Biochemically deleterious human NFKB1 variants underlie an autosomal dominant form of common variable immunodeficiency
Autosomal dominant (AD) NFKB1 deficiency is thought to be the most common genetic
etiology of common variable immunodeficiency (CVID). However, the causal link between …
etiology of common variable immunodeficiency (CVID). However, the causal link between …
Of mycelium and men: inherent human susceptibility to fungal diseases
DC Vinh - Pathogens, 2023 - mdpi.com
In medical mycology, the main context of disease is iatrogenic-based disease. However,
historically, and occasionally, even today, fungal diseases affect humans with no obvious …
historically, and occasionally, even today, fungal diseases affect humans with no obvious …
How do nuclear factor kappa B (NF-κB) 1 and NF-κB2 defects lead to the incidence of clinical and immunological manifestations of inborn errors of immunity?
N Fathi, H Mojtahedi, M Nasiri… - Expert Review of …, 2023 - Taylor & Francis
Introduction Genetic defects affect the manner of the immune system's development,
activation, and function. Nuclear factor-kappa B subunit 1 (NF-κB1) and NF-κB2 are involved …
activation, and function. Nuclear factor-kappa B subunit 1 (NF-κB1) and NF-κB2 are involved …
Defining the Role of Nuclear Factor (NF)-κB p105 Subunit in Human Macrophage by Transcriptomic Analysis of NFKB1 Knockout THP1 Cells
Since its discovery over 30 years ago the NF-ĸB family of transcription factors has gained the
status of master regulator of the immune response. Much of what we understand of the role …
status of master regulator of the immune response. Much of what we understand of the role …
Inborn errors of immunity: Recent progress
N Seth, KS Tuano, J Chinen - Journal of Allergy and Clinical Immunology, 2021 - Elsevier
Recent advances in the field of inborn errors of immunity (IEIs) have been wide in scope,
including progress in mechanisms of disease, diagnosis, and management. New gene …
including progress in mechanisms of disease, diagnosis, and management. New gene …
Mistuned NF-κB signaling in lymphocytes: lessons from relevant inborn errors of immunity
G Dabbah-Krancher, AL Snow - Clinical and Experimental …, 2023 - academic.oup.com
Inborn errors of immunity (IEIs) continuously remind us that multiple checks and balances
are built into the adaptive immune system to maintain homeostasis, ensuring effective …
are built into the adaptive immune system to maintain homeostasis, ensuring effective …
Case report and review of the literature: immune dysregulation in a large familial cohort due to a novel pathogenic RELA variant
K Lecerf, DC Koboldt, HS Kuehn, V Jayaraman… - …, 2023 - academic.oup.com
Objective To explore and define the molecular cause (s) of a multi-generational kindred
affected by Bechet's-like mucocutaneous ulcerations and immune dysregulation. Methods …
affected by Bechet's-like mucocutaneous ulcerations and immune dysregulation. Methods …
Nomogram for predicting early mortality after umbilical cord blood transplantation in children with inborn errors of immunity
P Wang, C Liu, Z Wei, W Jiang, H Sun, Y Wang… - Journal of Clinical …, 2023 - Springer
Purpose Pediatric patients with inborn errors of immunity (IEI) undergoing umbilical cord
blood transplantation (UCBT) are at risk of early mortality. Our aim was to develop and …
blood transplantation (UCBT) are at risk of early mortality. Our aim was to develop and …
Pomelo peel oil alleviates cerebral NLRP3 inflammasome activation in a cardiopulmonary resuscitation rat model
XS Zou, L Xie, WY Wang, GY Zhao… - Experimental and …, 2021 - spandidos-publications.com
The NLR family pyrin domain‑containing 3 (NLRP3) inflammasome, which is composed of
NLRP3, apoptosis‑associated speck‑like protein containing a CARD (ASC) and pro‑caspase …
NLRP3, apoptosis‑associated speck‑like protein containing a CARD (ASC) and pro‑caspase …
Clinical, Immunological, and Genetic Features in Patients with NFKB1 and NFKB2 Mutations: a Systematic Review
Background Inborn errors of immunity (IEIs) encompass various diseases with diverse
clinical and immunological symptoms. Determining the genotype–phenotype of different …
clinical and immunological symptoms. Determining the genotype–phenotype of different …