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Inflammasomes and Autoinflammation

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Textbook of Autoinflammation

Abstract

Inflammasomes are large intracellular multi-protein polymeric complexes comprised of sensors, adaptor proteins and caspases. As innate immune sensors capable of recognizing and rapidly responding to pathogen and metabolic danger signals, inflammasomes act as key modulators of initial immune responses. This chapter focuses on the known inflammasome complexes, how they assemble into a molecular platform for caspase-1 activation and ultimately lead to the release of pro-inflammatory cytokines in the context of the innate immune response. A brief discussion of the role for the inflammasomes in disease is included.

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Abbreviations

AIM2:

Absent in melanoma 2

ALR:

AIM2-like receptor

ANA:

Antinuclear antibody

ASC:

Apoptosis related speck-like protein containing CARD

ATP:

Adenosine triphosphate

Bid:

BH3 interacting-domain death agonist

CAPS:

Cryopyrin-associated periodic syndromes

CARD:

Caspase activation and recruitment domain

CRP:

C-reactive protein

DAMP:

Damage associated molecular patterns

FADD:

Fas-associated death domain

FCAS:

Familial cold autoinflammatory syndrome

FIIND:

Function to find domain

FMF:

Familial Mediterranean fever

GTP:

Guanosine-5′-triphosphate

GWAS:

Genome-wide association study

ICE:

Interleukin-1β converting enzyme

IFN:

Interferon

IGIF:

Interferon-gamma inducing factor

IL:

Interleukin

LDL:

Low density lipoprotein

LPS:

Lipopolysaccharide

LRRs:

Leucine-rich-repeats

MAS:

Macrophage-activation syndrome

MDP:

Muramyl-dipeptide

MSU:

Monosodium urate

NAIP:

NLR family, apoptosis inhibitory protein

NASH:

Nonalcoholic steatohepatitis

NBS:

Nucleotide binding site

NEK:

Nima-related kinase

NF-ĸB:

Nuclear factor–ĸB

NLR:

NOD-like receptor

NLRC:

NLR family CARD domain-containing protein

NOD:

Nucleotide-binding oligomerization domain

NOMID:

Neonatal-onset multisystem inflammatory disease

PAMP:

Pathogen-associated molecular patterns

PRR:

Pattern-recognition receptors

PYD:

Pyrin domain

ROS:

Reactive oxygen species

SNP:

Single-nucleotide polymorphism

TLR:

Toll-like receptor

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  1. University of California, San Diego, San Diego, CA, USA

    Lori Broderick

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  1. Lori Broderick
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  1. Pediatric Rheumatology Unit, Shaare Zedek Medical Center and Hebrew University, Jerusalem, Israel

    Philip J. Hashkes

  2. Department Paediatrics, Division of Rheumatology, The Hospital for Sick Children and University of Toronto, Toronto, ON, Canada

    Ronald M. Laxer

  3. Department of Internal Medicine, Radboudumc Expertisecenter for Immunodeficiency and Autoinflammation, Radboud University Medical Center, Nijmegen, The Netherlands

    Anna Simon

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Broderick, L. (2019). Inflammasomes and Autoinflammation. In: Hashkes, P., Laxer, R., Simon, A. (eds) Textbook of Autoinflammation. Springer, Cham. https://doi.org/10.1007/978-3-319-98605-0_5

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