Page 512 - Clinical Immunology_ Principles and Practice ( PDFDrive )

492 ParT fOur Immunological Deficiencies

Nucleus

FOXN1 CIITA RFX5
RFXAP
RFXANK
Cytoplasm

TAP TAPBP MHC

Antigen-presenting cell
Peptide
CD40
IL2 CD40L CD4
IL7 CD8
IL2Rβ
IL2Rα γc ξξ δεγ T cell
IL7Rα γc ORAI1

Ick LAT PLC1 PIP 2
JAK1 JAK3 JAK1 JAK3 CD45 ZAP-70 IP 3 Ca 2+
VAV SLP76 GRB2
CBL SOS Ca 2+
WASP
STAT DaG Calcineurin
P P
STAT Cytoskeleton Ras PKCθ
rearrangement
MAPK NF-κB NFAT
Cytoplasm

RAG1 ATM DCLREIC
RAG2 Nibrin MREIIA
LIG4 Cernunnos
Nucleus
fIG 35.2 T-Cell Signaling Cascade. Activation of T cells, such as following engagement of
peptide-major histocompatibility complex (MHC) with the T-cell receptor (TCR), involves signaling
through interleukin (IL) receptors, coreceptors, and calcium channels. Recruitment of additional
intracellular molecules trigger changes in gene expression, cytokine secretion, and receptor
expression. TAP, transporter associated with antigen presentation; TAPBP, TAP binding protein;
MHC, major histocompatibility complex; CIITA, class II MHC transactivator; IL7, interleukin (IL)-7;
IL7Rα, alpha chain of the IL7 receptor; CD40L, CD40 ligand; ZAP70, zeta chain–associated protein
kinase 70; JAK, Janus activated kinase; γc- gamma common chain of the IL-2 receptor; LCK,
lymphocyte-specific protein tyrosine kinase; STAT, signal transducer and activator of transcription;
CBL, Casitas B-lineage lymphoma; WASP, Wiskott-Aldrich syndrome protein; SLP76, SH2 domain
containing leukocyte protein of 76 kDa; LAT, linker for activation of T cells; PLC1, Phospholipase
C 1; GRB2, growth factor receptor–bound protein 2; DAG, diacylglycerol; RAS, Rat sarcoma;
MAPK, mitogen-activated protein kinase; PKC, protein kinase C; NF-κB, nuclear factor kappa-
light-chain-enhancer of activated B cells; NFAT, nuclear factor of activated T-cells; RAG, recombination
activating genes; LIG4, DNA ligase 4; ATM, ataxia telangiectasia mutated; DCLRE1C, DNA cross-link
repair 1C; MRE11A, MRE11 meiotic recombination 11 homologue A; RMRP, RNA component
of mitochondrial RNA-processing endoribonuclease.