How to build a fire: the genetics of autoinflammatory diseases

J Zhang, PY Lee, I Aksentijevich… - Annual Review of …, 2023 - annualreviews.org
Systemic autoinflammatory diseases (SAIDs) are a heterogeneous group of disorders
caused by excess activation of the innate immune system in an antigen-independent …

Potential roles of NLRP3 inflammasome in the pathogenesis of Kawasaki disease

A Shahi, S Afzali, Z Firoozi… - Journal of Cellular …, 2023 - Wiley Online Library
There is a heterogeneous group of rare illnesses that fall into the vasculitis category and are
characterized mostly by blood vessel inflammation. Ischemia and disrupted blood flow will …

ZDHHC5-mediated NLRP3 palmitoylation promotes NLRP3-NEK7 interaction and inflammasome activation

S Zheng, X Que, S Wang, Q Zhou, X Xing, L Chen… - Molecular cell, 2023 - cell.com
The nucleotide-binding domain (NBD), leucine-rich repeat (LRR), and pyrin domain (PYD)-
containing protein 3 (NLRP3) inflammasome is a critical mediator of the innate immune …

NLRP3 inflammasome: a key player in the pathogenesis of life-style disorders

R Ramachandran, A Manan, J Kim… - Experimental & Molecular …, 2024 - nature.com
Proinflammatory cytokines and chemokines play a crucial role in regulating the inflammatory
response, which is essential for the proper functioning of our immune system. When …

NLRP3 inflammasome mediates abnormal epithelial regeneration and distal lung remodeling in silica-induced lung fibrosis

H Zhou, Q Zhang, C Liu, J Fan… - International …, 2024 - spandidos-publications.com
NOD-like receptor protein 3 (NLRP3) inflammasome is closely related to silica particle-
induced chronic lung inflammation but its role in epithelial remodeling, repair and …

Anti-inflammatory effects of elexacaftor/tezacaftor/ivacaftor in adults with cystic fibrosis heterozygous for F508del

HH Jarosz-Griffiths, L Gillgrass, LR Caley, G Spoletini… - Plos one, 2024 - journals.plos.org
Inflammation is a key driver in the pathogenesis of cystic fibrosis (CF). We assessed the
effectiveness of elexacaftor/tezacaftor/ivacaftor (ETI) therapy on downregulating systemic …

Pathogenic variants in the NLRP3 LRR domain at position 861 are responsible for a boost-dependent atypical CAPS phenotype

A Fayand, M Cescato, L Le Corre, A Terré… - Journal of Allergy and …, 2023 - Elsevier
Background Cryopyrin-associated periodic syndrome (CAPS) is associated with NLRP3
pathogenic variants, mostly located in the NACHT (neuronal apoptosis inhibitor protein …

[HTML][HTML] Miltefosine as a PPM1A activator improves AD-like pathology in mice by alleviating tauopathy via microglia/neurons crosstalk

J Lv, X Shen, X Shen, X Li, Z Jin, X Ouyang, J Lu… - Brain, Behavior, & …, 2022 - Elsevier
Alzheimer's disease (AD) is a progressively neurodegenerative disease without effective
treatment. Here, we reported that the levels of expression and enzymatic activity of …

Cellular signaling, molecular activation, and regulation of auto-active inflammasomes: Insights from disease-associated variants

I Aksentijevich - Inflammasome Biology, 2023 - Elsevier
Erroneous activation of inflammasomes can lead to autoinflammatory diseases, a group of
genetically diverse but symptomatically similar disorders, termed inflammasomopathies. In …

Cellular activation, active inflammasomes: signaling, and regulation molecular insights

I Aksentijevich - … Biology: Fundamentals, Role in Disease States …, 2022 - books.google.com
Monogenic autoinflammatory disorders are linked to germline and somatic pathogenic
variants in several proteins that nucleate inflammasome complex. Associated phenotypes …