The pyrin inflammasome in health and disease
The pyrin inflammasome has evolved as an innate immune sensor to detect bacterial toxin-
induced Rho guanosine triphosphatase (Rho GTPase)-inactivation, a process that is similar …
induced Rho guanosine triphosphatase (Rho GTPase)-inactivation, a process that is similar …
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 …
S100A8/A9 is a marker for the release of neutrophil extracellular traps and induces neutrophil activation
EGG Sprenkeler, J Zandstra, ND van Kleef… - Cells, 2022 - mdpi.com
Neutrophils are the most abundant innate immune cells in the circulation and they are the
first cells recruited to sites of infection or inflammation. Almost half of the intracellular protein …
first cells recruited to sites of infection or inflammation. Almost half of the intracellular protein …
Whole-genome sequencing of a sporadic primary immunodeficiency cohort
Primary immunodeficiency (PID) is characterized by recurrent and often life-threatening
infections, autoimmunity and cancer, and it poses major diagnostic and therapeutic …
infections, autoimmunity and cancer, and it poses major diagnostic and therapeutic …
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 …
Dynamic transcriptome-proteome correlation networks reveal human myeloid differentiation and neutrophil-specific programming
AJ Hoogendijk, F Pourfarzad, CEM Aarts, ATJ Tool… - Cell reports, 2019 - cell.com
Human neutrophilic granulocytes form the largest pool of innate immune cells for host
defense against bacterial and fungal pathogens. The dynamic changes that accompany the …
defense against bacterial and fungal pathogens. The dynamic changes that accompany the …
T-cell defects in patients with ARPC1B germline mutations account for combined immunodeficiency
I Brigida, M Zoccolillo, MP Cicalese… - Blood, The Journal …, 2018 - ashpublications.org
ARPC1B is a key factor for the assembly and maintenance of the ARP2/3 complex that is
involved in actin branching from an existing filament. Germline biallelic mutations in …
involved in actin branching from an existing filament. Germline biallelic mutations in …
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 …
CLL/lymphoma 10 (BCL10)—MALT1 paracaspase (MALT1)[CBM] signalosome complex …
The formation and function of the neutrophil phagosome
E Naish, AJT Wood, AP Stewart… - Immunological …, 2023 - Wiley Online Library
Neutrophils are the most abundant circulating leukocyte and are crucial to the initial innate
immune response to infection. One of their key pathogen‐eliminating mechanisms is …
immune response to infection. One of their key pathogen‐eliminating mechanisms is …
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 …