Monogenic autoinflammatory disorders: conceptual overview, phenotype, and clinical approach
PA Nigrovic, PY Lee, HM Hoffman - Journal of Allergy and Clinical …, 2020 - Elsevier
Autoinflammatory diseases are conditions in which pathogenic inflammation arises primarily
through antigen-independent hyperactivation of immune pathways. First recognized just …
through antigen-independent hyperactivation of immune pathways. First recognized just …
Hematopoietic stem cell transplantation in primary immunodeficiency diseases: current status and future perspectives
Primary immunodeficiencies (PID) are disorders that for the most part result from mutations
in genes involved in immune host defense and immunoregulation. These conditions are …
in genes involved in immune host defense and immunoregulation. These conditions are …
Molecular mechanisms of phenotypic variability in monogenic autoinflammatory diseases
I Aksentijevich, O Schnappauf - Nature Reviews Rheumatology, 2021 - nature.com
Monogenic autoinflammatory diseases are a group of rheumatologic disorders caused by
dysregulation in the innate immune system. The molecular mechanisms of these disorders …
dysregulation in the innate immune system. The molecular mechanisms of these disorders …
Actin remodeling defects leading to autoinflammation and immune dysregulation
A growing number of monogenic immune-mediated diseases have been related to genes
involved in pathways of actin cytoskeleton remodeling. Increasing evidences associate …
involved in pathways of actin cytoskeleton remodeling. Increasing evidences associate …
Loss of ARPC1B impairs cytotoxic T lymphocyte maintenance and cytolytic activity
LO Randzavola, K Strege, M Juzans… - The Journal of …, 2019 - Am Soc Clin Investig
CD8 cytotoxic T lymphocytes (CTLs) rely on rapid reorganization of the branched F-actin
network to drive the polarized secretion of lytic granules, initiating target cell death during …
network to drive the polarized secretion of lytic granules, initiating target cell death during …
Formation of neutrophil extracellular traps requires actin cytoskeleton rearrangements
EGG Sprenkeler, ATJ Tool, SSV Henriet… - Blood, The Journal …, 2022 - ashpublications.org
Neutrophils are important effector cells in the host defense against invading
microorganisms. One of the mechanisms they use to eliminate pathogens is the release of …
microorganisms. One of the mechanisms they use to eliminate pathogens is the release of …
Moving towards a systems-based classification of innate immune-mediated diseases
S Savic, EA Caseley, MF McDermott - Nature Reviews Rheumatology, 2020 - nature.com
Autoinflammation as a distinct disease category was first reported in 1999 as a group of
monogenic disorders characterized by recurrent episodes of systemic and organ-specific …
monogenic disorders characterized by recurrent episodes of systemic and organ-specific …
Actin dynamics at the T cell synapse as revealed by immune-related actinopathies
The actin cytoskeleton is composed of dynamic filament networks that build adaptable local
architectures to sustain nearly all cellular activities in response to a myriad of stimuli …
architectures to sustain nearly all cellular activities in response to a myriad of stimuli …
Inborn errors of immunity manifesting as atopic disorders
M Vaseghi-Shanjani, KL Smith, RJ Sara… - Journal of Allergy and …, 2021 - Elsevier
Inborn errors of immunity are traditionally best known for enhancing susceptibility to
infections. However, allergic inflammation, among other types of immune dysregulation …
infections. However, allergic inflammation, among other types of immune dysregulation …
Identification of tumor antigens and immune subtypes of glioblastoma for mRNA vaccine development
The use of vaccines for cancer therapy is a promising immunotherapeutic strategy that has
been shown to be effective against various cancers. Vaccines directly target tumors but their …
been shown to be effective against various cancers. Vaccines directly target tumors but their …