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Front Immunol. 2013 Dec 9;4:440. doi: 10.3389/fimmu.2013.00440.

An Update on PYRIN Domain-Containing Pattern Recognition Receptors: From Immunity to Pathology.

Author information

1
Division of Rheumatology, Department of Medicine, Feinberg School of Medicine, Northwestern University , Chicago, IL , USA.
2
Division of Rheumatology, Department of Medicine, Feinberg School of Medicine, Northwestern University , Chicago, IL , USA ; Robert H. Lurie Comprehensive Cancer Center, Interdepartmental Immunobiology Center and Skin Disease Research Center, Feinberg School of Medicine, Northwestern University , Chicago, IL , USA.

Abstract

Cytosolic pattern recognition receptors (PRRs) sense a wide range of endogenous danger-associated molecular patterns as well as exogenous pathogen-associated molecular patterns. In particular, Nod-like receptors containing a pyrin domain (PYD), called NLRPs, and AIM2-like receptors (ALRs) have been shown to play a critical role in host defense by facilitating clearance of pathogens and maintaining a healthy gut microflora. NLRPs and ALRs both encode a PYD, which is crucial for relaying signals that result in an efficient innate immune response through activation of several key innate immune signaling pathways. However, mutations in these PRRs have been linked to the development of auto-inflammatory and autoimmune diseases. In addition, they have been implicated in metabolic diseases. In this review, we summarize the function of PYD-containing NLRPs and ALRs and address their contribution to innate immunity, host defense, and immune-linked diseases.

KEYWORDS:

AIM2-like receptor; ALR; NLR; Nod-like receptor; PYRIN domain; inflammasome; innate immunity; pattern recognition receptor

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