Research Information System
in: Physiology and Pathology of Immunology, Nima Rezaei, Editor, IntechOpen, London, pp.75-97, 2017
mmune regulation is an essential feature of immune responses. The
failure of such regulation results in allergic reactions and
debilitating autoimmune diseases that can be fatal. Furthermore, the
recent increase in the prevalence of the latter as well as the medical
severity makes this a subject of great medical interest. Autoimmunity
results from a breakdown in or the failure of the self-tolerance
mechanisms. Many genes have been identified in which mutations cause the
predisposition to autoinflammation and autoimmunity in human and in
animal models. The relatively small number of genes explored to date
unquestionably shows the challenges of identifying the associated genes
in outbred populations of humans. One chief contributing gene family to
both autoinflammatory and autoimmune diseases is the nucleotide-binding
and oligomerization domain (NOD)-like receptor (NLR) family. Ever since
their discovery, NLRs have drawn considerable attention for their
ability to form multiprotein complexes called inflammasomes and also for
their roles as NLRs, independent of inflammasome complexes. We herein
first revisit general characteristics of NLRs and inflammasomes. We then
couple this knowledge with the most recent findings related to
autoinflammatory and autoimmune diseases, while highlighting some
unanswered questions and future perspectives in elucidating NLR roles in
health and disease.