Keratoendotheliitis Fugax Hereditaria: A Novel Cryopyrin-Associated Periodic Syndrome Caused by a Mutation in the Nucleotide-Binding Domain, Leucine-Rich Repeat Family, Pyrin Domain-Containing 3 ( NLRP3 ) Gene

Turunen, Joni A.; Wedenoja, Juho; Repo, Pauliina; Järvinen, Reetta-Stiina; Jäntti, Johannes E; Mörtenhumer, Sanna; Riikonen, Antti S.; Lehesjoki, Anna‐Elina; Majander, Anna; Kivelä, Tero

doi:10.1016/j.ajo.2018.01.017

Cited by 31 publications

(55 citation statements)

References 36 publications

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“…To restore the normal tissue functions during the harmful inflammatory conditions, clearance of inflammatory cells, along with pro-inflammatory signaling pathways, are required ( 1 ). The nucleotide-binding oligomerization domain-like receptor family pyrin domain-containing proteins (NLRPs), specifically NLRP3, a cytosolic innate immune signaling receptor, have been detected at elevated levels in several inflammatory diseases, such as chronic infantile neurological cutaneous and articular (CINCA) syndrome, familial cold auto-inflammatory syndrome (FCAS), keratoendotheliitis fugax hereditaria, Muckle–Wells syndrome (MWS), neonatal onset multisystem inflammatory disease (NOMID) ( 2 , 3 ), and familial Mediterranean fever ( 4 ). In addition, NLRP3 has been associated with gout ( 5 ), type 2 diabetes, multiple sclerosis, atherosclerosis ( 6 ), and Alzheimer's, Parkinson's, and prion diseases ( 7 – 9 ).…”

Section: Introductionmentioning

confidence: 99%

Immunomodulatory Effects of Diterpenes and Their Derivatives Through NLRP3 Inflammasome Pathway: A Review

et al. 2020

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Nucleotide-binding oligomerization domain-like receptor family pyrin domain-containing protein (NLRP) inflammasomes are involved in the molecular pathogenesis of many diseases and disorders. Among NLRPs, the NLRP3 (in humans encoded by the NLRP3 gene) is expressed predominantly in macrophages as a component of the inflammasome and is associated with many diseases, including gout, type 2 diabetes, multiple sclerosis, atherosclerosis, and neurological diseases and disorders. Diterpenes containing repeated isoprenoid units in their structure are a member of some essential oils that possess diverse biological activities and are becoming a landmark in the field of drug discovery and development. This review sketches a current scenario of diterpenes or their derivatives acting through NLRPs, especially NLRP3-associated pathways with anti-inflammatory effects. For this, a literature survey on the subject has been undertaken using a number of known databases with specific keywords. Findings from the aforementioned databases suggest that diterpenes and their derivatives can exert anti-inflammatory effects via NLRPs-related pathways. Andrographolide, triptolide, kaurenoic acid, carnosic acid, oridonin, teuvincenone F, and some derivatives of tanshinone IIA and phorbol have been found to act through NLRP3 inflammasome pathways. In conclusion, diterpenes and their derivatives could be one of the promising compounds for the treatment of NLRP3-mediated inflammatory diseases and disorders.

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Section: Introductionmentioning

confidence: 99%

Immunomodulatory Effects of Diterpenes and Their Derivatives Through NLRP3 Inflammasome Pathway: A Review

et al. 2020

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“…First described in 1964 in Finnish families, patients experienced anterior chamber reactions multiple times per year with resultant pseudoguttae and patches of dark or absent endothelium [43,44]. A point mutation in the NLRP3 gene located on the long arm of chromosome 1 coding for crypoporin protein found largely in macrophages was isolated in these patients [45,46]. Multiple endothelial attacks can lead to endothelial decompensation.…”

Section: Inflammationsmentioning

confidence: 99%

Corneal Endothelial Decompensation

Gupta¹,

Deng²

2020

Klin Monbl Augenheilkd

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Endothelial decompensation can occur from a variety of insults to the endothelium that result in loss of stromal clarity. Direct insults to the endothelium commonly occur in inherited, inflammatory, traumatic, immunological, and infectious etiologies. These injuries may cause transient injury without decompensation, but repetitive injury or severe isolated injury can lead to permanent non-compensatory endothelial cell loss. Elevated intraocular pressure can induce stromal hydration, either primarily or secondarily. With partial and full thickness corneal transplants, chronic endothelial dysfunction can be treated surgically when medical therapy proves inadequate. Practitioners should be aware of the underlying causes for corneal endothelial injury.

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“…The receptor has shown interaction with ligands like 5,8,11,14-Eicosatetraenoic acid, methyl ester, (all-Z); 1-Hydroxy-1,7dimethyl-4-isopropyl-2,7-cyclodecadiene 4-Pregnen-6.beta.,11.beta., 17…”

Section: Binding Affinity Of the Compounds With 2z62mentioning

confidence: 99%

Evaluation of Medical Benefits of Coccinia Indica Through in-Silico Approach

Peter¹,

Kappagantu²,

Thripureshwari³

et al. 2020

Int Res J Pharm

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Coccinia indica is a vine that majorly grows in tropical climates. It is commonly used in south Asian cuisine and is known to show various medicinal properties. Various parts of the plant are used as a medicine such as roots, fruits and leaves. It is commonly known to cure disease such as liver disease, asthma, ulcer, urinary infection, allergy, bronchitis. In the current study, we try to understand the hepatoprotective and anti-arthritis of the plant via insilico docking methodologies. Firstly, the plant extract was subjected to GCMS analysis and the active components of the plant were obtained. The most reactive compounds amongst the result were chosen as ligands to conduct in-silico docking against the receptors of hepatoxicity and arthritis. Ligands like Pterin-6-carboxylic acid, 2r,3s-9-[[1,3-Dihydroxy-4-Fluoro-3-Butoxy]Methyl]Gu, D(17a)-Homo-C,18-dinorcard-20(22)-enolide, 14hydroxy-17a-methylene-3-oxo-, (5.beta.) -Showed potential interaction against chosen receptors. Coccinia indica active compound has shown better binding affinity between hepatotoxicity and arthritis receptors. The potential active compound of Coccinia indica can be further studied in animal models to demonstrate its beneficial activities.

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Keratoendotheliitis Fugax Hereditaria: A Novel Cryopyrin-Associated Periodic Syndrome Caused by a Mutation in the Nucleotide-Binding Domain, Leucine-Rich Repeat Family, Pyrin Domain-Containing 3 ( NLRP3 ) Gene

Abstract: Keratoendotheliitis fugax hereditaria is an autoinflammatory cryopyrin-associated periodic syndrome caused by a missense mutation c.61G>C in exon 1 of NLRP3 in Finnish patients. It is additionally expected to occur in other populations of European descent.

Cited by 31 publications

References 36 publications

Immunomodulatory Effects of Diterpenes and Their Derivatives Through NLRP3 Inflammasome Pathway: A Review

Immunomodulatory Effects of Diterpenes and Their Derivatives Through NLRP3 Inflammasome Pathway: A Review

Corneal Endothelial Decompensation

Evaluation of Medical Benefits of Coccinia Indica Through in-Silico Approach

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