Nlrp

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 13.11.2023

Synonym(s)

NIGHT domain leucine-rich repeat pyrin domain

Definition
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Acronym for "NACHT domain, leucine-rich repeat, pyrin domains" a subgroup of NOD-like receptors, key proteins of the inflammasome.

General information
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NLRP 1-3 form the central scaffold of a protein complex(inflammasome). This plays a central role in inflammatory processes.

Activation of the inflammasome leads to activation of a caspase1protease from its inactive precursor pro-caspase1. The caspase1 enzyme cleaves pro-IL-1beta to the active interleukin-1beta, which, once released, leads to fever and generalized inflammation.

Interleukin-1beta also releases the pro-inflammatory IL-6 in the vascular endothelium. IL-6 induces pathogenic Th17 cells. These in turn block anti-inflammatory regulatory T cells(Tregs) and thus lead to an increase in inflammation.

Genetic variations in the genomic region of NLRP1 are associated with a high risk of generalized vitiligo.

NLRP3 was previously referred to as cryopyrin when a change in the pyrin domain was present in patients with cold-induced fever. Disease patterns with mutations of the NLRP3 gene are therefore referred to as cryopyrin-associated periodic syndromes (CAPS) or cryopathies.

Literature
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  1. Greaney AJ et al (2015) Bacterial Exotoxins and the Inflammasome. Front Immunol6:570.
  2. Jo EK et al (20169 Molecular mechanisms regulating NLRP3 inflammasome activation. Cell Mol Immunol 13:148-159.
  3. Santana PT et al (2016) Is the inflammasome relevant for epithelial cell function? Microbes Infect 18:93-101.
  4. Shao BZ et al (2015) NLRP3 inflammasome and its inhibitors: a review. Front Pharmacol 6:262.

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Last updated on: 13.11.2023