IMR Press / FBL / Volume 6 / Issue 3 / DOI: 10.2741/gruen

Frontiers in Bioscience-Landmark (FBL) is published by IMR Press from Volume 26 Issue 5 (2021). Previous articles were published by another publisher on a subscription basis, and they are hosted by IMR Press on as a courtesy and upon agreement with Frontiers in Bioscience.

Human MHC class III and IV genes and disease associations
Show Less
1 Department of Pediatrics, Yale Child Health Research Center, Yale University School of Medicine, New Haven, CT 06520-8081, USA
2 Department of Genetics, Boyer Center for Molecular Medicine, Yale University School of Medicine
Front. Biosci. (Landmark Ed) 2001, 6(3), 960–972;
Published: 1 August 2001

The major histocompatibility complex (MHC) was initially defined as the genetic locus encoding the Class I and Class II highly polymorphic cell surface antigens that are now known to present antigen to matched sets of T cell receptors. Genes for several diverse complement components, specifically Bf, C2, and C4 were found between the Class I and II genes, in a region later dubbed Class III. More recently, several genes have been described that are encoded in the telomeric end of the Class III region and that appear to be involved in both global and specific inflammatory responses. Due to this commonality of function this gene-rich region was dubbed Class IV, and includes the TNF family, AIF1, and HSP70. The genes of the Class III and Class IV regions are sufficiently divergent in sequence and structure so that clustering is not explicable in terms of gene duplication or divergence. We present some of the newer pertinent information and puzzling features of the genes embraced in the Class IV region and discuss possible roles in specific autoimmune diseases linked to this region.

Back to top