ICOSL in host defense at epithelial barriers: lessons from ICOSLG deficiency

Curr Opin Immunol. 2021 Oct:72:21-26. doi: 10.1016/j.coi.2021.03.001. Epub 2021 Mar 21.

Abstract

Autosomal recessive mutations in Inducible T Cell Costimulator Ligand (ICOSLG) result in a combined immunodeficiency syndrome of humans, saliently marked by recurrent respiratory tract infections and significant disease with DNA-based viruses at epithelial barriers, including human papillomavirus (HPV). These features are also seen in persons with loss of function of the complementary gene, ICOS. The infection phenotypes associated with these natural experiments disclose a critical role of the corresponding proteins, ICOSL and ICOS, in human immunity at mucocutaneous barriers. Here, we review the syndromes of ICOSL and ICOS deficiency and explore the mechanisms by which the ICOSL:ICOS axis mediates epithelial host defenses.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Disease Resistance / genetics*
  • Disease Resistance / immunology
  • Disease Susceptibility / immunology
  • Epithelium / immunology*
  • Epithelium / metabolism*
  • Genetic Predisposition to Disease
  • Genotype
  • Germinal Center / immunology
  • Germinal Center / metabolism
  • Host-Pathogen Interactions / genetics*
  • Host-Pathogen Interactions / immunology
  • Humans
  • Inducible T-Cell Co-Stimulator Ligand / genetics*
  • Inducible T-Cell Co-Stimulator Ligand / metabolism*
  • Infections / etiology
  • Infections / metabolism
  • Mutation
  • Organ Specificity

Substances

  • ICOSLG protein, human
  • Inducible T-Cell Co-Stimulator Ligand