Variations in MHC Class II Antigen Processing and Presentation in Health and Disease

Emil R. Unanue, Vito Turk, Jacques Neefjes

Research output: Contribution to journalArticlepeer-review

154 Scopus citations


MHC class II (MHC-II) molecules are critical in the control of many immune responses. They are also involved in most autoimmune diseases and other pathologies. Here, we describe the biology of MHC-II and MHC-II variations that affect immune responses. We discuss the classic cell biology of MHC-II and various perturbations. Proteolysis is a major process in the biology of MHC-II, and we describe the various components forming and controlling this endosomal proteolytic machinery. This process ultimately determines the MHC-II-presented peptidome, including cryptic peptides, modified peptides, and other peptides that are relevant in autoimmune responses. MHC-II also variable in expression, glycosylation, and turnover. We illustrate that MHC-II is variable not only in amino acids (polymorphic) but also in its biology, with consequences for both health and disease.

Original languageEnglish
Pages (from-to)265-297
Number of pages33
JournalAnnual Review of Immunology
StatePublished - May 20 2016


  • Antigen presentation
  • Autoimmunity
  • Cathepsins
  • HLA
  • Inhibitors
  • MHC class II
  • Polymorphism
  • Processing


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