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 …

Primary immune regulatory disorders with an autoimmune lymphoproliferative syndrome-like phenotype: immunologic evaluation, early diagnosis and management

M López-Nevado, LI González-Granado… - Frontiers in …, 2021 - frontiersin.org
Primary immune regulatory disorders (PIRD) are associated with autoimmunity,
autoinflammation and/or dysregulation of lymphocyte homeostasis. Autoimmune …

Hypomorphic caspase activation and recruitment domain 11 (CARD11) mutations associated with diverse immunologic phenotypes with or without atopic disease

B Dorjbal, JR Stinson, CA Ma, MA Weinreich… - Journal of Allergy and …, 2019 - Elsevier
Background Caspase activation and recruitment domain 11 (CARD11) encodes a scaffold
protein in lymphocytes that links antigen receptor engagement with downstream signaling to …

The CBM-opathies—a rapidly expanding spectrum of human inborn errors of immunity caused by mutations in the CARD11-BCL10-MALT1 complex

HY Lu, BM Bauman, S Arjunaraja, B Dorjbal… - Frontiers in …, 2018 - frontiersin.org
The caspase recruitment domain family member 11 (CARD11 or CARMA1)—B cell
CLL/lymphoma 10 (BCL10)—MALT1 paracaspase (MALT1)[CBM] signalosome complex …

Human inborn errors of immunity to herpes viruses

E Jouanguy, V Béziat, TH Mogensen… - Current opinion in …, 2020 - Elsevier
Infections with any of the nine human herpes viruses (HHV) can be asymptomatic or life-
threatening. The study of patients with severe diseases caused by HHVs, in the absence of …

Assembly mechanism of the CARMA1–BCL10–MALT1–TRAF6 signalosome

L David, Y Li, J Ma, E Garner… - Proceedings of the …, 2018 - National Acad Sciences
The CARMA1–BCL10–MALT1 (CBM) signalosome is a central mediator of T cell receptor
and B cell receptor-induced NF-κB signaling that regulates multiple lymphocyte functions …

Hyper-IgE syndrome, 2021 update

Y Minegishi - Allergology International, 2021 - Elsevier
Clinically and pathologically, the patients with hyper-IgE syndrome present similar skin
manifestations to common atopic dermatitis. The original hyper-IgE syndrome is …

Stratification of systemic lupus erythematosus patients into three groups of disease activity progression according to longitudinal gene expression

D Toro‐Domínguez, J Martorell‐Marugán… - Arthritis & …, 2018 - Wiley Online Library
Objective The highly heterogeneous clinical presentation of systemic lupus erythematosus
(SLE) is characterized by the unpredictable occurrence of disease flares and organ damage …

Combined immunodeficiency and atopy caused by a dominant negative mutation in caspase activation and recruitment domain family member 11 (CARD11)

H Dadi, TA Jones, D Merico, N Sharfe, A Ovadia… - Journal of Allergy and …, 2018 - Elsevier
Background Combined immunodeficiency (CID) is a T-cell defect frequently presenting with
recurrent infections, as well as associated immune dysregulation manifesting as …