System level design of the ITER bolometer port plug cameras.

The ITER bolometer diagnostic is planned to have 550 lines of sight (LOS) distributed all over the vessel. 240 channels are provided by cameras mounted in two upper ports and in one equatorial port. This paper describes the current status of the system level design of the port cameras and the solutions proposed on how to implement all required camera components while meeting a multitude of competing requirements. Sensor holders, support structures, and different apertures depending on the camera type (pinhole or collimator), cable connectors, ceramic track plates, and many mineral insulated cables have to be integrated within a restricted space envelope to guarantee functionality. The design of the internal electrical interfaces and the external mechanical mountings will be described as well. Using the example of an upper port camera with 60 LOS, the assembly of the camera components is explained and two currently discussed architecture options for the remote handling maintenance scheme in the hot cell are compared.

Complete coding sequence, deduced primary structure, chromosomal localization, and structural analysis of murine aggrecan.

We have isolated and sequenced overlapping cDNA clones encoding the entire core protein of aggrecan (the large aggregating chondroitin sulfate/keratan sulfate proteoglycan of cartilage) from three chondrocyte cDNA libraries of BALB/c mice and localized the aggrecan gene in mouse chromosome 7. We determined 7386 bp of the cDNA sequence, including 132 and 854 nucleotides of 5' and 3' untranslated regions, respectively. The core protein precursor is 2132 amino acids long (M(r) 222,008), including a 19-residue secretory signal peptide. The overall amino acid sequence of the mouse aggrecan shows 91.6% identity to rat and 72.5% to human aggrecan. Comparison of the amino acid sequences of various domains and subdomain structures of mouse aggrecan to known sequences of other species and related proteins (versican, neurocan, link protein, and lymphocyte homing receptor CD44) revealed high levels of identity of the G1, G2, and G3 globular domains and relatively less conserved structures in the interglobular and glycosaminoglycan-attachment regions. Epidermal growth factor (EGF)-like module was detected in only a minor fraction of aggrecan clones, while the complement regulatory protein (CRP)-like domain was regularly expressed in all samples.

Expression of alternatively spliced epidermal growth factor-like domains in aggrecans of different species. Evidence for a novel module.

The carboxyl-terminal globular domain of human aggrecan has been shown previously to contain an alternatively spliced epidermal growth factor (EGF)-like module. We have used reverse transcription/polymerase chain reactions on cartilage-derived RNAs to investigate the heterogeneity in the EGF-like domain content of aggrecans from five species (mouse, rat, dog, bovine, and human). A novel alternatively spliced EGF-like module was detected in human aggrecan, establishing the presence of two of these domains. Highly homologous domains are present in aggrecans of other species, and the expression of these modules is identical (4-8%). They share significant homology with EGF-like domains of differentiation proteins and coagulation factors and have a putative calcium binding site. In contrast to this novel domain (EGF2), the previously described EGF-like module (EGF1) is expressed at a high level exclusively in human aggrecan. The expression of the two EGF-like domains in human aggrecan appears to be independent. Although the function of these domains is not understood, the uniform expression of the EGF2 domain may indicate a general role of this aggrecan module, while the expression of the EGF1 domain may reflect species specificity.

[Hyperimmunoglobulinemia E (Job) syndrome]. / Hyperimmunoglobulinaemia E (Jób) szindróma.

A patient with hyperimmunoglobulin E (Job's) syndrome is presented. The authors review the clinical and immunological characteristics of the disease and sum up the different explanations for the pathogenesis of the syndrome.

[Leukocyte migration inhibition test in celiac disease]. / A leukocyta migráció gátlás vizsgálata coeliakiában.

The leukocyte migration inhibition test is a method used to assess the cell-mediated immune function. The authors examined 251 samples for a period of 3 years; 169 samples from celiac patients and 82 were control. The sensitivity of this test was 34/35 (97%) in proved gluten sensitive patients, but this was found after repeating the test at different periods of time. According to these results the authors conclude that the efficacy of this test is less sensitive in the newly diagnosed celiac patients, which means that it is not suitable for screening purpose, but useful for detecting gliadin sensitivity during the diagnostic period of celiac disease (e. i. the 3 biopsies). LMT may be used to indicate the proper time for the 2nd and/or 3rd biopsy, and can also be used to reveal the gluten-free diet defaults. The authors agree with those who believe that this test cannot substitute the performance of the small intestinal biopsy.

Hormonal regulation of complement biosynthesis in human cell lines--II. Upregulation of the biosynthesis of complement components C3, factor B and C1 inhibitor by interleukin-6 and interleukin-1 in human hepatoma cell line.

The effect of interleukin (IL)-6 and IL-1 on the biosynthesis of complement components C3, factor B, C2, C4 and C1 inhibitor (C1 inh), as well as that of albumin, was studied in vitro in human hepatoma-derived cell line, HepG2. Measuring the amounts of secreted complement proteins we detected a significant upregulation of C3 by both hormones. The enhancement of the factor B and especially that of C1 inh production was predominant by IL-6. In our experimental system neither IL-1 nor IL-6 affected the biosynthesis of C2 and C4. Albumin secretion was significantly decreased only in the simultaneous presence of IL-1 and IL-6. Detection of the changes in the amounts of C3- and factor B-specific mRNA of HepG2 cells suggests a pretranslational regulation by these cytokines. The secretion of C3 and factor B was markedly potentiated when IL-1 and IL-6 were added together. However only the gene expression of factor B, but not of C3, was found to reveal synergism. IL-6 enhanced the in vitro production of C3 in mouse hepatocytes as well. This effect was greatly potentiated in the presence of histamine.

Hormonal regulation of complement biosynthesis in human cell lines--I. Androgens and gamma-interferon stimulate the biosynthesis and gene expression of C1 inhibitor in human cell lines U937 and HepG2.

C1 inhibitor (C1inh), a member of the serine protease inhibitor gene superfamily, is a glycosylated plasma protein inhibiting the proteolytic activities of C1r and C1s and involved in the regulation of coagulation, fibrinolysis and kinin-releasing systems. In this study, the in vitro effect of androgen hormones, dehydroepiandrosterone (DHEA), testosterone (TEST) and recombinant human gamma-interferon (gamma-IFN), has been determined on the production of C1inh in human cell lines. In both human monocytoid/histiocytoid cell line U937 and in hepatoma derived cell line HepG2, DHEA and TEST upregulated the gene expression and secretion of C1inh. The most pronounced effect was detected in the concn range 10(-7)-10(-9) M of the hormones. Under the same conditions DHEA and TEST had no detectable effect on the biosynthesis of C3, C2 and factor B by these cells, but DHEA at higher concn (10(-4) M) slightly increased that of C4 in HepG2 cells. Both in U937 and in HepG2 cells recombinant gamma-IFN markedly increased the gene expression and secretion of C1inh. This effect of gamma-IFN was abolished by histamine.

Unequal expression of complement C4A and C4B genes in rheumatoid synovial cells, human monocytoid and hepatoma-derived cell lines.

C4A and C4B are closely related homologous complement proteins encoded in the class III region of major histocompatibility complex (MHC). The regulation of their expression is under genetic and hormonal control. In this study we investigated the synovial fluid plasma ratio of C4A and C4B of rheumatoid (RA) and osteoarthritis (OA) patients, and a predominance of the C4B gene expression by the synovial macrophages of RA patients was demonstrated. To clarify the tissue specificity of the expression of C4A and C4B genes, human monocytoid cell line U937 and hepatoma-derived HepG2 cells were studied. The gene expression of C4A and C4B were markedly different in these cells since a relative predominance of C4B mRNA in U937 cells and excess of that of C4A in HepG2 cells were detected. Recombinant interferon-gamma (IFN-gamma) up-regulated the expression of C4A gene in both cells, but had apparently no effect on the C4B gene. Our results demonstrate dissimilar expression patterns for the two human C4 genes, suggesting different tissue specific regulation of human C4A and C4B.

Stimulation of histamine receptors of human monocytoid and hepatoma-derived cell lines and mouse hepatocytes modulates the production of the complement components C3, C4, factor B, and C2.

The influence of histamine (and the related agonists and antagonists) alone or in the presence of recombinant human interleukin 1 alpha (IL-1 alpha) and gamma interferon (IFN-gamma) was studied on the production of complement components C3, C2, factor B, and C4 in vitro with human monocytoid cell line U937, hepatoma-derived cell line HepG2, and mouse hepatocytes. Both U937 and HepG2 cells responded to histamine through H1 and H2 histamine receptors. The effect of histamine on the biosynthesis and gene expression of complement proteins was predominantly enhancing via the H1 histamine receptors and inhibitory through the H2 receptors. The actual predominance of the histamine receptor involved (and the outcome of the ligand interaction) seemed to be greatly affected by the simultaneous activation of the cells by IL-1 or IFN-gamma.

Effect of histamine on the T-cell colony formation of PHA-stimulated cells.

The effect of histamine on T-cell colony formation was studied in human peripheral blood mononuclear cells. Histamine inhibited dose-dependently (10(-4)-10(-6) M) the colony formation of PHA-stimulated T-cells. The inhibition was similar in normal controls and rheumatoid arthritis (RA) patients in spite of the fact that in RA the colony formation was significantly lower than in the normal controls. No increase of colony formation was observed at low concentrations (less than 10(-7) M). Impromidine was less effective than histamine, and pyridylethylamine (PEA) was inactive. Cimetidine counteracted the effect of histamine while chlorpheniramine did not. The results show that colony formation may be inhibited through H2-receptors. This action may be of importance in cellular interactions in tissues with high local histamine concentrations.

Effect of two synthetic alpha-gliadin peptides on lymphocytes in celiac disease: identification of a novel class of opioid receptors.

Two synthetic peptides containing residues 43-47 and 43-49 of alpha-gliadin were tested for inhibition of leukocyte migration in 47 patients with celiac disease. In nineteen patients, all on a normal diet, leukocyte migration was inhibited by the peptides and naloxone blocked this effect. In twenty-eight patients (24 of whom were on strict gluten-free diet) leukocyte migration was not affected by the peptides. Our results suggest that alpha-gliadin-(43-49), Tyr-Pro-Gln-Pro-Gln-Pro-Phe, is closely related to the active fragment, or to one of the active fragments of alpha-gliadin, and that it interacts with receptors that are similar to but not identical with the known opiate receptors.

Prognostic factors in acute lymphoid leukaemia of childhood. II. Cell surface markers.

Monoclonal sera have been used to determine the surface phaenotype of leukaemic cells during the last three years. Bone-marrow specimens of 57 children with recently diagnosed acute lymphoid leukaemia were examined; four cases were classified as T-cell leukaemia, 2 cases as B-cell leukaemia, in 37 cases cALLa was positive and fourteen children were classified as O-cell type, based on the absence of markers. Analysis of symptom-free survival revealed a very poor prognosis in B-cell leukaemia; there was no significant difference between the remaining groups. Within the cALLa positive cases L1 exhibited a markedly more favourable prognosis than L2.

Naloxone antagonises effect of alpha-gliadin on leucocyte migration in patients with coeliac disease.

The effect of naloxone on inhibition of leucocyte migration by alpha-gliadin was examined in 24 patients with coeliac disease. In all cases in which alpha-gliadin inhibited leucocyte migration, naloxone blocked this inhibitory effect, which suggests that the effect of gliadin on lymphocytes from patients with coeliac disease may be mediated through opioid-like receptors.