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Human CD14dim Monocytes Patrol and Sense Nucleic Acids and Viruses via TLR7 and TLR8 Receptors
TLDR
CD14dim monocytes were weak phagocytes and did not produce ROS or cytokines in response to cell-surface Toll-like receptors, but selectively produced TNF-α, IL-1β, and CCL3 inresponse to viruses and immune complexes containing nucleic acids, via a proinflammatory TLR7-TLR 8-MyD88-MEK pathway. Expand
Gene therapy of human severe combined immunodeficiency (SCID)-X1 disease.
TLDR
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. Expand
TLR3 Deficiency in Patients with Herpes Simplex Encephalitis
TLDR
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. Expand
B cell–helper neutrophils stimulate the diversification and production of immunoglobulin in the marginal zone of the spleen
TLDR
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. Expand
Primary Immunodeficiency Diseases: An Update on the Classification from the International Union of Immunological Societies Expert Committee for Primary Immunodeficiency
TLDR
This updated classification of primary immunodeficiency diseases, compiled by the ad hoc Expert Committee of the International Union of Immunological Societies, is meant to help in the diagnostic approach to patients with these diseases. Expand
Autoantibodies against IL-17A, IL-17F, and IL-22 in patients with chronic mucocutaneous candidiasis and autoimmune polyendocrine syndrome type I
TLDR
Findings suggest that auto-Abs against IL- 17A, IL-17F, and IL-22 may cause CMC in patients with APS-I. Expand
Gain-of-function human STAT1 mutations impair IL-17 immunity and underlie chronic mucocutaneous candidiasis
Whole-exome sequencing reveals activating STAT1 mutations in some patients with autosomal dominant chronic mucocutaneous candidiasis disease.
Interleukin-36-receptor antagonist deficiency and generalized pustular psoriasis.
TLDR
Aberrant interleukin-36Ra structure and function lead to unregulated secretion of inflammatory cytokines and generalized pustular psoriasis. Expand
Evolutionary Dynamics of Human Toll-Like Receptors and Their Different Contributions to Host Defense
TLDR
It is found that the intracellular TLRs—activated by nucleic acids and particularly specialized in viral recognition—have evolved under strong purifying selection, indicating their essential non-redundant role in host survival. Expand
Primary Immunodeficiency Diseases: An Update on the Classification from the International Union of Immunological Societies Expert Committee for Primary Immunodeficiency
TLDR
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. Expand
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