, Tc, Th, Th2, stem, mast cells, plasma cells, progenitor, tumor cells, phagocytes, neutrophils, monocytes, and macrophages.

Macrophage migration inhibitory factor (MIF) is an inflammatory cytokine secreted by several cell types, including mononuclear cells of the immune system and pituitary cells in the brain. Functional promoter polymorphisms in the MIF gene affect the clinical presentation of systemic sclerosis (SSc, scleroderma) and confer susceptibility to systemic lupus erythematosis (SLE). MIF counteracts cortisol-induced inhibition of inflammatory cytokine secretion, and employs the CD74 receptor.

Cytokines bind to specific membrane receptors:
a) Hematopoietin family receptors - dimers or trimers with conserved cysteines in their extracellular domains and a conserved Trp-Ser-X-Trp-Ser sequence.
The two subunits are i) cytokine-specific, and ii) signal transducing.
Cytokine binding promotes dimerization of the alpha and beta subunits, which then associate with cytoplasmic tyrosine kinases to phosphorylate proteins and activate mRNA transcription. Examples - receptors for IL-2 through IL-7 and GM-CSF.

Colony-stimulating factors (CSFs) are glycoprotein molecules that support growth of hematopoietic colonies. Interleukin 3 (IL-3) exhibits broad activity in the proliferation and differentiation of erythroid, megakaryocytic, and myeloid lineage progenitor cells.

Macrophage colony-stimulating factor (M-CSF, CSF1) acts selectively on the macrophage lineage, while granulocyte colony-stimulating factor (G-CSF) acts selectively on cells of the granulocyte lineage.

Granulocyte colony-stimulating factor (G-CSF) and granulocyte-macrophage colony-stimulating factor (GM-CSF) have pleiotropic activating effects on mature leukocytes, which can improve leukocyte function, facilitating eradication of microbial infections. G-CSF activates neutrophils, while GM-CSF activates neutrophils, eosinophils, and monocyte/macrophages.

G-CSF is a 19.6 kDA glycoprotein that exists in two forms, comprising either 174 or 180 amino-acids. It is produced in a number of different tissues and stimulates granulopoietic proliferation and differentiation in the bone marrow. GM-CSF is a 14.6 kDa globular protein of 128 amino acids that contains two intramolecular disulfide bonds and two potential N-linked glycosylation sites. It is produced by endothelial cells, monocytes, fibroblasts and T-lymphocytes. GM-CSF is a hematopoietic growth factor that stimulates the development of macrophages and neutrophils, and inhibits neutrophil migration while enhancing the functional activity of the mature end-cells. It also promotes the proliferation and development of early erythroid megakaryocytic and eosinophilic progenitor cells.

b) Interferon (IFN) family receptors have the conserved cysteine residues but not the Trp-Ser-X-Trp-Ser sequence, and include the receptors for IFNα, IFNb, and IFNγ.

c) Tumor Necrosis Factor family receptors possess four extracellular domains, and include receptors for soluble TNFα and TNFβ as well as membrane-bound CD40 (important for B cell activation and macrophage activation) and Fas (which signals the cell to undergo apoptosis). TNFα is lymphotoxin β (LT), where the lymphotoxins occur either as homotrimers of LT-α (LT-α3) or as heterotrimers of one LT-α subunit plus two LT-β subunits (LT-α,β2).[s]

d) Chemokine family receptors have seven transmembrane helices and interact with G protein. This family includes receptors for IL-8, MIP-1 and RANTES. Chemokine receptors CCR5 and CXCR4 are used by HIV to preferentially enter either macrophages or T cells.

Cytokines are often produced in cascades, as one cytokine stimulates its target cells to produce additional cytokines. Cytokines are redundant in their activity, in that different cytokines can stimulated similar functions. Different cell types may secrete the same cytokine, or for a single cytokine may act on several different cell types (pleiotropy). Cytokines can also act synergistically with two or more cytokines acting together, or antagonistically with cytokines causing opposing activities.

Interleukins comprise the largest group of cytokines, stimulating proliferation and differentiation of immune cells. The group includes Interleukin 1 (IL-1), which activates T cells; IL-2, which stimulates proliferation of antigen-activated T and B cells; IL-4, IL-5, and IL-6, which stimulate proliferation and differentiation of B cells; Interferon gamma (IFNγ), which activates macrophages; and IL-3, IL-7 and Granulocyte Monocyte Colony-Stimulating Factor (GM-CSF), which stimulate hematopoiesis.

Short half lives, low plasma concentrations, pleiotropy, and redundancy combine to make the isolation and characterization of cytokines difficult. Search for novel cytokines is often conducted at the DNA level, identifying genes that are similar to known cytokine genes.

actions : chemokine : chemokine family receptors : classification of cytokines : colony-stimulating factors (CSF) : cytokine cascade : cytokine effects : cytokine receptors : cytokine secreting cells : distance of cytokine action : granulocyte colony-stimulating factor (G-CSF) : granulocyte-macrophage colony-stimulating factor (GM-CSF) : hematopoietin family receptors : interleukins, interleukins as largest group : interferon family receptors (IFNR) : largest group of cytokines : lymphokine : lymphotoxins : macrophage colony-stimulating factor (M-CSF) : mechanism of cytokine action : monokine : receptors : search for novel cytokines : target cells : tumor necrosis family receptors (TNFR) ▲

Tables  Immune Cytokines  Cell signaling  Receptor Tyrosine Kinases(RTK)  Second Messengers  Phosphate-handling Enzymes  Cell Adhesion Molecules (CAM) .

· adenylyl (adenylate) cyclase ф antibodies ф antigen ф B cells · cadherins · calcium ions · cAMP-dependent protein kinase · CDKs ф cellular response · chemotaxis ф class-switch recombination ф clonal selection ф complement system ф costimulation · cyclin-dependent kinases · cytokine receptors · DAG ф dendritic cells · diacylglycerol · DNA ligases · ERKs · GPCRs · GPCR families ф granulocytes · guanylate cyclases · guanyl cyclase ф helper T cell ф hematopoiesis ф humoral immunity · Ig superfamily ф immune response ф inflammatory response · inositol triphosphate · integrins · IP3 ф leukocytes ф leukocyte adhesion cascade ф lymphokines ф macrophages · MAP kinases ф macrophages ф MHC ф migration · mitogen activated protein kinases ф monocytes ф neutrophils ф pathogens ф pattern-recognition receptors ф phagocyte · phosphatases · phosphodiesterases ф plasma cells · phospolipases · phosphorylation · PKA · PKC · phospholipase C-gamma · protein kinase A · protein kinase C · protein tyrosine kinases (PTKs) ф receptors · receptor tyrosine kinases · second messengers · second messenger cAMP · second messenger cGMP · selectins ф signaling · signal transduction ф surface receptors ф T cells · TNFs · two-component systems ·

Tables  Fc receptors  Immune Cytokines  Immunoglobulins  Interferons  Cell Adhesion Molecules  Cell signaling  Receptor Tyrosine Kinases (RTKs)  Receptor Signal Transduction  Second Messengers 

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