Sample records for hgo mutational hot

  1. Whole-genome sequencing in autism identifies hot spots for de novo germline mutation

    DEFF Research Database (Denmark)

    Michaelson, Jacob J.; Shi, Yujian; Gujral, Madhusudan


    De novo mutation plays an important role in autism spectrum disorders (ASDs). Notably, pathogenic copy number variants (CNVs) are characterized by high mutation rates. We hypothesize that hypermutability is a property of ASD genes and may also include nucleotide-substitution hot spots. We...

  2. One-step green synthesis and characterization of plant protein-coated mercuric oxide (HgO) nanoparticles: antimicrobial studies (United States)

    Das, Amlan Kumar; Marwal, Avinash; Sain, Divya; Pareek, Vikram


    The present study demonstrates the bioreductive green synthesis of nanosized HgO using flower extracts of an ornamental plant Callistemon viminalis. The flower extracts of Callistemon viminalis seem to be environmentally friendly, so this protocol could be used for rapid production of HgO. Till date, there is no report of synthesis of nanoparticles using flower extract of Callistemon viminalis. Mercuric acetate was taken as the metal precursor in the present experiment. The flower extract was found to act as a reducing as well as a stabilizing agent. The phytochemicals present in the flower extract act as reducing agent which include proteins, saponins, phenolic compounds, phytosterols, and flavonoids. FT-IR spectroscopy confirmed that the extract had the ability to act as a reducing agent and stabilizer for HgO nanoparticles. The formation of the plant protein-coated HgO nanoparticles was first monitored using UV-Vis absorption spectroscopy. The UV-Vis spectroscopy revealed the formation of HgO nanoparticles by exhibiting the typical surface plasmon absorption maxima at 243 nm. The average particle size formed ranges from 2 to 4 nm. The dried form of synthesized nanoparticles was further characterized using TGA, XRD, TEM, and FTIR spectroscopy. FT-IR spectra of synthesized HgO nanoparticles were performed to identify the possible bio-molecules responsible for capping and stabilization of nanoparticles, which confirm the formation of plant protein-coated HgO nanoparticles that is further corroborated by TGA study. The optical band gap of HgO nanoparticle was measured to be 2.48 eV using cutoff wavelength which indicates that HgO nanoparticles can be used in metal oxide semiconductor-based photovoltaic cells. A possible core-shell structure of the HgO nanobiocomposite has been proposed.

  3. Lack of mutational hot spots during decitabine-mediated HIV-1 mutagenesis. (United States)

    Rawson, Jonathan M O; Landman, Sean R; Reilly, Cavan S; Bonnac, Laurent; Patterson, Steven E; Mansky, Louis M


    Decitabine has previously been shown to induce lethal mutagenesis of human immunodeficiency virus type 1 (HIV-1). However, the factors that determine the susceptibilities of individual sequence positions in HIV-1 to decitabine have not yet been defined. To investigate this, we performed Illumina high-throughput sequencing of multiple amplicons prepared from proviral DNA that was recovered from decitabine-treated cells infected with HIV-1. We found that decitabine induced an ≈4.1-fold increase in the total mutation frequency of HIV-1, primarily due to a striking ≈155-fold increase in the G-to-C transversion frequency. Intriguingly, decitabine also led to an ≈29-fold increase in the C-to-G transversion frequency. G-to-C frequencies varied substantially (up to ≈80-fold) depending upon sequence position, but surprisingly, mutational hot spots (defined as upper outliers within the mutation frequency distribution) were not observed. We further found that every single guanine position examined was significantly susceptible to the mutagenic effects of decitabine. Taken together, these observations demonstrate for the first time that decitabine-mediated HIV-1 mutagenesis is promiscuous and occurs in the absence of a clear bias for mutational hot spots. These data imply that decitabine-mediated G-to-C mutagenesis is a highly effective antiviral mechanism for extinguishing HIV-1 infectivity.

  4. Mutation Hot Spots in Yeast Caused by Long-Range Clustering of Homopolymeric Sequences

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    Xin Ma


    Full Text Available Evolutionary theory assumes that mutations occur randomly in the genome; however, studies performed in a variety of organisms indicate the existence of context-dependent mutation biases. Sources of mutagenesis variation across large genomic contexts (e.g., hundreds of bases have not been identified. Here, we use high-coverage whole-genome sequencing of a conditional mismatch repair mutant line of diploid yeast to identify mutations that accumulated after 160 generations of growth. The vast majority of the mutations accumulated as insertion/deletions (in/dels in homopolymeric [poly(dA:dT] and repetitive DNA tracts. Surprisingly, the likelihood of an in/del mutation in a given poly(dA:dT tract is increased by the presence of nearby poly(dA:dT tracts in up to a 1,000 bp region centered on the given tract. Our work suggests that specific mutation hot spots can contribute disproportionately to the genetic variation that is introduced into populations and provides long-range genomic sequence context that contributes to mutagenesis.

  5. Mutational hot spot in the DSPP gene causing dentinogenesis imperfecta type II. (United States)

    Kim, Jung-Wook; Hu, Jan C-C; Lee, Jae-Il; Moon, Sung-Kwon; Kim, Young-Jae; Jang, Ki-Taeg; Lee, Sang-Hoon; Kim, Chong-Chul; Hahn, Se-Hyun; Simmer, James P


    The current system for the classification of hereditary defects of tooth dentin is based upon clinical and radiographic findings and consists of two types of dentin dysplasia (DD) and three types of dentinogenesis imperfecta (DGI). However, whether DGI type III should be considered a distinct phenotype or a variation of DGI type II is debatable. In the 30 years since the classification system was first proposed, significant advances have been made regarding the genetic etiologies of inherited dentin defects. DGI type II is recognized as an autosomal dominant disorder with almost complete penetrance and a low frequency of de novo mutations. We have identified a mutation (c.52G-->T, p.V18F) at the first nucleotide of exon 3 of the DSPP (dentin sialophosphoprotein) gene in a Korean family (de novo) and a Caucasian family. This mutation has previously been reported as causing DGI type II in a Chinese family. These findings suggest that this mutation site represents a mutational "hot spot" in the DSPP gene. The clinical and radiographic features of these two families include the classic phenotypes associated with both DGI type II and type III. Finding that a single mutation causes both phenotypic patterns strongly supports the conclusion that DGI type II and DGI type III are not separate diseases but rather the phenotypic variation of a single disease. We propose a modification of the current classification system such that the designation "hereditary opalescent dentin" or "DGI type II" should be used to describe both the DGI type II and type III phenotypes.

  6. Isocitrate dehydrogenase mutation hot spots in acute lymphoblastic leukemia and oral cancer

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    Jen-Yang Tang


    Full Text Available Isocitrate dehydrogenase (IDH encodes a nicotinamide adenine dinucleotide phosphate+-dependent enzyme for oxidative decarboxylation of isocitrate and has an essential role in the tricarboxylic acid cycle. Mutations of IDH1 and IDH2 have been identified in patients with glioma, leukemia, and other cancers. However, the incidence of IDH mutations in acute myeloid leukemia in Taiwan is much lower than that reported in Western countries. The reason for the difference is unknown and its clinical implications remain unclear. Acute lymphoblastic leukemia (ALL is a heterogenous hematopoietic malignancy. Oral squamous cell carcinoma (OSCC results from chronic carcinogen exposures and is highly prevalent in trucking workers, especially in southern Taiwan. Subtypes of both diseases require specific treatments, and molecular markers for developing tailored treatments are limited. High-resolution melting (HRM analysis is now a widely used methodology for rapid, accurate, and low-cost mutation scanning. In this study, 90 adults with OSC and 31 children with ALL were scanned by HRM analysis for IDH1 and IDH2 mutation hot spots. In ALL, the allele frequency was 3.23% in both IDH1 and IDH2. In OSCC, the allele frequency was 2.22% in IDH2. A synonymous mutation over pG313 (c.939A > G of IDH2 was found in both pediatric ALL and adult OSCC. Therefore, we concluded that mutations of IDH are uncommon in ALL and OSCC and are apparently not a major consideration when selecting treatment modalities.

  7. Identification of a Non-Gatekeeper Hot Spot for Drug-Resistant Mutations in mTOR Kinase

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    Tzung-Ju Wu


    Full Text Available Protein kinases are therapeutic targets for human cancer. However, “gatekeeper” mutations in tyrosine kinases cause acquired clinical resistance, limiting long-term treatment benefits. mTOR is a key cancer driver and drug target. Numerous small-molecule mTOR kinase inhibitors have been developed, with some already in human clinical trials. Given our clinical experience with targeted therapeutics, acquired drug resistance in mTOR is thought likely, but not yet documented. Herein, we describe identification of a hot spot (L2185 for drug-resistant mutations, which is distinct from the gatekeeper site, and a chemical scaffold refractory to drug-resistant mutations. We also provide new insights into mTOR kinase structure and function. The hot spot mutations are potentially useful as surrogate biomarkers for acquired drug resistance in ongoing clinical trials and future treatments and for the design of the next generation of mTOR-targeted drugs. Our study provides a foundation for further research into mTOR kinase function and targeting.

  8. Germline Stem Cell Competition, Mutation Hot Spots, Genetic Disorders, and Older Fathers. (United States)

    Arnheim, Norman; Calabrese, Peter


    Some de novo human mutations arise at frequencies far exceeding the genome average mutation rate. Examples include the common mutations at one or a few sites in the genes that cause achondroplasia, Apert syndrome, multiple endocrine neoplasia type 2B, and Noonan syndrome. These mutations are recurrent, provide a gain of function, are paternally derived, and are more likely to be transmitted as the father ages. Recent experiments have tested whether the high mutation frequencies are due to an elevated mutation rate per cell division, as expected, or to an advantage of the mutant spermatogonial stem cells over wild-type stem cells. The evidence, which includes the surprising discovery of testis mutation clusters, rules out the former model but not the latter. We propose how the mutations might alter spermatogonial stem cell function and discuss how germline selection contributes to the paternal age effect, the human mutational load, and adaptive evolution.

  9. HotSpot Wizard 2.0: automated design of site-specific mutations and smart libraries in protein engineering. (United States)

    Bendl, Jaroslav; Stourac, Jan; Sebestova, Eva; Vavra, Ondrej; Musil, Milos; Brezovsky, Jan; Damborsky, Jiri


    HotSpot Wizard 2.0 is a web server for automated identification of hot spots and design of smart libraries for engineering proteins' stability, catalytic activity, substrate specificity and enantioselectivity. The server integrates sequence, structural and evolutionary information obtained from 3 databases and 20 computational tools. Users are guided through the processes of selecting hot spots using four different protein engineering strategies and optimizing the resulting library's size by narrowing down a set of substitutions at individual randomized positions. The only required input is a query protein structure. The results of the calculations are mapped onto the protein's structure and visualized with a JSmol applet. HotSpot Wizard lists annotated residues suitable for mutagenesis and can automatically design appropriate codons for each implemented strategy. Overall, HotSpot Wizard provides comprehensive annotations of protein structures and assists protein engineers with the rational design of site-specific mutations and focused libraries. It is freely available at

  10. Identification of an mtDNA mutation hot spot in UV-induced mouse skin tumors producing altered cellular biochemistry. (United States)

    Jandova, Jana; Eshaghian, Alex; Shi, Mingjian; Li, Meiling; King, Lloyd E; Janda, Jaroslav; Sligh, James E


    There is increasing awareness of the role of mtDNA alterations in the development of cancer, as mtDNA point mutations are found at high frequency in a variety of human tumors. To determine the biological effects of mtDNA mutations in UV-induced skin tumors, hairless mice were irradiated to produce tumors, and the tumor mtDNAs were screened for single-nucleotide changes using temperature gradient capillary electrophoresis (TGCE), followed by direct sequencing. A mutation hot spot (9821insA) in the mitochondrially encoded tRNA arginine (mt-Tr) locus (tRNA(Arg)) was discovered in approximately one-third of premalignant and malignant skin tumors. To determine the functional relevance of this particular mutation in vitro, cybrid cell lines containing different mt-Tr (tRNA(Arg)) alleles were generated. The resulting cybrid cell lines contained the same nuclear genotype and differed only in their mtDNAs. The biochemical analysis of the cybrids revealed that the mutant haplotype is associated with diminished levels of complex I protein (CI), resulting in lower levels of baseline oxygen consumption and lower cellular adenosine triphosphate (ATP) production. We hypothesize that this specific mtDNA mutation alters cellular biochemistry, supporting the development of keratinocyte neoplasia.

  11. Biological impacts of "hot-spot" mutations of hepatitis B virus X proteins are genotype B and C differentiated

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    Xu Lin; Xiao Xu; Qing-Ling Huang; Yu-Qing Liu; Da-Li Zheng; Wan-Nan Chen; Jian-Yin Lin


    AIM: To investigate the biological impacts of "hot-spot"mutations on genotype B and C HBV X proteins (HBx).METHODS: Five types of"hot-spot" mutations of genotype B or C HBV X genes, which sequentially lead to the amino acid substitutions of HBx as I127T, F132Y, K130M+V131I,I127T+K130M+V131I, or K130M+V131I+F132Y, respectively,were generated by means of site-directed mutagenesis.To evaluate the anti-proliferative effects, HBx or related mutants' expression vectors were transfected separately to the Chang cells by lipofectamine, and the cells were cultured in hygromycin selective medium for 14 d, drug-resistant colonies were fixed with cold methanol, stained with Giemsa dyes and scored (increase of the colonies indicated the reduction of the anti-proliferation activity,and vice versa). Different types of HBx expression vectors were co-transfected separately with the reporter plasmid pCMVβ to Chang cells, which were lysed 48 h post-transfection and the intra-cellular β-galactosidase activities were monitored (increase of the β-galactosidase activities indicated the reduction of the transactivation activity, and vice versa). All data obtained were calculated by paired-samples t-test.RESULTS: As compared to standard genotype B HBx,mutants of I127T and I127T+K130M+V131I showed higher transactivation and anti-proliferative activities, while the mutants of F132Y, K130M+V131I, and K130M+V131I+F132Y showed lower activities. As compared to standard genotype C HBx, I127T mutant showed higher transactivation activity, while the other four types of mutants showed no differences. With regard to anti-proliferative activity,compared to standard genotype C HBx, F132Y and K130M+V131I mutants showed lower activities, and K130M+V131I +F132Y mutant, on the other hand, showed higher activity,while the mutants of I127T and I127T+K130M+V131I showed no differences.CONCLUSION: "Hot-spot" mutations affect the antiproliferation and transactivation activities of genotype B and/or C HBx, and

  12. A cancer-predisposing "hot spot" mutation of the fumarase gene creates a dominant negative protein. (United States)

    Lorenzato, Annalisa; Olivero, Martina; Perro, Mario; Brière, Jean Jacques; Rustin, Pierre; Di Renzo, Maria Flavia


    The Fumarase (Fumarate Hydratase, FH) is a tumor suppressor gene whose germline heterozygous mutations predispose to hereditary leiomyomatosis and renal cell cancer (HLRCC). The FH gene encodes an enzyme of the Krebs cycle, functioning as a homotetramer and catalyzing the hydration of fumarate to malate. Among the numerous FH mutations reported so far, the R190H missense mutation is the most frequent in HLRCC patients. Here we show the functional analyses of the R190H, in comparison to the better characterized E319Q mutation. We first expressed wild-type and mutated proteins in FH deficient human skin fibroblasts, using lentiviral vectors. The wild-type transgene was able to restore the FH enzymatic activity in cells, while the R190H- and E319Q-FH were not. More interestingly, when the same transgenes were expressed in normal, FH-proficient cells, only the R190H-FH reduced the endogenous FH enzymatic activity. By enforcing the expression of equal amount of wild-type and R190H-FH in the same cell, we showed that the mutated FH protein directly inhibited enzymatic activity by nearly abrogating the FH homotetramer formation. These data demonstrate the dominant negative effect of the R190H missense mutation in the FH gene and suggest that the FH tumor-suppressing activity might be impaired in cells carrying a heterozygous mutation. (c) 2007 Wiley-Liss, Inc.

  13. CTCF cis-regulates trinucleotide repeat instability in an epigenetic manner: a novel basis for mutational hot spot determination.

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    Randell T Libby


    Full Text Available At least 25 inherited disorders in humans result from microsatellite repeat expansion. Dramatic variation in repeat instability occurs at different disease loci and between different tissues; however, cis-elements and trans-factors regulating the instability process remain undefined. Genomic fragments from the human spinocerebellar ataxia type 7 (SCA7 locus, containing a highly unstable CAG tract, were previously introduced into mice to localize cis-acting "instability elements," and revealed that genomic context is required for repeat instability. The critical instability-inducing region contained binding sites for CTCF -- a regulatory factor implicated in genomic imprinting, chromatin remodeling, and DNA conformation change. To evaluate the role of CTCF in repeat instability, we derived transgenic mice carrying SCA7 genomic fragments with CTCF binding-site mutations. We found that CTCF binding-site mutation promotes triplet repeat instability both in the germ line and in somatic tissues, and that CpG methylation of CTCF binding sites can further destabilize triplet repeat expansions. As CTCF binding sites are associated with a number of highly unstable repeat loci, our findings suggest a novel basis for demarcation and regulation of mutational hot spots and implicate CTCF in the modulation of genetic repeat instability.

  14. Disease Mutations in the Ryanodine Receptor Central Region: Crystal Structures of a Phosphorylation Hot Spot Domain

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    Yuchi, Zhiguang; Lau, Kelvin; Van Petegem, Filip (UBC)


    Ryanodine Receptors (RyRs) are huge Ca{sup 2+} release channels in the endoplasmic reticulum membrane and form targets for phosphorylation and disease mutations. We present crystal structures of a domain in three RyR isoforms, containing the Ser2843 (RyR1) and Ser2808/Ser2814 (RyR2) phosphorylation sites. The RyR1 domain is the target for 11 disease mutations. Several of these are clustered near the phosphorylation sites, suggesting that phosphorylation and disease mutations may affect the same interface. The L2867G mutation causes a drastic thermal destabilization and aggregation at room temperature. Crystal structures for other disease mutants show that they affect surface properties and intradomain salt bridges. In vitro phosphorylation experiments show that up to five residues in one long loop of RyR2 can be phosphorylated by PKA or CaMKII. Docking into cryo-electron microscopy maps suggests a putative location in the clamp region, implying that mutations and phosphorylation may affect the allosteric motions within this area.

  15. Construction of a multiplex mutation hot spot PCR panel: the first step towards colorectal cancer genotyping on the GS Junior platform (United States)

    Péterfia, Bálint; Kalmár, Alexandra; Patai, Árpád V.; Csabai, István; Bodor, András; Micsik, Tamás; Wichmann, Barnabás; Egedi, Krisztina; Hollósi, Péter; Kovalszky, Ilona; Tulassay, Zsolt; Molnár, Béla


    Background: To support cancer therapy, development of low cost library preparation techniques for targeted next generation sequencing (NGS) is needed. In this study we designed and tested a PCR-based library preparation panel with limited target area for sequencing the top 12 somatic mutation hot spots in colorectal cancer on the GS Junior instrument. Materials and Methods: A multiplex PCR panel was designed to amplify regions of mutation hot spots in 12 selected genes (APC, BRAF, CTNNB1, EGFR, FBXW7, KRAS, NRAS, MSH6, PIK3CA, SMAD2, SMAD4, TP53). Amplicons were sequenced on a GS Junior instrument using ligated and barcoded adaptors. Eight samples were sequenced in a single run. Colonic DNA samples (8 normal mucosa; 33 adenomas; 17 adenocarcinomas) as well as HT-29 and Caco-2 cell lines with known mutation profiles were analyzed. Variants found by the panel on APC, BRAF, KRAS and NRAS genes were validated by conventional sequencing. Results: In total, 34 kinds of mutations were detected including two novel mutations (FBXW7 c.1740:C>G and SMAD4 c.413C>G) that have not been recorded in mutation databases, and one potential germline mutation (APC). The most frequently mutated genes were APC, TP53 and KRAS with 30%, 15% and 21% frequencies in adenomas and 29%, 53% and 29% frequencies in carcinomas, respectively. In cell lines, all the expected mutations were detected except for one located in a homopolymer region. According to re-sequencing results sensitivity and specificity was 100% and 92% respectively. Conclusions: Our NGS-based screening panel denotes a promising step towards low cost colorectal cancer genotyping on the GS Junior instrument. Despite the relatively low coverage, we discovered two novel mutations and obtained mutation frequencies comparable to literature data. Additionally, as an advantage, this panel requires less template DNA than sequence capture colon cancer panels currently available for the GS Junior instrument.

  16. The R304X mutation of the Aryl hydrocarbon receptor Interacting Protein gene in familial isolated pituitary adenomas: mutational Hot-Spot or founder effect?


    Occhi, G.; Jaffrain-Rea, M. L.; Trivellin, G.; Albiger, N; Ceccato, F.; De Menis, E.; Angelini, M.; Ferasin, S; Beckers, Albert; F. Mantero; Scaroni, C.


    Background: Mutations in the Aryl hydrocarbon receptor Interacting Protein (AIP) gene have been described in about 15% of kindreds with Familial Isolated Pituitary Adenomas (FIPA) and in a minority of early onset sporadic pituitary adenomas (PA). Among the AIP mutations reported so far, the R304X (AIPR304X) represents, together with the "Finnish mutation" Q14X, the most common one. Methods: Three AIPR304X Italian families, including a newly reported kindred, have been genotyped for 12 genetic...

  17. A duchenne muscular dystrophy gene hot spot mutation in dystrophin-deficient cavalier king charles spaniels is amenable to exon 51 skipping.

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    Gemma L Walmsley

    Full Text Available BACKGROUND: Duchenne muscular dystrophy (DMD, which afflicts 1 in 3500 boys, is one of the most common genetic disorders of children. This fatal degenerative condition is caused by an absence or deficiency of dystrophin in striated muscle. Most affected patients have inherited or spontaneous deletions in the dystrophin gene that disrupt the reading frame resulting in unstable truncated products. For these patients, restoration of the reading frame via antisense oligonucleotide-mediated exon skipping is a promising therapeutic approach. The major DMD deletion "hot spot" is found between exons 45 and 53, and skipping exon 51 in particular is predicted to ameliorate the dystrophic phenotype in the greatest number of patients. Currently the mdx mouse is the most widely used animal model of DMD, although its mild phenotype limits its suitability in clinical trials. The Golden Retriever muscular dystrophy (GRMD model has a severe phenotype, but due to its large size, is expensive to use. Both these models have mutations in regions of the dystrophin gene distant from the commonly mutated DMD "hot spot". METHODOLOGY/PRINCIPAL FINDINGS: Here we describe the severe phenotype, histopathological findings, and molecular analysis of Cavalier King Charles Spaniels with dystrophin-deficient muscular dystrophy (CKCS-MD. The dogs harbour a missense mutation in the 5' donor splice site of exon 50 that results in deletion of exon 50 in mRNA transcripts and a predicted premature truncation of the translated protein. Antisense oligonucleotide-mediated skipping of exon 51 in cultured myoblasts from an affected dog restored the reading frame and protein expression. CONCLUSIONS/SIGNIFICANCE: Given the small size of the breed, the amiable temperament and the nature of the mutation, we propose that CKCS-MD is a valuable new model for clinical trials of antisense oligonucleotide-induced exon skipping and other therapeutic approaches for DMD.

  18. Hepatitis B virus core protein with hot-spot mutations inhibit MxA gene transcription but has no effect on inhibition of virus replication by interferon α. (United States)

    Zhijian, Yu; Zhen, Huang; Fan, Zhang; Jin, Yang; Qiwen, Deng; Zhongming, Zeng


    It has been reported that hepatitis B virus (HBV) core protein (HBc) can inhibit the transcription of human interferon-induced MxA gene. In this study, we investigated whether HBc protein mutations at hot spots (L60V, S87G and I97L) could still inhibit MxA transcription and the potential significance of this inhibition in virus replication in vitro. Our data indicated that the IFN-induced MxA mRNA expression level and MxA promoter activity was significantly down-regulated by mutant protein of HBc(I97L), compared to WT and the other two mutated HBc proteins(L60V or S87G). However, in Huh7 cells stably expressing WT or the mutated HBc proteins (L60V, S87G or I97L), IFN-α could inhibit the extra- and intracellular HBV DNA level and HBsAg secretion to a similar level compared to that in cells transfected with control plasmids. In conclusion, HBc protein with I97L mutation may play an special role in suppressing the transcription of MxA gene. Moreover, the inhibitory effect on MxA gene transcription by the WT or mutated HBc proteins (L60V, S87G and I97L) has no impact on inhibition of HBV replication by IFN-α in Huh7 cells. The clinical significance of the inhibitory effect of MxA gene transcription by HBc protein requires further study.

  19. Comparison of HgO and CuSO4 as digestion catalysts in manual Kjeldahl determination of crude protein in animal feeds: collaborative study. (United States)

    Kane, P F


    The official AOAC manual Kjeldahl method for determining crude protein in animal feeds, 7.015, uses HgO as a catalyst in the digestion step. Because of environmental considerations, there is considerable interest in alternative catalysts. A collaborative study compares the official HgO-catalyzed method and an alternative using CuSO4. Fifty-four samples consisting of blind duplicates of closely matched pairs, representing a range of animal feed materials and 2 standard materials, were analyzed once by each method. Results were returned by 22 laboratories. Means and standard deviations between methods were comparable. The CuSO4-catalyzed method has been adopted official first action.

  20. Complete mitochondrial genome sequence of three Tetrahymena species reveals mutation hot spots and accelerated nonsynonymous substitutions in Ymf genes.

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    Mike M Moradian

    Full Text Available The ciliate Tetrahymena, a model organism, contains divergent mitochondrial (Mt genome with unusual properties, where half of its 44 genes still remain without a definitive function. These genes could be categorized into two major groups of KPC (known protein coding and Ymf (genes without an identified function. To gain insights into the mechanisms underlying gene divergence and molecular evolution of Tetrahymena (T. Mt genomes, we sequenced three Mt genomes of T.paravorax, T.pigmentosa, and T.malaccensis. These genomes were aligned and the analyses were carried out using several programs that calculate distance, nucleotide substitution (dn/ds, and their rate ratios (omega on individual codon sites and via a sliding window approach. Comparative genomic analysis indicated a conserved putative transcription control sequence, a GC box, in a region where presumably transcription and replication initiate. We also found distinct features in Mt genome of T.paravorax despite similar genome organization among these approximately 47 kb long linear genomes. Another significant finding was the presence of at least one or more highly variable regions in Ymf genes where majority of substitutions were concentrated. These regions were mutation hotspots where elevated distances and the dn/ds ratios were primarily due to an increase in the number of nonsynonymous substitutions, suggesting relaxed selective constraint. However, in a few Ymf genes, accelerated rates of nonsynonymous substitutions may be due to positive selection. Similarly, on protein level the majority of amino acid replacements occurred in these regions. Ymf genes comprise half of the genes in Tetrahymena Mt genomes, so understanding why they have not been assigned definitive functions is an important aspect of molecular evolution. Importantly, nucleotide substitution types and rates suggest possible reasons for not being able to find homologues for Ymf genes. Additionally, comparative genomic

  1. Comparison of HgO and CuSO4/TiO2 as catalysts in manual Kjeldahl digestion for determination of crude protein in animal feed: collaborative study. (United States)

    Kane, P F


    Because of environmental concerns about HgO, and because of lengthy digestion requirements for HgO and CuSO4, interest in alternative catalysts for the Kjeldahl determination of animal feeds remains high. A digestion system using a mixed CuSO4/TiO2 catalyst has been found to reduce digestion times to 40 min. A collaborative study was carried out to compare this system to the official AOAC HgO method, 7.015. Thirty-eight samples, consisting of blind duplicates of closely matched pairs and 2 standard materials, were analyzed once by each method. Results were received from 13 laboratories. Means and standard deviations of individual samples were comparable, with an overall difference of grand means of 0.005% protein. With only one exception, analyses of variance showed no significant method difference at the 95% confidence level. The CuSO4/TiO2 method has been approved interim official first action as an alternative method for determination of crude protein in animal feed.

  2. Hot Money, Hot Potato

    Institute of Scientific and Technical Information of China (English)


    International hot money flowing into Chinese capital markets has caught the attention of Chinese watchdogs The Chinese are not the only ones feasting on the thriving property and stock markets. Apparently, these markets are the targets of international h

  3. Two CpG mutational hot spots in the X-linked interleukin-2 receptor gamma chain gene (IL2RG) may cause 15% of all human severe combined immunodeficiency

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    Pepper, A.E.; Puck, J.M. [National Center for Human Genome Research, Bethseda, MD (United States); Liu, X. [Univ. of Pennsylvania School of Medicine, Philadelphia, PA (United States)


    Severe combined immunodeficiency (SCID), a syndrome of profoundly impaired cellular and humoral immune function, is caused by various autosomal gene defects, including adenosine deaminase (ADA) deficiency, as well as mutations in the X-linked IL2RG gene encoding the gamma chain of the lymphocyte receptor for IL-2. Mutational analysis of IL2RG was performed using genomic DNA from males with SCID referred from genetics and immunology centers. Single strand conformation polymorphisms (SSCP) were sought by PCR amplification of each of the 8 IL2RG exons using labelled flanking primers. Sequence of exons with aberrant SSCP detected a majority of unique deleterious IL2RG mutations in 30 unrelated SCID patients. However, multiple mutations were seen at CpG dinucleotides, known to be C{yields}T transversion sites. cDNA 690-691 in exon 5 was mutated in 4 patients, 1 patient with each of the C{sub 690}{yields}T causing an Arg{yields}Cys substitution, and 1 with G{sub 691}{yields}A causing Arg{yields}His. Two other patients had SCID caused by a single mutation in IL2RG exon 7. This C{sub 879}{yields}T, also in a CpG, changed an Arg to STOP, resulting in loss of the SH2-related intracellular domain. In addition to our patients, 1 patient with each of the C{sub 690} and the C{sub 879} mutations have been reported by others, giving an overall incidence of 20% from our lab and 21% from all reported IL2RG SCID mutations. While ADA defects account for approximately 15% of SCID, a striking male SCID predominance suggest up to 70% of the cases are X-linked, due to IL2RG mutation. Thus, screening for mutations at the 2 CpG hot spots we have found in IL2RG can identify the genotype of as many SCID cases as are found by ADA testing.

  4. Pressure fluctuations on the bed of surge tank at the H.P. Zimapan, Hgo., with different arrangements studied on hydraulic model, with the lowest operation conditions; Fluctuaciones de presion en la base del pozo de oscilacion del P.H. Zimapan Hgo., con diferentes arreglos estudiados en modelo hidraulico ante las condiciones minimas de operacion

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    Marengo Mogollon, H. [Facultad de Ingenieria, Universidad Nacional Autonoma de Mexico (Mexico)]. E-mail:; Ochoa Alvarez, F.J.; Cortes Cortes, C. [Comision Federal de Electricidad (Mexico)]. E-mail:;


    In this paper, the pressure fluctuations of the surge tank in the Zimapan Hydroelectric Project are compared in a hydraulic model. The shaft is located lateral, over the conduction tunnel and in the simple form (permitting the tunnel entering the shaft); with and without orifice plates taking into account the demand and supply condition of energy with the minimum level of water of the conduction. It was determined the hydraulic efficiency and it was found that it was the best constructive option. [Spanish] En este articulo se comparan las fluctuaciones de presion en el pozo de oscilacion del P.H. Zimapan, Hgo., Mexico las cuales fueron estudiadas en modelo hidraulico al considerar dicho pozo ubicado en diferentes posiciones; lateralmente y sobre el eje de la conduccion; pozo simple y con tuberia de conexion; con y sin placa de orificio, para maniobras de rechazo y demanda de carga de las turbinas de generacion con el nivel del agua correspondiente al NAMINO. Se determino la eficiencia hidraulica comparandola con las otras opciones encontrandose que la mejor opcion para el funcionamiento hidraulico es el pozo lateral, que ademas permite optimizar el procedimiento constructivo.

  5. Hot microswimmers (United States)

    Kroy, Klaus; Chakraborty, Dipanjan; Cichos, Frank


    Hot microswimmers are self-propelled Brownian particles that exploit local heating for their directed self-thermophoretic motion. We provide a pedagogical overview of the key physical mechanisms underlying this promising new technology. It covers the hydrodynamics of swimming, thermophoresis and -osmosis, hot Brownian motion, force-free steering, and dedicated experimental and simulation tools to analyze hot Brownian swimmers.

  6. Mutational analysis of the β-trefoil fold protein barley α-amylase/subtilisin inhibitor probes hot spots for the interaction with barley α-amylase

    DEFF Research Database (Denmark)

    Bønsager, Birgit Christine; Nielsen, P. K.; Abou Hachem, Maher;


    increased 2-3 orders of magnitude, whereas mutations on the other side of the inhibitor had virtually no effect. The mutants K140L, D150N, and E168T lost inhibitory activity, revealing the pivotal role of charge interactions for BASI activity on AMY2. A fully hydrated Ca(2+) at the AMY2-BASI interface...

  7. HOT 2012

    DEFF Research Database (Denmark)

    Lund, Henriette Romme

    Undersøgelse af, hvad der er hot - og hvad der burde være hot på læseområdet med 21 læsekyndige. Undersøgelsen er gennemført siden 2010. HOT-undersøgelsen er foretaget af Nationalt Videncenter for Læsning - Professionshøjskolerne i samarb. med Dansklærerforeningen...

  8. Mutational analysis of the β-trefoil fold protein barley α-amylase/subtilisin inhibitor probes hot spots for the interaction with barley α-amylase

    DEFF Research Database (Denmark)

    Bønsager, Birgit Christine; Nielsen, P. K.; Abou Hachem, Maher


    The barley alpha-amylase/subtilisin inhibitor (BASI) inhibits alpha-amylase 2 (AMY2) with subnanomolar affinity. The contribution of selected side chains of BASI to this high affinity is discerned in this study, and binding to other targets is investigated. Seven BASI residues along the AMY2-BASI...... interface and four residues in the putative protease-binding loop on the opposite side of the inhibitor were mutated. A total of 15 variants were compared with the wild type by monitoring the alpha-amylase and protease inhibitory activities using Blue Starch and azoalbumin, respectively, and the kinetics...

  9. A literature review of epidemiological studies on mutation hot spots of Chinese population with non-syndromic hearing loss%非综合征性聋突变热点的流行病学分析

    Institute of Scientific and Technical Information of China (English)

    李海波; 李琼; 李红; 陈瑛


    Objective: To identify the regional mutation hot spots characteristics, and then to establish a regional genetic screening programs. Method; Molecular epidemiological literatures about Chinese deafness gene GJB2,SLC26A4,mitochondrial DNA mutations from 2006 to the first half of 2011 were retrieved in Wanfang and Pubmed literature database. The primary data of these studies including the number of samples, demographic characteristics, mutation frequencies and so on,were analyzed statiscally. Result:Of 46 papers, 42 were included in this study. The patients all had non-syndromic sensorineural hearing loss and lived in 20 regions of China. A total of 18094 were counted and the average mutation frequencies were approximately 42.0%. The mutation frequencies of 235 delC were 16. 34%, 299-300 delAT were 4. 75% in GJB2 gene and IVS7-2A>G were 12. 60% , 2168A>G were 2. 32% in SLC26A4 gene and the mutation frequencies of 1555A>G, 1494C>T in mtDNA were 5. 21 %, 1. 11% respectively. The statistical discrepancy was significant among mutation frequencies in different regions by χ2test(P<0. 05). Conclusion:Molecular epidemiological statistics show that the 6 locus are the mutation hot spots in Chinese people with non-syndromic hearing loss and can be used for genetic screening according to the specific regional mutation characteristics.%目的:了解耳聋基因突变热点在中国患者中的分布特征,以建立针对突变热点的区域性基因筛查方案.方法:运用万方和Pubmed等检索2006-2011年上半年国内报道的中国各地区人群耳聋相关基因GJB2、SLC26A4、mtDNA突变流行病学文献,对文献中的样本量、样本特征、地域分布、突变频率等多个因素进行统计分析.结果:检索到相关文献46篇,纳入该统计分析42篇,研究区域涉及中国20个省、市、自治区和直辖市,调查人群均为非综合征型感音神经性聋患者;样本总量18 094例,GJB2 235 delC突变频率为16.34%,GJB2 299-300 del

  10. Hot Tickets (United States)

    Fox, Bette-Lee; Hoffert, Barbara; Kuzyk, Raya; McCormack, Heather; Williams, Wilda


    This article describes the highlights of this year's BookExpo America (BEA) held at the Los Angeles Convention Center. The attendees at BEA had not minded that the air was recycled, the lighting was fluorescent, and the food was bad. The first hot book sighting came courtesy of Anne Rice. Michelle Moran, author of newly published novel, "The…

  11. Hot Money,Hot Problems

    Institute of Scientific and Technical Information of China (English)


    After emerging from the economic doldrums, developing economies are now confronted with a new danger-a flood of international hot money. But how has the speculative capital circumvented regulatory controls and what are the consequences concerning the stability of the developing world? Zhao Zhongwei, a senior researcher with the Institute of World Politics and Economics at the Chinese Academy of Social Sciences, discussed these issues in an article recently published in the China Securities Journal. Edited excerpts follow

  12. Are 'hot spots' hot spots? (United States)

    Foulger, Gillian R.


    The term 'hot spot' emerged in the 1960s from speculations that Hawaii might have its origins in an unusually hot source region in the mantle. It subsequently became widely used to refer to volcanic regions considered to be anomalous in the then-new plate tectonic paradigm. It carried with it the implication that volcanism (a) is emplaced by a single, spatially restricted, mongenetic melt-delivery system, assumed to be a mantle plume, and (b) that the source is unusually hot. This model has tended to be assumed a priori to be correct. Nevertheless, there are many geological ways of testing it, and a great deal of work has recently been done to do so. Two fundamental problems challenge this work. First is the difficulty of deciding a 'normal' mantle temperature against which to compare estimates. This is usually taken to be the source temperature of mid-ocean ridge basalts (MORBs). However, Earth's surface conduction layer is ˜200 km thick, and such a norm is not appropriate if the lavas under investigation formed deeper than the 40-50 km source depth of MORB. Second, methods for estimating temperature suffer from ambiguity of interpretation with composition and partial melt, controversy regarding how they should be applied, lack of repeatability between studies using the same data, and insufficient precision to detect the 200-300 °C temperature variations postulated. Available methods include multiple seismological and petrological approaches, modelling bathymetry and topography, and measuring heat flow. Investigations have been carried out in many areas postulated to represent either (hot) plume heads or (hotter) tails. These include sections of the mid-ocean spreading ridge postulated to include ridge-centred plumes, the North Atlantic Igneous Province, Iceland, Hawaii, oceanic plateaus, and high-standing continental areas such as the Hoggar swell. Most volcanic regions that may reasonably be considered anomalous in the simple plate-tectonic paradigm have been

  13. Analysis on hot spot mutations of GJB2 gene and mitochondrial DNA 12S rRNA among consanguineous induced deafness families%近亲婚配致聋家系中患者GJB2和线粒体DNA 12S rRNA基因突变热点分析

    Institute of Scientific and Technical Information of China (English)

    杨威; 付四清; 董家曙; 王春芳; 陈观明


    Objective: To analyze the frequencies of hot spot mutations of the two common deafness gene - GJB2 and mitochondrial DNA 12S rRNA in the consanguineous induced deafness families. Methods: 25 consanguineous induced deafness families received comprehensive physical examination and pure tone test, 48 cases were found, 3 mi venous blood samples were obtained to extract DNA, polymerase chain reaction (PCR) and restriction fragment length polymorphism (RFLP) targeted to specific genes were performed, the cases with mutation were confirmed by gene sequencing. Results: 1 case was found with 235delC homozygous mutation of GJB2 gene, 3 cases were found with heterozygous mutation; no A 1555G mutation was found in mitochondrial DNA 12S rRNA. Conclusion: The most of consanguineous induced deafness are autosomal recessive inheritance, but the incidence of 235delC mutation of GJB2 gene is low.%目的:分析GJB2和线粒体DNA 12S rRNA两种常见耳聋致病基因突变热点在近亲婚配致聋家系患者中的发生频率.方法:对25个近亲结婚致聋核心家系的后代进行全面的体格检查和纯音测试,共发现患者48例,采静脉血3 ml,提取DNA,针对特定基因,进行聚合酶链式反应(Polymerase chain reaction,PCR)及限制性片段长度多态性(Restriction fragmentlength polymorphism,RFLP)分析,发现突变者测序验证.结果:发现GJB2基因235delC纯合突变1例,杂合突变3例;未发现线粒体DNA 12S rRNA A1555G突变.结论:近亲结婚家系中遗传性聋多为常染色体隐性遗传,但GJB2基因235delc突变的发生率较低.

  14. Hot-dome anemometry (United States)

    Thompson, Brian E.


    Hot-dome anemometry obtains three components of flow velocity using an array of sensors, specifically five hot films in the present contribution, which are mounted around the hemispherical tip of a cylindrical support. Calibration for speed and angle resembles that of hot wires and split films except that the procedures accommodate heat transfer dominated by forced convection from the surface of a sphere rather than single or multiple cylinders. Measurements are obtained with hot domes, conventional hot wires, and impact probes in the wake of a wing to quantify measurement uncertainties.

  15. Effect of different Thai traditional processing of various hot chili peppers on urethane-induced somatic mutation and recombination in Drosophila melanogaster: assessment of the role of glutathione transferase activity. (United States)

    Laohavechvanich, P; Kangsadalampai, K; Tirawanchai, N; Ketterman, A J


    Four different Thai traditional chili peppers, namely bird pepper (Capsicum frutescens), red chili spur peppers (Capsicum annuum), green bell peppers and sweet pepper (C. annuum) were investigated for their antimutagenic properties. Each chili was prepared in three formulations commonly used for chili food processing; raw paste (chili ground in water), pickled in vinegar or stir-fried in palm oil. Each sample was tested for its antimutagenic effect against urethane by using the somatic mutation and recombination of wing hair of Drosophila melanogaster as an indicator. Three-day-old larvae, trans-heterozygous for two genetic markers, multiple wing hairs mwh and orrigon (ORR;flr3), were exposed to urethane alone or in combination with each chili formulation. The various processing methods for chilies differentially extracted the antimutagenic chili components. The specific chili as well as the method of processing influenced the observed antimutagenic properties against urethane. This suggested each chili contains a unique complex mixture of many antimutagens. Co-treatment and pre-treatment experiments showed that both direct and indirect protective mechanisms are involved in an 'activation' process to give antimutagenesis effects. An association between antigenotoxicity and glutathione transferase activity could not be established.

  16. Detection and analysis of the hot spots of ATP7B gene mutation in the members of a family with an adult Wilson's disease%成人Wilson病一家系成员P型铜转运三磷酸腺苷酶基因突变热区的检测和分析

    Institute of Scientific and Technical Information of China (English)

    朱永豹; 黄元成; 郭威; 陈韬


    Objective To make a early gene diagnosis and analyze the mutation characteristics of ATP7B gene by detecting the hot spots of ATP7B gene mutation in Chinese family patients with Wilson's disease. Methods The genomic DNA of the family members were extracted from peripheral blood. The fragments of exon 8,1243 of ATP7B gene were amplified by polymerase chain reaction and PCR products were sequenced bidirectionally. Then we analyzed the results with BLAST software online. Results I 1N I 2( proband) , II1 and II2 have heterozygote missense mutation Arg778Leu(2333G > T) and heterozy-gote nonsense mutation Leu770Leu (2310C>G) on exon 8. I 1 、 I 2, I 3 and II1 have heterozygote mis-sense mutation Lys952Arg(2855A >G) on exon 12. All the subjects have no mutations on exon 13. Conclusion Exon 8、12,13 of ATP7B gene should be detected in the members of a family with a proband having Wilson' s disease, which will help to diagnose pre-symptomatic patients and carriers earlier.%目的 通过检测中国人Wilson病(WD)P型铜转运三磷酸腺苷酶(ATP7B)基因突变热区,对成人WD一家系成员进行早期基因诊断和突变特征分析.方法 提取该家系成员外周血基因组DNA,采用聚合酶链反应扩增ATP7B基因第8、12、13号外显子,并对扩增产物进行直接双向测序,然后应用在线BLAST软件分析.结果 Ⅰ1、Ⅰ2(先证者)、Ⅱ1和Ⅱ2第8号外显子存在Arg778Leu(2333G> T)错义杂合突变,且均伴有Leu770Leu( 2310C>G)同义杂合突变;Ⅰ1、Ⅰ 2、Ⅰ3和Ⅱ1第12号外显子存在Lys952Arg(2855A>G)错义杂合突变;所有受检者第13号外显子均未存在突变.结论 对有先证者的Wilson病家系成员应进行ATP7B基因第8、12、13号外显子检测,有助于早期发现症状前患者和携带者.

  17. Hot plasma dielectric tensor

    NARCIS (Netherlands)

    Westerhof, E.


    The hot plasma dielectric tensor is discussed in its various approximations. Collisionless cyclotron resonant damping and ion/electron Bernstein waves are discussed to exemplify the significance of a kinetic description of plasma waves.

  18. Mutations in the human TWIST gene. (United States)

    Gripp, K W; Zackai, E H; Stolle, C A


    Saethre-Chotzen syndrome is a relatively common craniosynostosis disorder with autosomal dominant inheritance. Mutations in the TWIST gene have been identified in patients with Saethre-Chotzen syndrome. The TWIST gene product is a transcription factor with DNA binding and helix-loop-helix domains. Numerous missense and nonsense mutations cluster in the functional domains, without any apparent mutational hot spot. Two novel point mutations and one novel polymorphism are included in this review. Large deletions including the TWIST gene have been identified in some patients with learning disabilities or mental retardation, which are not typically part of the Saethre-Chotzen syndrome. Comprehensive studies in patients with the clinical diagnosis of Saethre-Chotzen syndrome have demonstrated a TWIST gene abnormality in about 80%, up to 37% of which may be large deletions [Johnson et al., 1998]. The gene deletions and numerous nonsense mutations are suggestive of haploinsufficiency as the disease-causing mechanism. No genotype phenotype correlation was apparent.

  19. Infrared spectra, Raman laser, XRD, DSC/TGA and SEM investigations on the preparations of selenium metal, (Sb2O3, Ga2O3, SnO and HgO) oxides and lead carbonate with pure grade using acetamide precursors

    Indian Academy of Sciences (India)

    Moamen S Refat; Khaled M Elsabawy


    Ga2O3, Se metal, SnO, Sb2O3, HgO and PbCO3 are formed upon the reaction of acetamide aqueous solutions with Ga(NO3)3, SeO2, SnCl2, SbCl3, HgCl2 and Pb(NO3)2, respectively, at 90°C. Different amorphous or crystalline phases can be obtained depending upon the experimental conditions (molar ratios, metal salts and temperature). The chemical mechanisms for the formations of this metal, oxides or carbonate are discussed and the X-ray diffraction, scanning electron microscopy (SEM) and atomic force microscope (AFM) are described. The type of metal ions plays an important role in the decomposition of acetamide, leading to the formation of solid stable (metal, oxides or carbonate), soluble and gases species. These new precursors are more stable preventing the rapid precipitation of metal, oxides or carbonate. Furthermore, this route allows the formation of pure compounds in solutions.

  20. IR Hot Wave

    Energy Technology Data Exchange (ETDEWEB)

    Graham, T. B.


    The IR Hot Wave{trademark} furnace is a breakthrough heat treatment system for manufacturing metal components. Near-infrared (IR) radiant energy combines with IR convective heating for heat treating. Heat treatment is an essential process in the manufacture of most components. The controlled heating and cooling of a metal or metal alloy alters its physical, mechanical, and sometimes chemical properties without changing the object's shape. The IR Hot Wave{trademark} furnace offers the simplest, quickest, most efficient, and cost-effective heat treatment option for metals and metal alloys. Compared with other heat treatment alternatives, the IR Hot Wave{trademark} system: (1) is 3 to 15 times faster; (2) is 2 to 3 times more energy efficient; (3) is 20% to 50% more cost-effective; (4) has a {+-}1 C thermal profile compared to a {+-}10 C thermal profile for conventional gas furnaces; and (5) has a 25% to 50% smaller footprint.

  1. Hot Air Engines

    Directory of Open Access Journals (Sweden)

    P. Stouffs


    Full Text Available Invented in 1816, the hot-air engines have known significant commercial success in the nineteenth century, before falling into disuse. Nowadays they enjoy a renewed interest for some specific applications. The "hot-air engines" family is made up of two groups: Stirling engines and Ericsson engines. The operating principle of Stirling and Ericsson engines, their troubled history, their advantages and their niche applications are briefly presented, especially in the field of micro-combined heat and power, solar energy conversion and biomass energy conversion. The design of an open cycle Ericsson engine for solar application is proposed. A first prototype of the hot part of the engine has been built and tested. Experimental results are presented.

  2. Understanding what determines the frequency and pattern of human germline mutations. (United States)

    Arnheim, Norman; Calabrese, Peter


    Surprising findings about human germline mutation have come from applying new technologies to detect rare mutations in germline DNA, from analysing DNA sequence divergence between humans and closely related species, and from investigating human polymorphic variation. In this Review we discuss how these approaches affect our current understanding of the roles of sex, age, mutation hot spots, germline selection and genomic factors in determining human nucleotide substitution mutation patterns and frequencies. To enhance our understanding of mutation and disease, more extensive molecular data on the human germ line with regard to mutation origin, DNA repair, epigenetic status and the effect of newly arisen mutations on gamete development are needed.

  3. Ultrasonic Hot Embossing

    Directory of Open Access Journals (Sweden)

    Werner Karl Schomburg


    Full Text Available Ultrasonic hot embossing is a new process for fast and low-cost production of micro systems from polymer. Investment costs are on the order of 20.000 € and cycle times are a few seconds. Microstructures are fabricated on polymer foils and can be combined to three-dimensional systems by ultrasonic welding.

  4. What's Hot? What's Not? (United States)

    Buczynski, Sandy


    When Goldilocks finds three bowls of porridge at different temperatures in the three bears' house, she accurately assesses the situation and comes up with one of the most recognizable lines in children's literature," This porridge is too hot; this porridge is too cold; aahh, this porridge is just right!" Goldilocks' famous line is a perfect…

  5. Hot house bad house


    Azzopardi, Shaun


    Shaun Azzopardi met up with a team of researchers led by Eur. Ing. Charles Yousif to take the concrete block to the next level. It is more exciting than it sounds. Photography by Dr Edward Duca.

  6. Fending Off Hot Money

    Institute of Scientific and Technical Information of China (English)


    Amid uncertainties about the amount of hot money,the government strives to curb the harmful capital The benchmark Shanghai Composite Index was plagued by dips, climbs and dives as the stock market slumped from 3,186 to 2,838 points

  7. Hot Fuel Examination Facility (HFEF) (United States)

    Federal Laboratory Consortium — The Hot Fuel Examination Facility (HFEF) is one of the largest hot cells dedicated to radioactive materials research at Idaho National Laboratory (INL). The nation's...

  8. What Is Hot Yoga (Bikram)? (United States)

    Healthy Lifestyle Consumer health What is hot yoga? Answers from Edward R. Laskowski, M.D. Hot yoga is a vigorous form of yoga performed in a studio ... you check with your doctor before trying hot yoga if you have any health concerns. If you have heart disease, problems with ...

  9. Hot Subluminous Stars (United States)

    Heber, U.


    Hot subluminous stars of spectral type B and O are core helium-burning stars at the blue end of the horizontal branch or have evolved even beyond that stage. Most hot subdwarf stars are chemically highly peculiar and provide a laboratory to study diffusion processes that cause these anomalies. The most obvious anomaly lies with helium, which may be a trace element in the atmosphere of some stars (sdB, sdO) while it may be the dominant species in others (He-sdB, He-sdO). Strikingly, the distribution in the Hertzsprung-Russell diagram of He-rich versus He-poor hot subdwarf stars of the globular clusters ω Cen and NGC 2808 differ from that of their field counterparts. The metal-abundance patterns of hot subdwarfs are typically characterized by strong deficiencies of some lighter elements as well as large enrichments of heavy elements. A large fraction of sdB stars are found in close binaries with white dwarf or very low-mass main sequence companions, which must have gone through a common-envelope (CE) phase of evolution. Because the binaries are detached they provide a clean-cut laboratory to study this important but yet poorly understood phase of stellar evolution. Hot subdwarf binaries with sufficiently massive white dwarf companions are viable candidate progenitors of type Ia supernovae both in the double degenerate as well as in the single degenerate scenario as helium donors for double detonation supernovae. The hyper-velocity He-sdO star US 708 may be the surviving donor of such a double detonation supernova. Substellar companions to sdB stars have also been found. For HW Vir systems the companion mass distribution extends from the stellar into the brown dwarf regime. A giant planet to the acoustic-mode pulsator V391 Peg was the first discovery of a planet that survived the red giant evolution of its host star. Evidence for Earth-size planets to two pulsating sdB stars have been reported and circumbinary giant planets or brown dwarfs have been found around HW

  10. The hot Hagedorn Universe

    CERN Document Server

    Rafelski, Johann


    In the context of the half-centenary of Hagedorn temperature and the statistical bootstrap model (SBM) we present a short account of how these insights coincided with the establishment of the hot big-bang model (BBM) and helped resolve some of the early philosophical difficulties. We then turn attention to the present day context and show the dominance of strong interaction quark and gluon degrees of freedom in the early stage, helping to characterize the properties of the hot Universe. We focus attention on the current experimental insights about cosmic microwave background (CMB) temperature fluctuation, and develop a much improved understanding of the neutrino freeze-out, in this way paving the path to the opening of a direct connection of quark-gluon plasma (QGP) physics in the early Universe with the QCD-lattice, and the study of the properties of QGP formed in the laboratory.

  11. The hot chocolate effect

    Energy Technology Data Exchange (ETDEWEB)

    Crawford, Frank S. [Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States); Univ. of California, Berkeley, CA (United States)


    The "hot chocolate effect" was investigated quantitatively, using water. If a tall glass cylinder is filled nearly completely with water and tapped on the bottom with a softened mallet one can detect the lowest longitudinal mode of the water column, for which the height of the water column is one quarter wavelength. If the cylinder is rapidly filled with hot tap water containing dissolved air the pitch of that mode may descend by nearly three octaves during the first few seconds as the air comes out of solution and forms bubbles. Then the pitch gradually rises as the bubbles float to the top. A simple theoretical expression for the pitch ratio is derived and compared with experiment. The agreement is good to within the ten percent accuracy of the experiments.

  12. Hot chocolate effect

    Energy Technology Data Exchange (ETDEWEB)

    Crawford, F.S.


    The ''hot chocolate effect'' was investigated quantitatively, using water. If a tall glass cylinder is filled nearly completely with water and tapped on the bottom with a softened mallet one can detect the lowest longitudinal mode of the water column, for which the height of the water column is one-quarter wavelength. If the cylinder is rapidly filled with hot tap water containing dissolved air the pitch of that mode may descend by nearly three octaves during the first few seconds as the air comes out of solution and forms bubbles. Then the pitch gradually rises as the bubbles float to the top. A simple theoretical expression for the pitch ratio is derived and compared with experiment. The agreement is good to within the 10% accuracy of the experiments.

  13. Hot Spring Metagenomics

    Directory of Open Access Journals (Sweden)

    Olalla López-López


    Full Text Available Hot springs have been investigated since the XIX century, but isolation and examination of their thermophilic microbial inhabitants did not start until the 1950s. Many thermophilic microorganisms and their viruses have since been discovered, although the real complexity of thermal communities was envisaged when research based on PCR amplification of the 16S rRNA genes arose. Thereafter, the possibility of cloning and sequencing the total environmental DNA, defined as metagenome, and the study of the genes rescued in the metagenomic libraries and assemblies made it possible to gain a more comprehensive understanding of microbial communities—their diversity, structure, the interactions existing between their components, and the factors shaping the nature of these communities. In the last decade, hot springs have been a source of thermophilic enzymes of industrial interest, encouraging further study of the poorly understood diversity of microbial life in these habitats.

  14. Peppery Hot Bean Curd

    Institute of Scientific and Technical Information of China (English)


    Peppery Hot Bean Curd is a famous dish that originated in Chengdu,Sichuan Province.Dating back to the year under the reign of Emperor Tongzhi during the Qing Dynasty(1862-1875),a woman chef named Chen created this dish.In Chinese it is called Mapo Bean Curd. Ingredients:Three pieces of bean curd,100 grams lean pork,25 grams green soy beans or garlic

  15. Hot subluminous stars

    CERN Document Server

    Heber, Ulrich


    Hot subluminous stars of spectral type B and O are core helium-burning stars at the blue end of the horizontal branch or have evolved even beyond that stage. Strikingly, the distribution in the Hertzsprung-Russell diagram of He-rich vs. He-poor hot subdwarf stars of the globular clusters omega Cen and NGC~2808 differ from that of their field counterparts. The metal-abundance patterns of hot subdwarfs are typically characterized by strong deficiencies of some lighter elements as well as large enrichments of heavy elements. A large fraction of sdB stars are found in close binaries with white dwarf or very low-mass main sequence companions, which must have gone through a common-envelope phase of evolution.They provide a clean-cut laboratory to study this important but yet purely understood phase of stellar evolution. Substellar companions to sdB stars have also been found. For HW~Vir systems the companion mass distribution extends from the stellar into the brown dwarf regime. A giant planet to the pulsator V391 ...

  16. Solar Hot Water Heater (United States)


    The solar panels pictured below, mounted on a Moscow, Idaho home, are part of a domestic hot water heating system capable of providing up to 100 percent of home or small business hot water needs. Produced by Lennox Industries Inc., Marshalltown, Iowa, the panels are commercial versions of a collector co-developed by NASA. In an effort to conserve energy, NASA has installed solar collectors at a number of its own facilities and is conducting research to develop the most efficient systems. Lewis Research Center teamed with Honeywell Inc., Minneapolis, Minnesota to develop the flat plate collector shown. Key to the collector's efficiency is black chrome coating on the plate developed for use on spacecraft solar cells, the coating prevents sun heat from "reradiating," or escaping outward. The design proved the most effective heat absorber among 23 different types of collectors evaluated in a Lewis test program. The Lennox solar domestic hot water heating system has three main components: the array of collectors, a "solar module" (blue unit pictured) and a conventional water heater. A fluid-ethylene glycol and water-is circulated through the collectors to absorb solar heat. The fluid is then piped to a double-walled jacket around a water tank within the solar module.

  17. Jupiter's Hot, Mushy Moon (United States)

    Taylor, G. Jeffrey


    Jupiter's moon Io is the most volcanically active body in the Solar System. Observations by instruments on the Galileo spacecraft and on telescopes atop Mauna Kea in Hawai'i indicate that lava flows on Io are surprisingly hot, over 1200 oC and possibly as much as 1300 oC; a few areas might have lava flows as hot as 1500 oC. Such high temperatures imply that the lava flows are composed of rock that formed by a very large amount of melting of Io's mantle. This has led Laszlo Keszthelyi and Alfred S. McEwen of the University of Arizona and me to reawaken an old hypothesis that suggests that the interior of Io is a partially-molten mush of crystals and magma. The idea, which had fallen out of favor for a decade or two, explains high-temperature hot spots, mountains, calderas, and volcanic plains on Io. If correct, Io gives us an opportunity to study processes that operate in huge, global magma systems, which scientists believe were important during the early history of the Moon and Earth, and possibly other planetary bodies as well. Though far from proven, the idea that Io has a ocean of mushy magma beneath its crust can be tested with measurements by future spacecraft.

  18. PREFACE: Hot Quarks 2004 (United States)

    Antinori, Federico; Bass, Steffen A.; Bellwied, Rene; Ullrich, Thomas; Velkovska, Julia; Wiedemann, Urs


    Why another conference devoted to ultra-relativistic heavy-ion physics? As we looked around the landscape of the existing international conferences and workshops, we realized that there was not a single one tailored to the people who are most directly involved with the actual research work: students, post-docs, and junior faculty/research scientists. Of course there are schools, but that was not what we had in mind. We wanted a meeting where young researchers could come together to discuss in depth the physics that they are working on without any hindrance. The major conferences have very limited time for discussions which is often shared amongst the most established. This leaves little room for young people to ask their questions and to get the detailed feedback which they deserve and which satisfies their curiosity. A discussion-driven workshop, centering on those without whom there will be no future—that seemed like what was needed. And thus the Hot Quarks workshop was born. The aim of Hot Quarks was to enhance the direct exchange of scientific information among the younger members of the community, from both experiment and theory. Participation was by invitation only in order to emphasize the contributions from junior researchers. This approach makes the workshop unique among the many forums in the field. For young scientists it represented an opportunity for exposure that they would not have had in one of the major conferences. The hope is that this meeting has helped to stimulate the next generation of scientists in our field and, at the same time, strengthened their sense of community. It all came together from 18 24 July 2004, when the 77 participants met at The Inn at Snakedance in the Taos Ski Valley, New Mexico, USA, for the first Hot Quarks workshop. Photograph Participants gather in the sunshine at the foot of the Taos Ski Valley chairlift. By all accounts, Hot Quarks 2004 was a great success. Every participant had the opportunity to present her or

  19. CF Mutation Panel (United States)

    ... Testing; Cystic Fibrosis Transmembrane Conductance Regulator Mutation Analysis; CFTR Mutation Analysis Formal name: Cystic Fibrosis Gene Mutation ... an elevated immunoreactive trypsinogen (IRT) or positive sweat chloride test , to confirm the diagnosis of cystic fibrosis. ...

  20. Hot bitumen grouting rediscovered

    Energy Technology Data Exchange (ETDEWEB)

    Naudts, A. [ECO Grouting Specialists, Grand Valley, ON (Canada)


    The article extols the value of hot bitumen grouting, in conjunction with cement-based grout, as a fast, safe, environmentally-friendly and cost-effective sealant. A major advantage of bitumen grout is that blown bitumen will never wash out. The article discusses the properties and some applications of bitumen grout. A diagram shows an application of bitumen and cement-based grout at a large dam. Examples of preventing water flow in dams, in a coal mine and in a potash mine are also given.

  1. Mitochondrial DNA Mutations Associated with Aminoglycoside Ototoxicity

    Institute of Scientific and Technical Information of China (English)

    GUAN Min-Xin


    The mitochondrial 12S rRNA has been shown to be the hot spot for mutations associated with both aminoglycoside-induced and non-syndromic hearing loss. Of all the mutations, the homoplasmic A1555G and C1494T mutations at a highly conserved decoding region in the 12S rRNA have been associated with aminoglycoside-induced and non-syndromic hearing loss in many families worldwide. The A1555G or C1494T mutation is expected to form novel 1494C-G1555 or 1494U-A1555 base-pair at the highly conserved A-site of 12S rRNA. These transitions make the secondary structure of this RNA more closely resemble the corresponding region of bacterial 16S rRNA. Thus, the new U - A or G-C pair in 12S rRNA created by the C1494T or A1555G transition facilitates the binding of aminoglycosides, thereby accounting for the fact that the exposure to aminoglycosides can induce or worsen hearing loss in individuals carrying these mutations. Furthermore, the growth defect and impairment of mitochondrial translation were observed in cell lines carrying the A1555G or C1494T mutation in the presence of high concentration of aminoglycosides. In addition, nuclear modifier genes and mitochondrial haplotypes modulate the phenotypic manifestation of the A1555G and C1494T mutations. These observations provide the direct genetic and biochemical evidences that the A1555G or C1494T mutation is a pathogenic mtDNA mutation associated with aminoglycoside-induced and nonsyndromic hearing loss. Therefore, these data have been providing valuable information and technology to predict which individuals are at risk for ototoxicity, to improve the safety of aminoglycoside antibiotic therapy, and eventually to decrease the incidence of deafness.

  2. The molecular anatomy of spontaneous germline mutations in human testes. (United States)

    Qin, Jian; Calabrese, Peter; Tiemann-Boege, Irene; Shinde, Deepali Narendra; Yoon, Song-Ro; Gelfand, David; Bauer, Keith; Arnheim, Norman


    The frequency of the most common sporadic Apert syndrome mutation (C755G) in the human fibroblast growth factor receptor 2 gene (FGFR2) is 100-1,000 times higher than expected from average nucleotide substitution rates based on evolutionary studies and the incidence of human genetic diseases. To determine if this increased frequency was due to the nucleotide site having the properties of a mutation hot spot, or some other explanation, we developed a new experimental approach. We examined the spatial distribution of the frequency of the C755G mutation in the germline by dividing four testes from two normal individuals each into several hundred pieces, and, using a highly sensitive PCR assay, we measured the mutation frequency of each piece. We discovered that each testis was characterized by rare foci with mutation frequencies 10(3) to >10(4) times higher than the rest of the testis regions. Using a model based on what is known about human germline development forced us to reject (p < 10(-6)) the idea that the C755G mutation arises more frequently because this nucleotide simply has a higher than average mutation rate (hot spot model). This is true regardless of whether mutation is dependent or independent of cell division. An alternate model was examined where positive selection acts on adult self-renewing Ap spermatogonial cells (SrAp) carrying this mutation such that, instead of only replacing themselves, they occasionally produce two SrAp cells. This model could not be rejected given our observed data. Unlike the disease site, similar analysis of C-to-G mutations at a control nucleotide site in one testis pair failed to find any foci with high mutation frequencies. The rejection of the hot spot model and lack of rejection of a selection model for the C755G mutation, along with other data, provides strong support for the proposal that positive selection in the testis can act to increase the frequency of premeiotic germ cells carrying a mutation deleterious to an

  3. The molecular anatomy of spontaneous germline mutations in human testes.

    Directory of Open Access Journals (Sweden)

    Jian Qin


    Full Text Available The frequency of the most common sporadic Apert syndrome mutation (C755G in the human fibroblast growth factor receptor 2 gene (FGFR2 is 100-1,000 times higher than expected from average nucleotide substitution rates based on evolutionary studies and the incidence of human genetic diseases. To determine if this increased frequency was due to the nucleotide site having the properties of a mutation hot spot, or some other explanation, we developed a new experimental approach. We examined the spatial distribution of the frequency of the C755G mutation in the germline by dividing four testes from two normal individuals each into several hundred pieces, and, using a highly sensitive PCR assay, we measured the mutation frequency of each piece. We discovered that each testis was characterized by rare foci with mutation frequencies 10(3 to >10(4 times higher than the rest of the testis regions. Using a model based on what is known about human germline development forced us to reject (p < 10(-6 the idea that the C755G mutation arises more frequently because this nucleotide simply has a higher than average mutation rate (hot spot model. This is true regardless of whether mutation is dependent or independent of cell division. An alternate model was examined where positive selection acts on adult self-renewing Ap spermatogonial cells (SrAp carrying this mutation such that, instead of only replacing themselves, they occasionally produce two SrAp cells. This model could not be rejected given our observed data. Unlike the disease site, similar analysis of C-to-G mutations at a control nucleotide site in one testis pair failed to find any foci with high mutation frequencies. The rejection of the hot spot model and lack of rejection of a selection model for the C755G mutation, along with other data, provides strong support for the proposal that positive selection in the testis can act to increase the frequency of premeiotic germ cells carrying a mutation

  4. On the Hot Money Trail

    Institute of Scientific and Technical Information of China (English)


    The huge influx of international hot money is threatening inflation and affecting the country’s monetary policy In the last three months, the country’s financial supervisory departments have conducted frequent but atypical investi-gations of hot money.

  5. How hot is the sun

    Institute of Scientific and Technical Information of China (English)



    Do you know how hot thesun is? There are no solidsor liquids on the sun. Why not? The temperature onoutside the sun is more than 10, 000℃, and that at the centre is about 20, 000, 000℃.The sun is so hot that all thesolids and all the liquids havebeen turned into gases.

  6. The spectrum of Familial Mediterranean Fever (FMF) mutations. (United States)

    Touitou, I


    Familial Mediterranean Fever (FMF) is the prototype of a group of inherited inflammatory disorders. The gene (MEFV) responsible for this disease, comprises 10 exons and 781 codons. Twenty-nine mutations, most located in the last exon, have been identified so far. It is unclear whether all are true disease-causing mutations. Five founder mutations, V726A, M694V, M694I, M680I and E148Q account for 74% of FMF chromosomes from typical cases (Armenians, Arabs, Jews, and Turks). Rare mutations are preferentially found in populations not usually affected by FMF (eg Europeans not from the above ancestries). The various combinations of MEFV mutations define severe to mild genotypes. The trend is that genotypes including two mutations located within mutational 'hot-spots' (codons 680 or 694) of the gene are associated with severe phenotypes, whereas mild phenotypes are associated with some other mutations, E148Q being the mildest and least penetrant. Understanding the correlation between the FMF phenotype and genotype is further obscured by the existence of complex alleles, modifier loci, genetic heterogeneity and possible epigenetic factors. Additionally, mutations in the MEFV gene are thought to be involved in non FMF disorders. Carrier rates for FMF mutations may be as high as 1:3 in some populations, suggesting that the disease is underdiagnosed. This review update emphasises that both clinical and genetic features are to be taken into account for patient diagnosis, colchicine treatment and prognosis.

  7. Really Hot Stars (United States)


    Spectacular VLT Photos Unveil Mysterious Nebulae Summary Quite a few of the most beautiful objects in the Universe are still shrouded in mystery. Even though most of the nebulae of gas and dust in our vicinity are now rather well understood, there are some which continue to puzzle astronomers. This is the case of a small number of unusual nebulae that appear to be the subject of strong heating - in astronomical terminology, they present an amazingly "high degree of excitation". This is because they contain significant amounts of ions, i.e., atoms that have lost one or more of their electrons. Depending on the atoms involved and the number of electrons lost, this process bears witness to the strength of the radiation or to the impact of energetic particles. But what are the sources of that excitation? Could it be energetic stars or perhaps some kind of exotic objects inside these nebulae? How do these peculiar objects fit into the current picture of universal evolution? New observations of a number of such unusual nebulae have recently been obtained with the Very Large Telescope (VLT) at the ESO Paranal Observatory (Chile). In a dedicated search for the origin of their individual characteristics, a team of astronomers - mostly from the Institute of Astrophysics & Geophysics in Liège (Belgium) [1] - have secured the first detailed, highly revealing images of four highly ionized nebulae in the Magellanic Clouds, two small satellite galaxies of our home galaxy, the Milky Way, only a few hundred thousand light-years away. In three nebulae, they succeeded in identifying the sources of energetic radiation and to eludicate their exceptional properties: some of the hottest, most massive stars ever seen, some of which are double. With masses of more than 20 times that of the Sun and surface temperatures above 90 000 degrees, these stars are truly extreme. PR Photo 09a/03: Nebula around the hot star AB7 in the SMC. PR Photo 09b/03: Nebula near the hot Wolf-Rayet star BAT99

  8. Repetitious-Hot-Pressing Technique in Hot-Pressing Process

    Institute of Scientific and Technical Information of China (English)

    Shixue SONG; Xing AI; Wei GAO; Jun ZHAO


    A new pressing method was proposed for hot-pressing process. Experimental results indicated that the porosity in Al2O3/TiC/Ni/Mo (hereafter called Al2O3/TiC composite) composite compacts decreases by 6% after adopting this new technique,compared to traditional hot-pressing technique under the same sintering temperature. The flexural strength and Vickerhardness increase from 883 MPa to 980 MPa and from 16 GPa to 21.1 GPa, respectively. A theoretical model was given toanalyze the densification mechanism of the composite in the process of repetitious-hot-pressing.

  9. Prevalence of Mitochondrial 12S rRNA Mutations Associated with Aminoglycoside Ototoxicity (United States)

    Guan, Min-Xin


    The mitochondrial DNA (mtDNA) 12S rRNA is a hot spot for mutations associated with both aminoglycoside-induced and nonsyndromic hearing loss. Of those, the homoplasmic A1555G and C1494T mutations at a highly conserved decoding region of the 12S rRNA have been associated with hearing loss. These two mutations account for a significant number of…

  10. A germ-line-selective advantage rather than an increased mutation rate can explain some unexpectedly common human disease mutations. (United States)

    Choi, Soo-Kyung; Yoon, Song-Ro; Calabrese, Peter; Arnheim, Norman


    Two nucleotide substitutions in the human FGFR2 gene (C755G or C758G) are responsible for virtually all sporadic cases of Apert syndrome. This condition is 100-1,000 times more common than genomic mutation frequency data predict. Here, we report on the C758G de novo Apert syndrome mutation. Using data on older donors, we show that spontaneous mutations are not uniformly distributed throughout normal testes. Instead, we find foci where C758G mutation frequencies are 3-4 orders of magnitude greater than the remaining tissue. We conclude this nucleotide site is not a mutation hot spot even after accounting for possible Luria-Delbruck "mutation jackpots." An alternative explanation for such foci involving positive selection acting on adult self-renewing Ap spermatogonia experiencing the rare mutation could not be rejected. Further, the two youngest individuals studied (19 and 23 years old) had lower mutation frequencies and smaller foci at both mutation sites compared with the older individuals. This implies that the mutation frequency of foci increases as adults age, and thus selection could explain the paternal age effect for Apert syndrome and other genetic conditions. Our results, now including the analysis of two mutations in the same set of testes, suggest that positive selection can increase the relative frequency of premeiotic germ cells carrying such mutations, although individuals who inherit them have reduced fitness. In addition, we compared the anatomical distribution of C758G mutation foci with both new and old data on the C755G mutation in the same testis and found their positions were not correlated with one another.

  11. Hot Hydrogen Test Facility (United States)

    Swank, W. David; Carmack, Jon; Werner, James E.; Pink, Robert J.; Haggard, DeLon C.; Johnson, Ryan


    The core in a nuclear thermal rocket will operate at high temperatures and in hydrogen. One of the important parameters in evaluating the performance of a nuclear thermal rocket is specific impulse, ISP. This quantity is proportional to the square root of the propellant's absolute temperature and inversely proportional to square root of its molecular weight. Therefore, high temperature hydrogen is a favored propellant of nuclear thermal rocket designers. Previous work has shown that one of the life-limiting phenomena for thermal rocket nuclear cores is mass loss of fuel to flowing hydrogen at high temperatures. The hot hydrogen test facility located at the Idaho National Lab (INL) is designed to test suitability of different core materials in 2500°C hydrogen flowing at 1500 liters per minute. The facility is intended to test low activity uranium containing materials but is also suited for testing cladding and coating materials. In this first installment the facility is described. Automated data acquisition, flow and temperature control, vessel compatibility with various core geometries and overall capabilities are discussed.

  12. Charmonium in Hot Medium

    CERN Document Server

    Zhao, Xingbo


    We investigate charmonium production in the hot medium created by heavy-ion collisions by setting up a framework in which in-medium charmonium properties are constrained by thermal lattice QCD (lQCD) and subsequently implemented into kinetic approaches. A Boltzmann transport equation is employed to describe the time evolution of the charmonium phase space distribution with the loss and gain term accounting for charmonium dissociation and regeneration (from charm quarks), respectively. The momentum dependence of the charmonium dissociation rate is worked out. The dominant process for in-medium charmonium regeneration is found to be a 3-to-2 process. Its corresponding regeneration rates from different input charm-quark momentum spectra are evaluated. Experimental data on $J/\\psi$ production at CERN-SPS and BNL-RHIC are compared with our numerical results in terms of both rapidity-dependent inclusive yields and transverse momentum ($p_t$) spectra. Within current uncertainties from (interpreting) lQCD data and fr...

  13. Hot scalar electrodynamics as a toy model for hot QCD

    CERN Document Server

    Krämmer, U; Schulz, H; Kraemmer, Ulrike; Rebhan, Anton K; Schulz, Hermann


    Hot scalar electrodynamics is adopted as a toy model for a hot gluon plasma to display some aspects of the compulsory resummation of hard thermal loops when next-to-leading order quantities at soft momentum scales are to be calculated. [Talk given by A.K.R. at a one-day meeting dedicated to the memory of Tanguy ALTHERR, held on November 4, 1994 at CERN, Geneva. To appear in a Gedenkschrift published by World Scientific.

  14. Transgenic Animal Mutation Assays

    Institute of Scientific and Technical Information of China (English)

    Tao Chen; Ph.D.D.A.B.T.


    @@ The novel transgenic mouse and rat mutation assays have provided a tool for analyzing in vivo mutation in any tissue, thus permitting the direct comparison of cancer incidence with mutant frequency.

  15. Abstract 5324: Pan-cancer identification of mutated pathways and protein complexes

    DEFF Research Database (Denmark)

    Leiserson, Mark D.; Vandin, Fabio; Wu, Hsin-Ta


    that are mutually exclusive across the tumor cohort. There are numerous examples of mutually exclusive mutations between interacting proteins; e.g. BRAF and KRAS in colorectal cancer. Dendrix++ generalizes this idea to find larger groups of mutually exclusive mutations.We applied HotNet2 and Dendrix++ to whole......Large-scale cancer sequencing efforts such as The Cancer Genome Atlas (TCGA) and others have shown that tumors exhibit extensive mutational heterogeneity with relatively few genes mutated at significant frequency and many genes mutated in only a small number of individuals. This long tail...... phenomenon complicates the identification of driver mutations by their observed frequency. The long tail is explained in part by the fact that driver mutations target genes in signaling and regulatory pathways, and these pathways may be perturbed by different mutations in different tumors.We developed two...

  16. Small Friends of Hot Jupiters (United States)

    Nunez, Luis Ernesto; Johnson, John A.


    Hot Jupiters are Jupiter-sized gas giant exoplanets that closely orbit their host star in periods of about 10 days or less. Early models hypothesized that these exoplanets formed away from the star, then over time drifted to their characteristically closer locations. However, new theories predict that Hot Jupiters form at their close proximity during the process of core accretion (Batygin et al. 2015). In fact, a super-Earth and a Neptune-sized exoplanet have already been detected in the Hot Jupiter-hosting star WASP-47 (Becker et al. 2015). We will present our analysis of radial velocity time series plots to determine whether low-mass, short-period planets have been previously overlooked in systems of stars which host Hot Jupiters.The SAO REU program is funded in part by the National Science Foundation REU and Department of Defense ASSURE programs under NSF Grant no. 1262851.

  17. Hot dry rock geothermal energy (United States)

    Heiken, G.; Murphy, H.; Nunz, G.; Potter, R.


    Man-made geothermal systems are discussed which make it possible to extract heat from hot rocks in areas where natural fluids are insufficient for the development of hydrothermal energy. The location and magnitude of high- and low-temperature geothermal resources in the USA for such hot dry rock (HDR) systems are examined. An HDR concept is described in which water is injected into one of two nearly parallel wells connected at depth by man-made fractures; the injected water circulates through the fracture system, where it is heated by conduction from the hot rock, and hot fluid, which can be used for heating or for electric power generation, rises through the second well. Some heat-extraction experiments using the described concept are reviewed which are being conducted in a complex volcanic field in New Mexico. The economics of HDR energy is evaluated.

  18. Do scientists trace hot topics? (United States)

    Wei, Tian; Li, Menghui; Wu, Chensheng; Yan, Xiao-Yong; Fan, Ying; Di, Zengru; Wu, Jinshan


    Do scientists follow hot topics in their scientific investigations? In this paper, by performing analysis to papers published in the American Physical Society (APS) Physical Review journals, it is found that papers are more likely to be attracted by hot fields, where the hotness of a field is measured by the number of papers belonging to the field. This indicates that scientists generally do follow hot topics. However, there are qualitative differences among scientists from various countries, among research works regarding different number of authors, different number of affiliations and different number of references. These observations could be valuable for policy makers when deciding research funding and also for individual researchers when searching for scientific projects.

  19. Coulomb explosion of "hot spot"

    CERN Document Server

    Oreshkin, V I; Chaikovsky, S A; Artyomov, A P


    The study presented in this paper has shown that the generation of hard x rays and high-energy ions, which are detected in pinch implosion experiments, may be associated with the Coulomb explosion of the hot spot that is formed due to the outflow of the material from the pinch cross point. During the process of material outflow, the temperature of the hot spot plasma increases, and conditions arise for the plasma electrons to become continuously accelerated. The runaway of electrons from the hot spot region results in the buildup of positive space charge in this region followed by a Coulomb explosion. The conditions for the hot spot plasma electrons to become continuously accelerated have been revealed and estimates have been obtained for the kinetic energy of the ions generated by the Coulomb explosion.

  20. Quantitative spectroscopy of hot stars (United States)

    Kudritzki, R. P.; Hummer, D. G.


    A review on the quantitative spectroscopy (QS) of hot stars is presented, with particular attention given to the study of photospheres, optically thin winds, unified model atmospheres, and stars with optically thick winds. It is concluded that the results presented here demonstrate the reliability of Qs as a unique source of accurate values of the global parameters (effective temperature, surface gravity, and elemental abundances) of hot stars.

  1. Maize Mutator transposon

    Institute of Scientific and Technical Information of China (English)

    Yijun WANG; Mingliang XU; Dexiang DENG; Yunlong BIAN


    Transposable elements are widely distributed in eukaryotes. Due to its high copy numbers, high forward mutation rate and preferential insertion into low-copy DNA sequences, among others, the Mutator system has been widely used as a mutagen in genomic research. The discovery, classification, transposition specificity and epige-netic regulation of Mutator transposons were described. The application of Mutator tagging in plant genomic research was also presented. The role of Mu-like elements in genome evolution was briefly depicted. Moreover, the direction of Mutator transposon research in the future was discussed.

  2. Hot Alps (Invited) (United States)

    Speranza, F.; Minelli, L.; Pignatelli, A.; Gilardi, M.


    Although it is frequently assumed that crust of Alpine orogens is hot due to the occurrence of thick and young (hence radiogenic) crust, evidence on the thermal ranking of orogens is contradictory. Heat flow measurements from shallow wells (depth ≤ 1 km) in the Alps yield a relatively cold thermal regime of 50-80 mW/m2, but data are likely biased by meteoric cold-water circulation. Here we report on the spectral analysis of the aeromagnetic residuals of northern Italy to derive the Curie point depth (CPD), assumed to represent the 600°C isotherm depth. Airborne magnetics were acquired on whole Italy during the 1970s by the national oil company AGIP (now Eni). Data were gathered by several surveys carried out at 1000-13,300 feet (300-4000 m) altitude, with flight line spacing of 2-10 km. Surveys of the Alps and Po Plain (northern Italy) were obtained both with a line spacing of 5 km (and 5 km tie lines), at an altitude of 4000-5000 and 13,300 feet, respectively. To evaluate CPDs we used the centroid method (routinely adopted in recent CPD studies on East Asia and central-southern Europe) on 72 square windows of 100-110 km edge, with a 50% degree of superposition. CPDs vary between 16 and 38 km (22 km on average) in the Po Plain, located south of the Alps and representing the Adriatic-African foreland area. Conversely, the Alps yield very shallow CPDs, ranging between 6 and 15 km (10 km on average). CPDs fall systematically above local Moho depths, implying that magnetic source bottoms documented in this study do not represent a lithological boundary over non-magnetic peridotitic mantle, but can be safely associated with CPDs and the 600°C isotherm. CPDs from the Po Plain are in rough agreement with reported heat flow values of 25-60 mW/m2, and imply and average thermal conductivity (k) of the Po Plain crust of 1.5 W/m°K, at the lower bound of k values measured and inferred for the crust. Conversely, the average 10 km CPD documented in the Alps translates into

  3. IDH Mutation Analysis in Ewing Sarcoma Family Tumors

    Directory of Open Access Journals (Sweden)

    Ki Yong Na


    Full Text Available Background: Isocitrate dehydrogenase (IDH catalyzes the oxidative decarboxylation of isocitrate to yield α-ketoglutarate (α-KG with production of reduced nicotinamide adenine dinucleotide (NADH. Dysfunctional IDH leads to reduced production of α-KG and NADH and increased production of 2-hydroxyglutarate, an oncometabolite. This results in increased oxidative damage and stabilization of hypoxia-inducible factor α, causing cells to be prone to tumorigenesis. Methods: This study investigated IDH mutations in 61 Ewing sarcoma family tumors (ESFTs, using a pentose nucleic acid clamping method and direct sequencing. Results: We identified four cases of ESFTs harboring IDH mutations. The number of IDH1 and IDH2 mutations was equal and the subtype of IDH mutations was variable. Clinicopathologic analysis according to IDH mutation status did not reveal significant results. Conclusions: This study is the first to report IDH mutations in ESFTs. The results indicate that ESFTs can harbor IDH mutations in previously known hot-spot regions, although their incidence is rare. Further validation with a larger case-based study would establish more reliable and significant data on prevalence rate and the biological significance of IDH mutations in ESFTs.

  4. De novo mutation of keratin 9 gene in two Taiwanese patients with epidermolytic palmoplantar keratoderma. (United States)

    Yang, Mei-Hui; Lee, Julia Yu-Yun; Lin, Jeng-Hsien; Chao, Sheau-Chiou


    Epidermolytic palmoplantar keratoderma (EPPK) is an autosomal dominant hereditary disorder of keratinization. Recent molecular studies have shown that EPPK is caused by mutations in keratin 9 gene (K9). We report 2 unrelated sporadic cases of EPPK in Taiwanese, confirmed by histopathology and electron microscopy. A de novo mutation with a C to T transition at the first nucleotide of codon 162 in K9 was detected in both patients, but not in their parents. The mutation is expected to result in an arginine to tryptophan substitution (R162W) in the beginning region of the alpha-helical 1A domain of K9. Mutations in this region could disrupt keratin filament assembly, leading to degeneration or cytolysis of keratinocytes. Mutations of this arginine codon (R162W, R162Q) are common in pedigrees with EPPK. Our mutation analysis suggests that codon 162 in K9 gene is an important hot spot for mutation in EPPK.

  5. Software Simulation of Hot Tearing

    DEFF Research Database (Denmark)

    Andersen, S.; Hansen, P.N.; Hattel, Jesper Henri


    the solidification rate and the strain rate of the hot tear prone areas. But, until recently it was only possible to simulate the solidification rate, so that the criteria could not be used effectively.Today, with new software developments, it is possible to also simulate the strain rate in the hot tear prone areas....... With this additional information, the criteria can, for the first time, be used to their full potential.The purpose of this paper is to first give an introduction to a stress/strain simulation procedure that can be used in any foundry. Then, some results how to predict the hot cracking tendency in a casting are shown......, and the use of simulation to reduce this tendency is illustrated....

  6. Promethus Hot Leg Piping Concept

    Energy Technology Data Exchange (ETDEWEB)

    AM Girbik; PA Dilorenzo


    The Naval Reactors Prime Contractor Team (NRPCT) recommended the development of a gas cooled reactor directly coupled to a Brayton energy conversion system as the Space Nuclear Power Plant (SNPP) for NASA's Project Prometheus. The section of piping between the reactor outlet and turbine inlet, designated as the hot leg piping, required unique design features to allow the use of a nickel superalloy rather than a refractory metal as the pressure boundary. The NRPCT evaluated a variety of hot leg piping concepts for performance relative to SNPP system parameters, manufacturability, material considerations, and comparison to past high temperature gas reactor (HTGR) practice. Manufacturability challenges and the impact of pressure drop and turbine entrance temperature reduction on cycle efficiency were discriminators between the piping concepts. This paper summarizes the NRPCT hot leg piping evaluation, presents the concept recommended, and summarizes developmental issues for the recommended concept.

  7. Three kinds of mutation

    CERN Document Server

    Buan, Aslak Bakke; Thomas, Hugh


    For a finite dimensional hereditary algebra, we consider: exceptional sequences in the category of finite dimensional modules, silting objects in the bounded derived category, and m-cluster tilting objects in the m-cluster category. There are mutation operations on both the set of m-cluster tilting objects and the set of exceptional sequences. It is also possible to define a mutation operation for silting objects. We compare these three different notions of mutation.

  8. Swift, UVOT and Hot Stars

    CERN Document Server

    Siegel, Michael H; Hagen, Lea M Z; Hoversten, Erik A


    We present the results of our ongoing investigation into the properties of hot stars and young stellar populations using the Swift/UVOT telescope. We present UVOT photometry of open and globular clusters and show that UVOT is capable of characterizing a variety of rare hot stars, including Post-Asymptotic Giant Branch and Extreme Horizontal Branch Stars. We also present very early reults of our survey of stellar populations in the Small Magellanic Cloud. We find that the SMC has experienced recent bouts of star formation but constraining the exact star formation history will depend on finding an effective model of the reddening within the SMC.

  9. Monopole transitions in hot nuclei

    Energy Technology Data Exchange (ETDEWEB)

    Sujkowski, Z. [Soltan Inst. for Nuclear Studies, Otwock-Swierk (Poland)


    Monopole transitions can be a signature of shape changing in a hot, pulsating nucleus (the low energy E0 mode) and/or a measure of the compressibility of finite nuclei (GMR, the breathing mode). Experimental information pertaining to GMR is reviewed. Recipes for deducing the incompressibility modules for infinite nuclear matter from data on GMR are discussed. Astrophysical implications are outlined. The first attempts at locating the GMR strength in moderately hot nuclei are described. Prospects for improving the experimental techniques to make an observation of this strength in selected nuclei unambiguous are discussed. (author). 46 refs, 8 figs.

  10. Hot conditioning equipment conceptual design report

    Energy Technology Data Exchange (ETDEWEB)

    Bradshaw, F.W., Westinghouse Hanford


    This report documents the conceptual design of the Hot Conditioning System Equipment. The Hot conditioning System will consist of two separate designs: the Hot Conditioning System Equipment; and the Hot Conditioning System Annex. The Hot Conditioning System Equipment Design includes the equipment such as ovens, vacuum pumps, inert gas delivery systems, etc.necessary to condition spent nuclear fuel currently in storage in the K Basins of the Hanford Site. The Hot Conditioning System Annex consists of the facility of house the Hot Conditioning System. The Hot Conditioning System will be housed in an annex to the Canister Storage Building. The Hot Conditioning System will consist of pits in the floor which contain ovens in which the spent nuclear will be conditioned prior to interim storage.

  11. Diverse growth hormone receptor gene mutations in Laron syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Berg, M.A.; Francke, U. (Stanford Univ. School of Medicine, CA (United States)); Gracia, R.; Rosenbloom, A.; Toledo, S.P.A. (Univ. Autonoma, Madrid (Spain)); Chernausek, S. (Children' s Hospital Medical Center, Cincinnati, OH (United States)); Guevara-Aguirre, J. (Institute of Endocrinology, Metabolism, and Reproduction, Quito (Ecuador)); Hopp, M. (Univ. of Witwatersrand, Johannesburg (South Africa)); Rosenbloom, A.; Argente, J. (Univ. of Florida, Gainesville (United States)); Toledo, S.P.A. (Univ. of Sao Paulo (Brazil))


    To better understand the molecular genetic basis and genetic epidemiology of Laron syndrome (growth-hormone insensitivity syndrome), the authors analysed the growth-hormone receptor (GHR) genes of seven unrelated affected individuals from the United States, South America, Europe, and Africa. They amplified all nine GHR gene exons and splice junctions from these individuals by PCR and screened the products for mutations by using denaturing gradient gel electrophoresis (DGGE). They identified a single GHR gene fragment with abnormal DGGE results for each affected individual, sequenced this fragment, and, in each case, identified a mutation likely to cause Laron syndrome, including two nonsense mutations (R43X and R217X), two splice-junction mutations, (189-1 G to T and 71+1 G to A), and two frameshift mutations (46 del TT and 230 del TA or AT). Only one of these mutations, R43X, has been previously reported. Using haplotype analysis, they determined that this mutation, which involves a CpG dinucleotide hot spot, likely arose as a separate event in this case, relative to the two prior reports of R43X. Aside from R43X, the mutations identified are unique to patients from particular geographic regions. Ten GHR gene mutations have now been described in this disorder. The authors conclude that Laron syndrome is caused by diverse GHR gene mutations, including deletions, RNA processing defects, translational stop codons, and missense codons. All the identified mutations involve the extracellular domain of the receptor, and most are unique to particular families or geographic areas. 35 refs., 3 figs., 1 tab.

  12. Distinct Rayleigh scattering from hot spot mutant p53 proteins reveals cancer cells. (United States)

    Jun, Ho Joon; Nguyen, Anh H; Kim, Yeul Hong; Park, Kyong Hwa; Kim, Doyoun; Kim, Kyeong Kyu; Sim, Sang Jun


    The scattering of light redirects and resonances when an electromagnetic wave interacts with electrons orbits in the hot spot core protein and oscillated electron of the gold nanoparticles (AuNP). This report demonstrates convincingly that resonant Rayleigh scattering generated from hot spot mutant p53 proteins is correspondence to cancer cells. Hot spot mutants have unique local electron density changes that affect specificity of DNA binding affinity compared with wild types. Rayleigh scattering changes introduced by hot-spot mutations were monitored by localized surface plasmon resonance (LSPR) shift changes. The LSPR λmax shift for hot-spot mutants ranged from 1.7 to 4.2 nm for mouse samples and from 0.64 nm to 2.66 nm for human samples, compared to 9.6 nm and 15 nm for wild type and mouse and human proteins, respectively with a detection sensitivity of p53 concentration at 17.9 nM. It is interesting that hot-spot mutants, which affect only interaction with DNA, launches affinitive changes as considerable as wild types. These changes propose that hot-spot mutants p53 proteins can be easily detected by local electron density alterations that disturbs the specificity of DNA binding of p53 core domain on the surface of the DNA probed-nanoplasmonic sensor.

  13. Prediction of protein interaction hot spots using rough set-based multiple criteria linear programming. (United States)

    Chen, Ruoying; Zhang, Zhiwang; Wu, Di; Zhang, Peng; Zhang, Xinyang; Wang, Yong; Shi, Yong


    Protein-protein interactions are fundamentally important in many biological processes and it is in pressing need to understand the principles of protein-protein interactions. Mutagenesis studies have found that only a small fraction of surface residues, known as hot spots, are responsible for the physical binding in protein complexes. However, revealing hot spots by mutagenesis experiments are usually time consuming and expensive. In order to complement the experimental efforts, we propose a new computational approach in this paper to predict hot spots. Our method, Rough Set-based Multiple Criteria Linear Programming (RS-MCLP), integrates rough sets theory and multiple criteria linear programming to choose dominant features and computationally predict hot spots. Our approach is benchmarked by a dataset of 904 alanine-mutated residues and the results show that our RS-MCLP method performs better than other methods, e.g., MCLP, Decision Tree, Bayes Net, and the existing HotSprint database. In addition, we reveal several biological insights based on our analysis. We find that four features (the change of accessible surface area, percentage of the change of accessible surface area, size of a residue, and atomic contacts) are critical in predicting hot spots. Furthermore, we find that three residues (Tyr, Trp, and Phe) are abundant in hot spots through analyzing the distribution of amino acids.

  14. Gestational mutations in radiation carcinogenesis (United States)

    Meza, R.; Luebeck, G.; Moolgavkar, S.

    Mutations in critical genes during gestation could increase substantially the risk of cancer. We examine the consequences of such mutations using the Luebeck-Moolgavkar model for colorectal cancer and the Lea-Coulson modification of the Luria-Delbruck model for the accumulation of mutations during gestation. When gestational mutation rates are high, such mutations make a significant contribution to cancer risk even for adult tumors. Furthermore, gestational mutations ocurring at distinct times during emryonic developmemt lead to substantially different numbers of mutated cells at birth, with early mutations leading to a large number (jackpots) of mutated cells at birth and mutation occurring late leading to only a few mutated cells. Thus gestational mutations could confer considerable heterogeneity of the risk of cancer. If the fetus is exposed to an environmental mutagen, such as ionizing radiation, the gestational mutation rate would be expected to increase. We examine the consequences of such exposures during gestation on the subsequent development of cancer.

  15. Types of Hot Jupiter Atmospheres (United States)

    Bisikalo, Dmitry V.; Kaygorodov, Pavel V.; Ionov, Dmitry E.; Shematovich, Valery I.

    Hot Jupiters, i.e. exoplanet gas giants, having masses comparable to the mass of Jupiter and semimajor axes shorter than 0.1 AU, are a unique class of objects. Since they are so close to the host stars, their atmospheres form and evolve under the action of very active gas dynamical processes caused by the gravitational field and irradiation of the host star. As a matter of fact, the atmospheres of several of these planets fill their Roche lobes , which results in a powerful outflow of material from the planet towards the host star. The energy budget of this process is so important that it almost solely governs the evolution of hot Jupiters gaseous envelopes. Based on the years of experience in the simulations of gas dynamics in mass-exchanging close binary stars, we have investigated specific features of hot Jupiters atmospheres. The analytical estimates and results of 3D numerical simulations, discussed in this Chapter, show that the gaseous envelopes around hot Jupiters may be significantly non-spherical and, at the same time, stationary and long-lived. These results are of fundamental importance for the interpretation of observational data.

  16. Sources of antibiotics: Hot springs. (United States)

    Mahajan, Girish B; Balachandran, Lakshmi


    The discovery of antibiotics heralded an era of improved health care. However, the over-prescription and misuse of antibiotics resulted in the development of resistant strains of various pathogens. Since then, there has been an incessant search for discovering novel compounds from bacteria at various locations with extreme conditions. The soil is one of the most explored locations for bioprospecting. In recent times, hypersaline environments and symbiotic associations have been investigated for novel antimicrobial compounds. Among the extreme environments, hot springs are comparatively less explored. Many researchers have reported the presence of microbial life and secretion of antimicrobial compounds by microorganisms in hot springs. A pioneering research in the corresponding author's laboratory resulted in the identification of the antibiotic Fusaricidin B isolated from a hot spring derived eubacteria, Paenibacillus polymyxa, which has been assigned a new application for its anti-tubercular properties. The corresponding author has also reported anti-MRSA and anti-VRE activity of 73 bacterial isolates from hot springs in India. Copyright © 2016 Elsevier Inc. All rights reserved.

  17. The puzzling MILAGRO hot spots

    CERN Document Server

    Drury, Luke


    We discuss the reported detection by the MILAGRO experiment of localised hot spots in the cosmic ray arrival distribution and the difficulty of interpreting these observations. A model based on secondary neutron production in the heliotail is shown to fail. An alternative model based on loss-cone leakage through a magnetic trap from a local source region is proposed.

  18. Microsensor Hot-Film Anemometer (United States)

    Mcginley, Catherine B.; Stephens, Ralph; Hopson, Purnell; Bartlett, James E.; Sheplak, Mark; Spina, Eric F.


    Improved hot-film anemometer developed for making high-bandwidth turbulence measurements in moderate-enthalpy supersonic and hypersonic flows (e.g., NASP inlets and control surfaces, HSCT jet exhaust). Features include low thermal inertia, ruggedness, and reduced perturbation of flow.

  19. Mutation and premating isolation. (United States)

    Woodruff, R C; Thompson, J N


    While premating isolation might be traceable to different genetic mechanisms in different species, evidence supports the idea that as few as one or two genes may often be sufficient to initiate isolation. Thus, new mutation can theoretically play a key role in the process. But it has long been thought that a new isolation mutation would fail, because there would be no other individuals for the isolation-mutation-carrier to mate with. We now realize that premeiotic mutations are very common and will yield a cluster of progeny carrying the same new mutant allele. In this paper, we discuss the evidence for genetically simple premating isolation barriers and the role that clusters of an isolation mutation may play in initiating allopatric, and even sympatric, species divisions.

  20. Mutation spectrum of NF1 and clinical characteristics in 78 Korean patients with neurofibromatosis type 1. (United States)

    Ko, Jung Min; Sohn, Young Bae; Jeong, Seon Yong; Kim, Hyon-Ju; Messiaen, Ludwine M


    Neurofibromatosis type 1 (NF1) is one of the most common autosomal dominant disorders in humans. NF1 is caused by mutations of the NF1 gene. Mutation detection is complex owing to the large size of the NF1 gene, the presence of pseudogenes, and the great variety of mutations. Also, few probable genotype-phenotype correlations have been found in NF1. In this study 78 Korean patients from 60 families were screened for NF1 mutations. Mutation analysis of the entire coding region and flanking splice sites was carried out and included the use of a combination of reverse transcription polymerase chain reaction, multiplex ligation probe amplification, or fluorescence in situ hybridization. Mutation spectrum and genotype-phenotype relationship were assessed. Fifty-two distinct NF1 mutations were identified in 60 families. The mutations included 30 single base substitutions (12 missense and 18 nonsense), 11 missplicing mutations, seven small insertion or deletions, and four gross deletions. Sixteen (30.8%) mutations were novel; c.1A>G, c.2033_2034insC, c.2540T>C, c.4537C>T, c.5546G>A, c.6792C>A, and c.6792C>G were recurrently identified. The mutations were evenly distributed across exon 1 through intron 47 of NF1, and no mutational hot spots were found. A genotype-phenotype analysis suggests that there is no clear relationship between specific mutations and clinical features. This analysis revealed a wide spectrum of NF1 mutations in Korean patients. As technologies advance in molecular genetics, the mutation detection rate will increase. Considering that 30.8% of detected mutations were novel, exhaustive mutation analysis of NF1 may be an important tool in early diagnosis and genetic counseling.

  1. BRCA1 Gene Mutations in Chinese Families with Breast Cancer

    Institute of Scientific and Technical Information of China (English)

    Yurong Shi; Chenbin Li; Ruifang Niu; Xishan Hao; Xiangcheng Zhi; Liansheng Ning


    OBJECTIVE To investigate the frequency of BRCA1 gene mutations in breast cancer families in China.METHODS Genomic DNA was obtained by conventional techniques from the peripheral blood mononuclear cells collected from 94 persons derived from 45 breast cancer families. All participants gave written informed consent. The mutations in the BRCA1 gene were detected by the polymerase chain reaction and single stranded conformation polymorphism(PCR-SSCP). Then , the samples of interest were sent for direct DNA sequencing.RESULTS No mutation sites were found in exon 2 or 20 by DNA sequencing.Eight sites were found in exon 11 such as 2201C>T (Ser694Ser),3232A>G(Glu 1038Gly), 2201C >A/G (Ser694Arg), 2731C >T (Pro871Leu),2086A >T(Asn591lle) and three sites of 1584G>T (Glu424Stop). Three mutation sites were found in exon 16 which included 5106A >G (Met1663Val),5208delT(Stop 1639) and 4956A>G (Ser 1613Gly).CONCLUSION These mutation sites may be related to breast cancer, but more investigation is needed to determine whether the mutation sites are hot spots of mutations in Chinese familial breast cancer patients.

  2. A comprehensive catalogue of somatic mutations in cancer genomes. (United States)

    Friedberg, Errol C


    This Hot Topics contribution considers two recently published papers that demonstrate the utility of advanced DNA sequencing technologies for identifying classes of mutations other than base substitutions. Data are presented from genome analyses of immortalized cell lines derived from a malignant melanoma and a small cell carcinoma of the lung. Among other observations the studies suggest the operation of novel DNA repair mechanisms or modes. 2010 Elsevier B.V. All rights reserved.

  3. Bilateral frontoparietal polymicrogyria: a novel GPR56 mutation and an unusual phenotype. (United States)

    Santos-Silva, Rita; Passas, Armanda; Rocha, Carla; Figueiredo, Rita; Mendes-Ribeiro, Jose; Fernandes, Susana; Biskup, Saskia; Leão, Miguel


    Loss of function of GPR56 causes a specific brain malformation called the bilateral frontoparietal polymicrogyria (BFPP), which has typical clinical and neuroradiological findings. So far, 35 families and 26 independent mutations have been described.We present a Portuguese 5-year-old boy, born from nonconsanguineous parents, with BFPP. This patient has a novel GPR56 mutation (R271X) and an unusual phenotype, because he presents hot water epilepsy.To the best of our knowledge, this is the first reported case of BFPP evolving hot water epilepsy.

  4. Further Studies Of Hot-Wire Anemometry (United States)

    Mckenzie, Robert; Logan, Pamela; Bershader, Daniel


    Report discusses factors affecting readings of hot-wire anemometer in turbulent supersonic boundary layer. Represents extension of work described in "Hot-Wire Anemometry Versus Laser-Induced Fluorescence" (ARC-11802). Presents theoretical analysis of responses of hot-wire probe to changes in flow; also compares measurements by hot-wire probe with measurements of same flows by laser-induced fluorescence (LIF).

  5. OUT Success Stories: Solar Hot Water Technology (United States)

    Clyne, R.


    Solar hot water technology was made great strides in the past two decades. Every home, commercial building, and industrial facility requires hot water. DOE has helped to develop reliable and durable solar hot water systems. For industrial applications, the growth potential lies in large-scale systems, using flat-plate and trough-type collectors. Flat-plate collectors are commonly used in residential hot water systems and can be integrated into the architectural design of the building.

  6. Echinocandin failure case due to a yet unreported FKS mutation in Candida krusei

    DEFF Research Database (Denmark)

    Jensen, Rasmus Hare; Justesen, Ulrik Stenz; Rewes, Annika;

    Echinocandins are the preferred therapy for invasive infections due to Candida krusei. We present here a case of clinical failure involving C. krusei with a characteristic FKS1 hot spot mutation not previously reported in C. krusei that was isolated after 14 days of treatment. Anidulafungin MICs ...... were elevated by ≥5 dilution steps above the clinical breakpoint but by only 1 step for a Candida albicans isolate harboring the corresponding mutation, suggesting a notable species-specific difference in the MIC increase conferred by this mutation.......Echinocandins are the preferred therapy for invasive infections due to Candida krusei. We present here a case of clinical failure involving C. krusei with a characteristic FKS1 hot spot mutation not previously reported in C. krusei that was isolated after 14 days of treatment. Anidulafungin MICs...

  7. Mutation rates among RNA viruses


    Drake, John W.; Holland, John J.


    The rate of spontaneous mutation is a key parameter in modeling the genetic structure and evolution of populations. The impact of the accumulated load of mutations and the consequences of increasing the mutation rate are important in assessing the genetic health of populations. Mutation frequencies are among the more directly measurable population parameters, although the information needed to convert them into mutation rates is often lacking. A previous analysis of mutation rates in RNA viru...

  8. Identification of interacting hot spots in the beta3 integrin stalk using comprehensive interface design. (United States)

    Donald, Jason E; Zhu, Hua; Litvinov, Rustem I; DeGrado, William F; Bennett, Joel S


    Protein-protein interfaces are usually large and complementary surfaces, but specific side chains, representing energetic "hot spots," often contribute disproportionately to binding free energy. We used a computational method, comprehensive interface design, to identify hot spots in the interface between the stalk regions of the β3 and the complementary αIIb and αv integrin subunits. Using the Rosetta alanine-scanning and design algorithms to predict destabilizing, stabilizing, and neutral mutations in the β3 region extending from residues Lys(532) through Gly(690), we predicted eight alanine mutations that would destabilize the αIIbβ3 interface as well as nine predicted to destabilize the αvβ3 interface, by at least 0.3 kcal/mol. The mutations were widely and unevenly distributed, with four between residues 552 and 563 and five between 590 and 610, but none between 565 and 589, and 611 and 655. Further, mutations destabilizing the αvβ3 and αIIbβ3 interfaces were not identical. The predictions were then tested by introducing selected mutations into the full-length integrins expressed in Chinese hamster ovary cells. Five mutations predicted to destabilize αIIb and β3 caused fibrinogen binding to αIIbβ3, whereas three of four predicted to be neutral or stabilizing did not. Conversely, a mutation predicted to destabilize αvβ3, but not αIIbβ3 (D552A), caused osteopontin binding to αvβ3, but not fibrinogen binding to αIIbβ3. These results indicate that stability of the distal stalk interface is involved in constraining integrins in stable, inactive conformations. Further, they demonstrate the ability of comprehensive interface design to identify functionally significant integrin mutations.

  9. Thermometric Soots on Hot Jupiters?

    CERN Document Server

    Zahnle, K; Fortney, J J


    We use a 1D thermochemical and photochemical kinetics model to predict that the stratospheric chemistry of hot Jupiters should change dramatically as temperature drops from 1200 to 1000 K. At 1200 K methane is too unstable to reach the stratosphere in significant quantities, while thermal decomposition of water is a strong source of OH radicals that oxidize any hydrocarbons that do form to CO and CO$_2$. At 1000 K methane, although very reactive, survives long enough to reach the lower stratosphere, and the greater stability of water coupled with efficient scavenging of OH by H$_2$ raise the effective C/O ratio in the reacting gases above unity. Reduced products such as ethylene, acetylene, and hydrogen cyanide become abundant; such conditions favor polymerization and possible formation of PAHs and soots. Although low temperature is the most important factor favoring hydrocarbons in hot Jupiters, higher rates of vertical mixing and generally lower metallicities also favor organic synthesis. The peculiar prope...

  10. Measurement of Hot Electron Spectrum

    Institute of Scientific and Technical Information of China (English)

    LIYe-jun; SHANYu-sheng; ZHANGHai-feng; ZHANGJi; WANGLei-jian; TANGXiu-zhang


    The hot electron spectrum was measured using 180°electron magnetic spectrometer through the irradiation of solid Cu target by an intense, near infrared(744 nm), P-polarized light, femtosecond (120 fs) laser pulse with free pre-pulse, and the intensity of laser is 1016 W/cm2 with 45° incidence. And the spectrometer was located in the direction of laser right reflection.

  11. Graphene Hot-electron Transistors


    Vaziri, Sam


    Graphene base transistors (GBTs) have been, recently, proposed to overcome the intrinsic limitations of the graphene field effect transistors (GFETs) and exploit the graphene unique properties in high frequency (HF) applications. These devices utilize single layer graphene as the base material in the vertical hot-electron transistors. In an optimized GBT, the ultimate thinness of the graphene-base and its high conductivity, potentially, enable HF performance up to the THz region.  This thesis...


    Energy Technology Data Exchange (ETDEWEB)

    E.S. Connolly; G.D. Forsythe


    Advanced, coal-based power plants will require durable and reliable hot gas filtration systems to remove particulate contaminants from the gas streams to protect downstream components such as turbine blades from erosion damage. It is expected that the filter elements in these systems will have to be made of ceramic materials to withstand goal service temperatures of 1600 F or higher. Recent demonstration projects and pilot plant tests have indicated that the current generation of ceramic hot gas filters (cross-flow and candle configurations) are failing prematurely. Two of the most promising materials that have been extensively evaluated are clay-bonded silicon carbide and alumina-mullite porous monoliths. These candidates, however, have been found to suffer progressive thermal shock fatigue damage, as a result of rapid cooling/heating cycles. Such temperature changes occur when the hot filters are back-pulsed with cooler gas to clean them, or in process upset conditions, where even larger gas temperature changes may occur quickly and unpredictably. In addition, the clay-bonded silicon carbide materials are susceptible to chemical attack of the glassy binder phase that holds the SiC particles together, resulting in softening, strength loss, creep, and eventual failure.


    Institute of Scientific and Technical Information of China (English)

    CHANG Liang; ZHONG Su; ZHAO Nan; LIU Ping; ZHAO Yangyu; QIAO Jie


    Objective The study is to identify the carrier rate of common deafness mutation in Chinese pregnant women via detecting deafness gene mutations with gene chip. Methods The pregnant women in obstetric clinic without hearing impairment and hearing disorders family history were selected. The informed consent was signed. Peripheral blood was taken to extract genom-ic DNA. Application of genetic deafness gene chip for detecting 9 mutational hot spot of the most common 4 Chinese deafness genes, namely GJB2 (35delG,176del16bp, 235delC, 299delAT), GJB3 (C538T) ,SLC26A4 ( IVS72A>G, A2168G) and mito-chondrial DNA 12S rRNA (A1555G, C1494T) . Further genetic testing were provided to the spouses and newborns of the screened carriers. Results Peripheral blood of 430 pregnant women were detected,detection of deafness gene mutation carri-ers in 24 cases(4.2%), including 13 cases of the GJB2 heterozygous mutation, 3 cases of SLC26A4 heterozygous mutation, 1 cases of GJB3 heterozygous mutation, and 1 case of mitochondrial 12S rRNA mutation. 18 spouses and 17 newborns took fur-ther genetic tests, and 6 newborns inherited the mutation from their mother. Conclusion The common deafness genes muta-tion has a high carrier rate in pregnant women group,235delC and IVS7-2A>G heterozygous mutations are common.

  14. A pathway-centric survey of somatic mutations in Chinese patients with colorectal carcinomas.

    Directory of Open Access Journals (Sweden)

    Chao Ling

    Full Text Available Previous genetic studies on colorectal carcinomas (CRC have identified multiple somatic mutations in four candidate pathways (TGF-β, Wnt, P53 and RTK-RAS pathways on populations of European ancestry. However, it is under-studied whether other populations harbor different sets of hot-spot somatic mutations in these pathways and other oncogenes. In this study, to evaluate the mutational spectrum of novel somatic mutations, we assessed 41 pairs of tumor-stroma tissues from Chinese patients with CRC, including 29 colon carcinomas and 12 rectal carcinomas. We designed Illumina Custom Amplicon panel to target 43 genes, including genes in the four candidate pathways, as well as several known oncogenes for other cancers. Candidate mutations were validated by Sanger sequencing, and we further used SIFT and PolyPhen-2 to assess potentially functional mutations. We discovered 3 new somatic mutations in gene APC, TCF7L2, and PIK3CA that had never been reported in the COSMIC or NCI-60 databases. Additionally, we confirmed 6 known somatic mutations in gene SMAD4, APC, FBXW7, BRAF and PTEN in Chinese CRC patients. While most were previously reported in CRC, one mutation in PTEN was reported only in malignant endometrium cancer. Our study confirmed the existence of known somatic mutations in the four candidate pathways for CRC in Chinese patients. We also discovered a number of novel somatic mutations in these pathways, which may have implications for the pathogenesis of CRC.

  15. Mutations in GABRB3

    DEFF Research Database (Denmark)

    Møller, Rikke S; Wuttke, Thomas V; Helbig, Ingo


    OBJECTIVE: To examine the role of mutations in GABRB3 encoding the β3 subunit of the GABAA receptor in individual patients with epilepsy with regard to causality, the spectrum of genetic variants, their pathophysiology, and associated phenotypes. METHODS: We performed massive parallel sequencing...... of GABRB3 in 416 patients with a range of epileptic encephalopathies and childhood-onset epilepsies and recruited additional patients with epilepsy with GABRB3 mutations from other research and diagnostic programs. RESULTS: We identified 22 patients with heterozygous mutations in GABRB3, including 3...... probands from multiplex families. The phenotypic spectrum of the mutation carriers ranged from simple febrile seizures, genetic epilepsies with febrile seizures plus, and epilepsy with myoclonic-atonic seizures to West syndrome and other types of severe, early-onset epileptic encephalopathies...

  16. AIP mutations and gigantism. (United States)

    Rostomyan, Liliya; Potorac, Iulia; Beckers, Pablo; Daly, Adrian F; Beckers, Albert


    AIP mutations are rare in sporadic acromegaly but they are seen at a higher frequency among certain specific populations of pituitary adenoma patients (pituitary gigantism cases, familial isolated pituitary adenoma (FIPA) kindreds, and patients with macroadenomas who are diagnosed ≤30 years). AIP mutations are most prevalent in patients with pituitary gigantism (29% of this group were found to have mutations in AIP gene). These data support targeted genetic screening for AIP mutations/deletions in these groups of pituitary adenoma patients. Earlier diagnosis of AIP-related acromegaly-gigantism cases enables timely clinical evaluation and treatment, thereby improving outcomes in terms of excessive linear growth and acromegaly comorbidities. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  17. Mapping Mutations on Phylogenies

    DEFF Research Database (Denmark)

    Nielsen, Rasmus


    This chapter provides a short review of recent methodologies developed for mapping mutations on phylogenies. Mapping of mutations, or character changes in general, using the maximum parsimony principle has been one of the most powerful tools in phylogenetics, and it has been used in a variety...... of different applications, for example, in the detection of correlated evolution and to identify selection acting on DNA sequences. However, many uses of parsimony mappings have been criticized because they focus on only one of many possible mappings and/or because they do not incorporate statistical...... uncertainty in the mapping. Recently developed probabilistic methods can incorporate statistical uncertainty in the character mappings. In these methods, focus is on a probability distribution of mutational mappings instead of a single estimate of the mutational mapping....

  18. PRRT2 gene mutations (United States)

    Gardiner, Alice R.; Bhatia, Kailash P.; Stamelou, Maria; Dale, Russell C.; Kurian, Manju A.; Schneider, Susanne A.; Wali, G.M.; Counihan, Tim; Schapira, Anthony H.; Spacey, Sian D.; Valente, Enza-Maria; Silveira-Moriyama, Laura; Teive, Hélio A.G.; Raskin, Salmo; Sander, Josemir W.; Lees, Andrew; Warner, Tom; Kullmann, Dimitri M.; Wood, Nicholas W.; Hanna, Michael


    ABSTRACT Objective: The proline-rich transmembrane protein (PRRT2) gene was recently identified using exome sequencing as the cause of autosomal dominant paroxysmal kinesigenic dyskinesia (PKD) with or without infantile convulsions (IC) (PKD/IC syndrome). Episodic neurologic disorders, such as epilepsy, migraine, and paroxysmal movement disorders, often coexist and are thought to have a shared channel-related etiology. To investigate further the frequency, spectrum, and phenotype of PRRT2 mutations, we analyzed this gene in 3 large series of episodic neurologic disorders with PKD/IC, episodic ataxia (EA), and hemiplegic migraine (HM). Methods: The PRRT2 gene was sequenced in 58 family probands/sporadic individuals with PKD/IC, 182 with EA, 128 with HM, and 475 UK and 96 Asian controls. Results: PRRT2 genetic mutations were identified in 28 out of 58 individuals with PKD/IC (48%), 1/182 individuals with EA, and 1/128 individuals with HM. A number of loss-of-function and coding missense mutations were identified; the most common mutation found was the p.R217Pfs*8 insertion. Males were more frequently affected than females (ratio 52:32). There was a high proportion of PRRT2 mutations found in families and sporadic cases with PKD associated with migraine or HM (10 out of 28). One family had EA with HM and another large family had typical HM alone. Conclusions: This work expands the phenotype of mutations in the PRRT2 gene to include the frequent occurrence of migraine and HM with PKD/IC, and the association of mutations with EA and HM and with familial HM alone. We have also extended the PRRT2 mutation type and frequency in PKD and other episodic neurologic disorders. PMID:23077024

  19. ESA uncovers Geminga's `hot spot' (United States)


    16 July 2004 Astronomers using ESA’s X-ray observatory XMM-Newton have detected a small, bright ‘hot spot’ on the surface of the neutron star called Geminga, 500 light-years away. The hot spot is the size of a football field and is caused by the same mechanism producing Geminga’s X-ray tails. This discovery identifies the missing link between the X-ray and gamma-ray emission from Geminga. hi-res Size hi-res: 1284 kb Credits: ESA, P. Caraveo (IASF, Milan) Geminga's hot spot This figure shows the effects of charged particles accelerated in the magnetosphere of Geminga. Panel (a) shows an image taken with the EPIC instrument on board the XMM-Newton observatory. The bright tails, made of particles kicked out by Geminga’s strong magnetic field, trail the neutron star as it moves about in space. Panel (b) shows how electrically charged particles interact with Geminga’s magnetic field. For example, if electrons (blue) are kicked out by the star, positrons (in red) hit the star’s magnetic poles like in an ‘own goal’. Panel (c) illustrates the size of Geminga’s magnetic field (blue) compared to that of the star itself at the centre (purple). The magnetic field is tilted with respect to Geminga’s rotation axis (red). Panel (d) shows the magnetic poles of Geminga, where charged particles hit the surface of the star, creating a two-million degrees hot spot, a region much hotter than the surroundings. As the star spins on its rotation axis, the hot spot comes into view and then disappears, causing the periodic colour change seen by XMM-Newton. An animated version of the entire sequence can be found at: Click here for animated GIF [low resolution, animated GIF, 5536 KB] Click here for AVI [high resolution, AVI with DIVX compression, 19128 KB] hi-res Size hi-res: 371 kb Credits: ESA, P. Caraveo (IASF, Milan) Geminga's hot spot, panel (a) Panel (a) shows an image taken with the EPIC instrument on board the XMM-Newton observatory. The bright tails, made of

  20. Subquivers of mutation-acyclic quivers are mutation-acyclic

    CERN Document Server

    Warkentin, Matthias


    Quiver mutation plays a crucial role in the definition of cluster algebras by Fomin and Zelevinsky. It induces an equivalence relation on the set of all quivers without loops and two-cycles. A quiver is called mutation-acyclic if it is mutation-equivalent to an acyclic quiver. The aim of this note is to show that full subquivers of mutation-acyclic quivers are mutation-acyclic.

  1. Better living with hyper-mutation. (United States)

    Goodman, Myron F


    The simplest forms of mutations, base substitutions, typically have negative consequences, aside from their existential role in evolution and fitness. Hypermutations, mutations on steroids, occurring at frequencies of 10(-2) -10(-4) per base pair, straddle a domain between fitness and death, depending on the presence or absence of regulatory constraints. Two facets of hypermutation, one in Escherichia coli involving DNA polymerase V (pol V), the other in humans, involving activation-induced deoxycytidine deaminase (AID) are portrayed. Pol V is induced as part of the DNA-damage-induced SOS regulon, and is responsible for generating the lion's share of mutations when catalyzing translesion DNA synthesis (TLS). Four regulatory mechanisms, temporal, internal, conformational, and spatial, activate pol V to copy damaged DNA and then deactivate it. On the flip side of the coin, SOS-induced pols V, IV, and II mutate undamaged DNA, thus providing genetic diversity heightening long-term survival and evolutionary fitness. Fitness in humans is principally the domain of a remarkably versatile immune system marked by somatic hypermutations (SHM) in immunoglobulin variable (IgV) regions that ensure antibody (Ab) diversity. AID initiates SHM by deaminating C → U, favoring hot WRC (W = A/T, R = A/G) motifs. Since there are large numbers of trinucleotide motif targets throughout IgV, AID must exercise considerable catalytic restraint to avoid attacking such sites repeatedly, which would otherwise compromise diversity. Processive, random, and inefficient AID-catalyzed dC deamination simulates salient features of SHM, yet generates B-cell lymphomas when working at the wrong time in the wrong place. Environ. Mol. Mutagen. 57:421-434, 2016. © 2016 Wiley Periodicals, Inc.

  2. Structure of Hot Molecular Cores


    Rolffs, Rainer


    High-mass stars form deeply embedded in dense molecular gas, which they heat up and ionize due to their high energy output. During an early phase, the ionization is confined to small regions, and the stellar radiation is absorbed by dust. The high temperatures lead to the evaporation of ice mantles around dust grains, and many highly excited and complex molecules can be observed in these Hot Molecular Cores. At later stages, the whole molecular cloud is ionized and disrupted, and a...


    Energy Technology Data Exchange (ETDEWEB)

    Matthew R. June; John L. Hurley; Mark W. Johnson


    Iron aluminide hot gas filters have been developed using powder metallurgy techniques to form seamless cylinders. Three alloys were short-term corrosion tested in simulated IGCC atmospheres with temperatures between 925 F and 1200 F with hydrogen sulfide concentrations ranging from 783 ppm{sub v} to 78,300 ppm{sub v}. Long-term testing was conducted for 1500 hours at 925 F with 78,300 ppm{sub v}. The FAS and FAL alloys were found to be corrosion resistant in the simulated environments. The FAS alloy has been commercialized.

  4. Breakup Densities of Hot Nuclei. (United States)

    Viola, Vic


    Breakup densities of hot ^197Au-like residues have been deduced from the systematic trends of Coulomb parameters required to fit intermediate-mass-fragment kinetic-energy spectra. The results indicate emission from nuclei near normal nuclear density below an excitation energy E*/A .3ex˜x 5 MeV. Temperatures derived from these data with a density-dependent Fermi-gas model yield a nuclear caloric curve that is generally consistent with those derived from isotope ratios.

  5. Axion hot dark matter bounds

    CERN Document Server

    Raffelt, G; Mirizzi, A; Wong, Y Y Y


    We derive cosmological limits on two-component hot dark matter consisting of neutrinos and axions. We restrict the large-scale structure data to the safely linear regime, excluding the Lyman-alpha forest. We derive Bayesian credible regions in the two-parameter space consisting of m_a and sum(m_nu). Marginalizing over sum(m_nu) provides m_a<1.02 eV (95% CL). In the absence of axions the same data and methods give sum(m_nu)< 0.63 eV (95% CL).

  6. Hot moons and cool stars

    Directory of Open Access Journals (Sweden)

    Heller René


    Full Text Available The exquisite photometric precision of the Kepler space telescope now puts the detection of extrasolar moons at the horizon. Here, we firstly review observational and analytical techniques that have recently been proposed to find exomoons. Secondly, we discuss the prospects of characterizing potentially habitable extrasolar satellites. With moons being much more numerous than planets in the solar system and with most exoplanets found in the stellar habitable zone being gas giants, habitable moons could be as abundant as habitable planets. However, satellites orbiting planets in the habitable zones of cool stars will encounter strong tidal heating and likely appear as hot moons.

  7. Subminiature Hot-Wire Probes (United States)

    Westphal, R. V.; Lemos, F. R.; Ligrani, P. M.


    Class of improved subminiature hot-wire flow-measuring probes developed. Smaller sizes yield improved resolution in measurements of practical aerodynamic flows. Probe made in one-wire, two-perpendicular-wire, and three-perpendicular-wire version for measurement of one, two, or all three components of flow. Oriented and positioned on micromanipulator stage and viewed under microscope during fabrication. Tested by taking measurements in constant-pressure turbulent boundary layer. New probes give improved measurements of turbulence quantities near surfaces and anisotropies of flows strongly influence relative errors caused by phenomena related to spatial resolution.

  8. Hot Flow Anomalies at Venus (United States)

    Collinson, G. A.; Sibeck, David Gary; Boardsen, Scott A.; Moore, Tom; Barabash, S.; Masters, A.; Shane, N.; Slavin, J.A.; Coates, A.J.; Zhang, T. L.; Sarantos, M.


    We present a multi-instrument study of a hot flow anomaly (HFA) observed by the Venus Express spacecraft in the Venusian foreshock, on 22 March 2008, incorporating both Venus Express Magnetometer and Analyzer of Space Plasmas and Energetic Atoms (ASPERA) plasma observations. Centered on an interplanetary magnetic field discontinuity with inward convective motional electric fields on both sides, with a decreased core field strength, ion observations consistent with a flow deflection, and bounded by compressive heated edges, the properties of this event are consistent with those of HFAs observed at other planets within the solar system.

  9. The bacteriophage P1 hot gene, encoding a homolog of the E. coli DNA polymerase III theta subunit, is expressed during both lysogenic and lytic growth stages. (United States)

    Chikova, Anna K; Schaaper, Roel M


    The bacteriophage P1 hot gene product is a homolog of the theta subunit of E. coli DNA polymerase III. Previous studies with hot cloned on a plasmid have shown that Hot protein can substitute for theta, as evidenced by its stabilizing effect on certain dnaQ mutator mutants carrying an unstable pol III proofreading subunit (epsilon subunit). These results are consistent with Hot, like theta, being a replication protein involved in stabilizing the intrinsically unstable epsilon proofreading function. However, the function of hot for the viral life cycle is less clear. In the present study, we show that the hot gene is not essential. Based on its promoter structure, hot has been previously classified as a "late" phage gene, a property that is not easily reconciled with a presumed replication function. Here, we clarify this issue by demonstrating that P1 hot is actively expressed both during the lysogenic state and in the early stages of a lytic induction, in addition to its expression in the late stage of phage development. The results indicate that P1 hot has a complex expression pattern, compatible with a model in which Hot may affect the host replication machinery to benefit overall phage replication.

  10. Echinocandin failure case due to a yet unreported FKS mutation in Candida krusei

    DEFF Research Database (Denmark)

    Jensen, Rasmus Hare; Justesen, Ulrik Stenz; Rewes, Annika

    Echinocandins are the preferred therapy for invasive infections due to Candida krusei. We present here a case of clinical failure involving C. krusei with a characteristic FKS1 hot spot mutation not previously reported in C. krusei that was isolated after 14 days of treatment. Anidulafungin MICs...... were elevated by ≥5 dilution steps above the clinical breakpoint but by only 1 step for a Candida albicans isolate harboring the corresponding mutation, suggesting a notable species-specific difference in the MIC increase conferred by this mutation....

  11. Echinocandin Failure Case Due to a Previously Unreported FKS1 Mutation in Candida krusei

    DEFF Research Database (Denmark)

    Jensen, Rasmus Hare; Justesen, Ulrik Stenz; Rewes, Annika


    Echinocandins are the preferred therapy for invasive infections due to Candida krusei. We present here a case of clinical failure involving C. krusei with a characteristic FKS1 hot spot mutation not previously reported in C. krusei that was isolated after 14 days of treatment. Anidulafungin MICs...... were elevated by ≥5 dilution steps above the clinical breakpoint but by only 1 step for a Candida albicans isolate harboring the corresponding mutation, suggesting a notable species-specific difference in the MIC increase conferred by this mutation....

  12. Mutational spectrum drives the rise of mutator bacteria.

    Directory of Open Access Journals (Sweden)

    Alejandro Couce

    Full Text Available Understanding how mutator strains emerge in bacterial populations is relevant both to evolutionary theory and to reduce the threat they pose in clinical settings. The rise of mutator alleles is understood as a result of their hitchhiking with linked beneficial mutations, although the factors that govern this process remain unclear. A prominent but underappreciated fact is that each mutator allele increases only a specific spectrum of mutational changes. This spectrum has been speculated to alter the distribution of fitness effects of beneficial mutations, potentially affecting hitchhiking. To study this possibility, we analyzed the fitness distribution of beneficial mutations generated from different mutator and wild-type Escherichia coli strains. Using antibiotic resistance as a model system, we show that mutational spectra can alter these distributions substantially, ultimately determining the competitive ability of each strain across environments. Computer simulation showed that the effect of mutational spectrum on hitchhiking dynamics follows a non-linear function, implying that even slight spectrum-dependent fitness differences are sufficient to alter mutator success frequency by several orders of magnitude. These results indicate an unanticipated central role for the mutational spectrum in the evolution of bacterial mutation rates. At a practical level, this study indicates that knowledge of the molecular details of resistance determinants is crucial for minimizing mutator evolution during antibiotic therapy.

  13. Atmospheric Escape from Hot Jupiters

    CERN Document Server

    Murray-Clay, Ruth; Murray, Norman


    Photoionization heating from UV radiation incident on the atmospheres of hot Jupiters may drive planetary mass loss. We construct a model of escape that includes realistic heating and cooling, ionization balance, tidal gravity, and pressure confinement by the host star wind. We show that mass loss takes the form of a hydrodynamic ("Parker") wind, emitted from the planet's dayside during lulls in the stellar wind. When dayside winds are suppressed by the confining action of the stellar wind, nightside winds might pick up if there is sufficient horizontal transport of heat. A hot Jupiter loses mass at maximum rates of ~2 x 10^12 g/s during its host star's pre-main-sequence phase and ~2 x10^10 g/s during the star's main sequence lifetime, for total maximum losses of ~0.06% and ~0.6% of the planet's mass, respectively. For UV fluxes F_UV < 10^4 erg/cm^2/s, the mass loss rate is approximately energy-limited and is proportional to F_UV^0.9. For larger UV fluxes, such as those typical of T Tauri stars, radiative ...

  14. Hot Outflows in Galaxy Clusters

    CERN Document Server

    Kirkpatrick, C C


    The gas-phase metallicity distribution has been analyzed for the hot atmospheres of 29 galaxy clusters using {\\it Chandra X-ray Observatory} observations. All host brightest cluster galaxies (BCGs) with X-ray cavity systems produced by radio AGN. We find high elemental abundances projected preferentially along the cavities of 16 clusters. The metal-rich plasma was apparently lifted out of the BCGs with the rising X-ray cavities (bubbles) to altitudes between twenty and several hundred kiloparsecs. A relationship between the maximum projected altitude of the uplifted gas (the "iron radius") and jet power is found with the form $R_{\\rm Fe} \\propto P_{\\rm jet}^{0.45}$. The estimated outflow rates are typically tens of solar masses per year but exceed $100 ~\\rm M_\\odot ~yr^{-1}$ in the most powerful AGN. The outflow rates are 10% to 20% of the cooling rates, and thus alone are unable to offset a cooling inflow. Nevertheless, hot outflows effectively redistribute the cooling gas and may play a significant role at ...

  15. A Hot Spot in Coma

    CERN Document Server

    Donnelly, R H; Forman, W R; Jones, C; Churazov, E; Gilfanov, M R


    We study the temperature structure of the central part (r<18' ~0.7 h50**-1 Mpc) of the Coma cluster of galaxies using ASCA data. Two different analysis methods produce results in good agreement with each other and reveal the presence of interesting structures in the gas temperature distribution. Globally, the average temperature in the center of the cluster is 9.0 +/- 0.6 keV in good agreement with previous results. Superimposed on this, we find a cool area with temperatures of 4-6 keV associated with a filament of X-ray emission extending southeast from the cluster center detected by Vikhlinin and coworkers. We also find a hot spot with a temperature of around 13 keV displaced north from the central peak of emission. The distribution of the gas temperatures and relative specific entropies suggests that the cool features are most likely gas stripped from a galaxy group centered on NGC 4874 falling toward the core from outside, while the hot spot located ``ahead'' of this in-falling gas is due to shock heat...

  16. Leukemia-Associated Somatic Mutations Drive Distinct Patterns of Age-Related Clonal Hemopoiesis

    Directory of Open Access Journals (Sweden)

    Thomas McKerrell


    Full Text Available Clonal hemopoiesis driven by leukemia-associated gene mutations can occur without evidence of a blood disorder. To investigate this phenomenon, we interrogated 15 mutation hot spots in blood DNA from 4,219 individuals using ultra-deep sequencing. Using only the hot spots studied, we identified clonal hemopoiesis in 0.8% of individuals under 60, rising to 19.5% of those ≥90 years, thus predicting that clonal hemopoiesis is much more prevalent than previously realized. DNMT3A-R882 mutations were most common and, although their prevalence increased with age, were found in individuals as young as 25 years. By contrast, mutations affecting spliceosome genes SF3B1 and SRSF2, closely associated with the myelodysplastic syndromes, were identified only in those aged >70 years, with several individuals harboring more than one such mutation. This indicates that spliceosome gene mutations drive clonal expansion under selection pressures particular to the aging hemopoietic system and explains the high incidence of clonal disorders associated with these mutations in advanced old age.

  17. Silting mutation in triangulated categories

    CERN Document Server

    Aihara, Takuma


    In representation theory of algebras the notion of `mutation' often plays important roles, and two cases are well known, i.e. `cluster tilting mutation' and `exceptional mutation'. In this paper we focus on `tilting mutation', which has a disadvantage that it is often impossible, i.e. some of summands of a tilting object can not be replaced to get a new tilting object. The aim of this paper is to take away this disadvantage by introducing `silting mutation' for silting objects as a generalization of `tilting mutation'. We shall develope a basic theory of silting mutation. In particular, we introduce a partial order on the set of silting objects and establish the relationship with `silting mutation' by generalizing the theory of Riedmann-Schofield and Happel-Unger. We show that iterated silting mutation act transitively on the set of silting objects for local, hereditary or canonical algebras. Finally we give a bijection between silting subcategories and certain t-structures.

  18. Sanitary hot water; Eau chaude sanitaire

    Energy Technology Data Exchange (ETDEWEB)



    Cegibat, the information-recommendation agency of Gaz de France for building engineering professionals, has organized this conference meeting on sanitary hot water to present the solutions proposed by Gaz de France to meet its clients requirements in terms of water quality, comfort, energy conservation and respect of the environment: quantitative aspects of the hot water needs, qualitative aspects, presentation of the Dolce Vita offer for residential buildings, gas water heaters and boilers, combined solar-thermal/natural gas solutions, key-specifications of hot water distribution systems, testimony: implementation of a gas hot water reservoir and two accumulation boilers in an apartment building for young workers. (J.S.)

  19. Line Heat-Source Guarded Hot Plate (United States)

    Federal Laboratory Consortium — Description: The 1-meter guarded hot-plate apparatus measures thermal conductivity of building insulation. This facility provides for absolute measurement of thermal...

  20. Why Hot Money Is Sizzling in China

    Institute of Scientific and Technical Information of China (English)


    Hot money can cause serious problems for a country’s financial stability.Though analysts have controversial opinions about how hot money winds its way into China,most of them acknowledge that fast inflows and outflows of hot money have become an issue to be reckoned with.Their influences have been felt in capital and property markets as well as the overall macro-economic perfor- mance.Earlier this month,Economic Information Daily published an article about how the government tracks hot money flows and supervises them.Excerpts follow.

  1. Line Heat-Source Guarded Hot Plate (United States)

    Federal Laboratory Consortium — Description:The 1-meter guarded hot-plate apparatus measures thermal conductivity of building insulation. This facility provides for absolute measurement of thermal...


    DEFF Research Database (Denmark)


    The present invention relates to an isolated polynucleotide encoding at least a part of calmodulin and an isolated polypeptide comprising at least a part of a calmodulin protein, wherein the polynucleotide and the polypeptide comprise at least one mutation associated with a cardiac disorder. The ...... the binding of calmodulin to ryanodine receptor 2 and use of such compound in a treatment of an individual having a cardiac disorder. The invention further provides a kit that can be used to detect specific mutations in calmodulin encoding genes....


    DEFF Research Database (Denmark)


    The present invention relates to an isolated polynucleotide encoding at least a part of calmodulin and an isolated polypeptide comprising at least a part of a calmodulin protein, wherein the polynucleotide and the polypeptide comprise at least one mutation associated with a cardiac disorder...... the binding of calmodulin to ryanodine receptor 2 and use of such compound in a treatment of an individual having a cardiac disorder. The invention further provides a kit that can be used to detect specific mutations in calmodulin encoding genes....

  4. Are There Mutator Polymerases?

    Directory of Open Access Journals (Sweden)

    Miguel Garcia-Diaz


    Full Text Available DNA polymerases are involved in different cellular events, including genome replication and DNA repair. In the last few years, a large number of novel DNA polymerases have been discovered, and the biochemical analysis of their properties has revealed a long list of intriguing features. Some of these polymerases have a very low fidelity and have been suggested to play mutator roles in different processes, like translesion synthesis or somatic hypermutation. The current view of these processes is reviewed, and the current understanding of DNA polymerases and their role as mutator enzymes is discussed.


    Energy Technology Data Exchange (ETDEWEB)

    E.S. Connolly; G.D. Forsythe


    DuPont Lanxide Composites, Inc. undertook a sixty-month program, under DOE Contract DEAC21-94MC31214, in order to develop hot gas candle filters from a patented material technology know as PRD-66. The goal of this program was to extend the development of this material as a filter element and fully assess the capability of this technology to meet the needs of Pressurized Fluidized Bed Combustion (PFBC) and Integrated Gasification Combined Cycle (IGCC) power generation systems at commercial scale. The principal objective of Task 3 was to build on the initial PRD-66 filter development, optimize its structure, and evaluate basic material properties relevant to the hot gas filter application. Initially, this consisted of an evaluation of an advanced filament-wound core structure that had been designed to produce an effective bulk filter underneath the barrier filter formed by the outer membrane. The basic material properties to be evaluated (as established by the DOE/METC materials working group) would include mechanical, thermal, and fracture toughness parameters for both new and used material, for the purpose of building a material database consistent with what is being done for the alternative candle filter systems. Task 3 was later expanded to include analysis of PRD-66 candle filters, which had been exposed to actual PFBC conditions, development of an improved membrane, and installation of equipment necessary for the processing of a modified composition. Task 4 would address essential technical issues involving the scale-up of PRD-66 candle filter manufacturing from prototype production to commercial scale manufacturing. The focus would be on capacity (as it affects the ability to deliver commercial order quantities), process specification (as it affects yields, quality, and costs), and manufacturing systems (e.g. QA/QC, materials handling, parts flow, and cost data acquisition). Any filters fabricated during this task would be used for product qualification tests

  6. Melanomas of unknown primary have a mutation profile consistent with cutaneous sun-exposed melanoma. (United States)

    Dutton-Regester, Ken; Kakavand, Hojabr; Aoude, Lauren G; Stark, Mitchell S; Gartside, Michael G; Johansson, Peter; O'Connor, Linda; Lanagan, Cathy; Tembe, Varsha; Pupo, Gulietta M; Haydu, Lauren E; Schmidt, Christopher W; Mann, Graham J; Thompson, John F; Scolyer, Richard A; Hayward, Nicholas K


    Melanoma of unknown primary (MUP) is an uncommon phenomenon whereby patients present with metastatic disease without an evident primary site. To determine their likely site of origin, we combined exome sequencing from 33 MUPs to assess the total rate of somatic mutations and degree of UV mutagenesis. An independent cohort of 91 archival MUPs was also screened for 46 hot spot mutations highly prevalent in melanoma including BRAF, NRAS, KIT, GNAQ, and GNA11. Results showed that the majority of MUPs exhibited high somatic mutation rates, high ratios of C>T/G>A transitions, and a high rate of BRAF (45 of 101, 45%) and NRAS (32 of 101, 32%) mutations, collectively indicating a mutation profile consistent with cutaneous sun-exposed melanomas. These data suggest that a significant proportion of MUPs arise from regressed or unrecognized primary cutaneous melanomas or arise de novo in lymph nodes from nevus cells that have migrated from the skin.

  7. Msx1 Mutations (United States)

    Wang, Y.; Kong, H.; Mues, G.; D’Souza, R.


    Mutations in the transcription factors PAX9 and MSX1 cause selective tooth agenesis in humans. In tooth bud mesenchyme of mice, both proteins are required for the expression of Bmp4, which is the key signaling factor for progression to the next step of tooth development. We have previously shown that Pax9 can transactivate a 2.4-kb Bmp4 promoter construct, and that most tooth-agenesis-causing PAX9 mutations impair DNA binding and Bmp4 promoter activation. We also found that Msx1 by itself represses transcription from this proximal Bmp4 promoter, and that, in combination with Pax9, it acts as a potentiator of Pax9-induced Bmp4 transactivation. This synergism of Msx1 with Pax9 is significant, because it is currently the only documented mechanism for Msx1-mediated activation of Bmp4. In this study, we investigated whether the 5 known tooth-agenesis-causing MSX1 missense mutations disrupt this Pax9-potentiation effect, or if they lead to deficiencies in protein stability, protein-protein interactions, nuclear translocation, and DNA-binding. We found that none of the studied molecular mechanisms yielded a satisfactory explanation for the pathogenic effects of the Msx1 mutations, calling for an entirely different approach to the investigation of this step of odontogenesis on the molecular level. PMID:21297014

  8. Hot Extrusion of Aluminum Chips (United States)

    Tekkaya, A. Erman; Güley, Volkan; Haase, Matthias; Jäger, Andreas

    The process of hot extrusion is a promising approach for the direct recycling of aluminum machining chips to aluminum profiles. The presented technology is capable of saving energy, as remelting of aluminum chips can be avoided. Depending on the deformation route and process parameters, the chip-based aluminum extradates showed mechanical properties comparable or superior to cast aluminum billets extruded under the same conditions. Using different metal flow schemes utilizing different extrusion dies the mechanical properties of the profiles extruded from chips can be improved. The energy absorption capacity of the profiles the rectangular hollow profiles extruded from chips and as-cast billets were analyzed using the drop hammer test set-up. The formability of the profiles extruded from chips and as-cast material were compared using tube bending tests in a three-roller-bending machine.

  9. Resummations in hot scalar electrodynamics

    CERN Document Server

    Krämmer, U; Schulz, H


    The gauge-boson sector of perturbative scalar electrodynamics is investigated in detail as a testing ground for resummation methods in hot gauge theories. It also serves as a simple non-trivial reference system for the non-Abelian gluon plasma. The complete next-to-leading order contributions to the polarization tensor are obtained within the resummation scheme of Braaten and Pisarski. The simpler scheme proposed recently by Arnold and Espinosa is shown to apply to static quantities only, whereas Braaten-Pisarski resummation turns out to need modification for collective phenomena close to the light-cone. Finally, a recently proposed resummation of quasi-particle damping contributions is assessed critically.

  10. Hot topics in functional neuroradiology. (United States)

    Faro, S H; Mohamed, F B; Helpern, J A; Jensen, J H; Thulborn, K R; Atkinson, I C; Sair, H I; Mikulis, D J


    Functional neuroradiology represents a relatively new and ever-growing subspecialty in the field of neuroradiology. Neuroradiology has evolved beyond anatomy and basic tissue signal characteristics and strives to understand the underlying physiologic processes of central nervous system disease. The American Society of Functional Neuroradiology sponsors a yearly educational and scientific meeting, and the educational committee was asked to suggest a few cutting-edge functional neuroradiology techniques (hot topics). The following is a review of several of these topics and includes "Diffusion Tensor Imaging of the Pediatric Spinal Cord"; "Diffusional Kurtosis Imaging"; "From Standardization to Quantification: Beyond Biomarkers toward Bioscales as Neuro MR Imaging Surrogates of Clinical End Points"; Resting-State Functional MR Imaging"; and "Current Use of Cerebrovascular Reserve Imaging."

  11. Novel and recurrent EVC and EVC2 mutations in Ellis-van Creveld syndrome and Weyers acrofacial dyostosis. (United States)

    D'Asdia, Maria Cecilia; Torrente, Isabella; Consoli, Federica; Ferese, Rosangela; Magliozzi, Monia; Bernardini, Laura; Guida, Valentina; Digilio, Maria Cristina; Marino, Bruno; Dallapiccola, Bruno; De Luca, Alessandro


    Ellis van Creveld syndrome and Weyers acrofacial dysostosis are allelic disorders caused by mutations in EVC or EVC2 genes. We illustrate the results of direct analysis of whole EVC and EVC2 genes' coding regions in 32 unrelated families with clinical diagnosis of Ellis van Creveld syndrome and in 2 families with Weyers acrofacial dysostosis. We identified mutations in 27/32 (84%) cases with Ellis van Creveld syndrome and 2/2 cases with Weyers acrofacial dysostosis. Of the Ellis van Creveld syndrome cases, 20/27 (74%) had a mutation in EVC and 7/27 (26%) in EVC2 genes. The two subjects with Weyers acrofacial dysostosis had a heterozygous mutation in the last exon of EVC2. In total, we detected 25 independent EVC and 11 independent EVC2 mutations. Nineteen EVC mutations (19/25, 76%) and 4 EVC2 mutations (4/11, 36%) were novel. Also one EVC2 gene mutation found in Weyers acrofacial dysostosis was novel. In 5 unrelated cases with a clinical diagnosis of Ellis van Creveld syndrome, we did not find any mutation in either EVC or EVC2 genes. Current findings expand the Ellis van Creveld syndrome and Weyers acrofacial dysostosis mutation spectra, and provide further evidence that the last exon of EVC2 gene is a hot spot for Weyers acrofacial dysostosis mutations. Accordingly, EVC2 exon 22 should be analyzed with priority by mutation screening in individuals with a suspected diagnosis of Weyers acrofacial dysostosis.

  12. Hypothesizing an ancient Greek origin of the GJB2 35delG mutation: can science meet history? (United States)

    Kokotas, Haris; Grigoriadou, Maria; Villamar, Manuela; Giannoulia-Karantana, Aglaia; del Castillo, Ignacio; Petersen, Michael B


    One specific mutation of the GJB2 gene that encodes the connexin 26 protein, the 35delG mutation, has become a major interest among scientists who focus on the genetics of nonsyndromic hearing loss. The mutation accounts for the majority of GJB2 mutations detected in Caucasian populations and represents one of the most frequent disease mutations identified so far. The debate was so far between the arguments whether or not the 35delG mutation constitutes a mutational hot-spot or a founder effect; however, it was recently clarified that the latter seems the most likely. In an attempt to explore the origin and propagation of the 35delG mutation, several groups have reported the prevalence of the mutation and the carrier rates in different populations worldwide. It is now certain that the theory of a common founder prevails and that the highest carrier frequencies of the 35delG mutation are observed in southern European populations, giving rise to a discussion regarding the origin of the 35delG mutation. In this study, we discuss data previously published by our and other groups and also compare the haplotype distribution of the mutation in southern Europe, trying to understand the pathways of science and history and the conflict between them.

  13. Hot Dry Rock; Geothermal Energy

    Energy Technology Data Exchange (ETDEWEB)



    The commercial utilization of geothermal energy forms the basis of the largest renewable energy industry in the world. More than 5000 Mw of electrical power are currently in production from approximately 210 plants and 10 000 Mw thermal are used in direct use processes. The majority of these systems are located in the well defined geothermal generally associated with crustal plate boundaries or hot spots. The essential requirements of high subsurface temperature with huge volumes of exploitable fluids, coupled to environmental and market factors, limit the choice of suitable sites significantly. The Hot Dry Rock (HDR) concept at any depth originally offered a dream of unlimited expansion for the geothermal industry by relaxing the location constraints by drilling deep enough to reach adequate temperatures. Now, after 20 years intensive work by international teams and expenditures of more than $250 million, it is vital to review the position of HDR in relation to the established geothermal industry. The HDR resource is merely a body of rock at elevated temperatures with insufficient fluids in place to enable the heat to be extracted without the need for injection wells. All of the major field experiments in HDR have shown that the natural fracture systems form the heat transfer surfaces and that it is these fractures that must be for geothermal systems producing from naturally fractured formations provide a basis for directing the forthcoming but, equally, they require accepting significant location constraints on HDR for the time being. This paper presents a model HDR system designed for commercial operations in the UK and uses production data from hydrothermal systems in Japan and the USA to demonstrate the reservoir performance requirements for viable operations. It is shown that these characteristics are not likely to be achieved in host rocks without stimulation processes. However, the long term goal of artificial geothermal systems developed by systematic

  14. R31C GNRH1 mutation and congenital hypogonadotropic hypogonadism.

    Directory of Open Access Journals (Sweden)

    Luigi Maione

    Full Text Available Normosmic congenital hypogonadotropic hypogonadism (nCHH is a rare reproductive disease leading to lack of puberty and infertility. Loss-of-function mutations of GNRH1 gene are a very rare cause of autosomal recessive nCHH. R31C GNRH1 is the only missense mutation that affects the conserved GnRH decapeptide sequence. This mutation was identified in a CpG islet in nine nCHH subjects from four unrelated families, giving evidence for a putative "hot spot". Interestingly, all the nCHH patients carry this mutation in heterozygosis that strikingly contrasts with the recessive inheritance associated with frame shift and non-sense mutations. Therefore, after exclusion of a second genetic event, a comprehensive functional characterization of the mutant R31C GnRH was undertaken. Using different cellular models, we clearly demonstrate a dramatic reduction of the mutant decapeptide capacity to bind GnRH-receptor, to activate MAPK pathway and to trigger inositol phosphate accumulation and intracellular calcium mobilization. In addition it is less able than wild type to induce lh-beta transcription and LH secretion in gonadotrope cells. Finally, the absence of a negative dominance in vitro offers a unique opportunity to discuss the complex in vivo patho-physiology of this form of nCHH.

  15. Mutations in galactosemia

    Energy Technology Data Exchange (ETDEWEB)

    Reichardt, J.K.V. [Univ. of Southern California School of Medicine, Los Angeles, CA (United States)


    This Letter raises four issues concerning two papers on galactosemia published in the March 1995 of the Journal. First, table 2 in the paper by Elsas et al. incorrectly attributes seven galactose-l-phosphate uridyl transferase (GALT) mutations (S135L, L195P, K285N, N314D, R333W, R333G, and K334R). The table also fails to mention that others have reported the same two findings attributed to {open_quotes}Leslie et al.; Elsas et al. and in press{close_quotes} and {open_quotes}Leslie et al.; Elsas et al.{close_quotes} The first finding on the prevalence of the Q188R galactosemia mutation in the G/G Caucasian population has also been described by Ng et al., and the second finding on the correlation of the N314D GALT mutation with the Duarte variant was reported by Lin et al. Second, Elsas et al. suggest that the E203K and N314D mutations may {open_quotes}produce intra-allelic complementation when in cis{close_quotes}. This speculation is supported by the activity data of individual III-2 but is inconsistent with the activities of three other individuals I-1, II-1, and III-1 of the same pedigree. The GALT activity measured in these three individuals suggests a dominant negative effect of E203K in E203K-N314D chromosomes, since they all have less than normal activity. Thus, the preponderance of the data in this paper is at odds with the authors speculation. It is worth recalling that Lin et al. also identified four N314D GALT mutations on 95 galactosemic chromosomes examined. A similar situation also appears to be the case in proband III-1 (with genotype E203K-N314D/IVSC) in the Elsas et al. paper. 9 refs.

  16. Basics of Solar Heating & Hot Water Systems. (United States)

    American Inst. of Architects, Washington, DC.

    In presenting the basics of solar heating and hot water systems, this publication is organized from the general to the specific. It begins by presenting functional and operational descriptions of solar heating and domestic hot water systems, outlining the basic concepts and terminology. This is followed by a description of solar energy utilization…

  17. Hot Spot Removal System: System description

    Energy Technology Data Exchange (ETDEWEB)



    Hazardous wastes contaminated with radionuclides, chemicals, and explosives exist across the Department of Energy complex and need to be remediated due to environmental concerns. Currently, an opportunity is being developed to dramatically reduce remediation costs and to assist in the acceleration of schedules associated with these wastes by deploying a Hot Spot Removal System. Removing the hot spot from the waste site will remove risk driver(s) and enable another, more cost effective process/option/remedial alternative (i.e., capping) to be applied to the remainder of the site. The Hot Spot Removal System consists of a suite of technologies that will be utilized to locate and remove source terms. Components of the system can also be used in a variety of other cleanup activities. This Hot Spot Removal System Description document presents technologies that were considered for possible inclusion in the Hot Spot Removal System, technologies made available to the Hot Spot Removal System, industrial interest in the Hot Spot Removal System`s subsystems, the schedule required for the Hot Spot Removal System, the evaluation of the relevant technologies, and the recommendations for equipment and technologies as stated in the Plan section.

  18. Refractory Pellet for Hot Blast Stove

    Institute of Scientific and Technical Information of China (English)

    Wang Jing; Peng Xigao


    1 Scope This standard specifies the term,definition,classification,specification,technical requirements,test methods,quality appraisal procedures,packing,marking,transportation,storage,and quality certificate of refractory pellet for hot blast stove.This standard is applicable to refractory pellet for hot blast stove.

  19. Hot-dry-rock feasibility study

    Energy Technology Data Exchange (ETDEWEB)


    The hot-dry-rock project tasks are covered as follows: hot-dry-rock reservoir; generation facilities; water resources; transmission requirements; environmental issues; government and community institutional factors; leasing, ownership and management of facilities; regulations, permits, and laws; and financial considerations. (MHR)

  20. The Hot Hand Belief and Framing Effects (United States)

    MacMahon, Clare; Köppen, Jörn; Raab, Markus


    Purpose: Recent evidence of the hot hand in sport--where success breeds success in a positive recency of successful shots, for instance--indicates that this pattern does not actually exist. Yet the belief persists. We used 2 studies to explore the effects of framing on the hot hand belief in sport. We looked at the effect of sport experience and…

  1. Sprayformed Hot Work Steels for Rapid Tooling

    Institute of Scientific and Technical Information of China (English)

    Yunfeng Yang; Simo-Pekka Hannula


    The present work compares microstructures of hot work steels made by different processes, that is, by sprayforming,by casting, and a commercially supplied H13 steel. Material benefits are recognized by sprayforming hot working tools such as die inserts fo

  2. Improved Circuit For Hot-Film Anemometer (United States)

    Gray, David L.


    Circuit suitable for automation or computer control of setup and operation. Hot-film or hot-wire anemometer circuit features individual current drives for two arms of wheatstone bridge, plus other features that provide improved calibration and automated or computer-controlled operation.

  3. Hot-wire anemometer for spirography. (United States)

    Plakk, P; Liik, P; Kingisepp, P H


    The use of a constant temperature hot-wire anemometer flow sensor for spirography is reported. The construction, operating principles and calibration procedure of the apparatus are described, and temperature compensation method is discussed. Frequency response is studied. It is shown that this hot-wire flow transducer satisfies common demands with respect to accuracy, response time and temperature variations.

  4. Hot Mix Asphalt Recycling: Practices and Principles

    NARCIS (Netherlands)

    Mohajeri, M.


    Hot mix asphalt recycling has become common practice all over the world since the 1970s because of the crisis in oil prices. In the Netherlands, hot recycling has advanced to such an extent that in most of the mixtures more than 50% of reclaimed asphalt (RA) is allowed. These mixtures with such a hi

  5. HotSpot Software Test Plan

    Energy Technology Data Exchange (ETDEWEB)

    Walker, H; Homann, S G


    This Software Test Plan (STP) describes the procedures used to verify and validate that the HotSpot Health Physics Codes meet the requirements of its user base, which includes: (1) Users of the PC version of HotSpot conducting consequence assessment, hazard assessment and safety analysis calculations; and (2) Users of the NARAC Web and iClient software tools, which allow users to run HotSpot for consequence assessment modeling. This plan is intended to meet Critical Recommendation 2 from the Software Evaluation of HotSpot and DOE Safety Software Toolbox Recommendation for inclusion of HotSpot in the Department of Energy (DOE) Safety Software Toolbox. These users and sponsors of the HotSpot software and the organizations they represent constitute the intended audience for this document. HotSpot software is maintained for the Department of Energy Office of Emergency Operations by the National Atmospheric Release Advisory Center (NARAC) at Lawrence Livermore National Laboratory (LLNL). An overview of HotSpot and NARAC are provided.

  6. HotSpot Software Configuration Management Plan

    Energy Technology Data Exchange (ETDEWEB)

    Walker, H; Homann, S G


    This Software Configuration Management Plan (SCMP) describes the software configuration management procedures used to ensure that the HotSpot dispersion model meets the requirements of its user base, which includes: (1) Users of the PC version of HotSpot for consequence assessment, hazard assessment and safety analysis calculations; and (2) Users of the NARAC Web and iClient software tools, which allow users to run HotSpot for consequence assessment modeling These users and sponsors of the HotSpot software and the organizations they represent constitute the intended audience for this document. This plan is intended to meet Critical Recommendations 1 and 3 from the Software Evaluation of HotSpot and DOE Safety Software Toolbox Recommendation for inclusion of HotSpot in the Department of Energy (DOE) Safety Software Toolbox. HotSpot software is maintained for the Department of Energy Office of Emergency Operations by the National Atmospheric Release Advisory Center (NARAC) at Lawrence Livermore National Laboratory (LLNL). An overview of HotSpot and NARAC are provided.

  7. Evolutionary Stability Against Multiple Mutations

    CERN Document Server

    Ghatak, Anirban; Shaiju, A J


    It is known (see e.g. Weibull (1995)) that ESS is not robust against multiple mutations. In this article, we introduce robustness against multiple mutations and study some equivalent formulations and consequences.

  8. BRAF mutations in conjunctival melanoma

    DEFF Research Database (Denmark)

    Larsen, Ann-Cathrine; Dahl, Christina; Dahmcke, Christina M.


    Purpose: To investigate incidence, clinicopathological features and prognosis of BRAF-mutated conjunctival melanoma in Denmark. Furthermore, to determine BRAF mutations in paired premalignant lesions and evaluate immunohistochemical BRAF V600E oncoprotein detection. Methods: Data from 139 patients...

  9. 'Hot' cognition in major depressive disorder

    DEFF Research Database (Denmark)

    Miskowiak, Kamilla W; Carvalho, Andre F


    Major depressive disorder (MDD) is associated with significant cognitive dysfunction in both 'hot' (i.e. emotion-laden) and 'cold' (non-emotional) domains. Here we review evidence pertaining to 'hot' cognitive changes in MDD. This systematic review searched the PubMed and PsycInfo computerized...... in a fronto-limbic network with hyper-activity in limbic and ventral prefrontal regions paired with hypo-activity of dorsal prefrontal regions subserve these abnormalities. A cross-talk of 'hot' and 'cold' cognition disturbances in MDD occurs. Disturbances in 'hot cognition' may also contribute...... to the perpetuation of negative emotional states in MDD. Limited success in the identification of susceptibility genes in MDD has led to great research interest in identifying vulnerability biomarkers or endophenotypes. Emerging evidence points to the persistence of 'hot' cognition dysfunction during remission...

  10. [Analysis of PTPN11 mutation in children leukemia and its clinical significance]. (United States)

    Yang, San-Zhen; Chen, Bing-Qiang; Lu, Su-Ying; Zhang, Bi-Hong; Xue, Hong-Man; Chen, Chun


    This study was aimed to explore the frequency of PTPN11 mutation in children with leukemia and its clinical significance. Genomic DNAs were extracted from peripheral leukocytes of 131 patients with leukemia, including 101 cases of ALL, 26 cases of AML, 3 cases of CML and 1 case of juvenil myelomonocytic leukemia (JMML). The sequences of PTPN11 exons 3, 8, 13 were amplified by polymerase chain reaction (PCR), and the clinical characteristics of positive patients were analyzed. The results indicated that the PTPN11 mutation was found in 10 cases (9.9%) from newly diagnosed 101 cases of ALL. Grouping the newly diagnosed ALL children by various clinical features, it was found that the PTPN11 mutation did not show associations with sex, age, white blood cell (WBC) count, prednisone test sensitivity, clinical risk and disease recurrences at the first visit (P > 0.05). PTPN11 mutations were found in 2 cases out of 26 AML patients, including one AML-M(2) and one AML-M(4). No PTPN11 mutation in 3 CML patients was found. Exon 13 mutation of PTPN11 gene was found in 1 case of JMML. It is concluded that the E76 of exon 3 is the hot spot of PTPN11 mutation in children leukemia. The novel G503E (1508G > A) mutation is detected in one JMML patient. The PTPN11 mutation does not associate with the sex, age, WBC count, prednisone sensitive test and early recurrence.

  11. Primordial chemistry and enzyme evolution in a hot environment. (United States)

    Wolfenden, Richard


    Ever since the publication of Darwin's Origin of Species, questions have been raised about whether enough time has elapsed for living organisms to have reached their present level of complexity by mutation and natural selection. More recently, it has become apparent that life originated very early in Earth's history, and there has been controversy as to whether life originated in a hot or cold environment. This review describes evidence that rising temperature accelerates slow reactions disproportionately, and to a much greater extent than has been generally recognized. Thus, the time that would have been required for primordial chemistry to become established would have been abbreviated profoundly at high temperatures. Moreover, if the catalytic effect of a primitive enzyme (like that of modern enzymes) were to reduce a reaction's heat of activation, then the rate enhancement that it produced would have increased as the surroundings cooled, quite aside from changes arising from mutation (which is itself highly sensitive to temperature). Some nonenzymatic catalysts of slow reactions, including PLP as a catalyst of amino acid decarboxylation, and the Ce(IV) ion as a catalyst of phosphate ester hydrolysis, have been shown to meet that criterion. The work reviewed here suggests that elevated temperatures collapsed the time required for early evolution on Earth, furnishing an appropriate setting for exploring the vast range of chemical possibilities and for the rapid evolution of enzymes from primitive catalysts.

  12. A novel keratin 9 gene mutation (Asn160His) in a Taiwanese family with epidermolytic palmoplantar keratoderma. (United States)

    Lin, J-H; Lin, M-H; Yang, M-H; Chao, S-C


    Epidermolytic palmoplantar keratoderma (EPPK) is an autosomal dominant inherited disorder of keratinization. Recent molecular studies have shown that EPPK is caused by mutations in keratin 9 gene (KRT9). We report a Taiwanese family with EPPK with a novel mutation with an A-->C transition at the first nucleotide of codon 160 in KRT9. The mutation is predicted to result in an asparagine to histidine substitution (N160H) at the beginning of the alpha-helical 1A domain of keratin 9. Mutations in this region could disrupt keratin filament assembly, leading to degeneration or cytolysis of keratinocytes. Our mutation analysis confirms that codon 160 in KRT9 is one of the mutation hot spots in EPPK.

  13. Kin Selection - Mutation Balance

    DEFF Research Database (Denmark)

    Dyken, J. David Van; Linksvayer, Timothy Arnold; Wade, Michael J.


    Abstract Social conflict, in the form of intraspecific selfish "cheating" has been observed in a number of natural systems. However, a formal, evolutionary genetic theory of social cheating that provides an explanatory, predictive framework for these observations is lacking. Here we derive the kin...... selection-mutation balance, which provides an evolutionary null hypothesis for the statics and dynamics of cheating. When social interactions have linear fitness effects and Hamilton´s rule is satisfied, selection is never strong enough to eliminate recurrent cheater mutants from a population, but cheater...... lineages are transient and do not invade. Instead, cheating lineages are eliminated by kin selection but are constantly reintroduced by mutation, maintaining a stable equilibrium frequency of cheaters. The presence of cheaters at equilibrium creates a "cheater load" that selects for mechanisms of cheater...

  14. Cancer treatment: dealing with hot flashes and night sweats (United States)

    ... this page: // Cancer treatment: dealing with hot flashes and night sweats To ... this page, please enable JavaScript. Certain types of cancer treatments can cause hot flashes and night sweats. Hot ...

  15. Mutation of Auslander generators

    CERN Document Server

    Lada, Magdalini


    Let $\\Lambda$ be an artin algebra with representation dimension equal to three and $M$ an Auslander generator of $\\Lambda$. We show how, under certain assumptions, we can mutate $M$ to get a new Auslander generator whose endomorphism ring is derived equivalent to the endomorphism ring of $M$. We apply our results to selfinjective algebras with radical cube zero of infinite representation type, where we construct an infinite set of Auslander generators.

  16. Protein structure guided discovery of functional mutations across 19 cancer types (United States)

    Niu, Beifang; Scott, Adam D.; Sengupta, Sohini; Bailey, Matthew H.; Batra, Prag; Ning, Jie; Wyczalkowski, Matthew A.; Liang, Wen-Wei; Zhang, Qunyuan; McLellan, Michael D.; Sun, Sam Q.; Tripathi, Piyush; Lou, Carolyn; Ye, Kai; Mashl, R. Jay; Wallis, John; Wendl, Michael C.; Chen, Feng; Ding, Li


    Local concentrations of mutations are well-known in human cancers. However, their 3-dimensional (3D) spatial relationships have yet to be systematically explored. We developed a computational tool, HotSpot3D, to identify such spatial hotspots (clusters) and to interpret the potential function of variants within them. We applied HotSpot3D to >4,400 TCGA tumors across 19 cancer types, discovering >6,000 intra- and inter-molecular clusters, some of which showed tumor/tissue specificity. In addition, we identified 369 rare mutations from genes including TP53, PTEN, VHL, EGFR, and FBXW7 and 99 medium recurrence mutations from genes such as RUNX1, MTOR, CA3, PI3, and PTPN11, all residing within clusters having potential functional implications. As a proof of concept, we validated our predictions in EGFR using high throughput phosphorylation data and cell-line based experimental evaluation. Finally, drug-mutation cluster/network analysis predicted over 800 promising candidates of druggable mutations, raising new possibilities for designing personalized treatments for patients carrying specific mutations. PMID:27294619

  17. Sex and deleterious mutations. (United States)

    Gordo, Isabel; Campos, Paulo R A


    The evolutionary advantage of sexual reproduction has been considered as one of the most pressing questions in evolutionary biology. While a pluralistic view of the evolution of sex and recombination has been suggested by some, here we take a simpler view and try to quantify the conditions under which sex can evolve given a set of minimal assumptions. Since real populations are finite and also subject to recurrent deleterious mutations, this minimal model should apply generally to all populations. We show that the maximum advantage of recombination occurs for an intermediate value of the deleterious effect of mutations. Furthermore we show that the conditions under which the biggest advantage of sex is achieved are those that produce the fastest fitness decline in the corresponding asexual population and are therefore the conditions for which Muller's ratchet has the strongest effect. We also show that the selective advantage of a modifier of the recombination rate depends on its strength. The quantification of the range of selective effects that favors recombination then leads us to suggest that, if in stressful environments the effect of deleterious mutations is enhanced, a connection between sex and stress could be expected, as it is found in several species.

  18. Outflow from Hot Accretion Flows

    CERN Document Server

    Yuan, Feng; Wu, Maochun


    Numerical simulations of hot accretion flows have shown that the mass accretion rate decreases with decreasing radius. Two models have been proposed to explain this result. In the adiabatic inflow-outflow solution (ADIOS), it is thought to be due to the loss of gas in outflows. In the convection-dominated accretion flow (CDAF) model, it is explained as because that the gas is locked in convective eddies. In this paper we use hydrodynamical (HD) and magnetohydrodynamical (MHD) simulations to investigate which one is physical. We calculate and compare various properties of inflow (gas with an inward velocity) and outflow (gas with an outward velocity). Systematic and significant differences are found. For example, for HD flows, the temperature of outflow is higher than inflow; while for MHD flows, the specific angular momentum of outflow is much higher than inflow. We have also analyzed the convective stability of MHD accretion flow and found that they are stable. These results suggest that systematic inward an...

  19. Linear response of hot gluons (United States)

    Carrington, M. E.; Hansson, T. H.; Yamagishi, H.; Zahed, I.


    We reexamine the various schemes for calculating the linear response (the retarded Green's function) of a hot gluon plasma. The problems related to gauge invariance are discussed in detail, and results in different gauges are compared. We also point out some issues related to the very definition of a thermal ensemble in the presence of unphysical degrees of freedom. By culculating the retarded Green's function directly in real time, we explicitly study the effects of unphysical degrees of freedom in different gauges. Although there appears to be no unique way to define the response function, we find that several schemes can be questioned on formal grounds and that use of the background-field gauge (BFG) is the most satisfactory in this respect. We discuss two proposals to fix the gauge parameter (α) dependence in the BFG response function, the Vilkovisky-DeWitt effective action corresponding to the choice α = 0 (background Landau gauge), and the "gauge-invariant propagator" of Cornwall et al. corresponding to α = 1 (background Feynman gauge).

  20. Septin mutations in human cancers

    Directory of Open Access Journals (Sweden)

    Elias T Spiliotis


    Full Text Available Septins are GTP-binding proteins that are evolutionarily and structurally related to the RAS oncogenes. Septin expression levels are altered in many cancers and new advances point to how abnormal septin expression may contribute to the progression of cancer. In contrast to the RAS GTPases, which are frequently mutated and actively promote tumorigenesis, little is known about the occurrence and role of septin mutations in human cancers. Here, we review septin missense mutations that are currently in the Catalog of Somatic Mutations in Cancer (COSMIC database. The majority of septin mutations occur in tumors of the large intestine, skin, endometrium and stomach. Over 25% of the annotated mutations in SEPT2, SEPT4 and SEPT9 belong to large intestine tumors. From all septins, SEPT9 and SEPT14 exhibit the highest mutation frequencies in skin, stomach and large intestine cancers. While septin mutations occur with frequencies lower than 3%, recurring mutations in several invariant and highly conserved amino acids are found across different septin paralogs and tumor types. Interestingly, a significant number of these mutations occur in the GTP-binding pocket and septin dimerization interfaces. Future studies may determine how these somatic mutations affect septin structure and function, whether they contribute to the progression of specific cancers and if they could serve as tumor-specific biomarkers.

  1. Mucopolysaccharidosis IVA mutations in Chinese patients: 16 novel mutations. (United States)

    Wang, Zheng; Zhang, Weimin; Wang, Yun; Meng, Yan; Su, Liang; Shi, Huiping; Huang, Shangzhi


    Mucopolysaccharidosis IVA (MPS IVA; Morquio A syndrome) is a lysosomal storage disease caused by deficiency of N-acetylgalactosamine-6-sulfatase (GALNS) and transmitted as an autosomal recessive trait. This is the first systematic mutation screen in Chinese MPS IVA patients. Mutation detections in 24 unrelated Chinese MPS IVA patients were performed by PCR and direct sequencing of exons or the mRNA of GALNS. A total of 42 mutant alleles were identified, belonging to 27 different mutations. Out of the 27 mutations, 16 were novel, including 2 splicing mutations (c.567-1G>T and c.634-1G>A), 2 nonsense mutations (p.W325X and p.Q422X) and 12 missense mutations (p.T88I, p.H142R, p.P163H, p.G168L, p.H236D, p.N289S, p.T312A, p.G316V, p.A324E, p.L366P, p.Q422K and p.F452L). p.G340D was found to be a common mutation in the Chinese MPS IVA patients, accounting for 16.7% of the total number of mutant alleles. The results show that the mutations in Chinese MPS IVA patients are also family specific but have a different mutation spectrum as compared to those of other populations.

  2. A review on hot tearing of magnesium alloys

    Directory of Open Access Journals (Sweden)

    Jiangfeng Song


    Full Text Available Hot tearing is often a major casting defect in magnesium alloys and has a significant impact on the quality of their casting products. Hot tearing of magnesium alloys is a complex solidification phenomenon which is still not fully understood, it is of great importance to investigate the hot tearing behaviour of magnesium alloys. This review attempts to summarize the investigations on hot tearing of magnesium alloys over the past decades. The hot tearing criteria including recently developed Kou's criterion are summarized and compared. The numeric simulation and assessing methods of hot tearing, factors influencing hot tearing, and hot tearing susceptibility (HTS of magnesium alloys are discussed.

  3. Calreticulin Mutations in Myeloproliferative Neoplasms

    Directory of Open Access Journals (Sweden)

    Noa Lavi


    Full Text Available With the discovery of the JAK2V617F mutation in patients with Philadelphia chromosome-negative (Ph− myeloproliferative neoplasms (MPNs in 2005, major advances have been made in the diagnosis of MPNs, in understanding of their pathogenesis involving the JAK/STAT pathway, and finally in the development of novel therapies targeting this pathway. Nevertheless, it remains unknown which mutations exist in approximately one-third of patients with non-mutated JAK2 or MPL essential thrombocythemia (ET and primary myelofibrosis (PMF. At the end of 2013, two studies identified recurrent mutations in the gene encoding calreticulin (CALR using whole-exome sequencing. These mutations were revealed in the majority of ET and PMF patients with non-mutated JAK2 or MPL but not in polycythemia vera patients. Somatic 52-bp deletions (type 1 mutations and recurrent 5-bp insertions (type 2 mutations in exon 9 of the CALR gene (the last exon encoding the C-terminal amino acids of the protein calreticulin were detected and found always to generate frameshift mutations. All detected mutant calreticulin proteins shared a novel amino acid sequence at the C-terminal. Mutations in CALR are acquired early in the clonal history of the disease, and they cause activation of JAK/STAT signaling. The CALR mutations are the second most frequent mutations in Ph− MPN patients after the JAK2V617F mutation, and their detection has significantly improved the diagnostic approach for ET and PMF. The characteristics of the CALR mutations as well as their diagnostic, clinical, and pathogenesis implications are discussed in this review.

  4. Combining seismology and spectropolarimetry of hot stars

    CERN Document Server

    Neiner, Coralie; Mathis, Stéphane; Degroote, Pieter


    Asteroseismology and spectropolarimetry have allowed us to progress significantly in our understanding of the physics of hot stars over the last decade. It is now possible to combine these two techniques to learn even more information about hot stars and constrain their models. While only a few magnetic pulsating hot stars are known as of today and have been studied with both seismology and spectropolarimetry, new opportunities - in particular Kepler2 and BRITE - are emerging and will allow us to rapidly obtain new combined results.

  5. Polyakov loop modeling for hot QCD (United States)

    Fukushima, Kenji; Skokov, Vladimir


    We review theoretical aspects of quantum chromodynamics (QCD) at finite temperature. The most important physical variable to characterize hot QCD is the Polyakov loop, which is an approximate order parameter for quark deconfinement in a hot gluonic medium. Additionally to its role as an order parameter, the Polyakov loop has rich physical contents in both perturbative and non-perturbative sectors. This review covers a wide range of subjects associated with the Polyakov loop from topological defects in hot QCD to model building with coupling to the Polyakov loop.

  6. Screening in Hot Non-Abelian Plasma

    CERN Document Server

    Petreczky, P


    This thesis is devoted to the study of the screening masses in hot non-Abelian theories. Section 1 contain a brief introduction to the topic. In section 2 a detailed overview of the screening phenomena and their applications is given. In section 3 the screening masses are defined through the coupled gap equations. Section 4 deals with the determination of the screening masses of hot SU(2) gauge theory in the framework of the 3d lattice adjoint Higgs model considered as an effective theory. Finally in section 5 the screening masses of hot SU(2) Higgs model are examined.

  7. Biofilm formation in a hot water system

    DEFF Research Database (Denmark)

    Bagh, L.K.; Albrechtsen, Hans-Jørgen; Arvin, Erik


    The biofilm formation rate was measured in situ in a hot water system in an apartment building by specially designed sampling equipment, and the net growth of the suspended bacteria was measured by incubation of water samples with the indigeneous bacteria. The biofilm formation rate reached......, in the sludge, or in the water from the distribution system was negligible. This indicated that bacterial growth took place on the inner surfaces in the hot water system and biofilm formation and detachment of bacteria could account for most of the suspended bacteria actually measured in hot water. Therefore...

  8. Characterization of mutations causing rifampicin and isoniazid resistance of Mycobacterium tuberculosis in Syria. (United States)

    Madania, Ammar; Habous, Maya; Zarzour, Hana; Ghoury, Ifad; Hebbo, Barea


    In order to characterize mutations causing rifampicin and isoniazid resistance of M. tuberculosis in Syria, 69 rifampicin resistant (Rif(r)) and 72 isoniazid resistant (Inh(r)) isolates were screened for point mutations in hot spots of the rpoB, katG and inhA genes by DNA sequencing and real time PCR. Of 69 Rif(r) isolates, 62 (90%) had mutations in the rifampin resistance determining region (RRDR) of the rpoB gene, with codons 531 (61%), 526 (13%), and 516 (8.7%) being the most commonly mutated. We found two new mutations (Asp516Thr and Ser531Gly) described for the first time in the rpoB-RRDR in association with rifampicin resistance. Only one mutation (Ile572Phe) was found outside the rpoB-RRDR. Of 72 Inh(r) strains, 30 (41.6%) had a mutation in katGcodon315 (with Ser315Thr being the predominant alteration), and 23 (32%) harbored the inhA(-15C-->T) mutation. While the general pattern of rpoB-RRDR and katG mutations reflected those found worldwide, the prevalence of the inhA(-15C-->T mutation was above the value found in most other countries, emphasizing the great importance of testing the inhA(-15C-->T) mutation for prediction of isoniazid resistance in Syria. Sensitivity of a rapid test using real time PCR and 3'-Minor groove binder (MGB) probes in detecting Rif(r) and Inh(r) isolates was 90% and 69.4%, respectively. This demonstrates that a small set of MGB-probes can be used in real time PCR in order to detect most mutations causing resistance to rifampicin and isoniazid.

  9. Silica Brick for Hot Blast Stove

    Institute of Scientific and Technical Information of China (English)


    @@ 1 Scope This standard specifies the glossary and definition, marking, shape and dimension, technical requirements, test method, quality appraisal procedure, packing, label, transportation, storage and quality certification of silica brick for hot blast stove.

  10. Fireclay Refractory Bricks for Hot Blast Stove

    Institute of Scientific and Technical Information of China (English)

    Wang Jing; Zhang Yongfang; Chai Junlan


    @@ 1 Scope This standard specifies the classification, shape, dimension, technical requirements, test method,inspection rules, packing, marking,transportation,storage and quality certification of fireclay refractory bricks for hot blast stove.

  11. Hot Flashes amd Night Sweats (PDQ) (United States)

    ... Other Funding Find NCI funding for small business innovation, technology transfer, and contracts Training Cancer Training at ... remedies for hot flashes. These include dong quai , milk thistle , red clover , licorice root extract , and chaste ...

  12. Hot-Electron Transfer from Semiconductor Nanocrystals

    National Research Council Canada - National Science Library

    William A. Tisdale; Kenrick J. Williams; Brooke A. Timp; David J. Norris; Eray S. Aydil; X.-Y. Zhu


    ... that limits device efficiency. Although fabricating the semiconductor in a nanocrystalline morphology can slow this cooling, the transfer of hot carriers to electron and hole acceptors has not yet been thoroughly demonstrated...

  13. Thermal substructure of hot deformed austenite substructure

    Energy Technology Data Exchange (ETDEWEB)

    Bernshtejn, M.L.; Kaputkina, L.M.; Nikishov, N.A. (Moskovskij Inst. Stali i Splavov (USSR))


    Effect of hot working different regimes on formation of austenite structure and substructure of the 60N20 and 60Kh5G6 steels and kinetics of softening processes at postdeformation isothermal (at deformation temperature) heating, is investigated. It is shown, that variation of hot working regimes permits to obtain a wide range of structural and substructural austenite conditions. Rate decrease and temperature increase promotes obtaining after hot working and conservation under cooling conditions of developed polygonized substructure. Similar polygonized isotropic substructure with a rather low density of dislocations inside of subgrains promotes decelerating of initial stages of recrystallization development under conditions of hot working and regulated post-deformation heatings. Alloying by carbide-forming elements (chromium and manganese) delays development of recrystallization (in comparison with alloying with nickel), even if the steel is in the condition of single-phase solid solution.

  14. Hot-wire anemometry in transonic flow (United States)

    Horstman, C. C.; Rose, W. C.


    The use of hot-wire anemometry for obtaining fluctuating data in transonic flows has been evaluated. From hot-wire heat loss correlations based on previous transonic data, the sensitivity coefficients for velocity, density, and total temperature fluctuations have been calculated for a wide range of test conditions and sensor parameters. For sensor Reynolds number greater than 20 and high sensor overheat ratios, the velocity sensitivity remains independent of Mach number and equal to the density sensitivity. These conditions were verified by comparisons of predicted sensitivities with those from recent direct calibrations in transonic flows. Based on these results, techniques are presented to obtain meaningful measurements of fluctuating velocity, density, and Reynolds shear stress using hot-wire and hot-film anemometers. Example of these measurements are presented for two transonic boundary layers.

  15. Frequency Responses Of Hot-Wire Anemometers (United States)

    Watmuff, Jonathan H.


    Report describes theoretical study of frequency response of constant-temperature hot-wire anemometer, with view toward increasing frequency response while maintaining stable operation in supersonic flow. Effects of various circuit parameters discussed.

  16. Hot wire anemometry in transonic flow (United States)

    Horstman, C. C.; Rose, W. C.


    The use of hot-wire anemometry for obtaining fluctuating data in transonic flows has been evaluated. From hot-wire heat loss correlations based on previous transonic data, the sensitivity coefficients for velocity, density, and total temperature fluctuations have been calculated for a wide range of test conditions and sensor parameters. For sensor Reynolds numbers greater than 20 and high sensor overheat ratios, the velocity sensitivity remains independent of Mach number and equal to the density sensitivity. These conclusions were verified by comparisons of predicted sensitivities with those from recent direct calibrations in transonic flows. Based on these results, techniques are presented to obtain meaningful measurements of fluctuating velocity, density, and Reynolds shear stress using hot-wire and hot-film anemometers. Examples of these measurements are presented for two transonic boundary layers.

  17. Bouncing Balls and Hot Rod Races. (United States)

    Tibbs, Peggy; Sherrill, Donna

    This paper presents the Bouncing Ball Experiment which models quadratic and exponential functions, and the Hot Rod Races activity that explores velocity and acceleration. Activities include directions for the use of TI-82 and TI-83 calculators. (YDS)

  18. Hot Tub Rash (Pseudomonas Dermatitis/Folliculitis) (United States)

    ... Español [PDF - 1 page] "Hot Tub Rash" ( Pseudomonas Dermatitis / Folliculitis) If contaminated water comes in contact ... is often caused by infection with the germ Pseudomonas aeruginosa . This germ is common in the environment ( ...

  19. VT New Market Tax Credit - Hot Zones (United States)

    Vermont Center for Geographic Information — (Link to Metadata) The EconOther_NMTC layer delineates New Market Tax Credit (NMTC) "hot zones" and qualified counties and census tracts. This dataset is designed to...

  20. Semi-Supervised Transductive Hot Spot Predictor Working on Multiple Assumptions

    KAUST Repository

    Wang, Jim Jing-Yan


    Protein-protein interactions are critically dependent on just a few residues (“hot spots”) at the interfaces. Hot spots make a dominant contribution to the binding free energy and if mutated they can disrupt the interaction. As mutagenesis studies require significant experimental efforts, there exists a need for accurate and reliable computational hot spot prediction methods. Compared to the supervised hot spot prediction algorithms, the semi-supervised prediction methods can take into consideration both the labeled and unlabeled residues in the dataset during the prediction procedure. The transductive support vector machine has been utilized for this task and demonstrated a better prediction performance. To the best of our knowledge, however, none of the transductive semi-supervised algorithms takes all the three semisupervised assumptions, i.e., smoothness, cluster and manifold assumptions, together into account during learning. In this paper, we propose a novel semi-supervised method for hot spot residue prediction, by considering all the three semisupervised assumptions using nonlinear models. Our algorithm, IterPropMCS, works in an iterative manner. In each iteration, the algorithm first propagates the labels of the labeled residues to the unlabeled ones, along the shortest path between them on a graph, assuming that they lie on a nonlinear manifold. Then it selects the most confident residues as the labeled ones for the next iteration, according to the cluster and smoothness criteria, which is implemented by a nonlinear density estimator. Experiments on a benchmark dataset, using protein structure-based features, demonstrate that our approach is effective in predicting hot spots and compares favorably to other available methods. The results also show that our method outperforms the state-of-the-art transductive learning methods.

  1. [Mutations and their significance in the corepromoter region of hepatitis B virus]. (United States)

    Tu, H; Wen, Y; Xiong, S


    To study the mutations in the core promoter (CP) region of hepatitis B virus (HBV) in Chinese viral hepatits B patients. CP regions of 48 HBV strains were analysed by polymerase chain reaction (PCR) direct sequencing approach. 67% of the samples were detected to have point mutations in the CP region. The hot spots located in nt 1754-1766 and nt 1801-1811 while nucleotides in nt 1777-1800 and nt 1812-1836 were highly conserved. nt. 1764 mutation was present in HBeAg negative patients. There were 16 point mutations in region overlapping X gene, 9 of which lead to amino acid change. Though mutations in CP region of HBV appear frequently, the sequences associated with viral transcription are rarely changed. Point mutation at nt 1764 is related to HBeAg negative phenotype, but it is not the specific mutation of fulminant hepatitis. The importance of mutations in the X gene overlapping region needs to be further investigated.

  2. Mutations induced by monofunctional and bifunctional phosphoramide mustards in supF tRNA gene. (United States)

    Mudipalli, A; Maccubbin, A E; Nadadur, S S; Struck, R F; Gurtoo, H L


    The relative mutagenicity, nature of the mutations and the sequence specificity of mutations induced by the bifunctional alkylating agent, phosphoramide mustard (PM) and a monofunctional derivative, dechloroethyl phosphoramide mustard (dePM), were analyzed by the Ames test and by an in vitro shuttle vector mutagenesis assay. Both PM and dePM increased the mutation frequency above background in either assay. However, on an equimolar basis, dePM was less mutagenic than PM. In the in vitro shuttle vector mutagenesis assay, sequencing demonstrated that about 40% of the mutant plasmids contained more than one mutation in the supF tRNA gene segment of the plasmid. About 70% of the mutations observed in dePM-treated plasmids were single base substitutions with A:T and G:C base pairs being mutated at equivalent rates. In contrast, only about 50% of the mutations observed in PM-treated plasmids were single base substitutions, 80% of which involved G:C base pairs. Single base deletions and insertions were found in approximately equal proportions with both compounds; however, these lesions were in greater abundance in PM-treated plasmids. Putative hot-spots for mutation in the supF tRNA gene included base pairs at position 102 and 110 for PM and positions 170 and 171 for dePM.

  3. Hot ductility of continuously cast structural steels

    Energy Technology Data Exchange (ETDEWEB)

    Pytel, S.M. [Materials Science and Technology Institute, Cracow University of Technology, Cracow (Poland)


    The objective of this investigation was to explain the hot ductility of the structural steels characterized by different amount of carbon and morphology of sulfides. Two different rolling processes were simulated under computer controlled, high temperature deformation MTS system. Results of this study show that morphology of sulfides as well as temperature and amount of deformation are responsible for level of hot ductility of the steel tested. (author). 7 refs, 5 refs, 4 tabs.

  4. Sealed source dismantling hot cell - startup

    Energy Technology Data Exchange (ETDEWEB)

    Dellamano, Jose Claudio; Ferreira, Robson de Jesus, E-mail:, E-mail: [Instituto de Pesquisas Energeticas e Nucleares (IPEN/CNEN-SP), Sao Paulo, SP (Brazil). Gerencia de Rejeitos radioativos


    Sealed radioactive sources are widely used in many applications of nuclear technology and at the end of the useful life, most sources become radioactive waste. In Brazil, this waste is received by the Institutes of the National Nuclear Energy Commission and kept under centralized storage. The Waste Management Department at the Nuclear and Energy Research Institute is the main storage center, having received around 20,000 disused sources. A hot cell was designed and constructed to manage Co-60 spent sealed sources with activity up to 3.7 10{sup 1}0 Bq and other sources with equivalent activities. In the hot cell the sources are withdraw from their original shielding and transferred to a standard shielding for further disposal off. The original shielding disassembling is made outside the hot cell and after opening, it is transferred inside the hot cell and the sealed source is removed remotely. The source is checked in relation to external contamination and its activity is checked. After this, the source is positioned in the standard shielding located inside an overpack at the bottom of the hot cell. This paper describes some pre-operational tests carried out in it, that include: opening and closing doors and locks, checking of all electrical and pneumatic controls, the original shielding movement inside the hot-cell, dose rate measurements outside the hot-cell, insertion of the sealed sources inside the activity meter chamber, transferring the sealed source to the standard shielding, movement of the overpack with the standard shielding to outside of the hot-cell and plugging of the standard shielding. (author)

  5. Oxidation And Hot Corrosion Of ODS Alloy (United States)

    Lowell, Carl E.; Barrett, Charles A.


    Report reviews oxidation and hot corrosion of oxide-dispersion-strengthened (ODS) alloys, intended for use at high temperatures. Classifies environmental resistances of such alloys by rates of growth of oxides, volatilities of oxides, spalling of oxides, and limitations imposed by hot corrosion. Also discusses environmentally resistant coatings for ODS materials. Concludes ODS NICrAl and FeCrAl alloys highly resistant to oxidation and corrosion and can be used uncoated.

  6. Modeling deflagration waves out of hot spots (United States)

    Partom, Yehuda


    It is widely accepted that shock initiation and detonation of heterogeneous explosives comes about by a two-step process known as ignition and growth. In the first step a shock sweeping through an explosive cell (control volume) creates hot spots that become ignition sites. In the second step, deflagration waves (or burn waves) propagate out of those hot spots and transform the reactant in the cell into reaction products. The macroscopic (or average) reaction rate of the reactant in the cell depends on the speed of those deflagration waves and on the average distance between neighboring hot spots. Here we simulate the propagation of deflagration waves out of hot spots on the mesoscale in axial symmetry using a 2D hydrocode, to which we add heat conduction and bulk reaction. The propagation speed of the deflagration waves may depend on both pressure and temperature. It depends on pressure for quasistatic loading near ambient temperature, and on temperature at high temperatures resulting from shock loading. From the simulation we obtain deflagration fronts emanating out of the hot spots. For 8 to 13 GPa shocks, the emanating fronts propagate as deflagration waves to consume the explosive between hot spots. For higher shock levels deflagration waves may interact with the sweeping shock to become detonation waves on the mesoscale. From the simulation results we extract average deflagration wave speeds.

  7. Warm-plus-hot neutrino dark matter

    CERN Document Server

    Malaney, R A; Widrow, L M; Malaney, R A; Starkman, G D; Widrow, L


    We investigate a new hybrid-model universe containing two types of dark matter, one ``warm'' and the other ``hot''. The hot component is an ordinary light neutrino with mass \\sim 25h^2~eV while the warm component is a sterile neutrino with mass \\sim 700h^2~eV. The two types of dark matter arise entirely within the neutrino sector and do not require separate physical origins. We calculate the linear transfer functions for a representative sample of warm-plus-hot models. The transfer functions, and results from several observational tests of structure formation, are compared with those for the cold-plus-hot models that have been studied extensively in the literature. On the basis of these tests, we conclude that warm-plus-hot dark matter is essentially indistinguishable from cold-plus-hot dark matter, and therefore provides a viable scenario for large scale structure. We demonstrate that a neutrino mass matrix can be constructed which provides the requisite dark matter constituents, while remaining consistent w...

  8. Disaggregating Hot Water Use and Predicting Hot Water Waste in Five Test Homes

    Energy Technology Data Exchange (ETDEWEB)

    Henderson, Hugh [ARIES Collaborative, New York, NY (United States); Wade, Jeremy [ARIES Collaborative, New York, NY (United States)


    While it is important to make the equipment (or "plant") in a residential hot water system more efficient, the hot water distribution system also affects overall system performance and energy use. Energy wasted in heating water that is not used is estimated to be on the order of 10%-30% of total domestic hot water (DHW) energy use. This field monitoring project installed temperature sensors on the distribution piping (on trunks and near fixtures) in five houses near Syracuse, NY, and programmed a data logger to collect data at 5 second intervals whenever there was a hot water draw. This data was used to assign hot water draws to specific end uses in the home as well as to determine the portion of each hot water that was deemed useful (i.e., above a temperature threshold at the fixture). Overall, the procedures to assign water draws to each end use were able to successfully assign about 50% of the water draws, but these assigned draws accounted for about 95% of the total hot water use in each home. The amount of hot water deemed as useful ranged from low of 75% at one house to a high of 91% in another. At three of the houses, new water heaters and distribution improvements were implemented during the monitoring period and the impact of these improvements on hot water use and delivery efficiency were evaluated.

  9. Disaggregating Hot Water Use and Predicting Hot Water Waste in Five Test Homes

    Energy Technology Data Exchange (ETDEWEB)

    Henderson, H.; Wade, J.


    While it is important to make the equipment (or 'plant') in a residential hot water system more efficient, the hot water distribution system also affects overall system performance and energy use. Energy wasted in heating water that is not used is estimated to be on the order of 10 to 30 percent of total domestic hot water (DHW) energy use. This field monitoring project installed temperature sensors on the distribution piping (on trunks and near fixtures) and programmed a data logger to collect data at 5 second intervals whenever there was a hot water draw. This data was used to assign hot water draws to specific end uses in the home as well as to determine the portion of each hot water that was deemed useful (i.e., above a temperature threshold at the fixture). Five houses near Syracuse NY were monitored. Overall, the procedures to assign water draws to each end use were able to successfully assign about 50% of the water draws, but these assigned draws accounted for about 95% of the total hot water use in each home. The amount of hot water deemed as useful ranged from low of 75% at one house to a high of 91% in another. At three of the houses, new water heaters and distribution improvements were implemented during the monitoring period and the impact of these improvements on hot water use and delivery efficiency were evaluated.

  10. Filaggrin mutations and the skin

    Directory of Open Access Journals (Sweden)

    Dipankar De


    Full Text Available Filaggrin is very important in the terminal differentiation of the skin and the formation of cornified envelope in the stratum corneum. Several mutations in the filaggrin gene have been identified in the last decade, mostly from the European countries. Loss of function mutations in the filaggrin gene results in reduced production of filaggrin, depending on the type and site of mutation. Such mutations in the filaggrin gene have been shown to be the most significant genetic risk factor for development of atopic dermatitis and undoubtedly has a role in the pathogenesis of ichthyosis vulgaris. Though there is theoretical possibility of association with hand eczema and allergic contact dermatitis; in clinical studies, the strength of these associations was not significantly strong. In this review, we have discussed the structure and function of filaggrin, basic genetics, type of mutations in filaggrin gene, and association of such mutations with different dermatoses.

  11. Muller's ratchet with compensatory mutations

    CERN Document Server

    Pfaffelhuber, Peter; Wakolbinger, Anton


    We consider an infinite dimensional system of stochastic differential equations which describes the evolution of type frequencies in a large population. Random reproduction is modeled by a Wright-Fisher noise whose inverse diffusion coefficient $N$ corresponds to the total population size. The type of an individual is the number $k$ of deleterious mutations it carries. We assume that fitness of individuals carrying $k$ mutations is decreased by $\\alpha k$ for some $\\alpha >0$. Along the individual lines of descent, (new) mutations accumulate at rate $\\lambda$ per generation, and each of these mutations has a small probability $\\gamma$ per generation to disappear. While the case $\\gamma =0 $ is known as (the Fleming-Viot version of) {\\em Muller's ratchet}, the case $\\gamma > 0$ is referred to as that of {\\em compensatory mutations} in the biological literature. In the former case ($\\gamma=0$), an ever increasing number of mutations is accumulated over time, while in the latter ($\\gamma > 0$) this is prevented ...

  12. Filaggrin mutations and the skin. (United States)

    De, Dipankar; Handa, Sanjeev


    Filaggrin is very important in the terminal differentiation of the skin and the formation of cornified envelope in the stratum corneum. Several mutations in the filaggrin gene have been identified in the last decade, mostly from the European countries. Loss of function mutations in the filaggrin gene results in reduced production of filaggrin, depending on the type and site of mutation. Such mutations in the filaggrin gene have been shown to be the most significant genetic risk factor for development of atopic dermatitis and undoubtedly has a role in the pathogenesis of ichthyosis vulgaris. Though there is theoretical possibility of association with hand eczema and allergic contact dermatitis; in clinical studies, the strength of these associations was not significantly strong. In this review, we have discussed the structure and function of filaggrin, basic genetics, type of mutations in filaggrin gene, and association of such mutations with different dermatoses.

  13. Mutations induced in plant breeding

    Energy Technology Data Exchange (ETDEWEB)

    Barriga B, P. (Universidad Austral de Chile, Valdivia. Inst. de Produccion y Sanidad Vegetal)


    The most significant aspects of the use of ionizing radiations in plant breeding are reviewed. Aspects such as basic principles of mutation, expression and selection in obtention of mutants, methods for using induced mutations and sucess achieved with this methodology in plant breeding are reviewed. Results obtained in a program of induced mutation on wheat for high content of protein and lysine at the Universidad Austral de Chile are presented.

  14. Mutations in PIK3CA are infrequent in neuroblastoma

    Directory of Open Access Journals (Sweden)

    Mazanek Pavel


    Full Text Available Abstract Background Neuroblastoma is a frequently lethal pediatric cancer in which MYCN genomic amplification is highly correlated with aggressive disease. Deregulated MYC genes require co-operative lesions to foster tumourigenesis and both direct and indirect evidence support activated Ras signaling for this purpose in many cancers. Yet Ras genes and Braf, while often activated in cancer cells, are infrequent targets for activation in neuroblastoma. Recently, the Ras effector PIK3CA was shown to be activated in diverse human cancers. We therefore assessed PIK3CA for mutation in human neuroblastomas, as well as in neuroblastomas arising in transgenic mice with MYCN overexpressed in neural-crest tissues. In this murine model we additionally surveyed for Ras family and Braf mutations as these have not been previously reported. Methods Sixty-nine human neuroblastomas (42 primary tumors and 27 cell lines were sequenced for PIK3CA activating mutations within the C2, helical and kinase domain "hot spots" where 80% of mutations cluster. Constitutional DNA was sequenced in cases with confirmed alterations to assess for germline or somatic acquisition. Additionally, Ras family members (Hras1, Kras2 and Nras and the downstream effectors Pik3ca and Braf, were sequenced from twenty-five neuroblastomas arising in neuroblastoma-prone transgenic mice. Results We identified mutations in the PIK3CA gene in 2 of 69 human neuroblastomas (2.9%. Neither mutation (R524M and E982D has been studied to date for effects on lipid kinase activity. Though both occurred in tumors with MYCN amplification the overall rate of PIK3CA mutations in MYCN amplified and single-copy tumors did not differ appreciably (2 of 31 versus 0 of 38, respectively. Further, no activating mutations were identified in a survey of Ras signal transduction genes (including Hras1, Kras2, Nras, Pik3ca, or Braf genes in twenty-five neuroblastic tumors arising in the MYCN-initiated transgenic mouse model

  15. Analysis of EML4-ALK Gene Fusion Mutation in Patients 
with Non-small Cell Lung Cancer

    Directory of Open Access Journals (Sweden)

    Xuzhou WANG


    Full Text Available Background and objective Non-small cell lung cancer (NSCLC is the main type of lung cancer, and the related locus mutation detection research has become a hot direction of molecular targeted therapy, studying on gene mutation status of echinodem microtubule associated protein like 4-Anaplastic lymphoma kinase (EML4-ALK and epidermal growth factor receptor (EGFR, detecting the sensitivity of EML4-ALK gene fusion and gene mutation of EGFR. Methods EML4-ALK gene fusion in 85 cases of paraffin embedded tumor tissue and adjacent lung tissue was detected with the application of immunohistochemistry (IHC, Scorpions amplification refractory mutation system (Scorpions ARMS fluorescence quantitative PCR and fluorescence in situ hybridization (FISH technology, and EGFR gene in 18, 19, 20 and 21 exon mutation status was detected with the application of ARMS method. Results In 115 cases of NSCLC, IHC showed 32 cases with ALK (D5F3 expression, the expression rate was 27.8%; ARMS showed 27 cases with EML4-ALK fusion gene mutation, the mutation detection rate was 23.5%; 53 cases were detected with EGFR mutation, the mutation rate was 46%. While FISH showed 23 cases with EML4-ALK fusion gene mutation, the detection rate was 20%, slightly lower than the ARMS detection results, suggesting that ARMS more sensitive. Conclusion The application of IHC, ARMS fluorescence quantitative PCR and FISH technology can make a rapid and accurate evaluation of EML4-ALK gene fusion.


    DEFF Research Database (Denmark)

    Furbo, Simon


    A proposal for a test procedure for hot water comfort for solar heating systems for combined space heating and domestic hot water supply was worked out.......A proposal for a test procedure for hot water comfort for solar heating systems for combined space heating and domestic hot water supply was worked out....

  17. Positive selection for new disease mutations in the human germline: evidence from the heritable cancer syndrome multiple endocrine neoplasia type 2B.

    Directory of Open Access Journals (Sweden)

    Soo-Kyung Choi

    Full Text Available Multiple endocrine neoplasia type 2B (MEN2B is a highly aggressive thyroid cancer syndrome. Since almost all sporadic cases are caused by the same nucleotide substitution in the RET proto-oncogene, the calculated disease incidence is 100-200 times greater than would be expected based on the genome average mutation frequency. In order to determine whether this increased incidence is due to an elevated mutation rate at this position (true mutation hot spot or a selective advantage conferred on mutated spermatogonial stem cells, we studied the spatial distribution of the mutation in 14 human testes. In donors aged 36-68, mutations were clustered with small regions of each testis having mutation frequencies several orders of magnitude greater than the rest of the testis. In donors aged 19-23 mutations were almost non-existent, demonstrating that clusters in middle-aged donors grew during adulthood. Computational analysis showed that germline selection is the only plausible explanation. Testes of men aged 75-80 were heterogeneous with some like middle-aged and others like younger testes. Incorporating data on age-dependent death of spermatogonial stem cells explains the results from all age groups. Germline selection also explains MEN2B's male mutation bias and paternal age effect. Our discovery focuses attention on MEN2B as a model for understanding the genetic and biochemical basis of germline selection. Since RET function in mouse spermatogonial stem cells has been extensively studied, we are able to suggest that the MEN2B mutation provides a selective advantage by altering the PI3K/AKT and SFK signaling pathways. Mutations that are preferred in the germline but reduce the fitness of offspring increase the population's mutational load. Our approach is useful for studying other disease mutations with similar characteristics and could uncover additional germline selection pathways or identify true mutation hot spots.

  18. Positive selection for new disease mutations in the human germline: evidence from the heritable cancer syndrome multiple endocrine neoplasia type 2B. (United States)

    Choi, Soo-Kyung; Yoon, Song-Ro; Calabrese, Peter; Arnheim, Norman


    Multiple endocrine neoplasia type 2B (MEN2B) is a highly aggressive thyroid cancer syndrome. Since almost all sporadic cases are caused by the same nucleotide substitution in the RET proto-oncogene, the calculated disease incidence is 100-200 times greater than would be expected based on the genome average mutation frequency. In order to determine whether this increased incidence is due to an elevated mutation rate at this position (true mutation hot spot) or a selective advantage conferred on mutated spermatogonial stem cells, we studied the spatial distribution of the mutation in 14 human testes. In donors aged 36-68, mutations were clustered with small regions of each testis having mutation frequencies several orders of magnitude greater than the rest of the testis. In donors aged 19-23 mutations were almost non-existent, demonstrating that clusters in middle-aged donors grew during adulthood. Computational analysis showed that germline selection is the only plausible explanation. Testes of men aged 75-80 were heterogeneous with some like middle-aged and others like younger testes. Incorporating data on age-dependent death of spermatogonial stem cells explains the results from all age groups. Germline selection also explains MEN2B's male mutation bias and paternal age effect. Our discovery focuses attention on MEN2B as a model for understanding the genetic and biochemical basis of germline selection. Since RET function in mouse spermatogonial stem cells has been extensively studied, we are able to suggest that the MEN2B mutation provides a selective advantage by altering the PI3K/AKT and SFK signaling pathways. Mutations that are preferred in the germline but reduce the fitness of offspring increase the population's mutational load. Our approach is useful for studying other disease mutations with similar characteristics and could uncover additional germline selection pathways or identify true mutation hot spots.

  19. 促黄体激素受体突变导致的性器官发育异常%Disorders of sexual development caused by luteinizing hormone receptor mutations

    Institute of Scientific and Technical Information of China (English)

    Wai-Yee CHAN


    SUMMARY The Luteinizing hormone/chorionic gonadotropin receptor (LHR) plays a critical role in human male sexual development. Both gain-of-function and loss-of-function mutations of the LHR have been described. Gain-of-function mutations are dominant and cause constitutive activation of the receptor resulting in familial male-limited precocious puberty (FMPP). All activating mutations are single point mutations and are located in the transmembrane domain (TM). TM helix Ⅵ harbors the largest number of activating mutations with the codon of Asp-578 being the hot-spot of mutation. Besides causing abnormal sexual development, constitutively activated LHR may predispose an individual to the development of testicular neoplasia. The anti-thesis of FMPP is Leydig cell hypoplasia (LCH). This is caused by mutations that inactivate the LHR resulting in subnormal male sexual development or male pseudohermaphroditism. Inactivating mutations are recessive. The genetic cause of LCH is variable and there is no mutation hot-spot. Genotype-phenotype correlation can be identified in LCH with the milder form caused by mutated LHR with residual activity and the severe form caused by absence of signal transduction activity of the mutated receptor. Molecular diagnosis of the disorders caused by mutation of the LHR can be achieved by direct sequencing of the LHR gene.

  20. Experiments with the hot list strategy

    Energy Technology Data Exchange (ETDEWEB)

    Wos, L.


    Experimentation strongly suggests that, for attacking deep questions and hard problems with the assistance of an automated reasoning program, the more effective paradigms rely on the retention of deduced information. A significant obstacle ordinarily presented by such a paradigm is the deduction and retention of one or more needed conclusions whose complexity sharply delays their consideration. To mitigate the severity of the cited obstacle, the author formulates and features in this report the hot list strategy. The hot list strategy asks the researcher to choose, usually from among the input statements, one or more clauses that are conjectured to play a key role for assignment completion. The chosen clauses - conjectured to merit revisiting, again and again - are placed in an input list of clauses, called the hot list. When an automated reasoning program has decided to retain a new conclusion C - before any other clause is chosen to initiate conclusion drawing - the presence of a nonempty hot list (with an appropriate assignment of the input parameter known as heat) causes each inference rule in use to be applied to C together with the appropriate number of members of the hot list. Members of the hot list are used to complete applications of inference rules and not to initiate applications. The use of the hot list strategy thus enables an automated reasoning program to briefly consider a newly retained conclusion whose complexity would otherwise prevent its use for perhaps many CPU-hours. To give evidence of the value of the strategy, the author focuses on four contexts: (1) dramatically reducing the CPU time required to reach a desired goal; (2) finding a proof of a theorem that had previously resisted all but the more inventive automated attempts; (3) discovering a proof that is more elegant than previously known; and (4) answering a question that had steadfastly eluded researchers relying on an automated reasoning program.

  1. Spontaneous recurrent mutations and a complex rearrangement in the MECP2 gene in the light of current models of mutagenesis. (United States)

    Todorov, Tihomir; Todorova, Albena; Motoescu, Cristina; Dimova, Petia; Iancu, Daniela; Craiu, Dana; Stoian, Daniela; Barbarii, Ligia; Bojinova, Veneta; Mitev, Vanyo


    Mutations in the methyl-CpG-binding protein 2 (MECP2) gene are associated with Rett syndrome (RTT). The MECP2 gene has some unique characteristics: (1) it is mainly affected by de novo mutations, due to recurrent independent mutational events in a defined "hot spot" regions or positions; (2) complex mutational events along a single allele are frequently found in this gene; (3) most mutations arise on paternal X chromosome. The recurrent point mutations involve mainly CpG dinucleotides, where C>T transitions are explained by methylation-mediated deamination. The complex mutational events might be explained by the genomic architecture of the region involving the MECP2 gene. The finding that most spontaneous mutations arise on paternal X-chromosome supports the higher contribution of replication-mediated mechanism of mutagenesis. We present 9 types of mutations in the MECP2 gene, detected in a group of 22 Bulgarian and 6 Romanian classical RTT patients. Thirteen patients were clarified on molecular level (46.4%). The point mutations in our sample account for 61.5%. One intraexonic deletion was detected in the present study (7.7%). One novel insertion c.321_322insGAAG, p.(Lys107_Leu108insGluAlafs2*) was found (7.7%). Large deletions and complex mutations account for 23%. A novel complex mutational event c.[584_624del41insTT; 638delTinsCA] was detected in a Romanian patient. We discuss different types of the MECP2 mutations detected in our sample in the light of the possible mechanisms of mutagenesis. Complex gene rearrangements involving a combination of deletions and insertions have always been most difficult to detect, to specify precisely and hence to explain in terms of their underlying mutational mechanisms.

  2. Simplifying the detection of MUTYH mutations by high resolution melting analysis

    Directory of Open Access Journals (Sweden)

    López-Villar Isabel


    Full Text Available Abstract Background MUTYH-associated polyposis (MAP is a disorder caused by bi-allelic germline MUTYH mutation, characterized by multiple colorectal adenomas. In order to identify mutations in MUTYH gene we applied High Resolution Melting (HRM genotyping. HRM analysis is extensively employed as a scanning method for the detection of heterozygous mutations. Therefore, we applied HRM to show effectiveness in detecting homozygous mutations for these clinically important and frequent patients. Methods In this study, we analyzed phenotype and genotype data from 82 patients, with multiple (>= 10 synchronous (19/82 or metachronous (63/82 adenomas and negative APC study (except one case. Analysis was performed by HRM-PCR and direct sequencing, in order to identify mutations in MUTYH exons 7, 12 and 13, where the most prevalent mutations are located. In monoallelic mutation carriers, we evaluated entire MUTYH gene in search of another possible alteration. HRM-PCR was performed with strict conditions in several rounds: the first one to discriminate the heteroduplex patterns and homoduplex patterns and the next ones, in order to refine and confirm parameters. The genotypes obtained were correlated to phenotypic features (number of adenomas (synchronous or metachronous, colorectal cancer (CRC and family history. Results MUTYH germline mutations were found in 15.8% (13/82 of patients. The hot spots, Y179C (exon 7 and G396D (exon 13, were readily identified and other mutations were also detected. Each mutation had a reproducible melting profile by HRM, both heterozygous mutations and homozygous mutations. In our study of 82 patients, biallelic mutation is associated with being a carrier of ≥10 synchronous polyps (p = 0.05 and there is no association between biallelic mutation and CRC (p = 0.39 nor family history (p = 0.63. G338H non-pathogenic polymorphism (exon 12 was found in 23.1% (19/82 of patients. In all cases there was concordance between HRM (first

  3. Identification of the keratin K9 R162W mutation in patients of Italian origin with epidermolytic palmoplantar keratoderma. (United States)

    Terrinoni, Alessandro; Cocuroccia, Barbara; Gubinelli, Emanuela; Zambruno, Giovanna; Candi, Eleonora; Melino, Gerry; Girolomoni, Giampiero


    Epidermolytic palmoplantar keratoderma (EPPK) is an autosomal dominant skin disorder characterized by hyperkeratosis of the palms and soles associated with histologic findings of hyperkeratosis and epidermolysis. Ultrastructurally, there is vacuolization of the cytoplasm and abnormal keratin filament network with tonofilament clumping. EPPK is caused by mutations in the keratin 9 gene (KRT9), which is expressed exclusively in suprabasal keratinocytes of palmoplantar epidermis. The mutation R162W is the most frequent keratin 9 alteration reported in patients from different geographical areas. We present three unrelated Italian families affected by EPPK in which we confirmed the presence of the R162W mutation, by RT-PCR analysis followed by sequencing of the KRT9 gene, in all affected members. The finding of the same mutation in all patients, together with the previous reports of the disease, strongly suggest that position 162 of the KRT9 gene represents a mutation "hot-spot", probably due to the peculiarity of the sequence.

  4. A novel mutation of keratin 9 gene (R162P) in a Japanese family with epidermolytic palmoplantar keratoderma. (United States)

    Kon, Atsushi; Itagaki, Kae; Yoneda, Kozo; Takagaki, Keiichi


    Epidermolytic palmoplantar keratoderma (EPPK) is an autosomal dominant inherited skin disorder characterized by hyperkeratosis of the skin over the palms and soles. Mutations in keratin 9 gene (KRT9) have been demonstrated in EPPK. In this study, we screened a Japanese family with EPPK for KRT9 mutation by polymerase chain reaction amplification of genomic sequences, followed by heteroduplex analysis and direct nucleotide sequencing. The mutation consisted of a G-to-C transversion at codon 162 in exon 1, which was located in the hot spot of the mutations that have been reported previously (R162Q and R162W). However, the amino acid substitution was proline for arginine (R162P) in the 1A rod domain, the highly conserved helix initiation motif of keratin 9. Our result illustrates the repertoire of KRT9 mutation underlying the occurrence of EPPK in a Japanese family and is an important contribution to the investigation of the genotype/phenotype correlation.

  5. Four hot DOGs in the microwave

    CERN Document Server

    Frey, S; Gabányi, K É; An, T


    Hot dust-obscured galaxies (hot DOGs) are a rare class of hyperluminous infrared galaxies identified with the Wide-field Infrared Survey Explorer (WISE) satellite. The majority of them is at high redshifts (z~2-3), at the peak epoch of star formation in the Universe. Infrared, optical, radio, and X-ray data suggest that hot DOGs contain heavily obscured, extremely luminous active galactic nuclei (AGN). This class may represent a short phase in the life of the galaxies, signifying the transition from starburst- to AGN-dominated phases. Hot DOGs are typically radio-quiet, but some of them show mJy-level emission in the radio (microwave) band. We observed four hot DOGs using the technique of very long baseline interferometry (VLBI). The 1.7-GHz observations with the European VLBI Network (EVN) revealed weak radio features in all sources. The radio is free from dust obscuration and, at such high redshifts, VLBI is sensitive only to compact structures that are characteristic of AGN activity. In two cases (WISE J07...

  6. Influence of Hot Rolling Conditions on the Mechanical Properties of Hot Rolled TRIP Steel

    Institute of Scientific and Technical Information of China (English)

    LI Zhuang; WU Di


    Influence of hot rolling conditions on the mechanical properties of hot rolled TRIP steel was investigated.Thermomechanical control processing(TMCP)was conducted by using a laboratory hot rolling mill,in which three different kinds of finish rolling temperatures were applied.The results show that polygonal ferrite,granular bainite and larger amount of stabilized retained anstenite can be obtained by controlled rolling processes.The finer ferrite grain size is produced through the deformation induced transformation during deformation rather than after deformation,which affects the mechanical properties of hot rolled TRIP steel.Mechanical properties increase with decreasing finish rolling temperature due to the stabilization of retained austenite.Ultimate tensile strength(UTS),total elongation(TEL)and the product of ultimate tensile strength and total elongation(UTS×TEL)reaches optimal values(791 MPa,36% and 28 476 MPa%,respectively)whenthe specimen was hot rolled for 50% reduction at finish rolling temperature of 700℃.

  7. MPL mutations in myeloproliferative disorders

    DEFF Research Database (Denmark)

    Beer, Philip A.; Campbell, Peter J.; Scott, Linda M.


    Activating mutations of MPL exon 10 have been described in a minority of patients with idiopathic myelofibrosis (IMF) or essential thrombocythemia (ET), but their prevalence and clinical significance are unclear. Here we demonstrate that MPL mutations outside exon 10 are uncommon in platelet cDNA...

  8. Mutations in the 1A domain of keratin 9 in patients with epidermolytic palmoplantar keratoderma. (United States)

    Rothnagel, J A; Wojcik, S; Liefer, K M; Dominey, A M; Huber, M; Hohl, D; Roop, D R


    Epidermolytic palmoplantar keratoderma is an autosomal dominant skin disorder characterized by hyperkeratosis of the palms and soles. Ultrastructurally the disease exhibits abnormal keratin filament networks and tonofilament clumping like that found in the keratin disorders of epidermolysis bullosa simplex and epidermolytic hyperkeratosis. The disease has been mapped to chromosome 17q11-q23 in the region of the type 1 keratin gene locus and more recently mutations have been found in the palmoplantar specific keratin, keratin 9. We have analyzed six unrelated incidences of epidermolytic palmoplantar keratoderma for mutations in their keratin 9 genes. In two of these, we have identified mutations that alter critical residues within the highly conserved helix initiation motif at the beginning of the rod domain of keratin 9. In a three-generation Middle Eastern kindred we found a C to T transition at codon 162 that results in an arginine to tryptophan substitution at position 10 of the 1A alpha-helical domain, thus confirming this codon as a hot spot for mutation in keratin 9. The other mutation found involves a T to C transition at codon 167 that results in the expression of a serine residue in place of the normal leucine at position 15 of the 1A segment and is the first documentation of this mutation in this gene. The identification of these substitutions extends the current catalog of disease causing mutations in keratin 9.

  9. Radiation-Hydrodynamics of Hot Jupiter Atmospheres

    CERN Document Server

    Menou, Kristen


    Radiative transfer in planetary atmospheres is usually treated in the static limit, i.e., neglecting atmospheric motions. We argue that hot Jupiter atmospheres, with possibly fast (sonic) wind speeds, may require a more strongly coupled treatment, formally in the regime of radiation-hydrodynamics. To lowest order in v/c, relativistic Doppler shifts distort line profiles along optical paths with finite wind velocity gradients. This leads to flow-dependent deviations in the effective emission and absorption properties of the atmospheric medium. Evaluating the overall impact of these distortions on the radiative structure of a dynamic atmosphere is non-trivial. We present transmissivity and systematic equivalent width excess calculations which suggest possibly important consequences for radiation transport in hot Jupiter atmospheres. If winds are fast and bulk Doppler shifts are indeed important for the global radiative balance, accurate modeling and reliable data interpretation for hot Jupiter atmospheres may p...

  10. Kepler constraints on planets near hot Jupiters

    Energy Technology Data Exchange (ETDEWEB)

    Steffen, Jason H.; /Fermilab; Ragozzine, Darin; /Harvard-Smithsonian Ctr. Astrophys.; Fabrycky, Daniel C.; /UC, Santa Cruz, Astron. Astrophys.; Carter, Joshua A.; /Harvard-Smithsonian Ctr. Astrophys.; Ford, Eric B.; /Florida U.; Holman, Matthew J.; /Harvard-Smithsonian Ctr. Astrophys.; Rowe, Jason F.; /NASA, Ames; Welsh, William F.; /San Diego State U., Astron. Dept.; Borucki, William J.; /NASA, Ames; Boss, Alan P.; /Carnegie Inst., Wash., D.C., DTM; Ciardi, David R.; /Caltech /Harvard-Smithsonian Ctr. Astrophys.


    We present the results of a search for planetary companions orbiting near hot Jupiter planet candidates (Jupiter-size candidates with orbital periods near 3 d) identified in the Kepler data through its sixth quarter of science operations. Special emphasis is given to companions between the 2:1 interior and exterior mean-motion resonances. A photometric transit search excludes companions with sizes ranging from roughly two-thirds to five times the size of the Earth, depending upon the noise properties of the target star. A search for dynamically induced deviations from a constant period (transit timing variations) also shows no significant signals. In contrast, comparison studies of warm Jupiters (with slightly larger orbits) and hot Neptune-size candidates do exhibit signatures of additional companions with these same tests. These differences between hot Jupiters and other planetary systems denote a distinctly different formation or dynamical history.

  11. Archaeal diversity in Icelandic hot springs

    DEFF Research Database (Denmark)

    Kvist, Thomas; Ahring, Birgitte Kiær; Westermann, Peter


    Whole-cell density gradient extractions from three solfataras (pH 2.5) ranging in temperature from 81 to 90 degrees C and one neutral hot spring (81 degrees C, pH 7) from the thermal active area of Hveragerethi (Iceland) were analysed for genetic diversity and local geographical variation...... of Archaea by analysis of amplified 16S rRNA genes. In addition to the three solfataras and the neutral hot spring, 10 soil samples in transects of the soil adjacent to the solfataras were analysed using terminal restriction fragment length polymorphism (t-RFLP). The sequence data from the clone libraries...... enzymes AluI and BsuRI. The sequenced clones from this solfatara belonged to Sulfolobales, Thermoproteales or were most closest related to sequences from uncultured Archaea. Sequences related to group I.1b were not found in the neutral hot spring or the hyperthermophilic solfatara (90 degrees C)....

  12. Hot carrier degradation in semiconductor devices

    CERN Document Server


    This book provides readers with a variety of tools to address the challenges posed by hot carrier degradation, one of today’s most complicated reliability issues in semiconductor devices.  Coverage includes an explanation of carrier transport within devices and book-keeping of how they acquire energy (“become hot”), interaction of an ensemble of colder and hotter carriers with defect precursors, which eventually leads to the creation of a defect, and a description of how these defects interact with the device, degrading its performance. • Describes the intricacies of hot carrier degradation in modern semiconductor technologies; • Covers the entire hot carrier degradation phenomenon, including topics such as characterization, carrier transport, carrier-defect interaction, technological impact, circuit impact, etc.; • Enables detailed understanding of carrier transport, interaction of the carrier ensemble with the defect precursors, and an accurate assessment of how the newly created defects imp...

  13. Hot exciton dissociation in polymer solar cells. (United States)

    Grancini, G; Maiuri, M; Fazzi, D; Petrozza, A; Egelhaaf, H-J; Brida, D; Cerullo, G; Lanzani, G


    The standard picture of photovoltaic conversion in all-organic bulk heterojunction solar cells predicts that the initial excitation dissociates at the donor/acceptor interface after thermalization. Accordingly, on above-gap excitation, the excess photon energy is quickly lost by internal dissipation. Here we directly target the interfacial physics of an efficient low-bandgap polymer/PC(60)BM system. Exciton splitting occurs within the first 50 fs, creating both interfacial charge transfer states (CTSs) and polaron species. On high-energy excitation, higher-lying singlet states convert into hot interfacial CTSs that effectively contribute to free-polaron generation. We rationalize these findings in terms of a higher degree of delocalization of the hot CTSs with respect to the relaxed ones, which enhances the probability of charge dissociation in the first 200 fs. Thus, the hot CTS dissociation produces an overall increase in the charge generation yield.

  14. Modeling of large area hot embossing

    CERN Document Server

    Worgull, M; Marcotte, J -P; Hétu, J -F; Heckele, M


    Today, hot embossing and injection molding belong to the established plastic molding processes in microengineering. Based on experimental findings, a variety of microstructures have been replicated so far using the processes. However, with increasing requirements regarding the embossing surface and the simultaneous decrease of the structure size down into the nanorange, increasing know-how is needed to adapt hot embossing to industrial standards. To reach this objective, a German-Canadian cooperation project has been launched to study hot embossing theoretically by a process simulation and experimentally. The present publication shall report about the first results of the simulation - the modeling and simulation of large area replication based on an eight inch microstructured mold.

  15. Kepler constraints on planets near hot Jupiters

    CERN Document Server

    Steffen, Jason H; Fabrycky, Daniel C; Carter, Joshua A; Ford, Eric B; Holman, Matthew J; Rowe, Jason F; Welsh, William F; Borucki, William J; Boss, Alan P; Ciardi, David R; Quinn, Samuel N


    We present the results of a search for planetary companions orbiting near hot Jupiter planet candidates (Jupiter-size candidates with orbital periods near 3 days) identified in the Kepler data through its sixth quarter of science operations. Special emphasis is given to companions between the 2:1 interior and exterior mean-motion resonances. A photometric transit search excludes companions with sizes ranging from roughly 2/3 to 5 times the size of the Earth, depending upon the noise properties of the target star. A search for dynamically induced deviations from a constant period (transit timing variations or TTVs) also shows no significant signals. In contrast, comparison studies of warm Jupiters (with slightly larger orbits) and hot Neptune-size candidates do exhibit signatures of additional companions with these same tests. These differences between hot Jupiters and other planetary systems denote a distinctly different formation or dynamical history.

  16. Cool and hot flux ropes, their helicity (United States)

    Nindos, Alexander


    We will review recent indirect and direct evidence for the existence of magnetic flux ropes in the solar atmosphere. Magnetic flux ropes may appear as S-shaped or reverse S-shaped (sigmoidal) structures in regions that are likely to erupt, and may also show in nonlinear force-free field extrapolations that use data from photospheric vector magnetograms as boundary condition. The availability of high sensitivity data recorded with unprecedented spatial and temporal resolution in hot EUV wavelengths by the Atmospheric Imaging Assembly (AIA) onboard the Solar Dynamics Observatory (SDO) has revealed the existence of coherent structures identified as hot flux ropes. In this presentation, we will review the properties of both cool and hot flux ropes with an emphasis on the frequency of their occurrence in large flares and on their magnetic helicity content.

  17. Angular response of hot wire probes (United States)

    di Mare, L.; Jelly, T. O.; Day, I. J.


    A new equation for the convective heat loss from the sensor of a hot-wire probe is derived which accounts for both the potential and the viscous parts of the flow past the prongs. The convective heat loss from the sensor is related to the far-field velocity by an expression containing a term representing the potential flow around the prongs, and a term representing their viscous effect. This latter term is absent in the response equations available in the literature but is essential in representing some features of the observed response of miniature hot-wire probes. The response equation contains only four parameters but it can reproduce, with great accuracy, the behaviour of commonly used single-wire probes. The response equation simplifies the calibration the angular response of rotated slanted hot-wire probes: only standard King’s law parameters and a Reynolds-dependent drag coefficient need to be determined.

  18. Hot Carrier extraction with plasmonic broadband absorbers

    CERN Document Server

    Ng, Charlene; Dligatch, Svetlana; Roberts, Ann; Davis, Timothy J; Mulvaney, Paul; Gomez, Daniel E


    Hot charge carrier extraction from metallic nanostructures is a very promising approach for applications in photo-catalysis, photovoltaics and photodetection. One limitation is that many metallic nanostructures support a single plasmon resonance thus restricting the light-to-charge-carrier activity to a spectral band. Here we demonstrate that a monolayer of plasmonic nanoparticles can be assembled on a multi-stack layered configuration to achieve broad-band, near-unit light absorption, which is spatially localised on the nanoparticle layer. We show that this enhanced light absorbance leads to $\\sim$ 40-fold increases in the photon-to-electron conversion efficiency by the plasmonic nanostructures. We developed a model that successfully captures the essential physics of the plasmonic hot-electron charge generation and separation in these structures. This model also allowed us to establish that efficient hot carrier extraction is limited to spectral regions where the photons possessing energies higher than the S...

  19. Hot Stars Old-Fashioned or Trendy?

    CERN Document Server

    Pauldrach, A W A


    Spectroscopic analyses with the intention of the interpretation of the UV-spectra of the brightest stars as individuals - supernovae - or as components of star-forming regions - massive O stars - provide a powerful tool with great astrophysical potential for the determination of extragalactic distances and of the chemical composition of star-forming galaxies even at high redshifts. The perspectives of already initiated work with the new generation of tools for quantitative UV-spectroscopy of Hot Stars that have been developed during the last two decades are presented and the status of the continuing effort to construct corresponding models for Hot Star atmospheres is reviewed. Because the physics of the atmospheres of Hot Stars are strongly affected by velocity expansion dominating the spectra at all wavelength ranges, hydrodynamic model atmospheres for O-type stars and explosion models for Supernovae of Type Ia are necessary as basis for the synthesis and analysis of the spectra. It is shown that stellar par...

  20. Distribution map of hot springs in Japan

    Energy Technology Data Exchange (ETDEWEB)

    Sumi, K.


    This map (scale 1:2,000,000) provides the distribution and locations of hot springs in Japan. A hot spring is defined as hot water, mineral water, vapor or other gases (excluding natural gases containing hydrocarbons as the major component) issuing from underground at a temperature of 25/sup 0/C or higher and/or containing substances listed on the map in specific concentrations. Springs are classified according to their chemical composition. Each class of spring is assigned one of five different symbols (per class) according to its temperature. Where appropriate, the geologic age of the spring location is identified. A comprehensive place name index is provided in both Japanese and English transliteration. The map is also isothermically graduated in HFU and references are given for descriptive textual materials that may be used as supplements.

  1. Kepler constraints on planets near hot Jupiters. (United States)

    Steffen, Jason H; Ragozzine, Darin; Fabrycky, Daniel C; Carter, Joshua A; Ford, Eric B; Holman, Matthew J; Rowe, Jason F; Welsh, William F; Borucki, William J; Boss, Alan P; Ciardi, David R; Quinn, Samuel N


    We present the results of a search for planetary companions orbiting near hot Jupiter planet candidates (Jupiter-size candidates with orbital periods near 3 d) identified in the Kepler data through its sixth quarter of science operations. Special emphasis is given to companions between the 21 interior and exterior mean-motion resonances. A photometric transit search excludes companions with sizes ranging from roughly two-thirds to five times the size of the Earth, depending upon the noise properties of the target star. A search for dynamically induced deviations from a constant period (transit timing variations) also shows no significant signals. In contrast, comparison studies of warm Jupiters (with slightly larger orbits) and hot Neptune-size candidates do exhibit signatures of additional companions with these same tests. These differences between hot Jupiters and other planetary systems denote a distinctly different formation or dynamical history.

  2. Four hot DOGs in the microwave (United States)

    Frey, Sándor; Paragi, Zsolt; Gabányi, Krisztina Éva; An, Tao


    Hot dust-obscured galaxies (hot DOGs) are a rare class of hyperluminous infrared galaxies identified with the Wide-field Infrared Survey Explorer (WISE) satellite. The majority of them are at high redshifts (z ˜ 2-3), at the peak epoch of star formation in the Universe. Infrared, optical, radio, and X-ray data suggest that hot DOGs contain heavily obscured, extremely luminous active galactic nuclei (AGN). This class may represent a short phase in the life of the galaxies, signifying the transition from starburst- to AGN-dominated phases. Hot DOGs are typically radio-quiet, but some of them show mJy-level emission in the radio (microwave) band. We observed four hot DOGs using the technique of very long baseline interferometry (VLBI). The 1.7 GHz observations with the European VLBI Network (EVN) revealed weak radio features in all sources. The radio is free from dust obscuration and, at such high redshifts, VLBI is sensitive only to compact structures that are characteristic of AGN activity. In two cases (WISE J0757+5113, WISE J1603+2745), the flux density of the VLBI-detected components is much smaller than the total flux density, suggesting that ˜70-90 per cent of the radio emission, while still dominated by AGN, originates from angular scales larger than that probed by the EVN. The source WISE J1146+4129 appears a candidate compact symmetric object, and WISE J1814+3412 shows a 5.1 kpc double structure, reminiscent of hotspots in a medium-sized symmetric object. Our observations support that AGN residing in hot DOGs may be genuine young radio sources where starburst and AGN activities coexist.

  3. Glucokinase gene mutations: structural and genotype-phenotype analyses in MODY children from South Italy.

    Directory of Open Access Journals (Sweden)

    Nadia Tinto

    Full Text Available BACKGROUND: Maturity onset diabetes of the young type 2 (or GCK MODY is a genetic form of diabetes mellitus provoked by mutations in the glucokinase gene (GCK. METHODOLOGY/PRINCIPAL FINDINGS: We screened the GCK gene by direct sequencing in 30 patients from South Italy with suspected MODY. The mutation-induced structural alterations in the protein were analyzed by molecular modeling. The patients' biochemical, clinical and anamnestic data were obtained. Mutations were detected in 16/30 patients (53%; 9 of the 12 mutations identified were novel (p.Glu70Asp, p.Phe123Leu, p.Asp132Asn, p.His137Asp, p.Gly162Asp, p.Thr168Ala, p.Arg392Ser, p.Glu290X, p.Gln106_Met107delinsLeu and are in regions involved in structural rearrangements required for catalysis. The prevalence of mutation sites was higher in the small domain (7/12: approximately 59% than in the large (4/12: 33% domain or in the connection (1/12: 8% region of the protein. Mild diabetic phenotypes were detected in almost all patients [mean (SD OGTT = 7.8 mMol/L (1.8] and mean triglyceride levels were lower in mutated than in unmutated GCK patients (p = 0.04. CONCLUSIONS: The prevalence of GCK MODY is high in southern Italy, and the GCK small domain is a hot spot for MODY mutations. Both the severity of the GCK mutation and the genetic background seem to play a relevant role in the GCK MODY phenotype. Indeed, a partial genotype-phenotype correlation was identified in related patients (3 pairs of siblings but not in two unrelated children bearing the same mutation. Thus, the molecular approach allows the physician to confirm the diagnosis and to predict severity of the mutation.

  4. Mutational meltdown in laboratory yeast populations

    NARCIS (Netherlands)

    Zeyl, C.; Mizesko, M.; Visser, de J.A.G.M.


    In small or repeatedly bottlenecked populations, mutations are expected to accumulate by genetic drift, causing fitness declines. In mutational meltdown models, such fitness declines further reduce population size, thus accelerating additional mutation accumulation and leading to extinction. Because

  5. Thermal tides on a hot Jupiter

    Directory of Open Access Journals (Sweden)

    Hsieh H.-F.


    Full Text Available Following the linear analysis laid out by Gu & Ogilvie 2009 (hereafter GO09, we investigate the dynamical response of a non-synchronized hot Jupiter to stellar irradiation. Besides the internal and Rossby waves considered by GO09, we study the Kelvin waves excited by the diurnal Fourier harmonic of the prograde stellar irradiation. We also present a 2-dimensional plot of internal waves excited by the semi-diurnal component of the stellar irradiation and postulate that thermal bulges may arise in a hot Jupiter. Whether our postulation is valid and is consistent with the recent results from Arras & Socrates (2009b requires further investigation.

  6. NLTE wind models of hot subdwarf stars

    CERN Document Server

    Krticka, Jiri; 10.1007/s10509-010-0385-z


    We calculate NLTE models of stellar winds of hot compact stars (central stars of planetary nebulae and subdwarf stars). The studied range of subdwarf parameters is selected to cover a large part of these stars. The models predict the wind hydrodynamical structure and provide mass-loss rates for different abundances. Our models show that CNO elements are important drivers of subdwarf winds, especially for low-luminosity stars. We study the effect of X-rays and instabilities on these winds. Due to the line-driven wind instability, a significant part of the wind could be very hot.

  7. Axions as Hot and Cold Dark Matter

    CERN Document Server

    Jeong, Kwang Sik; Takahashi, Fuminobu


    The presence of a hot dark matter component has been hinted at 3 sigma by a combination of the results from different cosmological observations. We examine a possibility that pseudo Nambu-Goldstone bosons account for both hot and cold dark matter components. We show that the QCD axions can do the job for the axion decay constant f_a < O(10^10) GeV, if they are produced by the saxion decay and the domain wall annihilation. We also investigate the cases of thermal QCD axions, pseudo Nambu-Goldstone bosons coupled to the standard model sector through the Higgs portal, and axions produced by modulus decay.

  8. Centrally active nonhormonal hot flash therapies. (United States)

    Loprinzi, Charles L; Stearns, Vered; Barton, Debra


    Given the problems associated with hormonal therapy, and the prominent problem of hot flashes in menopausal women, there is a need for nonhormonal agents to alleviate hot flashes. Several compounds that appear to act on the central nervous system have been investigated. Potential mechanisms for their effects on hot flashes have been described. Bellergal (no longer available on the US market, where it was known as Bellergal-S), a combination preparation sedative that consists of low-dose phenobarbital, ergotamine tartrate, and levorotatory alkaloids of belladonna, is an old agent that was popular approximately 20 years ago; however, there is limited suggestion of efficacy for this agent. Clonidine, an older antihypertensive drug, is another centrally active agent that has been studied. Randomized trials have demonstrated that it clearly works for reducing hot flashes, but the magnitude of efficacy is somewhat limited. Toxicity from this agent limits its utility in the clinic. Methyldopa is another centrally active agent that has been studied but to a more limited degree. It appears to have minimal efficacy and too much toxicity to make it clinically useful. Anecdotal observations from a number of sources suggested that newer antidepressants can alleviate hot flashes. This led to pilot trials of venlafaxine and paroxetine, with results suggesting benefit from both drugs. Subsequently, randomized, placebo-controlled, double-blind clinical trials of venlafaxine, paroxetine, and fluoxetine were conducted. All 3 of these clinical trials demonstrated statistically significant reductions in hot flashes with these newer antidepressants compared with placebo. Pilot trials of citalopram and mirtazapine, 2 other newer antidepressants, have also suggested efficacy. Toxicity evaluations have suggested that these agents are, again, well tolerated by the majority of patients. A recent trial, however, was unable to demonstrate any benefit for fluoxetine or citalopram over a placebo

  9. Hot dry rock venture risks investigation:

    Energy Technology Data Exchange (ETDEWEB)


    This study assesses a promising resource in central Utah as the potential site of a future commerical hot dry rock (HDR) facility for generating electricity. The results indicate that, if the HDR reservoir productivity equals expectations based on preliminary results from research projects to date, a 50 MWe HDR power facility at Roosevelt Hot Springs could generate power at cost competitive with coal-fired plants. However, it is imperative that the assumed productivity be demonstrated before funds are committed for a commercial facility. 72 refs., 39 figs., 38 tabs.

  10. Validation of the Hot Strip Mill Model

    Energy Technology Data Exchange (ETDEWEB)

    Richard Shulkosky; David Rosberg; Jerrud Chapman


    The Hot Strip Mill Model (HSMM) is an off-line, PC based software originally developed by the University of British Columbia (UBC) and the National Institute of Standards and Technology (NIST) under the AISI/DOE Advanced Process Control Program. The HSMM was developed to predict the temperatures, deformations, microstructure evolution and mechanical properties of steel strip or plate rolled in a hot mill. INTEG process group inc. undertook the current task of enhancing and validating the technology. With the support of 5 North American steel producers, INTEG process group tested and validated the model using actual operating data from the steel plants and enhanced the model to improve prediction results.

  11. Hot-carrier effects in MOS devices

    CERN Document Server

    Takeda, Eiji; Miura-Hamada, Akemi


    The exploding number of uses for ultrafast, ultrasmall integrated circuits has increased the importance of hot-carrier effects in manufacturing as well as for other technological applications. They are rapidly movingout of the research lab and into the real world.This book is derived from Dr. Takedas book in Japanese, Hot-Carrier Effects, (published in 1987 by Nikkei Business Publishers). However, the new book is much more than a translation. Takedas original work was a starting point for developing this much more complete and fundamental text on this increasingly important topic. The new work

  12. Novel V600E BRAF mutations in imatinib-naive and imatinib-resistant gastrointestinal stromal tumors. (United States)

    Agaram, Narasimhan P; Wong, Grace C; Guo, Tianhua; Maki, Robert G; Singer, Samuel; Dematteo, Ronald P; Besmer, Peter; Antonescu, Cristina R


    BRAF and NRAS are commonly mutated in cancer and represent the most frequent genetic events in malignant melanoma. More recently, a subset of melanomas was shown to overexpress KIT and harbor KIT mutations. Although most gastrointestinal stromal tumors (GISTs) exhibit activating mutations in either KIT or PDGFRA, about 10% of the cases lack mutations in these genes. It is our hypothesis following the melanoma model that mutations in BRAF or NRAS may play a role in wild-type GIST pathogenesis. Alterations in RAS/MEK/ERK pathway may also be involved in development of imatinib resistance in GIST, particularly in tumors lacking secondary KIT or PDGFRA mutations. Imatinib-naive wild-type GISTs from 61 patients, including 15 children and 28 imatinib-resistant tumors without secondary KIT mutations were analyzed. Screening for hot spots mutations in BRAF (exons 11 and 15) and NRAS (exons 2 and 3) was performed. A BRAF exon 15 V600E was identified in 3 of 61 GIST patients, who shared similar clinical features, being 49- to 55-years-old females and having their tumors located in the small bowel. The tumors were strongly KIT immunoreactive and had a high risk of malignancy. An identical V600E BRAF mutation was also identified in one of 28 imatinib resistant GIST lacking a defined mechanism of drug resistance. In conclusion, we identified a primary BRAF V600E mutations in 7% of adult GIST patients, lacking KIT/PDGFRA mutations. The BRAF-mutated GISTs show predilection for small bowel location and high risk of malignancy. A secondary V600E BRAF mutation could represent an alternative mechanism of imatinib resistance. Kinase inhibitors targeting BRAF may be effective therapeutic options in this molecular GIST subset.

  13. [Frequency, spectrum, and functional significance of TP53 mutations in patients with diffuse large B-cell lymphoma]. (United States)

    Voropaeva, E N; Pospelova, T I; Voevoda, M I; Maksimov, V N


    A comparative analysis of oncogene mutations shows that variations in their frequency, spectrum, and hot-spot locations depends on the type of tumor and the ethnic origin of the population studied. The current version of the IARC TP53 Mutation Database lacks information about the frequency and spectrum of TP53 mutations in patients with DLBCL in Russia. The aim of this study was to assess the frequency and functional significance of TP53 mutations in patients with DLBCL in Novosibirsk. The TP53 coding sequence and the adjacent intron regions were analyzed by direct sequencing in the tumor material from 74 patients with DLBCL. Mutations of the TP53 coding sequence were found in 18 (24.3%) patients. These data are consistent with the frequency of TP53  mutations observed in other studies. The spectrum of nucleotide substitutions found in DLBCL specimens corresponded to that described in the IARC TP53 Mutation Database. According to bioinformatic data and to reported experiments in vitro, most of the mutations detected result in the production of functionally inactive p53. Our results show that DLBCL progression is accompanied by the functional selection for mutations in TP53 exons 5-8.

  14. A specific superoxide dismutase mutation is on the same genetic background in sporadic and familial cases of amyotrophic lateral sclerosis

    Energy Technology Data Exchange (ETDEWEB)

    Hayward, C.; Brock, D.J.H. [Univ. of Edinburgh (United Kingdom); Swingler, R.J. [Dundee Royal Infirmary (United Kingdom)] [and others


    Amyotrophic lateral sclerosis (ALS) is a degenerative disease of motor neurons, causing progressive muscular atrophy, weakness, and death from respiratory failure, often within 2-3 years. Although most cases are sporadic, some 5%-10% are inherited as autosomal dominants with age-dependent penetrance. An ALS locus has been mapped to chromosome 21q, and causative mutations identified in the Cu/Zn superoxide dismutase (SOD1) gene. A majority of SOD1 mutations have been found in cases with a clear family history of ALS. However, we and others have also described SOD1 mutations in patients where the disease appears to be sporadic. This is especially true for the missense mutation in codon 113 of the SOD1 gene, which substitutes threonine for isoleucine (I113T). One explanation for this finding is that this codon is a mutational hot spot with sporadic cases representing new mutations. Another is that the inherited nature of the cases is disguised by the reduced penetrance of this specific mutation. We have now shown that each of six unrelated cases of I113T mutation that we have collected in the Scottish population occurs on the same genetic background. Association analysis of multiple flanking loci on chromosome 21q supports the conclusion of a founder effect, with the original mutational event occurring {ge}10 generations ago. 12 refs., 1 fig., 1 tab.

  15. Identification of CFTR Gene Mutations in Chinese Patients with Congenital Obstructive Azoospermia

    Institute of Scientific and Technical Information of China (English)

    曾国华; 吴开俊; 梅骅; 庄广伦


    Objective To analyze the frequency and hot spot of CFTR gene mutations in Chinese patients with congenital obstructive azoospermia Materials & Methods Mutations in CFTR exon 2,3,4,5,6a,8,10,11,12,13,15A 17b, 19A,20,21and 23 were detected. PCR-single strand conformation poly-morphism (SSCP) and direct sequencing were performed on 32 patients with congenital bilateral absence of the vas deferens (CBAVD), 17 patients with congenital unilateral absence of the vas deferens (CUAVD) and 50 normal Chinese.Results No CFTR gene mutations were detected in 50 normal Chinese. One CBAVD patient exhibited an abnormal band on SSCP for exon 10 of the CFTR gene and subsequent DNA sequencing showed a 3 bp deletion at position 1 653~ 1 655, which caused the deletion of a single amino acid, phenyalanine, in codon 508, i. e. , △F 508. A shift mutation was detected in another CBAVD patient in exon 2, a 1 bp deletion at position 225, 225 delC. One CUAVD patient exhibited an abnormal band on SSCP for exon 17 b of CFTR gene. Subsequent DNA sequencing showed a C-to-A transversion at position 3 295, which led to a predicted change of Leusine (codon 1 055,CUU) to Isoleucine (codon AUU), L1055I.Conclusion CFTR mutation could be detected in Chinese patients with congenital obstructive azoospermia. But no hot spots of mutations are discovered. 225 delC and L1055I are identified as two novel mutations, which are found only in Chinese.

  16. Minisequencing mitochondrial DNA pathogenic mutations

    Directory of Open Access Journals (Sweden)

    Carracedo Ángel


    Full Text Available Abstract Background There are a number of well-known mutations responsible of common mitochondrial DNA (mtDNA diseases. In order to overcome technical problems related to the analysis of complete mtDNA genomes, a variety of different techniques have been proposed that allow the screening of coding region pathogenic mutations. Methods We here propose a minisequencing assay for the analysis of mtDNA mutations. In a single reaction, we interrogate a total of 25 pathogenic mutations distributed all around the whole mtDNA genome in a sample of patients suspected for mtDNA disease. Results We have detected 11 causal homoplasmic mutations in patients suspected for Leber disease, which were further confirmed by standard automatic sequencing. Mutations m.11778G>A and m.14484T>C occur at higher frequency than expected by change in the Galician (northwest Spain patients carrying haplogroup J lineages (Fisher's Exact test, P-value Conclusion We here developed a minisequencing genotyping method for the screening of the most common pathogenic mtDNA mutations which is simple, fast, and low-cost. The technique is robust and reproducible and can easily be implemented in standard clinical laboratories.

  17. HNPCC: Six new pathogenic mutations

    Directory of Open Access Journals (Sweden)

    Epplen Joerg T


    Full Text Available Abstract Background Hereditary non-polyposis colorectal cancer (HNPCC is an autosomal dominant disease with a high risk for colorectal and endometrial cancer caused by germline mutations in DNA mismatch-repair genes (MMR. HNPCC accounts for approximately 2 to 5% of all colorectal cancers. Here we present 6 novel mutations in the DNA mismatch-repair genes MLH1, MSH2 and MSH6. Methods Patients with clinical diagnosis of HNPCC were counselled. Tumor specimen were analysed for microsatellite instability and immunohistochemistry for MLH1, MSH2 and MSH6 protein was performed. If one of these proteins was not detectable in the tumor mutation analysis of the corresponding gene was carried out. Results We identified 6 frameshift mutations (2 in MLH1, 3 in MSH2, 1 in MSH6 resulting in a premature stop: two mutations in MLH1 (c.2198_2199insAACA [p.N733fsX745], c.2076_2077delTG [p.G693fsX702], three mutations in MSH2 (c.810_811delGT [p.C271fsX282], c.763_766delAGTGinsTT [p.F255fsX282], c.873_876delGACT [p.L292fsX298] and one mutation in MSH6 (c.1421_1422dupTG [p.C475fsX480]. All six tumors tested for microsatellite instability showed high levels of microsatellite instability (MSI-H. Conclusions HNPCC in families with MSH6 germline mutations may show an age of onset that is comparable to this of patients with MLH1 and MSH2 mutations.

  18. Radiation mutation breeding

    Energy Technology Data Exchange (ETDEWEB)

    Song, Hi Sup; Kim, Jae Sung; Kim, Jin Kyu; Shin, In Chul; Lim, Young Taek


    In order to develop an advanced technical knowledge for the selection of better mutants, some of the crops were irradiated and the mutation rate, the survival rate and the method for selction of a mutant were studied. Furthermore, this study aimed to obtain basic data applicable to the development of genetic resources by evaluation and analysis the specific character for selection of the superior mutant and its plant breeding. 1. selection of the mutant with a superior resistance against environment in the principal crops 1) New varieties of mutant rices such as Wonpyeongbyeo, Wongwangbyeo, Winmibyeo, and heogseon chalbeyeo (sticky forma) were registered in the national variety list and made an application to crop variety protection right. They are under review now. 2) We also keep on studying on the number of a grain of 8 lines of excellent mutant rice for the purpose of improvement of breeding . 3) We selected 3 lines which have a resistance to pod and stem blight in large soybean, 31 lines with small grain size and higher yield, 112 lines of soybean of cooking, 7 lines of low lipoxygenase content, and 12 lines with decreased phytic acid content by 20 % compared to the previous level. 2. Selection of advanced Mugunwha (Rose of Sharon) mutant 1) Bagseul, a new variety of mutant, was developed and 30 plantlets of it are being proliferated. 2) Fifty-three lines of a mutant having a various morphologies were selected.

  19. Influence of Hot Deformation and Subsequent Austempering on the Mechanical Properties of Hot Rolled Multiphase Steel

    Institute of Scientific and Technical Information of China (English)

    Zhuang LI; Di WU


    Influence of hot deformation and subsequent austempering on the mechanical properties of hot rolled multiphase steel was investigated. Thermo-mechanical control processing (TMCP) was conducted by using a laboratory hot rolling mill, where three different kinds of finishing rolling reduction, and austemperings with various isothermal holding duration were applied. The results have shown that a multiphase microstructure consisting of polygonal ferrite, granular bainite and larger amount of stabilized retained austenite can be obtained by controlled rolling processes. Mechanical properties increase with increasing the amount of deformation because of the stabilization of retained austenite. Ultimate tensile strength (σb), total elongation (δ)36% and 28476 MPa%, respectively) at optimal processes.

  20. Estimation of Hot Electron Relaxation Time in GaN Using Hot Electron Transistors (United States)

    Dasgupta, Sansaptak; Lu, Jing; Nidhi; Raman, Ajay; Hurni, Christophe; Gupta, Geetak; Speck, James S.; Mishra, Umesh K.


    In this paper, we report for the first time an estimation of hot electron relaxation time in GaN using electrical measurements. Hot electron transistors (HETs) with GaN as the base layer and different base-emitter barrier-height configurations and base thicknesses were fabricated. Common-base measurements were performed to extract the differential transfer ratio, and an exponential decay of the transfer ratio with increasing base thickness was observed. A hot electron mean free path was extracted from the corresponding exponential fitting and a relaxation time was computed, which, for low energy injection, matched well with theoretically predicted relaxation times based on longitudinal optical (LO) phonon scattering.

  1. How mixing during hot water draw-offs influence the thermal performance of small solar domestic hot water systems

    DEFF Research Database (Denmark)

    Furbo, Simon; Shah, Louise Jivan


    CFD calculations on the mixing during hot water draw-offs in vertical hot water tanks with different diameters have been carried out. The calculations, which were carried out with the same cold water inlet design, showed that the extent of mixing is strongly influenced by the tank diameter. The e...... consideration of the required hot water comfort.......CFD calculations on the mixing during hot water draw-offs in vertical hot water tanks with different diameters have been carried out. The calculations, which were carried out with the same cold water inlet design, showed that the extent of mixing is strongly influenced by the tank diameter....... The extent of mixing is increasing for increasing tank diameter. Further, calculations of the yearly thermal performance of small solar domestic hot water systems with hot water tanks with different mixing rates during hot water draw-offs were carried out. Both solar domestic hot water systems with mantle...

  2. Mutations induced by ultraviolet light

    Energy Technology Data Exchange (ETDEWEB)

    Pfeifer, Gerd P. [Department of Biology, Beckman Research Institute, City of Hope, Duarte, CA 91010 (United States)]. E-mail:; You, Young-Hyun [Department of Biology, Beckman Research Institute, City of Hope, Duarte, CA 91010 (United States); Besaratinia, Ahmad [Department of Biology, Beckman Research Institute, City of Hope, Duarte, CA 91010 (United States)


    The different ultraviolet (UV) wavelength components, UVA (320-400 nm), UVB (280-320 nm), and UVC (200-280 nm), have distinct mutagenic properties. A hallmark of UVC and UVB mutagenesis is the high frequency of transition mutations at dipyrimidine sequences containing cytosine. In human skin cancers, about 35% of all mutations in the p53 gene are transitions at dipyrimidines within the sequence 5'-TCG and 5'-CCG, and these are localized at several mutational hotspots. Since 5'-CG sequences are methylated along the p53 coding sequence in human cells, these mutations may be derived from sunlight-induced pyrimidine dimers forming at sequences that contain 5-methylcytosine. Cyclobutane pyrimidine dimers (CPDs) form preferentially at dipyrimidines containing 5-methylcytosine when cells are irradiated with UVB or sunlight. In order to define the contribution of 5-methylcytosine to sunlight-induced mutations, the lacI and cII transgenes in mouse fibroblasts were used as mutational targets. After 254 nm UVC irradiation, only 6-9% of the base substitutions were at dipyrimidines containing 5-methylcytosine. However, 24-32% of the solar light-induced mutations were at dipyrimidines that contain 5-methylcytosine and most of these mutations were transitions. Thus, CPDs forming preferentially at dipyrimidines with 5-methylcytosine are responsible for a considerable fraction of the mutations induced by sunlight in mammalian cells. Using mouse cell lines harboring photoproduct-specific photolyases and mutational reporter genes, we showed that CPDs (rather than 6-4 photoproducts or other lesions) are responsible for the great majority of UVB-induced mutations. An important component of UVB mutagenesis is the deamination of cytosine and 5-methylcytosine within CPDs. The mutational specificity of long-wave UVA (340-400 nm) is distinct from that of the shorter wavelength UV and is characterized mainly by G to T transversions presumably arising through mechanisms

  3. Towards Chemical Constraints on Hot Jupiter Migration

    CERN Document Server

    Madhusudhan, Nikku; Kennedy, Grant M


    The origin of hot Jupiters -- gas giant exoplanets orbiting very close to their host stars -- is a long-standing puzzle. Planet formation theories suggest that such planets are unlikely to have formed in-situ but instead may have formed at large orbital separations beyond the snow line and migrated inward to their present orbits. Two competing hypotheses suggest that the planets migrated either through interaction with the protoplanetary disk during their formation, or by disk-free mechanisms such as gravitational interactions with a third body. Observations of eccentricities and spin-orbit misalignments of hot Jupiter systems have been unable to differentiate between the two hypotheses. In the present work, we suggest that chemical depletions in hot Jupiter atmospheres might be able to constrain their migration mechanisms. We find that sub-solar carbon and oxygen abundances in Jovian-mass hot Jupiters around Sun-like stars are hard to explain by disk migration. Instead, such abundances are more readily expla...

  4. Moving hot cell for LMFBR type reactor

    Energy Technology Data Exchange (ETDEWEB)

    Kanbe, Mitsuru


    A moving hot cell for an LMFBR type reactor is made movable on a reactor operation floor between a position just above the reactor container and a position retreated therefrom. Further, it comprises an overhung portion which can incorporate a spent fuel just thereunder, and a crane for moving a fuel assembly between a spent fuel cask and a reactor container. Further, an opening/closing means having a shielding structure is disposed to the bottom portion and the overhung portion thereof, to provide a sealing structure, in which only the receiving port for the spent fuel cask faces to the inner side, and the cask itself is disposed at the outside. Upon exchange of fuels, the movable hot cell is placed just above the reactor to take out the spent fuels, so that a region contaminated with primary sodium is limited within the hot cell. On the other hand, upon maintenance and repair for equipments, the hot cell is moved, thereby enabling to provide a not contaminated reactor operation floor. (N.H.).

  5. Esophageal thermal injury by hot adlay tea. (United States)

    Go, Hoon; Yang, Hyeon Woong; Jung, Sung Hee; Park, Young A; Lee, Jung Yun; Kim, Sae Hee; Lim, Sin Hyung


    Reversible thermal injury to the esophagus as the result of drinking hot liquids has been reported to generate alternating white and red linear mucosal bands, somewhat reminiscent of a candy cane. This phenomenon is associated with chest pain, dysphagia, odynophagia, and epigastric pain. Here, we report a case of thermal injury to the esophageal and oral cavity due to the drinking of hot tea, including odynophagia and dysphagia. A 69-year-old man was referred due to a difficulty in swallowing which had begun a week prior to referral. The patient, at the time of admission, was unable to swallow even liquids. He had recently suffered from hiccups, and had consumed five cups of hot adlay tea one week prior to admission, as a folk remedy for the hiccups. Upon physical examination, the patient's oral cavity evidenced mucosal erosion, hyperemia, and mucosa covered by a whitish pseudomembrane. Nonspecific findings were detected on the laboratory and radiological exams. Upper endoscopy revealed diffuse hyperemia, and erosions with thick and whitish pseudomembraneous mucosa on the entire esophagus. The stomach and duodenum appeared normal. We diagnosed the patient with thermal esophageal injury inflicted by the hot tea. He was treated with pantoprazole, 40 mg/day, for 14 days, and evidenced significant clinical and endoscopic improvement.

  6. Six Hot Topics in Planetary Astronomy


    Jewitt, David


    Six hot topics in modern planetary astronomy are described: 1) lightcurves and densities of small bodies 2) colors of Kuiper belt objects and the distribution of the ultrared matter 3) spectroscopy and the crystallinity of ice in the outer Solar system 4) irregular satellites of the giant planets 5) the Main Belt Comets and 6) comets and meteor stream parents.

  7. Comment on Breakup Densities of Hot Nuclei

    CERN Document Server

    Viola, V E; Natowitz, J B; Yennello, S J


    In [1,2]the observed decrease in spectral peak energies of IMFs emitted from hot nuclei was interpreted in terms of a breakup density that decreased with increasing energy. Subsequently, Raduta et al. [3] performed MMM simulations that showed decreasing spectral peaks could be obtained at constant density. In this letter we examine this apparent inconsistency.

  8. Transport properties of hot gluonic matter

    CERN Document Server

    Bluhm, Marcus


    We discuss the temperature dependence of the scaled jet quenching parameter of hot gluonic matter within a quasiparticle approach. A pronounced maximum in the vicinity of the transition temperature is observed, where the ratio of the scaled jet quenching parameter and the inverse specific shear viscosity increases above typical values for weakly coupled systems.

  9. Extracting hot carriers from photoexcited semiconductor nanocrystals

    Energy Technology Data Exchange (ETDEWEB)

    Zhu, Xiaoyang


    This research program addresses a fundamental question related to the use of nanomaterials in solar energy -- namely, whether semiconductor nanocrystals (NCs) can help surpass the efficiency limits, the so-called “Shockley-Queisser” limit, in conventional solar cells. In these cells, absorption of photons with energies above the semiconductor bandgap generates “hot” charge carriers that quickly “cool” to the band edges before they can be utilized to do work; this sets the solar cell efficiency at a limit of ~31%. If instead, all of the energy of the hot carriers could be captured, solar-to-electric power conversion efficiencies could be increased, theoretically, to as high as 66%. A potential route to capture this energy is to utilize semiconductor nanocrystals. In these materials, the quasi-continuous conduction and valence bands of the bulk semiconductor become discretized due to confinement of the charge carriers. Consequently, the energy spacing between the electronic levels can be much larger than the highest phonon frequency of the lattice, creating a “phonon bottleneck” wherein hot-carrier relaxation is possible via slower multiphonon emission. For example, hot-electron lifetimes as long as ~1 ns have been observed in NCs grown by molecular beam epitaxy. In colloidal NCs, long lifetimes have been demonstrated through careful design of the nanocrystal interfaces. Due to their ability to slow electronic relaxation, semiconductor NCs can in principle enable extraction of hot carriers before they cool to the band edges, leading to more efficient solar cells.

  10. Hot Carrier Extraction from Multilayer Graphene. (United States)

    Urcuyo, Roberto; Duong, Dinh Loc; Sailer, Patrick; Burghard, Marko; Kern, Klaus


    Hot carriers in semiconductor or metal nanostructures are relevant, for instance, to enhance the activity of oxide-supported metal catalysts or to achieve efficient photodetection using ultrathin semiconductor layers. Moreover, rapid collection of photoexcited hot carriers can improve the efficiency of solar cells, with a theoretical maximum of 85%. Because of the long lifetime of secondary excited electrons, graphene is an especially promising two-dimensional material to harness hot carriers for solar-to-electricity conversion. However, the photoresponse of thus far realized graphene photoelectric devices is mainly governed by thermal effects, which yield only a very small photovoltage. Here, we report a Gr-TiOx-Ti heterostructure wherein the photovoltaic effect is predominant. By doping the graphene, the open circuit voltage reaches values up to 0.30 V, 2 orders of magnitude larger than for devices relying upon the thermoelectric effect. The photocurrent turned out to be limited by trap states in the few-nanometer-thick TiOx layer. Our findings represent a first valuable step toward the integration of graphene into third-generation solar cells based upon hot carrier extraction.

  11. Hot-dry-rock geothermal resource 1980

    Energy Technology Data Exchange (ETDEWEB)

    Heiken, G.; Goff, F.; Cremer, G. (ed.)


    The work performed on hot dry rock (HDR) geothermal resource evaluation, site characterization, and geophysical exploration techniques is summarized. The work was done by region (Far West, Pacific Northwest, Southwest, Rocky Mountain States, Midcontinent, and Eastern) and limited to the conterminous US.

  12. Geothermal energy and hot springs in Ethiopia

    Energy Technology Data Exchange (ETDEWEB)

    Koga, T. (Hot Springs Therapeutics Research Institute, Kyushu, Univ., Japan)


    The hot springs in Ethiopia are concentrated in two areas: the North Afar depression and adjacent Red Sea shore, and a geothermal field 100 km from northeast to southwest in the central part of Ethiopia. The latter extends not only to the Great Rift Valley but also to the Aden Gulf. In the lake district in the central Great Rift Valley, there are a number of hot springs on the lake shore. These are along NE-SW fault lines, and the water is a sodium bicarbonate-type rich in HCO/sub 3/ and Na but low in C1 and Ca. In Dallol in the North Afar depression, CO/sub 2/-containing hot springs with high temperatures (110/sup 0/C) and a specific gravity of 1.4, were observed. In the South Afar depression, located in the northeastern part of the Rift Valley, there are many active volcanoes and hot springs between the lake district and the Danakil depression. The spring water is a sodium bicarbonate saline type. Nine graphs and maps are included.

  13. Advances in hot gas filtration technology

    Energy Technology Data Exchange (ETDEWEB)

    Weber, C.

    The past decade has seen the introduction of new filter media specifically designed for 'hot-gas' filtration. These media are available as woven or knitted fabrics and as non-wovens, i.e. needled felts. Needlefelted fabrics have proven so highly successful in the dedusting of hot gases that they are widely used nowadays in this new and necessary technology. Hot-gas filtration offers advantages in, for example, the saving or recycling of energy, the elimination of the cooling process, and the short-circuiting of process steps. This paper gives a survey of the types of textile fibres available for hot-gas filtration from the more recently developed organic fibres to refractory fibres. It describes, compares and contrasts their salient properties and lists the uses to which they may be put. It concentrates on such fibres which are generally referred to as 'high performance materials', since they are expected to provide satisfactory performance under extreme conditions of temperature, chemical environment and mechanical stress. It touches on filtration theory governing the collection mechanism. 9 refs., 7 figs., 3 tabs.

  14. Hot-dry-rock geothermal resource 1980

    Energy Technology Data Exchange (ETDEWEB)

    Heiken, G.; Goff, F.; Cremer, G. (ed.)


    The work performed on hot dry rock (HDR) geothermal resource evaluation, site characterization, and geophysical exploration techniques is summarized. The work was done by region (Far West, Pacific Northwest, Southwest, Rocky Mountain States, Midcontinent, and Eastern) and limited to the conterminous US.

  15. Heat stress protection in abnormally hot environments.

    CSIR Research Space (South Africa)

    Schutte, PC


    Full Text Available The present report presents the findings of SIMRAC project GAP 045 entitled ‘Heat stress protection in abnormally hot environments’. It is intended as a reference to develop guidelines which, in turn would assist mine management in establishing safe...

  16. Hot Jupiters and Super-Earths

    CERN Document Server

    Mustill, Alexander James; Johansen, Anders


    We explore the role of dynamics in shaping planetary system multiplicities, focussing on two particular problems. (1) We propose that the lack of close-in super-Earths in hot Jupiter systems is a signature of the migration history of the hot Jupiters and helps to discriminate between different mechanisms of migration. We present N-body simulations of dynamical migration scenarios where proto-hot Jupiters are excited to high eccentricities prior to tidal circularisation and orbital decay. We show that in this scenario, the eccentric giant planet typically destroys planets in the inner system, in agreement with the observed lack of close super-Earth companions to hot Jupiters. (2) We explore the role of the dynamics of outer systems in affecting the multiplicities of close-in systems such as those discovered by Kepler. We consider specifically the effects of planet--planet scattering and Kozai perturbations on an exterior giant planet on the architecture of the inner system, and evaluate the ability of such sce...

  17. High Temperature Chemistry at NASA: Hot Topics (United States)

    Jacobson, Nathan S.


    High Temperature issues in aircraft engines Hot section: Ni and Co based Superalloys Oxidation and Corrosion (Durability) at high temperatures. Thermal protection system (TPS) and RCC (Reinforced Carbon-Carbon) on the Space Shuttle Orbiter. High temperatures in other worlds: Planets close to their stars.

  18. Hydrodynamic Overview at Hot Quarks 2016

    CERN Document Server

    Noronha-Hostler, Jacquelyn


    This presents an overview of relativistic hydrodynamic modeling in heavy-ion collisions prepared for Hot Quarks 2016, at South Padre Island, TX, USA. The influence of the initial state and viscosity on various experimental observables are discussed. Specific problems that arise in the hydrodynamical modeling at the Beam Energy Scan are briefly discussed.

  19. Solar-powered hot-air system (United States)


    Solar-powered air heater supplies part or all of space heating requirements of residential or commercial buildings and is interfaced with air to water heat exchanger to heat domestic hot water. System has potential application in drying agricultural products such as cotton, lumber, corn, grains, and peanuts.

  20. HotMobile 2008: Postconference Report

    NARCIS (Netherlands)

    Lindqvist, J.; Pawar, P.; Stuntebeck, E.; Hong, J.


    HotMobile 2008 presented a two-day program on mobile computing systems and applications. The authors focuses on the sessions on sensors, modularity, wireless, security, systems, and screens. The mobile device is the most amazing invention in history and that it has had the largest impact on human ki

  1. Mutational profiling reveals PIK3CA mutations in gallbladder carcinoma

    Directory of Open Access Journals (Sweden)

    Bardeesy Nabeel


    Full Text Available Abstract Background The genetics of advanced biliary tract cancers (BTC, which encompass intra- and extra-hepatic cholangiocarcinomas as well as gallbladder carcinomas, are heterogeneous and remain to be fully defined. Methods To better characterize mutations in established known oncogenes and tumor suppressor genes we tested a mass spectrometric based platform to interrogate common cancer associated mutations across a panel of 77 formalin fixed paraffin embedded archived BTC cases. Results Mutations among three genes, KRAS, NRAS and PIK3CA were confirmed in this cohort. Activating mutations in PIK3CA were identified exclusively in GBC (4/32, 12.5%. KRAS mutations were identified in 3 (13% intra-hepatic cholangiocarcinomas and 1 (33% perihillar cholangiocarcinoma but were not identified in gallbladder carcinomas and extra-hepatic cholangiocarcinoma. Conclusions The presence of activating mutations in PIK3CA specifically in GBC has clinical implications in both the diagnosis of this cancer type, as well as the potential utility of targeted therapies such as PI3 kinase inhibitors.

  2. Microbial hotspots and hot moments in soil (United States)

    Kuzyakov, Yakov; Blagodatskaya, Evgenia


    Soils are the most heterogeneous parts of the biosphere, with an extremely high differentiation of properties and processes within nano- to macroscales. The spatial and temporal heterogeneity of input of labile organics by plants creates microbial hotspots over short periods of time - the hot moments. We define microbial hotspots as small soil volumes with much faster process rates and much more intensive interactions compared to the average soil conditions. Such hotspots are found in the rhizosphere, detritusphere, biopores (including drilosphere) and on aggregate surfaces, but hotspots are frequently of mixed origin. Hot moments are short-term events or sequences of events inducing accelerated process rates as compared to the averaged rates. Thus, hotspots and hot moments are defined by dynamic characteristics, i.e. by process rates. For this hotspot concept we extensively reviewed and examined the localization and size of hotspots, spatial distribution and visualization approaches, transport of labile C to and from hotspots, lifetime and process intensities, with a special focus on process rates and microbial activities. The fraction of active microorganisms in hotspots is 2-20 times higher than in the bulk soil, and their specific activities (i.e. respiration, microbial growth, mineralization potential, enzyme activities, RNA/DNA ratio) may also be much higher. The duration of hot moments in the rhizosphere is limited and is controlled by the length of the input of labile organics. It can last a few hours up to a few days. In the detritusphere, however, the duration of hot moments is regulated by the output - by decomposition rates of litter - and lasts for weeks and months. Hot moments induce succession in microbial communities and intense intra- and interspecific competition affecting C use efficiency, microbial growth and turnover. The faster turnover and lower C use efficiency in hotspots counterbalances the high C inputs, leading to the absence of strong

  3. Hot-Jupiter Breakfasts Realign Stars (United States)

    Kohler, Susanna


    Two researchers at the University of Chicago have recently developed a new theory to explain an apparent dichotomy in the orbits of planets around cool vs. hot stars. Their model proposes that the spins of cool stars are affected when they ingest hot Jupiters (HJs) early in their stellar lifetimes. A Puzzling Dichotomy: In exoplanet studies, there is a puzzling difference observed between planet orbits around cool and hot (those with Teff ≥ 6250 K) stars: the orbital planes of planets around cool stars are primarily aligned with the host star's spin, whereas the orbital planes of planets around hot stars seem to be randomly distributed. Previous attempts to explain this dichotomy have focused on tidal interactions between the host star and the planets observed in the system. Now Titos Matsakos and Arieh Königl have taken these models a step further — by including in their calculations not only the effects of observed planets, but also those of HJs that may have been swallowed by the star long before we observed the systems. Modeling Meals: Plots of the distribution of the obliquity λ for hot Jupiters around cool hosts (upper plot) and hot hosts (lower plot). The dashed line shows the initial distribution, the bins show the model prediction for the final distribution after the systems evolve, and the black dots show the current observational data. [Matsakos & Königl, 2015]" class="size-thumbnail wp-image-223" height="386" src="" width="260" /> Plots of the distribution of the obliquity λ for hot Jupiters around cool hosts (upper plot) and hot hosts (lower plot). The dashed line shows the initial distribution, the bins show the model prediction for the final distribution after the systems evolve, and the black dots show the current observational data. [Matsakos & Königl, 2015] The authors' model assumes that as HJs are formed and migrate inward through the protoplanetary disk, they stall out near

  4. [Analysis of GALNS gene mutation in thirty-eight Chinese patients with mucopolysaccharidosis type IVA]. (United States)

    Ye, Jun; Lei, Hong-lin; Zhang, Hui-wen; Qiu, Wen-juan; Han, Lian-shu; Wang, Yu; Li, Xiao-yan; Gu, Xue-fan


    Mucopolysaccharidosis (MPS) type IVA (MPS IVA) is an autosomal recessive lysosomal storage disease caused by deficiency of N-acetylgalactosamine-6-sulfate sulfatase (GALNS) needed to degrade glycosaminoglycanes (GAGs), accumulation of GAGs in the tissue resulting in disorder of function. So far, the small number of articles about clinical study of Chinese MPS IVA were published and only one paper about gene mutation analysis was published. This study aimed to investigate the mutation spectrum and characteristic of GALNS gene in Chinese patients with MPS IVA who were diagnosed in our hospital. Thirty-eight patients from 36 families (male 17, female 21) were diagnosed as MPS IVA by GALNS activity determination [(0.85 ± 1.33) nmol/(17 h·mg)] and clinical symptoms during 2006-2012. The average age of diagnosis was (5.7 ± 3.6) years. Mutation analysis of GALNS gene performed performed by PCR-direct DNA sequencing for 38 patients. PCR-restriction fragment length polymorphism analysis was used for validating novel mutation, and also to assess amino acid conservation for novel missense variants in five different species. PolyPhen-2 tool was used to predict the possible impact of missense mutations on the structure and function of the human GALNS protein, etc. Analysis of GALNS activity and gene mutation in amniotic fluid were performed to provide the prenatal diagnosis for some families with MPS type IVA. (1) Thirty-eight kinds of mutation in GALNS gene were identified in 38 patients of them, 71% were missense mutations. p. M318R was a hot-spot mutation (21%) tested. Five kinds of mutation i.e., p. P163H, p.G168L, p. A324E, p. L366P and p. F452L were only found in Chinese patients with MPS IVA. Eighteen kinds of novel mutation were detected including p. E315K, p.G304D, p.R251Q, p.Y240C, p.G161E, p.N32D, p.L390P, p. D60E, p. P420S, W403C/T404S, p.L454P, for p.W405X, p. M1I, c.409_ c.420del12, c.1176_1178del3, c.1046delG, c.1188delG and IVS9-2A>C. (2) The polymorphism of

  5. Geothermal Exploration in Hot Springs, Montana

    Energy Technology Data Exchange (ETDEWEB)

    Toby McIntosh, Jackola Engineering


    The project involves drilling deeper in the Camp Aqua well dri lled in June 1982 as part of an effort to develop an ethanol plant. The purpose of the current drill ing effort is to determine if water at or above 165°F exists for the use in low temperature resource power generation. Previous geothermal resource study efforts in and around Hot Springs , MT and the Camp Aqua area (NE of Hot Springs) have been conducted through the years. A confined gravel aquifer exists in deep alluvium overlain by approximately 250 of si lt and c lay deposits from Glacial Lake Missoula. This gravel aquifer overlies a deeper bedrock aquifer. In the Camp Aqua area several wel l s exist in the gravel aquifer which receives hot water f rom bedrock fractures beneath the area. Prior to this exploration, one known well in the Camp Aqua area penetrated into the bedrock without success in intersecting fractures transporting hot geothermal water. The exploration associated with this project adds to the physical knowledge database of the Camp Aqua area. The dri l l ing effort provides additional subsurface information that can be used to gain a better understanding of the bedrock formation that i s leaking hot geothermal water into an otherwise cold water aquifer. The exi s t ing well used for the explorat ion is located within the center of the hottest water within the gravel aquifer. This lent i t sel f as a logical and economical location to continue the exploration within the existing well. Faced with budget constraints due to unanticipated costs, changing dril l ing techniques stretched the limited project resources to maximize the overa l l well depth which f e l l short of original project goals. The project goal of finding 165°F or hotter water was not achieved; however the project provides additional information and understanding of the Camp Aqua area that could prove valuable in future exploration efforts

  6. Markov models for accumulating mutations

    CERN Document Server

    Beerenwinkel, Niko


    We introduce and analyze a waiting time model for the accumulation of genetic changes. The continuous time conjunctive Bayesian network is defined by a partially ordered set of mutations and by the rate of fixation of each mutation. The partial order encodes constraints on the order in which mutations can fixate in the population, shedding light on the mutational pathways underlying the evolutionary process. We study a censored version of the model and derive equations for an EM algorithm to perform maximum likelihood estimation of the model parameters. We also show how to select the maximum likelihood poset. The model is applied to genetic data from different cancers and from drug resistant HIV samples, indicating implications for diagnosis and treatment.

  7. Dynamical Mutation of Dark Energy

    CERN Document Server

    Abramo, L R; Liberato, L; Rosenfeld, R


    We discuss the intriguing possibility that dark energy may change its equation of state in situations where large dark energy fluctuations are present. We show indications of this dynamical mutation in some generic models of dark energy.

  8. Hot-Carrier Seebeck Effect: Diffusion and Remote Detection of Hot Carriers in Graphene (United States)

    Sierra, Juan F.; Neumann, Ingmar; Costache, Marius V.; Valenzuela, Sergio O.


    We investigate hot carrier propagation across graphene using an electrical nonlocal injection/detection method. The device consists of a monolayer graphene flake contacted by multiple metal leads. Using two remote leads for electrical heating, we generate a carrier temperature gradient that results in a measurable thermoelectric voltage VNL across the remaining (detector) leads. Due to the nonlocal character of the measurement, VNL is exclusively due to the Seebeck effect. Remarkably, a departure from the ordinary relationship between Joule power P and VNL, VNL ~ P, becomes readily apparent at low temperatures, representing a fingerprint of hot-carrier dominated thermoelectricity. By studying VNL as a function of bias, we directly determine the carrier temperature and the characteristic cooling length for hot-carrier propagation, which are key parameters for a variety of new applications that rely on hot-carrier transport.

  9. WESF hot cells waste minimization criteria hot cells window seals evaluation

    Energy Technology Data Exchange (ETDEWEB)

    Walterskirchen, K.M.


    WESF will decouple from B Plant in the near future. WESF is attempting to minimize the contaminated solid waste in their hot cells and utilize B Plant to receive the waste before decoupling. WESF wishes to determine the minimum amount of contaminated waste that must be removed in order to allow minimum maintenance of the hot cells when they are placed in ''laid-up'' configuration. The remaining waste should not cause unacceptable window seal deterioration for the remaining life of the hot cells. This report investigates and analyzes the seal conditions and hot cell history and concludes that WESF should remove existing point sources, replace cerium window seals in F-Cell and refurbish all leaded windows (except for A-Cell). Work should be accomplished as soon as possible and at least within the next three years.

  10. New Insights in Preservation of Meteorites in Hot Deserts: The Oldest Hot Desert Meteorite Collection. (United States)

    Hutzler, A.; Rochette, P.; Bourlès, D.; Gattacceca, J.; Merchel, S.; Jull, A. J. T.; Valenzuela, M.


    Terrestrial ages of a subset of a chilean meteorite collection have been determined with cosmogenic nuclides. We show here that provided the environnement is favorable enough, hot desert meteorites can survive over a million year.

  11. Laboratory spectra of hot molecules: Data needs for hot super-Earth exoplanets (United States)

    Tennyson, Jonathan; Yurchenko, Sergei N.


    The majority of stars are now thought to support exoplanets. Many of those exoplanets discovered thus far are categorized as rocky objects with an atmosphere. Most of these objects are however hot due to their short orbital period. Models suggest that water is the dominant species in their atmospheres. The hot temperatures are expected to turn these atmospheres into a (high pressure) steam bath containing remains of melted rock. The spectroscopy of these hot rocky objects will be very different from that of cooler objects or hot gas giants. Molecules suggested to be important for the spectroscopy of these objects are reviewed together with the current status of the corresponding spectroscopic data. Perspectives of building a comprehensive database of linelist/cross sections applicable for atmospheric models of rocky super-Earths as part of the ExoMol project are discussed. The quantum-mechanical approaches used in linelist productions and their challenges are summarized.

  12. Hot-Carrier Seebeck Effect: Diffusion and Remote Detection of Hot Carriers in Graphene. (United States)

    Sierra, Juan F; Neumann, Ingmar; Costache, Marius V; Valenzuela, Sergio O


    We investigate hot carrier propagation across graphene using an electrical nonlocal injection/detection method. The device consists of a monolayer graphene flake contacted by multiple metal leads. Using two remote leads for electrical heating, we generate a carrier temperature gradient that results in a measurable thermoelectric voltage V(NL) across the remaining (detector) leads. Due to the nonlocal character of the measurement, V(NL) is exclusively due to the Seebeck effect. Remarkably, a departure from the ordinary relationship between Joule power P and V(NL), V(NL) ∼ P, becomes readily apparent at low temperatures, representing a fingerprint of hot-carrier dominated thermoelectricity. By studying V(NL) as a function of bias, we directly determine the carrier temperature and the characteristic cooling length for hot-carrier propagation, which are key parameters for a variety of new applications that rely on hot-carrier transport.

  13. Effect of Controlled Cooling After Hot Rolling on Mechanical Properties of Hot Rolled TRIP Steel

    Institute of Scientific and Technical Information of China (English)

    WU Di; LI Zhuang; L(U) Hui-sheng


    A three-step cooling pattern on the runout table (ROT) was conducted for the hot rolled TRIP steel. Microstructural evolution during thermomechanical controlled processing (TMCP) was investigated. Proeessing condition of controlled cooling on a ROT in the laboratory rolling mill was discussed. The results indicated that the microstructure containing polygonal ferrite, granular bainite and a significant amount of the stable retained austenite can be obtained through three-step cooling on the ROT after hot rolling. TMCP led to ferrite grain refinement. Controlled cooling after hot rolling resulted in the stability of the remaining austenite and a satisfactory TRIP effect. Excellent mechanical properties were obtained through TMCP for the hot rolled TRIP steel.

  14. PPARγ mutations, lipodystrophy and diabetes. (United States)

    Astapova, Olga; Leff, Todd


    The focus of this review is the lipodystrophy syndrome caused by mutation in the PPARγ nuclear receptor - partial familial lipodystrophy FPLD3. To provide a broader context for how these mutations act to generate the clinical features of partial lipodystrophy we will review the basic biology of PPARγ and also survey the set PPARγ genetic variants that do not cause lipodystrophy, but are nonetheless associated with clinically related syndromes, specifically type 2 diabetes.

  15. Gene mutations in hepatocellular adenomas

    DEFF Research Database (Denmark)

    Raft, Marie B; Jørgensen, Ernö N; Vainer, Ben


    is associated with bi-allelic mutations in the TCF1 gene and morphologically has marked steatosis. β-catenin activating HCA has increased activity of the Wnt/β-catenin pathway and is associated with possible malignant transformation. Inflammatory HCA is characterized by an oncogene-induced inflammation due....... This review offers an overview of the reported gene mutations associated with hepatocellular adenomas together with a discussion of the diagnostic and prognostic value....

  16. Mutations causative of familial hypercholesterolaemia

    DEFF Research Database (Denmark)

    Benn, Marianne; Watts, Gerald F; Tybjærg-Hansen, Anne


    AIMS: Ideally, familial hypercholesterolaemia (FH) is diagnosed by testing for mutations that decrease the catabolism of low-density lipoprotein (LDL) cholesterol; however, genetic testing is not universally available. The aim of the present study was to assess the frequency and predictors of FH....... The prevalence of the four FH mutations was 0.18% (1:565), suggesting a total prevalence of FH mutations of 0.46% (1:217). Using the Dutch Lipid Clinic Network (DLCN) criteria, odds ratios for an FH mutation were 439 (95% CI: 170-1 138) for definite FH, 90 (53-152) for probable FH, and 18 (13-25) for possible FH......-cholesterol concentration to discriminate between mutation carriers and non-carriers was 4.4 mmol/L. CONCLUSION: Familial hypercholesterolaemia-causing mutations are estimated to occur in 1:217 in the general population and are best identified by a definite or probable phenotypic diagnosis of FH based on the DLCN criteria...

  17. [Clinical features and acid alpha-glucosidase gene mutation in 7 Chinese patients with glycogen storage disease type II]. (United States)

    Liu, Qi; Zhao, Juan; Wang, Zhao-xia; Zhang, Wei; Yuan, Yun


    To explore the clinical features and acid alpha-glucosidase (GAA) gene mutations of Chinese patients with glycogen storage disease typeII(GSDII). Seven patients with GSDII were diagnosed by muscle pathology examination at Department of Neurology, Peking University First Hospital from 2003 to 2011. One patient with infant-onset presented development retardation, generalized muscle weakness, dyspnea, cardiomegaly and hepatomegaly. Six cases were of late-onset ranging from 1 to 29 years. Their main clinical features included progressive muscle weakness. Two patients developed respiratory insufficiency. Increased serum creatine kinase was detected in all of them. Electromyography studies showed myopathic (n = 5) and neuropathic (n = 1) changes. Muscle biopsies showed basophilic vacuoles in muscle fibers containing a large amounts of glycogen on electron microscopy. GAA gene mutation was detected by direct sequencing of polymerase chain reaction (PCR) product. Novel mutations were screened in 100 normal controls. GAA gene mutations were found in all of them, including 10 point mutations and 1 frameshift deletion. Six mutations (p. P361L, p. P266S, p.R437C, p.R600C, p.W746S and p.W746*) have been reported before. And five novel mutations (p.R168Q, p.R168P, p.E521V, p.R594H and c.827_845del) were found in this study. None of these novel mutations were found in 100 normal controls except for p.R168Q mutation in two normal controls. p. P361L and p.W746* were detected in two unrelated GSDII patients while other mutations were carried by only one patient. In our study, we found several novel GAA mutations in Chinese patients with GSDII. No hot spot mutation of GAA gene existed in our patient group. However, p. P266S, p. P361L and p.R437C might be associated with late-onset GSDII.

  18. Detection of Deafness-Causing Mutations in the Greek Mitochondrial Genome

    Directory of Open Access Journals (Sweden)

    Haris Kokotas


    Full Text Available Mitochondrion harbors its own DNA, known as mtDNA, encoding certain essential components of the mitochondrial respiratory chain and protein synthesis apparatus. mtDNA mutations have an impact on cellular ATP production and many of them are undoubtedly a factor that contributes to sensorineural deafness, including both syndromic and non-syndromic forms. Hot spot regions for deafness mutations are the MTRNR1 gene, encoding the 12S rRNA, the MTTS1 gene, encoding the tRNA for Ser(UCN, and the MTTL1 gene, encoding the tRNA for Leu(UUR. We investigated the impact of mtDNA mutations in the Greek hearing impaired population, by testing a cohort of 513 patients suffering from childhood onset prelingual or postlingual, bilateral, sensorineural, syndromic or non-syndromic hearing loss of any degree for six mitochondrial variants previously associated with deafness. Screening involved the MTRNR1 961delT/insC and A1555G mutations, the MTTL1 A3243G mutation, and the MTTS1 A7445G, 7472insC and T7510C mutations. Although two patients were tested positive for the A1555G mutation, we failed to identify any subject carrying the 961delT/insC, A3243G, A7445G, 7472insC, or T7510C mutations. Our findings strongly support our previously raised conclusion that mtDNA mutations are not a major risk factor for sensorineural deafness in the Greek population.

  19. On the Usefulness of Radioactive Hot Springs in Mars Exploration (United States)

    Rask, J. C.; Bywaters, K. F.; Magnuson, T. S.


    We report on a systematic characterization of the radiation environment, water temperatures, and microbial systems of Worswick Hot Springs, as a model for future characterization of polar hot spring environments.

  20. Children in Hot Cars Result in Fatal Consequences

    Medline Plus

    Full Text Available ... Share this! Home » Health Tips » Holiday and Seasonal Children in Hot Cars Result in Fatal Consequences Emergency ... the overwhelming dangers associated with leaving anyone, especially children in hot, unventilated vehicles during the summer. Children ...

  1. Children in Hot Cars Result in Fatal Consequences (United States)

    ... this! Home » Health Tips » Holiday and Seasonal Children in Hot Cars Result in Fatal Consequences Emergency physicians are warning the public ... throbbing headache, dizziness, nausea, confusion and disorientation. READ IN SUMMER Children in Hot Cars Result in Fatal ...

  2. Children in Hot Cars Result in Fatal Consequences

    Medline Plus

    Full Text Available ... of Emergency Phycisians Toggle navigation Emergency 101 Is it an Emergency? Emergency Care or Urgent Care? When ... being left alone in a hot vehicle. “Putting it bluntly, leaving your child in a hot car ...

  3. Children in Hot Cars Result in Fatal Consequences

    Medline Plus

    Full Text Available ... Tips » Holiday and Seasonal Children in Hot Cars Result in Fatal Consequences Emergency physicians are warning the ... the country die each year as a direct result of being left alone in a hot vehicle. “ ...

  4. Children in Hot Cars Result in Fatal Consequences

    Medline Plus

    Full Text Available ... of Emergency Phycisians Toggle navigation Emergency 101 Is it an Emergency? Emergency Care or Urgent Care? When ... being left alone in a hot vehicle. “Putting it bluntly, leaving your child in a hot car ...

  5. Children in Hot Cars Result in Fatal Consequences

    Medline Plus

    Full Text Available ... Health Tips » Holiday and Seasonal Children in Hot Cars Result in Fatal Consequences Emergency physicians are warning ... it bluntly, leaving your child in a hot car is like leaving your child in a lit ...

  6. Hot-Film and Hot-Wire Anemometry for a Boundary Layer Active Flow Control Test (United States)

    Lenahan, Keven C.; Schatzman, David M.; Wilson, Jacob Samuel


    Unsteady active flow control (AFC) has been used experimentally for many years to minimize bluff-body drag. This technology could significantly improve performance of rotorcraft by cleaning up flow separation. It is important, then, that new actuator technologies be studied for application to future vehicles. A boundary layer wind tunnel was constructed with a 1ft-x-3ft test section and unsteady measurement instrumentation to study how AFC manipulates the boundary layer to overcome adverse pressure gradients and flow separation. This unsteady flow control research requires unsteady measurement methods. In order to measure the boundary layer characteristics, both hot-wire and hot-film Constant Temperature Anemometry is used. A hot-wire probe is mounted in the flow to measure velocity while a hot-film array lays on the test surface to measure skin friction. Hot-film sensors are connected to an anemometer, a Wheatstone bridge circuit with an output that corresponds to the dynamic flow response. From this output, the time varying flow field, turbulence, and flow reversal can be characterized. Tuning the anemometers requires a fan test on the hot-film sensors to adjust each output. This is a delicate process as several variables drastically affect the data, including control resistance, signal input, trim, and gain settings.

  7. Predictions of hot spot residues at protein-protein interfaces using support vector machines.

    Directory of Open Access Journals (Sweden)

    Stefano Lise

    Full Text Available Protein-protein interactions are critically dependent on just a few 'hot spot' residues at the interface. Hot spots make a dominant contribution to the free energy of binding and they can disrupt the interaction if mutated to alanine. Here, we present HSPred, a support vector machine(SVM-based method to predict hot spot residues, given the structure of a complex. HSPred represents an improvement over a previously described approach (Lise et al, BMC Bioinformatics 2009, 10:365. It achieves higher accuracy by treating separately predictions involving either an arginine or a glutamic acid residue. These are the amino acid types on which the original model did not perform well. We have therefore developed two additional SVM classifiers, specifically optimised for these cases. HSPred reaches an overall precision and recall respectively of 61% and 69%, which roughly corresponds to a 10% improvement. An implementation of the described method is available as a web server at It is free to non-commercial users.

  8. 21 CFR 880.6085 - Hot/cold water bottle. (United States)


    ... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Hot/cold water bottle. 880.6085 Section 880.6085... Devices § 880.6085 Hot/cold water bottle. (a) Identification. A hot/cold water bottle is a device intended for medical purposes that is in the form of a container intended to be filled with hot or cold...

  9. Thiamine-responsive megaloblastic anemia: identification of novel compound heterozygotes and mutation update. (United States)

    Bergmann, Anke K; Sahai, Inderneel; Falcone, Jill F; Fleming, Judy; Bagg, Adam; Borgna-Pignati, Caterina; Casey, Robin; Fabris, Luca; Hexner, Elizabeth; Mathews, Lulu; Ribeiro, Maria Leticia; Wierenga, Klaas J; Neufeld, Ellis J


    To determine causative mutations and clinical status of 7 previously unreported kindreds with TRMA syndrome, (thiamine-responsive megaloblastic anemia, online Mendelian inheritance in man, no. 249270), a recessive disorder of thiamine transporter Slc19A2. Genomic DNA was purified from blood, and SLC19A2 mutations were characterized by sequencing polymerase chain reaction-amplified coding regions and intron-exon boundaries of all probands. Compound heterozygotes were further analyzed by sequencing parents, or cloning patient genomic DNA, to ascertain that mutations were in trans. We detected 9 novel SLC19A2 mutations. Of these, 5 were missense, 3 were nonsense, and 1 was insertion. Five patients from 4 kindreds were compound heterozygotes, a finding not reported previously for this disorder, which has mostly been found in consanguineous kindreds. SLC19A2 mutation sites in TRMA are heterogeneous; with no regional "hot spots." TRMA can be caused by heterozygous compound mutations; in these cases, the disorder is found in outbred populations. To the extent that heterozygous patients were ascertained at older ages, a plausible explanation is that if one or more allele(s) is not null, partial function might be preserved. Phenotypic variability may lead to underdiagnosis or diagnostic delay, as the average time between the onset of symptoms and diagnosis was 8 years in this cohort.

  10. THIAMINE RESPONSIVE MEGALOBLASTIC ANEMIA: Identification of novel compound heterozygotes and mutation update (United States)

    Neuwirth, Anke K.; Sahai, Inderneel; Falcone, Jill F.; Fleming, Judy; Bagg, Adam; Borgna-Pignatti, Caterina; Casey, Robin; Fabris, Luca; Hexner, Elizabeth; Mathews, Lulu; Ribeiro, Maria Leticia; Wierenga, Klaas J.; Neufeld, Ellis J.


    Objective To determine causative mutations and clinical status of seven previously unreported kindreds with TRMA syndrome, (Thiamine Responsive Megaloblastic Anemia, OMIM #249270), a recessive disorder of thiamine transporter Slc19A2. Study design Genomic DNA was purified from blood, and SLC19A2 mutations were characterized by sequencing PCR-amplified coding regions and intron-exon boundaries of all probands. Compound heterozygotes were further analyzed by sequencing parents, or cloning patient genomic DNA, to ascertain that mutations were in trans. Results We detected 9 novel SLC19A2 mutations. Of these, five were missense, three nonsense, and one insertion. Five patients from four kindreds were compound heterozygotes, a finding not reported previously for this disorder, which has mostly been found in consanguineous kindreds. Conclusion SLC19A2 mutation sites in TRMA are heterogeneous; with no regional “hot spots”. TRMA can be caused by heterozygous compound mutations; in these cases, the disorder is found in outbred populations. To the extent that heterozygous patients were ascertained at older ages, a plausible explanation is that if one or more allele(s) is not null, partial function might be preserved. Phenotypic variability may lead to underdiagnosis or diagnostic delay, as the average time between the onset of symptoms and diagnosis was 8 years in this cohort. PMID:19643445

  11. Overexpression and promoter mutation of the TERT gene in malignant pleural mesothelioma. (United States)

    Tallet, A; Nault, J-C; Renier, A; Hysi, I; Galateau-Sallé, F; Cazes, A; Copin, M-C; Hofman, P; Andujar, P; Le Pimpec-Barthes, F; Zucman-Rossi, J; Jaurand, M-C; Jean, D


    Malignant pleural mesothelioma (MPM) is a very aggressive tumor with no known curative treatment. Better knowledge of the molecular mechanisms of mesothelial carcinogenesis is required to develop new therapeutic strategies. MPM, like all cancer cells, needs to maintain telomere length to prevent senescence. Previous studies suggested that the telomere lengthening mechanism in MPM is based mainly on telomerase activity. For this reason, we focused on the key catalytic enzyme, TERT (telomerase reverse transcriptase), by analyzing its gene expression in MPM and by studying the mechanism underlying its upregulation. We used our large collection of MPM composed of 61 MPM in culture and 71 frozen MPM tumor samples. Evaluation of TERT mRNA expression by quantitative RT-PCR showed overexpression in MPM in culture compared with normal mesothelial cells, and in MPM tumor samples compared with normal pleura. We identified a 'hot spot' of mutations in the TERT gene core promoter in both MPM in culture and in MPM tumor samples with an overall frequency of 15%. Furthermore, data clearly identified mutation in the TERT promoter as a mechanism of TERT mRNA upregulation in MPM. In contrast, gene copy number amplification was not associated with TERT overexpression. Then, we analyzed the clinicopathological, etiological and genetic characteristics of MPM with mutations in the TERT promoter. TERT promoter mutations were more frequent in MPM with sarcomatoid histologic subtype (Ppromoter mutations, which lead to TERT mRNA upregulation. This is the first recurrent gain-of-function oncogenic mutations identified in MPM.

  12. Installation package for a solar heating and hot water system (United States)


    Development and installation of two commercial solar heating and hot water systems are reported. The systems consist of the following subsystems: collector, storage, transport, hot water, auxiliary energy and controls. General guidelines are provided which may be utilized in development of detailed installation plans and specifications. In addition, operation, maintenance and repair of a solar heating and hot water system instructions are included.

  13. Discovery of feature-based hot spots using supervised clustering (United States)

    Ding, Wei; Stepinski, Tomasz F.; Parmar, Rachana; Jiang, Dan; Eick, Christoph F.


    Feature-based hot spots are localized regions where the attributes of objects attain high values. There is considerable interest in automatic identification of feature-based hot spots. This paper approaches the problem of finding feature-based hot spots from a data mining perspective, and describes a method that relies on supervised clustering to produce a list of hot spot regions. Supervised clustering uses a fitness function rewarding isolation of the hot spots to optimally subdivide the dataset. The clusters in the optimal division are ranked using the interestingness of clusters that encapsulate their utility for being hot spots. Hot spots are associated with the top ranked clusters. The effectiveness of supervised clustering as a hot spot identification method is evaluated for four conceptually different clustering algorithms using a dataset describing the spatial distribution of ground ice on Mars. Clustering solutions are visualized by specially developed raster approximations. Further assessment of the ability of different algorithms to yield hot spots is performed using raster approximations. Density-based clustering algorithm is found to be the most effective for hot spot identification. The results of the hot spot discovery by supervised clustering are comparable to those obtained using the G* statistic, but the new method offers a high degree of automation, making it an ideal tool for mining large datasets for the existence of potential hot spots.

  14. How mixing during hot water draw-offs influence the thermal performance of small solar domestic hot water systems

    DEFF Research Database (Denmark)

    Furbo, Simon; Shah, Louise Jivan


    CFD calculations on the mixing during hot water draw-offs in vertical hot water tanks with different diameters have been carried out. The calculations, which were carried out with the same cold water inlet design, showed that the extent of mixing is strongly influenced by the tank diameter....... The extent of mixing is increasing for increasing tank diameter. Further, calculations of the yearly thermal performance of small solar domestic hot water systems with hot water tanks with different mixing rates during hot water draw-offs were carried out. Both solar domestic hot water systems with mantle......, and that a decreased auxiliary volume in the tanks and an increased height/diameter ratio of the tanks will increase the thermal performance of the systems. The investigations showed further, that mixing during hot water draw-offs decreases the thermal performance of solar domestic hot water systems. The mixing...

  15. De novo mutation in the DSPP gene associated with dentinogenesis imperfecta type II in a Japanese family. (United States)

    Kida, Miyuki; Tsutsumi, Tomonori; Shindoh, Masanobu; Ikeda, Hisami; Ariga, Tadashi


    Dentinogenesis imperfecta (DGI) type II is one of the most common dominantly inherited dentin defects, in which both the primary and permanent teeth are affected. Here, we report a Japanese family with autosomal-dominant DGI type II, including both molecular genetic defects and pathogenesis with histological analysis. Mutation analysis revealed a mutation (c.53T>A, p.V18D, g.1192T>A) involving the second nucleotide of the first codon within exon 3 of the dentin sialophosphoprotein (DSPP) gene. This mutation has previously been reported in a Korean family. Thus far, 24 allelic DSPP mutations have been reported, and this is the seventh mutation involving the DSPP V18 residue. Among those, only one other was shown to be caused by a de novo mutation, and that mutation also affected the V18 amino acid residue. The DSPP V18 residue is highly conserved among other mammalian species. These findings thus suggest that the V18 amino acid might be a sensitive mutational hot spot, playing a critical role in the pathogenesis of DGI.

  16. Prediction of hot spot residues at protein-protein interfaces by combining machine learning and energy-based methods

    Directory of Open Access Journals (Sweden)

    Pontil Massimiliano


    Full Text Available Abstract Background Alanine scanning mutagenesis is a powerful experimental methodology for investigating the structural and energetic characteristics of protein complexes. Individual amino-acids are systematically mutated to alanine and changes in free energy of binding (ΔΔG measured. Several experiments have shown that protein-protein interactions are critically dependent on just a few residues ("hot spots" at the interface. Hot spots make a dominant contribution to the free energy of binding and if mutated they can disrupt the interaction. As mutagenesis studies require significant experimental efforts, there is a need for accurate and reliable computational methods. Such methods would also add to our understanding of the determinants of affinity and specificity in protein-protein recognition. Results We present a novel computational strategy to identify hot spot residues, given the structure of a complex. We consider the basic energetic terms that contribute to hot spot interactions, i.e. van der Waals potentials, solvation energy, hydrogen bonds and Coulomb electrostatics. We treat them as input features and use machine learning algorithms such as Support Vector Machines and Gaussian Processes to optimally combine and integrate them, based on a set of training examples of alanine mutations. We show that our approach is effective in predicting hot spots and it compares favourably to other available methods. In particular we find the best performances using Transductive Support Vector Machines, a semi-supervised learning scheme. When hot spots are defined as those residues for which ΔΔG ≥ 2 kcal/mol, our method achieves a precision and a recall respectively of 56% and 65%. Conclusion We have developed an hybrid scheme in which energy terms are used as input features of machine learning models. This strategy combines the strengths of machine learning and energy-based methods. Although so far these two types of approaches have mainly been

  17. Axions as hot and cold dark matter

    Energy Technology Data Exchange (ETDEWEB)

    Jeong, Kwang Sik [Deutsches Elektronen-Synchrotron (DESY), Hamburg (Germany); Kawasaki, Masahiro [Tokyo Univ., Kashiwa (Japan). Inst. for Cosmic Ray Research; Tokyo Univ., Kashiwa (Japan). Kavli IPMU, TODIAS; Takahashi, Fuminobu [Tohoku Univ., Sendai (Japan). Dept. of Physics; Tokyo Univ., Kashiwa (Japan). Kavli IPMU, TODIAS


    The presence of a hot dark matter component has been hinted at 3{sigma} by a combination of the results from different cosmological observations. We examine a possibility that pseudo Nambu- Goldstone bosons account for both hot and cold dark matter components. We show that the QCD axions can do the job for the axion decay constant f{sub a}

  18. Hot subdwarf formation: Confronting theory with observation

    Directory of Open Access Journals (Sweden)

    Geier S.


    Full Text Available The formation of hot subdwarf stars is still unclear. Both single-star and binary scenarios have been proposed to explain the properties of these evolved stars situated at the extreme blue end of the horizontal branch. The observational evidence gathered in the last decade, which revealed high fractions of binaries, shifted the focus from the single-star to the binary formation scenarios. Common envelope ejection, stable Roche lobe overflow and the merger of helium white dwarfs seemed to be sufficient to explain the formation of both the binary as well as the remaining single hot subdwarfs. However, most recent and rather unexpected observations challenge the standard binary evolution scenarios.

  19. Hot cheese: a processed Swiss cheese model. (United States)

    Li, Y; Thimbleby, H


    James Reason's classic Swiss cheese model is a vivid and memorable way to visualise how patient harm happens only when all system defences fail. Although Reason's model has been criticised for its simplicity and static portrait of complex systems, its use has been growing, largely because of the direct clarity of its simple and memorable metaphor. A more general, more flexible and equally memorable model of accident causation in complex systems is needed. We present the hot cheese model, which is more realistic, particularly in portraying defence layers as dynamic and active - more defences may cause more hazards. The hot cheese model, being more flexible, encourages deeper discussion of incidents than the simpler Swiss cheese model permits.

  20. Hot Particles Attract in a Cold Bath

    CERN Document Server

    Tanaka, Hidenori; Brenner, Michael P


    Controlling interactions out of thermodynamic equilibrium is crucial for designing addressable and functional self-organizing structures. These active interactions also underpin collective behavior in biological systems. Here we study a general setting of active particles in a bath of passive particles, and demonstrate a novel mechanism for long ranged attraction between active particles. The mechanism operates when the translational persistence length of the active particle motion is smaller than the particle diameter. In this limit, the system reduces to particles of higher diffusivity ("hot" particles) in a bath of particles with lower diffusivity ("cold" particles). This attractive interaction arises as a hot particle pushes cold particles away to create a large hole around itself, and the holes interact via a depletion-like attraction even though all particles have the same size. Although the mechanism occurs outside the parameter range of typical biological organisms, the mechanism could be realized in ...


    Institute of Scientific and Technical Information of China (English)

    LIU Junshan; WANG Liding; LIU Chong; LUO Yi


    Fabrication of microchannels on polymethylmethacrylate (PMMA) substrates using novel microfabrication methods is demonstrated. The image of microchannels is transferred from a silicon master possessing the inverse image of the microchannel to a PMMA plate by using hot embossing methods. The silicon master is electrostatically bonded to a Pyrex 7740 glass wafer, which improves the device yield from about 20 devices to hundreds of devices per master. Effects of embossing temperature, pressure and time on the accuracy of replication are systematically studied using the orthogonal factorial design. According to the suggested experimental model, the time for the whole embossing procedure is shorten from about 20 min to 6 min, and the accuracy of replication is 99.3%.The reproducibility of the hot embossing method is evaluated using 10 channels on different microfluidic devices, with variations of 1.4 % in depth and 1.8% in width.

  2. An Optimization Study of Hot Stamping Operation (United States)

    Ghoo, Bonyoung; Umezu, Yasuyoshi; Watanabe, Yuko; Ma, Ninshu; Averill, Ron


    In the present study, 3-dimensional finite element analyses for hot-stamping processes of Audi B-pillar product are conducted using JSTAMP/NV and HEEDS. Special attention is paid to the optimization of simulation technology coupling with thermal-mechanical formulations. Numerical simulation based on FEM technology and optimization design using the hybrid adaptive SHERPA algorithm are applied to hot stamping operation to improve productivity. The robustness of the SHERPA algorithm is found through the results of the benchmark example. The SHERPA algorithm is shown to be far superior to the GA (Genetic Algorithm) in terms of efficiency, whose calculation time is about 7 times faster than that of the GA. The SHERPA algorithm could show high performance in a large scale problem having complicated design space and long calculation time.

  3. The Escaping Upper Atmospheres of Hot Jupiters (United States)

    Davidson, Eric; Jones, Gabrielle; Uribe, Ana; Carson, Joseph


    Hot Jupiters are massive gaseous planets which orbit closely to their parent star. The strong stellar irradiation at these small orbital separations causes the temperature of the upper atmosphere of the planet to rise. This can cause the planet's atmosphere to escape into space, creating an exoplanet outflow. We ascertained which factors determine the presence and structure of these outflows by creating one dimensional simulations of the density, pressure, velocity, optical depth, and neutral fraction of hot Jupiter atmospheres. This was done for planets of masses and radii ranging from 0.5-1.5 Mj and 0.5-1.5 Rj. We found the outflow rate to be highest for a planet of 0.5 Mj and 1.5 Rj at 5.3×10-14 Mj/Yr. We also found that the higher the escape velocity, the lower the chance of the planet having an outflow.

  4. Coiling Temperature Control in Hot Strip Mill (United States)

    Imanari, Hiroyuki; Fujiyama, Hiroaki

    Coiling temperature is one of the most significant factors in products of hot strip mill to determine material properties such as strength, toughness of steel, so it is very important to achieve accurate coiling temperature control (CTC). Usually there are a few pyrometers on the run out table in hot strip mill, therefore temperature model and its adapting system have large influences on the accuracy of CTC. Also unscheduled change of rolling speed has a bad effect to keep coiling temperature as its target. Newly developed CTC system is able to get very accurate coiling temperature against uncertain factors and disturbances by adopting easily identified temperature model, learning method and dynamic set up function. The features of the CTC system are discussed with actual data, and the effectiveness of the system is shown by actual control results.

  5. Hot deformation behavior of FGH96 superalloys

    Institute of Scientific and Technical Information of China (English)

    Jiantao Liu; Guoquan Liu; Benfu Hu; Yuepeng Song; Ziran Qin; Yiwen Zhang


    The hot deformation behavior of FGH96 superalloys at 1070-1170℃ and 5×10-4-2×10-1 s-1 were investigated by means of the isothermal compression tests at a Gleeble-1500 thermal mechanical simulator. The results show that dynamic recovery acts as the main softening mechanism below 2×10-3 s-1, whereas dynamic recrystallization acts as the main softening mechanism above 2×10-3 s-1during deformation; the temperature increase caused by the deformation and the corresponding softening stress is negligible; the thermal-mechanical constitutive model to describe the hot deformation behavior is given, and the value of the apparent deformation activation energy (Qdef) is determined to be 354.93 kJ/mol.

  6. Research Hot Spots of Black Peanut

    Institute of Scientific and Technical Information of China (English)


    Taking the research papers on black peanut in National Knowledge Infrastructure(CNKI)as data sources,we determine the research hot spots on black peanut using word frequency analysis,and analyze the main research directions that change over the years.The results show that the relevant researches on black peanut are mainly concentrated in six themes(study of varieties,physiological property,cultivation management,development prospects,relationship with agriculture,and molecular level);varieties,physiology,cultivation and other aspects are the focus of current research hot spots;the researches on selection and breeding of variety and various physiological and ecological mechanisms,still need a breakthrough.

  7. Hot water, fresh beer, and salt

    Energy Technology Data Exchange (ETDEWEB)

    Crawford, F.S. (Lawrence Berkeley Laboratory, Berkeley, CA (USA) Physics Department, University of California, Berkeley, CA (USA))


    In the hot chocolate effect'' the best musical scales (those with the finest tone quality, largest range, and best tempo) are obtained by adding salt to a glass of hot water supersaturated with air. Good scales can also be obtained by adding salt to a glass of freshly opened beer (supersaturated with CO{sub 2}) provided you first (a) get rid of much of the excess CO{sub 2} so as to produce smaller, hence slower, rising bubbles, and (b) get rid of the head of foam, which damps the standing wave and ruins the tone quality. Finally the old question, Do ionizing particles produce bubbles in fresh beer '' is answered experimentally.

  8. Hot water can freeze faster than cold?!?

    CERN Document Server

    Jeng, M


    We review the Mpemba effect, where intially hot water freezes faster than initially cold water. While the effect appears impossible at first sight, it has been seen in numerous experiments, was reported on by Aristotle, Francis Bacon, and Descartes, and has been well-known as folklore around the world. It has a rich and fascinating history, which culminates in the dramatic story of the secondary school student, Erasto Mpemba, who reintroduced the effect to the twentieth century scientific community. The phenomenon, while simple to describe, is deceptively complex, and illustrates numerous important issues about the scientific method: the role of skepticism in scientific inquiry, the influence of theory on experiment and observation, the need for precision in the statement of a scientific hypothesis, and the nature of falsifiability. We survey proposed theoretical mechanisms for the Mpemba effect, and the results of modern experiments on the phenomenon. Studies of the observation that hot water pipes are more ...

  9. Enabling Technologies for Ceramic Hot Section Components

    Energy Technology Data Exchange (ETDEWEB)

    Venkat Vedula; Tania Bhatia


    Silicon-based ceramics are attractive materials for use in gas turbine engine hot sections due to their high temperature mechanical and physical properties as well as lower density than metals. The advantages of utilizing ceramic hot section components include weight reduction, and improved efficiency as well as enhanced power output and lower emissions as a result of reducing or eliminating cooling. Potential gas turbine ceramic components for industrial, commercial and/or military high temperature turbine applications include combustor liners, vanes, rotors, and shrouds. These components require materials that can withstand high temperatures and pressures for long duration under steam-rich environments. For Navy applications, ceramic hot section components have the potential to increase the operation range. The amount of weight reduced by utilizing a lighter gas turbine can be used to increase fuel storage capacity while a more efficient gas turbine consumes less fuel. Both improvements enable a longer operation range for Navy ships and aircraft. Ceramic hot section components will also be beneficial to the Navy's Growth Joint Strike Fighter (JSF) and VAATE (Versatile Affordable Advanced Turbine Engines) initiatives in terms of reduced weight, cooling air savings, and capability/cost index (CCI). For DOE applications, ceramic hot section components provide an avenue to achieve low emissions while improving efficiency. Combustors made of ceramic material can withstand higher wall temperatures and require less cooling air. Ability of the ceramics to withstand high temperatures enables novel combustor designs that have reduced NO{sub x}, smoke and CO levels. In the turbine section, ceramic vanes and blades do not require sophisticated cooling schemes currently used for metal components. The saved cooling air could be used to further improve efficiency and power output. The objectives of this contract were to develop technologies critical for ceramic hot section

  10. Baroclinic Instability on Hot Extrasolar Planets

    CERN Document Server

    Polichtchouk, Inna


    We investigate baroclinic instability in flow conditions relevant to hot extrasolar planets. The instability is important for transporting and mixing heat, as well as for influencing large-scale variability on the planets. Both linear normal mode analysis and non-linear initial value calculations are carried out -- focusing on the freely-evolving, adiabatic situation. Using a high-resolution general circulation model (GCM) which solves the traditional primitive equations, we show that large-scale jets similar to those observed in current GCM simulations of hot extrasolar giant planets are likely to be baroclinically unstable on a timescale of few to few tens of planetary rotations, generating cyclones and anticyclones that drive weather systems. The growth rate and scale of the most unstable mode obtained in the linear analysis are in qualitative, good agreement with the full non-linear calculations. In general, unstable jets evolve differently depending on their signs (eastward or westward), due to the chang...

  11. Assessing the Biological Contribution to Mineralized Cap Formation in the Little Hot Creek Hot Spring System (United States)

    Floyd, J. G.; Beeler, S. R.; Mors, R. A.; Kraus, E. A.; 2016, G.; Piazza, O.; Frantz, C. M.; Loyd, S. J.; Berelson, W.; Stevenson, B. S.; Marenco, P. J.; Spear, J. R.; Corsetti, F. A.


    Hot spring environments exhibit unique redox/physical gradients that may create favorable conditions for the presence of life and commonly contain mineral precipitates that could provide a geologic archive of such ecosystems on Earth and potentially other planets. However, it is critical to discern biologic from abiotic formation mechanisms if hot spring-associated minerals are to be used as biosignatures. The study of modern hot spring environments where mineral formation can be directly observed is necessary to better interpret the biogenicity of ancient/extraterrestrial examples. Little Hot Creek (LHC), a hot spring located in the Long Valley Caldera, California, contains mineral precipitates composed of a carbonate base covered with amorphous silica and minor carbonate in close association with microbial mats/biofilms. Geological, geochemical, and microbiological techniques were integrated to investigate the role of biology in mineral formation at LHC. Geochemical measurements indicate that the waters of the spring are near equilibrium with respect to carbonate and undersaturated with respect to silica, implying additional processes are necessary to initiate cap formation. Geochemical modeling, integrating elemental and isotopic data from hot spring water and mineral precipitates, indicate that the abiotic processes of degassing and evaporation drive mineral formation at LHC, without microbial involvement. However, petrographic analysis of LHC caps revealed microbial microfabrics within silica mineral phases, despite the fact that microbial metabolism was not required for mineral precipitation. Our results show that microorganisms in hot spring environments can shape mineral precipitates even in the absence of a control on authigenesis, highlighting the need for structural as well as geochemical investigation in similar systems.

  12. Hot Strip Laminar Cooling Control Model

    Institute of Scientific and Technical Information of China (English)

    WANG Jun; WANG Guo-dong; LIU Xiang-hua


    The control model of laminar cooling system for hot strip, including air-cooling model, water-cooling model, temperature distribution model along thickness direction, feedforward control model, feedback control model and self-learning model, was introduced. PID arithmetic and Smith predictor controller were applied to feedback control. The sample of model parameter classification was given. The calculation process was shown by flow chart. The model has been proved to be simple, effective and of high precision.

  13. Virus Silicification under Simulated Hot Spring Conditions (United States)

    Laidler, James R.; Stedman, Kenneth M.


    Silicification of organisms in silica-depositing environments can impact both their ecology and their presence in the fossil record. Although microbes have been silicified under laboratory and environmental conditions, viruses have not. Bacteriophage T4 was successfully silicified under laboratory conditions that closely simulated those found in silica-depositing hot springs. Virus morphology was maintained, and a clear elemental signature of phosphorus was detected by energy-dispersive X-ray spectrophotometry (EDS).

  14. Hot Corrosion of Cobalt-Base Alloys (United States)


    scale is similar to that which has already been proposed for cobalt . The oxide ions would react with the Al203 to form aluminate ions in the Na2S04...resistance of cobalt -base and nickel-base alloys. The contract was accomplished under the technical direction of Dr. H. C. Graham of the Aerospace Research...Oxidized Specimens RESULTS AND DISCUSSION 1. INTRODUCfiON 2. SODIUM SULFATE INDUCED HOT CORROSION OF COBALT a. Introduction b. Experimental c

  15. Constraint Cooling of Hot Rolled Coil

    Institute of Scientific and Technical Information of China (English)

    WANG Li-juan; ZHANG Chun-li


    The layer thermal conductivity during constraint cooling of hot rolled coil was described by using equivalent thermal conductivity model and finite element method. Two radial stress concentration zones in constraint cooled coil were shown by numerical analysis, and the tension stress was assumed to be the main factor to induce stress corrosion. The experimental results show that the longer the water cooling time is, the smaller the grain size and the more uniform the grains are.

  16. Low Energy Air Conditioning for Hot Climates


    Almutairi, Hamad Hhn


    Fossil fuels are the major sources of electrical power generation in the world. Among all fossil fuels, oil is considered as the most sought-after fuel. The burden on countries that provide subsidized electricity produced from oil-fired power plants is noteworthy. Kuwait is a notable example of these countries. Electricity in Kuwait is heavily consumed by residential air conditioning, which comprises 60% of the total electricity generated at peak times on a hot summer day. From this perspecti...


    Directory of Open Access Journals (Sweden)



    Full Text Available The questions of consolidation of nanopowders concerning hot compaction by pressing activated by electric current action are considered. Mechanisms of grain boundary creep-sliding which are sequentially prevalent in a forming of compacted structures under influence of temperature factor and in the presence of a direct electric heating are discussed. Structural-transformational sources and conditions of forming of high physical-mechanical properties of nanopowder refractory solid-state products are described.

  18. Detection of a new mutation (1467-A) for the pedigree with mucopolysaccharidosis type Ⅱ from a Chinese family

    Institute of Scientific and Technical Information of China (English)

    GUO Yibin; PAN Jingxin; DU Chuanshu


    Mucopolysaccharidosis type II is of high genetic heterogeneity. PCR-DNA sequencing was used to study the mutation hot spots in the IDS gene of a Chinese MPS II pedigree. A new mutation (1467-A) not yet reported worldwide was detected. This mutation located at 448th codon in the coding region of exon 9 deletes one "A" at the end of 1467 bp (cDNA). The frame-shift mutation makes the peptide chain shorten from amino acids 550 to 459, probably altering the configuration of IDS enzyme protein remarkably and lowering the activation of IDS greatly. Therefore it is supposed to be the direct cause of the patient with MPS II and to be a necessary premise for prenatal gene diagnosis.

  19. Hot Dry Rock Geothermal Energy Development Program

    Energy Technology Data Exchange (ETDEWEB)

    Smith, M.C.; Hendron, R.H.; Murphy, H.D.; Wilson, M.G.


    During Fiscal Year 1987, emphasis in the Hot Dry Rock Geothermal Energy Development Program was on preparations for a Long-Term Flow Test'' of the Phase II'' or Engineering'' hot dry rock energy system at Fenton Hill, New Mexico. A successful 30-day flow test of the system during FY86 indicated that such a system would produce heat at a temperature and rate that could support operation of a commercial electrical power plant. However, it did not answer certain questions basic to the economics of long-term operation, including the rate of depletion of the thermal reservoir, the rate of water loss from the system, and the possibility of operating problems during extended continuous operation. Preparations for a one-year flow test of the system to answer these and more fundamental questions concerning hot dry rock systems were made in FY87: design of the required surface facilities; procurement and installation of some of their components; development and testing of slimline logging tools for use through small-diameter production tubing; research on temperature-sensitive reactive chemical tracers to monitor thermal depletion of the reservoir; and computer simulations of the 30-day test, extended to modeling the planned Long-Term Flow Test. 45 refs., 34 figs., 5 tabs.

  20. One-phonon excitations in hot nuclei

    Energy Technology Data Exchange (ETDEWEB)

    Vdovin, A.I.; Kosov, D.S. [Joint Institute for Nuclear Research, Moscow (Russian Federation)


    Equations of the random-phase approximation for hot nuclei are derived in the formalism of thermo-field dynamics. The model Hamiltonian used in the derivation involves separable effective interactions in the particle-particle and particl-hole channels. This initial Hamiltonian that forms the basis of the quasiparticle-phonon model at T = O is expressed in terms of thermal quasiparticles by means of two successive canonical transformations, i.e., by the conventional and thermal Bogolyubov transformations. The coefficients in the transformations are determined by minimizing the free-energy potential of a hot nucleus in the thermal vacuum state. The part that describes excitations of the hot nucleus in the random-phase approximation is then singled out in the resulting Hamiltonian at T {ne} O. The variational principle is used to derive equations for one-phonon excitations. If the Hamiltonian involves only residual particle-hole interaction, these equations coincide with those obtained earlier by means of Green`s functions and linearization of the equations of motion. The approach developed here can be used to extend the treatment beyond the random-phase approximation. 15 refs.

  1. Thermal Processes Governing Hot-Jupiter Radii

    CERN Document Server

    Spiegel, David S


    There have been many proposed explanations for the larger-than-expected radii of some transiting hot Jupiters, including either stellar or orbital energy deposition deep in the atmosphere or deep in the interior. In this paper, we explore the important influences on hot-Jupiter radius evolution of (i) additional heat sources in the high atmosphere, the deep atmosphere, and deep in the convective interior; (ii) consistent cooling of the deep interior through the planetary dayside, nightside, and poles; (iii) the degree of heat redistribution to the nightside; and (iv) the presence of an upper atmosphere absorber inferred to produce anomalously hot upper atmospheres and inversions in some close-in giant planets. In particular, we compare the radius expansion effects of atmospheric and deep-interior heating at the same power levels and derive the power required to achieve a given radius increase when night-side cooling is incorporated. We find that models that include consistent day/night cooling are more simila...

  2. Nuclear track radiography of 'hot' aerosol particles

    CERN Document Server

    Boulyga, S F; Kievets, M K; Lomonosova, E M; Zhuk, I V; Yaroshevich, O I; Perelygin, V P; Petrova, R I; Brandt, R; Vater, P


    Nuclear track radiography was applied to identify aerosol 'hot' particles which contain elements of nuclear fuel and fallout after Chernobyl NPP accident. For the determination of the content of transuranium elements in radioactive aerosols the measurement of the alpha-activity of 'hot' particles by SSNTD was used in this work, as well as radiography of fission fragments formed as a result of the reactions (n,f) and (gamma,f) in the irradiation of aerosol filters by thermal neutrons and high energy gamma quanta. The technique allowed the sizes and alpha-activity of 'hot' particles to be determined without extracting them from the filter, as well as the determination of the uranium content and its enrichment by sup 2 sup 3 sup 5 U, sup 2 sup 3 sup 9 Pu and sup 2 sup 4 sup 1 Pu isotopes. Sensitivity of determination of alpha activity by fission method is 5x10 sup - sup 6 Bq per particle. The software for the system of image analysis was created. It ensured the identification of track clusters on an optical imag...

  3. Hot electron transport and current sensing (United States)

    Abraham, Mathew Cheeran

    The effect of hot electrons on momentum scattering rates in a two-dimensional electron gas is critically examined. It is shown that with hot electrons it is possible to explore the temperature dependence of individual scattering mechanisms not easily probed under equilibrium conditions; both the Bloch-Gruneisen (BG) phonon scattering phenomena and the reduction in impurity scattering are clearly observed. The theoretical calculations are consistent with the results obtained from hot electrons experiments. As a function of bias current, a resistance peak is formed in a 2DEG if the low temperature impurity limited mobilities muI( T = 0) is comparable to muph(TBG ) the phonon limited mobility at the critical BG temperature. In this case, as the bias current is increased, the electron temperature Te rises due to Joule heating and the rapid increase in phonon scattering can be detected before the effect of the reduction in impurity scattering sets in. If muI(T = 0) wire defined in a 2DEG. Concurrently, an appropriate current imaging technique to detect this transition is sought. A rigorous evaluation of magnetic force microscopy (MFM) as a possible candidate to detect Poiseuille electronic flow was conducted, and a method that exploits the mechanical resonance of the MFM cantilever was implemented to significantly improve its current sensitivity.

  4. Theory of hot electron photoemission from graphene (United States)

    Ang, Lay Kee; Liang, Shijun

    Motivated by the development of Schottky-type photodetectors, some theories have been proposed to describe how the hot carriers generated by the incident photon are transported over the Schottky barrier through the internal photoelectric effect. One of them is Fowler's law proposed as early as 1931, which studied the temperature dependence of photoelectric curves of clean metals. This law is very successful in accounting for mechanism of detecting photons of energy lower than the band gap of semiconductor based on conventional metal/semiconductor Schottky diode. With the goal of achieving better performance, graphene/silicon contact-based- graphene/WSe2 heterostructure-based photodetectors have been fabricated to demonstrate superior photodetection efficiency. However, the theory of how hot electrons is photo-excited from graphene into semiconductor remains unknown. In the current work, we first examine the photoemission process from suspended graphene and it is found that traditional Einstein photoelectric effect may break down for suspended graphene due to the unique linear band structure. Furthermore, we find that the same conclusion applies for 3D graphene analog (e.g. 3D topological Dirac semi-metal). These findings are very useful to further improve the performance of graphene-based photodetector, hot-carrier solar cell and other kinds of sensor.

  5. Nucleosynthesis in Hot and Dense Media

    CERN Document Server

    Masood, Samina S


    We study the finite temperature and density effects on beta decay rates to compute their contributions to nucleosynthesis. QED type corrections to beta decay from the hot and dense background are estimated in terms of the statistical corrections to the self-mass of an electron. For this purpose, we re-examine the hot and dense background contributions to the electron mass and compute its effect to the beta decay rate, helium yield, energy density of the universe as well as the change in neutrino temperature from the first order contribution to the self-mass of electrons during these processes. We explicitly show that the thermal contribution to the helium abundance at T = m of a cooling universe 0.045 % is higher than the corresponding contribution to helium abundance of a heating universe 0.031% due to the existence of hot fermions before the beginning of nucleosynthesis and their absence after the nucleosynthesis, in the early universe. Thermal contribution to helium abundance was a simple quadratic functio...

  6. Hot-Gas Filter Ash Characterization Project

    Energy Technology Data Exchange (ETDEWEB)

    Swanson, M.L.; Hurley, J.P.; Dockter, B.A.; O`Keefe, C.A.


    Large-scale hot-gas filter testing over the past 10 years has revealed numerous cases of cake buildup on filter elements that has been difficult, if not impossible, to remove. At times, the cake can blind or bridge between candle filters, leading to filter failure. Physical factors, including particle-size distribution, particle shape, the aerodynamics of deposition, and system temperature, contribute to the difficulty in removing the cake, but chemical factors such as surface composition and gas-solid reactions also play roles in helping to bond the ash to the filters or to itself. This project is designed to perform the research necessary to determine the fuel-, sorbent-, and operations-related conditions that lead to blinding or bridging of hot-gas particle filters. The objectives of the project are threefold: (1) Determine the mechanisms by which a difficult-to-clean ash is formed and how it bridges hot-gas filters (2) Develop a method to determine the rate of bridging based on analyses of the feed coal and sorbent, filter properties, and system operating conditions and (3) Suggest and test ways to prevent filter bridging.

  7. Hot gas in clusters of galaxies

    Energy Technology Data Exchange (ETDEWEB)

    Doroshkevich, A.G.; Klypin, A.A.


    The origin, space distribution, and emission of the hot intergalactic gas in galaxy clusters are discussed. This gas may have been left over from the period when the clusters were formed. According to the adiabatic theory of galaxy formation, primordial gas would have become heated and compressed into hot, thin pancake-shaped structures, representing protoclusters. As the primordial gas crosses the shock waves bounding the protocluster, its temperature and entropy will rise significantly. The gas not consumed in the formation of galaxies will remain in the hot phase, enriched with heavy elements from supernova explosions in the cluster members and from galactic winds. This gas will retain the entropy acquired during the contraction process, but its temperature will be governed by the depth of the cluster potential well. The primordial entropy of intracluster gas is estimated from this theory, and the results are compared with x-ray observations of clusters. The best fit is achieved for a nuclear region of 0.3-Mpc radius. Temperature fluctuations = (1--2) x 10/sup -4/ in the microwave background radiation are inferred, depending only weakly on the model parameters.

  8. Illuminating Hot Jupiters in caustic crossing

    CERN Document Server

    Sajadian, Sedighe


    In recent years a large number of Hot Jupiters orbiting in a very close orbit around the parent stars have been explored with the transit and doppler effect methods. Here in this work we study the gravitational microlensing effect of a binary lens on a parent star with a Hot Jupiter revolving around it. Caustic crossing of the planet makes enhancements on the light curve of the parent star in which the signature of the planet can be detected by high precision photometric observations. We use the inverse ray shooting method with tree code algorithm to generate the combined light curve of the parent star and the planet. In order to investigate the probability of observing the planet signal, we do a Monte-Carlo simulation and obtain the observational optical depth of $\\tau \\sim 10^{-8}$. We show that about ten years observations of Galactic Bulge with a network of telescopes will enable us detecting about ten Hot Jupiter with this method. Finally we show that the observation of the microlensing event in infra-re...

  9. Fast cold gas in hot AGN outflows

    CERN Document Server

    Costa, Tiago; Haehnelt, Martin


    Observations of the emission from spatially extended cold gas around bright high-redshift QSOs reveal surprisingly large velocity widths exceeding 2000 km s^(-1), out to projected distances as large as 30 kpc. The high velocity widths have been interpreted as the signature of powerful AGN-driven outflows. Naively, these findings appear in tension with hydrodynamic models in which AGN-driven outflows are energy-driven and thus very hot with typical temperatures T = 10^6-7 K. Using the moving-mesh code Arepo, we perform 'zoom-in' cosmological simulations of a z = 6 QSO and its environment, following black hole growth and feedback via energy-driven outflows. In the simulations, the QSO host galaxy is surrounded by a clumpy circum-galactic medium pre-enriched with metals due to supernovae-driven galactic outflows. As a result, part of the AGN-driven hot outflowing gas can cool radiatively, leading to large amounts (> 10^9 M_sun) of cold gas comoving with the hot bipolar outflow. This results in velocity widths of...

  10. Hot Super Earths: disrupted young jupiters?

    CERN Document Server

    Nayakshin, Sergei


    Recent {\\em Kepler} observations revealed an unexpected abundance of "hot" Earth-size to Neptune-size planets in the inner $0.02-0.2$ AU from their parent stars. We propose that these smaller planets are the remnants of massive giant planets that migrated inward quicker than they could contract. We show that such disruptions naturally occur in the framework of the Tidal Downsizing hypothesis for planet formation. We find that the characteristic planet-star separation at which such "hot disruptions" occur is $R \\approx 0.03-0.2$ AU. This result is independent of the planet's embryo mass but is dependent on the accretion rate in the disc. At high accretion rates, $\\dot M \\simgt 10^{-6}\\msun$ yr$^{-1}$, the embryo is unable to contract quickly enough and is disrupted. At late times, when the accretion rate drops to $\\dot M \\simlt 10^{-8} \\msun$ yr$^{-1}$, the embryos migrate sufficiently slow to not be disrupted. These "late arrivals" may explain the well known population of hot jupiters. If type I migration reg...

  11. Common Β- Thalassaemia Mutations in

    Directory of Open Access Journals (Sweden)

    P Azarfam


    Full Text Available Introduction: β –Thalassaemia was first explained by Thomas Cooly as Cooly’s anaemia in 1925. The β- thalassaemias are hereditary autosomal disorders with decreased or absent β-globin chain synthesis. The most common genetic defects in β-thalassaemias are caused by point mutations, micro deletions or insertions within the β-globin gene. Material and Methods: In this research , 142 blood samples (64 from childrens hospital of Tabriz , 15 samples from Shahid Gazi hospital of Tabriz , 18 from Urumia and 45 samples from Aliasghar hospital of Ardebil were taken from thalassaemic patients (who were previously diagnosed .Then 117 non-familial samples were selected . The DNA of the lymphocytes of blood samples was extracted by boiling and Proteinase K- SDS procedure, and mutations were detected by ARMS-PCR methods. Results: From the results obtained, eleven most common mutations,most of which were Mediterranean mutations were detected as follows; IVS-I-110(G-A, IVS-I-1(G-A ،IVS-I-5(G-C ,Frameshift Codon 44 (-C,( codon5(-CT,IVS-1-6(T-C, IVS-I-25(-25bp del ,Frameshift 8.9 (+G ,IVS-II-1(G-A ,Codon 39(C-T, Codon 30(G-C the mutations of the samples were defined. The results showed that Frameshift 8.9 (+G, IVS-I-110 (G-A ,IVS-II-I(G-A, IVS-I-5(G-C, IVS-I-1(G-A , Frameshift Codon 44(-C , codon5(-CT , IVS-1-6(T-C , IVS-I-25(-25bp del with a frequency of 29.9%, 25.47%,17.83%, 7.00%, 6.36% , 6.63% , 3.8% , 2.5% , 0.63% represented the most common mutations in North - west Iran. No mutations in Codon 39(C-T and Codon 30(G-C were detected. Cunclusion: The frequency of the same mutations in patients from North - West of Iran seems to be different as compared to other regions like Turkey, Pakistan, Lebanon and Fars province of Iran. The pattern of mutations in this region is more or less the same as in the Mediterranean region, but different from South west Asia and East Asia.

  12. [Founder mutation in Lynch syndrome]. (United States)

    Cajal, Andrea R; Piñero, Tamara A; Verzura, Alicia; Santino, Juan Pablo; Solano, Angela R; Kalfayan, Pablo G; Ferro, Alejandra; Vaccaro, Carlos


    Lynch syndrome is the most frequent syndrome in hereditary colorectal cancer, a family-specific deleterious mutations in genes encoding DNA reparation proteins: MLH1 (mutL homolog 1), MSH2, MSH6 (mutS homolog 2 y 6, respectively), PMS2 (PMS1 homolog 2, mismatch repair system component) y MUTYH (mutY DNA glycosylase). The c.2252_2253delAA, p.Lys751Serfs*3 mutation in MLH1 gene segregates with a haplotype reported in the northern region of Italy and whose origin was attributed to a founder effect. This mutation co-segregates with typical characteristics of Lynch syndrome, including early age at onset and multiple primary tumors in the same individual, a high frequency of pancreatic cancer, high microsatellite instability and lack of PMS2 expression. This report describes a mutation in an Argentinian patient with endometrioid adenocarcinoma of uterus. Her first-degree relatives had a history of colon cancer diagnosed before 50 years, fulfilling the Amsterdam Criteria I and Lynch syndrome II. The high pathogenicity associated to this mutation makes necessary the study of all members from families with hereditary cancer, allowing pre-symptomatic genetic diagnosis, early assessment and the instauration of preventive treatments.

  13. SQSTM1 Mutations and Glaucoma.

    Directory of Open Access Journals (Sweden)

    Todd E Scheetz

    Full Text Available Glaucoma is the most common cause of irreversible blindness worldwide. One subset of glaucoma, normal tension glaucoma (NTG occurs in the absence of high intraocular pressure. Mutations in two genes, optineurin (OPTN and TANK binding kinase 1 (TBK1, cause familial NTG and have known roles in the catabolic cellular process autophagy. TKB1 encodes a kinase that phosphorylates OPTN, an autophagy receptor, which ultimately activates autophagy. The sequestosome (SQSTM1 gene also encodes an autophagy receptor and also is a target of TBK1 phosphorylation. Consequently, we hypothesized that mutations in SQSTM1 may also cause NTG. We tested this hypothesis by searching for glaucoma-causing mutations in a cohort of NTG patients (n = 308 and matched controls (n = 157 using Sanger sequencing. An additional 1098 population control samples were also analyzed using whole exome sequencing. A total of 17 non-synonymous mutations were detected which were not significantly skewed between cases and controls when analyzed separately, or as a group (p > 0.05. These data suggest that SQSTM1 mutations are not a common cause of NTG.

  14. Whistler Solitons in Plasma with Anisotropic Hot Electron Admixture (United States)

    Khazanov, G. V.; Krivorutsky, E. N.; Gallagher, D. L.


    The longitudinal and transverse modulation instability of whistler waves in plasma, with a small admixture of hot anisotropic electrons, is discussed. If the hot particles temperature anisotropy is positive, it is found that, in such plasma, longitudinal perturbations can lead to soliton formation for frequencies forbidden in cold plasma. The soliton is enriched by hot particles. The frequency region unstable to transverse modulation in cold plasma in the presence of hot electrons is divided by stable domains. For both cases the role of hot electrons is more significant for whistlers with smaller frequencies.

  15. Lubrication in Hot Tube Extrusion of Superalloys and Ti Alloys

    Institute of Scientific and Technical Information of China (English)


    Tubular products made of superalloys and titanium alloys usually work in high temperature environment and applied heavy loading. Hot extrusion is the best technology to form tubular billets with fine microstructures and good mechanical properties. Lubrication is one of the key techniques in hot extrusion, glass lubricants are most suitable for hot extrusion. Lubrication technique in hot extrusion is dealt with in this paper, the lubrication principle of hot tube extrusion is presented. Experiments of glass lubricated backward tube extrusion of titanium alloys and forward tube extrusion of superalloys are also discussed.

  16. Accuracy Of Hot-Wire Anemometry In Supersonic Turbulence (United States)

    Logan, Pamela; Mckenzie, Robert L.; Bershader, Daniel


    Sensitivity of hot-wire probe compared to laser-induced-florescence measurements. Report discusses factors affecting readings of hot-wire anemometer in turbulent supersonic boundary layer. Presents theoretical analysis of responses of hot-wire probe to changes in flow; also compares measurements by hot-wire probe with measurements of same flows by laser-induced fluorescence (LIF). Because LIF provides spatially and temporally resolved data on temperature, density, and pressure, provides independent means to determine responses of hot-wire anemometers to these quantities.

  17. Driven by Mutations: The Predictive Value of Mutation Subtype in EGFR-Mutated Non-Small Cell Lung Cancer. (United States)

    Castellanos, Emily; Feld, Emily; Horn, Leora


    EGFR-mutated NSCLC is a genetically heterogeneous disease that includes more than 200 distinct mutations. The implications of mutational subtype for both prognostic and predictive value are being increasingly understood. Although the most common EGFR mutations-exon 19 deletions or L858R mutations-predict sensitivity to EGFR tyrosine kinase inhibitors (TKIs), it is now being recognized that outcomes may be improved in patients with exon 19 deletions. Additionally, 10% of patients will have an uncommon EGFR mutation, and response to EGFR TKI therapy is highly variable depending on the mutation. Given the growing recognition of the genetic and clinical variation seen in this disease, the development of comprehensive bioinformatics-driven tools to both analyze response in uncommon mutation subtypes and inform clinical decision making will be increasingly important. Clinical trials of novel EGFR TKIs should prospectively account for the presence of uncommon mutation subtypes in study design.

  18. Successful switch from insulin to oral sulfonylurea therapy in HNF1A-MODY Tunisian patient with the P291fsinsC mutation. (United States)

    Khelifa, Souhaïra Ben; Dendana, Azza; Barboura, Ilhem; Khochtali, Ines; Chahed, Hinda; Ferchichi, Selima; Miled, Abdelhedi


    The hot spot mutation P291fsinsC was identified for the first time in a 26 years old Tunisian woman. The low serum level of high C-reactive protein was helpful to target the HNF1A gene. Due to the molecular diagnosis, the change from insulin to sulfonylurea therapy was performed successfully.

  19. RAS testing practices and RAS mutation prevalence among patients with metastatic colorectal cancer: results from a Europe-wide survey of pathology centres

    NARCIS (Netherlands)

    Boleij, A.; Tack, V.; Taylor, A.; Kafatos, G.; Jenkins-Anderson, S.; Tembuyser, L.; Dequeker, E.; Krieken, J.H.J.M. van


    BACKGROUND: Treatment options for patients with metastatic colorectal cancer (mCRC) include anti-epithelial growth factor therapies, which, in Europe, are indicated in patients with RAS wild-type tumours only and require prior mutation testing of "hot-spot" codons in exons 2, 3 and 4 of KRAS and

  20. A series of West European patients with severe cardiac and skeletal myopathy associated with a de novo R406W mutation in desmin. (United States)

    Dagvadorj, Ayush; Olivé, Montse; Urtizberea, Jean-Andoni; Halle, Martin; Shatunov, Alexey; Bönnemann, Carsten; Park, Kye-Yoon; Goebel, Hans H; Ferrer, Isidro; Vicart, Patrick; Dalakas, Marinos C; Goldfarb, Lev G


    Desminopathy is a familial or sporadic cardiac and skeletal muscular dystrophy associated with mutations in desmin. We have previously characterized a de novo desmin R406W mutation in a patient of European origin with early onset muscle weakness in the lower extremities and atrioventricular conduction block requiring a permanent pacemaker. The disease relentlessly progressed resulting in severe incapacity within 5 years after onset. We have now identified three other patients with early onset rapidly progressive cardiac and skeletal myopathy caused by this same desmin R406W mutation. The mutation was present in each studied patient, but not in their parents or other unaffected family members, indicating that the mutation in all four cases was generated de novo. The patients' mutation-carrying chromosomes showed no similarity, suggesting that the R406W mutation has occurred independently. These observations strongly confirm that the de novo R406W desmin mutation is the genetic basis for early-onset cardiac and skeletal myopathy in patients with sporadic disease and indicate that desmin position 406 is a hot spot for spontaneous mutations. The high pathogenic potential of this mutation can be explained by its location in the highly conserved YRKLLEGEE motif at the C-terminal end of the 2B helix that has a critical role in the process of desmin filament assembly.

  1. Rapid Mutation Scanning of Genes Associated with Familial Cancer Syndromes Using Denaturing High-Performance Liquid Chromatography

    Directory of Open Access Journals (Sweden)

    Deborah J. Marsh


    Full Text Available Germline mutations in tumor suppressor genes, or less frequently oncogenes, have been identified in up to 19 familial cancer syndromes including Li-Fraumeni syndrome, familial paraganglioma, familial adenomatous polyposis coli and breast and ovarian cancers. Multiple genes have been associated with some syndromes as approximately 26 genes have been linked to the development of these familial cancers. With this increased knowledge of the molecular determinants of familial cancer comes an equal expectation for efficient genetic screening programs. We have trialled denaturing highperformance liquid chromatography (dHPLC as a tool for rapid germline mutation scanning of genes implicated in three familial cancer syndromes - Cowden syndrome (PTEN mutation, multiple endocrine neoplasia type 2 (RET mutation and von Hippel-Lindau disease (VHL mutation. Thirty-two mutations, including 21 in PTEN, 9 in RET plus a polymorphism, and 2 in VHL, were analyzed using the WAVE DNA fragment analysis system with 100% detection efficiency. In the case of the tumor suppressor gene PTEN, mutations were scattered along most of the gene. However, mutations in the RET proto-oncogene associated with multiple endocrine neoplasia type 2 were limited to specific clusters or “hot spots”. The use of GC-clamped primers to scan for mutations scattered along PTEN exons was shown to greatly enhance the sensitivity of detection of mutant hetero- and homoduplex peaks at a single denaturation temperature compared to fragments generated using non-GC-clamped primers. Thus, when scanning tumor suppressor genes for germline mutation using dHPLC, the incorporation of appropriate GCclamped primers will likely increase the efficiency of mutation detection.

  2. NOTCH1 and FBXW7 mutations favor better outcome in pediatric South Indian T-cell acute lymphoblastic leukemia. (United States)

    Natarajan, Valliyammai; Bandapalli, Obul R; Rajkumar, Thangarajan; Sagar, Tenali Gnana; Karunakaran, Nirmala


    The NOTCH1 signaling pathway is essential for hematopoiesis and a critical regulatory step for T-cell proliferation and maturation. The E3 ubiquitin ligase FBXW7 controls NOTCH1 protein stability. Mutations in NOTCH1/FBXW7 activate NOTCH signaling and are of prognostic significance in patients with T-cell acute lymphoblastic leukemia (T-ALL). In this study we analyzed NOTCH1 and FBXW7 mutations in 50 South Indian T-ALL patients treated by a modified ALL BFM 95 regimen. The hot spot exons (HD-N, HD-C, TAD, and PEST) of NOTCH1 and exons 9 of the 10 of FBXW7 were polymerase chain reaction amplified and sequenced. In total, 20 of the 50 (40%) T-ALL patients revealed heterozygous mutations in the NOTCH1 domains, and a predominance of missense mutations in HD-N (70%) and PEST (15%) domains. FBXW7 mutations were detected in 5 of the 50 (10%) T-ALL patients. T-ALL patients with NOTCH1/FBXW7 mutations expressed higher protein level of NOTCH1 compared with patients without NOTCH1/FBXW7 mutations. Six of the mutations detected in NOTCH1 were not reported previously. When tested in a Dual Luciferase Renilla reporter assay some of these conferred increased NOTCH activity, suggesting that these are activating mutations. Importantly, 13 of the 20 (65%) NOTCH1/FBXW7-mutated T-ALL patients showed a good prednisone response (P=0.01) and a better clinical outcome compared with NOTCH1/FBXW7 nonmutated patients (P=0.03). These data suggest that NOTCH1/FBXW7 mutations are present in T-ALL patients from Southern India and may be useful biomarkers to predict prognosis in T-ALL.

  3. Ultraviolet spectroscopy of the hot interstellar medium (United States)

    Indebetouw, Remy

    I study the hot phase of the interstellar medium (ISM) in our Galaxy. The lithium-like ions of common metals are a powerful tracer of gas between the hot (106 K) and cooler (104 K) phases of the ISM, and are particularly sensitive to dynamical processes because gas at several 105 K cools very rapidly. These ions are usually produced in nonequilibrium processes such as shocks, evaporative interfaces, or rapidly cooling gas. There are two different approaches to studying the hot ISM via Li-like ions---analysis of the microphysics in a well-defined location in the Galaxy, and observation of a large part of the Galaxy searching for global trends. This thesis describes two experiments which follow these two approaches. Chapter 2 describes a sounding rocket experiment which could perform simultaneous ultra-high spectroscopy of C IV, N V, and O VI. In particular, it was to study the interface between the local bubble, a diffuse region of the Galaxy in which the Sun is located, and denser neighboring gas. I redesigned, integrated, and directed the flight of the payload, which in addition to its scientific goals was the first space demonstration of a low-order echelle spectrograph. Chapter 3 describes a survey of N V, O VI, and C IV in the Galactic halo using data from the Far Ultraviolet Spectroscopic Explorer and the Hubble Space Telescope. Searching for global trends, I found a general trend of higher ionization (lower N V/O VI column density ratio) at larger positive line-of-sight velocities. I modeled the various physical situations in which Li-like ions are produced, and found that the observed trend is qualitatively consistent with a cooling Galactic fountain flow which rises, cools, and recombines as it returns to the disk. The observed trend is also consistent with shocks moving towards the observer, and with observing through a conductive interface, looking from the hot gas into cooler gas. The latter geometry is consistent with the solar system being inside a hot

  4. Microbial pathways for the mobilization of mercury as Hg(O) in anoxic subsurface environments

    Energy Technology Data Exchange (ETDEWEB)

    Barkay, Tamar


    The goal of our project which was initiated in June 2005 is focused on the presence of merA in microbial communities of anoxic environments and the effect of anaerobic respiratory pathways on MR expression and activities. The following progress has been made to date: PCR primers were designed to span the known phylogenetic range of merA genes of Gram-negative bacteria. In control experiments, these primers successfully amplified a 288 bp region at the 3? end of previously characterized merA genes from Shewanella putrefaciens pMERPH, Acidithiobacillus ferrooxidans, Pseudomonas stutzeri pPB, Tn5041, Pseudomonas sp. K-62, and Serratia marcescens pDU1358.


    Human exposure to mercury vapor (Hg0) has been reported to result in sensory disturbances. Developmental exposure to toxicants may result in long-lasting changes in neural function. This manuscript describes the lack of effect of gestational exposure of Long Evans rats to Hg0 on ...

  6. Microbial pathways for the mobilization of mercury as Hg(O) in anoxic subsurface environments

    Energy Technology Data Exchange (ETDEWEB)

    Barkay, Tamar


    The goal of our project which was initiated in June 2005 is focused on the presence of merA in microbial communities of anoxic environments and the effect of anaerobic respiratory pathways on MR expression and activities. The following progress has been made to date: PCR primers were designed to span the known phylogenetic range of merA genes of Gram-negative bacteria. In control experiments, these primers successfully amplified a 288 bp region at the 3? end of previously characterized merA genes from Shewanella putrefaciens pMERPH, Acidithiobacillus ferrooxidans, Pseudomonas stutzeri pPB, Tn5041, Pseudomonas sp. K-62, and Serratia marcescens pDU1358.

  7. Rapid generation of hypomorphic mutations (United States)

    Arthur, Laura L.; Chung, Joyce J.; Jankirama, Preetam; Keefer, Kathryn M.; Kolotilin, Igor; Pavlovic-Djuranovic, Slavica; Chalker, Douglas L.; Grbic, Vojislava; Green, Rachel; Menassa, Rima; True, Heather L.; Skeath, James B.; Djuranovic, Sergej


    Hypomorphic mutations are a valuable tool for both genetic analysis of gene function and for synthetic biology applications. However, current methods to generate hypomorphic mutations are limited to a specific organism, change gene expression unpredictably, or depend on changes in spatial-temporal expression of the targeted gene. Here we present a simple and predictable method to generate hypomorphic mutations in model organisms by targeting translation elongation. Adding consecutive adenosine nucleotides, so-called polyA tracks, to the gene coding sequence of interest will decrease translation elongation efficiency, and in all tested cell cultures and model organisms, this decreases mRNA stability and protein expression. We show that protein expression is adjustable independent of promoter strength and can be further modulated by changing sequence features of the polyA tracks. These characteristics make this method highly predictable and tractable for generation of programmable allelic series with a range of expression levels. PMID:28106166

  8. Neutral Evolution of Mutational Robustness

    CERN Document Server

    Van Nimwegen, E; Huynen, M; Nimwegen, Erik van; Crutchfield, James P.; Huynen, Martijn


    We introduce and analyze a general model of a population evolving over a network of selectively neutral genotypes. We show that the population's limit distribution on the neutral network is solely determined by the network topology and given by the principal eigenvector of the network's adjacency matrix. Moreover, the average number of neutral mutant neighbors per individual is given by the matrix spectral radius. This quantifies the extent to which populations evolve mutational robustness: the insensitivity of the phenotype to mutations. Since the average neutrality is independent of evolutionary parameters---such as, mutation rate, population size, and selective advantage---one can infer global statistics of neutral network topology using simple population data available from {\\it in vitro} or {\\it in vivo} evolution. Populations evolving on neutral networks of RNA secondary structures show excellent agreement with our theoretical predictions.

  9. Origin and distribution of the BRCA2-8765delAG mutation in breast cancer

    Directory of Open Access Journals (Sweden)

    Baldinu Paola


    Full Text Available Abstract Background The BRCA2-8765delAG mutation was firstly described in breast cancer families from French-Canadian and Jewish-Yemenite populations; it was then reported as a founder mutation in Sardinian families. We evaluated both the prevalence of the BRCA2-8765delAG variant in Sardinia and the putative existence of a common ancestral origin through a haplotype analysis of breast cancer family members carrying such a mutation. Methods Eight polymorphic microsatellite markers (D13S1250, centromeric, to D13S267, telomeric spanning the BRCA2 gene locus were used for the haplotype analysis. Screening for the 8765delAG mutation was performed by PCR-based amplification of BRCA2-exon 20, followed by automated sequencing. Results Among families with high recurrence of breast cancer (≥ 3 cases in first-degree relatives, those from North Sardinia shared the same haplotype whereas the families from French Canadian and Jewish-Yemenite populations presented distinct genetic assets at the BRCA2 locus. Screening for the BRCA2-8765delAG variant among unselected and consecutively-collected breast cancer patients originating from the entire Sardinia revealed that such a mutation is present in the northern part of the island only [9/648 (1.4% among cases from North Sardinia versus 0/493 among cases from South Sardinia]. Conclusion The BRCA2-8765delAG has an independent origin in geographically and ethnically distinct populations, acting as a founder mutation in North but not in South Sardinia. Since BRCA2-8765delAG occurs within a triplet repeat sequence of AGAGAG, our study further confirmed the existence of a mutational hot-spot at this genomic position (additional genetic factors within each single population might be involved in generating such a mutation.

  10. Effect of mutation and genetic background on drug resistance in Mycobacterium tuberculosis. (United States)

    Fenner, Lukas; Egger, Matthias; Bodmer, Thomas; Altpeter, Ekkehardt; Zwahlen, Marcel; Jaton, Katia; Pfyffer, Gaby E; Borrell, Sonia; Dubuis, Olivier; Bruderer, Thomas; Siegrist, Hans H; Furrer, Hansjakob; Calmy, Alexandra; Fehr, Jan; Stalder, Jesica Mazza; Ninet, Béatrice; Böttger, Erik C; Gagneux, Sebastien


    Bacterial factors may contribute to the global emergence and spread of drug-resistant tuberculosis (TB). Only a few studies have reported on the interactions between different bacterial factors. We studied drug-resistant Mycobacterium tuberculosis isolates from a nationwide study conducted from 2000 to 2008 in Switzerland. We determined quantitative drug resistance levels of first-line drugs by using Bactec MGIT-960 and drug resistance genotypes by sequencing the hot-spot regions of the relevant genes. We determined recent transmission by molecular methods and collected clinical data. Overall, we analyzed 158 isolates that were resistant to isoniazid, rifampin, or ethambutol, 48 (30.4%) of which were multidrug resistant. Among 154 isoniazid-resistant strains, katG mutations were associated with high-level and inhA promoter mutations with low-level drug resistance. Only katG(S315T) (65.6% of all isoniazid-resistant strains) and inhA promoter -15C/T (22.7%) were found in molecular clusters. M. tuberculosis lineage 2 (includes Beijing genotype) was associated with any drug resistance (adjusted odds ratio [OR], 3.0; 95% confidence interval [CI], 1.7 to 5.6; P mutations (OR, 6.4; 95% CI, 2.0 to 20.7; P = 0.002). We found that the genetic strain background influences the level of isoniazid resistance conveyed by particular mutations (interaction tests of drug resistance mutations across all lineages; P tuberculosis drug resistance mutations were associated with various levels of drug resistance and transmission, and M. tuberculosis lineages were associated with particular drug resistance-conferring mutations and phenotypic drug resistance. Our study also supports a role for epistatic interactions between different drug resistance mutations and strain genetic backgrounds in M. tuberculosis drug resistance.

  11. Mutations of the phenylalanine hydroxylase gene in patients with phenylketonuria in Shanxi, China

    Directory of Open Access Journals (Sweden)

    Yong-An Zhou


    Full Text Available The variation in mutations in exons 3, 6, 7, 11 and 12 of the phenylalanine hydroxylase (PAH gene was investigated in 59 children with phenylketonuria (PKU and 100 normal children. Three single nucleotide polymorphisms were detected by sequence analysis. The mutational frequencies of cDNA 696, cDNA 735 and cDNA 1155 in patients were 96.2%, 76.1% and 7.6%, respectively, whereas in healthy children the corresponding frequencies were 97.0%, 77.3% and 8.3%. In addition, 81 mutations accounted for 61.0% of the mutant alleles. R111X, H64 > TfsX9 and S70 del accounted for 5.1%, 0.8% and 0.8% mutation of alleles in exon 3, whereas EX6-96A > G accounted for 10.2% mutation of alleles in exon 6. R243Q had the highest incidence in exon 7 (12.7%, followed by Ivs7 +2T>A (5.1% and T278I (2.5%. G247V, R252Q, L255S, R261Q and E280K accounted for 0.8% while Y356X and V399V accounted for 5.9% and 5.1%, respectively, in exon 11. R413P and A434D accounted for 5.9% and 2.5%, respectively, in exon 12. Seventy-two variant alleles accounted for the 16 mutations observed here. The mutation characteristics and distributions demonstrated that EX6-96A > G and R243Q were the hot regions for mutations in the PAH gene in Shanxi patients with PKU.

  12. A large Norwegian family with inherited malignant melanoma, multiple atypical nevi, and CDK4 mutation. (United States)

    Molven, Anders; Grimstvedt, Magne B; Steine, Solrun J; Harland, Mark; Avril, Marie-Françoise; Hayward, Nicholas K; Akslen, Lars A


    Mutations in two loci encoding cell-cycle-regulatory proteins have been shown to cause familial malignant melanoma. About 20% of melanoma-prone families bear a mutation in the CDKN2A locus, which encodes two unrelated proteins, p16INK4A and p14ARF. Mutations in the other locus, CDK4, are much rarer and have been linked to the disease in only three families worldwide. In the 1960s, a large Norwegian pedigree with multiple atypical nevi and malignant melanomas was identified. Subsequently, six generations and more than 100 family members were traced and 20 cases of melanoma verified. In this article, we report that CDK4 codon 24 is mutated from CGT to CAT (Arg24His) in this unusually large melanoma kindred. Intriguingly, one of the family members had ocular melanoma, but the CDK4 mutation could not be detected in archival tissue samples from this subject. Thus, the case of ocular melanoma in this family was sporadic, suggesting an etiology different from that of the skin tumors. The CDK4 mutation in the Norwegian family was identical to that in melanoma families in France, Australia, and England. Haplotype analysis using microsatellite markers flanking the CDK4 gene and single-nucleotide polymorphisms within the gene did not support the possibility that there was a common founder, but rather indicated at least two independent mutational events. All CDK4 melanoma families known to date have a substitution of amino acid 24. In addition to resulting from selection pressure, this observation may be explained by the CG dinucleotide of codon 24 representing a mutational hot spot in the CDK4 gene.

  13. Mutations in ANKH cause chondrocalcinosis. (United States)

    Pendleton, Adrian; Johnson, Michelle D; Hughes, Anne; Gurley, Kyle A; Ho, Andrew M; Doherty, Michael; Dixey, Josh; Gillet, Pierre; Loeuille, Damien; McGrath, Rodney; Reginato, Antonio; Shiang, Rita; Wright, Gary; Netter, Patrick; Williams, Charlene; Kingsley, David M


    Chondrocalcinosis (CC) is a common cause of joint pain and arthritis that is caused by the deposition of calcium-containing crystals within articular cartilage. Although most cases are sporadic, rare familial forms have been linked to human chromosomes 8 (CCAL1) or 5p (CCAL2) (Baldwin et al. 1995; Hughes et al. 1995; Andrew et al. 1999). Here, we show that two previously described families with CCAL2 have mutations in the human homolog of the mouse progressive ankylosis gene (ANKH). One of the human mutations results in the substitution of a highly conserved amino acid residue within a predicted transmembrane segment. The other creates a new ATG start site that adds four additional residues to the ANKH protein. Both mutations segregate completely with disease status and are not found in control subjects. In addition, 1 of 95 U.K. patients with sporadic CC showed a deletion of a single codon in the ANKH gene. The same change was found in a sister who had bilateral knee replacement for osteoarthritis. Each of the three human mutations was reconstructed in a full-length ANK expression construct previously shown to regulate pyrophosphate levels in cultured cells in vitro. All three of the human mutations showed significantly more activity than a previously described nonsense mutation that causes severe hydroxyapatite mineral deposition and widespread joint ankylosis in mice. These results suggest that small sequence changes in ANKH are one cause of CC and joint disease in humans. Increased ANK activity may explain the different types of crystals commonly deposited in human CCAL2 families and mutant mice and may provide a useful pharmacological target for treating some forms of human CC.

  14. Allele Specific Locked Nucleic Acid Quantitative PCR (ASLNAqPCR): An Accurate and Cost-Effective Assay to Diagnose and Quantify KRAS and BRAF Mutation (United States)

    Morandi, Luca; de Biase, Dario; Visani, Michela; Cesari, Valentina; De Maglio, Giovanna; Pizzolitto, Stefano; Pession, Annalisa; Tallini, Giovanni


    The use of tyrosine kinase inhibitors (TKIs) requires the testing for hot spot mutations of the molecular effectors downstream the membrane-bound tyrosine kinases since their wild type status is expected for response to TKI therapy. We report a novel assay that we have called Allele Specific Locked Nucleic Acid quantitative PCR (ASLNAqPCR). The assay uses LNA-modified allele specific primers and LNA-modified beacon probes to increase sensitivity, specificity and to accurately quantify mutations. We designed primers specific for codon 12/13 KRAS mutations and BRAF V600E, and validated the assay with 300 routine samples from a variety of sources, including cytology specimens. All were analyzed by ASLNAqPCR and Sanger sequencing. Discordant cases were pyrosequenced. ASLNAqPCR correctly identified BRAF and KRAS mutations in all discordant cases and all had a mutated/wild type DNA ratio below the analytical sensitivity of the Sanger method. ASLNAqPCR was 100% specific with greater accuracy, positive and negative predictive values compared with Sanger sequencing. The analytical sensitivity of ASLNAqPCR is 0.1%, allowing quantification of mutated DNA in small neoplastic cell clones. ASLNAqPCR can be performed in any laboratory with real-time PCR equipment, is very cost-effective and can easily be adapted to detect hot spot mutations in other oncogenes. PMID:22558339

  15. Mutations determining mitomycin resistance in Bacillus subtilis. (United States)

    Iyer, V N


    Iyer, V. N. (Microbiology Research Institute, Canada Department of Agriculture, Ottawa, Canada). Mutations determining mitomycin resistance in Bacillus subtilis. J. Bacteriol. 92:1663-1669. 1966.-The pattern of development of genetic resistance in Bacillus subtilis to mitomycin C was studied, and spontaneous single and multistep mutants were obtained. The transmission and expression of these mutations in sensitive strains proved possible by means of genetic transformation. The mutations were genetically studied in relation to a chromosomal mutation, mac-1, which confers resistance to the macrolide antibiotic erythromycin and which has been previously localized in the early-replicating segment of the B. subtilis chromosome. The results indicate that all of three primary mutations studied in this manner, as well as a secondary and tertiary mutation derived from one of the primary mutations, are clustered in this early-replicating segment. It appears that the secondary and tertiary mutations enhance the resistance conferred by the primary mutation, apparently without themselves conferring any resistance.

  16. Hot particulate removal and desulfurization results from the METC integrated gasification and hot gas cleanup facility

    Energy Technology Data Exchange (ETDEWEB)

    Rockey, J.M.


    The Morgantown Energy Technology Center (METC) is conducting experimental testing using a 10-inch diameter fluid-bed gasifier (FBG) and modular hot gas cleanup rig (MGCR) to develop advanced methods for removing contaminants in hot coal gasifier gas streams for commercial development of integrated gasification combined-cycle (IGCC) power systems. The program focus is on hot gas particulate removal and desulfurization technologies that match the temperatures and pressures of the gasifier, cleanup system, and power generator. The purpose of this poster is to present the program objectives and results of the work conducted in cooperation with industrial users and vendors to meet the vision for IGCC of reducing the capital cost per kilowatt to $1050 and increasing the plant efficiency to 52% by the year 2010.

  17. Isocitrate dehydrogenase mutations in gliomas. (United States)

    Waitkus, Matthew S; Diplas, Bill H; Yan, Hai


    Over the last decade, extraordinary progress has been made in elucidating the underlying genetic causes of gliomas. In 2008, our understanding of glioma genetics was revolutionized when mutations in isocitrate dehydrogenase 1 and 2 (IDH1/2) were identified in the vast majority of progressive gliomas and secondary glioblastomas (GBMs). IDH enzymes normally catalyze the decarboxylation of isocitrate to generate α-ketoglutarate (αKG), but recurrent mutations at Arg(132) of IDH1 and Arg(172) of IDH2 confer a neomorphic enzyme activity that catalyzes reduction of αKG into the putative oncometabolite D-2-hydroxyglutate (D2HG). D2HG inhibits αKG-dependent dioxygenases and is thought to create a cellular state permissive to malignant transformation by altering cellular epigenetics and blocking normal differentiation processes. Herein, we discuss the relevant literature on mechanistic studies of IDH1/2 mutations in gliomas, and we review the potential impact of IDH1/2 mutations on molecular classification and glioma therapy.

  18. LMNA mutations in progeroid syndromes. (United States)

    Huang, Shurong; Kennedy, Brian K; Oshima, Junko


    Segmental progeroid syndromes are disorders in which affected individuals. present various features that suggest accelerated ageing. The two best-known examples are Hutchinson-Gilford progeria syndrome (HGPS, 'Progeria of childhood') and Werner syndrome (WS, 'Progeria of the adult'). A novel, recurrent de novo mutation in the LMNA gene, responsible for the majority of HGPS cases, results in an in-frame deletion of 50 amino acids, including endoproteolytic sites required for processing of prelamin A to mature lamin A protein. Another mutation results in a 35 amino acid in-frame deletion with a milder HGPS phenotype. WRN, the gene responsible for the majority of WS cases, encodes a multifunctional nuclear protein with exonuclease and helicase activities and may participate in optimizing DNA repair/recombination. A subset of WS patients do not show mutations at the WRN locus (atypical WS), but show heterozygous amino acid substitutions in the heptad repeat region of lamin A. Structural analysis suggests that mutations in atypical WS may interfere with protein-protein interactions. When compared to WRN-mutant WS, LMNA-mutant atypical WS patients appear to show earlier onset and possibly more severe ageing-related symptoms.

  19. Mutations in RARS cause hypomyelination

    NARCIS (Netherlands)

    Wolf, Nicole I.; Salomons, Gajja S.; Rodenburg, Richard J.; Pouwels, Petra J. W.; Schieving, Jolanda H.; Derks, Terry G. J.; Fock, Johanna M.; Rump, Patrick; van Beek, Daphne M.; van der Knaap, Marjo S.; Waisfisz, Quinten


    Hypomyelinating disorders of the central nervous system are still a diagnostic challenge, as many patients remain without genetic diagnosis. Using magnetic resonance imaging (MRI) pattern recognition and whole exome sequencing, we could ascertain compound heterozygous mutations in RARS in 4 patients

  20. Mutations in RARS cause hypomyelination

    NARCIS (Netherlands)

    Wolf, Nicole I.; Salomons, Gajja S.; Rodenburg, Richard J.; Pouwels, Petra J. W.; Schieving, Jolanda H.; Derks, Terry G. J.; Fock, Johanna M.; Rump, Patrick; van Beek, Daphne M.; van der Knaap, Marjo S.; Waisfisz, Quinten

    Hypomyelinating disorders of the central nervous system are still a diagnostic challenge, as many patients remain without genetic diagnosis. Using magnetic resonance imaging (MRI) pattern recognition and whole exome sequencing, we could ascertain compound heterozygous mutations in RARS in 4 patients

  1. Mutations in RARS cause hypomyelination

    NARCIS (Netherlands)

    Wolf, N.I.; Salomons, G.S.; Rodenburg, R.J.; Pouwels, P.J.; Schieving, J.H.; Derks, T.G.; Fock, J.M.; Rump, P.; Beek, D.M. van; Knaap, M.S. van der; Waisfisz, Q.


    Hypomyelinating disorders of the central nervous system are still a diagnostic challenge, as many patients remain without genetic diagnosis. Using magnetic resonance imaging (MRI) pattern recognition and whole exome sequencing, we could ascertain compound heterozygous mutations in RARS in 4 patients

  2. An evaluation of lead concentrations in imported hot sauces. (United States)

    Berger Ritchie, Jennifer A; Gerstenberger, Shawn L


    In the last decade, the U.S. Food and Drug Administration (FDA) has issued several warnings and recalls for food products that exceed FDA standards for lead. Products containing chili peppers and salt were often suspected as sources of lead contamination, and included items such as candy that are routinely investigated. However, products such as hot sauces that contain similar ingredients have not been the focus of evaluations. This study quantified lead concentrations in imported hot sauces, evaluated product compliance to existing United States standards, and calculated potential dietary lead exposure for children using the Integrated Exposure Uptake Biokinetic Model. Finally, recommendations for reducing the risk of lead exposure from hot sauces are provided. Twenty-five (25) bottles of imported hot sauces manufactured in Mexico and South America were purchased in Clark County, Nevada. All hot sauces were analyzed for lead concentrations, pH, and leaded packaging. Hot sauces were analyzed by inductively coupled plasma mass spectrometry and packaging was analyzed using x-ray fluorescence technology. Four brands of hot sauces (16%) exceeded 0.1 ppm lead, the current FDA action level for lead in candy. Hot sauces with lead concentrations >0.1 ppm lead contained salt and were manufactured in Mexico. Subsequent analysis of additional lots of hot sauces exceeding 0.1 ppm lead revealed inconsistent lead concentrations between and within manufacturer lots. The lead concentrations of the plastic hot sauce lids ranged from below the limit of detection to 2,028 ppm lead. There was no association between lead concentrations in hot sauces and pepper type. These results indicate the need for more rigorous screening protocols for products imported from Mexico, the establishment of an applicable standard for hot sauce, and resources to allow for the enforcement of existing food safety policies. The data reported herein represent the first known investigation of lead

  3. [History of hot spring bath treatment in China]. (United States)

    Hao, Wanpeng; Wang, Xiaojun; Xiang, Yinghong; Gu Li, A Man; Li, Ming; Zhang, Xin


    As early as the 7th century B.C. (Western Zhou Dynasty), there is a recording as 'spring which contains sulfur could treat disease' on the Wentang Stele written by WANG Bao. Wenquan Fu written by ZHANG Heng in the Easten Han Dynasty also mentioned hot spring bath treatment. The distribution of hot springs in China has been summarized by LI Daoyuan in the Northern Wei Dynasty in his Shuijingzhu which recorded hot springs in 41 places and interpreted the definition of hot spring. Bencao Shiyi (by CHEN Cangqi, Tang Dynasty) discussed the formation of and indications for hot springs. HU Zai in the Song Dynasty pointed out distinguishing hot springs according to water quality in his book Yuyin Conghua. TANG Shenwei in the Song Dynasty noted in Jingshi Zhenglei Beiji Bencao that hot spring bath treatment should be combined with diet. Shiwu Bencao (Ming Dynasty) classified hot springs into sulfur springs, arsenicum springs, cinnabar springs, aluminite springs, etc. and pointed out their individual indications. Geologists did not start the work on distribution and water quality analysis of hot springs until the first half of the 20th century. There are 972 hot springs in Wenquan Jiyao (written by geologist ZHANG Hongzhao and published in 1956). In July 1982, the First National Geothermal Conference was held and it reported that there were more than 2600 hot springs in China. Since the second half of the 20th century, hot spring sanatoriums and rehabilitation centers have been established, which promoted the development of hot spring bath treatment.

  4. IDH mutations in acute myeloid leukemia. (United States)

    Rakheja, Dinesh; Konoplev, Sergej; Medeiros, L Jeffrey; Chen, Weina


    Acute myeloid leukemia is a heterogeneous group of diseases. Mutations of the isocitrate dehydrogenase (IDH) genes represent a novel class of point mutations in acute myeloid leukemia. These mutations prevent oxidative decarboxylation of isocitrate to α-ketoglutarate and confer novel enzymatic activity, facilitating the reduction of α-ketoglutarate to d-2-hydroxyglutarate, a putative oncometabolite. IDH1/IDH2 mutations are heterozygous, and their combined frequency is approximately 17% in unselected acute myeloid leukemia cases, 27% in cytogenetically normal acute myeloid leukemia cases, and up to 67% in acute myeloid leukemia cases with cuplike nuclei. These mutations are largely mutually exclusive. Despite many similarities of IDH1 and IDH2 mutations, it is possible that they represent distinct molecular or clinical subgroups of acute myeloid leukemia. All known mutations involve arginine (R), in codon 132 of IDH1 or codon 140 or 172 of IDH2. IDH1(R132) and IDH2(R140) mutations are frequently accompanied by normal cytogenetics and NPM1 mutation, whereas IDH2(R172) is frequently the only mutation detected in acute myeloid leukemia. There is increasing evidence that the prognostic impact of IDH1/2 mutations varies according to the specific mutation and also depends on the context of concurrent mutations of other genes. IDH1(R132) mutation may predict poor outcome in a subset of patients with molecular low-risk acute myeloid leukemia, whereas IDH2(R172) mutations confer a poor prognosis in patients with acute myeloid leukemia. Expression of IDH1/2 mutants induces an increase in global DNA hypermethylation and inhibits TET2-induced cytosine 5-hydroxymethylation, DNA demethylation. These data suggest that IDH1/2 mutations constitute a distinct mutational class in acute myeloid leukemia, which affects the epigenetic state, an important consideration for the development of therapeutic agents.

  5. Hot rolling of thick uranium molybdenum alloys (United States)

    DeMint, Amy L.; Gooch, Jack G.


    Disclosed herein are processes for hot rolling billets of uranium that have been alloyed with about ten weight percent molybdenum to produce cold-rollable sheets that are about one hundred mils thick. In certain embodiments, the billets have a thickness of about 7/8 inch or greater. Disclosed processes typically involve a rolling schedule that includes a light rolling pass and at least one medium rolling pass. Processes may also include reheating the rolling stock and using one or more heavy rolling passes, and may include an annealing step.

  6. Glueball dynamics in the hot plasma

    Energy Technology Data Exchange (ETDEWEB)

    Kochelev, Nikolai [Chinese Academy of Sciences, Institute of Modern Physics, Lanzhou (China); Joint Institute for Nuclear Research, Bogoliubov Laboratory of Theoretical Physics, Dubna, Moscow region (Russian Federation)


    We discuss the glueball contribution to the equation of state (EoS) of hot gluon matter below and above T{sub c}. We show that the strong variation of the masses of the scalar and pseudoscalar glueballs near T{sub c} is determining the thermodynamics of the SU(3) gauge theory. We provide arguments to justify that these glueballs become massless at T{sub G} ∼ 1.1 T{sub c}, a phenomenon which is crucial to understand the mysterious behavior of the trace anomaly found in lattice calculations. (orig.)

  7. Hot tensile tests of Inconel 718 (United States)


    The physical metallurgy of near-solidus integranular cracking in Inconel 718 welds was investigated. The data, although inconclusive, suggest at least two mechanisms which might explain intergranular cracking (microfissuring) in the heat-affected zone of several high temperature alloys. One theory is based on the separation of intergranular liquid while the other involves mechanical failure of solid ligaments surrounded by intergranular liquid. Both mechanisms concentrate strain in the grain boundaries resulting in low strain (1%) intergranular brittleness. The mechanisms reported might also pertain to the physical metallurgy of casting, powder metallurgy sintering and hot isostatic pressing.

  8. Hot Fuel Examination Facility/South

    Energy Technology Data Exchange (ETDEWEB)


    This document describes the potential environmental impacts associated with proposed modifications to the Hot Fuel Examination Facility/South (HFEF/S). The proposed action, to modify the existing HFEF/S at the Argonne National Laboratory-West (ANL-W) on the Idaho National Engineering Laboratory (INEL) in southeastern Idaho, would allow important aspects of the Integral Fast Reactor (IFR) concept, offering potential advantages in nuclear safety and economics, to be demonstrated. It would support fuel cycle experiments and would supply fresh fuel to the Experimental Breeder Reactor-II (EBR-II) at the INEL. 35 refs., 12 figs., 13 tabs.

  9. Hot Deformation Behavior of 2124 Al Alloy

    Institute of Scientific and Technical Information of China (English)

    S.Ramanathan; R.Karthikeyan; V.Deepak Kumar; G.Ganesan


    The mechanical behavior of 2124 Al alloy produced by powder metallurgy was investigated with compression test at different temperatures and strain rates. The tests were performed in the temperature range of 300℃~500℃ and at strain rates from 0.001 s-1 to 1.0 s-1. The compression flow curves exhibited an initial sharp increase with strain, followed by monotonous hardening. The maximum stress decreased with decreasing strain rate and increasing temperature. The hot deformation characteristics of the material were studied using processing maps. The domain of safety and unsafe regime were identified and validated through microstructural examination.

  10. Intelligent Control on Hot Strip Coiling Temperature

    Institute of Scientific and Technical Information of China (English)


    A new intelligent control scheme for hot strip coiling temperature is presented. In this scheme, the prediction model of finishing temperature and the presetting model of main cooling zone are establish based on BP neural network, the feed-forward open-loop control model of main cooling zone is constructed based on T-S fuzzy neural network, a new improved structure of T-S fuzzy neural network is developed, and the feedback close-loop control model of precision cooling zone is obtained based on fuzzy control. The effectiveness of the proposed scheme has been demonstrated by computer simulation with a satisfactory result.

  11. Double giant dipole resonance in hot nuclei

    Energy Technology Data Exchange (ETDEWEB)

    Cinausero, M.; Rizzi, V.; Viesti, G.; Fabris, D.; Lunardon, M.; Moretto, S.; Nebbia, G.; Pesente, S.; Barbui, M.; Fioretto, E.; Prete, G.; Bracco, A.; Camera, F.; Million, B.; Leoni, S.; Wieland, O.; Benzoni, G.; Brambilla, S.; Airoldi, A.; Maj, A.; Kmiecik, M


    Signals from Double Dipole Giant Resonances (DGDR) in hot nuclei have been searched in a {gamma}-{gamma} coincidence experiment using the HECTOR array at the Laboratori Nazionali di Legnaro. The experimental single {gamma}-ray spectrum and the projection of the {gamma}-{gamma} matrix have been compared with a standard Monte Carlo Statistical Model code including only the single GDR excitation. These calculations have been used as background to determine the extra-yield associated with the DGDR de-excitation. Results have been compared with a previous experiment confirming the presence of the DGDR excitation in fusion-evaporation reactions.

  12. When hot water freezes before cold

    CERN Document Server

    Katz, J I


    I suggest that the origin of the Mpemba effect (the freezing of hot water before cold) is freezing-point depression by solutes, either gaseous or solid, whose solubility decreases with increasing temperature so that they are removed when water is heated. They are concentrated ahead of the freezing front by zone refining in water that has not been heated, reduce the temperature of the freezing front, and thereby reduce the temperature gradient and heat flux, slowing the progress of the front. I present a simple calculation of this effect, and suggest experiments to test this hypothesis.

  13. Concerning thermal tides on hot Jupiters

    CERN Document Server

    Goodman, Jeremy


    By analogy with a mechanism proposed by Gold and Soter to explain the retrograde rotation of Venus, Arras and Socrates suggest that thermal tides may excite hot jovian exoplanets into nonsynchronous rotation, and perhaps also noncircular orbits. It is shown here that because of the absence of a solid surface above the convective core of a jovian planet, the coupling of the gravitational and thermal tides vanishes to zeroth order in the ratio of the atmospheric scale height to the planetary radius. At the next order, the effect probably has the sign opposite to that claimed by the latter authors, hence reinforcing synchronous and circular orbits.

  14. The "hot money" phenomenon in Brazil

    Directory of Open Access Journals (Sweden)

    Mylène Gaulard


    Full Text Available Because of its high interest rates, Brazil attracts more and more speculative capital flows, called "hot money", under the form of foreign loans, direct or portfolio investments. Actually, the country is directly involved in a carry-trade strategy that tends to appreciate the real, what penalizes the Brazilian exportations of manufactured products. Moreover, capital inflows are extremely volatile, and their departure, causing a fall in loans granted to the Brazilian private banks, could provoke a dangerous burst of the speculative bubble they have contributed to form in the Brazilian real estate sector.

  15. Going ballistic: Graphene hot electron transistors (United States)

    Vaziri, S.; Smith, A. D.; Östling, M.; Lupina, G.; Dabrowski, J.; Lippert, G.; Mehr, W.; Driussi, F.; Venica, S.; Di Lecce, V.; Gnudi, A.; König, M.; Ruhl, G.; Belete, M.; Lemme, M. C.


    This paper reviews the experimental and theoretical state of the art in ballistic hot electron transistors that utilize two-dimensional base contacts made from graphene, i.e. graphene base transistors (GBTs). Early performance predictions that indicated potential for THz operation still hold true today, even with improved models that take non-idealities into account. Experimental results clearly demonstrate the basic functionality, with on/off current switching over several orders of magnitude, but further developments are required to exploit the full potential of the GBT device family. In particular, interfaces between graphene and semiconductors or dielectrics are far from perfect and thus limit experimental device integrity, reliability and performance.

  16. Contribution to advanced hot wire wind sensing


    Kowalski, Lukasz


    La consulta íntegra de la tesi, inclos l'article no comunicat públicament per drets d'autor, es pot realitzar prèvia petició a l'Arxiu de la UPC The thermal anemometry is a method which allows to estimate wind magnitude be the mean of measuring heat transfer to the ambient in a forced convection process. For Earth's atmosphere condition, this method is typically applied to the hot wires made of temperature dependent electrical conductor, typically platinum or tungsten, which working with o...

  17. Hardware Trojan by Hot Carrier Injection

    CERN Document Server

    Shiyanovskii, Y; Papachristou, C; Weyer, D; Clay, W


    This paper discusses how hot carrier injection (HCI) can be exploited to create a trojan that will cause hardware failures. The trojan is produced not via additional logic circuitry but by controlled scenarios that maximize and accelerate the HCI effect in transistors. These scenarios range from manipulating the manufacturing process to varying the internal voltage distribution. This new type of trojan is difficult to test due to its gradual hardware degradation mechanism. This paper describes the HCI effect, detection techniques and discusses the possibility for maliciously induced HCI trojans.

  18. Control of dephasing in rotationally hot molecules (United States)

    Bartram, David; Ivanov, Misha


    We consider a rotationally hot diatomic molecule as an example of an open quantum system, where molecular vibrational wave packets are subject to dephasing due to rovibrational coupling. We report analytical and numerical results addressing whether the dephasing rate can be controlled by adjustment of the initial wave packet phases. It appears that over long time scales, phase-only control is not possible, but for earlier time scales the possibility of phase-only control of dephasing remains. In addition, we point out that the time dependence of the dephasing process depends significantly upon the degeneracy of the rotational environment states.

  19. Palaeoclimatology: evidence for hot early oceans? (United States)

    Shields, Graham A; Kasting, James F


    The oxygen isotopes in sedimentary cherts (siliceous sediments) have been used to argue that the Precambrian oceans were hot--with temperatures of up to 70 degrees C at 3.3 Gyr before present. Robert and Chaussidon measure silicon isotopes in cherts and arrive at a similar conclusion. We suggest here that both isotope trends may be caused by variations in seawater isotope composition, rather than in ocean temperatures. If so, then the climate of the early Earth may have been temperate, as it is today, and therefore more consistent with evidence for Precambrian glaciations and with constraints inferred from biological evolution.

  20. Stream instabilities in relativistically hot plasma

    CERN Document Server

    Shaisultanov, Rashid; Eichler, David


    The instabilities of relativistic ion beams in a relativistically hot electron background are derived for general propagation angles. It is shown that the Weibel instability in the direction perpendicular to the streaming direction is the fastest growing mode, and probably the first to appear, consistent with the aligned filaments that are seen in PIC simulations. Oblique, quasiperpendicular modes grow almost as fast, as the growth rate varies only moderately with angle, and they may distort or corrugate the filaments after the perpendicular mode saturates.

  1. Hot Air Drying of Green Table Olives

    Directory of Open Access Journals (Sweden)

    Sayit Sargin


    Full Text Available The characteristics of hot air-drying of green table olives (Domat variety by using a tray dryer were studied. Air temperature varied from 40 to 70 °C with an air velocity of 1 m/s. Drying rate curves were determined and quality of dried green olives was evaluated by instrumental analysis (bulk density, particle density, porosity, shrinkage, moisture content, water activity, colour value, protein content, oil content, peroxide value and acidity. Consumers’ acceptance test and microbiological analysis were also applied.

  2. Automated inspection of hot steel slabs (United States)

    Martin, Ronald J.


    The disclosure relates to a real time digital image enhancement system for performing the image enhancement segmentation processing required for a real time automated system for detecting and classifying surface imperfections in hot steel slabs. The system provides for simultaneous execution of edge detection processing and intensity threshold processing in parallel on the same image data produced by a sensor device such as a scanning camera. The results of each process are utilized to validate the results of the other process and a resulting image is generated that contains only corresponding segmentation that is produced by both processes.

  3. Disease-causing mutations in exon 11 of the medium-chain acyl-CoA dehydrogenase gene

    DEFF Research Database (Denmark)

    Andresen, B S; Jensen, T G; Bross, P


    spot. Here we describe the results from sequence analysis of exon 11 and part of the flanking introns from 36 compound heterozygous patients with MCAD deficiency. We have identified four previously unknown disease-causing mutations (M301T, S311R, R324X, and E359X) and two silent mutations in exon 11......Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is the most commonly recognized defect of the mitochondrial beta-oxidation in humans. It is a potentially fatal, autosomal recessive inherited defect. Most patients with MCAD deficiency are homozygous for a single disease-causing mutation (G985......), causing a change from lysine to glutamate at position 304 (K304E) in the mature MCAD. Only seven non-G985 mutations, all of which are rare, have been reported. Because the G985 mutation and three of the non-G985 mutations are located in exon 11, it has been suggested that this exon may be a mutational hot...

  4. Identification of APC gene mutations in Italian adenomatous polyposis coli patients by PCR-SSCP analysis

    Energy Technology Data Exchange (ETDEWEB)

    Varesco, L.; Gismondi, V.; James, R.; Casarino, L.; De Benedetti, L.; Bafico, A.; Allegretti, A.; Aste, H. (Istituto Nazionale per la Ricerca sul Cancro, Genoa (Italy)); Robertson, M.; Groden, J.; White, R. (Univ. of Utah, Salt Lake City (United States)); Grammatico, P.; De Sanctis, S.; Sciarra, A.; Del Porto, G. (Universita di Roma, Rome (Italy)); Bertario, L.; Sala, P.; Rossetti, C.; Illeni, M.T. (Istituto Nazionale Tumori, Milan (Italy)); Sassatelli, R.; Ponz de Leon, M. (Universita di Modena (Italy)); Biasco, G. (Universita di Bologna (Italy)); Ferrara, G.B. (Istituto Nazionale per la Ricerca sul Cancro, Genoa (Italy) Universita di Napoli, Naples (Italy))


    The APC gene is a putative human tumor-suppressor gene responsible for adenomatous polyposis coli (APC), an inherited, autosomal dominant predisposition to colon cancer. It is also implicated in the development of sporadic colorectal tumors. The characterization of APC gene mutations in APC patients is clinically important because DNA-based tests can be applied for presymptomatic diagnosis once a specific mutation has been identified in a family. Moreover, the identification of the spectrum of APC gene mutations in patients is of great interest in the study of the biological properties of the APC gene product. The authors analyzed the entire coding region of the APC gene by the PCR-single-strand conformation polymorphism method in 42 unrelated Italian APC patients. Mutations were found in 12 cases. These consist of small (5-14 bp) base-pair deletions leading to frameshifts; all are localized within exon 15. Two of these deletions, a 5-bp deletion at position 3183-3187 and a 5-bp deletion at position 3926-3930, are present in 3/42 and 7/42 cases of the series, respectively, indicating the presence of mutational hot spots at these two sites. 17 refs., 2 figs., 1 tab.

  5. Prevalence of mutations in APC, CTNNB1, and BRAF in Tunisian patients with sporadic colorectal cancer. (United States)

    Bougatef, Karim; Ouerhani, Slah; Moussa, Amel; Kourda, Nadia; Coulet, Florence; Colas, Chrystelle; Lahely, Yannick Blondeau; Najjar, Tawfik; Ben Jilani, Sarra; Benammar-Elgaaied, Amel; Soubrier, Florent; Marrakchi, Raja


    Sporadic colorectal tumorigenesis is caused by alterations in the Wnt (APC, CTNNB1) and Ras pathways. Our objective was to analyze the occurrence of these genetic alterations in relation to tumor and patient characteristics. The prevalence of somatic alteration in the hot-spot regions of the APC, BRAF, and CTNNB1 genes was investigated in 48 unselected and unrelated Tunisian patients with sporadic colorectal cancer, and the association between the molecular features at these genes in relation to tumor and patient characteristics (age at diagnosis, sex, tumor localization, stage, and differentiation) was analyzed. Loss of heterozygosity was observed at the APC locus in 52% of the analyzed tumors. 6 novel mutations were detected by polymerase chain reaction sequencing in the mutation cluster region of the APC gene. No mutations were observed in the CTNNB1 gene in any tumor, but 8% of tumors harbored mutation in the BRAF gene. Clinicopathological analyses showed an association between APC point mutations and the earliest occurrence of sporadic colorectal cancer. The findings confirm the heterogeneity of APC gene alteration and also reveal a particular profile of this pathology among Tunisian patients that confirms the epidemiological data for this country.

  6. Structure Based Thermostability Prediction Models for Protein Single Point Mutations with Machine Learning Tools. (United States)

    Jia, Lei; Yarlagadda, Ramya; Reed, Charles C


    Thermostability issue of protein point mutations is a common occurrence in protein engineering. An application which predicts the thermostability of mutants can be helpful for guiding decision making process in protein design via mutagenesis. An in silico point mutation scanning method is frequently used to find "hot spots" in proteins for focused mutagenesis. ProTherm ( is a public database that consists of thousands of protein mutants' experimentally measured thermostability. Two data sets based on two differently measured thermostability properties of protein single point mutations, namely the unfolding free energy change (ddG) and melting temperature change (dTm) were obtained from this database. Folding free energy change calculation from Rosetta, structural information of the point mutations as well as amino acid physical properties were obtained for building thermostability prediction models with informatics modeling tools. Five supervised machine learning methods (support vector machine, random forests, artificial neural network, naïve Bayes classifier, K nearest neighbor) and partial least squares regression are used for building the prediction models. Binary and ternary classifications as well as regression models were built and evaluated. Data set redundancy and balancing, the reverse mutations technique, feature selection, and comparison to other published methods were discussed. Rosetta calculated folding free energy change ranked as the most influential features in all prediction models. Other descriptors also made significant contributions to increasing the accuracy of the prediction models.

  7. Structure Based Thermostability Prediction Models for Protein Single Point Mutations with Machine Learning Tools.

    Directory of Open Access Journals (Sweden)

    Lei Jia

    Full Text Available Thermostability issue of protein point mutations is a common occurrence in protein engineering. An application which predicts the thermostability of mutants can be helpful for guiding decision making process in protein design via mutagenesis. An in silico point mutation scanning method is frequently used to find "hot spots" in proteins for focused mutagenesis. ProTherm ( is a public database that consists of thousands of protein mutants' experimentally measured thermostability. Two data sets based on two differently measured thermostability properties of protein single point mutations, namely the unfolding free energy change (ddG and melting temperature change (dTm were obtained from this database. Folding free energy change calculation from Rosetta, structural information of the point mutations as well as amino acid physical properties were obtained for building thermostability prediction models with informatics modeling tools. Five supervised machine learning methods (support vector machine, random forests, artificial neural network, naïve Bayes classifier, K nearest neighbor and partial least squares regression are used for building the prediction models. Binary and ternary classifications as well as regression models were built and evaluated. Data set redundancy and balancing, the reverse mutations technique, feature selection, and comparison to other published methods were discussed. Rosetta calculated folding free energy change ranked as the most influential features in all prediction models. Other descriptors also made significant contributions to increasing the accuracy of the prediction models.

  8. Development of plant mutation techniques using ion beam

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Si Yong; Park, In Sook; Song, Hi Sup; Kim, Dong Sub; Kim, Soo Yeon


    It has been reported that ion beam with high liner energy transfer (LET) show relative high biological effectiveness (RBE) and more effective for induced plant mutation than low LET radiation i.e., X-rays, gamma rays and electrons. This study was conducted to induce mutation of in vitro cultured orchid and Chrysanthemum using proton beam of the MC-50 cyclotron (50 MeV) at the Korea Institute of Radiological and Medical Science. In vitro cultured stems of chrysanthemum(cv. Migok) and protocom-like bodies(PLBs) of Dendrobium orchid (cv. Kingianum) placed in the plastic petridish (5.5cm in diameter) with agar medium were irradiated by the proton beam with various dose ranges of 10, 25, 50, 100 Gy under the condition of 5nA beam current. Those irradiated plants were transferred to subculture media and then investigated growth characteristics. Shoot growth of chrysanthemum and orchid was decreased by increase of irradiation dose. In particular, new shoot formation was hardly founded over 50Gy in chrysanthemum and 100 Gy in orchid. Some leaf mutants were observed at the 25 Gy and 50 Gy irradiated PLBs of the orchid. The dry seeds of hot pepper, rapeseed, rice and perilla also were irradiated with proton beam of MC-50 cyclotron and then measured germination rate and early growth of M1 plants compared with gamma ray irradiation.

  9. Mutations induced by dacarbazine activated with cytochrome P-450. (United States)

    Mudipalli, A; Nadadur, S S; Maccubbin, A E; Gurtoo, H L


    The mutagenicity of the antitumor drug dacarbazine (DTIC) is due to alkylation of cellular DNA by metabolites resulting from the metabolism of this drug by the mixed function oxidase system. In the present study, we used an in vitro shuttle vector assay to study the base and sequence specificity of mutagenesis by DTIC. The shuttle vector plasmid pSP189 was treated with DTIC (1-2.5 mM) in vitro in a reconstituted cytochrome P-450 system at 37 degrees C for either 30 or 60 min. SupF tRNA gene insert contained in the plasmid was sequenced after replication of the drug-treated plasmid in human Ad 293 cells followed by amplification in indicator bacteria. Mutagenesis of DTIC in this system was dependent upon the presence of the cytochrome P-450 reconstituted system and NADPH. Mutations induced by DTIC included single base substitutions (35%), single base deletions (30.5%), single base insertions (19.4%) and large deletions (13.8%). Among the substitutions, transversions and transitions were in the ratio of 1:0.7. Base pairs 108 and 127 in the SupF tRNA of the pSP189 were identified as mutational hot spots.

  10. [Neurofibromatosis type 1. Splicing mutation detected by MLPA and DNA sequencing in Argentina]. (United States)

    Laurito, Sergio; Di Pierri, José; Roqué, María


    Neurofibromatosis type 1 (NF1) is a dominant autosomic genetic disorder, with a birth incidence of 1 in 2500-3000. Diagnosis is difficult because of the size of gene NF1 that has few hot-spots sites, the absence of a clear genotype-phenotype relation, and a heterogeneous clinical manifestation. A NF1 suspected case from Jujuy province was analyzed by multiplex ligation-dependent probe amplification (MLPA). Mestizo female teenage (Amerindian/European), with a maxilar osteoma, lumbar lordosis, cutaneous neurofibromas and café au lait spots. MLPA detected an alteration in exon 13 of the NF1 gene. By sequencing of exon 13, a missense mutation (NM_000267.3:c.1466A>G) was found which introduces an aberrant splicing site and is registered as pathogenic in the clinical variants database of NCBI. As far as we are aware, this is the first report of a NF1 mutation in mestizo population of Northwest Argentina. 1466A>G has been described before in patients of European origin, suggesting that the affected site could be a hot-spot site of the gene. For countries as Argentina, with limited availability of molecular diagnostic methods, we propose a diagnosis algorithm by starting the mutational analysis of NF1 with MLPA. This methodology is relatively simple and of low cost, avoiding to send samples abroad for genetic analyses.

  11. Age-dependent germline mosaicism of the most common noonan syndrome mutation shows the signature of germline selection. (United States)

    Yoon, Song-Ro; Choi, Soo-Kung; Eboreime, Jordan; Gelb, Bruce D; Calabrese, Peter; Arnheim, Norman


    Noonan syndrome (NS) is among the most common Mendelian genetic diseases (∼1/2,000 live births). Most cases (50%-84%) are sporadic, and new mutations are virtually always paternally derived. More than 47 different sites of NS de novo missense mutations are known in the PTPN11 gene that codes for the protein tyrosine phosphatase SHP-2. Surprisingly, many of these mutations are recurrent with nucleotide substitution rates substantially greater than the genome average; the most common mutation, c.922A>G, is at least 2,400 times greater. We examined the spatial distribution of the c.922A>G mutation in testes from 15 unaffected men and found that the mutations were not uniformly distributed across each testis as would be expected for a mutation hot spot but were highly clustered and showed an age-dependent germline mosaicism. Computational modeling that used different stem cell division schemes confirmed that the data were inconsistent with hypermutation, but consistent with germline selection: mutated spermatogonial stem cells gained an advantage that allowed them to increase in frequency. SHP-2 interacts with the transcriptional activator STAT3. Given STAT3's function in mouse spermatogonial stem cells, we suggest that this interaction might explain the mutant's selective advantage by means of repression of stem cell differentiation signals. Repression of STAT3 activity by cyclin D1 might also play a previously unrecognized role in providing a germline-selective advantage to spermatogonia for the recurrent mutations in the receptor tyrosine kinases that cause Apert syndrome and MEN2B. Looking at recurrent mutations driven by germline selection in different gene families can help highlight common causal signaling pathways.

  12. Tilting mutation of Brauer tree algebras

    CERN Document Server

    Aihara, T


    We define tilting mutations of symmetric algebras as the endomorphism algebras of Okuyama-Rickard complexes. For Brauer tree algebras, we give an explicit description of the change of Brauer trees under mutation.

  13. Identifying driver mutations in sequenced cancer genomes

    DEFF Research Database (Denmark)

    Raphael, Benjamin J; Dobson, Jason R; Oesper, Layla


    High-throughput DNA sequencing is revolutionizing the study of cancer and enabling the measurement of the somatic mutations that drive cancer development. However, the resulting sequencing datasets are large and complex, obscuring the clinically important mutations in a background of errors, noise......, and random mutations. Here, we review computational approaches to identify somatic mutations in cancer genome sequences and to distinguish the driver mutations that are responsible for cancer from random, passenger mutations. First, we describe approaches to detect somatic mutations from high-throughput DNA...... sequencing data, particularly for tumor samples that comprise heterogeneous populations of cells. Next, we review computational approaches that aim to predict driver mutations according to their frequency of occurrence in a cohort of samples, or according to their predicted functional impact on protein...

  14. Hot dry rock geothermal potential of Roosevelt Hot Springs area: review of data and recommendations

    Energy Technology Data Exchange (ETDEWEB)

    East, J.


    The Roosevelt Hot Springs area in west-central Utah possesses several features indicating potential for hot dry rock (HDR) geothermal development. The area is characterized by extensional tectonics and a high regional heat flow of greater than 105 mW/m/sup 2/. The presence of silicic volcanic rocks as young as 0.5 to 0.8 Myr and totaling 14 km/sup 3/ in volume indicates underlying magma reservoirs may be the heat source for the thermal anomaly. Several hot dry wells have been drilled on the periphery of the geothermal field. Information obtained on three of these deep wells shows that they have thermal gradients of 55 to 60/sup 0/C/km and bottom in impermeable Tertiary granitic and Precambrian gneissic units. The Tertiary granite is the preferred HDR reservoir rock because Precambrian gneissic rocks possess a well-developed banded foliation, making fracture control over the reservoir more difficult. Based on a fairly conservative estimate of 160 km/sup 2/ for the thermal anomaly present at Roosevelt Hot Springs, the area designated favorable for HDR geothermal exploration may be on the order of seven times or more than the hydrogeothermal area currently under development.

  15. Variability in Hot Carbon-Dominated Atmosphere (hot DQ) White Dwarfs: Rapid Rotation?

    CERN Document Server

    Williams, Kurtis A; Winget, D E; Falcon, Ross E; Bierwagon, Michael


    Hot white dwarfs with carbon-dominated atmospheres (hot DQs) are a cryptic class of white dwarfs. In addition to their deficiency of hydrogen and helium, most of these stars are highly magnetic, and a large fraction vary in luminosity. This variability has been ascribed to nonradial pulsations, but increasing data call this explanation into question. We present studies of short-term variability in seven hot DQ white dwarfs. Three (SDSS J1426+5752, SDSS J2200-0741, and SDSS J2348-0942) were known to be variable. Their photometric modulations are coherent over at least two years, and we find no evidence for variability at frequencies that are not harmonics. We present the first time-series photometry for three additional hot DQs (SDSS J0236-0734, SDSS J1402+3818, and SDSS J1615+4543); none are observed to vary, but the signal-to-noise is low. Finally, we present high speed photometry for SDSS J0005-1002, known to exhibit a 2.1 d photometric variation; we do not observe any short-term variability. Monoperiodicit...

  16. Formation and X-ray emission from Hot Bubbles in Planetary Nebulae. I. Hot Bubble formation

    CERN Document Server

    Toalá, J A


    We carry out high resolution two-dimensional radiation-hydrodynamic numerical simulations to study the formation and evolution of hot bubbles inside planetary nebulae (PNe). We take into account the evolution of the stellar parameters, wind velocity and mass-loss rate from the final thermal pulses during the asymptotic giant branch (AGB) through to the post-AGB stage for a range of initial stellar masses. The instabilities that form at the interface between the hot bubble and the swept-up AGB wind shell lead to hydrodynamical interactions, photoevaporation flows and opacity variations. We explore the effects of hydrodynamical mixing combined with thermal conduction at this interface on the dynamics, photoionization, and emissivity of our models. We find that even models without thermal conduction mix significant amounts of mass into the hot bubble. When thermal conduction is not included, hot gas can leak through the gaps between clumps and filaments in the broken swept-up AGB shell and this depressurises the...

  17. Organic Molecules in the Galactic Center. Hot Core Chemistry without Hot Cores

    CERN Document Server

    Requena-Torres, M A; Martín, S; Martín-Pintado, J; Rodr'iguez-Fern'andez, N J; Rodríguez-Franco, A


    To study the origin of the large abundances of complex organic molecules in the Galactic center (GC), we have carried out a systematic study of the complex organic molecules CH3OH, C2H5OH, (CH3)2O, HCOOCH3, HCOOH, CH3COOH, H2CO and CS toward 40 GC molecular clouds. Using the LTE approximation, we have derived the physical properties of GC molecular clouds and the abundances of the complex molecules.The CH3OH abundance between clouds varies by nearly two orders of magnitude from 2.4x10^{-8} to 1.1x10^{-6}. The abundance of the other complex organic molecules relative to that of CH3OH is basically independent of the CH3OH abundance, with variations of only a factor of 4-8. The abundances of complex organic molecules in the GC are compared with those measured in hot cores and hot corinos, in which these complex molecules are also abundant. We find that both the abundance and the abundance ratios of the complex molecules relative to CH3OH in hot cores are similar to that found in the GC clouds. However, hot corin...

  18. Hot spots and hot moments in riparian zones: Potential for improved water quality management (United States)

    Philippe Vidon; Craig Allan; Douglas Burns; Tim P. Duval; Noel Gurwick; Shreeram Inamdar; Richard Lowrance; Judy Okay; Durelle Scott; Stephen Sebestyen


    Biogeochemical and hydrological processes in riparian zones regulate contaminant movement to receiving waters and often mitigate the impact of upland sources of contaminants on water quality. These heterogeneous processes have recently been conceptualized as "hot spots and moments" of retention, degradation, or production. Nevertheless, studies investigating...

  19. Orion KL: The hot core that is not a "Hot Core"

    CERN Document Server

    Zapata, Luis A; Menten, Karl M


    We present sensitive high angular resolution submillimeter and millimeter observations of torsionally/vibrationally highly excited lines of the CH$_3$OH, HC$_3$N, SO$_2$, and CH$_3$CN molecules and of the continuum emission at 870 and 1300 $\\mu$m from the Orion KL region, made with the Submillimeter Array (SMA). These observations plus recent SMA CO J=3-2 and J=2-1 imaging of the explosive flow originating in this region, which is related to the non-hierarchical disintegration of a massive young stellar system, suggest that the molecular Orion "Hot Core" is a pre-existing density enhancement heated from the outside by the explosive event -- unlike in other hot cores we do not find any self-luminous submillimeter, radio or infrared source embedded in the hot molecular gas. Indeed, we do not observe filamentary CO flow structures or "fingers" in the shadow of the hot core pointing away from the explosion center. The low-excitation CH$_3$CN emission shows the typical molecular heart-shaped structure, traditional...

  20. Effect of hot acid hydrolysis and hot chlorine dioxide stage on bleaching effluent biodegradability. (United States)

    Gomes, C M; Colodette, J L; Delantonio, N R N; Mounteer, A H; Silva, C M


    The hot acid hydrolysis followed by chlorine dioxide (A/D*) and hot chlorine dioxide (D*) technologies have proven very useful for bleaching of eucalyptus kraft pulp. Although the characteristics and biodegradability of effluents from conventional chlorine dioxide bleaching are well known, such information is not yet available for effluents derived from hot acid hydrolysis and hot chorine dioxide bleaching. This study discusses the characteristics and biodegradability of such effluents. Combined whole effluents from the complete sequences DEpD, D*EpD, A/D*EpD and ADEpD, and from the pre-bleaching sequences DEp, D*Ep, A/D*Ep and ADEp were characterized by quantifying their colour, AOX and organic load (BOD, COD, TOC). These effluents were also evaluated for their treatability by simulation of an activated sludge system. It was concluded that treatment in the laboratory sequencing batch reactor was efficient for removal of COD, BOD and TOC of all effluents. However, colour increased after biological treatment, with the greatest increase found for the effluent produced using the AD technology. Biological treatment was less efficient at removing AOX of effluents from the sequences with D*, A/D* and AD as the first stages, when compared to the reference D stage; there was evidence of the lower treatability of these organochlorine compounds from these sequences.

  1. Hot stamping advanced manufacturing technology of lightweight car body

    CERN Document Server

    Hu, Ping; He, Bin


    This book summarizes the advanced manufacturing technology of original innovations in hot stamping of lightweight car body. A detailed description of the technical system and basic knowledge of sheet metal forming is given, which helps readers quickly understand the relevant knowledge in the field. Emphasis has been placed on the independently developed hot stamping process and equipment, which help describe the theoretical and experimental research on key problems involving stress field, thermal field and phase transformation field in hot stamping process. Also, a description of the formability at elevated temperature and the numerical simulation algorithms for high strength steel hot stamping is given in combination with the experiments. Finally, the book presents some application cases of hot stamping technology such as the lightweight car body design using hot stamping components and gradient hardness components, and the cooling design of the stamping tool. This book is intended for researchers, engineers...

  2. Meteorology of Jupiter's Equatorial Hot Spots and Plumes from Cassini

    CERN Document Server

    Choi, David S; Vasavada, Ashwin R; Simon-Miller, Amy A


    We present an updated analysis of Jupiter's equatorial meteorology from Cassini observations. For two months preceding the spacecraft's closest approach, the Imaging Science Subsystem (ISS) onboard regularly imaged the atmosphere. We created time-lapse movies from this period in order to analyze the dynamics of equatorial hot spots and their interactions with adjacent latitudes. Hot spots are quasi-stable, rectangular dark areas on visible-wavelength images, with defined eastern edges that sharply contrast with surrounding clouds, but diffuse western edges serving as nebulous boundaries with adjacent equatorial plumes. Hot spots exhibit significant variations in size and shape over timescales of days and weeks. Some of these changes correspond with passing vortex systems from adjacent latitudes interacting with hot spots. Strong anticyclonic gyres present to the south and southeast of the dark areas appear to circulate into hot spots. Impressive, bright white plumes occupy spaces in between hot spots. Compact...

  3. Inactivating CUX1 mutations promote tumorigenesis



    A major challenge for cancer genetics is to determine which low frequency somatic mutations are drivers of tumorigenesis. Here we interrogate the genomes of 7,651 diverse human cancers to identify novel drivers and find inactivating mutations in the homeodomain transcription factor CUX1 (cut-like homeobox 1) in ~1-5% of tumors. Meta-analysis of CUX1 mutational status in 2,519 cases of myeloid malignancies reveals disruptive mutations associated with poor survival, highlighting the clinical si...

  4. Elliptical instability in hot Jupiter systems

    CERN Document Server

    Cébron, David; Gal, Patrice Le; Moutou, Claire; Leconte, J; Sauret, Alban


    Several studies have already considered the influence of tides on the evolution of systems composed of a star and a close-in companion to tentatively explain different observations such as the spin-up of some stars with hot Jupiters, the radius anomaly of short orbital period planets and the synchronization or quasi-synchronization of the stellar spin in some extreme cases. However, the nature of the mechanism responsible for the tidal dissipation in such systems remains uncertain. In this paper, we claim that the so-called elliptical instability may play a major role in these systems, explaining some systematic features present in the observations. This hydrodynamic instability, arising in rotating flows with elliptical streamlines, is suspected to be present in both planet and star of such systems, which are elliptically deformed by tides. The presence and the influence of the elliptical instability in gaseous bodies, such as stars or hot Jupiters, are most of the time neglected. In this paper, using numeri...

  5. Considerations for choosing HOT lane delineation techniques

    Energy Technology Data Exchange (ETDEWEB)

    Hlavacek, I.; Vitek, M. [Texas Univ., Austin, TX (United States); Carrizales, J.; Machemehl, R. [Texas Dept. of Transportation, Austin, TX (Canada)


    Transportation agencies are looking for ways to move more vehicles with fewer tax dollars. One innovative technique is managed lanes. These are special purpose lanes on highways with controlled access to allow manipulation of service parameters. An example of a managed facility, high-occupancy toll (HOT) lanes are gaining support as a tool for encouraging higher vehicle occupancy while dealing with many of the shortcomings of high-occupancy vehicle (HOV) lanes. Because managed lanes, including HOT lanes, are controlled access facilities and must be separated from general purpose lanes, one of the principle issues is the type of delineation technique that should be used. Though the specifics of delineating a managed lane are numerous, and allow for a wide variety of possibilities, most delineation techniques used can be categorized into three broad types: concrete barriers, buffer separation, and plastic posts. This paper presented the advantages, disadvantages, required and desirable conditions for implementation of each type. The project involved assembling an expert panel to gather collective knowledge of factors and to share experiences and make recommendations regarding delineation device implementation. Panel recommendations and secondary source information were presented as conceptual guidance regarding managed lane delineation measures. Buffer type delineators were found to be least costly in terms of both initial and maintenance costs, and concrete barriers provide the best means of controlling access so they are the best means of guaranteeing toll collection from all users. 11 refs.

  6. METC CFD simulations of hot gas filtration

    Energy Technology Data Exchange (ETDEWEB)

    O`Brien, T.J.


    Computational Fluid Dynamic (CFD) simulations of the fluid/particle flow in several hot gas filtration vessels will be presented. These simulations have been useful in designing filtration vessels and in diagnosing problems with filter operation. The simulations were performed using the commercial code FLUENT and the METC-developed code MFIX. Simulations of the initial configuration of the Karhula facility indicated that the dirty gas flow over the filter assemblage was very non-uniform. The force of the dirty gas inlet flow was inducing a large circulation pattern that caused flow around the candles to be in opposite directions on opposite sides of the vessel. By introducing a system of baffles, a more uniform flow pattern was developed. This modification may have contributed to the success of the project. Several simulations of configurations proposed by Industrial Filter and Pump were performed, varying the position of the inlet. A detailed resolution of the geometry of the candles allowed determination of the flow between the individual candles. Recent simulations in support of the METC/CeraMem Cooperative Research and Development Agreement have analyzed the flow in the vessel during the cleaning back-pulse. Visualization of experiments at the CeraMem cold-flow facility provided confidence in the use of CFD. Extensive simulations were then performed to assist in the design of the hot test facility being built by Ahlstrom/Pyropower. These tests are intended to demonstrate the CeraMem technology.

  7. Hot and turbulent gas in clusters (United States)

    Schmidt, W.; Engels, J. F.; Niemeyer, J. C.; Almgren, A. S.


    The gas in galaxy clusters is heated by shock compression through accretion (outer shocks) and mergers (inner shocks). These processes additionally produce turbulence. To analyse the relation between the thermal and turbulent energies of the gas under the influence of non-adiabatic processes, we performed numerical simulations of cosmic structure formation in a box of 152 Mpc comoving size with radiative cooling, UV background, and a subgrid scale model for numerically unresolved turbulence. By smoothing the gas velocities with an adaptive Kalman filter, we are able to estimate bulk flows towards cluster cores. This enables us to infer the velocity dispersion associated with the turbulent fluctuation relative to the bulk flow. For haloes with masses above 1013 M⊙, we find that the turbulent velocity dispersions averaged over the warm-hot intergalactic medium (WHIM) and the intracluster medium (ICM) are approximately given by powers of the mean gas temperatures with exponents around 0.5, corresponding to a roughly linear relation between turbulent and thermal energies and transonic Mach numbers. However, turbulence is only weakly correlated with the halo mass. Since the power-law relation is stiffer for the WHIM, the turbulent Mach number tends to increase with the mean temperature of the WHIM. This can be attributed to enhanced turbulence production relative to dissipation in particularly hot and turbulent clusters.

  8. The Origin of Retrograde Hot Jupiters (United States)

    Naoz, Smadar; Farr, W.; Lithwick, Y.; Rasio, F.; Teyssandier, J.


    The search for extra-solar planets has led to the surprising discovery of many Jupiter-like planets in very close proximity to their host star, the so-called ``hot Jupiters'' (HJ). Even more surprisingly, many of these HJs have orbits that are eccentric or highly inclined with respect to the equator of the star, and some (about 25%) even orbiting counter to the spin direction of the star. This poses a unique challenge to all planet formation models. We show that secular interactions between Jupiter-like planet and another perturber in the system can easily produce retrograde HJ orbits. We show that in the frame of work of secular hierarchical triple system (the so-called Kozai mechanism) the inner orbit's angular momentum component parallel to the total angular momentum (i.e., the z-component of the inner orbit angular momentum) need not be constant. In fact, it can even change sign, leading to a retrograde orbit. A brief excursion to very high eccentricity during the chaotic evolution of the inner orbit allows planet-star tidal interactions to rapidly circularize that orbit, decoupling the planets and forming a retrograde hot Jupiter. We estimate the relative frequencies of retrograde orbits and counter to the stellar spin orbits using Monte Carlo simulations, and find that the they are consistent with the observations. The high observed incidence of planets orbiting counter to the stellar spin direction may suggest that planet--planet secular interactions are an important part of their dynamical history.

  9. The long hot summer of the tokamak

    CERN Document Server

    Kendl, Alexander


    What have the probability for fine weather in summer and the possibility for a future use of nuclear fusion as a practically unlimited and clean energy source got in common? The answer is in the particular nature underlying both physical systems: both the atmosphere and hot magnetized fusion plasmas are determined by similar processes of structure formation in quasi-two-dimensional periodic nonlinear dynamical systems. Self-organization of waves and vortices on small scales in both cases leads to large-scale flows, which are, depending on conditions, either stable for a long time - or can break apart intermittently and expel large vortex structures. In the case of earth's atmosphere, a potential stabilization of the polar jet stream over northern Europe by warming in early summer leads to a high probability for stable hot midsummer weather in central Europe. The efficient utilization of nuclear fusion in a power plant also depends if a stabilization of such zonal flows ("H mode") may be sustained by heating o...

  10. Ethylene oxide and Acetaldehyde in hot cores

    CERN Document Server

    Occhiogrosso, A; Herbst, E; Viti, S; Ward, M D; Price, S D; Brown, W A


    [Abridged] Ethylene oxide and its isomer acetaldehyde are important complex organic molecules because of their potential role in the formation of amino acids. Despite the fact that acetaldehyde is ubiquitous in the interstellar medium, ethylene oxide has not yet been detected in cold sources. We aim to understand the chemistry of the formation and loss of ethylene oxide in hot and cold interstellar objects (i) by including in a revised gas-grain network some recent experimental results on grain surfaces and (ii) by comparison with the chemical behaviour of its isomer, acetaldehyde. We test the code for the case of a hot core. The model allows us to predict the gaseous and solid ethylene oxide abundances during a cooling-down phase prior to star formation and during the subsequent warm-up phase. We can therefore predict at what temperatures ethylene oxide forms on grain surfaces and at what temperature it starts to desorb into the gas phase. The model reproduces the observed gaseous abundances of ethylene oxid...

  11. Hot Dog and Butterfly, Nereidum Montes (United States)


    Some of the pictures returned from Mars by the Mars Orbiter Camera (MOC) onboard the Mars Global Surveyor (MGS) spacecraft show features that--at a glance--resemble familiar, non-geological objects on Earth. For example, the picture above at the left shows several low, relatively flat-topped hills (mesas) on the floor of a broad valley among the mountains of the Nereidum Montes region, northeast of Argyre Planitia. One of the mesas seen here looks like half of a butterfly (upper subframe on right). Another hill looks something like a snail or a hot dog wrapped and baked in a croissant roll (lower subframe on right). These mesas were formed by natural processes and are most likely the eroded remnants of a formerly more extensive layer of bedrock. In the frame on the left, illumination is from the upper left and the scene covers an area 2.7 km (1.7 miles) wide by 6.8 km (4.2 miles) high. The 'butterfly' is about 800 meters (875 yards) in length and the 'hot dog' is about 1 km (0.62 miles) long. Malin Space Science Systems and the California Institute of Technology built the MOC using spare hardware from the Mars Observer mission. MSSS operates the camera from its facilities in San Diego, CA. The Jet Propulsion Laboratory's Mars Surveyor Operations Project operates the Mars Global Surveyor spacecraft with its industrial partner, Lockheed Martin Astronautics, from facilities in Pasadena, CA and Denver, CO.

  12. Sugar cane bagasse prehydrolysis using hot water

    Directory of Open Access Journals (Sweden)

    D. Abril


    Full Text Available Results are presented on the hot water prehydrolysis of sugar cane bagasse for obtaining ethanol by fermentation. The experimental study consisted of the determination of the effect of temperature and time of prehydrolysis on the extraction of hemicelluloses, with the objective of selecting the best operating conditions that lead to increased yield of extraction with a low formation of inhibitors. The study, carried out in a pilot plant scale rotational digester, using a 3² experimental design at temperatures of 150-190ºC and times of 60-90 min, showed that it is possible to perform the hot water prehydrolysis process between 180-190ºC in times of 60-82 min, yielding concentrations of xylose > 35 g/L, furfural < 2.5 g/L, phenols from soluble lignin < 1.5 g/L, and concentrations < 3.0 g/L of hemicelluloses in the cellolignin residue. These parameters of temperature and prehydrolysis time could be used for the study of the later hydrolysis and fermentation stages of ethanol production from sugar cane bagasse.

  13. SMA millimeter observations of Hot Molecular Cores

    CERN Document Server

    Hernández-Hernández, Vicente; Kurtz, Stan; Garay, Guido


    We present Submillimeter Array observations, in the 1.3 mm continuum and the CH_3CN(12-11) line of 17 hot molecular cores associated with young high-mass stars. The angular resolution of the observations ranges from 1".0 to 4".0. The continuum observations reveal large (>3500 AU) dusty structures with gas masses from 7 to 375 Msun, that probably surround multiple young stars. The CH_3CN line emission is detected toward all the molecular cores at least up to the K=6-component and is mostly associated with the emission peaks of the dusty objects. We used the multiple K-components of the CH_3CN and both the rotational diagram method and a simultaneous synthetic LTE model with the XCLASS program to estimate the temperatures and column densities of the cores. For all sources, we obtained reasonable fits from XCLASS by using a model that combines two components: an extended and warm envelope, and a compact hot core of molecular gas, suggesting internal heating by recently formed massive stars. The rotational temper...

  14. Hot workability of five commercial magnesium alloys

    Energy Technology Data Exchange (ETDEWEB)

    Mwembela, A.; McQueen, H.J.; Herba, E.; Sauerborn, M. (Concordia Univ., Montreal, PQ (Canada))


    The mechanical forming of Mg alloys can provide products with superior mechanical properties and in certain shapes increased productivity and economy. To assist in optimizing such manufacturing, the hot working characteristics of AZ91, AZ63, AZ31, AZ31-Mn and ZK60 were determined by hot torsion in the range 180-450 C and 0.1-1.0 s[sup -1]. The dependence of the flow stress on temperature and strain rate was determined for the purpose of process modeling for force calculation. A sinh-Arrhenius relationship fitted the data successfully with an activation energy of about 140 kJ/mol similar to that in creep. Similarly, the dependence of the fracture strain was measured to develop temperature-strain-rate maps of the forming limits. In the optical microstructures, twinning was gradually displaced by diffuse slip and dynamic recrystallization as the temperature rose above 300 C in association with the marked rise in ductility. In the observations by TEM with rising temperature, the twins became less sharp and regular as a dynamically recovered substructure more uniformly filled both them and the matrix grains. The variations in dislocation density which led to serrated grain boundaries also gave rise to the dynamic nucleation in their vicinity. the refined grain structure and retained substructure endow the product with strength and toughness. (orig.)

  15. The Hot Inner Disk of FU Ori

    CERN Document Server

    Zhu, Zhaohuan; Calvet, Nuria; Hernandez, Jesus; Muzerolle, James; Tannirkulam, Ajay-Kumar


    We have constructed a detailed radiative transfer disk model which reproduces the main features of the spectrum of the outbursting young stellar object FU Orionis from ~ 4000 angstrom, to ~ 8 micron. Using an estimated visual extinction Av~1.5, a steady disk model with a central star mass ~0.3 Msun and a mass accretion rate ~ 2e-4 Msun/yr, we can reproduce the spectral energy distribution of FU Ori quite well. With the mid-infrared spectrum obtained by the Infrared Spectrograph (IRS) on board the Spitzer Space Telescope, we estimate that the outer radius of the hot, rapidly accreting inner disk is ~ 1 AU using disk models truncated at this outer radius. Inclusion of radiation from a cooler irradiated outer disk might reduce the outer limit of the hot inner disk to ~ 0.5 AU. In either case, the radius is inconsistent with a pure thermal instability model for the outburst. Our radiative transfer model implies that the central disk temperature Tc > 1000 K out to ~ 0.5 - 1 AU, suggesting that the magnetorotationa...

  16. A novel fully automated molecular diagnostic system (AMDS for colorectal cancer mutation detection.

    Directory of Open Access Journals (Sweden)

    Shiro Kitano

    Full Text Available BACKGROUND: KRAS, BRAF and PIK3CA mutations are frequently observed in colorectal cancer (CRC. In particular, KRAS mutations are strong predictors for clinical outcomes of EGFR-targeted treatments such as cetuximab and panitumumab in metastatic colorectal cancer (mCRC. For mutation analysis, the current methods are time-consuming, and not readily available to all oncologists and pathologists. We have developed a novel, simple, sensitive and fully automated molecular diagnostic system (AMDS for point of care testing (POCT. Here we report the results of a comparison study between AMDS and direct sequencing (DS in the detection of KRAS, BRAF and PI3KCA somatic mutations. METHODOLOGY/PRINCIPAL FINDING: DNA was extracted from a slice of either frozen (n = 89 or formalin-fixed and paraffin-embedded (FFPE CRC tissue (n = 70, and then used for mutation analysis by AMDS and DS. All mutations (n = 41 among frozen and 27 among FFPE samples detected by DS were also successfully (100% detected by the AMDS. However, 8 frozen and 6 FFPE samples detected as wild-type in the DS analysis were shown as mutants in the AMDS analysis. By cloning-sequencing assays, these discordant samples were confirmed as true mutants. One sample had simultaneous "hot spot" mutations of KRAS and PIK3CA, and cloning assay comfirmed that E542K and E545K were not on the same allele. Genotyping call rates for DS were 100.0% (89/89 and 74.3% (52/70 in frozen and FFPE samples, respectively, for the first attempt; whereas that of AMDS was 100.0% for both sample sets. For automated DNA extraction and mutation detection by AMDS, frozen tissues (n = 41 were successfully detected all mutations within 70 minutes. CONCLUSIONS/SIGNIFICANCE: AMDS has superior sensitivity and accuracy over DS, and is much easier to execute than conventional labor intensive manual mutation analysis. AMDS has great potential for POCT equipment for mutation analysis.

  17. Two novel mutations of CLCN7 gene in Chinese families with autosomal dominant osteopetrosis (type II). (United States)

    Zheng, Hui; Shao, Chong; Zheng, Yan; He, Jin-Wei; Fu, Wen-Zhen; Wang, Chun; Zhang, Zhen-Lin


    Autosomal dominant osteopetrosis type II (ADO-II) is a heritable bone disorder characterized by osteosclerosis, predominantly involving the spine (vertebral end-plate thickening, or rugger-jersey spine), the pelvis ("bone-within-bone" structures) and the skull base. Chloride channel 7 (CLCN7) has been reported to be the causative gene. In this study, we aimed to identify the pathogenic mutation in four Chinese families with ADO-II. All 25 exons of the CLCN7 gene, including the exon-intron boundaries, were amplified and sequenced directly in four probands from the Chinese families with ADO-II. The mutation site was then identified in other family members and 250 healthy controls. In family 1, a known missense mutation c.296A>G in exon 4 of CLCN7 was identified in the proband, resulting in a tyrosine (UAU) to cysteine (UGU) substitution at p.99 (Y99C); the mutation was also identified in his affected father. In family 2, a novel missense mutation c.865G>C in exon 10 was identified in the proband, resulting in a valine (GUC) to leucine (CUC) substitution at p.289 (V289L); the mutation was also identified in her healthy mother and sister. In family 3, a novel missense mutation c.1625C>T in exon 17 of CLCN7 was identified in the proband, resulting in an alanine (GCG) to valine (GUG) substitution at p.542 (A542V); the mutation was also identified in her father. In family 4, a hot spot, R767W (c.2299C>T, CGG>TGG), in exon 24 was found in the proband which once again proved the susceptibility of the site or the similar genetic background in different races. Moreover, two novel mutations, V289L and A542V, occurred at a highly conserved position, found by a comparison of the protein sequences from eight vertebrates, and were predicted to have a pathogenic effect by PolyPhen-2 software, which showed "probably damaging" with a score of approximately 1. These mutation sites were not identified in 250 healthy controls. Our present findings suggest that the novel missense

  18. A study in Polish patients with cardiomyopathy emphasizes pathogenicity of phospholamban (PLN) mutations at amino acid position 9 and low penetrance of heterozygous null PLN mutations. (United States)

    Truszkowska, Grażyna T; Bilińska, Zofia T; Kosińska, Joanna; Śleszycka, Justyna; Rydzanicz, Małgorzata; Sobieszczańska-Małek, Małgorzata; Franaszczyk, Maria; Bilińska, Maria; Stawiński, Piotr; Michalak, Ewa; Małek, Łukasz A; Chmielewski, Przemysław; Foss-Nieradko, Bogna; Machnicki, Marcin M; Stokłosa, Tomasz; Ponińska, Joanna; Szumowski, Łukasz; Grzybowski, Jacek; Piwoński, Jerzy; Drygas, Wojciech; Zieliński, Tomasz; Płoski, Rafał


    In humans mutations in the PLN gene, encoding phospholamban - a regulator of sarcoplasmic reticulum calcium ATPase (SERCA), cause cardiomyopathy with prevalence depending on the population. Our purpose was to identify PLN mutations in Polish cardiomyopathy patients. We studied 161 unrelated subjects referred for genetic testing for cardiomyopathies: 135 with dilated cardiomyopathy, 22 with hypertrophic cardiomyopathy and 4 with other cardiomyopathies. In 23 subjects multiple genes were sequenced by next generation sequencing and in all subjects PLN exons were analyzed by Sanger sequencing. Control group included 200 healthy subjects matched with patients for ethnicity, sex and age. Large deletions/insertions were screened by real time polymerase chain reaction. We detected three different heterozygous mutations in the PLN gene: a novel null c.9_10insA:(p.Val4Serfs*15) variant and two missense variants: c.25C > T:(p.Arg9Cys) and c.26G > T:(p.Arg9Leu). The (p.Val4Serfs*15) variant occurred in the patient with Wolff-Parkinson-White syndrome in whom the diagnosis of cardiomyopathy was not confirmed and his mother who had concentric left ventricular remodeling but normal left ventricular mass and function. We did not detect large deletions/insertions in PLN in cohort studied. In Poland, similar to most populations, PLN mutations rarely cause cardiomyopathy. The 9(th) PLN residue is apparently a mutation hot spot whereas a single dose of c.9_10insA, and likely other null PLN mutations, cause the disease only with low penetrance or are not pathogenic.

  19. A novel Lys141Thr mutation in small heat shock protein 22 (HSPB8) gene in Charcot-Marie-Tooth disease type 2L. (United States)

    Nakhro, Khriezhanuo; Park, Jin-Mo; Kim, Ye Jin; Yoon, Bo Ram; Yoo, Jeong Hyun; Koo, Heasoo; Choi, Byung-Ok; Chung, Ki Wha


    Charcot-Marie-Tooth disease (CMT) is a group of clinically and genetically heterogeneous peripheral neuropathies. HSPB8 gene encodes heat shock protein 22 (HSP22) which belongs to the superfamily of small stress induced proteins. Mutations in HSPB8 are implicated to CMT2L and distal hereditary motor neuropathy 2A (dHMN2A). All three reported HSPB8 mutations are interestingly located in the Lys141 residue. In the present study, we examined a Korean axonal CMT patient who presented distal limb atrophy, sensory loss, areflexia, and axonal loss of large myelinated fibers. Whole exome sequencing identified a novel missense mutation c.422A>C (p.Lys141Thr) in HSPB8 as the underlying cause of the CMT2 patient. The mutation was regarded as a de novo case because both unaffected parents have no such mutation. The patient with HSPB8 mutation is the first case in Koreans. Clinical heterogeneities have been revealed in patients with Lys141 mutation; the present patient revealed similar phenotype of CMT2L. In addition, the lower limb MRI revealed a similarity between our HSPB8 and HSPB1 patients. It seems that the Lys141 site in the alpha-crystallin domain of HSPB8 is regarded as a mutational hot spot for peripheral neuropathy development, and mutations even in the same codon can exhibit different CMT phenotypes. Copyright © 2013 Elsevier B.V. All rights reserved.

  20. A novel solar hot plate for cooking

    Energy Technology Data Exchange (ETDEWEB)

    Rincon Mejia, Eduardo A; Osorio Jaramillo, Fidel A [Facultad de Ingenieria, UAEMex, Toluca, Edo. (Mexico)


    In Mexico and other developing countries, the use of firewood as combustible for cooking has contributed to deforestation and desertification of large zones. This is due to the lack of alternative combustibles for the poor inhabitants of the countryside and remote areas. In this paper, a new solar hot plate, intended for contributing to solve this problem, is presented. It can be used for cooking not only a great variety of prehispanic and traditional meals, like tortillas, fried meat and vegetables, but also hot cakes, bacon, eggs, steaks and fries. The hot plate solar cooker, called Tolocatzin, consists of a horizontal metallic plate, which is heated from both of its top and bottom surfaces by concentrated sun light from multicompound concentrator based on nonimaging optics, and built with nine ordinary plane glass-silvered, and two curved aluminum mirrors, so it can be manufactured easily in a small factory or at home. For an acceptance angle of 15 Celsius degrees, which allows the concentration of sun light without sun-tracking for about one hour, it can reach temperatures up to 240 Celsius degrees in a few minutes. This temperature is high enough for cooking almost all fried or grilled meals. The design was optimized using ray-trace procedures. The operational experience with early prototypes has shown that the Tolocatzin solar hot plate does an excellent cooking job and could really be massively used in sunny countries. [Spanish] En Mexico y otros paises en desarrollo, el uso de la madera como combustible para cocinar ha contribuido a la deforestacion y desertificacion de grandes zonas. Esto es debido a la falta de combustibles alternativos por parte de los habitantes pobres del campo y de areas remotas. En este articulo se presenta una nueva placa solar que tiene el proposito de contribuir a resolver este problema. Puede ser usada para cocinar no solamente una gran variedad de comidas prehispanicas y tradicionales, como tortillas, carne frita y verduras sino


    Energy Technology Data Exchange (ETDEWEB)

    Williams, Kurtis A.; Bierwagen, Michael [Department of Physics and Astrophysics, Texas A and M University-Commerce, P.O. Box 3011, Commerce, TX, 75429 (United States); Montgomery, M. H.; Winget, D. E.; Falcon, Ross E., E-mail: [Department of Astronomy, University of Texas, 1 University Station C1400, Austin, TX, 78712 (United States)


    Hot white dwarfs (WDs) with carbon-dominated atmospheres (hot DQs) are a cryptic class of WDs. In addition to their deficiency of hydrogen and helium, most of these stars are highly magnetic, and a large fraction vary in luminosity. This variability has been ascribed to nonradial pulsations, but increasing data call this explanation into question. We present studies of short-term variability in seven hot DQ WDs. Three (SDSS J1426+5752, SDSS J2200−0741, and SDSS J2348−0942) were known to be variable. Their photometric modulations are coherent over at least two years, and we find no evidence for variability at frequencies that are not harmonics. We present the first time-series photometry for three additional hot DQs (SDSS J0236−0734, SDSS J1402+3818, and SDSS J1615+4543); none are observed to vary, but the signal-to-noise is low. Finally, we present high speed photometry for SDSS J0005−1002, known to exhibit a 2.1-day photometric variation; we do not observe any short-term variability. Monoperiodicity is rare among pulsating WDs, so we contemplate whether the photometric variability is due to rotation rather than pulsations; similar hypotheses have been raised by other researchers. If the variability is due to rotation, then hot DQ WDs as a class contain many rapid rotators. Given the lack of companions to these stars, the origin of any fast rotation is unclear—both massive progenitor stars and double degenerate merger remnants are possibilities. We end with suggestions of future work that would best clarify the nature of these rare, intriguing objects.

  2. Design package for solar domestic hot water system

    Energy Technology Data Exchange (ETDEWEB)



    Information used to evaluate the initial design of the Elcam, Inc., Solar Domestic Hot Water System is presented. Included are such items as the system performance specification, detailed design drawings and other information. Elcam, Inc., has developed two solar heated prototype hot water systems and two heat exchangers. The hot water systems consist of the following subsystems: collector, storage, control, transport, auxiliary energy, and government-furnished Site Data Acquisition. The two systems are installed at Tempe, Arizona, and San Diego, California.

  3. Quality of scintillating fibres after hot bump shrinking

    CERN Document Server

    Rodrigues Cavalcante, Ana Barbara; Joram, Christian


    Shrinking the diameter of fibre bumps by a hot drawing tool requires to run the fibre through the hot tool over its full length, bearing the risk of a degradation of the fibre performance. In this study we demonstrated that the hot bump shrinking method has no visible effect on the optical attenuation length, the light yield following ionising radiation, the diameter, the mechanical stability and the integrity of the cladding. For the latter, even a small positive impact was observed.

  4. Dynamic Multiaxial Response of a Hot-Pressed Aluminum Nitride (United States)


    Dynamic Multiaxial Response of a Hot-Pressed Aluminum Nitride by Guangli Hu, C. Q. Chen, K. T. Ramesh, and J. W. McCauley ARL-RP-0487...Laboratory Aberdeen Proving Ground, MD 21005-5066 ARL-RP-0487 June 2014 Dynamic Multiaxial Response of a Hot-Pressed Aluminum Nitride...3. DATES COVERED (From - To) January 2010–January 2013 4. TITLE AND SUBTITLE Dynamic Multiaxial Response of a Hot-Pressed Aluminum Nitride 5a

  5. Design package for solar domestic hot water system

    Energy Technology Data Exchange (ETDEWEB)



    Information used to evaluate the initial design of the Elcam, Inc., Solar Domestic Hot Water System is presented. Included are such items as the system performance specification, detailed design drawings and other information. Elcam, Inc., has developed two solar heated prototype hot water systems and two heat exchangers. The hot water systems consist of the following subsystems: collector, storage, control, transport, auxiliary energy, and government-furnished Site Data Acquisition. The two systems are installed at Tempe, Arizona, and San Diego, California.

  6. Plastome Mutations and Recombination Events in Barley Chloroplast Mutator Seedlings. (United States)

    Landau, Alejandra; Lencina, Franco; Pacheco, María G; Prina, Alberto R


    The barley chloroplast mutator (cpm) is an allele of a nuclear gene that when homozygous induces several types of cytoplasmically inherited chlorophyll deficiencies. In this work, a plastome Targeting Induced Local Lesions in Genomes (TILLING) strategy based on mismatch digestion was used on families that carried the cpm genotype through many generations. Extensive scanning of 33 plastome genes and a few intergenic regions was conducted. Numerous polymorphisms were detected on both genic and intergenic regions. The detected polymorphisms can be accounted for by at least 61 independent mutational events. The vast majority of the polymorphisms originated in substitutions and small indels (insertions/deletions) in microsatellites. The rpl23 and the rps16 genes were the most polymorphic. Interestingly, the variation observed in the rpl23 gene consisted of several combinations of 5 different one nucleotide polymorphisms. Besides, 4 large indels that have direct repeats at both ends were also observed, which appear to be originated from recombinational events. The cpm mutation spectrum suggests that the CPM gene product is probably involved in plastome mismatch repair. The numerous subtle molecular changes that were localized in a wide range of plastome sites show the cpm as a valuable source of plastome variability for plant research and/or plant breeding. Moreover, the cpm mutant appears to be an interesting experimental material for investigating the mechanisms responsible for maintaining the stability of plant organelle DNA.

  7. Biological evolution model with conditional mutation rates (United States)

    Saakian, David B.; Ghazaryan, Makar; Bratus, Alexander; Hu, Chin-Kun


    We consider an evolution model, in which the mutation rates depend on the structure of population: the mutation rates from lower populated sequences to higher populated sequences are reduced. We have applied the Hamilton-Jacobi equation method to solve the model and calculate the mean fitness. We have found that the modulated mutation rates, directed to increase the mean fitness.

  8. Novel and recurrent tyrosine aminotransferase gene mutations in tyrosinemia type II. (United States)

    Hühn, R; Stoermer, H; Klingele, B; Bausch, E; Fois, A; Farnetani, M; Di Rocco, M; Boué, J; Kirk, J M; Coleman, R; Scherer, G


    Tyrosinemia type II (Richner-Hanhart syndrome, RHS) is a disorder of autosomal recessive inheritance characterized by keratitis, palmoplantar hyperkeratosis, mental retardation, and elevated blood tyrosine levels. The disease results from deficiency in hepatic tyrosine aminotransferase (TAT). We have previously described one deletion and six different point mutations in four RHS patients. We have now analyzed the TAT genes in a further seven unrelated RHS families from Italy, France, the United Kingdom, and the United States. We have established PCR conditions for the amplification of all twelve TAT exons and have screened the products for mutations by direct sequence analysis or by first performing single-strand conformation polymorphism analysis. We have thus identified the presumably pathological mutations in eight RHS alleles, including two nonsense mutations (R57X, E411X) and four amino acid substitutions (R119W, L201R, R433Q, R433W). Only the R57X mutation, which was found in one Scottish and two Italian families, has been previously reported in another Italian family. Haplotype analysis indicates that this mutation, which involves a CpG dinucleotide hot spot, has a common origin in the three Italian families but arose independently in the Scottish family. Two polymorphisms have also been detected, viz., a protein polymorphism, P15S, and a silent substitution S103S (TCG-->TCA). Expression of R433Q and R433W demonstrate reduced activity of the mutant proteins. In all, twelve different TAT gene mutations have now been identified in tyrosinemia type II.

  9. Co-mutation of p53, K-ras genes and accumulation of p53 protein and its correlation to clinicopathological features in rectal cancer

    Institute of Scientific and Technical Information of China (English)

    Zhi-Zhong Pan; De-Sen Wan; Gong Chen; Li-Ren Li; Zhen-Hai Lu; Bi-Jun Huang


    AIM: To determine the accuracy of p53 gene mutations predicted by overexpression of p53 protein immunohistochemically,and to investigate the co-mutation of p53 and K-rasgenes in rectal cancer and its effect on promoting malignant biologic behaviors of tumors.METHODS: Ninety-seven specimens of rectal cancer were surgically resected in our hospital from August 1996 to October 1997. The hot mutation areas of p53 gene (in exons 5-8) and K-ras gene (in codon 5/12 and 13) were detected with polymerase chain reaction-single strand conformation polymorphism (PCR-SSCP), and overexpression of p53 protein was detected with immunohistochemistry (IHC) in the 97 specimens of rectal cancer. Correlation between gene mutations and tumor clinicopathologic factors was studied, and survival analysis was penfomed as well.RESULTS: There were 36 cases of p53 gene mutations in 61 p53 protein positive cases, and 21 cases of p53 gene non-mutation in 36 p53 protein negative cases respectively.The coincidence rate of p53 gene mutation by IHC method with PCR-SSCP method was 58.8% (57/97). The mutation rate of p53 gene was 52.6% (51/97), while K-ras gene mutation was observed in codons 12 and 13 in 61 cases with a mutation rate of 62.9% (61/97). Single gene mutation of p53 or K-raswas found in 32 cases. Both p53 and K-ras gene mutation were found in 48 cases. Statistical analysis showed that p53 and K-rasgene mutations were not related to the clinicopathologic factors, including tumor size, gross tumor type, histological classification, differentiation, invasion to intestinal veins, lymphatics and nerves, invasive depth to wall, lymph node metastasis, and Dukes' stages (P>0.05).The survival in patients with no gene mutation, single gene mutation and both gene mutations were similar (P>0.05).CONCLUSION: IHC has a certain false positive and false negative rate in detecting p53 gene mutations. Malignant biological behaviours of rectal cancer are not enhanced by p53 and K-rasgene mutations. Co-mutation

  10. Hot ductility of medium carbon steel with vanadium

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Chang-Hoon, E-mail: [Korea Institute of Materials Science, Changwon, Gyeongnam 642-831 (Korea, Republic of); Park, Jun-Young [Korea Institute of Materials Science, Changwon, Gyeongnam 642-831 (Korea, Republic of); Chung, JunHo; Park, Dae-Bum; Jang, Jin-Young; Huh, Sungyul; Ju Kim, Sung [R& D Center, Hyundai Steel Company, Dangjin, Chungnam 343-823 (Korea, Republic of); Kang, Jun-Yun; Moon, Joonoh; Lee, Tae-Ho [Korea Institute of Materials Science, Changwon, Gyeongnam 642-831 (Korea, Republic of)


    Hot ductility of medium carbon steel containing 0.52 wt% of carbon and 0.11 wt% of vanadium was investigated using a hot tensile test performed up to fracture. The hot ductility was evaluated by measuring the reduction of area of the fractured specimens, which were strained at a variety of test temperatures in a range of 600–1100 °C at a strain rate of 2×10{sup −3}/s. The hot ductility was excellent in a temperature range of 950–1100 °C, followed by a decrease of the hot ductility below 950 °C. The hot ductility continued to drop as the temperature was lowered to 600 °C. The loss of hot ductility in a temperature range of 800–950 °C, which is above the Ae{sub 3} temperature, was due to V(C,N) precipitation at austenite grain boundaries. The further decline of hot ductility between 700 °C and 750 °C resulted from the transformation of ferrite films decorating austenite grain boundaries. The hot ductility continued to decrease at 650 °C or less, owing to ferrite films and the pearlite matrix, which is harder than ferrite. The pearlite was transformed from austenite due to relatively high carbon content.

  11. Hot complaint intelligent classification based on text mining

    Directory of Open Access Journals (Sweden)

    XIA Haifeng


    Full Text Available The complaint recognizer system plays an important role in making sure the correct classification of the hot complaint,improving the service quantity of telecommunications industry.The customers’ complaint in telecommunications industry has its special particularity which should be done in limited time,which cause the error in classification of hot complaint.The paper presents a model of complaint hot intelligent classification based on text mining,which can classify the hot complaint in the correct level of the complaint navigation.The examples show that the model can be efficient to classify the text of the complaint.

  12. Investigation of the Hot Plasticity of Duplex Stainless Steel

    Institute of Scientific and Technical Information of China (English)

    LIN Gang; ZHANG Zhi-xia; SONG Hong-wei; TONG Jun; ZHOU Can-dong


    Hot plasticity of a nitrogen alloyed 25Cr-7Ni-4 Mo duplex stainless steel was investigated.The results indicate that thc main factors affecting the hot plasticity of duplex stainless steel are listed as follows:coalescent force of phase interface,microstructure,and the phase ratio and difference between the mechanicsl propertms of ferrite and austenite.The heat treatment and sulphur contents have a notable effect on the hot plasticity.The reasonable heat treatrnents and the irlcreased interfacial coalescent force will effectively enhance the hot plasticity of duplex stainless steel.

  13. Harvesting the loss: surface plasmon-based hot electron photodetection

    Directory of Open Access Journals (Sweden)

    Li Wei


    Full Text Available Although the nonradiative decay of surface plasmons was once thought to be only a parasitic process within the plasmonic and metamaterial communities, hot carriers generated from nonradiative plasmon decay offer new opportunities for harnessing absorption loss. Hot carriers can be harnessed for applications ranging from chemical catalysis, photothermal heating, photovoltaics, and photodetection. Here, we present a review on the recent developments concerning photodetection based on hot electrons. The basic principles and recent progress on hot electron photodetectors are summarized. The challenges and potential future directions are also discussed.

  14. Numerical simulation of hot stamping of side impact beam

    Institute of Scientific and Technical Information of China (English)

    Guo Yihui; Ma Mingtu; Fang Gang; Song Leifeng; Liu Qiang; Wang Xiaona; Zhou Dianwu


    Ls-DYNA software is adopted to conduct research of numerical simulation on hot stamping of side impact beam to calculate the temperature field distribution, stress field distribution, forming limit diagram (FLD) figure, etc. in the course of hot stamping so as to predict and analyze the formability of parts. ProCAST software is employed to conduct research of numerical simulation on solid quenching course concerning hot stamping to calculate temperature field distri- bution of tools and component of muhiple stamping cycles. The results obtained from numerical simulation can provide significant reference value to hot stamping part design, formability predication and tools cooling system design.

  15. TCOF1 mutation database: novel mutation in the alternatively spliced exon 6A and update in mutation nomenclature. (United States)

    Splendore, Alessandra; Fanganiello, Roberto D; Masotti, Cibele; Morganti, Lucas S C; Passos-Bueno, M Rita


    Recently, a novel exon was described in TCOF1 that, although alternatively spliced, is included in the major protein isoform. In addition, most published mutations in this gene do not conform to current mutation nomenclature guidelines. Given these observations, we developed an online database of TCOF1 mutations in which all the reported mutations are renamed according to standard recommendations and in reference to the genomic and novel cDNA reference sequences ( We also report in this work: 1) results of the first screening for large deletions in TCOF1 by Southern blot in patients without mutation detected by direct sequencing; 2) the identification of the first pathogenic mutation in the newly described exon 6A; and 3) statistical analysis of pathogenic mutations and polymorphism distribution throughout the gene.

  16. Anaerobically Grown Escherichia coli Has an Enhanced Mutation Rate and Distinct Mutational Spectra (United States)

    Shewaramani, Sonal; Finn, Thomas J.; Kassen, Rees; Rainey, Paul B.


    Oxidative stress is a major cause of mutation but little is known about how growth in the absence of oxygen impacts the rate and spectrum of mutations. We employed long-term mutation accumulation experiments to directly measure the rates and spectra of spontaneous mutation events in Escherichia coli populations propagated under aerobic and anaerobic conditions. To detect mutations, whole genome sequencing was coupled with methods of analysis sufficient to identify a broad range of mutational classes, including structural variants (SVs) generated by movement of repetitive elements. The anaerobically grown populations displayed a mutation rate nearly twice that of the aerobic populations, showed distinct asymmetric mutational strand biases, and greater insertion element activity. Consistent with mutation rate and spectra observations, genes for transposition and recombination repair associated with SVs were up-regulated during anaerobic growth. Together, these results define differences in mutational spectra affecting the evolution of facultative anaerobes. PMID:28103245

  17. PAX6 mutations: genotype-phenotype correlations

    Directory of Open Access Journals (Sweden)

    Hanson Isabel M


    Full Text Available Abstract Background The PAX6 protein is a highly conserved transcriptional regulator that is important for normal ocular and neural development. In humans, heterozygous mutations of the PAX6 gene cause aniridia (absence of the iris and related developmental eye diseases. PAX6 mutations are archived in the Human PAX6 Allelic Variant Database, which currently contains 309 records, 286 of which are mutations in patients with eye malformations. Results We examined the records in the Human PAX6 Allelic Variant Database and documented the frequency of different mutation types, the phenotypes associated with different mutation types, the contribution of CpG transitions to the PAX6 mutation spectrum, and the distribution of chain-terminating mutations in the open reading frame. Mutations that introduce a premature termination codon into the open reading frame are predominantly associated with aniridia; in contrast, non-aniridia phenotypes are typically associated with missense mutations. Four CpG dinucleotides in exons 8, 9, 10 and 11 are major mutation hotspots, and transitions at these CpG's account for over half of all nonsense mutations in the database. Truncating mutations are distributed throughout the PAX6 coding region, except for the last half of exon 12 and the coding part of exon 13, where they are completely absent. The absence of truncating mutations in the 3' part of the coding region is statistically significant and is consistent with the idea that nonsense-mediated decay acts on PAX6 mutant alleles. Conclusion The PAX6 Allelic Variant Database is a valuable resource for studying genotype-phenotype correlations. The consistent association of truncating mutations with the aniridia phenotype, and the distribution of truncating mutations in the PAX6 open reading frame, suggests that nonsense-mediated decay acts on PAX6 mutant alleles.

  18. Mutation Clusters from Cancer Exome. (United States)

    Kakushadze, Zura; Yu, Willie


    We apply our statistically deterministic machine learning/clustering algorithm *K-means (recently developed in to 10,656 published exome samples for 32 cancer types. A majority of cancer types exhibit a mutation clustering structure. Our results are in-sample stable. They are also out-of-sample stable when applied to 1389 published genome samples across 14 cancer types. In contrast, we find in- and out-of-sample instabilities in cancer signatures extracted from exome samples via nonnegative matrix factorization (NMF), a computationally-costly and non-deterministic method. Extracting stable mutation structures from exome data could have important implications for speed and cost, which are critical for early-stage cancer diagnostics, such as novel blood-test methods currently in development.

  19. Tailoring the metabolism against mutations (United States)

    Gulbahce, Natali; Motter, Adilson E.; Almaas, Eivind; Barabasi, Albert Laszlo


    In the post-genomic era, organisms can be modelled at the whole-cell level in silico via steady state methods to describe their metabolic capabilities. We use two such methods, Flux Balance Analysis and Minimization of Metabolic Adjustment to explore the behavior of cells (of E. coli and S. cerevisiae) after severe mutations. We propose experimentally feasible ways of modifying the underlying biochemical reaction network of a mutant cell such that cell functionality, in particular growth rate, is significantly improved.

  20. AIM cryocooler developments for HOT detectors (United States)

    Rühlich, I.; Mai, M.; Withopf, A.; Rosenhagen, C.


    Significantly increased FPA temperatures for both Mid Wave and Long Wave IR detectors, i.e. HOT detectors, which have been developed in recent years are now leaving the development phase and are entering real application. HOT detectors allowing to push size weight and power (SWaP) of Integrated Detectors Cooler Assemblies (IDCA's) to a new level. Key component mainly driving achievable weight, volume and power consumption is the cryocooler. AIM cryocooler developments are focused on compact, lightweight linear cryocoolers driven by compact and high efficient digital cooler drive electronics (DCE) to also achieve highest MTTF targets. This technology is using moving magnet driving mechanisms and dual or single piston compressors. Whereas SX030 which was presented at SPIE in 2012 consuming less 3 WDC to operate a typical IDCA at 140K, next smaller cooler SX020 is designed to provide sufficient cooling power at detector temperature above 160K. The cooler weight of less than 200g and a total compressor length of 60mm makes it an ideal solution for all applications with limited weight and power budget, like in handheld applications. For operating a typical 640x512, 15μm MW IR detector the power consumption will be less than 1.5WDC. MTTF for the cooler will be in excess of 30,000h and thus achieving low maintenance cost also in 24/7 applications. The SX020 compressor is based on a single piston design with integrated passive balancer in a new design achieves very low exported vibration in the order of 100mN in the compressor axis. AIM is using a modular approach, allowing the chose between 5 different compressor types for one common Stirling expander. The 6mm expander with a total length of 74mm is now available in a new design that fits into standard dewar bores originally designed for rotary coolers. Also available is a 9mm coldfinger in both versions. In development is an ultra-short expander with around 35mm total length to achieve highest compactness. Technical

  1. Pathogenic mutations in Parkinson disease. (United States)

    Tan, Eng-King; Skipper, Lisa M


    Parkinson disease (PD; Parkinson's) is the second most common neurodegenerative disease, characterized by the progressive loss of dopamine neurons and the accumulation of Lewy bodies. Increasing evidence suggests that deficits in mitochondrial function, oxidative and nitrosative stress, the accumulation of aberrant or misfolded proteins, and ubiquitin-proteasome system (UPS) dysfunction may represent the principal molecular pathways that commonly underlie the pathogenesis. The relative role of genetic and environmental factors has been the focus of research and debate. The recent discovery of a number of disease-causing genes (SNCA, Parkin/PARK2, UCHL1, PINK1, DJ1/PARK7, and LRRK2) in familial and sporadic forms of PD has provided considerable insights into the pathophysiology of this complex disorder. The frequency of these gene mutations may vary according to ethnicity and to the specific gene. A gene dosage effect is observed in some cases, and the phenotype of some of the mutation carriers closely resembles typical PD. Penetrance of some of the recurrent mutations is incomplete and may vary with age. Further research to unravel the etiopathology could identify biochemical or genetic markers for potential neuroprotective trials. (c) 2007 Wiley-Liss, Inc.

  2. LHON: Mitochondrial Mutations and More. (United States)

    Kirches, E


    Leber's hereditary optic neuropathy (LHON) is a mitochondrial disorder leading to severe visual impairment or even blindness by death of retinal ganglion cells (RGCs). The primary cause of the disease is usually a mutation of the mitochondrial genome (mtDNA) causing a single amino acid exchange in one of the mtDNA-encoded subunits of NADH:ubiquinone oxidoreductase, the first complex of the electron transport chain. It was thus obvious to accuse neuronal energy depletion as the most probable mediator of neuronal death. The group of Valerio Carelli and other authors have nicely shown that energy depletion shapes the cell fate in a LHON cybrid cell model. However, the cybrids used were osteosarcoma cells, which do not fully model neuronal energy metabolism. Although complex I mutations may cause oxidative stress, a potential pathogenetic role of the latter was less taken into focus. The hypothesis of bioenergetic failure does not provide a simple explanation for the relatively late disease onset and for the incomplete penetrance, which differs remarkably between genders. It is assumed that other genetic and environmental factors are needed in addition to the 'primary LHON mutations' to elicit RGC death. Relevant nuclear modifier genes have not been identified so far. The review discusses the unresolved problems of a pathogenetic hypothesis based on ATP decline and/or ROS-induced apoptosis in RGCs.

  3. A hot topic: temperature sensitive sodium channelopathies. (United States)

    Egri, Csilla; Ruben, Peter C


    Perturbations to body temperature affect almost all cellular processes and, within certain limits, results in minimal effects on overall physiology. Genetic mutations to ion channels, or channelopathies, can shift the fine homeostatic balance resulting in a decreased threshold to temperature induced disturbances. This review summarizes the functional consequences of currently identified voltage-gated sodium (NaV) channelopathies that lead to disorders with a temperature sensitive phenotype. A comprehensive knowledge of the relationships between genotype and environment is not only important for understanding the etiology of disease, but also for developing safe and effective treatment paradigms.

  4. Mutation induction by ion beams in plants

    Energy Technology Data Exchange (ETDEWEB)

    Tanaka, Atsushi [Japan Atomic Energy Research Inst., Takasaki, Gunma (Japan). Takasaki Radiation Chemistry Research Establishment


    The effect of ion beams such as C, He, and Ne ions was investigated on the mutation induction in plants with the expectation that ion beams of high linear energy transfer (LET) can frequently produce large DNA alternation such as inversion, translocation and large deletion rather than point mutation. Mutation frequency was investigated using Arabidopsis visible phenotype loci and was 8 to 33 fold higher for 220 MeV carbon ions than for electrons. Mutation spectrum was investigated on the flower color of chrysanthemum cv to find that flower mutants induced by ion beams show complex and stripe types rather than single color. Polymerase chain reaction analysis was performed to investigate DNA alteration of mutations. In conclusion, the characteristics of ion beams for the mutation induction are 1) high frequency, 2) broad mutation spectrum, and 3) novel mutants. (S. Ohno)

  5. Androgen receptor gene mutation, rearrangement, polymorphism. (United States)

    Eisermann, Kurtis; Wang, Dan; Jing, Yifeng; Pascal, Laura E; Wang, Zhou


    Genetic aberrations of the androgen receptor (AR) caused by mutations, rearrangements, and polymorphisms result in a mutant receptor that has varied functions compared to wild type AR. To date, over 1,000 mutations have been reported in the AR with most of these being associated with androgen insensitivity syndrome (AIS). While mutations of AR associated with prostate cancer occur less often in early stage localized disease, mutations in castration-resistant prostate cancer (CRPC) patients treated with anti-androgens occur more frequently with 10-30% of these patients having some form of mutation in the AR. Resistance to anti-androgen therapy usually results from gain-of-function mutations in the LBD such as is seen with bicalutamide and more recently with enzalutamide (MDV3100). Thus, it is crucial to investigate these new AR mutations arising from drug resistance to anti-androgens and other small molecule pharmacological agents.

  6. Hot Springs-Garrison Fiber Optic Project

    Energy Technology Data Exchange (ETDEWEB)


    Bonneville Power Administration (BPA) is proposing to upgrade its operational telecommunications system between the Hot Springs Substation and the Garrison Substation using a fiber optic system. The project would primarily involve installing 190 kilometers (120 miles) of fiber optic cable on existing transmission structures and installing new fiber optic equipment in BPA`s substation yards and control houses. BPA prepared an environmental assessment (EA) evaluating the proposed action. This EA was published in October 1994. The EA identifies a number of minor impacts that might occur as a result of the proposed action, as well as some recommended mitigation measures. This Mitigation Action Plan (MAP) identifies specific measures to avoid, minimize, or compensate for impacts identified in the EA.

  7. Hot Cell Facility (HCF) Safety Analysis Report

    Energy Technology Data Exchange (ETDEWEB)



    This Safety Analysis Report (SAR) is prepared in compliance with the requirements of DOE Order 5480.23, Nuclear Safety Analysis Reports, and has been written to the format and content guide of DOE-STD-3009-94 Preparation Guide for U. S. Department of Energy Nonreactor Nuclear Safety Analysis Reports. The Hot Cell Facility is a Hazard Category 2 nonreactor nuclear facility, and is operated by Sandia National Laboratories for the Department of Energy. This SAR provides a description of the HCF and its operations, an assessment of the hazards and potential accidents which may occur in the facility. The potential consequences and likelihood of these accidents are analyzed and described. Using the process and criteria described in DOE-STD-3009-94, safety-related structures, systems and components are identified, and the important safety functions of each SSC are described. Additionally, information which describes the safety management programs at SNL are described in ancillary chapters of the SAR.

  8. Hot and Turbulent Gas in Clusters

    CERN Document Server

    Schmidt, Wolfram; Niemeyer, Jens C; Almgren, Ann S


    The gas in galaxy clusters is heated by shock compression through accretion (outer shocks) and mergers (inner shocks). These processes additionally produce turbulence. To analyse the relation between the thermal and turbulent energies of the gas under the influence of non-adiabatic processes, we performed numerical simulations of cosmic structure formation in a box of 152 Mpc comoving size with radiative cooling, UV background, and a subgrid scale model for numerically unresolved turbulence. By smoothing the gas velocities with an adaptive Kalman filter, we are able to estimate bulk flows toward cluster cores. This enables us to infer the velocity dispersion associated with the turbulent fluctuation relative to the bulk flow. For halos with masses above $10^{13}\\,M_\\odot$, we find that the turbulent velocity dispersions averaged over the warm-hot intergalactic medium (WHIM) and the intracluster medium (ICM) are approximately given by powers of the mean gas temperatures with exponents around 0.5, corresponding...

  9. Hot gas path component cooling system (United States)

    Lacy, Benjamin Paul; Bunker, Ronald Scott; Itzel, Gary Michael


    A cooling system for a hot gas path component is disclosed. The cooling system may include a component layer and a cover layer. The component layer may include a first inner surface and a second outer surface. The second outer surface may define a plurality of channels. The component layer may further define a plurality of passages extending generally between the first inner surface and the second outer surface. Each of the plurality of channels may be fluidly connected to at least one of the plurality of passages. The cover layer may be situated adjacent the second outer surface of the component layer. The plurality of passages may be configured to flow a cooling medium to the plurality of channels and provide impingement cooling to the cover layer. The plurality of channels may be configured to flow cooling medium therethrough, cooling the cover layer.

  10. Viscosity: From air to hot nuclei

    Indian Academy of Sciences (India)

    Nguyen Dinh Dang


    After a brief review of the history of viscosity from classical to quantal fluids, a discussion of how the shear viscosity of a finite hot nucleus is calculated directly from the width and energy of the giant dipole resonance (GDR) of the nucleus is given in this paper. The ratio / with s being the entropy volume density, is extracted from the experimental systematic of GDR in copper, tin and lead isotopes at finite temperature . These empirical results are compared with the results predicted by several independent models, as well as with almost model-independent estimations. Based on these results, it is concluded that the ratio / in medium and heavy nuclei decreases with increasing to reach (1.3−4)$×\\hbar/(4 k_B)$ at = 5 MeV, which is almost the same as that obtained for quark-gluon plasma at > 170 MeV.

  11. HotSpotter? Neutron/Gamma Detector

    Energy Technology Data Exchange (ETDEWEB)

    Bell, Z.W.


    The HotSpotter{trademark} Neutron/Gamma Detector combines in a single detecting module high sensitivity to gamma rays up to 3 MeV and sensitivity to neutrons. Using a 15 mm cubic CdWO{sub 4} (cadmium tungstate) crystal mounted on a 25 mm photomultiplier, the instrument realizes a factor of 5 increased photopeak efficiency over NaI(Tl) at 1 MeV, and a factor of 2 improvement over CsI(Tl). The addition of a 0.5 mm layer of {sup 10}B- impregnated epoxy covering the crystal provides neutron sensitivity without sacrificing gamma ray spectroscopic characteristics. Neutrons are detected by the presence of the 478 keV gamma from the {sup 10}B(n,{alpha}){sup 7}Li* reaction. In this paper, we describe the electronics and software of the instrument, and some of its characteristics.

  12. Why do hot subdwarf stars pulsate?

    CERN Document Server

    Geier, S


    Hot subdwarf B stars (sdBs) are the stripped cores of red giants located at the bluest extension of the horizontal branch. Several different kinds of pulsators are found among those stars. The mechanism that drives those pulsations is well known and the theoretically predicted instability regions for both the short-period p-mode and the long-period g-mode pulsators match the observed distributions fairly well. However, it remains unclear why only a fraction of the sdB stars pulsate, while stars with otherwise very similar parameters do not show pulsations. From an observers perspective I review possible candidates for the missing parameter that makes sdB stars pulsate or not.

  13. Power control for hot gas engines (United States)

    Macglashan, W. F. (Inventor)


    A hot gas engine in which the expander piston of the engine is connected to an expander crankshaft. A displacer piston of the engine is connected to a separate displacer crankshaft which may or may not be coaxial with the expander crankshaft. A phase angle control mechanism used as a power control for changing the phase angle between the expander and displacer crankshaft is located between the two crankshafts. The phase angle control mechanism comprises a differential type mechanism comprised of a pair of gears, as for example, bevel gears, one of which is connected to one end of the expander crankshaft and the other of which is connected to the opposite end of the displacer crankshaft. A mating bevel gear is disposed in meshing engagement with the first two level gears to provide a phase angle control between the two crankshafts. Other forms of differential mechanisms may be used including conventional spur gears connected in a differential type arrangement.

  14. A microsatellite platform for hot spot detection

    Energy Technology Data Exchange (ETDEWEB)

    Walter, I.; Briess, K.; Baerwald, W.; Lorenz, E.; Skrbek, W.; Schrandt, F. [DLR, Berlin (Germany). Inst. of Space Sensor Technology & Planetary Exploration


    The main payload of the BIRD micro-satellite is the newly developed hot spot recognition system. It's a dual-channel instrument for middle and thermal infrared imagery based on cooled MCT line detectors. The miniaturisation by integrated detector/cooler assemblies provides a highly efficient design. Since the launch in October 2001 from SHAR/India the BIRD payload, claiming 30% of the BIRD mass of 92 kg, is fully operational. Among others forest fires (Australia), volcanoes (Etna, Chile) and burning coal mines (China) have been detected and their parameters like size, temperature and energy release could be determined. As the status of the payload system is satisfactorily it has a potential to be applied in new missions with the help of modern detector technology.

  15. Hot Galactic Arms Point To Vicious Cycle (United States)


    NASA's Chandra X-ray Observatory has revealed the aftermath of a titanic explosion that wracked the elliptical galaxy known as NGC 4636. This eruption could be the latest episode in a cycle of violence that is triggered by gas falling into a central massive black hole. Chandra's images of NGC 4636 show spectacular symmetric arms, or arcs, of hot gas extending 25,000 light years into a huge cloud of multimillion-degree-Celsius gas that envelopes the galaxy. At a temperature of 10 million degrees, the arms are 30 percent hotter than the surrounding gas cloud. "The temperature jump, together with the symmetry and scale of the arms, suggests that we are observing the effects of a tremendous outburst that occurred in the center of the galaxy," said Christine Jones of the Harvard-Smithsonian Center for Astrophysics in Cambridge, Mass., lead author of a paper on these observations scheduled for publication in Astrophysical Journal Letters. "The energy of this explosion would be the equivalent of several hundred thousand supernovas." The arms appear to be the leading edges of a galaxy-sized shock wave that is racing outward at 700 kilometers per second, or 1.6 million miles per hour. At this speed, it would take 3 million years for the structures to attain their present size. Cavities detected in the hot gas cloud to the east and west of the center of the galaxy support the shockwave explanation. The authors suggest that the explosion is part of a majestic cosmic feedback process that keeps the galaxy in a state of turmoil. Over a period of a few million years, a hot gas cloud that envelops the stars in the galaxy cools and falls inward toward a central, massive black hole. The feeding of the black hole by the infalling material leads to an explosion that heats the hot gaseous envelope, starting the cycle anew. NGC 4636 NGC 4636 Background Subtracted This feedback cycle may explain one puzzling feature of the galaxy - the lack of a strong radio source of the type that is

  16. GDR in Hot Nuclei: New Measurements (United States)

    Camera, F.; Kmiecik, M.; Wieland, O.; Benzoni, G.; Bracco, A.; Brambilla, S.; Crespi, F.; Mason, P.; Moroni, A.; Million, B.; Leoni, S.; Maj, A.; Styczen, J.; Brekiesz, M.; Meczynski, W.; Zieblinski, M.; Gramegna, F.; Barlini, S.; Kravchuk, V. L.; Lanchais, A. L.; Mastinu, P. F.; Bruno, M.; D'Agostino, M.; Geraci, E.; Ordine, A.; Casini, G.; Chiari, M.


    The measured properties of the Giant Dipole Resonance in hot rotating nuclei are successfully described with the model of thermal fluctuations, even though there are still some open problems especially at very low (T 2.5MeV) temperatures and missing data in some mass regions. Recent experimental works have addressed more specific problems regarding the nuclear shape and its behaviour in very particular and delimited phase space regions. In this paper will be discussed new exclusive measurements of the GDR γ decay in heavy 216Rn nuclei (where the shape of nuclei surviving fission have been probed) and some preliminary data on the 132Ce nuclei at very high excitation energy.

  17. Disappearance of collective motion in hot nuclei

    Energy Technology Data Exchange (ETDEWEB)

    Santonocito, D.; Piattelli, P.; Agodi, C.; Alba, R.; Bellia, G.; Coniglione, R.; Del Zoppo, A.; Finocchiaro, P.; Hongmei, F.; Maiolino, C.; Migneco, E.; Sapienza, P. [INFN-LNS, Laboratori Nazionali del Sud, Catania (Italy); Blumenfeld, Y.; Delaunay, F.; Frascaria, N.; Lima, V.; Scarpaci, J. [Institut de Physique Nucleaire, IN2P3-CNRS, 91 - Orsay (France); Migneco, E. [Catania Univ., Dipartimento di Fisica (Italy)


    The evolution of the GDR {gamma} yield for excitation energies between 160 and 300 MeV has been investigated in nuclei of mass A {approx} 126 through the reactions {sup 116}Sn + {sup 12}C and {sup 24}Mg at 17 and 23 A*MeV. Gamma-rays were detected with MEDEA array in coincidence with residues detected in MACISTE. The evolution of the GDR parameters has been investigated as a function of the linear momentum transferred to the fused system. This procedure allowed us to select hot nuclei produced in incomplete fusion reactions with different excitation energies. The analysis of the {gamma} spectra and their comparison with CASCADE calculations is presented. Results suggest a saturation of the GDR yield between 200 and 300 MeV excitation energy. (authors)

  18. Optical NEP in Hot-Electron Nanobolometers

    CERN Document Server

    Karasik, Boris S


    For the first time, we have measured the optical noise equivalent power (NEP) in titanium (Ti) superconducting hot-electron nanobolometers (nano-HEBs). The bolometers were 2{\\mu}mx1{\\mu}mx20nm and 1{\\mu}mx1{\\mu}mx20nm planar antenna-coupled devices. The measurements were done at {\\lambda} = 460 {\\mu}m using a cryogenic black body radiation source delivering optical power from a fraction of a femtowatt to a few 100s of femtowatts. A record low NEP = 3x10^{-19} W/Hz^{1/2} at 50 mK has been achieved. This sensitivity meets the requirements for SAFARI instrument on the SPICA telescope. The ways for further improvement of the nano-HEB detector sensitivity are discussed.

  19. Telescopic nanotube device for hot nanolithography (United States)

    Popescu, Adrian; Woods, Lilia M


    A device for maintaining a constant tip-surface distance for producing nanolithography patterns on a surface using a telescopic nanotube for hot nanolithography. An outer nanotube is attached to an AFM cantilever opposite a support end. An inner nanotube is telescopically disposed within the outer nanotube. The tip of the inner nanotube is heated to a sufficiently high temperature and brought in the vicinity of the surface. Heat is transmitted to the surface for thermal imprinting. Because the inner tube moves telescopically along the outer nanotube axis, a tip-surface distance is maintained constant due to the vdW force interaction, which in turn eliminates the need of an active feedback loop.

  20. Protodiscs around Hot Magnetic Rotator Stars

    CERN Document Server

    Maheswaran, M


    We develop equations and obtain solutions for the structure and evolution of a protodisc region that is initially formed with no radial motion and super-Keplerian rotation speed when wind material from a hot rotating star is channelled towards its equatorial plane by a dipole-type magnetic field. Its temperature is around $10^7$K because of shock heating and the inflow of wind material causes its equatorial density to increase with time. The centrifugal force and thermal pressure increase relative to the magnetic force and material escapes at its outer edge. The protodisc region of a uniformly rotating star has almost uniform rotation and will shrink radially unless some instability intervenes. In a star with angular velocity increasing along its surface towards the equator, the angular velocity of the protodisc region decreases radially outwards and magnetorotational instability (MRI) can occur within a few hours or days. Viscosity resulting from MRI will readjust the angular velocity distribution of the pro...

  1. Rydberg blockade in a hot atomic beam (United States)

    Yoshida, S.; Burgdörfer, J.; Zhang, X.; Dunning, F. B.


    The dipole blockade of very-high-n , n ˜300 , strontium 5 s n f 1F3 Rydberg atoms in a hot atomic beam is studied. For such high n , the blockade radius can exceed the linear dimensions of the excitation volume. Rydberg atoms formed inside the excitation volume can, upon leaving the region, continue to suppress excitation until they have moved farther away than the blockade radius. Moreover, the high density of states originating from the many magnetic sublevels associated with the F states results in a small but finite probability of excitation of L =3 n 1F3 atom pairs at small internuclear separations below the blockade radius. We demonstrate that these effects can be distinguished from one another by the distinct features they imprint on the Mandel Q parameter as a function of the duration of the exciting laser.

  2. Lifetime of quasiparticles in hot QED plasmas

    CERN Document Server

    Blaizot, J P; Blaizot, Jean Paul; Iancu, Edmond


    The calculation of the lifetime of quasiparticles in a QED plasma at high temperature remains plagued with infrared divergences, even after one has taken into account the screening corrections. The physical processes responsible for these divergences are the collisions involving the exchange of very soft, unscreened, magnetic photons, whose contribution is enhanced by the thermal Bose-Einstein occupation factor. The self energy diagrams which diverge in perturbation theory contain no internal fermion loops, but an arbitrary number of internal magnetostatic photon lines. By generalizing the Bloch-Nordsieck model at finite temperature, we can resum all the singular contributions of such diagrams, and obtain the correct long time behaviour of the retarded fermion propagator in the hot QED plasma: S_R(t)\\sim \\exp\\{-\\alpha T \\, t\\, \\ln\\omega_pt\\}, where \\omega_p=eT/3 is the plasma frequency and \\alpha=e^2/4\\pi.

  3. Subarcsecond Imaging of Hot Cores with BIMA

    CERN Document Server

    Gibb, A G; Mundy, L G


    We present 1.4-mm BIMA observations with subarcsecond resolution of the bright dust and molecular line emission from hot cores associated with a sample of four ultracompact HII regions: G9.62+0.19, G10.47-0.03, G29.96-0.02, G31.41+0.31. Density power laws can reproduce the observed continuum emission but break down on scales smaller than 2000 AU. A total of 38 transitions from 18 species are detected, with G10.47 and G31.41 showing the greatest number of lines. In particular, these sources display emission from two collisionally-excited transitions of methanol lying more than 950 K above the ground state. Outflows traced by H2S emission provide evidence for embedded exciting sources and the observed morphology of molecular lines is consistent with internal heating of the cores.

  4. Approach to hot bending process simulation

    Energy Technology Data Exchange (ETDEWEB)

    Carmignani, B.; Daneri, A.; Toselli, G. [ENEA, Bologna (Italy). Centro Ricerche Energia `E. Clementel` - Area Energia e Innovazione


    An approach to the simulation of the thermal shaping or bending of large steel sheets, by ABAQUS/Standard code, will be presented. A thermal source representation, which can produce a temperature distribution, adequate to the processes which must be considered, has been set up. Some problems connected with the hot sheet shaping or bending process simulation have been approached and calculations have been executed in order to single out how to perform the sheet heating, so that the required sheet shape may be obtained. The results for one reference model for different source situations and one heating line, object of the first phase of the analyses performed, will be presented and discussed. The work will be presented at the 8th International Abaqus Users` Conference at Paris, 31 May - 2 June 1995.

  5. Hot carrier injection degradation under dynamic stress

    Institute of Scientific and Technical Information of China (English)

    Ma Xiao-Hua; Cao Yan-Rong; Hao Yue; Zhang Yue


    In this paper, we have studied hot carrier injection (HCI) under alternant stress. Under different stress modes, different degradations are obtained from the experiment results. The different alternate stresses can reduce or enhance the HC effect, which mainly depends on the latter condition of the stress cycle. In the stress mode A (DC stress with electron injection), the degradation keeps increasing. In the stress modes B (DC stress and then stress with the smallest gate injection) and C (DC stress and then stress with hole injection under Vg=0V and Vd = 1.8 V), recovery appears in the second stress period. And in the stress mode D (DC stress and then stress with hole injection under Vg = -1.8 V and Vd = 1.8 V), as the traps filled in by holes can be smaller or greater than the generated interface states, the continued degradation or recovery in different stress periods can be obtained.

  6. Hot Isostatic Pressing of 60-Nitinol (United States)

    Stanford, Malcolm K.


    The effects of varying the time, temperature and pressure during consolidation of 60-Nitinol (Nickel Titanium alloy) by hot isostatic pressing (HIP) were examined. Six HIP cycles with a cycle time of either 2 or 20 hours, temperature of 900 or 1000 degrees Centigrade, and a chamber pressure of either 100 or 200 millipascals were used. The cycle representing the shortest cycle time at the highest temperature and pressure (2 hours/1000 degrees Centigrade/200 millipascals) produced material with the highest hardness (720 Vickers Pyramid Number (HV)). A modest increase in average grain size and significant porosity reduction were observed in material subjected to the longest cycle time at the highest temperature, regardless of the pressure applied. The intent of this study is to facilitate the technology transfer involved in the processing of this material.

  7. Magnetic spots on hot massive stars

    CERN Document Server

    Cantiello, Matteo


    Hot luminous stars show a variety of phenomena in their photospheres and winds which still lack clear physical explanation. Among these phenomena are photospheric turbulence, line profile variability (LPV), non-thermal emission, non-radial pulsations, discrete absorption components (DACs) and wind clumping. Cantiello et al. (2009) argued that a convection zone close to the stellar surface could be responsible for some of these phenomena. This convective zone is caused by a peak in the opacity associated with iron-group elements and is referred to as the "iron convection zone" (FeCZ). Assuming dynamo action producing magnetic fields at equipartition in the FeCZ, we investigate the occurrence of subsurface magnetism in OB stars. Then we study the surface emergence of these magnetic fields and discuss possible observational signatures of magnetic spots. Simple estimates are made using the subsurface properties of massive stars, as calculated in 1D stellar evolution models. We find that magnetic fields of suffici...

  8. Hot multiboundary wormholes from bipartite entanglement

    CERN Document Server

    Marolf, Donald; Peach, Alex; Ross, Simon F


    We analyze the 1+1 CFT states dual to hot (time-symmetric) 2+1 multiboundary AdS wormholes. These are black hole geometries with high local temperature, $n \\ge 1$ asymptotically-AdS$_3$ regions, and arbitrary internal topology. The dual state at $t=0$ is defined on $n$ circles. We show these to be well-described by sewing together tensor networks corresponding to thermofield double states. As a result, the entanglement is spatially localized and bipartite: away from particular boundary points ("vertices") any small connected region $A$ of the boundary CFT is entangled only with another small connected region $B$, where $B$ may lie on a different circle or may be a different part of the same circle. We focus on the pair-of-pants case, from which more general cases may be constructed. We also discuss finite-temperature corrections, where we note that the states involve a code subspace in each circle.

  9. Graphene vertical hot-electron terahertz detectors

    Energy Technology Data Exchange (ETDEWEB)

    Ryzhii, V., E-mail: [Research Institute for Electrical Communication, Tohoku University, Sendai 980-8577 (Japan); Center for Photonics and Infrared Engineering, Bauman Moscow State Technical University and Institute of Ultra High Frequency Semiconductor Electronics, Russian Academy of Sciences, Moscow 111005 (Russian Federation); Satou, A.; Otsuji, T. [Research Institute for Electrical Communication, Tohoku University, Sendai 980-8577 (Japan); Ryzhii, M. [Department of Computer Science and Engineering, University of Aizu, Aizu-Wakamatsu 965-8580 (Japan); Mitin, V. [Department of Electrical Engineering, University at Buffalo, Buffalo, New York 1460-1920 (United States); Shur, M. S. [Departments of Electrical, Electronics, and Systems Engineering and Physics, Applied Physics, and Astronomy, Rensselaer Polytechnic Institute, Troy, New York 12180 (United States)


    We propose and analyze the concept of the vertical hot-electron terahertz (THz) graphene-layer detectors (GLDs) based on the double-GL and multiple-GL structures with the barrier layers made of materials with a moderate conduction band off-set (such as tungsten disulfide and related materials). The operation of these detectors is enabled by the thermionic emissions from the GLs enhanced by the electrons heated by incoming THz radiation. Hence, these detectors are the hot-electron bolometric detectors. The electron heating is primarily associated with the intraband absorption (the Drude absorption). In the frame of the developed model, we calculate the responsivity and detectivity as functions of the photon energy, GL doping, and the applied voltage for the GLDs with different number of GLs. The detectors based on the cascade multiple-GL structures can exhibit a substantial photoelectric gain resulting in the elevated responsivity and detectivity. The advantages of the THz detectors under consideration are associated with their high sensitivity to the normal incident radiation and efficient operation at room temperature at the low end of the THz frequency range. Such GLDs with a metal grating, supporting the excitation of plasma oscillations in the GL-structures by the incident THz radiation, can exhibit a strong resonant response at the frequencies of several THz (in the range, where the operation of the conventional detectors based on A{sub 3}B{sub 5} materials, in particular, THz quantum-well detectors, is hindered due to a strong optical phonon radiation absorption in such materials). We also evaluate the characteristics of GLDs in the mid- and far-infrared ranges where the electron heating is due to the interband absorption in GLs.

  10. Phototrophy in Mildly Acidic Hot Spring Ecosystems (United States)

    Fecteau, K.; Boyd, E. S.; Shock, E.


    Microbial light-driven reduction of carbon in continental hydrothermal ecosystems is restricted to environments at temperatures less than 73 °C. In circumneutral and alkaline systems bacterial phototrophs (cyanobacteria and anoxygenic phototrophs) are suggested to be principally responsible for this activity whereas algal (i.e., eukaryotic) phototrophs are thought to be responsible for this activity in acidic systems. In Yellowstone National Park numerous examples of phototrophic microbial communities exist at high and low pH, while hot springs with intermediate pH (values 3-5) are rare and commonly dilute. It is thought that the transition from algal photosynthesis to bacterial photosynthesis occurs within this pH range. To test this hypothesis, we sequenced bacterial and eukaryal small subunit ribosomal RNA genes, analyzed pigments, and performed comprehensive geochemical measurements from 12 hot springs within this pH realm. At all sites, the largest phototrophic population was either comprised of Cyanobacteria or affiliated with the algal order Cyanidiales, which are ubiquitous in acidic springs, yet abundant sequences of both lineages were present in 8 of the 12 sites. Nevertheless, some of these samples exceeded the known temperature limit of the algae (56 °C), suggesting that these populations are dead or inactive. Indeed, one site yielded evidence for a large Cyanidiales population as the only phototrophs present, yet an experiment at the time of sampling failed to demonstrate light-driven carbon fixation, and analysis of extracted pigments showed a large amount of the chlorophyll degradation product pheophorbide a and very little intact chlorophyll, indicating photosynthesis occurred at this site when conditions were different. Our observations illustrate the dynamic nature of these systems that may be transiently conducive to photosynthesis, which may open niches for phototrophs of both domains and likely played a role in the evolution of photosynthesis.

  11. SMA millimeter observations of hot molecular cores

    Energy Technology Data Exchange (ETDEWEB)

    Hernández-Hernández, Vicente; Zapata, Luis; Kurtz, Stan [Centro de Radioastronomía y Astrofísica, Universidad Nacional Autónoma de México, Apdo. Postal 3-72 (Xangari), 58090 Morelia, Michoacán (Mexico); Garay, Guido, E-mail: [Departamento de Astronomía, Universidad de Chile, Camino del Observatorio 1515, Las Condes, Santiago (Chile)


    We present Submillimeter Array observations in the 1.3 mm continuum and the CH{sub 3}CN (12 {sub K}-11 {sub K}) line of 17 hot molecular cores associated with young high-mass stars. The angular resolution of the observations ranges from 1.''0 to 4.''0. The continuum observations reveal large (>3500 AU) dusty structures with gas masses from 7 to 375 M {sub ☉}, which probably surround multiple young stars. The CH{sub 3}CN line emission is detected toward all the molecular cores at least up to the K = 6 component and is mostly associated with the emission peaks of the dusty objects. We used the multiple K-components of the CH{sub 3}CN and both the rotational diagram method and a simultaneous synthetic local thermodynamic equilibrium model with the XCLASS program to estimate the temperatures and column densities of the cores. For all sources, we obtained reasonable fits from XCLASS by using a model that combines two components: an extended and warm envelope and a compact hot core of molecular gas, suggesting internal heating by recently formed massive stars. The rotational temperatures lie in the range of 40-132 K and 122-485 K for the extended and compact components, respectively. From the continuum and CH{sub 3}CN results, we infer fractional abundances from 10{sup –9} to 10{sup –7} toward the compact inner components, which increase with the rotational temperature. Our results agree with a chemical scenario in which the CH{sub 3}CN molecule is efficiently formed in the gas phase above 100-300 K, and its abundance increases with temperature.

  12. Oncogene mutational profile in nasopharyngeal carcinoma

    Directory of Open Access Journals (Sweden)

    Zhang ZC


    Full Text Available Zi-Chen Zhang,1,* Sha Fu,1,* Fang Wang,1 Hai-Yun Wang,1 Yi-Xin Zeng,2 Jian-Yong Shao11Department of Molecular Diagnostics, 2Department of Experimental Research, Sun Yat-sen University Cancer Center, State Key Laboratory of Oncology in South China, Collaborative Innovation Center of Cancer Medicine, Guangzhou, People's Republic of China *These authors contributed equally to this work Abstract: Nasopharyngeal carcinoma (NPC is a common tumor in Southern China, but the oncogene mutational status of NPC patients has not been clarified. Using time-of-flight mass spectrometry, 238 mutation hotspots in 19 oncogenes were examined in 123 NPC patients. The relationships between mutational status and clinical data were assessed with a χ2 or Fisher's exact test. Survival analysis was performed using the Kaplan–Meier method with the log-rank test. In 123 patients, 21 (17.1% NPC tumors were positive for mutations in eight oncogenes: six patients had PIK3CA mutations (4.9%, five NRAS mutations (4.1%, four KIT mutations (3.3%, two PDGFRA mutations (1.6%, two ABL mutations (1.6%, and one with simultaneous mutations in HRAS, EGFR, and BRAF (1%. Patients with mutations were more likely to relapse or develop metastasis than those with wild-type alleles (P=0.019. No differences or correlations were found in other clinical characteristics or in patient survival. No mutations were detected in oncogenes AKT1, AKT2, CDK, ERBB2, FGFR1, FGFR3, FLT3, JAK2, KRAS, MET, and RET. These results demonstrate an association between NPC and mutations in NRAS, KIT, PIK3CA, PDGFRA, and ABL, which are associated with patient relapse and metastasis. Keywords: NPC, oncogene, mutation

  13. Molecular characterization of rpoB gene mutations in rifampicine-resistant Mycobacterium tuberculosis isolates from tuberculosis patients in Belarus. (United States)

    Titov, Leonid P; Zakerbostanabad, Saeed; Slizen, Veranika; Surkova, Larisa; Taghikhani, Mohammad; Bahrmand, Ahmadreza


    The aim of this study was to investigate the frequency, location and type of rpoB mutations in Mycobacterium tuberculosis isolated from patients in Belarus. Tuberculosis cases are increasing every year in Belarus. Moreover, resistance to anti-tuberculosis drugs, especially to rifampicine, has increased. In this study, 44 rifampicine-resistance M. tuberculosis clinical isolates (including multidrug-resistant isolates) were subjected to DNA sequencing analysis of the hypervariable region (hot-spot) of the rpoB gene originating from different geographical regions in Belarus. Sixteen different types of mutations were identified. The most common point mutations were in codons 510 (47.7%), 526 (45.5%), 523 (40.86%) and 531 (29.5%). Eleven isolates (27.7%) carried one mutation and 23 (52%), 7 (16%), 3 (7%) of isolates carried 2, 3 and 4 mutations, respectively. A characteristic, prominent finding of this study was high frequency of multiple mutations in different codons of the rpoB gene (27.7%) and also the detection of unusual types of mutations in the 510 codon, comprising CAG mutations (deletion or changing, to CTG, CAC or CAT). In our study, the change TTG in codon 531 was found in 92% of isolates and the change TGC in 8% of isolates. A TAC change in codon 526 was not found, but the GAC change was found in all isolates. Isolates of M. tuberculosis isolated in Belarus were characterized by the wide spectrum of the important mutations and might belong to the epidemic widespread clones.

  14. Hot-Deformation Behavior and Hot-Processing Maps of AISI 410 Martensitic Stainless Steel (United States)

    Qi, Rong-Sheng; Jin, Miao; Guo, Bao-Feng; Liu, Xin-Gang; Chen, Lei


    The compressive deformation behaviors of 410 martensitic stainless steel were investigated on a Gleeble-1500 thermomechanical simulator, and the experimental stress-strain data were obtained. The measured flow stress was corrected for friction and temperature. A constitutive equation that accounts for the influence of strain was established, and the hot-processing maps at different strain were plotted. The microstructure evolution of the hot-deformation process was studied on the basis of microstructural observations at high temperatures. Phase-transformation experiments on 410 steel were conducted at high temperatures to elucidate the effects of temperature on the delta-ferrite content. The initial forging temperature and optimum process parameters were obtained on the basis of the processing map and the changes in the delta-ferrite content at high temperatures.

  15. Update on the KELT Transit Survey: Hot Planets around Hot, Bright Stars (United States)

    Gaudi, B. Scott; KELT Collaboration


    The KELT Transit Survey consists of a pair of small-aperture, wide-angle automated telescope located at Winer Observatory in Sonoita, Arizona and the South African Astronomical Observatory (SAAO) in Sutherland, South Africa. Together, they are surveying roughly 60% of the sky for transiting planets. By virtue of their small apertures (42 mm) and large fields-of-view (26 degrees x 26 degrees), KELT is most sensitive to hot Jupiters transiting relatively bright (V~8-11), and thus relatively hot stars. Roughly half of the dwarf stars targeted by KELT are hotter than 6250K; such stars pose novel challenges, but also provide unique opportunities. I will provide an update on the most recent companions discovered by KELT, focusing in detail on a few particularly interesting systems. KELT is a joint collaboration between the Ohio State University, Vanderbilt University, and Lehigh University. This work was partially supported by NSF CAREER grant AST-1056524.

  16. Kelley Hot Spring Geothermal Project: Kelly Hot Spring Agricultural Center conceptual design

    Energy Technology Data Exchange (ETDEWEB)

    Longyear, A.B. (ed.)


    The proposed core activity in the Kelly Hot Spring Agricultural Center is a nominal 1200 sow swine raising complex. The swine raising is to be a totally confined operation for producing premium pork in controlled environment facilities that utilize geothermal energy. The complex will include a feedmill for producing the various feed formulae required for the animals from breeding through gestation, farrowing, nursery, growing and finishing. The market animals are shipped live by truck to slaughter in Modesto, California. A complete waste management facility will include manure collection from all raising areas, transport via a water flush sysem to methane (biogas) generators, manure separation, settling ponds and disposition of the surplus agricultural quality water. The design is based upon the best commercial practices in confined swine raising in the US today. The most unique feature of the facility is the utilization of geothermal hot water for space heating and process energy throughout the complex.

  17. 36 CFR 7.18 - Hot Springs National Park. (United States)


    ... 36 Parks, Forests, and Public Property 1 2010-07-01 2010-07-01 false Hot Springs National Park. 7.18 Section 7.18 Parks, Forests, and Public Property NATIONAL PARK SERVICE, DEPARTMENT OF THE INTERIOR SPECIAL REGULATIONS, AREAS OF THE NATIONAL PARK SYSTEM § 7.18 Hot Springs National Park. (a)...

  18. Heat Losses Evaluation for Domestic Hot Water Distribution Systems

    Directory of Open Access Journals (Sweden)

    Theodor Mateescu


    Full Text Available In sanitary systems assembly, domestic hot water distribution supply networks represent an important weight for energetically balance.par This paper presents, in an analytical and graphical manner, the computational tools needed for domestic hot water piping system behavior characterization in different functional and structural assumptions.

  19. 46 CFR 177.970 - Protection against hot piping. (United States)


    ... 46 Shipping 7 2010-10-01 2010-10-01 false Protection against hot piping. 177.970 Section 177.970 Shipping COAST GUARD, DEPARTMENT OF HOMELAND SECURITY (CONTINUED) SMALL PASSENGER VESSELS (UNDER 100 GROSS TONS) CONSTRUCTION AND ARRANGEMENT Rails and Guards § 177.970 Protection against hot piping....

  20. 46 CFR 116.970 - Protection against hot piping. (United States)


    ... 46 Shipping 4 2010-10-01 2010-10-01 false Protection against hot piping. 116.970 Section 116.970 Shipping COAST GUARD, DEPARTMENT OF HOMELAND SECURITY (CONTINUED) SMALL PASSENGER VESSELS CARRYING MORE... ARRANGEMENT Rails and Guards § 116.970 Protection against hot piping. Piping, including valves, pipe...