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 …

30 years of NF-κB: a blossoming of relevance to human pathobiology

Q Zhang, MJ Lenardo, D Baltimore - Cell, 2017 - cell.com
NF-κB was discovered 30 years ago as a rapidly inducible transcription factor. Since that
time, it has been found to have a broad role in gene induction in diverse cellular responses …

[HTML][HTML] Loss-of-function nuclear factor κB subunit 1 (NFKB1) variants are the most common monogenic cause of common variable immunodeficiency in Europeans

P Tuijnenburg, HL Allen, SO Burns, D Greene… - Journal of allergy and …, 2018 - Elsevier
Background The genetic cause of primary immunodeficiency disease (PID) carries
prognostic information. Objective We conducted a whole-genome sequencing study …

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 …

Intrinsic antiproliferative activity of the innate sensor STING in T lymphocytes

S Cerboni, N Jeremiah, M Gentili… - Journal of Experimental …, 2017 - rupress.org
Activation of the cyclic dinucleotide sensor stimulator of interferon (IFN) genes (STING) is
critical for IFN and inflammatory gene expression during innate immune responses …

Severe combined immunodeficiencies and related disorders

A Fischer, LD Notarangelo, B Neven… - Nature reviews Disease …, 2015 - nature.com
Severe combined immunodeficiencies (SCIDs) comprise a group of rare, monogenic
diseases that are characterized by an early onset and a profound block in the development …

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 …

Inherited CARD9 deficiency: invasive disease caused by ascomycete fungi in previously healthy children and adults

E Corvilain, JL Casanova, A Puel - Journal of clinical immunology, 2018 - Springer
Autosomal recessive CARD9 deficiency underlies life-threatening, invasive fungal infections
in otherwise healthy individuals normally resistant to other infectious agents. In less than 10 …

Cellular and molecular mechanisms breaking immune tolerance in inborn errors of immunity

G Sogkas, F Atschekzei, IR Adriawan… - Cellular & Molecular …, 2021 - nature.com
In addition to susceptibility to infections, conventional primary immunodeficiency disorders
(PIDs) and inborn errors of immunity (IEI) can cause immune dysregulation, manifesting as …

[HTML][HTML] Inborn errors of human B cell development, differentiation, and function

SG Tangye, T Nguyen, EK Deenick, VL Bryant… - Journal of Experimental …, 2023 - rupress.org
B cells develop from hematopoietic stem cells in the bone marrow. Once generated, they
serve multiple roles in immune regulation and host defense. However, their most important …