Die häufigsten Fiebersyndrome und autoinflammatorischen Erkrankungen im Erwachsenenalter DOI
Anne Pankow, Martin Krusche

Deleted Journal, Journal Year: 2024, Volume and Issue: unknown

Published: Dec. 2, 2024

Autoinflammatorische Erkrankungen zeichnen sich durch entzündliche Manifestationen in verschiedenen Organsystemen aus, wobei wiederkehrende Fieberschübe, muskuloskeletale Beschwerden, gastrointestinale und kutane Symptome, begleitet von serologischen Entzündungszeichen, häufig auftreten. Die autoinflammatorischen umfassen seltene monogenetische Entitäten sowie multifaktorielle/polygene Krankheiten, die mit variabler Symptomatik im Laufe der Zeit manifestieren können. Beispiele für autoinflammatorische sind das familiäre Mittelmeerfieber (FMF), Cryopyrin-assoziierte periodische Syndrom (CAPS), TNF(Tumor-Nekrose-Faktor)-Rezeptor-assoziierte (TRAPS) neu beschriebene VEXAS-Syndrom. Bei den nichtmonogenetisch determinierten wichtigsten Vertreter Erwachsenenalter adulte Form Still-Erkrankung (AOSD) Schnitzler-Syndrom, bei denen eher eine polygenetische Suszeptibilität epigenetische Faktoren Rolle spielen.

The Role of Inflammasome in Abdominal Aortic Aneurysm and Its Potential Drugs DOI Open Access

Suyu Pi,

Sizheng Xiong,

Yan Yuan

et al.

International Journal of Molecular Sciences, Journal Year: 2024, Volume and Issue: 25(9), P. 5001 - 5001

Published: May 3, 2024

Abdominal aortic aneurysm (AAA) has been recognized as a serious chronic inflammatory degenerative disease in recent years. At present, there is no other effective intervention except surgical treatment for AAA. With the aging of human population, its incidence increasing year by year, posing threat to health. Modern studies suggest that vascular response core process AAA occurrence and development. Inflammasome, multiprotein complex located cytoplasm, mediates expression various cytokines like interleukin (IL)-1β IL-18, thus plays pivotal role inflammation regulation. Therefore, inflammasome may exert crucial influence on progression This article reviews some mechanism investigate then summarizes several potential drugs targeting AAA, aiming provide new ideas clinical prevention beyond methods.

Language: Английский

Citations

8

Implication of the LRR Domain in the Regulation and Activation of the NLRP3 Inflammasome DOI Creative Commons
Margaux Cescato,

Yixiang Y.J. Zhu,

Laurent Le Corre

et al.

Cells, Journal Year: 2024, Volume and Issue: 13(16), P. 1365 - 1365

Published: Aug. 16, 2024

The NLRP3 inflammasome is a critical component of the innate immune response. activation tightly controlled process involving an initial priming to express NLRP3, pro-IL-1 β, and pro-IL-18, followed by signal. precise mechanism not fully understood due diverse range activators, yet it effectively orchestrates caspase-1, which subsequently triggers release proinflammatory cytokines IL-1β IL-18. dysregulation can lead variety inflammatory diseases, highlighting its significant role in response disease pathogenesis. divided into three domains: PYD, NACHT, LRR domains. This review focuses on domain detailing structural characteristics, function pathogen sensing, degradation process, involvement auto-inhibition activation. Additionally, we discuss impact mutations within found atypical Cryopyrin-Associated Periodic Syndromes (CAPS), clinical relevance this domain.

Language: Английский

Citations

4

p16INK4a Aggravated Sepsis-associated Cardiac Injury by Inhibiting the PI3K/AKT Pathway and Inducing Redox Imbalance DOI
Baihong Li, Wei Wang, Xiaoyan Wang

et al.

Journal of Cardiovascular Translational Research, Journal Year: 2025, Volume and Issue: unknown

Published: Jan. 14, 2025

Language: Английский

Citations

0

Rapid validation of NLRP3 gain-of-function variants in cryopyrin-associated periodic syndromes DOI
Alex Quach, Yunyu Lao, Alexander Troelnikov

et al.

Journal of Allergy and Clinical Immunology, Journal Year: 2025, Volume and Issue: unknown

Published: Jan. 1, 2025

Language: Английский

Citations

0

A structural atlas of death domain fold proteins reveals their versatile roles in biology and function DOI Creative Commons

Evan Wu,

Ankita T. Kandalkar, Julian Ehrmann

et al.

Proceedings of the National Academy of Sciences, Journal Year: 2025, Volume and Issue: 122(8)

Published: Feb. 20, 2025

Death domain fold (DDF) superfamily proteins are critically important players in pathways of cell death and inflammation. DDFs often essential scaffolding domains receptors, adaptors, or effectors these by mediating homo- hetero-oligomerization including helical filament assembly. At the downstream ends pathways, effector oligomerization brings enzyme into proximity for their dimerization activation. Hundreds structures have been solved. However, a comprehensive understanding is lacking. In this article, we report curation DDF structural atlas as public website (deathdomain.org) deduce common distinct principles DDF-mediated among four families (death DD, DED, caspase recruitment CARD, pyrin PYD). We further annotate genome-wide based on AlphaFold-predicted models protein sequences. These studies reveal mechanistic rules widely distributed superfamily.

Language: Английский

Citations

0

Neutrophilic Urticarial Dermatosis: A Window into Systemic Inflammation and Autoimmune Disorders DOI

Xiaoxuan Cai,

Yadan Zheng,

Changyi Yang

et al.

Clinical Reviews in Allergy & Immunology, Journal Year: 2025, Volume and Issue: 68(1)

Published: May 6, 2025

Language: Английский

Citations

0

Clustering of NLRP3 induced by membrane or protein scaffolds promotes inflammasome assembly DOI Creative Commons
Elvira Boršić, Taja Železnik Ramuta, Sara Orehek

et al.

Nature Communications, Journal Year: 2025, Volume and Issue: 16(1)

Published: May 27, 2025

Language: Английский

Citations

0

CRISPR-Cpf1 system and its applications in animal genome editing DOI

Yawei Han,

Zisen Jia,

Keli Xu

et al.

Molecular Genetics and Genomics, Journal Year: 2024, Volume and Issue: 299(1)

Published: Aug. 1, 2024

Language: Английский

Citations

1

Die häufigsten Fiebersyndrome und autoinflammatorischen Erkrankungen im Erwachsenenalter DOI
Anne Pankow, Martin Krusche

Zeitschrift für Rheumatologie, Journal Year: 2024, Volume and Issue: 83(5), P. 363 - 375

Published: May 27, 2024

Citations

0

Modulating NLRP3 splicing with antisense oligonucleotides to control pathological inflammation DOI Creative Commons

Roni Klein,

Janset Onyuru,

Estela M. Viera

et al.

bioRxiv (Cold Spring Harbor Laboratory), Journal Year: 2024, Volume and Issue: unknown

Published: Sept. 6, 2024

Inflammation has an essential role in healing. However, over-active inflammation disrupts normal cellular functions and can be life-threatening when not resolved. The NLRP3 inflammasome, a component of the innate immune system, is intracellular multiprotein complex that senses stress-associated signals, and, for this reason promising therapeutic target treating unresolved, pathogenic inflammation. Alternative splicing

Language: Английский

Citations

0