The TNF family of ligands and receptors: communication modules in the immune system and beyond

C Dostert, M Grusdat, E Letellier… - Physiological …, 2019 - journals.physiology.org
The tumor necrosis factor (TNF) and TNF receptor (TNFR) superfamilies (TNFSF/TNFRSF)
include 19 ligands and 29 receptors that play important roles in the modulation of cellular …

Molecular basis and therapeutic implications of CD40/CD40L immune checkpoint

TT Tang, X Cheng, B Truong, LZ Sun, XF Yang… - Pharmacology & …, 2021 - Elsevier
The CD40 receptor and its ligand CD40L is one of the most critical molecular pairs of the
stimulatory immune checkpoints. Both CD40 and CD40L have a membrane form and a …

Practice parameter for the diagnosis and management of primary immunodeficiency

FA Bonilla, DA Khan, ZK Ballas, J Chinen… - Journal of Allergy and …, 2015 - Elsevier
The American Academy of Allergy, Asthma & Immunology (AAAAI) and the American
College of Allergy, Asthma & Immunology (ACAAI) have jointly accepted responsibility for …

Overview of immunodeficiency disorders

N Raje, C Dinakar - Immunology and Allergy Clinics, 2015 - immunology.theclinics.com
In the past, primary immunodeficiency disorders (PIDs) have been described as diseases
caused by 1 or more defects of the immune system, leading to increased susceptibility to …

The hyper IgM syndromes: Epidemiology, pathogenesis, clinical manifestations, diagnosis and management

R Yazdani, S Fekrvand, S Shahkarami, G Azizi… - Clinical …, 2019 - Elsevier
Abstract Hyper Immunoglobulin M syndrome (HIGM) is a rare primary immunodeficiency
disorder characterized by low or absent levels of serum IgG, IgA, IgE and normal or …

[HTML][HTML] ESCMID Study Group for Infections in Compromised Hosts (ESGICH) Consensus Document on the safety of targeted and biological therapies: an infectious …

L Drgona, C Gudiol, S Lanini, B Salzberger… - Clinical Microbiology …, 2018 - Elsevier
Background The present review is part of the ESCMID Study Group for Infections in
Compromised Hosts (ESGICH) Consensus Document on the safety of targeted and …

Modeling, optimization, and comparable efficacy of T cell and hematopoietic stem cell gene editing for treating hyper‐IgM syndrome

V Vavassori, E Mercuri, GE Marcovecchio… - EMBO Molecular …, 2021 - embopress.org
Precise correction of the CD40LG gene in T cells and hematopoietic stem/progenitor cells
(HSPC) holds promise for treating X‐linked hyper‐IgM Syndrome (HIGM1), but its actual …

Activating PI3Kδ mutations in a cohort of 669 patients with primary immunodeficiency

M Elgizouli, DM Lowe, C Speckmann… - Clinical & …, 2016 - academic.oup.com
The gene PIK3CD codes for the catalytic subunit of phosphoinositide 3-kinase δ (PI3K δ),
and is expressed solely in leucocytes. Activating mutations of PIK3CD have been described …

Congenital neutropenia and primary immunodeficiency diseases

J Spoor, H Farajifard, N Rezaei - Critical reviews in oncology/hematology, 2019 - Elsevier
Neutropenia is a dangerous and potentially fatal condition that renders patients vulnerable
to recurrent infections. Its severity is commensurate with the absolute count of neutrophil …

Hyper IgM syndrome: a report from the USIDNET registry

EA Leven, P Maffucci, HD Ochs, PR Scholl… - Journal of clinical …, 2016 - Springer
Abstract Purpose The United States Immunodeficiency Network (USIDNET) patient registry
was used to characterize the presentation, genetics, phenotypes, and treatment of patients …