NF-κB: at the borders of autoimmunity and inflammation

L Barnabei, E Laplantine, W Mbongo… - Frontiers in …, 2021 - frontiersin.org
The transcription factor NF-κB regulates multiple aspects of innate and adaptive immune
functions and serves as a pivotal mediator of inflammatory response. In the first part of this …

Targeting NF-κB pathway for the therapy of diseases: mechanism and clinical study

H Yu, L Lin, Z Zhang, H Zhang, H Hu - Signal transduction and targeted …, 2020 - nature.com
NF-κB pathway consists of canonical and non-canonical pathways. The canonical NF-κB is
activated by various stimuli, transducing a quick but transient transcriptional activity, to …

Human inborn errors of immunity: 2019 update on the classification from the International Union of Immunological Societies Expert Committee

SG Tangye, W Al-Herz, A Bousfiha, T Chatila… - Journal of clinical …, 2020 - Springer
We report the updated classification of Inborn Errors of Immunity/Primary
Immunodeficiencies, compiled by the International Union of Immunological Societies Expert …

Type 2 immunity in the skin and lungs

CA Akdis, PD Arkwright, MC Brüggen, W Busse… - Allergy, 2020 - Wiley Online Library
There has been extensive progress in understanding the cellular and molecular
mechanisms of inflammation and immune regulation in allergic diseases of the skin and …

[HTML][HTML] CARD–BCL-10–MALT1 signalling in protective and pathological immunity

J Ruland, L Hartjes - Nature Reviews Immunology, 2019 - nature.com
Abstract CARD protein–BCL-10–MALT1 (CBM) signalosomes are multiprotein signalling
platforms that control immune and inflammatory pathways in most tissues. After exposure to …

STAT3 hyper-IgE syndrome—an update and unanswered questions

C Tsilifis, AF Freeman, AR Gennery - Journal of clinical immunology, 2021 - Springer
The hyper-IgE syndromes (HIES) are a heterogeneous group of inborn errors of immunity
sharing manifestations including increased infection susceptibility, eczema, and raised …

A recessive form of hyper-IgE syndrome by disruption of ZNF341-dependent STAT3 transcription and activity

V Béziat, J Li, JX Lin, CS Ma, P Li, A Bousfiha… - Science …, 2018 - science.org
Heterozygosity for human signal transducer and activator of transcription 3 (STAT3)
dominant-negative (DN) mutations underlies an autosomal dominant form of hyper …

An integrated taxonomy for monogenic inflammatory bowel disease

C Bolton, CS Smillie, S Pandey, R Elmentaite, G Wei… - Gastroenterology, 2022 - Elsevier
Background & aims Monogenic forms of inflammatory bowel disease (IBD) illustrate the
essential roles of individual genes in pathways and networks safeguarding immune …

Pediatric Evans syndrome is associated with a high frequency of potentially damaging variants in immune genes

J Hadjadj, N Aladjidi, H Fernandes… - Blood, The Journal …, 2019 - ashpublications.org
Evans syndrome (ES) is a rare severe autoimmune disorder characterized by the
combination of autoimmune hemolytic anemia and immune thrombocytopenia. In most …

Genetics and epigenetics of atopic dermatitis: an updated systematic review

MJ Martin, M Estravís, A García-Sánchez, I Dávila… - Genes, 2020 - mdpi.com
Background: Atopic dermatitis is a common inflammatory skin disorder that affects up to 15–
20% of the population and is characterized by recurrent eczematous lesions with intense …