Pattern Recognition Receptors in Autoinflammation

  • Victor Saavedra
  • Fiona Moghaddas
  • Eicke LatzEmail author
  • Seth L. MastersEmail author


The immune system is essential for maintenance of tissue homeostasis. This task requires that immune cells detect and respond to dyshomeostatic states (when homeostasis has broken down) that can occur during invasion of the host with pathogenic microbes, after sterile trauma of tissues or during metabolic derangements. Research in the field of innate immunity has uncovered many molecular mechanisms by which the immune system can prevent the spread of infection, restore damaged tissues and respond to altered metabolism. These pathways involve different classes of pattern recognition receptors, some of which can directly detect minimal motifs (patterns) that are common to multiple pathogens or types of damaged cells. Here, we summarize the general concepts that have been developed to explain how immune recognition of dyshomeostasis is achieved and discuss our current knowledge of the innate immune signaling receptors that are known to directly bind ligands.


Toll-like receptor (TLR) Nucleotide-binding oligomerization domain (NOD) NOD-like receptor (NLR) C-type lectin receptor (CLR) RIG-I-like receptor (RLR) Pattern recognition receptor (PRR) 



Adenosine deaminase acting on RNA 1


Aicardi-Goutières syndrome


Absent in melanoma 2


Apoptosis related speck-like protein containing CARD


Adenosine triphosphate


Blau syndrome


Caspase activation and recruitment domain


Crohn’s disease


Cyclic dinucleotides


Cyclic GMP-AMP


cGAMP synthase


C-type lectin receptor


Clustered regularly interspaced short palindromic repeats


C-type lectin like domain


Damage associated molecular patterns


Diaminopimelic acid


Dendritic cell-specific intercellular adhesion molecule-3-grabbing non-integrin


Double-stranded DNA


Double-stranded RNA


Early onset sarcoidosis


Fc receptor gamma chain


Guanosine triphosphate


Hyaluronic acid


Homeostasis-altering molecular processes


Hematopoietic expression, interferon-inducible nature, and nuclear localization


Human immunodeficiency virus


High mobility group box 1


High molecular weight


Herpes simplex encephalitis


Heat shock protein


Inflammatory bowel disease




Interferon alpha/beta receptor 1


IκB kinase




Interferon regulatory factor 3


Immunoreceptor tyrosine-based activation motif


Immunoreceptor tyrosine-based inhibition motif


Inhibitor of NF-ĸB


Janus kinase




Laboratory of genetics and physiology 2


Low molecular weight


Lectin-like oxidized LDL receptor 1






MyD88 adaptor like (= TIRAP)


Mitogen-activated protein kinase 1


Mitochondrial antiviral signaling


Mouse cytomegalovirus


Myeloid differentiation factor 2


Melanoma differentiation-associated protein 5


Muramyl dipeptide


Myeloid inhibitory C-type lectin


Macrophage-inducible C-type lectin


Micro RNA


Messenger RNA


Monosodium urate


Myeloid differentiation primary response gene 88


Nucleotide binding site


Nuclear factor of activated T-cells


Nuclear factor–ĸB


Natural killer


NOD-like receptor


NOD-like receptor protein


Nucleotide-binding oligomerization domain


Oxidized 1-palmitoyl-2-arachidonoyl-sn-glycero-3-phosphorylcholine


Pathogen-associated molecular patterns


Peripheral blood mononuclear cells


Plasmacytoid dendritic cells


Primary immunodeficiency


PYD-only protein


Pattern-recognition receptors


Pyrin domain


Retinoic acid-inducible gene 1


Receptor-interacting serine/threonine kinase 2


RIG-I-like receptor


Ribonucleic acid


Reactive oxygen species


Sterile α- and armadillo motif containing protein


STING-associated vasculopathy with onset in infancy


Src homology region 2


SH2 domain-containing phosphatase


Small interfering RNA


Singleton-Merten syndrome


Single nucleotide polymorphism


Single stranded RNA


Signal transducer and activator of transcription


Stimulator of interferon genes


TANK-binding kinase 1


Mitochondrial transcription factor A


Toll/IL-1 receptor


TIR domain containing adaptor protein (= MAL)


Toll-like receptor


Transmembrane protein 173


TRIF-related adaptor molecule


Three-prime repair exonuclease 1


TIR-domain containing adaptor protein inducing IFN-β (=TICAM1)


Transfer RNA


Unc-93 homologue B1


Whole exome sequencing


Wild type


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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Institute of Innate ImmunityUniversity Hospital BonnBonnGermany
  2. 2.Inflammation DivisionThe Walter and Eliza Hall Institute of Medical ResearchParkvilleAustralia
  3. 3.Department of Medical BiologyThe University of MelbourneParkvilleAustralia
  4. 4.Department of MedicineUniversity of Massachusetts Medical SchoolWorcesterUSA
  5. 5.German Center for Neurodegenerative DiseasesBonnGermany

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