Overvoltage protection by point-plane spark gaps

In electron-beam-controlled discharge CO/sup 2/ lasers, such as those used in the Antares and Helios laser-fusion drivers at the Los Alamos Scientific Laboratory (LASL), protection needs to be provided against possible damage due to overvoltage. A passive (self-breakdown) point-plane spark gap has been developed and successfully used in the Helios power amplifiers which operate at voltages up to 300 kV. A gap of similar design is planned for use in the Antares power amplifiers which operate at 550 kV. These gaps must reliably hold off the normal discharge voltage, but break down with short delay if overvoltaged, diverting the discharge energy to a resistor. A prototype of the Antares gap has been built and is undergoing tests. Parameters being investigated include voltage polarity, gap spacing, gas composition, and gas pressure. Results of these measurements and the operational experience of the Helios gaps will be presented

Autosomal dominant pseudohypoaldosteronism type 1 with a novel splice site mutation in MR gene

<p>Abstract</p> <p>Background</p> <p>Autosomal dominant pseudohypoaldosteronism type 1 (PHA1) is a rare inherited condition that is characterized by renal resistance to aldosterone as well as salt wasting, hyperkalemia, and metabolic acidosis. Renal PHA1 is caused by mutations of the human mineralcorticoid receptor gene (<it>MR</it>), but it is a matter of debate whether <it>MR </it>mutations cause mineralcorticoid resistance via haploinsufficiency or dominant negative mechanism. It was previously reported that in a case with nonsense mutation the mutant mRNA was absent in lymphocytes because of nonsense mediated mRNA decay (NMD) and therefore postulated that haploinsufficiency alone can give rise to the PHA1 phenotype in patients with truncated mutations.</p> <p>Methods and Results</p> <p>We conducted genomic DNA analysis and mRNA analysis for familial PHA1 patients extracted from lymphocytes and urinary sediments and could detect one novel splice site mutation which leads to exon skipping and frame shift result in premature termination at the transcript level. The mRNA analysis showed evidence of wild type and exon-skipped RT-PCR products.</p> <p>Conclusion</p> <p>mRNA analysis have been rarely conducted for PHA1 because kidney tissues are unavailable for this disease. However, we conducted RT-PCR analysis using mRNA extracted from urinary sediments. We could demonstrate that NMD does not fully function in kidney cells and that haploinsufficiency due to NMD with premature termination is not sufficient to give rise to the PHA1 phenotype at least in this mutation of our patient. Additional studies including mRNA analysis will be needed to identify the exact mechanism of the phenotype of PHA.</p

Critical Points in the Management of Pseudohypoaldosteronism Type 1

Pseudohypoaldosteronism type 1 (PHA-1, MIM #264350) is caused by defective transepithelial sodium transport. Affected patients develop life-threatening neonatal-onset salt loss, hyperkalemia, acidosis, and elevated aldosterone levels due to end-organ resistance to aldosterone. In this report, we present a patient diagnosed as PHA-1 who had clinical and laboratory findings compatible with the diagnosis and had genetically proven autosomal recessive PHA-1. The patient received high doses of sodium supplementation and potassium-lowering therapies; however, several difficulties were encountered in the management of this case. The aim of this presentation was to point out the potential pitfalls in the treatment of such patients in the clinical practice and to recommend solutions

Exile Vol. XLV No. 2

43rd Year Title Page 3 Epigraph by Ezra Pound 5 Table of Contents 7 Contributors Notes 74-75 Editorial Board 76 INTERVIEWS The Art of Hearing: Interview with Stanley Plumly by Alison Stine \u2700 23-27 ART Self-Portrait by Angela Bliss \u2799 8 For a Living by Angela Bliss \u2799 12 Untitled by Frazier Taylor \u2702 22 Untitled by Amy Deaner \u2799 29 Perfect Knee by David Tulkin \u2701 34 Untitled by Amy Deaner \u2799 43 Still Light by Angela Bliss \u2799 62 Hiding Nature by Amy Deaner \u2799 64 Self-Portrait A by Sarah Leyrer \u2701 73 POETRY Bolted Back by Michelle Grindstaff \u2702 9 Squall by Georgia Riepe \u2702 10 Loaves and Fishes by Maeghan Demmons \u2701 11 World Cafe by Katie Kroner \u2701 28 Gurney Surfer by Tom Hankinson \u2702 31 Japanese Beetles by Alison Stine \u2700 32-33 Shoveling by Bekah Taylor \u2700 40 Tobacco Country by K. Moore \u2701 41 Winton Place by Rachel Colina \u2702 42 Bottom of the Ninth by Michelle Grindstaff \u2702 61 Fall Burning by Alison Stine \u2700 63 rocking by Bekah Taylor \u2700 71 The Armor of the Beach by Georgia Riepe \u2702 72 PROSE In the Aisles of the Night by Tom Dussel \u2701 13-21 From Those Uninvolved by Justin Walker \u2799 30 Frame by Paul Durica \u2700 35-39 The Rose by Rachel Bolton \u2799 44-60 Stop at the Soldier by Hillary Campbell \u2700 65-70 All submissions are reviewed on an anonymous basis, and all editorial decisions are shared equally among the members of the Editorial Board. -76 Cover Art Untitled by Kris Lewis \u2799 / Back Cover Art Figure 25 by Todd Gys \u2799 -76 Printed by Printing Arts Press -7

The USNO-B Catalog

USNO-B is an all-sky catalog that presents positions, proper motions, magnitudes in various optical passbands, and star/galaxy estimators for 1,042,618,261 objects derived from 3,643,201,733 separate observations. The data were obtained from scans of 7,435 Schmidt plates taken for the various sky surveys during the last 50 years. USNO-B1.0 is believed to provide all-sky coverage, completeness down to V = 21, 0.2 arcsecond astrometric accuracy at J2000, 0.3 magnitude photometric accuracy in up to five colors, and 85% accuracy for distinguishing stars from non-stellar objects. A brief discussion of various issues is given here, but the actual data are available from http://www.nofs.navy.mil and other sites.Comment: Accepted by Astronomical Journa

Using a Modelling Language to Describe the Quality of Life Goals of People Living with Dementia

Although now well established, our information systems engineering theories and methods are applied only rarely in disciplines beyond systems development. This paper reports the application of the i* goal modelling language to describe the types of and relationships between quality of life goals of people living with dementia. Published social care frameworks to manage and improve the lives of people with dementia were reviewed to synthesize, for the first time, a comprehensive conceptual model of the types of goals of people living with dementia. Although the quality of life goal model was developed in order to construct automated reasoning capabilities in a new digital toolset that people with dementia can use for life planning, the multi-stage modelling exercise provided valuable insights into quality of life and dementia care practices of both researchers and experienced practitioners in the field

Androgen Receptor Function Links Human Sexual Dimorphism to DNA Methylation

Sex differences are well known to be determinants of development, health and disease. Epigenetic mechanisms are also known to differ between men and women through X-inactivation in females. We hypothesized that epigenetic sex differences may also result from sex hormone functions, in particular from long-lasting androgen programming. We aimed at investigating whether inactivation of the androgen receptor, the key regulator of normal male sex development, is associated with differences of the patterns of DNA methylation marks in genital tissues. To this end, we performed large scale array-based analysis of gene methylation profiles on genomic DNA from labioscrotal skin fibroblasts of 8 males and 26 individuals with androgen insensitivity syndrome (AIS) due to inactivating androgen receptor gene mutations. By this approach we identified differential methylation of 167 CpG loci representing 162 unique human genes. These were significantly enriched for androgen target genes and low CpG content promoter genes. Additional 75 genes showed a significant increase of heterogeneity of methylation in AIS compared to a high homogeneity in normal male controls. Our data show that normal and aber

Aged PROP1 Deficient Dwarf Mice Maintain ACTH Production

Humans with PROP1 mutations have multiple pituitary hormone deficiencies (MPHD) that typically advance from growth insufficiency diagnosed in infancy to include more severe growth hormone (GH) deficiency and progressive reduction in other anterior pituitary hormones, eventually including adrenocorticotropic hormone (ACTH) deficiency and hypocortisolism. Congenital deficiencies of GH, prolactin, and thyroid stimulating hormone have been reported in the Prop1null (Prop1-/-) and the Ames dwarf (Prop1df/df) mouse models, but corticotroph and pituitary adrenal axis function have not been thoroughly investigated. Here we report that the C57BL6 background sensitizes mutants to a wasting phenotype that causes approximately one third to die precipitously between weaning and adulthood, while remaining homozygotes live with no signs of illness. The wasting phenotype is associated with severe hypoglycemia. Circulating ACTH and corticosterone levels are elevated in juvenile and aged Prop1 mutants, indicating activation of the pituitary-adrenal axis. Despite this, young adult Prop1 deficient mice are capable of responding to restraint stress with further elevation of ACTH and corticosterone. Low blood glucose, an expected side effect of GH deficiency, is likely responsible for the elevated corticosterone level. These studies suggest that the mouse model differs from the human patients who display progressive hormone loss and hypocortisolism