T Cell CD3ζ Deficiency Enables Multiorgan Tissue Inflammation

T cell CD3ζ deficiency enables multiorgan tissue inflammation.

Although a population of T cells with CD3ζ chain deficiency has been found in patients with systemic lupus erythematosus, rheumatoid arthritis, cancer, and infectious disease, the role of CD3ζ chain in the disease pathogenesis remains unknown. To understand the contribution of CD3ζ deficiency to the expression of organ injury, we have performed the following studies. We used CD3ζ-deficient mice...

T Cell CD3z Deficiency Enables Multiorgan Tissue Inflammation

TBNK Severe Combined Immunodeficiency Caused by Complete Deficiency of the CD3ζ Subunit of the T Cell Antigen Receptor Complex Running Title: Human SCID Due to Complete CD3ζ Deficiency

CD3ζ is a subunit of the T cell antigen receptor (TCR) complex required for its assembly and surface expression that also plays an important role in TCR-mediated signal transduction. We report here a patient with TBNK severe combined immunodeficiency (SCID) who was homozygous for a single C insertion following nucleotide 411 in exon 7 of the CD3ζ gene. The few T cells present contained no detec...

Multiorgan Inflammation Effector T and B Cell Responses Leading to Results in Unbalanced Regulatory and Deficiency of MALT1 Paracaspase Activity

Dhavalkumar D. Patel and Thomas Calzascia Catherine H. Régnier, Tak W. Mak, Pamela S. Ohashi, Natasa Zamurovic, Kathy D. McCoy, Antonius Rolink, Traggiai, Dirk Brenner, Anne Brüstle, Michael St. Paul, Weckbecker, Gerhard Zenke, Bernd Kinzel, Elisabetta Katopodis, Barbara Nuesslein-Hildesheim, Gisbert Christoph Burkhart, Janet Dawson, Satoru Niwa, Andreas Paul A. Smith, Marc Bigaud, Ursula Junke...

Neutrophil myeloperoxidase regulates T-cell-driven tissue inflammation in mice by inhibiting dendritic cell function.

Myeloperoxidase (MPO) is important in intracellular microbial killing by neutrophils but extracellularly causes tissue damage. Its role in adaptive immunity and T-cell-mediated diseases is poorly understood. Here, T-cell responses in lymph nodes (LNs) were enhanced by MPO deletion or in vivo inhibition, causing enhanced skin delayed-type hypersensitivity and antigen (Ag)-induced arthritis. Resp...