IMMUNOPATHOLOGIE

 

Déficiences Immunitaires Héréditaires,

 

Cytokines pro-inflammatoires et leur recepteurs

 

FUNCTIONS

IL-1a

 

 

macrophages, NK cells,

dendritic cells, B cells,

T cells

kD protein; 159

amino acid residues.

fever and acute phase pro-tein

synthesis; . thymocyte

and T cell activation and B cell

growth, differentiation, and

immunoglobulin secretion.

Binds and acts through the IL-1R

Type I.

IL-1b

mono-cytes,

macrophages, NK

cells, dendritic cells,

B cells, T cells

 

kD protein; 153

amino acid residues.

 

fever and acute phase pro-tein

synthesis; . thymocyte

and T cell activation and B cell

growth, differentiation, and

immunoglobulin secretion.

Binds and acts through the IL-1R

Type I.

IL-6

 

cell types includ-ing

monocytes,

macrophages, B cells,

T cells, granulocytes,

mast cells, chondro-cytes,

osteoblasts, vas-cular

endothelial cells,

fibroblasts, ker-atinocytes,

astrocytes

kD glycoprotein,

185 amino acid

residues.

 

acute phase protein synth.;

. thymocyte and T cell activa-tion;

. B cell growth, differen-tiation

and Ig production. Acts

through the IL-6Ra/gp130 het-erodimer.

IFN-g

 

cell and NK cells

kD glycoprotein

subunit, 146 amino

acid residues, homod-imer

. Macrophage and

NK cell function; . MHC class I

and II cell surface antigen

expression. Binds and acts

through the heterodimeric

IFN-gRa,b.

TNF-a

 

cell types includ-ing

monocytes,

macrophages, neu-trophils,

NK cells, B

cells, T cells, astrocytes,

microglial cells, fibrob-lasts.

kD glycoprotein

subunit, 171 amino

acid residues.

Expressed as cell sur-face

homotrimer; shed

in soluble form by

enzymatic cleavage.

fever and septic shock; cyto-toxic

for many tumor cell

types; activates macrophages

and neutrophils; . MHC class I

and II cell surface antigen

expression, acts through TNFRI

and TNFRII.

LT- a3

cells, B cells, fibrob-lasts,

astrocytes,

endothelial cells.

 

kD subunits,

homotrimer

 

form of LT-a acts

through TNFRI and TNFII, cyto-toxic

for many tumor cell

types, inhibits angiogenesis,

activates macrophages and

neutrophils.

IL-1R Type I

CD121a

 

level expres-sion

on most cells.

 

kD Type I transmem-brane

glycoprotein,

569 amino acid

residues. A soluble

form of the IL-1RI

extracellular domain

exists.

 

affinity receptor for bioac-tive

IL-1a, IL-1b and non-bioac-tive

IL-1 receptor antagonist.

Complexes with IL-1 Receptor

Accessory Protein to form a

high affinity receptor complex

that mediates the cellular

response to IL-1.

IL-1R Type II

CD121b

 

some

T-cells, myeloid

cells, some epithe-lial

tissues

 

kD Type I trans-membrane

glycopro-tein,

398 amino acid

residues. A soluble

form of the IL-1RII

extracellular domain

exists.

 

IL-1a, IL-1b & IL-1 recep-tor

antagonist. No known sig-nalling

function, IL-1 decoy

receptor.

IL-6Ra

CD126

 

T cells,

and activated B

cells. Low on rest-ing

B cells. Little

or no expression

on NK cells,

granulocytes,

and erythrocytes.

Hepatocytes.

kD Type I transmem-brane

glycoprotein,

449 amino acid

residues. A soluble

form of the IL-6Ra

extracellular domain

exists.

 

receptor for IL-6.

Complex formed between IL-6

and IL-6Ra associates with

gp130 (CD130) to form a sig-naling

receptor for IL-6.

GP130

CD130

 

cells, B cells, NK

cells, monocytes,

granulocytes

 

kD Type I trans-membrane

glycopro-tein,

896 amino acid

residues

 

with the complex of

IL-6 and IL-6Ra (CD126) to

form a signaling receptor for

IL-6. The gp130 subunit does

not bind IL-6 by itself.

Common signaling subunit of

receptors for IL-6, IL-11,

Oncostatin M, LIF, CNTF, CT-1

receptors.

IFN-gRa

CD119

 

expres-sion

on B cells, T

cells, NK cells,

monocytes, granu-locytes,

platelets,

epithelial cells,

endothelium,

many tumor cells.

Not expressed by

erythrocytes.

kD Type I trans-membrane

glycopro-tein,

489 amino acid

residues. A soluble

form of the IFN-gRa

extracellular domain

exists.

 

for ligand binding

and trafficking through the

cell; it is necessary but not

sufficient for signaling.

IFN-gRb

 

expression:

B cells, T cells, NK

cells, monocytes,

granulocytes,

platelets, epithelial

cells, endothelium,

many tumor cells.

Not expressed by

erythrocytes.

kD Type I transmem-brane

glycoprotein,

489 amino acid

residues.

 

of the complex

formed between the ligand

and the IFN-g receptor a sub-unit;

required for signal trans-duction

upon ligand ligation,

TNFR Type I

CD120a

 

 

by most

nucleated cell

types. Not

expressed by ery-throcytes.

kD Type I transmem-brane

glycoprotein,

435 amino acid

residues. A soluble

form of the human

TNFRI extracellular

domain exists

for both TNF-a and

LT-a3 (TNF-b). An 80 AA

residue ‘death domain’ trig-gers

apototic pathway. Soluble

TNFRI blocks TNF-a and LT-a 3

activities.

TNFR Type II

CD120b

 

by most

nucleated cell

types. Upregulated

expression by acti-vated

T and B lym-phocytes.

Not

expressed by ery-throcytes

kD Type I transmem-brane

glycoprotein,

435 amino acid

residues. A soluble

form if the human

TNFRII extracellular

domain exists.

 

for both TNF-a and

LT-a3 (TNF-b).