Major Histocompatibility Complex

  • Large genomic family of membrane-bound glycoproteins that play important role in immune system and autoimmunity; main role to present protein antigen to T cells
  •    
     
    Remember: (MHC) 1 × 8 (CD8 T cell) = (MHC) 2 × 4 (CD4 T cell)
     
       
    Three classes: MHC I (present on all nucleated cells), MHC II (present only on antigen-presenting cells or APCs), and MHC III
  • MHC I:
    • Process/bind intracellular antigens
    • Antigens are taken up and degraded in cytosol of cell
    • MHC I recognized by CD8 T cells
    • Main types of MHC I genes: HLA-A, HLA-B, and HLA-C
    • MHC II
    • Process/bind extracellular proteins via degradation in endocytic vesicles (of APCs)
    • MHC II recognized by CD4 T cells
    • MHC II genes: HLA-DP, DLA-DQ, and HLA-DR
    • Know following diseases with HLA associations (Table 1-17)
   
 
Table 1-17 MHC I-Associated Diseases
 HLAAssociated Diseases
 
HLA-B8
Oral lichen planus, dermatitis herpetiformis
 
HLA-B13
Guttate psoriasis, erythrodermic psoriasis
 
HLA-B17
Guttate psoriasis, erythrodermic psoriasis
 
HLA-B27
Psoriasis vulgaris, psoriatic arthritis, ankylosing spondylitis, reactive arthritis (Reiter disease), acrodermatitis continua of Hallopeau, lichen planus
 
HLA-B51
Behcet’s disease, lichen planus
 
HLA-B57 (Bw57)
Oral lichen planus (in English patients)
 
HLA-Cw6
(Most definitive for psoriasis)
Psoriasis (influences early onset)
    
 
   


   
 
Table 1-18 MHC II-Associated Diseases
 HLA Associated Diseases
 
HLA-DR1
Lichen planus (oral and cutaneous)
 
HLA-DR2
Lupus
 
HLA-DR3 (DRw3)
Dermatitis herpetiformis, pemphigus gestationis, lupus erythematosus, lichen planus
 
HLA-DR4 (DRw4)
Pemphigus vulgaris, idiopathic chronic urticaria, pemphigoid gestationis, rheumatoid arthritis
 
HLA-DR6 (DRw6)
Pemphigus vulgaris
 
HLA-DR10
Lichen planus
 
HLA-DQ2 (DQw2)
Dermatitis herpetiformis (strongest association)
 
HLA-DQ8
Dermatitis herpetiformis, chronic urticaria