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Updated: Jan 15 2022

Structure and Function of MHC

  • Introduction
    • Major histocompatibility complexes (MHC) are cell surface expressed proteins that
      • bind to protein peptides in a surface groove
      • present bound peptides to
        • receptors on circulating T-cells
      • participate in the
        • activation of the adaptive immune response
    • MHC are encoded by HLA genes that are localized to several different genetic regions
      • different HLA regions encode for different types of MHC
    • Allogenic MHC can be recognized by T-cells of the adaptive immune system
      • mismatch of MHC is an important cause of transplant rejection
  • MHC Classes
    • There are two different classes of MHC encoded in the human genome
      • class I MHC usually presents
        • endogenous antigens to CD8+ killer T-cells
      • class II MHC usually presents
        • endocytosed antigens to CD4+ helper T-cells
      • antigens may be "cross-presented" by the other MHC class in some cases
    • The two MHC classes differ in several characteristics including
      • HLA loci that encodes the gene
      • sites of expression
      • protein structure
      • antigen loading site
      • antigen loading mechanism
      • binding partners
      • Differences Between MHC Classes
      • Feature
      • MHC Class I
      • MHC Class II
      • HCA Loci
      • HLA-A
      • HLA-B
      • HLA-C
      • One letter
      • HLA-DP
      • HLA-DQ
      • HLA-DR
      • Two letters
      • Site of expression
      • All nucleated cells
      • Platelets
      • Antigen presenting cells
      • Protein structure
      • 1 long chain composing antigen binding site 
      • 1 short chain called β2 - microglobulin
      • 2 equal length chains
      • Additional invariant chain
      • Antigen loading site
      • Rough endoplasmic reticulum (RER)
      • Acidified endosomes
      • Loading mechanism
      • Endogenous proteins are degraded by the proteosome
      • Antigenic peptides are transported into the RER by the TAP transporter 
      • Antigens bind to MHC molecules directly
      • Exogenous proteins are endocytosed from the cell surface
      • Antigens are produced by degradation within the endosomal compartment
      • Antigens bind to MHC molecules after release of the invariant chain
      • Binding Partners
      • T-cell receptor
      • CD8 co-receptor
      • T-cell receptor
      • CD4 co-receptor
  • Disease Associations
    • Each individual possesses a set of MHC class I and class II subtypes
      • these "haplotypes" define the peptides that will be recognized
    • Some haplotypes are associated with specific diseases because they may
      • preferentially bind to certain self-peptides
      • lead to a failure of immune tolerance
      • MHC Subtypes Associated With Diseases
      • Class I Haplotype
      • Diseases
      • Class II Haplotype
      • Diseases
      • A3
      • Hemochromatosis
      • DQ2/DQ8
      • Celiac disease
      • B8
      • Addison disease
      • Myasthenia gravis
      • Graves disease
      • DR2
      • Multiple sclerosis
      • Hay fever
      • Systemic lupus erythematosis
      • Goodpasture syndrome 
      • B27
      • Psoriatic arthritis
      • Ankylosing spondylitis
      • Inflammatory bowel disease
      • Reactive arthritis
      • DR3
      • Diabetes melliutus type I
      • Systemic lupus erythematosis
      • Graves disease
      • Hashimoto thyroiditis
      • Addison disease
      • DR4
      • Rheumatoid arthritis
      • Diabetes mellitus type 1
      • Addison disease
      • DR5
      • Pernicious anemia
      • Hashimoto thyroiditis
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