[HTML][HTML] Loss-of-function nuclear factor κB subunit 1 (NFKB1) variants are the most common monogenic cause of common variable immunodeficiency in Europeans
Background The genetic cause of primary immunodeficiency disease (PID) carries prognostic
information. Objective We conducted a whole-genome sequencing study assessing a …
information. Objective We conducted a whole-genome sequencing study assessing a …
Combined immunodeficiency with severe inflammation and allergy caused by ARPC1B deficiency
TW Kuijpers, ATJ Tool, I van der Bijl… - Journal of Allergy and …, 2017 - jacionline.org
2. Feldman RE, Lam AC, Sadow PM, Bleier BS. P-glycoprotein is a marker of tissue eosinophilia
and radiographic inflammation in chronic rhinosinusitis without nasal polyps. Int Forum …
and radiographic inflammation in chronic rhinosinusitis without nasal polyps. Int Forum …
Proteomic screen identifies IGFBP7 as a novel component of endothelial cell-specific Weibel-Palade bodies
D Van Breevoort, EL van Agtmaal… - Journal of proteome …, 2012 - ACS Publications
Vascular endothelial cells contain unique storage organelles, designated Weibel-Palade
bodies (WPBs), that deliver inflammatory and hemostatic mediators to the vascular lumen in …
bodies (WPBs), that deliver inflammatory and hemostatic mediators to the vascular lumen in …
Activated neutrophils exert myeloid-derived suppressor cell activity damaging T cells beyond repair
CEM Aarts, IH Hiemstra, EP Béguin… - Blood …, 2019 - ashpublications.org
Myeloid-derived suppressor cells (MDSCs) have the capacity to suppress T-cell–mediated
immune responses and impact the clinical outcome of cancer, infections, and transplantation …
immune responses and impact the clinical outcome of cancer, infections, and transplantation …
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 …
A combined immunodeficiency with severe infections, inflammation, and allergy caused by ARPC1B deficiency
S Volpi, MP Cicalese, P Tuijnenburg… - Journal of Allergy and …, 2019 - jacionline.org
To the Editor: Recently, a novel syndrome of combined immunodeficiency, allergy, and ‘‘auto’’inflammation
caused by mutations in the ARPC1B gene has been reported. 1-4 Analysis of …
caused by mutations in the ARPC1B gene has been reported. 1-4 Analysis of …
Potassium leakage primes stored erythrocytes for phosphatidylserine exposure and shedding of pro‐coagulant vesicles
P Burger, E Kostova, E Bloem… - British journal of …, 2013 - Wiley Online Library
During storage, erythrocytes undergo changes that alter their clearance and function after
transfusion and there is increasing evidence that these changes contribute to the …
transfusion and there is increasing evidence that these changes contribute to the …
Hemolysis in the spleen drives erythrocyte turnover
TRL Klei, J Dalimot, B Nota, M Veldthuis… - Blood, The Journal …, 2020 - ashpublications.org
Red pulp macrophages (RPMs) of the spleen mediate turnover of billions of senescent
erythrocytes per day. However, the molecular mechanisms involved in sequestration of …
erythrocytes per day. However, the molecular mechanisms involved in sequestration of …
Uptake of blood coagulation factor VIII by dendritic cells is mediated via its C1 domain
BACKGROUND: The uptake and processing of blood coagulation factor VIII (FVIII) by
antigen-presenting cells and the subsequent presentation of FVIII-derived peptides to CD4 + T …
antigen-presenting cells and the subsequent presentation of FVIII-derived peptides to CD4 + T …
HLA-DR-presented peptide repertoires derived from human monocyte-derived dendritic cells pulsed with blood coagulation factor VIII
Activation of T-helper cells is dependent upon the appropriate presentation of antigen-derived
peptides on MHC class II molecules expressed on antigen presenting cells. In the current …
peptides on MHC class II molecules expressed on antigen presenting cells. In the current …