NF-κB essential modifier (NEMO), also known as IKK-γ, is a member of the I-κB kinase complex responsible for phosphorylating I-κB, allowing the release and activation of NF-κB. Boys with an expressed NEMO mutation have an X-linked syndrome characterized by hypohidrotic ectodermal dysplasia with immune deficiency (HED-ID). The immunophenotype resulting from NEMO mutation is highly variable, with deficits in both T and B cell responses. We evaluated three patients with NEMO mutations (L153R, Q403X, and C417R) and HED-ID who had evidence of defective CD40 signaling. All three patients had normal percentages of peripheral blood NK cells, but impaired NK cell cytotoxic activity. This was not due to a generalized defect in cytotoxicity because antibody-dependent cellular cytotoxicity was intact. This abnormality was partially reversed by in vitro addition of IL-2, which was also able to induce NF-κB activation. In one patient with recurrent cytomegalovirus infections, administration of IL-2 partially corrected the NK cell killing deficit. These data suggest that NEMO participates in signaling pathways leading to NK cell cytotoxicity and that IL-2 can activate NF-κB and partially overcome the NK cell defect in patients with NEMO mutations

NF-κB essential modifier (NEMO), also known as IKK-γ, is a member of the I-κB kinase complex responsible for phosphorylating I-κB, allowing the release and activation of NF-κB. Boys with an expressed NEMO mutation have an X-linked syndrome characterized by hypohidrotic ectodermal dysplasia with immune deficiency (HED-ID). The immunophenotype resulting from NEMO mutation is highly variable, with deficits in both T and B cell responses. We evaluated three patients with NEMO mutations (L153R, Q403X, and C417R) and HED-ID who had evidence of defective CD40 signaling. All three patients had normal percentages of peripheral blood NK cells, but impaired NK cell cytotoxic activity. This was not due to a generalized defect in cytotoxicity because antibody-dependent cellular cytotoxicity was intact. This abnormality was partially reversed by in vitro addition of IL-2, which was also able to induce NF-κB activation. In one patient with recurrent cytomegalovirus infections, administration of IL-2 partially corrected the NK cell killing deficit. These data suggest that NEMO participates in signaling pathways leading to NK cell cytotoxicity and that IL-2 can activate NF-κB and partially overcome the NK cell defect in patients with NEMO mutations.

The supernatant fluid of axenically grown E. histolytica inhibits chemotaxis, chemokinesis and random mobility of human mononuclear phagocytes (MP) as measured in Boyden chambers. Human polymorphonuclear phagocytes (PMN) locomotion is apparently unaffected. The factor was found in comparable amounts in the supernatant fluid of axenic cultures of four E. histolytica strains that differed in their human pathogenicity and virulence, as well as in two entamoebas non-pathogenic for man. This dialysable and thermolabile MP-locomotion inhibiting entamoeba product (EP) can be absorbed out by incubation with MP, but not with lymphocytes, while partial absorption was observed using PMN. The MP-locomotion inhibitory effect of this EP was cancelled by inhibiting protein synthesis in the MP by means of cycloheximide. In vivo, this EP caused a delay in MP migration in Rebuck skin windows. The molecular weight of this EP lies between 478 and 765 by gel-sieve chromatography. Our results suggest a direct effect upon the cytoskeletal and locomotive apparatus of the MP. This MP-locomotion inhibiting EP could contribute to the paucity of the inflammatory reaction observed in the advanced stages of invasive amoebiasis and consequently also to the lack of scar tissue formation upon healing of amoebic lesions.

Objective: Only a few studies have investigated variations of different markers for inflammatory processes during the physiological menstrual cycle. The results are conflicting, particularly concerning the correlation between the marker leptin and steroid hormones. The aim of the study was to investigate the inflammatory markers C-reactive protein (CRP) and leptin in the serum of healthy, normally ovulating women and to correlate these with each other and with the hormones of the gonadal axis. A cycle-dependence of the markers studied would imply an exact timing of the blood sampling for clinical needs. Design: Observational study investigating the two inflammatory markers CRP and leptin in relation to the hormonal pattern of the gonadal axis during the normal cycle. Methods: Ovulatory cycles of 36 healthy, young, normo-androgenic women, having a normal body mass index were evaluated. Serum concentrations of leptin and CRP, as well as of follicle-stimulating hormone, luteinising hormone, 17b-oestradiol, progesterone, prolactin (PRL) and free testosterone were measured every 1-2 days during one full cycle. Results: Serum levels of leptin and CRP behaved differently during ovulatory cycles, with higher concentrations for leptin only during certain phases. Significant correlations were found in the follicular phase between leptin and PRL and leptin and free testosterone. Conclusions: Leptin levels change during the menstrual cycle. Leptin levels are more stable on cycle days 1-5 than later in the cycle. For precise cycle-independent measurements, these fluctuations have to be taken into account. There is no similar cyclic pattern for CRP.European Journal of Endocrinology 155 137-142

Leptin and C-reactive protein levels change significantly during assisted reproductive treatment. In contrast to estradiol they are, however, not a marker of success.