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Human CD14dim Monocytes Patrol and Sense Nucleic Acids and Viruses via TLR7 and TLR8 Receptors
Gene therapy of human severe combined immunodeficiency (SCID)-X1 disease.
A gene therapy trial for SCID-X1 was initiated, based on the use of complementary DNA containing a defective gammac Moloney retrovirus-derived vector and ex vivo infection of CD34+ cells, which provided full correction of disease phenotype and clinical benefit.
Autoantibodies against type I IFNs in patients with life-threatening COVID-19
A means by which individuals at highest risk of life-threatening COVID-19 can be identified is identified, and the hypothesis that neutralizing auto-Abs against type I IFNs may underlie critical CO VID-19 is tested.
Inborn errors of type I IFN immunity in patients with life-threatening COVID-19
The COVID Human Genetic Effort established to test the general hypothesis that life-threatening COVID-19 in some or most patients may be caused by monogenic inborn errors of immunity to SARS-CoV-2 with incomplete or complete penetrance finds an enrichment in variants predicted to be loss-of-function (pLOF), with a minor allele frequency <0.001.
TLR3 Deficiency in Patients with Herpes Simplex Encephalitis
Human TLR3 appears to be redundant in host defense to most microbes but is vital for natural immunity to HSV-1 in the CNS, which suggests that neurotropic viruses have contributed to the evolutionary maintenance ofTLR3.
Kawasaki-like multisystem inflammatory syndrome in children during the covid-19 pandemic in Paris, France: prospective observational study
An unusually high proportion of the affected children and adolescents had gastrointestinal symptoms, Kawasaki disease shock syndrome, and were of African ancestry in this study, suggesting that the ongoing outbreak of Kawasaki-like multisystem inflammatory syndrome in the Paris area might be related to SARS-CoV-2.
Primary Immunodeficiency Diseases: An Update on the Classification from the International Union of Immunological Societies Expert Committee for Primary Immunodeficiency
The updated classification of primary immunodeficiencies (PIDs) compiled by the Expert Committee of the International Union of Immunological Societies acts as a current reference of the knowledge of these conditions and is an important aid for the molecular diagnosis of patients with these rare diseases.
Autoantibodies against IL-17A, IL-17F, and IL-22 in patients with chronic mucocutaneous candidiasis and autoimmune polyendocrine syndrome type I
- A. Puel, R. Döffinger, J. Casanova
- Medicine, BiologyThe Journal of experimental medicine
- 15 February 2010
Findings suggest that auto-Abs against IL- 17A, IL-17F, and IL-22 may cause CMC in patients with APS-I.
B cell–helper neutrophils stimulate the diversification and production of immunoglobulin in the marginal zone of the spleen
Neutrophils around the marginal zone (MZ) of the spleen, a B cell area specialized in T cell–independent immunoglobulin responses to circulating antigen, are identified, which indicates that neutrophils generate an innate layer of antimicrobial immunoglOBulin defense by interacting with MZ B cells.
Interleukin-36-receptor antagonist deficiency and generalized pustular psoriasis.
Aberrant interleukin-36Ra structure and function lead to unregulated secretion of inflammatory cytokines and generalized pustular psoriasis.