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Autosomal dominant periodic fever syndromes are characterized by unexplained episodes of fever and severe localized inflammation. In seven affected families, we found six different missense mutations of the 55 kDa tumor necrosis factor receptor (TNFR1), five of which disrupt conserved extracellular disulfide bonds. Soluble plasma TNFR1 levels in patients(More)
Antibodies to glutamic acid decarboxylase (GAD) are found in about 40% of patients with stiff man syndrome. A new assay involving immunoprecipitation of (125)I-glutamic acid decarboxylase was used to measure anti-GAD antibodies in 18 patients with stiff man syndrome. Of the eight serum samples from patients with stiff man syndrome, that had previously been(More)
BACKGROUND We have previously shown that NK (CD56+CD3-) and NKT-like (CD56+CD3+) cells are reduced in both numbers and cytotoxicity in peripheral blood. The aim of the present study was to investigate their numbers and function within induced sputum. METHODS Induced sputum cell numbers and intracellular granzyme B and perforin were analysed by flow(More)
BACKGROUND There is mounting evidence that perforin and granzymes are important mediators in the lung destruction seen in COPD. We investigated the characteristics of the three main perforin and granzyme containing peripheral cells, namely CD8+ T lymphocytes, natural killer (NK; CD56+CD3-) cells and NKT-like (CD56+CD3+) cells. METHODS Peripheral blood(More)
BACKGROUND CD8(+) T-lymphocytes, natural killer T-like cells (NKT-like cells, CD56(+)CD3(+)) and natural killer cells (NK cells, CD56(+)CD3(-)) are the three main classes of human killer cells and they are implicated in the pathogenesis of chronic obstructive pulmonary disease (COPD). Activation of these cells can initiate immune responses by virtue of(More)
Mutations in TNFRSF1A encoding TNF receptor 1 (TNFR1) cause the autosomal dominant TNF receptor-associated periodic syndrome (TRAPS): a systemic autoinflammatory disorder. Misfolding, intracellular aggregation, and ligand-independent signaling by mutant TNFR1 are central to disease pathophysiology. Our aim was to understand the extent of signaling pathway(More)
Autoimmunity may contribute to the pathogenesis of chronic obstructive pulmonary disease (COPD). Studies have identified disease-specific autoantibodies (DSAAbs) in COPD patients, but natural autoantibodies (NAAbs) may also play a role. Previous studies have concentrated on circulating autoantibodies, but lung-associated autoantibodies may be most(More)
The Rapid Temporal Survey (RATS) explores the faint, variable sky. Our observations search a parameter space which, until now, has never been exploited from the ground. Our strategy involves observing the sky close to the Galactic plane with wide-field CCD cameras. An exposure is obtained approximately every minute with the total observation of each field(More)
Biomarkers, including cytokines, can help in the diagnosis, prognosis, and prediction of treatment response across a wide range of disease settings. Consequently, the recent emergence of protein microarray technology, which is able to quantify a range of inflammatory mediators in a large number of samples simultaneously, has become highly desirable.(More)