WorldWideScience

Sample records for linear integrated sachs-wolfe

  1. CMB anisotropies at all orders: the non-linear Sachs-Wolfe formula

    OpenAIRE

    Roldan, Omar

    2017-01-01

    We obtain the non-linear generalization of the Sachs-Wolfe + integrated Sachs-Wolfe (ISW) formula describing the CMB temperature anisotropies. Our formula is valid at all orders in perturbation theory, is also valid in all gauges and includes scalar, vector and tensor modes. A direct consequence of our results is that the maps of the logarithmic temperature anisotropies are much cleaner than the usual CMB maps, because they automatically remove many secondary anisotropies. This can for instan...

  2. CMB anisotropies at all orders: the non-linear Sachs-Wolfe formula

    International Nuclear Information System (INIS)

    Roldan, Omar

    2017-01-01

    We obtain the non-linear generalization of the Sachs-Wolfe + integrated Sachs-Wolfe (ISW) formula describing the CMB temperature anisotropies. Our formula is valid at all orders in perturbation theory, is also valid in all gauges and includes scalar, vector and tensor modes. A direct consequence of our results is that the maps of the logarithmic temperature anisotropies are much cleaner than the usual CMB maps, because they automatically remove many secondary anisotropies. This can for instance, facilitate the search for primordial non-Gaussianity in future works. It also disentangles the non-linear ISW from other effects. Finally, we provide a method which can iteratively be used to obtain the lensing solution at the desired order.

  3. CMB anisotropies at all orders: the non-linear Sachs-Wolfe formula

    Energy Technology Data Exchange (ETDEWEB)

    Roldan, Omar, E-mail: oaroldan@if.ufrj.br [Instituto de Física, Universidade Federal do Rio de Janeiro, 21941-972, Rio de Janeiro, RJ (Brazil)

    2017-08-01

    We obtain the non-linear generalization of the Sachs-Wolfe + integrated Sachs-Wolfe (ISW) formula describing the CMB temperature anisotropies. Our formula is valid at all orders in perturbation theory, is also valid in all gauges and includes scalar, vector and tensor modes. A direct consequence of our results is that the maps of the logarithmic temperature anisotropies are much cleaner than the usual CMB maps, because they automatically remove many secondary anisotropies. This can for instance, facilitate the search for primordial non-Gaussianity in future works. It also disentangles the non-linear ISW from other effects. Finally, we provide a method which can iteratively be used to obtain the lensing solution at the desired order.

  4. Planck 2015 results: XXI. The integrated Sachs-Wolfe effect

    DEFF Research Database (Denmark)

    Ade, P. A R; Aghanim, N.; Arnaud, M.

    2016-01-01

    This paper presents a study of the integrated Sachs-Wolfe (ISW) effect from the Planck 2015 temperature and polarization data release. This secondary cosmic microwave background (CMB) anisotropy caused by the large-scale time-evolving gravitational potential is probed from different perspectives....

  5. Integrated Sachs-Wolfe effect in a quintessence cosmological model: Including anisotropic stress of dark energy

    International Nuclear Information System (INIS)

    Wang, Y. T.; Xu, L. X.; Gui, Y. X.

    2010-01-01

    In this paper, we investigate the integrated Sachs-Wolfe effect in the quintessence cold dark matter model with constant equation of state and constant speed of sound in dark energy rest frame, including dark energy perturbation and its anisotropic stress. Comparing with the ΛCDM model, we find that the integrated Sachs-Wolfe (ISW)-power spectrums are affected by different background evolutions and dark energy perturbation. As we change the speed of sound from 1 to 0 in the quintessence cold dark matter model with given state parameters, it is found that the inclusion of dark energy anisotropic stress makes the variation of magnitude of the ISW source uncertain due to the anticorrelation between the speed of sound and the ratio of dark energy density perturbation contrast to dark matter density perturbation contrast in the ISW-source term. Thus, the magnitude of the ISW-source term is governed by the competition between the alterant multiple of (1+3/2xc-circumflex s 2 ) and that of δ de /δ m with the variation of c-circumflex s 2 .

  6. Integrated Sachs-Wolfe effect versus redshift test for the cosmological parameters

    Science.gov (United States)

    Kantowski, R.; Chen, B.; Dai, X.

    2015-04-01

    We describe a method using the integrated Sachs-Wolfe (ISW) effect caused by individual inhomogeneities to determine the cosmological parameters H0, Ωm , and ΩΛ, etc. This ISW-redshift test requires detailed knowledge of the internal kinematics of a set of individual density perturbations, e.g., galaxy clusters and/or cosmic voids, in particular their density and velocity profiles, and their mass accretion rates. It assumes the density perturbations are isolated and embedded (equivalently compensated) and makes use of the newly found relation between the ISW temperature perturbation of the cosmic microwave background (CMB) and the Fermat potential of the lens. Given measurements of the amplitudes of the temperature variations in the CMB caused by such clusters or voids at various redshifts and estimates of their angular sizes or masses, one can constrain the cosmological parameters. More realistically, the converse is more likely, i.e., if the background cosmology is sufficiently constrained, measurement of ISW profiles of clusters and voids (e.g., hot and cold spots and rings) can constrain dynamical properties of the dark matter, including accretion, associated with such lenses and thus constrain the evolution of these objects with redshift.

  7. Embedded Lensing Time Delays, the Fermat Potential, and the Integrated Sachs-Wolfe Effect

    Science.gov (United States)

    Chen, Bin; Kantowski, Ronald; Dai, Xinyu

    2015-05-01

    We derive the Fermat potential for a spherically symmetric lens embedded in a Friedman-Lemaître-Robertson-Walker cosmology and use it to investigate the late-time integrated Sachs-Wolfe (ISW) effect, i.e., secondary temperature fluctuations in the cosmic microwave background (CMB) caused by individual large-scale clusters and voids. We present a simple analytical expression for the temperature fluctuation in the CMB across such a lens as a derivative of the lens’ Fermat potential. This formalism is applicable to both linear and nonlinear density evolution scenarios, to arbitrarily large density contrasts, and to all open and closed background cosmologies. It is much simpler to use and makes the same predictions as conventional approaches. In this approach the total temperature fluctuation can be split into a time-delay part and an evolutionary part. Both parts must be included for cosmic structures that evolve and both can be equally important. We present very simple ISW models for cosmic voids and galaxy clusters to illustrate the ease of use of our formalism. We use the Fermat potentials of simple cosmic void models to compare predicted ISW effects with those recently extracted from WMAP and Planck data by stacking large cosmic voids using the aperture photometry method. If voids in the local universe with large density contrasts are no longer evolving we find that the time delay contribution alone predicts values consistent with the measurements. However, we find that for voids still evolving linearly, the evolutionary contribution cancels a significant part of the time delay contribution and results in predicted signals that are much smaller than recently observed.

  8. Probing the integrated Sachs-Wolfe effect using embedded lens models

    Science.gov (United States)

    Chen, B.; Kantowski, R.

    2015-04-01

    The photometry profile of the integrated Sachs-Wolfe (ISW) effect, recently obtained by the Planck consortium by stacking patches of cosmic microwave background (CMB) sky maps around a large number of cosmic voids, contains a cold ring at about half the void's effective radius surrounded by a hot ring near the void's boundary. The source of the temperature structure is assumed to be the ISW effect but the exact cause of the ringed structure is not currently well understood, particularly the outer hot ring. Numerical simulations have suggested that hot/cold ring structures can be produced by motions associated with nonlinear growths of cosmic structures whose gravitational potentials produce the ISW effect. We have recently developed the embedded lens theory and the Fermat potential formalism which can be used to model the ISW effect caused by intervening individual lens inhomogeneities evolving arbitrarily. This theory only requires knowledge of the void's projected mass profile as a function of the passing CMB photons' impact radius and the rate of change of that mass distribution at passage. We present two simple embedded void lens models with evolving mass densities and investigate the ISW effect caused by these lenses. Both models possess expanding mass shells which produce hot rings around central cold regions, consistent with the recent observations. By adding a small overdensity at the void's center we can produce the slight positive temperature excess hinted at in Planck's photometric results. We conclude that the embedded lens theory and the Fermat potential formalism is well suited for modeling the ISW effect.

  9. Magnification-temperature correlation: The dark side of integrated Sachs-Wolfe measurements

    International Nuclear Information System (INIS)

    LoVerde, Marilena; Hui, Lam; Gaztanaga, Enrique

    2007-01-01

    Integrated Sachs-Wolfe (ISW) measurements, which involve cross-correlating the microwave background anisotropies with the foreground large-scale structure (e.g. traced by galaxies/quasars), have proven to be an interesting probe of dark energy. We show that magnification bias, which is the inevitable modulation of the foreground number counts by gravitational lensing, alters both the scale dependence and amplitude of the observed ISW signal. This is true especially at high redshifts because (1) the intrinsic galaxy-temperature signal diminishes greatly back in the matter-dominated era, (2) the lensing efficiency increases with redshift and (3) the number count slope generally steepens with redshift in a magnitude limited sample. At z > or approx. 2, the magnification-temperature correlation dominates over the intrinsic galaxy-temperature correlation and causes the observed ISW signal to increase with redshift, despite dark energy subdominance--a result of the fact that magnification probes structures all the way from the observer to the sources. Ignoring magnification bias therefore can lead to (significantly) erroneous conclusions about dark energy. While the lensing modulation opens up an interesting high z window for ISW measurements, high redshift measurements are not expected to add much new information to low redshift ones if dark energy is indeed the cosmological constant. This is because lensing introduces significant covariance across redshifts. The most compelling reasons for pursuing high redshift ISW measurements are to look for potential surprises such as early dark energy domination or signatures of modified gravity. We conclude with a discussion of existing measurements, the highest redshift of which is at the margin of being sensitive to the magnification effect. We also develop a formalism which might be of more general interest: to predict biases in estimating parameters when certain physical effects are ignored in interpreting observations

  10. Updated tomographic analysis of the integrated Sachs-Wolfe effect and implications for dark energy

    Science.gov (United States)

    Stölzner, Benjamin; Cuoco, Alessandro; Lesgourgues, Julien; Bilicki, Maciej

    2018-03-01

    We derive updated constraints on the integrated Sachs-Wolfe (ISW) effect through cross-correlation of the cosmic microwave background with galaxy surveys. We improve with respect to similar previous analyses in several ways. First, we use the most recent versions of extragalactic object catalogs, SDSS DR12 photometric redshift (photo-z ) and 2MASS Photo-z data sets, as well as those employed earlier for ISW, SDSS QSO photo-z and NVSS samples. Second, we use for the first time the WISE × SuperCOSMOS catalog, which allows us to perform an all-sky analysis of the ISW up to z ˜0.4 . Third, thanks to the use of photo-z s , we separate each data set into different redshift bins, deriving the cross-correlation in each bin. This last step leads to a significant improvement in sensitivity. We remove cross-correlation between catalogs using masks which mutually exclude common regions of the sky. We use two methods to quantify the significance of the ISW effect. In the first one, we fix the cosmological model, derive linear galaxy biases of the catalogs, and then evaluate the significance of the ISW using a single parameter. In the second approach we perform a global fit of the ISW and of the galaxy biases varying the cosmological model. We find significances of the ISW in the range 4.7 - 5.0 σ thus reaching, for the first time in such an analysis, the threshold of 5 σ . Without the redshift tomography we find a significance of ˜4.0 σ , which shows the importance of the binning method. Finally we use the ISW data to infer constraints on the dark energy redshift evolution and equation of state. We find that the redshift range covered by the catalogs is still not optimal to derive strong constraints, although this goal will be likely reached using future datasets such as from Euclid, LSST, and SKA.

  11. Planck 2015 results: XXI. The integrated Sachs-Wolfe effect

    International Nuclear Information System (INIS)

    Ade, P. A. R.; Aghanim, N.; Arnaud, M.; Ashdown, M.; Aumont, J.

    2016-01-01

    Here, this paper presents a study of the integrated Sachs-Wolfe (ISW) effect from the Planck 2015 temperature and polarization data release. This secondary cosmic microwave background (CMB) anisotropy caused by the large-scale time-evolving gravitational potential is probed from different perspectives. The CMB is cross-correlated with different large-scale structure (LSS) tracers: radio sources from the NVSS catalogue; galaxies from the optical SDSS and the infrared WISE surveys; and the Planck 2015 convergence lensing map. The joint cross-correlation of the CMB with the tracers yields a detection at 4σ where most of the signal-to-noise is due to the Planck lensing and the NVSS radio catalogue. In fact, the ISW effect is detected from the Planck data only at ≈3σ (through the ISW-lensing bispectrum), which is similar to the detection level achieved by combining the cross-correlation signal coming from all the galaxy catalogues mentioned above. We study the ability of the ISW effect to place constraints on the dark-energy parameters; in particular, we show that Ω Λ is detected at more than 3σ. This cross-correlation analysis is performed only with the Planck temperature data, since the polarization scales available in the 2015 release do not permit significant improvement of the CMB-LSS cross-correlation detectability. Nevertheless, the Planck polarization data are used to study the anomalously large ISW signal previously reported through the aperture photometry on stacked CMB features at the locations of known superclusters and supervoids, which is in conflict with ΛCDM expectations. We find that the current Planck polarization data do not exclude that this signal could be caused by the ISW effect. In addition, the stacking of the Planck lensing map on the locations of superstructures exhibits a positive cross-correlation with these large-scale structures. Finally, we have improved our previous reconstruction of the ISW temperature fluctuations by combining

  12. Fermat Potentials of Embedded Lensing, the Integrated Sachs-Wolfe Effect, and Weak-Lensing of CMB by Cosmic Voids

    Science.gov (United States)

    Chen, Bin; Kantowski, R.; Dai, X.

    2014-01-01

    We have developed an accurate gravitational lens theory for an inhomogeneity embedded in an otherwise homogeneous universe, which to the lowest order is applicable to any mass distribution. We derive the Fermat potential for a spherically symmetric lens embedded in a FLRW cosmology and use it to investigate the late-time integrated Sachs-Wolfe effect (ISW) caused by individual large scale inhomogeneities, in particular, cosmic voids. We present a simple analytical expression for the CMB temperature fluctuation across such a lens as the derivative of the lens Fermat potential. Our formalism is applicable to both linear and nonlinear density evolution scenarios, to arbitrarily large density contrasts, and to all open and closed background cosmologies. Our results are particularly useful for modeling ISW effects extracted through stacking large numbers of cosmic voids and clusters (that is, the aperture photometry method). For structures co-expanding with the background cosmology, i.e., for time-independent density contrasts, we find that the gravitational lensing time delay alone can produce fluctuations of the order of seen in recent observations by WMAP and Planck. We revisit the possibility of explaining the non-Gaussian cold spot on the south hemisphere via the Rees-Sciama effect of a large cosmic void using constraints obtained from the most recent void catalogs and our new void-lensing formalism, and compare it with other explanations such as a collapsing cosmic texture. We also study the remapping of primordial CMB anisotropies, the weak-lensing shear, and magnification caused by void lensing.

  13. SAChES: Scalable Adaptive Chain-Ensemble Sampling.

    Energy Technology Data Exchange (ETDEWEB)

    Swiler, Laura Painton [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Ray, Jaideep [Sandia National Lab. (SNL-CA), Livermore, CA (United States); Ebeida, Mohamed Salah [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Huang, Maoyi [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Hou, Zhangshuan [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Bao, Jie [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Ren, Huiying [Pacific Northwest National Lab. (PNNL), Richland, WA (United States)

    2017-08-01

    We present the development of a parallel Markov Chain Monte Carlo (MCMC) method called SAChES, Scalable Adaptive Chain-Ensemble Sampling. This capability is targed to Bayesian calibration of com- putationally expensive simulation models. SAChES involves a hybrid of two methods: Differential Evo- lution Monte Carlo followed by Adaptive Metropolis. Both methods involve parallel chains. Differential evolution allows one to explore high-dimensional parameter spaces using loosely coupled (i.e., largely asynchronous) chains. Loose coupling allows the use of large chain ensembles, with far more chains than the number of parameters to explore. This reduces per-chain sampling burden, enables high-dimensional inversions and the use of computationally expensive forward models. The large number of chains can also ameliorate the impact of silent-errors, which may affect only a few chains. The chain ensemble can also be sampled to provide an initial condition when an aberrant chain is re-spawned. Adaptive Metropolis takes the best points from the differential evolution and efficiently hones in on the poste- rior density. The multitude of chains in SAChES is leveraged to (1) enable efficient exploration of the parameter space; and (2) ensure robustness to silent errors which may be unavoidable in extreme-scale computational platforms of the future. This report outlines SAChES, describes four papers that are the result of the project, and discusses some additional results.

  14. Tay-Sachs - resources

    Science.gov (United States)

    The following organizations provide information on Tay-Sachs disease : US National Library of Medicine -- ghr.nlm.nih.gov/condition/tay-sachs-disease March of Dimes -- www.marchofdimes.org/complications/tay-sachs- ...

  15. Tay-Sachs Disease

    Science.gov (United States)

    Tay-Sachs disease is a rare, inherited disease. It is a type of lipid metabolism disorder. It causes too ... cells, causing mental and physical problems. . Infants with Tay-Sachs disease appear to develop normally for the first few ...

  16. Survey on serum acetylecholinesterase (SAChE activity in selected farming communities at risk of organophosphate exposure, Southern Ghana

    Directory of Open Access Journals (Sweden)

    Ato Kwamena Tetteh

    2017-12-01

    Material and Methods ― The study was cross-sectional, involving 963 participants who consented to being screened for serum acetylcholinesterase (SAChE activity, using the dry chemistry test strips. The SAChE activity was then categorized into normal, low or high risk of developing symptoms associated with OP poisoning. Results ― The total group included 54.5% females and 45.5% males. Majority were crop farmers (46.3% and students/pupils (30.8%. The remaining 22.9% consisted mainly of traders, teachers, fishermen/fish processors, drivers and dressmakers. Very low SAChE activity unit of 30 U/ml. By this, a significant majority had either low or high risk of developing OP poisoning (χ2=4.43, 95%CI: 4.12–4.65, p=0.014 compared with those with normal activity. Most of the participants were in the 10–19 years age group of which 15.6% were in the high risk category, while 13.6% had low risk of OP poisoning. Reduced SAChE activity was predominant among all age groups compared with those with normal activity of the enzyme. As well, 27.3% who were females had reduced SAChE activity rate <30 U/ml (high risk of OP poisoning. Similarly, 23.4% of males were also at high risk of OP poisoning. There was however no statistical significance between the sex with regards to the numbers at risk of OP poisoning (χ2=4.80, 95% CI: 4.25–4.75, p=0.160. Conclusion ― This study provides some evidence of exposure, deduced from reduced SAChE activity, which potentially puts participants at a high risk of developing symptoms associated with OP poisoning. A follow-up study might be able to suggest to physicians in Ghana considering diagnostics of OP poisoning, especially for clients from farming communities where pesticides are used extensively.

  17. The metabolism of Tay-Sachs ganglioside: catabolic studies with lysosomal enzymes from normal and Tay-Sachs brain tissue

    Science.gov (United States)

    Tallman, John F.; Johnson, William G.; Brady, Roscoe O.

    1972-01-01

    The catabolism of Tay-Sachs ganglioside, N-acetylgalactosaminyl- (N-acetylneuraminosyl) -galactosylglucosylceramide, has been studied in lysosomal preparations from normal human brain and brain obtained at biopsy from Tay-Sachs patients. Utilizing Tay-Sachs ganglioside labeled with 14C in the N-acetylgalactosaminyl portion or 3H in the N-acetylneuraminosyl portion, the catabolism of Tay-Sachs ganglioside may be initiated by either the removal of the molecule of N-acetylgalactosamine or N-acetylneuraminic acid. The activity of the N-acetylgalactosamine-cleaving enzyme (hexosaminidase) is drastically diminished in such preparations from Tay-Sachs brain whereas the activity of the N-acetylneuraminic acid-cleaving enzyme (neuraminidase) is at a normal level. Total hexosaminidase activity as measured with an artificial fluorogenic substrate is increased in tissues obtained from patients with the B variant form of Tay-Sachs disease and it is virtually absent in the O-variant patients. The addition of purified neuraminidase and various purified hexosaminidases exerted only a minimal synergistic effect on the hydrolysis of Tay-Sachs ganglioside in the lysosomal preparations from the control or patient with the O variant of Tay-Sachs disease. Images PMID:4639018

  18. A Dantzig-Wolfe Decomposition Algorithm for Linear Economic MPC of a Power Plant Portfolio

    DEFF Research Database (Denmark)

    Standardi, Laura; Edlund, Kristian; Poulsen, Niels Kjølstad

    2012-01-01

    Future power systems will consist of a large number of decentralized power producers and a large number of controllable power consumers in addition to stochastic power producers such as wind turbines and solar power plants. Control of such large scale systems requires new control algorithms....... In this paper, we formulate the control of such a system as an Economic Model Predictive Control (MPC) problem. When the power producers and controllable power consumers have linear dynamics, the Economic MPC may be expressed as a linear program and we apply Dantzig-Wolfe decomposition for solution...

  19. Ethical Issues with Genetic Testing for Tay-Sachs.

    Science.gov (United States)

    Clayton, Tricia

    Several genetic disorders are specific to Jewish heritage; one of the most devastating is Tay-Sachs disease.Tay-Sachs is a fatal hereditary disease, causing progressive neurological problems for which there is no cure. Ethical issues surrounding genetic testing for Tay-Sachs within the Jewish community continue to be complex and multifaceted. A perspective of Tay-Sachs, using rights-based ethics and virtue ethics as a theoretical framework, is explored.

  20. Basic versus applied research: Julius Sachs (1832-1897) and the experimental physiology of plants.

    Science.gov (United States)

    Kutschera, Ulrich

    2015-01-01

    The German biologist Julius Sachs was the first to introduce controlled, accurate, quantitative experimentation into the botanical sciences, and is regarded as the founder of modern plant physiology. His seminal monograph Experimental-Physiologie der Pflanzen (Experimental Physiology of Plants) was published 150 y ago (1865), when Sachs was employed as a lecturer at the Agricultural Academy in Poppelsdorf/Bonn (now part of the University). This book marks the beginning of a new era of basic and applied plant science. In this contribution, I summarize the achievements of Sachs and outline his lasting legacy. In addition, I show that Sachs was one of the first biologists who integrated bacteria, which he considered to be descendants of fungi, into the botanical sciences and discussed their interaction with land plants (degradation of wood etc.). This "plant-microbe-view" of green organisms was extended and elaborated by the laboratory botanist Wilhelm Pfeffer (1845-1920), so that the term "Sachs-Pfeffer-Principle of Experimental Plant Research" appears to be appropriate to characterize this novel way of performing scientific studies on green, photoautotrophic organisms (embryophytes, algae, cyanobacteria).

  1. Prenatal Diagnosis Of Tay-Sachs Disease

    Directory of Open Access Journals (Sweden)

    Özgür Özyüncü

    2010-04-01

    CONCLUSION: Tay-Sachs disease can be diagnosed prenatally by measuring hexosaminidase enzyme activity in fetal tissue samples with an acceptable complication rate. Prenatal diagnosis should be offered to families who have affected siblings with Tay-Sachs disease.

  2. Energy in the Kantowski–Sachs space-time using teleparallel ...

    Indian Academy of Sciences (India)

    Energy in the Kantowski–Sachs space-time using teleparallel geometry ... Kantowski–Sachs metric; teleparallelism; gravitational energy. Abstract. The purpose of this paper is to examine the energy content of the inflationary Universe described by Kantowski–Sachs space-time in quasilocal approach of teleparallel gravity ...

  3. The bystander effect model of Brenner and Sachs fitted to lung cancer data in 11 cohorts of underground miners, and equivalence of fit of a linear relative risk model with adjustment for attained age and age at exposure

    International Nuclear Information System (INIS)

    Little, M P

    2004-01-01

    Bystander effects following exposure to α-particles have been observed in many experimental systems, and imply that linearly extrapolating low dose risks from high dose data might materially underestimate risk. Brenner and Sachs (2002 Int. J. Radiat. Biol. 78 593-604; 2003 Health Phys. 85 103-8) have recently proposed a model of the bystander effect which they use to explain the inverse dose rate effect observed for lung cancer in underground miners exposed to radon daughters. In this paper we fit the model of the bystander effect proposed by Brenner and Sachs to 11 cohorts of underground miners, taking account of the covariance structure of the data and the period of latency between the development of the first pre-malignant cell and clinically overt cancer. We also fitted a simple linear relative risk model, with adjustment for age at exposure and attained age. The methods that we use for fitting both models are different from those used by Brenner and Sachs, in particular taking account of the covariance structure, which they did not, and omitting certain unjustifiable adjustments to the miner data. The fit of the original model of Brenner and Sachs (with 0 y period of latency) is generally poor, although it is much improved by assuming a 5 or 6 y period of latency from the first appearance of a pre-malignant cell to cancer. The fit of this latter model is equivalent to that of a linear relative risk model with adjustment for age at exposure and attained age. In particular, both models are capable of describing the observed inverse dose rate effect in this data set

  4. Structural consequences of amino acid substitutions causing Tay-Sachs disease.

    Science.gov (United States)

    Ohno, Kazuki; Saito, Seiji; Sugawara, Kanako; Sakuraba, Hitoshi

    2008-08-01

    To determine the structural changes in the alpha-subunit of beta-hexosaminidase due to amino acid substitutions causing Tay-Sachs disease, we built structural models of mutant alpha-subunits resulting from 33 missense mutations (24 infantile and 9 late-onset), and analyzed the influence of each amino acid replacement on the structure by calculating the number of atoms affected and determining the solvent-accessible surface area of the corresponding amino acid residue in the wild-type alpha-subunit. In the infantile Tay-Sachs group, the number of atoms influenced by a mutation was generally larger than that in the late-onset Tay-Sachs group in both the main chain and the side chain, and residues associated with the mutations found in the infantile Tay-Sachs group tended to be less solvent-accessible than those in the late-onset Tay-Sachs group. Furthermore, color imaging determined the distribution and degree of the structural changes caused by representative amino acid substitutions, and that there were also differences between the infantile and late-onset Tay-Sachs disease groups. Structural study is useful for elucidating the basis of Tay-Sachs disease.

  5. A Dantzig-Wolfe decomposition algorithm for linear economic model predictive control of dynamically decoupled subsystems

    DEFF Research Database (Denmark)

    Sokoler, Leo Emil; Standardi, Laura; Edlund, Kristian

    2014-01-01

    This paper presents a warm-started Dantzig–Wolfe decomposition algorithm tailored to economic model predictive control of dynamically decoupled subsystems. We formulate the constrained optimal control problem solved at each sampling instant as a linear program with state space constraints, input...... limits, input rate limits, and soft output limits. The objective function of the linear program is related directly to the cost of operating the subsystems, and the cost of violating the soft output constraints. Simulations for large-scale economic power dispatch problems show that the proposed algorithm...... is significantly faster than both state-of-the-art linear programming solvers, and a structure exploiting implementation of the alternating direction method of multipliers. It is also demonstrated that the control strategy presented in this paper can be tuned using a weighted ℓ1-regularization term...

  6. Planck 2013 results. XII. Diffuse component separation

    DEFF Research Database (Denmark)

    Ade, P. A. R.; Aghanim, N.; Armitage-Caplan, C.

    2014-01-01

    of primordial non-Gaussianity and statistical isotropy, the integrated Sachs-Wolfe effect, gravitational lensing, and searches for topological defects. We test four foreground-cleaned CMB maps derived using qualitatively different component separation algorithms. The quality of our reconstructions is evaluated...

  7. An inducible mouse model of late onset Tay-Sachs disease.

    Science.gov (United States)

    Jeyakumar, Mylvaganam; Smith, David; Eliott-Smith, Elena; Cortina-Borja, Mario; Reinkensmeier, Gabriele; Butters, Terry D; Lemm, Thorsten; Sandhoff, Konrad; Perry, V Hugh; Dwek, Raymond A; Platt, Frances M

    2002-08-01

    Mouse models of the G(M2) gangliosidoses, Tay-Sachs and Sandhoff disease, are null for the hexosaminidase alpha and beta subunits respectively. The Sandhoff (Hexb-/-) mouse has severe neurological disease and mimics the human infantile onset variant. However, the Tay-Sachs (Hexa-/-) mouse model lacks an overt phenotype as mice can partially bypass the blocked catabolic pathway and escape disease. We have investigated whether a subset of Tay-Sachs mice develop late onset disease. We have found that approximately 65% of the mice develop one or more clinical signs of the disease within their natural life span (n = 52, P disease at an earlier age (n = 21, P Tay-Sachs mice confirmed that pregnancy induces late onset Tay-Sachs disease. Onset of symptoms correlated with reduced up-regulation of hexosaminidase B, a component of the bypass pathway.

  8. Recent results and perspectives on cosmology and fundamental physics from microwave surveys

    DEFF Research Database (Denmark)

    Burigana, Carlo; Battistelli, Elia Stefano; Benetti, Micol

    2016-01-01

    Recent cosmic microwave background (CMB) data in temperature and polarization have reached high precision in estimating all the parameters that describe the current so-called standard cosmological model. Recent results about the integrated Sachs-Wolfe (ISW) effect from CMB anisotropies, galaxy su...

  9. The National Tay Sachs and Allied Diseases Association.

    Science.gov (United States)

    Zeitlin, Paula

    1986-01-01

    The National Tay-Sachs and Allied Diseases Association is involved in education, research, and prevention of Tay-Sachs, an inherited metabolic disorder which destroys the central nervous system, and over 30 related disorders. The group features a parent peer group network and a support group for carrier couples. (CL)

  10. National Tay-Sachs and Allied Diseases Association, Inc.

    Science.gov (United States)

    Exceptional Parent, 1977

    1977-01-01

    Reviewed are the history and organization, purpose and programs, and public services of the National Tay-Sachs and Allied Diseases Association, an organization geared toward eradicating Tay-Sachs disease (a hereditary disorder affecting primarily Jewish infants which generally leads to deterioration and death by the child's fifth year). (SBH)

  11. [Tay-Sachs disease in non-Jewish infant in Israel].

    Science.gov (United States)

    Nadim, Nasser

    2012-01-01

    Tay-Sachs disease, also known as GM2 gangliosidosis or Hexosaminidase A deficiency is an autosomal recessive genetic fatal disorder. The disease is known to appear in East European Ashkenazi Jews, North African Jews, and Quebec French Canadians exclusively, but, with different frequency and type of mutation. Its most common variant is the infantile type Tay-Sachs disease. Juvenile and late-onset forms of the disease are infrequent and slowly progressive. At nearly 3 to 6 months old, a baby with Tay-Sachs progressively loses his motor skills and attentiveness. Startle responses and hyperreflexia become prominent, especially on eliciting deep patellar and Achilles reflexes, as a consequence of neurodegeneration of the upper motor neuron. Other systemic damage ensues gradually; seizures, blindness, spasticity of limbs, inability to swallow and breathe, and eventually the baby dies at 1-4 years of age. All Tay-Sachs patients have a "cherry red spot", easily seen in the macula area of the retina, using an ophthalmoscope. The "cherry red spot" is the only normal part of the retina in these sick babies. The case presented here emphasizes that Tay-Sachs disease is sometimes misdiagnosed at first visits even by an experienced clinician, because of his lack of awareness that this disease is not exclusively a Jewish disease.

  12. Planck 2015 results. XXI. The integrated Sachs-Wolfe effect

    CERN Document Server

    Ade, P.A.R.; Arnaud, M.; Ashdown, M.; Aumont, J.; Baccigalupi, C.; Banday, A.J.; Barreiro, R.B.; Bartolo, N.; Basak, S.; Battaner, E.; Benabed, K.; Benoît, A.; Benoit-Lévy, A.; Bernard, J.-P.; Bersanelli, M.; Bielewicz, P.; Bock, J.J.; Bonaldi, A.; Bonavera, L.; Bond, J.R.; Borrill, J.; Bouchet, F.R.; Bucher, M.; Burigana, C.; Butler, R.C.; Calabrese, E.; Cardoso, J.-F.; Casaponsa, B.; Catalano, A.; Challinor, A.; Chamballu, A.; Chiang, H.C.; Christensen, P.R.; Church, S.; Clements, D.L.; Colombi, S.; Colombo, L.P.L.; Combet, C.; Couchot, F.; Coulais, A.; Crill, B.P.; Curto, A.; Cuttaia, F.; Danese, L.; Davies, R.D.; Davis, R.J.; de Bernardis, P.; de Rosa, A.; de Zotti, G.; Delabrouille, J.; Désert, F.-X.; Diego, J.M.; Dole, H.; Donzelli, S.; Doré, O.; Douspis, M.; Ducout, A.; Dupac, X.; Efstathiou, G.; Elsner, F.; Enßlin, T.A.; Eriksen, H.K.; Fergusson, J.; Fernandez-Cobos, R.; Finelli, F.; Forni, O.; Frailis, M.; Fraisse, A.A.; Franceschi, E.; Frejsel, A.; Galeotta, S.; Galli, S.; Ganga, K.; Génova-Santos, R.T.; Giard, M.; Giraud-Héraud, Y.; Gjerløw, E.; González-Nuevo, J.; Górski, K.M.; Gratton, S.; Gregorio, A.; Gruppuso, A.; Gudmundsson, J.E.; Hansen, F.K.; Hanson, D.; Harrison, D.L.; Henrot-Versillé, S.; Hernández-Monteagudo, C.; Herranz, D.; Hildebrandt, S.R.; Hivon, E.; Hobson, M.; Holmes, W.A.; Hornstrup, A.; Hovest, W.; Huffenberger, K.M.; Hurier, G.; Ilić, S.; Jaffe, A.H.; Jaffe, T.R.; Jones, W.C.; Juvela, M.; Keihänen, E.; Keskitalo, R.; Kisner, T.S.; Kneissl, R.; Knoche, J.; Kunz, M.; Kurki-Suonio, H.; Lagache, G.; Lähteenmäki, A.; Lamarre, J.-M.; Langer, M.; Lasenby, A.; Lattanzi, M.; Lawrence, C.R.; Leonardi, R.; Lesgourgues, J.; Levrier, F.; Liguori, M.; Lilje, P.B.; Linden-Vørnle, M.; López-Caniego, M.; Lubin, P.M.; Ma, Y.-Z.; Macías-Pérez, J.F.; Maggio, G.; Maino, D.; Mandolesi, N.; Mangilli, A.; Marcos-Caballero, A.; Maris, M.; Martin, P.G.; Martínez-González, E.; Masi, S.; Matarrese, S.; McGehee, P.; Meinhold, P.R.; Melchiorri, A.; Mendes, L.; Mennella, A.; Migliaccio, M.; Mitra, S.; Miville-Deschênes, M.-A.; Moneti, A.; Montier, L.; Morgante, G.; Mortlock, D.; Moss, A.; Munshi, D.; Murphy, J.A.; Naselsky, P.; Nati, F.; Natoli, P.; Netterfield, C.B.; Nørgaard-Nielsen, H.U.; Noviello, F.; Novikov, D.; Novikov, I.; Oxborrow, C.A.; Paci, F.; Pagano, L.; Pajot, F.; Paoletti, D.; Pasian, F.; Patanchon, G.; Perdereau, O.; Perotto, L.; Perrotta, F.; Pettorino, V.; Piacentini, F.; Piat, M.; Pierpaoli, E.; Pietrobon, D.; Plaszczynski, S.; Pointecouteau, E.; Polenta, G.; Popa, L.; Pratt, G.W.; Prézeau, G.; Prunet, S.; Puget, J.-L.; Rachen, J.P.; Reach, W.T.; Rebolo, R.; Reinecke, M.; Remazeilles, M.; Renault, C.; Renzi, A.; Ristorcelli, I.; Rocha, G.; Rosset, C.; Rossetti, M.; Roudier, G.; Rubiño-Martín, J.A.; Rusholme, B.; Sandri, M.; Santos, D.; Savelainen, M.; Savini, G.; Schaefer, B.M.; Scott, D.; Seiffert, M.D.; Shellard, E.P.S.; Spencer, L.D.; Stolyarov, V.; Stompor, R.; Sudiwala, R.; Sunyaev, R.; Sutton, D.; Suur-Uski, A.-S.; Sygnet, J.-F.; Tauber, J.A.; Terenzi, L.; Toffolatti, L.; Tomasi, M.; Tristram, M.; Tucci, M.; Tuovinen, J.; Valenziano, L.; Valiviita, J.; Van Tent, F.; Vielva, P.; Villa, F.; Wade, L.A.; Wandelt, B.D.; Wehus, I.K.; Yvon, D.; Zacchei, A.

    2016-01-01

    This paper presents a study of the ISW effect from the Planck 2015 temperature and polarization data release. The CMB is cross-correlated with different LSS tracers: the NVSS, SDSS and WISE catalogues, and the Planck 2015 convergence lensing map. This cross-correlation yields a detection at $4\\,\\sigma$, where most of the signal-to-noise is due to the Planck lensing and NVSS. In fact, the ISW effect is detected only from the Planck data (through the ISW-lensing bispectrum) at $\\approx 3\\,\\sigma$, which is similar to the detection level achieved by combining the cross-correlation signal coming from all the catalogues. This cross-correlation analysis is performed only with the Planck temperature data, since the polarization scales available in the 2015 release do not permit significant improvement of the CMB-LSS cross-correlation detectability. Nevertheless, polarization data is used to study the anomalously large ISW signal previously reported through the aperture photometry on stacked CMB features at the locat...

  13. Planck 2013 results. XIX. The integrated Sachs-Wolfe effect

    CERN Document Server

    Ade, P.A.R.; Armitage-Caplan, C.; Arnaud, M.; Ashdown, M.; Atrio-Barandela, F.; Aumont, J.; Baccigalupi, C.; Banday, A.J.; Barreiro, R.B.; Bartlett, J.G.; Bartolo, N.; Battaner, E.; Benabed, K.; Benoit, A.; Benoit-Levy, A.; Bernard, J.P.; Bersanelli, M.; Bielewicz, P.; Bobin, J.; Bock, J.J.; Bonaldi, A.; Bonavera, L.; Bond, J.R.; Borrill, J.; Bouchet, F.R.; Bridges, M.; Bucher, M.; Burigana, C.; Butler, R.C.; Cardoso, J.F.; Catalano, A.; Challinor, A.; Chamballu, A.; Chiang, L.Y.; Chiang, H.C.; Christensen, P.R.; Church, S.; Clements, D.L.; Colombi, S.; Colombo, L.P.L.; Couchot, F.; Coulais, A.; Crill, B.P.; Curto, A.; Cuttaia, F.; Danese, L.; Davies, R.D.; Davis, R.J.; de Bernardis, P.; de Rosa, A.; de Zotti, G.; Delabrouille, J.; Delouis, J.M.; Desert, F.X.; Dickinson, C.; Diego, J.M.; Dolag, K.; Dole, H.; Donzelli, S.; Dore, O.; Douspis, M.; Dupac, X.; Efstathiou, G.; Ensslin, T.A.; Eriksen, H.K.; Fergusson, J.; Finelli, F.; Forni, O.; Fosalba, P.; Frailis, M.; Franceschi, E.; Frommert, M.; Galeotta, S.; Ganga, K.; Genova-Santos, R.T.; Giard, M.; Giardino, G.; Giraud-Heraud, Y.; Gonzalez-Nuevo, J.; Gorski, K.M.; Gratton, S.; Gregorio, A.; Gruppuso, A.; Hansen, F.K.; Hanson, D.; Harrison, D.; Henrot-Versille, S.; Hernandez-Monteagudo, C.; Herranz, D.; Hildebrandt, S.R.; Hivon, E.; Ho, S.; Hobson, M.; Holmes, W.A.; Hornstrup, A.; Hovest, W.; Huffenberger, K.M.; Ilic, S.; Jaffe, T.R.; Jaffe, A.H.; Jasche, J.; Jones, W.C.; Juvela, M.; Keihanen, E.; Keskitalo, R.; Kisner, T.S.; Knoche, J.; Knox, L.; Kunz, M.; Kurki-Suonio, H.; Lagache, G.; Lahteenmaki, A.; Lamarre, J.M.; Langer, M.; Lasenby, A.; Laureijs, R.J.; Lawrence, C.R.; Leahy, J.P.; Leonardi, R.; Lesgourgues, J.; Liguori, M.; Lilje, P.B.; Linden-Vornle, M.; Lopez-Caniego, M.; Lubin, P.M.; Macias-Perez, J.F.; Maffei, B.; Maino, D.; Mandolesi, N.; Mangilli, A.; Marcos-Caballero, A.; Maris, M.; Marshall, D.J.; Martin, P.G.; Martinez-Gonzalez, E.; Masi, S.; Matarrese, S.; Matthai, F.; Mazzotta, P.; Meinhold, P.R.; Melchiorri, A.; Mendes, L.; Mennella, A.; Migliaccio, M.; Mitra, S.; Miville-Deschenes, M.A.; Moneti, A.; Montier, L.; Morgante, G.; Mortlock, D.; Moss, A.; Munshi, D.; Naselsky, P.; Nati, F.; Natoli, P.; Netterfield, C.B.; Norgaard-Nielsen, H.U.; Noviello, F.; Novikov, D.; Novikov, I.; Osborne, S.; Oxborrow, C.A.; Paci, F.; Pagano, L.; Pajot, F.; Paoletti, D.; Partridge, B.; Pasian, F.; Patanchon, G.; Perdereau, O.; Perotto, L.; Perrotta, F.; Piacentini, F.; Piat, M.; Pierpaoli, E.; Pietrobon, D.; Plaszczynski, S.; Pointecouteau, E.; Polenta, G.; Ponthieu, N.; Popa, L.; Poutanen, T.; Pratt, G.W.; Prezeau, G.; Prunet, S.; Puget, J.L.; Rachen, J.P.; Racine, B.; Rebolo, R.; Reinecke, M.; Remazeilles, M.; Renault, C.; Renzi, A.; Ricciardi, S.; Riller, T.; Ristorcelli, I.; Rocha, G.; Rosset, C.; Roudier, G.; Rowan-Robinson, M.; Rubino-Martin, J.A.; Rusholme, B.; Sandri, M.; Santos, D.; Savini, G.; Schaefer, B.M.; Schiavon, F.; Scott, D.; Seiffert, M.D.; Shellard, E.P.S.; Spencer, L.D.; Starck, J.L.; Stolyarov, V.; Stompor, R.; Sudiwala, R.; Sunyaev, R.; Sureau, F.; Sutton, D.; Suur-Uski, A.S.; Sygnet, J.F.; Tauber, J.A.; Tavagnacco, D.; Terenzi, L.; Toffolatti, L.; Tomasi, M.; Tristram, M.; Tucci, M.; Tuovinen, J.; Umana, G.; Valenziano, L.; Valiviita, J.; Van Tent, B.; Varis, J.; Viel, M.; Vielva, P.; Villa, F.; Vittorio, N.; Wade, L.A.; Wandelt, B.D.; White, M.; Xia, J.Q.; Yvon, D.; Zacchei, A.; Zonca, A.

    2014-01-01

    Based on CMB maps from the 2013 Planck Mission data release, this paper presents the detection of the ISW effect, i.e., the correlation between the CMB and large-scale evolving gravitational potentials. The significance of detection ranges from 2 to 4 sigma, depending on which method is used. We investigate three separate approaches, which cover essentially all previous studies, as well as breaking new ground. (i) Correlation of the CMB with the Planck reconstructed gravitational lensing potential (for the first time). This detection is made using the lensing-induced bispectrum; the correlation between lensing and the ISW effect has a significance close to 2.5 sigma. (ii) Cross-correlation with tracers of LSS, yielding around 3 sigma significance, based on a combination of radio (NVSS) and optical (SDSS) data. (iii) Aperture photometry on stacked CMB fields at the locations of known large-scale structures, which yields a 4 sigma signal when using a previously explored catalogue, but shows strong discrepancies...

  14. Planck 2013 results. XIX. The integrated Sachs-Wolfe effect

    DEFF Research Database (Denmark)

    Ade, P. A. R.; Aghanim, N.; Armitage-Caplan, C.

    2014-01-01

    they can be compared, these measurements are compatible with previous work using data from WMAP, where these scales have been mapped to the limits of cosmic variance. Planck's broader frequency coverage allows for better foreground cleaning and confirms that the signal is achromatic, which makes...

  15. A 2D view of Wolf-Rayet Galaxies

    OpenAIRE

    Srivastava, S.; Kehrig, C.; Kantharia, N. G.; Pérez-Montero, E.; Vílchez, J. M.; Iglesias-Páramo, J.; Janardhan, P.

    2015-01-01

    The main objective of this work is to investigate the evolution of massive stars, and the interplay between them and the ionized gas for a sample of local metal-poor Wolf-Rayet galaxies. Optical integral field spectrocopy was used in combination with multi-wavelength radio data. Combining optical and radio data, we locate Wolf-Rayet stars and supernova remnants across the Wolf-Rayet galaxies to study the spatial correlation between them. This study will shed light on the massive star fo...

  16. No N = 4 strings on Wolf spaces

    International Nuclear Information System (INIS)

    Gates, S.J. Jr.; Ketov, S.V.

    1995-02-01

    We generalize the standard N=2 supersymmetric Kazama-Suzuki coset construction to the N=4 case by requiring the non-linear (Goddard-Schwimmer) N=4 quasi-superconformal algebra to be realized on cosets. The constraints that we find allow very simple geometrical interpretation and have the Wolf spaces as their natural solutions. Our results obtained by using components-level superconformal field theory methods are fully consistent with standard results about N=4 supersymmetric two-dimensional nonlinear sigma-models and N=4 WZNW models on Wolf spaces. We construct the actions for the latter and express the quaternionic structure, appearing in the N=4 coset solution, in terms of the symplectic structure associated with the underlying Freudenthal triple system. Next, we gauge the N=4 QSCA and build a quantum BRST charge for the N=4 string propagating on a Wolf space. Surprisingly, the BRST charge nilpotency conditions rule out the non-trivial Wolf spaces as consistent string backgrounds. (orig.)

  17. Rapid detection of fetal Mendelian disorders: Tay-Sachs disease.

    Science.gov (United States)

    Guetta, Esther; Peleg, Leah

    2008-01-01

    Tay-Sachs disease is an autosomal recessive storage disease caused by the impaired activity of the lysosomal enzyme hexosaminidase A. In this fatal disease, the sphingolipid GM2 ganglioside accumulates in the neurons. Due to high carrier rates and the severity of the disease, population screening and prenatal diagnosis of Tay-Sachs disease are routinely carried out in Israel. Laboratory diagnosis of Tay-Sachs is carried out with biochemical and DNA-based methods in peripheral and umbilical cord blood, amniotic fluid, and chorionic villi samples. The assay of hexosaminidase A (Hex A) activity is carried out with synthetic substrates, 4-methylumbelliferyl-6-sulfo-N-acetyl-beta-glucosaminide (4-MUGS) and 4-methylumbelliferil-N-acetyl-beta-glucosamine (4-MUG), and the DNA-based analysis involves testing for the presence of specific known mutations in the alpha-subunit gene of Hex A. Prenatal diagnosis of Tay-Sachs disease is accomplished within 24-48 h from sampling. The preferred strategy is to simultaneously carry out enzymatic analysis in the amniotic fluid supernatant or in chorionic villi and molecular DNA-based testing in an amniotic fluid cell-pellet or in chorionic villi.

  18. Tay-Sachs and Sandhoff Diseases

    Science.gov (United States)

    ... more common in these groups: Creole of northern Argentina Metis Indians in Saskatchewan, Canada Lebanese If you or your partner is part of these ethnic groups, or you have a family history of Tay-Sachs disease or Sandhoff disease, tell ...

  19. Enfermedad de Tay-Sachs

    Directory of Open Access Journals (Sweden)

    Albia Josefina Pozo Alonso

    Full Text Available La enfermedad de Tay-Sachs es un trastorno neurodegenerativo progresivo de herencia autosómica recesiva. Se debe a la deficiencia de la enzima β-hexosaminidasa A, que provoca una acumulación de gangliósidos GM2 en los lisosomas. Se incluye dentro de las esfingolipidosis. De las esfingolipidosis que presentan mancha rojo cereza en la mácula, la enfermedad de Tay-Sachs es la única en la que no se evidencia hepatoesplenomegalia. La variante más frecuente se inicia en la lactancia. Se presenta un lactante del sexo masculino al que se le realizó el diagnóstico de esta entidad a los 8 meses de edad. A partir de los 4 meses comenzó a presentar una reacción de sobresalto. A los 6 meses comenzó a perder habilidades previamente adquiridas y crisis epilépticas mioclónicas. Se constató una disminución de la actividad específica de la enzima hexosaminidasa A en leucocitos.

  20. Camera Traps on Wildlife Crossing Structures as a Tool in Gray Wolf (Canis lupus) Management - Five-Years Monitoring of Wolf Abundance Trends in Croatia.

    Science.gov (United States)

    Šver, Lidija; Bielen, Ana; Križan, Josip; Gužvica, Goran

    2016-01-01

    The conservation of gray wolf (Canis lupus) and its coexistence with humans presents a challenge and requires continuous monitoring and management efforts. One of the non-invasive methods that produces high-quality wolf monitoring datasets is camera trapping. We present a novel monitoring approach where camera traps are positioned on wildlife crossing structures that channel the animals, thereby increasing trapping success and increasing the cost-efficiency of the method. In this way we have followed abundance trends of five wolf packs whose home ranges are intersected by a motorway which spans throughout the wolf distribution range in Croatia. During the five-year monitoring of six green bridges we have recorded 28 250 camera-events, 132 with wolves. Four viaducts were monitored for two years, recording 4914 camera-events, 185 with wolves. We have detected a negative abundance trend of the monitored Croatian wolf packs since 2011, especially severe in the northern part of the study area. Further, we have pinpointed the legal cull as probable major negative influence on the wolf pack abundance trends (linear regression, r2 > 0.75, P Croatia until there is more data proving population stability. In conclusion, quantitative methods, such as the one presented here, should be used as much as possible when assessing wolf abundance trends.

  1. Proton MR spectroscopy in three children with Tay-Sachs disease.

    Science.gov (United States)

    Aydin, Kubilay; Bakir, Baris; Tatli, Burak; Terzibasioglu, Ege; Ozmen, Meral

    2005-11-01

    Tay-Sachs disease is an inherited metabolic disease caused by the accumulation of GM(2) gangliosides in the central nervous system. Deficiency of hexosaminidase A leads to the accumulation of gangliosides in neurons, axons and glial cells. To present the cranial MRI and proton MR spectroscopy findings of children of Tay-Sachs disease. Three children aged 10, 20 and 21 months were examined. On T2-weighted MR images there were hyperintense signal changes in the basal ganglia and cerebral white matter. MR spectroscopy demonstrated an increase in myoinositol/creatine and choline/creatine ratios with a decrease in the N-acetyl aspartate/creatine ratio. The spectroscopy findings support demyelination, gliosis and neuronal loss in the neuropathological process of Tay-Sachs disease.

  2. Proton MR spectroscopy in three children with Tay-Sachs disease

    Energy Technology Data Exchange (ETDEWEB)

    Aydin, Kubilay; Bakir, Baris; Terzibasioglu, Ege [Istanbul University, Neuroradiology Division, Department of Radiology, Istanbul (Turkey); Tatli, Burak; Ozmen, Meral [Istanbul University, Department of Paediatric Neurology, Istanbul (Turkey)

    2005-11-01

    Tay-Sachs disease is an inherited metabolic disease caused by the accumulation of GM{sub 2} gangliosides in the central nervous system. Deficiency of hexosaminidase A leads to the accumulation of gangliosides in neurons, axons and glial cells. To present the cranial MRI and proton MR spectroscopy findings of children of Tay-Sachs disease. Three children aged 10, 20 and 21 months were examined. On T2-weighted MR images there were hyperintense signal changes in the basal ganglia and cerebral white matter. MR spectroscopy demonstrated an increase in myoinositol/creatine and choline/creatine ratios with a decrease in the N-acetyl aspartate/creatine ratio. The spectroscopy findings support demyelination, gliosis and neuronal loss in the neuropathological process of Tay-Sachs disease. (orig.)

  3. Proton MR spectroscopy in three children with Tay-Sachs disease

    International Nuclear Information System (INIS)

    Aydin, Kubilay; Bakir, Baris; Terzibasioglu, Ege; Tatli, Burak; Ozmen, Meral

    2005-01-01

    Tay-Sachs disease is an inherited metabolic disease caused by the accumulation of GM 2 gangliosides in the central nervous system. Deficiency of hexosaminidase A leads to the accumulation of gangliosides in neurons, axons and glial cells. To present the cranial MRI and proton MR spectroscopy findings of children of Tay-Sachs disease. Three children aged 10, 20 and 21 months were examined. On T2-weighted MR images there were hyperintense signal changes in the basal ganglia and cerebral white matter. MR spectroscopy demonstrated an increase in myoinositol/creatine and choline/creatine ratios with a decrease in the N-acetyl aspartate/creatine ratio. The spectroscopy findings support demyelination, gliosis and neuronal loss in the neuropathological process of Tay-Sachs disease. (orig.)

  4. A critique of Jeffrey D. Sachs's The end of poverty.

    Science.gov (United States)

    Henwood, Doug

    2006-01-01

    Jeffrey Sachs's The End of Poverty is a manifesto and how-to guide on ending extreme poverty around the world; it promotes the U.N. Millennium Development Goals. Sachs achieved fame with his policy package for the "stabilization" of Bolivia (which did nothing to relieve Bolivia's poverty), and became advisor to the Yeltsin government in Russia and to Poland, Slovenia, and Estonia as they began their transitions to capitalism (the last three mixed successes; Russia a thorough disaster). Sachs later became more prominent as a critic of development orthodoxy, and was economic advisor to the Jubilee 2000 movement. The End of Poverty is full of sharp critiques of Western imperialism, but his views on the rest of the development business are more conventional.

  5. Effective gene therapy in an authentic model of Tay-Sachs-related diseases.

    Science.gov (United States)

    Cachón-González, M Begoña; Wang, Susan Z; Lynch, Andrew; Ziegler, Robin; Cheng, Seng H; Cox, Timothy M

    2006-07-05

    Tay-Sachs disease is a prototypic neurodegenerative disease. Lysosomal storage of GM2 ganglioside in Tay-Sachs and the related disorder, Sandhoff disease, is caused by deficiency of beta-hexosaminidase A, a heterodimeric protein. Tay-Sachs-related diseases (GM2 gangliosidoses) are incurable, but gene therapy has the potential for widespread correction of the underlying lysosomal defect by means of the secretion-recapture cellular pathway for enzymatic complementation. Sandhoff mice, lacking the beta-subunit of hexosaminidase, manifest many signs of classical human Tay-Sachs disease and, with an acute course, die before 20 weeks of age. We treated Sandhoff mice by stereotaxic intracranial inoculation of recombinant adeno-associated viral vectors encoding the complementing human beta-hexosaminidase alpha and beta subunit genes and elements, including an HIV tat sequence, to enhance protein expression and distribution. Animals survived for >1 year with sustained, widespread, and abundant enzyme delivery in the nervous system. Onset of the disease was delayed with preservation of motor function; inflammation and GM2 ganglioside storage in the brain and spinal cord was reduced. Gene delivery of beta-hexosaminidase A by using adeno-associated viral vectors has realistic potential for treating the human Tay-Sachs-related diseases.

  6. Sachês antimicrobianos em pós-colheita de manga

    Directory of Open Access Journals (Sweden)

    Eber Antonio Alves Medeiros

    2011-10-01

    Full Text Available Objetivou-se com este trabalho desenvolver sachês incorporados com óleos essenciais avaliando as propriedades físico-químicas e microbiológicas de mangas armazenadas em saco de papel contendo estes sachês em seu interior. Os óleos essenciais de orégano (Origanum vulgaris e capim-limão (Cymbopogon citratus foram incorporados em saches, e sua atividade antimicrobiana foi testada nos fungos Colletotrichum gloeosporides, Lasiodiplodia theobromae, Xanthomonas campestris pv. mangiferae indica, Alternaria alternata. Frutos de manga 'Tommy Atkins' foram acondicionados individualmente em sacos de papel contendo em seu interior um sachê antimicrobiano e mantidos a 25 °C ± 2 °C e UR 80% ± 5% por nove dias. Os parâmetros cor da epiderme, firmeza de polpa, sólidos solúveis, acidez titulável e pH sofreram pouca influência dos óleos essenciais de orégano e capim limão, indicando que a presença do óleo essencial não altera as características físico-químicas da polpa de manga. Os sachês ativos incorporados com óleos essenciais de orégano e capim limão apresentaram controle no crescimento dos microrganismos testados, sendo o capim-limão mais eficiente, reduzindo em aproximadamente 2 ciclos Log a contagem de mesófilos aeróbios e fungos filamentosos e leveduras em relação ao tratamento controle empregado.

  7. 'Cherry red spot' in a patient with Tay-Sachs disease: case report.

    Science.gov (United States)

    Aragão, Ricardo Evangelista Marrocos de; Ramos, Régia Maria Gondim; Pereira, Felipe Bezerra Alves; Bezerra, Andreya Ferreira Rodrigues; Fernandes, Daniel Nogueira

    2009-01-01

    Tay-Sachs disease is an autosomal recessive disorder of sphingolipid metabolism, caused by enzyme hexosaminidase A deficiency that leads to an accumulation of GM2 in neurocytes which results in progressive loss of neurological function. The accumulation of lipid in retinal ganglion cells that leads to a chalk-white appearance of the fundus called 'cherry red spot' is the hallmark of Tay-Sachs disease. It is also seen in others neurometabolic diseases as well as in central retinal artery occlusion. This case reports a child with Tay-Sachs disease in a family with four previous similar deaths without diagnostic.

  8. EMBEDDED LENSING TIME DELAYS, THE FERMAT POTENTIAL, AND THE INTEGRATED SACHS–WOLFE EFFECT

    Energy Technology Data Exchange (ETDEWEB)

    Chen, Bin; Kantowski, Ronald; Dai, Xinyu, E-mail: bchen3@fsu.edu [Homer L. Dodge Department of Physics and Astronomy, University of Oklahoma, Norman, OK 73019 (United States)

    2015-05-01

    We derive the Fermat potential for a spherically symmetric lens embedded in a Friedman–Lemaître–Robertson–Walker cosmology and use it to investigate the late-time integrated Sachs–Wolfe (ISW) effect, i.e., secondary temperature fluctuations in the cosmic microwave background (CMB) caused by individual large-scale clusters and voids. We present a simple analytical expression for the temperature fluctuation in the CMB across such a lens as a derivative of the lens’ Fermat potential. This formalism is applicable to both linear and nonlinear density evolution scenarios, to arbitrarily large density contrasts, and to all open and closed background cosmologies. It is much simpler to use and makes the same predictions as conventional approaches. In this approach the total temperature fluctuation can be split into a time-delay part and an evolutionary part. Both parts must be included for cosmic structures that evolve and both can be equally important. We present very simple ISW models for cosmic voids and galaxy clusters to illustrate the ease of use of our formalism. We use the Fermat potentials of simple cosmic void models to compare predicted ISW effects with those recently extracted from WMAP and Planck data by stacking large cosmic voids using the aperture photometry method. If voids in the local universe with large density contrasts are no longer evolving we find that the time delay contribution alone predicts values consistent with the measurements. However, we find that for voids still evolving linearly, the evolutionary contribution cancels a significant part of the time delay contribution and results in predicted signals that are much smaller than recently observed.

  9. EMBEDDED LENSING TIME DELAYS, THE FERMAT POTENTIAL, AND THE INTEGRATED SACHS–WOLFE EFFECT

    International Nuclear Information System (INIS)

    Chen, Bin; Kantowski, Ronald; Dai, Xinyu

    2015-01-01

    We derive the Fermat potential for a spherically symmetric lens embedded in a Friedman–Lemaître–Robertson–Walker cosmology and use it to investigate the late-time integrated Sachs–Wolfe (ISW) effect, i.e., secondary temperature fluctuations in the cosmic microwave background (CMB) caused by individual large-scale clusters and voids. We present a simple analytical expression for the temperature fluctuation in the CMB across such a lens as a derivative of the lens’ Fermat potential. This formalism is applicable to both linear and nonlinear density evolution scenarios, to arbitrarily large density contrasts, and to all open and closed background cosmologies. It is much simpler to use and makes the same predictions as conventional approaches. In this approach the total temperature fluctuation can be split into a time-delay part and an evolutionary part. Both parts must be included for cosmic structures that evolve and both can be equally important. We present very simple ISW models for cosmic voids and galaxy clusters to illustrate the ease of use of our formalism. We use the Fermat potentials of simple cosmic void models to compare predicted ISW effects with those recently extracted from WMAP and Planck data by stacking large cosmic voids using the aperture photometry method. If voids in the local universe with large density contrasts are no longer evolving we find that the time delay contribution alone predicts values consistent with the measurements. However, we find that for voids still evolving linearly, the evolutionary contribution cancels a significant part of the time delay contribution and results in predicted signals that are much smaller than recently observed

  10. Gravitational corrections to light propagation in a perturbed FLRW universe and corresponding weak-lensing spectra

    Science.gov (United States)

    Cuesta-Lazaro, Carolina; Quera-Bofarull, Arnau; Reischke, Robert; Schäfer, Björn Malte

    2018-06-01

    When the gravitational lensing of the large-scale structure is calculated from a cosmological model a few assumptions enter: (i) one assumes that the photons follow unperturbed background geodesics, which is usually referred to as the Born approximation, (ii) the lenses move slowly, (iii) the source-redshift distribution is evaluated relative to the background quantities, and (iv) the lensing effect is linear in the gravitational potential. Even though these approximations are small individually they could sum up, especially since they include local effects such as the Sachs-Wolfe and peculiar motion, but also non-local ones like the Born approximation and the integrated Sachs-Wolfe effect. In this work, we will address all points mentioned and perturbatively calculate the effect on a tomographic cosmic shear power spectrum of each effect individually as well as all cross-correlations. Our findings show that each effect is at least 4-5 orders of magnitude below the leading order lensing signal. Finally, we sum up all effects to estimate the overall impact on parameter estimation by a future cosmological weak-lensing survey such as Euclid in a wcold dark matter cosmology with parametrization Ωm, σ8, ns, h, w0, and wa, using five tomographic bins. We consistently find a parameter bias of 10-5, which is therefore completely negligible for all practical purposes, confirming that other effects such as intrinsic alignments, magnification bias and uncertainties in the redshift distribution will be the dominant systematic source in future surveys.

  11. Cosmic microwave and infrared backgrounds cross-correlation for ISW detection

    International Nuclear Information System (INIS)

    Ilić, S

    2014-01-01

    We have investigated the cross-correlation between the cosmic infrared and microwave backgrounds (CIB and CMB) anisotropies through the integrated Sachs-Wolfe effect. We have first described the CIB anisotropies using a linearly biased power spectrum, then derive the theoretical angular power spectrum of the CMB-CIB cross-correlation for different instruments and frequencies. We have discussed the detectability of the ISW signal by performing a signal-to-noise (SNR) analysis with our predicted spectra. The significances obtained range from 6σ to 7σ in an ideal case, depending on the frequency; in realistic cases which account for the presence of noise including astrophysical contaminants, the results span the range 2 – 5σ, depending strongly on the major contribution to the noise term

  12. "Cherry red spot" in a patient with Tay-Sachs disease: case report "Mácula em cereja" em paciente com doença de Tay-Sachs: relato de caso

    Directory of Open Access Journals (Sweden)

    Ricardo Evangelista Marrocos de Aragão

    2009-08-01

    Full Text Available Tay-Sachs disease is an autosomal recessive disorder of sphingolipid metabolism, caused by enzime hexosaminidase A deficiency that leads to an accumulation of GM2 in neurocytes which results in progressive loss of neurological function. The accumulation of lipid in retinal ganglion cells that leads to a chalk-white appearance of the fundus called "cherry red spot" is the hallmark of Tay-Sachs disease. It is also seen in others neurometabolic diseases as well as in central retinal artery occlusion. This case reports a child with Tay-Sachs disease in a family with four previous similar deaths without diagnostic.Tay-Sachs é uma doença autossômica recessiva, caracterizada pela deficiência da enzima hexosaminidase A levando ao acúmulo de esfingolipídios (GM2 em células neuronais que resulta em uma perda progressiva da função neurológica. O acúmulo de lipídios em células ganglionais da retina leva a uma aparência de mácula em cereja, característica do fundo de olho de pessoas acometidas. "Mácula em cereja" também pode ser vista em outras doenças neurometabólicas e em oclusão da artéria central da retina. Este trabalho relata o caso de um paciente com doença de Tay-Sachs em uma família com história de quatro óbitos por causas semelhantes sem diagnóstico.

  13. Neutrino mass, dark energy, and the linear growth factor

    International Nuclear Information System (INIS)

    Kiakotou, Angeliki; Lahav, Ofer; Elgaroey, Oystein

    2008-01-01

    We study the degeneracies between neutrino mass and dark energy as they manifest themselves in cosmological observations. In contradiction to a popular formula in the literature, the suppression of the matter power spectrum caused by massive neutrinos is not just a function of the ratio of neutrino to total mass densities f ν =Ω ν /Ω m , but also each of the densities independently. We also present a fitting formula for the logarithmic growth factor of perturbations in a flat universe, f(z,k;f ν ,w,Ω DE )≅[1-A(k)Ω DE f ν +B(k)f ν 2 -C(k)f ν 3 ]Ω m α (z), where α depends on the dark energy equation of state parameter w. We then discuss cosmological probes where the f factor directly appears: peculiar velocities, redshift distortion, and the integrated Sachs-Wolfe effect. We also modify the approximation of Eisenstein and Hu [Astrophys. J. 511, 5 (1999)] for the power spectrum of fluctuations in the presence of massive neutrinos and provide a revised code [http://www.star.ucl.ac.uk/∼lahav/nu m atter p ower.f].

  14. Tay-Sach disease with "cherry-red spot"--first reported case in Malaysia.

    Science.gov (United States)

    Chan, L Y; Balasubramaniam, S; Sunder, R; Jamalia, R; Karunakar, T V N; Alagaratnam, J

    2011-12-01

    We present a rare case of Tay-Sachs disease with retinal 'cherry-red spots' in a 19-month-old Malay child. Molecular genetic studies confirmed the diagnosis. The case highlights that 'cherry-red spot' is a useful clinical clue in Tay-Sachs disease and several other lysosomal storage disorders. It serves as an ideal illustration of the eye as a window to inborn error of metabolism.

  15. Tay Sachs and Related Storage Diseases: Family Planning

    Science.gov (United States)

    Schneiderman, Gerald; And Others

    1978-01-01

    Based on interviews with 24 families, the article discusses family planning and the choices available to those families in which a child has previously died from Tay-Sachs or related lipid storage diseases. (IM)

  16. Segregation of Tay-Sachs and Sandhoff alleles in a non-Jewish family.

    OpenAIRE

    Lane, A B; Young, E; Jenkins, T

    1980-01-01

    A non-Jewish family is presented in which the genes for Tay-Sachs disease and Sandhoff disease are segregating. Individuals heterozygous for both alleles have low serum and white cell total hexosaminidase levels together with a proportion of heat-labile hexosaminidase A (HEX A) which falls in the normal range. The individuals would not be detected as carriers of Tay-Sachs disease or Sandhoff disease in a population screening program.

  17. Distribution of a pseudodeficiency allele among Tay-Sachs carriers

    Energy Technology Data Exchange (ETDEWEB)

    Tomczak, J.; Grebner, E.E. (Thomas Jefferson Univ., Philadelphia, PA (United States)); Boogen, C. (Univ. of Essen Medical School (Germany))

    1993-08-01

    Recently Triggs-Raine et al. (1992) identified a new mutation in the gene coding for the [alpha]-subunit of [beta]-hexosaminidase A (hex A), the enzyme whose deficiency causes Tay-Sachs disease. This mutation, a C[sub 739]-to-T transition in exon 7, results in an altered enzyme that is active (albeit at reduced levels) in cells but that has essentially no activity in serum. This so-called pseudodeficient allele was first detected in compound heterozygotes who also carried a Tay-Sachs disease allele and therefore had no detectable hex A in their serum but who were in good health. Carriers of this apparently benign mutation are generally indistinguishable from carriers of a lethal mutation by means of routine enzyme-based screening tests, because the product of the pseudodeficient allele is not detectable in serum and has decreased activity in cells. This suggests that some individuals who have been classified as Tay-Sachs carriers are actually carriers of the pseudodeficient allele and are not at risk to have a child affected with Tay-Sachs disease. The pseudodeficient allele may also be responsible for some inconclusive diagnoses, where leukocyte values fall below the normal range but are still above the carrier range. The fact that there are now two mutant alleles (the psuedodeficient and the adult) that are indistinguishable from the lethal infantile mutations by means of enzyme assay yet that are phenotypically very different and that together may account for as much as 12% of enzyme-defined carriers on the basis of the data here suggests that DNA analysis should be part of a comprehensive screening program. It will be particularly useful to identify the mutations in couples at risk, before they undergo prenatal diagnosis. DNA analysis will also resolve some inconclusive diagnoses.

  18. Outcomes of arthroscopic "Remplissage": capsulotenodesis of the engaging large Hill-Sachs lesion

    Directory of Open Access Journals (Sweden)

    Mayo Lee

    2011-06-01

    Full Text Available Abstract Background A Hill-Sachs lesion of the humeral head after a shoulder dislocation is clinically insignificant in most cases. However, a sizable defect will engage with the anterior rim of the glenoid and cause instability even after anterior glenoid reconstruction. The purpose of this study was to evaluate the outcome of arthroscopic capsulotenodesis of the posterior capsule and infraspinatus tendon ("remplissage" to seal a large engaging Hill-Sachs lesion in an unstable shoulder. Methods This was a prospective follow-up study of patients who underwent arthroscopic surgery for recurrent shoulder instability with a large engaging Hill-Sachs lesion from 2007 to 2009. The clinical results were measured preoperatively and postoperatively with the Simple Shoulder test (SST and the Rowe score for instability. Results Eleven patients met the inclusion criteria of this study. The mean follow-up time was 30 months (range 24 to 35 months. At the last follow-up, significant improvement was observed in both scores with no recurrent dislocations. The mean SST improved from 6.6 to 11 (p Conclusions Arthroscopic remplissage for shoulder instability is an effective soft tissue technique to seal a large engaging Hill-Sachs lesion with respect to recurrence rate, range of motion and shoulder function.

  19. Cross-correlating CMB temperature fluctuations with high-energy γ-ray from Dark-Matter annihilation

    International Nuclear Information System (INIS)

    Pieri, L.

    2013-01-01

    In this paper we compute the Integrated Sachs-Wolfe effect due to the presence of dark-matter structures on cosmological scale. We cross-correlate the CMB temperature fluctuations with the extragalactic high-energy γ-ray flux map obtained with FERMI-LAT. We find a null signal consistent with the theory and conclude that the presence of halos and subhalos at galactic and extragalactic scale, if not excluded, will be hardly discoverable.

  20. Segregation of Tay-Sachs and Sandhoff alleles in a non-Jewish family.

    Science.gov (United States)

    Lane, A B; Young, E; Jenkins, T

    1980-01-01

    A non-Jewish family is presented in which the genes for Tay-Sachs disease and Sandhoff disease are segregating. Individuals heterozygous for both alleles have low serum and white cell total hexosaminidase levels together with a proportion of heat-labile hexosaminidase A (HEX A) which falls in the normal range. The individuals would not be detected as carriers of Tay-Sachs disease or Sandhoff disease in a population screening program. PMID:7446530

  1. The frequency of Tay-Sachs disease causing mutations in the Brazilian Jewish population justifies a carrier screening program.

    Science.gov (United States)

    Rozenberg, R; Pereira, L da V

    2001-07-05

    Tay-Sachs disease is an autosomal recessive disease characterized by progressive neurologic degeneration, fatal in early childhood. In the Ashkenazi Jewish population the disease incidence is about 1 in every 3,500 newborns and the carrier frequency is 1 in every 29 individuals. Carrier screening programs for Tay-Sachs disease have reduced disease incidence by 90% in high-risk populations in several countries. The Brazilian Jewish population is estimated at 90,000 individuals. Currently, there is no screening program for Tay-Sachs disease in this population. To evaluate the importance of a Tay-Sachs disease carrier screening program in the Brazilian Jewish population by determining the frequency of heterozygotes and the acceptance of the program by the community. Laboratory of Molecular Genetics--Institute of Biosciences--Universidade de São Paulo. 581 senior students from selected Jewish high schools. Molecular analysis of Tay-Sachs disease causing mutations by PCR amplification of genomic DNA, followed by restriction enzyme digestion. Among 581 students that attended educational classes, 404 (70%) elected to be tested for Tay-Sachs disease mutations. Of these, approximately 65% were of Ashkenazi Jewish origin. Eight carriers were detected corresponding to a carrier frequency of 1 in every 33 individuals in the Ashkenazi Jewish fraction of the sample. The frequency of Tay-Sachs disease carriers among the Ashkenazi Jewish population of Brazil is similar to that of other countries where carrier screening programs have led to a significant decrease in disease incidence. Therefore, it is justifiable to implement a Tay-Sachs disease carrier screening program for the Brazilian Jewish population.

  2. The white matter of the human cerebrum: Part I The occipital lobe by Heinrich Sachs

    Science.gov (United States)

    Forkel, Stephanie J.; Mahmood, Sajedha; Vergani, Francesco; Catani, Marco

    2015-01-01

    This is the first complete translation of Heinrich Sachs' outstanding white matter atlas dedicated to the occipital lobe. This work is accompanied by a prologue by Prof Carl Wernicke who for many years was Sachs' mentor in Breslau and enthusiastically supported his work. PMID:25527430

  3. Linear integrated circuits

    CERN Document Server

    Carr, Joseph

    1996-01-01

    The linear IC market is large and growing, as is the demand for well trained technicians and engineers who understand how these devices work and how to apply them. Linear Integrated Circuits provides in-depth coverage of the devices and their operation, but not at the expense of practical applications in which linear devices figure prominently. This book is written for a wide readership from FE and first degree students, to hobbyists and professionals.Chapter 1 offers a general introduction that will provide students with the foundations of linear IC technology. From chapter 2 onwa

  4. Three-dimensional computed tomography measurement accuracy of varying Hill-Sachs lesion size.

    Science.gov (United States)

    Ho, Anthony; Kurdziel, Michael D; Koueiter, Denise M; Wiater, J Michael

    2018-02-01

    The glenoid track concept has been proposed to correlate shoulder stability with bone loss. Accurate assessment of Hill-Sachs lesion size preoperatively may affect surgical planning and postoperative outcomes; however, no measurement method has been universally accepted. This study aimed to assess the accuracy and reliability of measuring Hill-Sachs lesion sizes using 3-dimensional (3D) computed tomography (CT). Nine polyurethane humerus bone substitutes were used to create Hill-Sachs lesions of varying sizes with a combination of lesion depth (shallow, intermediate, and deep) and width (small, medium, and large). Specimens were scanned with a clinical CT scanner for size measurements and a micro-CT scanner for measurement of true lesion size. Six evaluators repeated measurements twice in a 2-week interval. Scans were measured by use of 3D CT reconstructions for length, width, and Hill-Sachs interval and with use of 2D CT for depth. The interclass correlation coefficient evaluated interobserver and intraobserver variability and percentage error, and Student t-tests assessed measurement accuracy. Interclass correlation coefficient reliability demonstrated strong agreement for all variables measured (0.856-0.975). Percentage error between measured length and measured depth and the true measurement significantly varied with respect to both lesion depth (P = .003 and P = .005, respectively) and lesion size (P = .049 and P = .004, respectively). The 3D CT imaging is effective and reproducible in determining lesion size. Determination of Hill-Sachs interval width is also reliable when it is applied to the glenoid track concept. Measured values on 3D and 2-dimensional imaging using a conventional CT scanner may slightly underestimate true measurements. Copyright © 2017 Journal of Shoulder and Elbow Surgery Board of Trustees. Published by Elsevier Inc. All rights reserved.

  5. [Sandhoff's and Tay-Sachs disease--based on our own cases].

    Science.gov (United States)

    Kokot, Witold; Raczyńska, Krystyna; Krajka-Lauer, Jarosława; Iwaszkiewicz-Bilikiewicz, Barbara; Wierzba, Jolanta

    2004-01-01

    The authors described two infant with Sandhoff's and Tay-Sachs disease. Tay-Sachs disease is well-known inherited disease leading to an accumulation of gangliosides in the brain and retina. Sandhoffs disease (GM2 gangliosidosis type 0) was diagnosed in an infant, in whom a progressive neurological disorder and cherry-red foveal spots were developed. In addition, to the general clinical examination, indirect ophthalnoscopy, blood white cells enzymatic examination are used to make definitive diagnosis. In this cases, the early eye fundus examination allowed us to make the proper diagnosis. The fundus change is characterized by the "cherry-red spot" in the central area.

  6. The frequency of Tay-Sachs disease causing mutations in the Brazilian Jewish population justifies a carrier screening program

    Directory of Open Access Journals (Sweden)

    Roberto Rozenberg

    Full Text Available CONTEXT: Tay-Sachs disease is an autosomal recessive disease characterized by progressive neurologic degeneration, fatal in early childhood. In the Ashkenazi Jewish population the disease incidence is about 1 in every 3,500 newborns and the carrier frequency is 1 in every 29 individuals. Carrier screening programs for Tay-Sachs disease have reduced disease incidence by 90% in high-risk populations in several countries. The Brazilian Jewish population is estimated at 90,000 individuals. Currently, there is no screening program for Tay-Sachs disease in this population. OBJECTIVE: To evaluate the importance of a Tay-Sachs disease carrier screening program in the Brazilian Jewish population by determining the frequency of heterozygotes and the acceptance of the program by the community. SETTING: Laboratory of Molecular Genetics - Institute of Biosciences - Universidade de São Paulo. PARTICIPANTS: 581 senior students from selected Jewish high schools. PROCEDURE: Molecular analysis of Tay-Sachs disease causing mutations by PCR amplification of genomic DNA, followed by restriction enzyme digestion. RESULTS: Among 581 students that attended educational classes, 404 (70% elected to be tested for Tay-Sachs disease mutations. Of these, approximately 65% were of Ashkenazi Jewish origin. Eight carriers were detected corresponding to a carrier frequency of 1 in every 33 individuals in the Ashkenazi Jewish fraction of the sample. CONCLUSION: The frequency of Tay-Sachs disease carriers among the Ashkenazi Jewish population of Brazil is similar to that of other countries where carrier screening programs have led to a significant decrease in disease incidence. Therefore, it is justifiable to implement a Tay-Sachs disease carrier screening program for the Brazilian Jewish population.

  7. Wolf-Rayet Stars

    Science.gov (United States)

    Hamann, Wolf-Rainer; Sander, Andreas; Todt, Helge

    Nearly 150 years ago, the French astronomers Charles Wolf and Georges Rayet described stars with very conspicuous spectra that are dominated by bright and broad emission lines. Meanwhile termed Wolf-Rayet Stars after their discoverers, those objects turned out to represent important stages in the life of massive stars. As the first conference in a long time that was specifically dedicated to Wolf-Rayet stars, an international workshop was held in Potsdam, Germany, from 1.-5. June 2015. About 100 participants, comprising most of the leading experts in the field as well as as many young scientists, gathered for one week of extensive scientific exchange and discussions. Considerable progress has been reported throughout, e.g. on finding such stars, modeling and analyzing their spectra, understanding their evolutionary context, and studying their circumstellar nebulae. While some major questions regarding Wolf-Rayet stars still remain open 150 years after their discovery, it is clear today that these objects are not just interesting stars as such, but also keystones in the evolution of galaxies. These proceedings summarize the talks and posters presented at the Potsdam Wolf-Rayet workshop. Moreover, they also include the questions, comments, and discussions emerging after each talk, thereby giving a rare overview not only about the research, but also about the current debates and unknowns in the field. The Scientific Organizing Committee (SOC) included Alceste Bonanos (Athens), Paul Crowther (Sheffield), John Eldridge (Auckland), Wolf-Rainer Hamann (Potsdam, Chair), John Hillier (Pittsburgh), Claus Leitherer (Baltimore), Philip Massey (Flagstaff), George Meynet (Geneva), Tony Moffat (Montreal), Nicole St-Louis (Montreal), and Dany Vanbeveren (Brussels).

  8. Wolf Point Substation, Roosevelt County, Montana

    International Nuclear Information System (INIS)

    1991-05-01

    The Western Area Power Administration (Western), an agency of the United States Department of Energy, is proposing to construct the 115-kV Wolf Point Substation near Wolf Point in Roosevelt County, Montana (Figure 1). As part of the construction project, Western's existing Wolf Point Substation would be taken out of service. The existing 115-kV Wolf Point Substation is located approximately 3 miles west of Wolf Point, Montana (Figure 2). The substation was constructed in 1949. The existing Wolf Point Substation serves as a ''Switching Station'' for the 115-kV transmission in the region. The need for substation improvements is based on operational and reliability issues. For this environmental assessment (EA), the environmental review of the proposed project took into account the removal of the old Wolf Point Substation, rerouting of the five Western lines and four lines from the Cooperatives and Montana-Dakota Utilities Company, and the new road into the proposed substation. Reference to the new proposed Wolf Point Substation in the EA includes these facilities as well as the old substation site. The environmental review looked at the impacts to all resource areas in the Wolf Point area. 7 refs., 6 figs

  9. Wolf prize in physics

    CERN Document Server

    Piran, Tsvi

    2016-01-01

    The Wolf Foundation began its activities in 1976, with an initial endowment donated by the Wolf family. Within a very short period of time after its initiation, the Wolf prize has become one of the major signs for recognition of scientific achievements and excellence. This volume is devoted to a selection of Wolf Prize laureates in Physics and each has included two respective major publications as well as a commentary written by the laureate describing his scientific career. Readers around the world are provided a unique opportunity to get a glimpse of how scientific processes work in physics, and to comprehend how these laureates have left an indelible imprint on scientific history.

  10. A pseudodeficiency allele common in non-Jewish Tay-Sachs carriers: Implications for carrier screening

    Energy Technology Data Exchange (ETDEWEB)

    Triggs-Raine, B.L.; Akerman, B.R.; Gravel, R.A. (McGill Univ.-Montreal Children' s Hospital Research Institute, Montreal, Quebec (Canada)); Mules, E.H.; Thomas, G.H.; Dowling, C.E. (Johns Hopkins School of Medicine, Baltimore, MD (United States)); Kaback, M.M.; Lim-Steele, J.S.T. (Univ. of California, San Diego, CA (United States)); Natowicz, M.R. (Eunice Kennedy Shriver Center for Mental Retardation, Waltham, MA (United States)); Grebner, E.E. (Thomas Jefferson Univ., Philadelphia, PA (United States)); Navon, R.R. (Tel-Aviv Univ., Kfar-Sava (Israel)); Welch, J.P. (Dalhousie Univ., Halifax, Nova, Scotia (Canada)); Greenberg, C.R. (Univ. of Manitoba, Winnipeg (Canada))

    1992-10-01

    Deficiency of [beta]-hexosaminidase A (Hex A) activity typically results in Tay-Sachs disease. However, healthy subjects found to be deficient in Hex A activity (i.e., pseudodeficient) by means of in vitro biochemical tests have been described. The authors analyzed the HEXA gene of one pseudodeficient subject and identified both a C[sub 739]-to-T substitution that changes Arg[sub 247][yields]Trp on one allele and a previously identified Tay-Sachs disease mutation of the second allele. Six additional pseudodeficient subjects were found to have the C[sub 739]-to-T but for none of 36 Jewish enzyme-defined carries who did not have one of three known mutations common to this group. The C[sub 739]-to-T allele, together with a [open quotes]true[close quotes] Tay-Sachs disease allele, causes Hex A pseudodeficiency. Given both the large proportion of non-Jewish carriers with this allele and that standard biochemical screening cannot differentiate between heterozygotes for the C[sub 739]-to-T mutations and Tay-Sachs disease carriers, DNA testing for this mutation in at-risk couples is essential. This could prevent unnecessary or incorrect prenatal diagnoses. 40 refs., 3 figs., 4 tabs.

  11. The white matter of the human cerebrum: part I The occipital lobe by Heinrich Sachs.

    Science.gov (United States)

    Forkel, Stephanie J; Mahmood, Sajedha; Vergani, Francesco; Catani, Marco

    2015-01-01

    This is the first complete translation of Heinrich Sachs' outstanding white matter atlas dedicated to the occipital lobe. This work is accompanied by a prologue by Prof Carl Wernicke who for many years was Sachs' mentor in Breslau and enthusiastically supported his work. Copyright © 2014 The Authors. Published by Elsevier Ltd.. All rights reserved.

  12. Higher spin currents in orthogonal Wolf space

    International Nuclear Information System (INIS)

    Ahn, Changhyun; Paeng, Jinsub

    2015-01-01

    For the N=4 superconformal coset theory by ((SO(N+4))/(SO(N)×SU(2)))×U(1) (that contains an orthogonal Wolf space) with N = 4, the N=2 WZW affine current algebra is obtained. The 16 generators (or 11 generators) of the large N=4 linear (or nonlinear) superconformal algebra are described by these WZW affine currents explicitly. Along the line of large N=4 holography, the extra 16 currents with spins (2,(5/2),(5/2),3), ((5/2),3,3,(7/2)), ((5/2),3,3,(7/2)), and (3,(7/2),(7/2),4) are obtained in terms of the WZW affine currents. The lowest spin of this N=4 multiplet is two rather than one, which is for a unitary Wolf space. The operator product expansions between the above 11 currents and these extra 16 higher spin currents are found explicitly. (paper)

  13. A Top-Down Account of Linear Canonical Transforms

    Directory of Open Access Journals (Sweden)

    Kurt Bernardo Wolf

    2012-06-01

    Full Text Available We contend that what are called Linear Canonical Transforms (LCTs should be seen as a part of the theory of unitary irreducible representations of the '2+1' Lorentz group. The integral kernel representation found by Collins, Moshinsky and Quesne, and the radial and hyperbolic LCTs introduced thereafter, belong to the discrete and continuous representation series of the Lorentz group in its parabolic subgroup reduction. The reduction by the elliptic and hyperbolic subgroups can also be considered to yield LCTs that act on functions, discrete or continuous in other Hilbert spaces. We gather the summation and integration kernels reported by Basu and Wolf when studiying all discrete, continuous, and mixed representations of the linear group of 2×2 real matrices. We add some comments on why all should be considered canonical.

  14. New hybrid conjugate gradient methods with the generalized Wolfe line search.

    Science.gov (United States)

    Xu, Xiao; Kong, Fan-Yu

    2016-01-01

    The conjugate gradient method was an efficient technique for solving the unconstrained optimization problem. In this paper, we made a linear combination with parameters β k of the DY method and the HS method, and putted forward the hybrid method of DY and HS. We also proposed the hybrid of FR and PRP by the same mean. Additionally, to present the two hybrid methods, we promoted the Wolfe line search respectively to compute the step size α k of the two hybrid methods. With the new Wolfe line search, the two hybrid methods had descent property and global convergence property of the two hybrid methods that can also be proved.

  15. Kantowski-Sachs Einstein-æther perfect fluid models

    Energy Technology Data Exchange (ETDEWEB)

    Latta, Joey [Department of Mathematics and Statistics, Dalhousie University, Halifax, Nova Scotia, B3H 3J5 (Canada); Leon, Genly [Instituto de Física, Pontificia Universidad de Católica de Valparaíso, Casilla 4950, Valparaíso (Chile); Paliathanasis, Andronikos, E-mail: lattaj@mathstat.dal.ca, E-mail: genly.leon@pucv.cl, E-mail: anpaliat@phys.uoa.gr [Instituto de Ciencias Físicas y Matemáticas, Universidad Austral de Chile, Valdivia (Chile)

    2016-11-01

    We investigate Kantowski-Sachs models in Einstein-æ ther theory with a perfect fluid source using the singularity analysis to prove the integrability of the field equations and dynamical system tools to study the evolution. We find an inflationary source at early times, and an inflationary sink at late times, for a wide region in the parameter space. The results by A.A. Coley, G. Leon, P. Sandin and J. Latta ( JCAP 12 (2015) 010), are then re-obtained as particular cases. Additionally, we select other values for the non-GR parameters which are consistent with current constraints, getting a very rich phenomenology. In particular, we find solutions with infinite shear, zero curvature, and infinite matter energy density in comparison with the Hubble scalar. We also have stiff-like future attractors, anisotropic late-time attractors, or both, in some special cases. Such results are developed analytically, and then verified by numerics. Finally, the physical interpretation of the new critical points is discussed.

  16. Non-adiabatic perturbations in Ricci dark energy model

    International Nuclear Information System (INIS)

    Karwan, Khamphee; Thitapura, Thiti

    2012-01-01

    We show that the non-adiabatic perturbations between Ricci dark energy and matter can grow both on superhorizon and subhorizon scales, and these non-adiabatic perturbations on subhorizon scales can lead to instability in this dark energy model. The rapidly growing non-adiabatic modes on subhorizon scales always occur when the equation of state parameter of dark energy starts to drop towards -1 near the end of matter era, except that the parameter α of Ricci dark energy equals to 1/2. In the case where α = 1/2, the rapidly growing non-adiabatic modes disappear when the perturbations in dark energy and matter are adiabatic initially. However, an adiabaticity between dark energy and matter perturbations at early time implies a non-adiabaticity between matter and radiation, this can influence the ordinary Sachs-Wolfe (OSW) effect. Since the amount of Ricci dark energy is not small during matter domination, the integrated Sachs-Wolfe (ISW) effect is greatly modified by density perturbations of dark energy, leading to a wrong shape of CMB power spectrum. The instability in Ricci dark energy is difficult to be alleviated if the effects of coupling between baryon and photon on dark energy perturbations are included

  17. Infections and parasitic diseases of the gray wolf and their potential effects on wolf populations in North America.

    Science.gov (United States)

    Brand, C.J.; Pybus, M.J.; Ballard, W.B.; Peterson, R.O.

    1995-01-01

    Numerous infections and parasitic diseases have been reported for the gray wolf, including more than 10 viral, bacterial, and mycotic disease and more than 70 species of helminths and ectoparasites. However, few studies have documented the role of diseases in population dynamics. Disease can affect wolf populations directly by causing mortality or indirectly by affecting physiological and homeostatic processes, thriftiness, reproduction, behavior, or social structure. In addition, wolves are hosts to diseases that can affect prey species, thus affecting wolf populations indirectly by reducing prey abundance or increasing vulnerability to predation. Diseases such as canine distemper and infectious canine hepatitis are enzootic in wolf populations, whereas rabies occurs in wolves primarily as a result of transmission from other species such as artic and red foxes. Contact between wolves and domestic pets and livestock may affect the composition of diseases in wolves and their effects on wolf populations. Dogs were suspected of introducing lice and canine parovirus to several wolf populations. THe potential for disease to affect wolf populations and other wild and domestic animals should be considered in wolf management plans, particularly in plans for reintroduction of wolves to area within their former range.

  18. Lyso-GM2 ganglioside: a possible biomarker of Tay-Sachs disease and Sandhoff disease.

    Science.gov (United States)

    Kodama, Takashi; Togawa, Tadayasu; Tsukimura, Takahiro; Kawashima, Ikuo; Matsuoka, Kazuhiko; Kitakaze, Keisuke; Tsuji, Daisuke; Itoh, Kohji; Ishida, Yo-Ichi; Suzuki, Minoru; Suzuki, Toshihiro; Sakuraba, Hitoshi

    2011-01-01

    To find a new biomarker of Tay-Sachs disease and Sandhoff disease. The lyso-GM2 ganglioside (lyso-GM2) levels in the brain and plasma in Sandhoff mice were measured by means of high performance liquid chromatography and the effect of a modified hexosaminidase (Hex) B exhibiting Hex A-like activity was examined. Then, the lyso-GM2 concentrations in human plasma samples were determined. The lyso-GM2 levels in the brain and plasma in Sandhoff mice were apparently increased compared with those in wild-type mice, and they decreased on intracerebroventricular administration of the modified Hex B. The lyso-GM2 levels in plasma of patients with Tay-Sachs disease and Sandhoff disease were increased, and the increase in lyso-GM2 was associated with a decrease in Hex A activity. Lyso-GM2 is expected to be a potential biomarker of Tay-Sachs disease and Sandhoff disease.

  19. Tay Sachs disease: an autopsy case report.

    Science.gov (United States)

    Jadhav, Meenal Vitthal; Landge, Meenal P; Sawaimoon, Satyakam K; Harke, Arun B; Deshmukh, Sanjay D

    2005-10-01

    This report describes a case report of a postmortem performed on a 5-year old patient of Tay-Sachs disease, presenting with failure to thrive, muscular flaccidity, and cherry-red spots on macula on fundoscopy. There was no history of similarly affected sibling or any other family member. The diagnosis was confirmed by enzyme studies. At postmortem, there was no organomegaly. The brain, on microscopy, showed vacuolated swollen neurons.

  20. Useful tools for non-linear systems: Several non-linear integral inequalities

    Czech Academy of Sciences Publication Activity Database

    Agahi, H.; Mohammadpour, A.; Mesiar, Radko; Vaezpour, M. S.

    2013-01-01

    Roč. 49, č. 1 (2013), s. 73-80 ISSN 0950-7051 R&D Projects: GA ČR GAP402/11/0378 Institutional support: RVO:67985556 Keywords : Monotone measure * Comonotone functions * Integral inequalities * Universal integral Subject RIV: BA - General Mathematics Impact factor: 3.058, year: 2013 http://library.utia.cas.cz/separaty/2013/E/mesiar-useful tools for non-linear systems several non-linear integral inequalities.pdf

  1. Linear integral equations and soliton systems

    International Nuclear Information System (INIS)

    Quispel, G.R.W.

    1983-01-01

    A study is presented of classical integrable dynamical systems in one temporal and one spatial dimension. The direct linearizations are given of several nonlinear partial differential equations, for example the Korteweg-de Vries equation, the modified Korteweg-de Vries equation, the sine-Gordon equation, the nonlinear Schroedinger equation, and the equation of motion for the isotropic Heisenberg spin chain; the author also discusses several relations between these equations. The Baecklund transformations of these partial differential equations are treated on the basis of a singular transformation of the measure (or equivalently of the plane-wave factor) occurring in the corresponding linear integral equations, and the Baecklund transformations are used to derive the direct linearization of a chain of so-called modified partial differential equations. Finally it is shown that the singular linear integral equations lead in a natural way to the direct linearizations of various nonlinear difference-difference equations. (Auth.)

  2. Observational and evolutionary aspects of Wolf-Rayet stars

    International Nuclear Information System (INIS)

    Vanbeveren, D.

    1980-01-01

    The author considers (i) the binary status of Wolf-Rayet stars, (ii) the evolutionary status of Wolf-Rayet stars, (iii) the chemical abundances of Wolf-Rayet stars and (iv) evolutionary models for some known Wolf-Rayet systems. (G.T.H.)

  3. Lyso-GM2 ganglioside: a possible biomarker of Tay-Sachs disease and Sandhoff disease.

    Directory of Open Access Journals (Sweden)

    Takashi Kodama

    Full Text Available To find a new biomarker of Tay-Sachs disease and Sandhoff disease. The lyso-GM2 ganglioside (lyso-GM2 levels in the brain and plasma in Sandhoff mice were measured by means of high performance liquid chromatography and the effect of a modified hexosaminidase (Hex B exhibiting Hex A-like activity was examined. Then, the lyso-GM2 concentrations in human plasma samples were determined. The lyso-GM2 levels in the brain and plasma in Sandhoff mice were apparently increased compared with those in wild-type mice, and they decreased on intracerebroventricular administration of the modified Hex B. The lyso-GM2 levels in plasma of patients with Tay-Sachs disease and Sandhoff disease were increased, and the increase in lyso-GM2 was associated with a decrease in Hex A activity. Lyso-GM2 is expected to be a potential biomarker of Tay-Sachs disease and Sandhoff disease.

  4. The enigma of lone wolf terrorism: an assessment

    NARCIS (Netherlands)

    Spaaij, R.

    2010-01-01

    Lone wolf terrorism remains an ambiguous and enigmatic phenomenon. The boundaries of lone wolf terrorism are fuzzy and arbitrary. This article aims to define and analyze the main features and patterns of lone wolf terrorism in fifteen countries. Lone wolf terrorism is shown to be more prevalent in

  5. The Difficulty of Saying "I": Translation and Censorship of Christa Wolf's Der geteilte Himmel

    Directory of Open Access Journals (Sweden)

    Katharina von Ankum

    1993-06-01

    Full Text Available The end of the GDR in 1990 triggered a vivid literary debate in Germany which focused on the interrelationship of politics, literature, and criticism. In this context, the work of Christa Wolf was attacked as primary example of self-censorship and collaboration. In my article, I argue that Wolf became the target of literary criticism largely because of her attempt to express female subjectivity in her texts. In my contrastive analysis of Der geteilte Himmel (1963 and its English translation (1965, I read Wolf's text as an initial attempt at a "socialist modernism." The continued value of this and subsequent works by Wolf lies in the accuracy and complexity with which she probes human behavior under adverse historical circumstances. Even a text like Der geteilte Himmel , which on a surface level reads merely like a political vote for socialism in the GDR as well as the writer's support for the division of Germany, eludes the binary opposition of East/West, them/us that critics have used to categorize Wolf's work. The hybrid nature of the text serves as example of Wolf's sincerity as a writer, evidence of her personal integrity, as well as her relentless commitment to a social alternative.

  6. Quantum field theory of the universe in the Kantowski-Sachs space-time

    International Nuclear Information System (INIS)

    Shen, Y.; Tan, Z.

    1996-01-01

    In this paper, the quantum field theory of the universe in the Kantowski-Sachs space-time is studied. An analogue of proceedings in quantum field theory is applied in curved space-time to the Kantowski-Sachs space-time, obtaining the wave function of the universe satisfied the Wheeler-DeWitt equation. Regarding the wave function as a universe field in the minisuperspace, the authors can not only overcome the difficulty of the probabilistic interpretation in quantum cosmology, but also come to the conclusion that there is multiple production of universes. The average number of the produced universes from nothing is calculated. The distribution of created universe is given. It is the Planckian distribution

  7. Denali Park wolf studies: Implications for Yellowstone

    Science.gov (United States)

    Mech, L. David; Meier, Thomas J.; Burch, John W.

    1991-01-01

    The Northern Rocky Mountain Wolf Recovery Plan approved by the U.S. Fish and Wildlife Service (1987) recommends re-establishment of wolves (Canis lupus) in Yellowstone National Park. Bills proposing wolf re-establishment in the Park have been introduced into the U.S. House and Senate. However, several questions have been raised about the possible effects of wolf re-establishment on other Yellowstone Park fauna, on human use of the Park and on human use of surrounding areas. Thus the proposed wolf re-establishment remains controversial.Information pertinent to some of the above questions is available from a current study of wolf ecology in Denali National Park and Preserve, Alaska, which we began in 1986. Although Denali Park differs from Yellowstone in several ways, it is also similar enough in important respects to provide insight into questions raised about wolf re-establishment in Yellowstone.

  8. Energy in the Kantowski–Sachs space-time using teleparallel ...

    Indian Academy of Sciences (India)

    Inflation is again considered to be one of the early phases of the Universe [10]. For testing gravitational energy–momentum tensor of the teleparallel theory in quasilocal approach and also in the. Hamiltonian structure of TEGR, it is intended to calculate the total energy of inflationary. Kantowski–Sachs-type Universe. Again ...

  9. Geospatial Analysis of Grey Wolf Movement Patterns

    Science.gov (United States)

    Sur, D.

    2017-12-01

    The grey wolf is a top predator that lives across a diverse habitat, ranging from Europe to North America. They often hunt in packs, preferring caribou, deer and elk as prey. Currently, many gray wolves live in Denali National Park and Preserve. In this study, several wolf packs were studied in three distinct regions of Denali. The purpose of my research was to investigate the links between wolf habitat, movement patterns, and prey thresholds. These are needed for projecting future population, growth and distribution of wolves in the studied region. I also investigated the effect wolves have on the ecological structure of the communities they inhabit. In the study I carried out a quantitative analysis of wolf population trends and daily distance movement by utilizing an analysis of variance (ANOVA) in the program JmpPro12 (SAS Institute, Crary, NC) to assess regional differences in pack size, wolf density, average daily distance moved. I found a clear link between the wolf habitat and prey thresholds; the habitat directly influences the types of prey available. However there was no link between the daily distance movement, the wolf habitat and prey density.

  10. Correlation of augmented startle reflex with brainstem electrophysiological responses in Tay-Sachs disease.

    Science.gov (United States)

    Nakamura, Sadao; Saito, Yoshiaki; Ishiyama, Akihiko; Sugai, Kenji; Iso, Takashi; Inagaki, Masumi; Sasaki, Masayuki

    2015-01-01

    To clarify the evolution of an augmented startle reflex in Tay-Sachs disease and compare the temporal relationship between this reflex and brainstem evoked potentials. Clinical and electrophysiological data from 3 patients with Tay-Sachs disease were retrospectively collected. The augmented startle reflex appeared between the age of 3 and 17 months and disappeared between the age of 4 and 6 years. Analysis of brainstem auditory evoked potentials revealed that poor segregation of peak I, but not peak III, coincided with the disappearance of the augmented startle reflex. A blink reflex with markedly high amplitude was observed in a patient with an augmented startle reflex. The correlation between the augmented startle reflex and the preservation of peak I but not peak III supports the theory that the superior olivary nucleus is dispensable for this reflex. The blink reflex with high amplitudes may represent augmented excitability of reticular formation at the pontine tegmentum in Tay-Sachs disease, where the pattern generators for the augmented startle and blink reflexes may functionally overlap. Copyright © 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  11. Bianchi - I, II, VIII, IX and Kantowski-Sachs-like cosmological models with perfect fluid and electromagnetic fields with conductivity current

    International Nuclear Information System (INIS)

    Portugal, R.

    1984-01-01

    Three processes of solutions of the Einstein-Maxwell equations for Bianchi - I, II, VIII, IX and Kantowski-Sachs-like cosmological models with perfect fluid in magnetohydrolodynamical regimem are presented. Diagonal Bianchi-like models are considered with two anisotropy direction in the maximum. Solutions are found for Bianchi-II and IX-like models with energy conditions to be analyzed. Solutions are found for Bianchi-IX and Kantowski-Sachs-Like models with positive electric conductivity and satisfering to the predominant energy conditions. Solutions are formed for isotropic Kantowski-Sachs-Like models satisfering to the equation of state p=λρ, 0 0, admiting, in addition to the perfect fluid, electric field only. It is shown that a class of Bertotti-Robinson-like solutions is unstable by perturbations and it is carried in Kantowski-Sachs-like models with non-null electric conductivity. (L.C.) [pt

  12. Gravitational, shear and matter waves in Kantowski-Sachs cosmologies

    Energy Technology Data Exchange (ETDEWEB)

    Keresztes, Zoltán; Gergely, László Á. [Department of Theoretical Physics, University of Szeged, Tisza Lajos krt 84-86, Szeged 6720 (Hungary); Forsberg, Mats; Bradley, Michael [Department of Physics, UmeåUniversity (Sweden); Dunsby, Peter K.S., E-mail: zkeresztes@titan.physx.u-szeged.hu, E-mail: forsberg.mats.a.b@gmail.com, E-mail: michael.bradley@physics.umu.se, E-mail: peter.dunsby@uct.ac.za, E-mail: gergely@physx.u-szeged.hu [Astrophysics, Cosmology and Gravity Centre (ACGC), University of Cape Town, Rondebosch 7701, Cape Town (South Africa)

    2015-11-01

    A general treatment of vorticity-free, perfect fluid perturbations of Kantowski-Sachs models with a positive cosmological constant are considered within the framework of the 1+1+2 covariant decomposition of spacetime. The dynamics is encompassed in six evolution equations for six harmonic coefficients, describing gravito-magnetic, kinematic and matter perturbations, while a set of algebraic expressions determine the rest of the variables. The six equations further decouple into a set of four equations sourced by the perfect fluid, representing forced oscillations and two uncoupled damped oscillator equations. The two gravitational degrees of freedom are represented by pairs of gravito-magnetic perturbations. In contrast with the Friedmann case one of them is coupled to the matter density perturbations, becoming decoupled only in the geometrical optics limit. In this approximation, the even and odd tensorial perturbations of the Weyl tensor evolve as gravitational waves on the anisotropic Kantowski-Sachs background, while the modes describing the shear and the matter density gradient are out of phase dephased by π /2 and share the same speed of sound.

  13. THREE PLANETS ORBITING WOLF 1061

    Energy Technology Data Exchange (ETDEWEB)

    Wright, D. J.; Wittenmyer, R. A.; Tinney, C. G.; Bentley, J. S.; Zhao, Jinglin, E-mail: duncan.wright@unsw.edu.au [Department of Astronomy and Australian Centre for Astrobiology, School of Physics, University of New South Wales, NSW 2052 (Australia)

    2016-02-01

    We use archival HARPS spectra to detect three planets orbiting the M3 dwarf Wolf 1061 (GJ 628). We detect a 1.36 M{sub ⊕} minimum-mass planet with an orbital period P = 4.888 days (Wolf 1061b), a 4.25 M{sub ⊕} minimum-mass planet with orbital period P = 17.867 days (Wolf 1061c), and a likely 5.21 M{sub ⊕} minimum-mass planet with orbital period P = 67.274 days (Wolf 1061d). All of the planets are of sufficiently low mass that they may be rocky in nature. The 17.867 day planet falls within the habitable zone for Wolf 1061 and the 67.274 day planet falls just outside the outer boundary of the habitable zone. There are no signs of activity observed in the bisector spans, cross-correlation FWHMs, calcium H and K indices, NaD indices, or Hα indices near the planetary periods. We use custom methods to generate a cross-correlation template tailored to the star. The resulting velocities do not suffer the strong annual variation observed in the HARPS DRS velocities. This differential technique should deliver better exploitation of the archival HARPS data for the detection of planets at extremely low amplitudes.

  14. Wolf-Rayet Stars in Starburst Galaxies

    OpenAIRE

    Mas-Hesse, J. Miguel; Kunth, Daniel; Cervino, Miguel

    1999-01-01

    Wolf-Rayet stars have been detected in a large number of galaxies experiencing intense bursts of star formation. All stars initially more massive than a certain, metallicity-dependent, value are believed to experience the Wolf-Rayet phase at the end of their evolution, just before collapsing in supernova explosion. The detection of Wolf-Rayet stars puts therefore important constraints on the evolutionary status of starbursts, the properties of their Initial Mass Functions and their star forma...

  15. Neurocognitive testing in late-onset Tay-Sachs disease: a pilot study.

    Science.gov (United States)

    Elstein, D; Doniger, G M; Simon, E; Korn-Lubetzki, I; Navon, R; Zimran, A

    2008-08-01

    To test neurocognitive function in patients with late-onset Tay-Sachs disease (LOTS) using a computerized system to assess whether cognition is a clinically relevant outcome measure of possible therapeutic intervention in LOTS. Ten adults with Tay-Sachs disease were administered at least one battery of the Mindstreams Neurotrax system for evaluation of cognitive function. Six sub-scores and a Global Cognitive Score (GCS) were tabulated. A disease specific severity score was also devised with six domains. Despite identical genotypes, all patients but the two oldest had > or = 3/6 sub-scores one standard deviation below normal mean (100); verbal and executive functions were most affected. The severity score measured other functions. Because of provocative findings on re-testing in patients exposed to miglustat, and despite the very small cohort, cognitive function may be an appropriate and clinically relevant outcome measure for future therapeutic interventions in LOTS.

  16. Winter severity and wolf predation on a formerly wolf-free elk herd

    Science.gov (United States)

    Mech, L. David; Smith, Douglas W.; Murphy, Kerry M.; MacNulty, Daniel R.

    2001-01-01

    We studied wolf (Canis lupus) predation on elk (Cervus elaphus) in Yellowstone National Park from 17 March to 15 April 1997 (severe winter conditions) and from 2 to 31 March 1998 (mild winter conditions) 2-3 years after wolves were reintroduced to the park. Elk composed 91 % of 117 kills. Data comparisons for 1997 versus 1998 were: hunting success rate, 26% versus 15%; kill rate, 17.1 kg/wolf/day versus 6.1; percent of kill consumed in first day, 7 versus 86; percent femur marrow fat of adult kills, 27 versus 70; calf:adult ratios of kills, 2:33 versus 17:23; sex ratio of kills, 14M:19F versus 17M:6F; mean age of elk killed, males 6.1 years, females 15.2 versus males, 4.8, females 13.0. Winter severity influenced the wolf-elk relationship more than the naivete of the elk herd to predation by wolves.

  17. Bear-baiting may exacerbate wolf-hunting dog conflict.

    Science.gov (United States)

    Bump, Joseph K; Murawski, Chelsea M; Kartano, Linda M; Beyer, Dean E; Roell, Brian J

    2013-01-01

    The influence of policy on the incidence of human-wildlife conflict can be complex and not entirely anticipated. Policies for managing bear hunter success and depredation on hunting dogs by wolves represent an important case because with increasing wolves, depredations are expected to increase. This case is challenging because compensation for wolf depredation on hunting dogs as compared to livestock is less common and more likely to be opposed. Therefore, actions that minimize the likelihood of such conflicts are a conservation need. We used data from two US states with similar wolf populations but markedly different wolf/hunting dog depredation patterns to examine the influence of bear hunting regulations, bear hunter to wolf ratios, hunter method, and hunter effort on wolf depredation trends. Results indicated that the ratio of bear hunting permits sold per wolf, and hunter method are important factors affecting wolf depredation trends in the Upper Great Lakes region, but strong differences exist between Michigan and Wisconsin related in part to the timing and duration of bear-baiting (i.e., free feeding). The probability that a wolf depredated a bear-hunting dog increases with the duration of bear-baiting, resulting in a relative risk of depredation 2.12-7.22× greater in Wisconsin than Michigan. The net effect of compensation for hunting dog depredation in Wisconsin may also contribute to the difference between states. These results identified a potential tradeoff between bear hunting success and wolf/bear-hunting dog conflict. These results indicate that management options to minimize conflict exist, such as adjusting baiting regulations. If reducing depredations is an important goal, this analysis indicates that actions aside from (or in addition to) reducing wolf abundance might achieve that goal. This study also stresses the need to better understand the relationship among baiting practices, the effect of compensation on hunter behavior, and depredation

  18. Detection of local non-Gaussianity with future observations

    International Nuclear Information System (INIS)

    Li Hong; Liu Jie

    2012-01-01

    In this Letter we estimate the primordial non-Gaussianity (PNG) by simulating future observations. We use the Large Sky Area Multi-Object Fiber Spectroscopic Telescope (LAMOST) as an example and focus on the cross correlation signal between the galaxies and the Integrate Sachs-Wolfe (ISW) effect of CMB. Our result is optimistical. It shows the potential of LAMOST, particularly its quasar survey, in probing for the PNG by ISW - galaxy cross correlation. This study is particularly relevant because LAMOST is almost parallel to the timetable of the upcoming high precision Planck satellite.

  19. Tay-Sachs disease with conspicuous cranial computerized tomographic appearances

    International Nuclear Information System (INIS)

    Watanabe, Kishichiro; Mukawa, Akio; Muto, Kazuhiko; Nishikawa, Jiro; Takahashi, Shigeko.

    1985-01-01

    An autopsy case of a 3-year-old female infant with Tay-Sachs disease was presented. A cherry red spot in the fundus and a deficiency of N-acetyl-β-hexosaminidase A in the white blood cells were revealed soon after admission at the age of one year. Her parents and sister were found to be healthy carriers. The patient showed a typical clinical course with marked cranial swelling. In addition to the marked ballooning of neurons on light microscope, membranous cytoplasmic body (MCB) on electron microscope and abnormal accumulation of GM 2 ganglioside in the cerebral cortex by thin layer chromatography were confirmed in the autopsy specimens. In the late stage of her clinical course, the cranial computerized tomography (CT) demonstrated symmetric and deep-wavy hyperdense cerebral cortical zones, diffuse hypodensity and diminished volume of cerebral white matter, mild to moderate ventricular dilatation, and a small cerebellum and brainstem. These conspicuous appearances of the cranial CT seem to be characteristic of Tay-Sachs disease in the late stage, and they are derived from abnormal accumulation of GM 2 ganglioside in the cerebral cortex, and diffuse intense demyelination (dysmyelinating demyelination) of the cerebral white matter. (author)

  20. Evaluating prey switching in wolf-ungulate systems.

    Science.gov (United States)

    Garrott, Robert A; Bruggeman, Jason E; Becker, Matthew S; Kalinowski, Steven T; White, P J

    2007-09-01

    Wolf restoration has become a widely accepted conservation and management practice throughout North America and Europe, though the ecosystem effects of returning top carnivores remain both scientific and societal controversies. Mathematical models predicting and describing wolf-ungulate interactions are typically limited to the wolves' primary prey, with the potential for prey switching in wolf-multiple-ungulate systems only suggested or assumed by a number of investigators. We used insights gained from experiments on small taxa and field data from ongoing wolf-ungulate studies to construct a model of predator diet composition for a wolf-elk-bison system in Yellowstone National Park, Wyoming, USA. The model explicitly incorporates differential vulnerability of the ungulate prey types to predation, predator preference, differences in prey biomass, and the possibility of prey switching. Our model demonstrates wolf diet shifts with changes in relative abundance of the two prey, with the dynamics of this shift dependent on the combined influences of preference, differential vulnerability, relative abundances of prey, and whether or not switching occurs. Differences in vulnerability between elk and bison, and strong wolf preference for elk, result in an abrupt dietary shift occurring only when elk are very rare relative to bison, whereas incorporating switching initiates the dietary shift more gradually and at higher bison-elk ratios. We demonstrate how researchers can apply these equations in newly restored wolf-two-prey systems to empirically evaluate whether prey switching is occurring. Each coefficient in the model has a biological interpretation, and most can be directly estimated from empirical data collected from field studies. Given the potential for switching to dramatically influence predator-prey dynamics and the wide range of expected prey types and abundances in some systems where wolves are present and/or being restored, we suggest that this is an

  1. Choroidal Coloboma in a Case of Tay-Sachs Disease

    OpenAIRE

    Ahmed, Nasreen Raees; Tripathy, Koushik; Kumar, Vivek; Gogia, Varun

    2014-01-01

    Coloboma as an ocular finding has been documented in various syndromes. Here we have a case of infantile Tay-Sachs disease associated with unilateral choroidal coloboma. To the best of our knowledge, such an association has not been documented in the literature. Whether such an association is a matter of chance or signifies the involvement of ganglioside metabolism in ocular embryogenesis remains to be elucidated.

  2. Choroidal coloboma in a case of tay-sachs disease.

    Science.gov (United States)

    Ahmed, Nasreen Raees; Tripathy, Koushik; Kumar, Vivek; Gogia, Varun

    2014-01-01

    Coloboma as an ocular finding has been documented in various syndromes. Here we have a case of infantile Tay-Sachs disease associated with unilateral choroidal coloboma. To the best of our knowledge, such an association has not been documented in the literature. Whether such an association is a matter of chance or signifies the involvement of ganglioside metabolism in ocular embryogenesis remains to be elucidated.

  3. Tay-Sachs and Sandhoff diseases: enzymatic diagnosis in dried blood spots on filter paper: retrospective diagnoses in newborn-screening cards.

    Science.gov (United States)

    Chamoles, Néstor A; Blanco, Mariana; Gaggioli, Daniela; Casentini, Carina

    2002-04-01

    Tay-Sachs disease (TSD), Sandhoff disease (SD) and variants are caused by deficient activity of the lysosomal enzymes hexosaminidase A (HA) and total hexosaminidase (TH) (hexosaminidase A plus B), respectively. For diagnosis, these enzymes are usually measured in plasma or extracts of leukocytes. We describe methods for the assay of hexosaminidase A and total hexosaminidase activities in dried blood spots (DBSs) on filter paper. We studied 163 healthy controls, 9 Tay-Sachs patients, 4 Sandhoff patients, 18 obligate carriers and the newborn-screening cards from two patients with Tay-Sachs and one patient with Sandhoff disease. To tubes containing a 3-mm-diameter blood spot, we added elution liquid and substrate solution. After incubation at 37 degrees C, the amount of hydrolyzed product was compared with a calibrator to allow the quantification of enzyme activity. The described methodology is useful to distinguish patients with Tay-Sachs disease or Sandhoff disease from carriers and controls using samples that are sufficiently stable to be transported to the testing laboratory by mail. The diagnosis of both diseases from a newborn-screening card (NSC) was clearly demonstrated, even after storage for up to 38 months at room temperature. The newborn-screening card has been added to the biological materials that allow the identification of patients with Tay-Sachs disease and Sandhoff disease.

  4. The relationships between the maned wolf and people

    OpenAIRE

    Consorte-McCrea, A.

    2013-01-01

    The maned wolf is a large carnivore, the largest one in South America. Although it was described as a wolf by early agents of the Portuguese crown, it is monophyletic and therefore difficult to mistake with any other species. The fact that the maned wolf is unique and distinctive is important if its image is to represent the Cerrado biome and all of its dwindling biodiversity. Do people’s relationships with the maned wolf make it charismatic and likable enough to earn the badge of flagship sp...

  5. Preconception risk assessment for thalassaemia, sickle cell disease, cystic fibrosis and Tay-Sachs disease.

    Science.gov (United States)

    Hussein, Norita; Weng, Stephen F; Kai, Joe; Kleijnen, Jos; Qureshi, Nadeem

    2018-03-14

    Globally, about five per cent of children are born with congenital or genetic disorders. The most common autosomal recessive conditions are thalassaemia, sickle cell disease, cystic fibrosis and Tay-Sachs disease, with higher carrier rates in specific patient populations. Identifying and counselling couples at genetic risk of the conditions before pregnancy enables them to make fully informed reproductive decisions, with some of these choices not being available if genetic counselling is only offered in an antenatal setting. This is an update of a previously published review. To assess the effectiveness of systematic preconception genetic risk assessment to improve reproductive outcomes in women and their partners who are identified as carriers of thalassaemia, sickle cell disease, cystic fibrosis and Tay-Sachs disease in healthcare settings when compared to usual care. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Trials Registers. In addition, we searched for all relevant trials from 1970 (or the date at which the database was first available if after 1970) to date using electronic databases (MEDLINE, Embase, CINAHL, PsycINFO), clinical trial databases (National Institutes of Health, Clinical Trials Search portal of the World Health Organization, metaRegister of controlled clinical trials), and hand searching of key journals and conference abstract books from 1998 to date (European Journal of Human Genetics, Genetics in Medicine, Journal of Community Genetics). We also searched the reference lists of relevant articles, reviews and guidelines and also contacted subject experts in the field to request any unpublished or other published trials.Date of latest search of the registers: 20 June 2017.Date of latest search of all other sources: 16 November 2017. Any randomised or quasi-randomised controlled trials (published or unpublished) comparing reproductive outcomes of systematic preconception genetic risk assessment for thalassaemia, sickle

  6. İki Kardeşte Görülen Tay-Sachs Hastalığı

    OpenAIRE

    KÜÇÜKÖDÜK, Ş.

    2010-01-01

    In view of two cases of autosomal recessive Tay-Sachs disease which affected two siblings, genetic consultation has been once more emphasized. The clinical features of this entity has been reviewed under the information obtained from recent literature. Otozomal resesif geçiş gösteren ve iki kardeşide etkiliyen Tay-Sachs hastalığında, genetik danışmanın önemi bir kez daha vurgulandı. Bu hastalığın klinik özellikleri literatür bilgilerinin ışığı altında gözden geçirildi....

  7. Tay-Sachs disease: high gene frequency in a non-Jewish population.

    Science.gov (United States)

    Kelly, T E; Chase, G A; Kaback, M M; Kumor, K; McKusick, V A

    1975-01-01

    A non-Amish "Pennsylvania Dutch" semi-isolate was found to have a high frequency of Tay-Sachs gene. This high frequency could be ascribed to founder effect and may represent, in microcosm, how this mechanism could have produced the high gene frequency among Ashkenazi Jews. PMID:803011

  8. 78 FR 24447 - Goldman Sachs Trust, et al.; Notice of Application

    Science.gov (United States)

    2013-04-25

    ... SECURITIES AND EXCHANGE COMMISSION [Investment Company Act Release No. 30471; 812-14075] Goldman Sachs Trust, et al.; Notice of Application April 19, 2013. AGENCY: Securities and Exchange Commission..., or cause more than 10% of the acquired company's voting stock to be owned by investment companies and...

  9. Bear-baiting may exacerbate wolf-hunting dog conflict.

    Directory of Open Access Journals (Sweden)

    Joseph K Bump

    Full Text Available BACKGROUND: The influence of policy on the incidence of human-wildlife conflict can be complex and not entirely anticipated. Policies for managing bear hunter success and depredation on hunting dogs by wolves represent an important case because with increasing wolves, depredations are expected to increase. This case is challenging because compensation for wolf depredation on hunting dogs as compared to livestock is less common and more likely to be opposed. Therefore, actions that minimize the likelihood of such conflicts are a conservation need. METHODOLOGY/PRINCIPAL FINDINGS: We used data from two US states with similar wolf populations but markedly different wolf/hunting dog depredation patterns to examine the influence of bear hunting regulations, bear hunter to wolf ratios, hunter method, and hunter effort on wolf depredation trends. Results indicated that the ratio of bear hunting permits sold per wolf, and hunter method are important factors affecting wolf depredation trends in the Upper Great Lakes region, but strong differences exist between Michigan and Wisconsin related in part to the timing and duration of bear-baiting (i.e., free feeding. The probability that a wolf depredated a bear-hunting dog increases with the duration of bear-baiting, resulting in a relative risk of depredation 2.12-7.22× greater in Wisconsin than Michigan. The net effect of compensation for hunting dog depredation in Wisconsin may also contribute to the difference between states. CONCLUSIONS/SIGNIFICANCE: These results identified a potential tradeoff between bear hunting success and wolf/bear-hunting dog conflict. These results indicate that management options to minimize conflict exist, such as adjusting baiting regulations. If reducing depredations is an important goal, this analysis indicates that actions aside from (or in addition to reducing wolf abundance might achieve that goal. This study also stresses the need to better understand the relationship

  10. Mathematical methods linear algebra normed spaces distributions integration

    CERN Document Server

    Korevaar, Jacob

    1968-01-01

    Mathematical Methods, Volume I: Linear Algebra, Normed Spaces, Distributions, Integration focuses on advanced mathematical tools used in applications and the basic concepts of algebra, normed spaces, integration, and distributions.The publication first offers information on algebraic theory of vector spaces and introduction to functional analysis. Discussions focus on linear transformations and functionals, rectangular matrices, systems of linear equations, eigenvalue problems, use of eigenvectors and generalized eigenvectors in the representation of linear operators, metric and normed vector

  11. Diagnosis and molecular characterization of non-classic forms of Tay-Sachs disease in Brazil.

    Science.gov (United States)

    Rozenberg, R; Kok, F; Burin, M G; Sá Miranda, M C; Vasques, C; Henriques-Souza, A M M; Giugliani, R; Vainzof, Mariz; Pereira, L V

    2006-06-01

    Molecular analysis of five Brazilian families, including eight patients presenting with nonclassic Tay-Sachs disease, was performed to identify frequent causative mutations and their correlation with clinical course. Three patients were affected by the B1 subacute variant and were shown to carry the R178H mutation (the DN allele), which is also common among Portuguese patients. Two of them were compound heterozygotes, whereas the third presented with the mutation in both alleles. Since Brazil was a Portuguese colony for over two centuries, common ancestry might be the probable explanation. The fourth patient presented with a juvenile phenotype and carries the R499H mutation, which has been reported only once worldwide and is associated with residual enzyme activity, responsible for a slower clinical course. The fifth family, of an Ashkenazi Jewish background, showed an extensive intrafamilial clinical variability among three affected sibs presenting with muscle atrophy, ataxia, and psychiatric symptoms. They were first diagnosed as having atypical spinal muscular atrophy and, subsequently, spinocerebellar ataxia, but, recently, the diagnosis of late-onset Tay-Sachs disease was confirmed based on reduced plasma hexosaminidase A activity and the G269S/InsTATC1278 genotype. It is therefore highly recommended to test patients with a similar clinical history for Tay-Sachs disease. In the same family, one first cousin committed suicide at the age of 24 years, presenting with a clinical phenotype that suggested an undiagnosed case and highlighting the effect of the intrafamilial clinical variability in delaying a prompt diagnosis. It is now recognized that his parents are, in fact, a carrier couple. Additionally, another relative had been previously identified as a heterozygote in a Tay-Sachs disease screening program, but the information was not shared among the family. Since this information might anticipate diagnosis and genetic counseling, it is advisable that

  12. Choroidal Coloboma in a Case of Tay-Sachs Disease

    Directory of Open Access Journals (Sweden)

    Nasreen Raees Ahmed

    2014-01-01

    Full Text Available Coloboma as an ocular finding has been documented in various syndromes. Here we have a case of infantile Tay-Sachs disease associated with unilateral choroidal coloboma. To the best of our knowledge, such an association has not been documented in the literature. Whether such an association is a matter of chance or signifies the involvement of ganglioside metabolism in ocular embryogenesis remains to be elucidated.

  13. Gamma-Ray Bursts from tidally spun-up Wolf-Rayet stars?

    OpenAIRE

    Detmers, R. G.; Langer, N.; Podsiadlowski, Ph.; Izzard, R. G.

    2008-01-01

    Context. The collapsar model requires rapidly rotating Wolf-Rayet stars as progenitors of long gamma-ray bursts. However, Galactic Wolf-Rayet stars rapidly lose angular momentum due to their intense stellar winds. Aims. We investigate whether the tidal interaction of a Wolf-Rayet star with a compact object in a binary system can spin up the Wolf-Rayet star enough to produce a collapsar. Methods. We compute the evolution of close Wolf-Rayet binaries, including tidal angular momentum exchange, ...

  14. Seeded hot dark matter models with inflation

    Science.gov (United States)

    Gratsias, John; Scherrer, Robert J.; Steigman, Gary; Villumsen, Jens V.

    1993-01-01

    We examine massive neutrino (hot dark matter) models for large-scale structure in which the density perturbations are produced by randomly distributed relic seeds and by inflation. Power spectra, streaming velocities, and the Sachs-Wolfe quadrupole fluctuation are derived for this model. We find that the pure seeded hot dark matter model without inflation produces Sachs-Wolfe fluctuations far smaller than those seen by COBE. With the addition of inflationary perturbations, fluctuations consistent with COBE can be produced. The COBE results set the normalization of the inflationary component, which determines the large-scale (about 50/h Mpc) streaming velocities. The normalization of the seed power spectrum is a free parameter, which can be adjusted to obtain the desired fluctuations on small scales. The power spectra produced are very similar to those seen in mixed hot and cold dark matter models.

  15. Splice junction mutation in some Ashkenazi Jews with Tay-Sachs disease: Evidence against a single defect within this ethnic group

    International Nuclear Information System (INIS)

    Myerowitz, R.

    1988-01-01

    Tay-Sachs disease is an inherited disorder in which the α chain of the lysosomal enzyme β-N-acetylhexosaminidase A bears the mutation. Ashkenazi Jews are found to be carriers for a severe type of Tay-Sachs disease, the classic form, 10 times more frequently than the general population. Ashkenazi Jewish patients with classic Tay-Sachs disease have appeared to be clinically and biochemically identical, and the usual assumption has been that they harbor the same α-chain mutation. The author has isolated the α-chain gene from an Ashkenazi Jewish patient, GM2968, with classic Tay-Sachs disease and compared its nucleotide sequences with that of the normal α-chain gene in the promoter region, exon and splice junction regions, and polyadenylylation signal area. Only one difference was observed between these sequences. The alteration is presumed to be functionally significant and to result in aberrant mRNA splicing. Utilizing the polymerase chain reaction to amplify the region encompassing the mutation, the author developed an assay to screen patients and heterozygote carriers for this mutation. Surprisingly, in each of two Ashkenazi patients, only one α-chain allele harbored the splice junction mutation. Only one parent of each of these patients was positive for the defect. Another Ashkenazi patient did not bear this mutation at all nor did either of the subject's parents. The data are consistent with the presence of more than one mutation underlying the classic form of Tay-Sachs disease in the Ashkenazi Jewish population

  16. Computer simulation of wolf-removal strategies for animal damage control

    Science.gov (United States)

    Robert G. Haight; Laurel E. Travis; Kevin Nimerfro; L. David Mech

    2002-01-01

    Because of the sustained growth of the gray wolf (Canis lupus) population in the western Great Lakes region of the United States, management agencies are anticipating gray wolf removal from the federal endangered species list and are proposing strategies for wolf management. Strategies are needed that would balance conflicting public demands for wolf...

  17. The Wolf-Rayet stars

    International Nuclear Information System (INIS)

    Cherepashchuk, A.M.; Khaliullin, K.F.

    1983-01-01

    One may say that the observational evidence now available for Wolf-Rayet stars, particularly the results gained by analyzing eclipsing binary systems prove that at least some of these objects are helium stars. At the same time, the low electron temperatures in the emission-line zones, the indisputable evidence of a highly extended atmosphere and the steady loss of material by these stars all strongly support the fluorescence model proposed by a previous study, whereby Wolf-Rayet envelopes would owe their luminosity to conversion of the short-wavelength radiation emitted by the hot stellar core

  18. Wolf predation on caribou calves in Denali National Park, Alaska

    Science.gov (United States)

    Adams, Layne G.; Dale, B.; Mech, L. David; Carbyn, Ludwig N.; Fritts, Steven H.; Seip, Dale R.

    1995-01-01

    During 1987-1991, 29 to 45 radio-collared caribou cows were monitored daily during calving each year and their calves were radio-collared (n = 147 calves) to investigate calf production and survival. We determined characteristics of wolf predation on caribou calves and, utilizing information from a companion wolf study, evaluated the role of spacing by caribou cows in minimizing wolf predation on neonates (calves wolf abundance doubled. On average, 49% of the neonates died, ranging from 30% in 1987 to 71% in 1991. Overall, wolves killed 22% of the neonates produced and were the most important mortality agent. Wolves preyed on calves primarily during six days following the peak of calving and usually killed calves five to 15 days old. The mortality rate for neonates was strongly inversely correlated with average birthweight. Neonatal losses to wolves were also correlated with birthweight but not spring wolf density or mean calving elevation. Caribou concentrated on a calving ground when spring snow conditions allowed and adjusted their distribution on the calving ground depending on snow conditions and wolf distribution and abundance. Even though the wolf population doubled, the exposure of caribou calves to wolf predation did not increase, when spacing by caribou at the wolf pack territory scale was accounted for.

  19. Rob Wolf 1947-2010

    CERN Multimedia

    TE Department

    2010-01-01

    We deeply regret to announce the death of Mr Rob WOLF on 20.06.2010. Mr Rob Wolf, born on 07.06.1947, worked in the TE Department and had been employed at CERN since 15.09.1973. The Director-General has sent his family a message of condolence on behalf of the CERN staff. Social Affairs Human Resources Department Rob Wolf passed away on Sunday 20 June after a long illness. Born in June 1947 in The Netherlands, he joined CERN as a fellow in September 1972 after obtaining his doctoral qualification from the University of Leiden (NL) on the degradation of superconducting materials in ionizing radiation. He was a member of the CERN personnel since 1st March 1975. Rob immediately assumed full responsibility for the measurement and qualification of the superconducting materials for the low-beta magnet project of the ISR, and was then tasked with the manufacture of the corrector windings of these magnets. Throughout his working life he remained a reference in these areas. Later, Rob took on the responsibi...

  20. Integral and Multidimensional Linear Distinguishers with Correlation Zero

    DEFF Research Database (Denmark)

    Bogdanov, Andrey; Leander, Gregor; Nyberg, Kaisa

    2012-01-01

    Zero-correlation cryptanalysis uses linear approximations holding with probability exactly 1/2. In this paper, we reveal fundamental links of zero-correlation distinguishers to integral distinguishers and multidimensional linear distinguishers. We show that an integral implies zero-correlation li...... weak key assumptions. © International Association for Cryptologic Research 2012....

  1. Splice junction mutation in some Ashkenazi Jews with Tay-Sachs disease: Evidence against a single defect within this ethnic group

    Energy Technology Data Exchange (ETDEWEB)

    Myerowitz, R. (National Institute of Diabetes, Digestive and Kidney Diseases, Bethesda, MD (USA))

    1988-06-01

    Tay-Sachs disease is an inherited disorder in which the {alpha} chain of the lysosomal enzyme {beta}-N-acetylhexosaminidase A bears the mutation. Ashkenazi Jews are found to be carriers for a severe type of Tay-Sachs disease, the classic form, 10 times more frequently than the general population. Ashkenazi Jewish patients with classic Tay-Sachs disease have appeared to be clinically and biochemically identical, and the usual assumption has been that they harbor the same {alpha}-chain mutation. The author has isolated the {alpha}-chain gene from an Ashkenazi Jewish patient, GM2968, with classic Tay-Sachs disease and compared its nucleotide sequences with that of the normal {alpha}-chain gene in the promoter region, exon and splice junction regions, and polyadenylylation signal area. Only one difference was observed between these sequences. The alteration is presumed to be functionally significant and to result in aberrant mRNA splicing. Utilizing the polymerase chain reaction to amplify the region encompassing the mutation, the author developed an assay to screen patients and heterozygote carriers for this mutation. Surprisingly, in each of two Ashkenazi patients, only one {alpha}-chain allele harbored the splice junction mutation. Only one parent of each of these patients was positive for the defect. Another Ashkenazi patient did not bear this mutation at all nor did either of the subject's parents. The data are consistent with the presence of more than one mutation underlying the classic form of Tay-Sachs disease in the Ashkenazi Jewish population.

  2. Role of the physician in screening for carriers of Tay-Sachs disease.

    Science.gov (United States)

    Lowden, J. A.

    1978-01-01

    A screening test for carriers of Tay-Sachs disease has been available in Toronto for more than 6 years. In that time more than 11 000 Jewish residents have been tested. Most had requested testing after hearing about the screening program from friends or the media; few had been advised by their physicians to be tested. To sample the attitudes of physicians in Toronto towards carrier screening, we studied questionnaire responses of 42 physicians whose practices were composed largely of Jewish patients. Only 31% regularly advised their young adult Jewish patients to have a carrier screening test but 76% said they had patients who asked if they should be tested. Of the 14 (33%) who had had one or more patients with Tay-Sachs disease 6 did not advise carrier testing. There was a positive correlation between specialty training and support for the screening program. Methods for increasing physician advocacy of these programs are discussed. PMID:709448

  3. Kantowski--Sachs cosmological models as big-bang models

    International Nuclear Information System (INIS)

    Weber, E.

    1985-01-01

    In the presence of a nonzero cosmological constant Λ, we classify the anisotropic cosmological models of the Kantowski--Sachs type by means of the quantities epsilon 2 0 , q 0 , summation 0 corresponding, respectively, to the relative root-mean-square deviation from isotropy, the deceleration parameter, and the density parameter of the perfect fluid at a given time t = t 0 . We obtain for Λ>0 a set of big-bang models of zero measure as well as a set of cosmological models of nonzero measure evolving toward the de Sitter solution

  4. Black Hole Entropy from Bondi-Metzner-Sachs Symmetry at the Horizon.

    Science.gov (United States)

    Carlip, S

    2018-03-09

    Near the horizon, the obvious symmetries of a black hole spacetime-the horizon-preserving diffeomorphisms-are enhanced to a larger symmetry group with a three-dimensional Bondi-Metzner-Sachs algebra. Using dimensional reduction and covariant phase space techniques, I investigate this augmented symmetry and show that it is strong enough to determine the black hole entropy in any dimension.

  5. Is science in danger of sanctifying the wolf?

    Science.gov (United States)

    Mech, L. David

    2012-01-01

    Historically the wolf (Canis lupus) was hated and extirpated from most of the contiguous United States. The federal Endangered Species Act fostered wolf protection and reintroduction which improved the species' image. Wolf populations reached biological recovery in the Northern Rocky Mountains and upper Midwest, and the animal has been delisted from the Endangered Species List in those areas. Numerous studies in National Parks suggest that wolves, through trophic cascades, have caused ecosystems to change in ways many people consider positive. Several studies have been conducted in Yellowstone National Park where wolf interactions with their prey, primarily elk (Cervus elaphus), are thought to have caused reduction of numbers or changes in movements and behavior. Some workers consider the latter changes to have led to a behaviorally-mediated trophic cascade. Either the elk reduction or the behavioral changes are hypothesized to have fostered growth in browse, primarily willows (Salix spp.) and aspen (Populus spp.), and that growth has resulted in increased beavers (Castor Canadensis), songbirds, and hydrologic changes. The wolf's image thus has gained an iconic cachet. However, later research challenges several earlier studies' findings such that earlier conclusions are now controversial; especially those related to causes of browse regrowth. In any case, any such cascading effects of wolves found in National Parks would have little relevance to most of the wolf range because of overriding anthropogenic influences there on wolves, prey, vegetation, and other parts of the food web. The wolf is neither a saint nor a sinner except to those who want to make it so.

  6. Rapid identification of HEXA mutations in Tay-Sachs patients.

    Science.gov (United States)

    Giraud, Carole; Dussau, Jeanne; Azouguene, Emilie; Feillet, François; Puech, Jean-Philippe; Caillaud, Catherine

    2010-02-19

    Tay-Sachs disease (TSD) is a recessively inherited neurodegenerative disorder due to mutations in the HEXA gene resulting in a beta-hexosaminidase A (Hex A) deficiency. The purpose of this study was to characterize the molecular abnormalities in patients with infantile or later-onset forms of the disease. The complete sequencing of the 14 exons and flanking regions of the HEXA gene was performed with a unique technical condition in 10 unrelated TSD patients. Eleven mutations were identified, including five splice mutations, one insertion, two deletions and three single-base substitutions. Four mutations were novel: two splice mutations (IVS8+5G>A, IVS2+4delAGTA), one missense mutation in exon 6 (c.621T>G (p.D207E)) and one small deletion (c.1211-1212delTG) in exon 11 resulting in a premature stop codon at residue 429. The c.621T>G missense mutation was found in a patient presenting an infantile form. Its putative role in the pathogenesis of TSD is suspected as residue 207 is highly conserved in human, mouse and rat. Moreover, structural modelling predicted changes likely to affect substrate binding and catalytic activity of the enzyme. The time-saving procedure reported here could be useful for the characterization of Tay-Sachs-causing mutations, in particular in non-Ashkenazi patients mainly exhibiting rare mutations. Copyright (c) 2010 Elsevier Inc. All rights reserved.

  7. Late-onset Tay-Sachs disease: adverse effects of medications and implications for treatment.

    Science.gov (United States)

    Shapiro, B E; Hatters-Friedman, S; Fernandes-Filho, J A; Anthony, K; Natowicz, M R

    2006-09-12

    The authors conducted a retrospective and brief prospective study of adverse effects of approximately 350 medications in 44 adults with late-onset Tay-Sachs disease (LOTS). Some medications were relatively safe, whereas others, particularly haloperidol, risperidone, and chlorpromazine, were associated with neurologic worsening.

  8. Wolf-Hirschhorn Syndrome

    African Journals Online (AJOL)

    Extract Studio

    she had the distinctive facial features of Wolf-Hirschhorn syndrome that included hyperteleorism, ... The PDA noted at birth had closed by 3 months and there was no other cardiac .... followed by pulmonary stenosis, ventricular septal defects,.

  9. Temporary Efficacy of Pyrimethamine in Juvenile-Onset Tay-Sachs Disease Caused by 2 Unreported HEXA Mutations in the Indian Population.

    Science.gov (United States)

    Udwadia-Hegde, Anaita; Hajirnis, Omkar

    2017-01-01

    Juvenile Tay-Sachs disease is rarer than other forms of Tay-Sachs disease and is usually seen in children between the age of 2 and 10 years. Pyrimethamine as a pharmacological chaperone was used to increase β-hexosaminidase A activity in this patient. We describe a patient with Tay-Sachs disease from the Indian population, a juvenile case who presented with developmental regression starting at the age of three, initially with motor followed by language regression. She is currently incapacitated with severe behavioral issues. This brief communication gives an insight into the efficacy of pharmacological chaperones. It also describes two unreported mutations in hexosaminidase A gene from the Indian population. After commencing Pyrimethamine, though initial benefits with increase in levels corresponded with briefly halting the motor regression, the observed increase was only transient and not associated with discernible beneficial neurological or psychiatric effects.

  10. Gamma-ray bursts from tidally spun-up Wolf-Rayet stars?

    NARCIS (Netherlands)

    Detmers, R.G.; Langer, N.; Podsiadlowski, Ph.; Izzard, R.G.

    2008-01-01

    Context. The collapsar model requires rapidly rotating Wolf-Rayet stars as progenitors of long gamma-ray bursts. However, Galactic Wolf-Rayet stars rapidly lose angular momentum due to their intense stellar winds. Aims. We investigate whether the tidal interaction of a Wolf-Rayet star with a compact

  11. Tay-Sachs disease: current perspectives from Australia

    Directory of Open Access Journals (Sweden)

    Lew RM

    2015-01-01

    Full Text Available Raelia M Lew,1,7 Leslie Burnett,2,3,4 Anné L Proos,2 Martin B Delatycki5,6 1Department of Obstetrics and Gynecology, QEII Research Institute for Mothers and Infants, The University of Sydney, Australia; 2NSW Health Pathology North, Royal North Shore Hospital, St Leonards, Australia; 3SEALS, Prince of Wales Hospital, Randwick, Australia; 4Sydney Medical School-Northern, Royal North Shore Hospital E25, University of Sydney, Sydney, Australia; 5Department of Clinical Genetics, Austin Health, Heidelberg, Australia; 6Bruce Lefroy Centre for Genetic Health Research, Murdoch Childrens Research Institute, Parkville, Australia; 7Department of Obstetrics and Gynaecology, Dentistry and Health Sciences, Faculty of Medicine, The University of Melbourne, Melbourne, Australia Abstract: Tay-Sachs disease (TSD is a fatal, recessively inherited neurodegenerative condition of infancy and early childhood. Although rare in most other populations, the carrier frequency is one in 25 in Ashkenazi Jews. Australian high-school-based TSD preconception genetic screening programs aim to screen, educate, and optimize reproductive choice for participants. These programs have demonstrated high uptake, low psychological morbidity, and have been shown to result in fewer than expected Jewish TSD-affected births over 18 years of operation. The majority of Jewish individuals of reproductive age outside of the high school screening program setting in Australia have not accessed screening. Recent recommendations advocate supplementing the community high school screening programs with general practitioner- and obstetrician-led genetic screening of Ashkenazi Jewish individuals for TSD and other severe recessive diseases for which this group is at risk. Massively parallel DNA sequencing is expected to become the testing modality of choice over the coming years. Keywords: Tay-Sachs disease, genetic screening, Australia

  12. Late-onset Tay-Sachs disease.

    Science.gov (United States)

    Barritt, Andrew W; Anderson, Stuart J; Leigh, P Nigel; Ridha, Basil H

    2017-10-01

    We discuss the assessment and differential diagnoses of a young adult Hungarian man with a 1-year history of a progressive and symmetric amyotrophic lateral sclerosis-like syndrome, along with irregular action tremor and stimulus-sensitive myoclonus of the arms. MR scan of the brain showed isolated cerebellar atrophy and formal neuropsychometric testing identified significant subclinical deficits in attention, processing speed and memory. We suspected a form of GM 2 gangliosidosis, and white cell enzyme analysis showed markedly reduced enzymatic activity of β-hexosaminidase A. Genetic testing subsequently revealed two heterozygous pathogenic mutations in the HEXA gene (c.1499delT p.(Leu500fs) and c.805G>A p.(Gly269Ser)), confirming the very rare diagnosis of adult-onset Tay-Sachs disease. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  13. Temporary Efficacy of Pyrimethamine in Juvenile-Onset Tay-Sachs Disease Caused by 2 Unreported HEXA Mutations in the Indian Population

    Directory of Open Access Journals (Sweden)

    Anaita Udwadia-Hegde MD, DCH, MRCPCH

    2017-01-01

    Full Text Available Background: Juvenile Tay-Sachs disease is rarer than other forms of Tay-Sachs disease and is usually seen in children between the age of 2 and 10 years. Pyrimethamine as a pharmacological chaperone was used to increase β-hexosaminidase A activity in this patient. Patient: We describe a patient with Tay-Sachs disease from the Indian population, a juvenile case who presented with developmental regression starting at the age of three, initially with motor followed by language regression. She is currently incapacitated with severe behavioral issues. Conclusion: This brief communication gives an insight into the efficacy of pharmacological chaperones. It also describes two unreported mutations in hexosaminidase A gene from the Indian population. After commencing Pyrimethamine, though initial benefits with increase in levels corresponded with briefly halting the motor regression, the observed increase was only transient and not associated with discernible beneficial neurological or psychiatric effects.

  14. Hot mantles, moderate photospheres for Wolf-Rayet stars

    International Nuclear Information System (INIS)

    Underhill, A.B.

    1982-01-01

    The amount of continuous energy from Wolf-Rayet stars and the shape of the continuous spectrum from the ultraviolet to the near infrared correspond to effective temperatures in the range 25000 to 30000 K. The value of log g is of the order of 4.0 +- 0.5. Thus the photospheres of Wolf-Rayet stars correspond to those of moderately hot stars. The line spectra of Wolf-Rayet stars, however, indicate that electron temperatures greater than 30000 K occur in the outer atmospheres or mantles of these stars. Here outflow is important. (Auth.)

  15. Optical spectrophotometry of Wolf-Rayet galaxies

    Science.gov (United States)

    Vacca, William D.; Conti, Peter S.

    1992-01-01

    We have obtained long-slit optical spectra of 10 Wolf-Rayet galaxies and four other starburst galaxies. Using the nebular emission lines we have determined the electron temperatures, electron densities, extinctions, oxygen abundances, mass of ionized hydrogen, and numbers of ionizing photons due to hot stars in these galaxies. The various forbidden line ratios clearly indicate a stellar origin for the emission-line spectrum. From the flux of the broad He II 4686 A emission feature we have estimated the number of Wolf-Rayet stars present. We have accounted for the contribution of these stars to the total ionizing flux and have calculated the ratio of the number of these stars to the number of O stars. Wolf-Rayet galaxies are among the youngest examples of the starburst phenomenon, which we observed at a propitious moment.

  16. Elk migration patterns and human activity influence wolf habitat use in the Greater Yellowstone Ecosystem.

    Science.gov (United States)

    Nelson, Abigail A; Kauffman, Matthew J; Middleton, Arthur D; Jimenez, Michael D; McWhirter, Douglas E; Barber, Jarrett; Gerow, Kenneth

    2012-12-01

    Identifying the ecological dynamics underlying human-wildlife conflicts is important for the management and conservation of wildlife populations. In landscapes still occupied by large carnivores, many ungulate prey species migrate seasonally, yet little empirical research has explored the relationship between carnivore distribution and ungulate migration strategy. In this study, we evaluate the influence of elk (Cervus elaphus) distribution and other landscape features on wolf (Canis lupus) habitat use in an area of chronic wolf-livestock conflict in the Greater Yellowstone Ecosystem, USA. Using three years of fine-scale wolf (n = 14) and elk (n = 81) movement data, we compared the seasonal habitat use of wolves in an area dominated by migratory elk with that of wolves in an adjacent area dominated by resident elk. Most migratory elk vacate the associated winter wolf territories each summer via a 40-60 km migration, whereas resident elk remain accessible to wolves year-round. We used a generalized linear model to compare the relative probability of wolf use as a function of GIS-based habitat covariates in the migratory and resident elk areas. Although wolves in both areas used elk-rich habitat all year, elk density in summer had a weaker influence on the habitat use of wolves in the migratory elk area than the resident elk area. Wolves employed a number of alternative strategies to cope with the departure of migratory elk. Wolves in the two areas also differed in their disposition toward roads. In winter, wolves in the migratory elk area used habitat close to roads, while wolves in the resident elk area avoided roads. In summer, wolves in the migratory elk area were indifferent to roads, while wolves in resident elk areas strongly avoided roads, presumably due to the location of dens and summering elk combined with different traffic levels. Study results can help wildlife managers to anticipate the movements and establishment of wolf packs as they expand into areas

  17. Impact of gene patents and licensing practices on access to genetic testing and carrier screening for Tay-Sachs and Canavan disease.

    Science.gov (United States)

    Colaianni, Alessandra; Chandrasekharan, Subhashini; Cook-Deegan, Robert

    2010-04-01

    Genetic testing for Tay-Sachs and Canavan disease is particularly important for Ashkenazi Jews, because both conditions are more frequent in that population. This comparative case study was possible because of different patenting and licensing practices. The role of DNA testing differs between Tay-Sachs and Canavan diseases. The first-line screening test for Tay-Sachs remains an enzyme activity test rather than genotyping. Genotyping is used for preimplantation diagnosis and confirmatory testing. In contrast, DNA-based testing is the basis for Canavan screening and diagnosis. The HEXA gene for Tay-Sachs was cloned at the National Institutes of Health, and the gene was patented but has not been licensed. The ASPA gene for Canavan disease was cloned and patented by Miami Children's Hospital. Miami Children's Hospital did not inform family members and patient groups that had contributed to the gene discovery that it was applying for a patent, and pursued restrictive licensing practices when a patent issued in 1997. This led to intense controversy, litigation, and a sealed, nonpublic 2003 settlement that apparently allowed for nonexclusive licensing. A survey of laboratories revealed a possible price premium for ASPA testing, with per-unit costs higher than for other genetic tests in the Secretary's Advisory Committee on Genetics, Health, and Society case studies. The main conclusion from comparing genetic testing for Tay-Sachs and Canavan diseases, however, is that patenting and licensing conducted without communication with patients and advocates cause mistrust and can lead to controversy and litigation, a negative model to contrast with the positive model of patenting and licensing for genetic testing of cystic fibrosis.

  18. The effect of a combined glenoid and Hill-Sachs defect on glenohumeral stability: a biomechanical cadaveric study using 3-dimensional modeling of 142 patients.

    Science.gov (United States)

    Arciero, Robert A; Parrino, Anthony; Bernhardson, Andrew S; Diaz-Doran, Vilmaris; Obopilwe, Elifho; Cote, Mark P; Golijanin, Petr; Mazzocca, Augustus D; Provencher, Matthew T

    2015-06-01

    Bone loss in anterior glenohumeral instability occurs on both the glenoid and the humerus; however, existing biomechanical studies have evaluated glenoid and humeral head defects in isolation. Thus, little is known about the combined effect of these bony lesions in a clinically relevant model on glenohumeral stability. The purpose of this study was to determine the biomechanical efficacy of a Bankart repair in the setting of bipolar (glenoid and humeral head) bone defects determined via computer-generated 3-dimensional (3D) modeling of 142 patients with recurrent anterior shoulder instability. The null hypothesis was that adding a bipolar bone defect will have no effect on glenohumeral stability after soft tissue Bankart repair. Controlled laboratory study. A total of 142 consecutive patients with recurrent anterior instability were analyzed with 3D computed tomography scans. Two Hill-Sachs lesions were selected on the basis of volumetric size representing the 25th percentile (0.87 cm(3); small) and 50th percentile (1.47 cm(3); medium) and printed in plastic resin with a 3D printer. A total of 21 cadaveric shoulders were evaluated on a custom shoulder-testing device permitting 6 degrees of freedom, and the force required to translate the humeral head anteriorly 10 mm at a rate of 2.0 mm/s with a compressive load of 50 N was determined at 60° of glenohumeral abduction and 60° of external rotation. All Bankart lesions were made sharply from the 2- to 6-o'clock positions for a right shoulder. Subsequent Bankart repair with transosseous tunnels using high-strength suture was performed. Hill-Sachs lesions were made in the cadaver utilizing a plastic mold from the exact replica off the 3D printer. Testing was conducted in the following sequence for each specimen: (1) intact, (2) posterior capsulotomy, (3) Bankart lesion, (4) Bankart repair, (5) Bankart lesion with 2-mm glenoid defect, (6) Bankart repair, (7) Bankart lesion with 2-mm glenoid defect and Hill-Sachs lesion

  19. Expanding the spectrum of HEXA mutations in Indian patients with Tay-Sachs disease.

    Science.gov (United States)

    Sheth, Jayesh; Mistri, Mehul; Datar, Chaitanya; Kalane, Umesh; Patil, Shekhar; Kamate, Mahesh; Shah, Harshuti; Nampoothiri, Sheela; Gupta, Sarita; Sheth, Frenny

    2014-01-01

    Tay-Sachs disease is an autosomal recessive neurodegenerative disorder occurring due to impaired activity of β-hexosaminidase-A (EC 3.2.1.52), resulting from the mutation in HEXA gene. Very little is known about the molecular pathology of TSD in Indian children except for a few mutations identified by us. The present study is aimed to determine additional mutations leading to Tay-Sachs disease in nine patients confirmed by the deficiency of β-hexosaminidase-A (C (D175A) and c.805G>C (p.G269R) in one case; and one small 1 bp deletion c.426delT (p.F142LfsX57) and one splice site mutation c.459+4A>C in the other two cases respectively. None of these mutations were detected in 100 chromosomes from healthy individuals of the same ethnic group. Three previously reported missense mutations, (i) c.532C>T (p.R178C), (ii) c.964G>T (p.D322Y), and (iii) c.1385A>T (p.E462V); two nonsense mutations (i) c.709C>T (p.Q237X) and (ii) c.1528C>T (p.R510X), one 4 bp insertion c.1277_1278insTATC (p.Y427IfsX5) and one splice site mutation c.459+5G>A were also identified in six cases. We observe from this study that novel mutations are more frequently observed in Indian patients with Tay-Sachs disease with clustering of ~ 73% of disease causing mutations in exons 5 to 12. This database can be used for a carrier rate screening in the larger population of the country.

  20. Wolf Creek Generating Station containment model

    International Nuclear Information System (INIS)

    Nguyen, D.H.; Neises, G.J.; Howard, M.L.

    1995-01-01

    This paper presents a CONTEMPT-LT/28 containment model that has been developed by Wolf Creek Nuclear Operating Corporation (WCNOC) to predict containment pressure and temperature behavior during the postulated events at Wolf Creek Generating Station (WCGS). The model has been validated using data provided in the WCGS Updated Safety Analysis Report (USAR). CONTEMPT-LT/28 model has been used extensively at WCGS to support plant operations, and recently, to support its 4.5% thermal power uprate project

  1. Observations of southern Wolf-Rayet stars. I

    International Nuclear Information System (INIS)

    Niemela, V.S.

    1976-01-01

    The Wolf-Rayet star HD 90657 is found to be a spectroscopic binary with a WN5+O6 spectrum and a period of 6.456 days. Preliminary orbital elements show an elliptic orbit and a mass ratio Msub(WR)/Msub(O) approximately 0.5. Evidence is presented for the Wolf-Rayet atmosphere being accelerated outwards and not being spherically symmetric. (Auth.)

  2. Substrate reduction therapy in the infantile form of Tay-Sachs disease.

    Science.gov (United States)

    Bembi, B; Marchetti, F; Guerci, V I; Ciana, G; Addobbati, R; Grasso, D; Barone, R; Cariati, R; Fernandez-Guillen, L; Butters, T; Pittis, M G

    2006-01-24

    Substrate reduction therapy (SRT) with miglustat has been proposed for treatment of some lysosomal storage disorders. Based on the positive experience in Gaucher disease and experimental data in Tay-Sachs (TSD) and Sandhoff animal models, the authors investigated the clinical efficacy of SRT in two patients with infantile TSD. SRT could not arrest the patients' neurologic deterioration. However, a significant drug concentration in CSF as well as macrocephaly prevention were observed.

  3. 26Al yields from rotating Wolf--Rayet star models

    OpenAIRE

    Vuissoz, C.; Meynet, G.; Knoedlseder, J.; Cervino, M.; Schaerer, D.; Palacios, A.; Mowlavi, N.

    2003-01-01

    We present new $^{26}$Al stellar yields from rotating Wolf--Rayet stellar models which, at solar metallicity, well reproduce the observed properties of the Wolf-Rayet populations. These new yields are enhanced with respect to non--rotating models, even with respect to non--rotating models computed with enhanced mass loss rates. We briefly discuss some implications of the use of these new yields for estimating the global contribution of Wolf-Rayet stars to the quantity of $^{26}$Al now present...

  4. Severe anemia caused by babesiosis in a maned wolf (Chrysocyon brachyurus).

    Science.gov (United States)

    Phair, Kristen A; Carpenter, James W; Smee, Nicole; Myers, Carl B; Pohlman, Lisa M

    2012-03-01

    An 8-yr-old, captive, spayed, female maned wolf (Chrysocyon brachyurus) developed progressive lethargy and weakness over a 24-hr period. Clinical signs included vomiting, recumbency, horizontal nystagmus, possible blindness, pale icteric mucus membranes, and port-wine colored urine. A complete blood cell count revealed severe anemia (packed cell volume [PCV], 6%) and intraerythrocytic piroplasms consistent with a Babesia species. Polymerase chain reaction testing later confirmed babesiosis. The wolf was treated with imidocarb dipropionate, antibiotics, and fluid therapy. A whole-blood transfusion from a sibling maned wolf also was performed. Despite aggressive treatment, the wolf failed to improve and was euthanized. To the authors' knowledge, this is the first documented case of babesiosis in a captive maned wolf in North America. Surveillance of infectious diseases in captive and wild maned wolf populations should be expanded to include screening for Babesia species. Tick control also should be implemented to prevent and decrease transmission of the disease to this endangered species.

  5. Wolf Attack Probability: A Theoretical Security Measure in Biometric Authentication Systems

    Science.gov (United States)

    Une, Masashi; Otsuka, Akira; Imai, Hideki

    This paper will propose a wolf attack probability (WAP) as a new measure for evaluating security of biometric authentication systems. The wolf attack is an attempt to impersonate a victim by feeding “wolves” into the system to be attacked. The “wolf” means an input value which can be falsely accepted as a match with multiple templates. WAP is defined as a maximum success probability of the wolf attack with one wolf sample. In this paper, we give a rigorous definition of the new security measure which gives strength estimation of an individual biometric authentication system against impersonation attacks. We show that if one reestimates using our WAP measure, a typical fingerprint algorithm turns out to be much weaker than theoretically estimated by Ratha et al. Moreover, we apply the wolf attack to a finger-vein-pattern based algorithm. Surprisingly, we show that there exists an extremely strong wolf which falsely matches all templates for any threshold value.

  6. Late onset Tay-Sachs disease in mice with targeted disruption of the Hexa gene: behavioral changes and pathology of the central nervous system.

    Science.gov (United States)

    Miklyaeva, Elena I; Dong, Weijia; Bureau, Alexandre; Fattahie, Roya; Xu, Yongqin; Su, Meng; Fick, Gordon H; Huang, Jing-Qi; Igdoura, Suleiman; Hanai, Nobuo; Gravel, Roy A

    2004-03-19

    Tay-Sachs disease is an autosomal recessive neurodegenerative disease resulting from a block in the hydrolysis of GM2 ganglioside, an intermediate in ganglioside catabolism. The mouse model of Tay-Sachs disease (Hexa -/-) has been described as behaviorally indistinguishable from wild type until at least 1 year of age due to a sialidase-mediated bypass of the metabolic defect that reduces the rate of GM2 ganglioside accumulation. In this study, we have followed our mouse model to over 2 years of age and have documented a significant disease phenotype that is reminiscent of the late onset, chronic form of human Tay-Sachs disease. Onset occurs at 11-12 months of age and progresses slowly, in parallel with increasing storage of GM2 ganglioside. The disease is characterized by hind limb spasticity, weight loss, tremors, abnormal posture with lordosis, possible visual impairment, and, late in the disease, muscle weakness, clasping of the limbs, and myoclonic twitches of the head. Immunodetection of GM2 ganglioside showed that storage varies widely in different regions, but is most intense in pyriform cortex, hippocampus (CA3 field, subiculum), amygdala, hypothalamus (paraventricular supraoptic, ventromedial and arcuate nuclei, and mammilary body), and the somatosensory cortex (layer V) in 1- to 2-year-old mutant mice. We suggest that the Tay-Sachs mouse model is a phenotypically valid model of disease and may provide for a reliable indicator of the impact of therapeutic strategies, in particular geared to the late onset, chronic form of human Tay-Sachs disease.

  7. Characterization of two Turkish beta-hexosaminidase mutations causing Tay-Sachs disease.

    Science.gov (United States)

    Ozkara, Hatice Asuman; Sandhoff, Konrad

    2003-04-01

    Two homoallelic mutations have recently been identified in the alpha-subunit of hexosaminidase A (EC 3.2.1.52) causing the infantile form of Tay-Sachs disease in Turkish patients. Both of these mutations, a 12 bp deletion (1096-1107 or 1098-1108 or 1099-1109) in exon 10 and a point mutation (G1362 to A, Gly454 to Asp) in exon 12, are located in the catalytic domain of the hexosaminidase alpha-chain. In order to determine whether these mutations affect the function of the catalytic domain or result in an instable protein, both mutant cDNAs were overexpressed in COS-1 cells. As judged by Western blotting, transfections of wild-type cDNA produced pro-alpha-chain and mature alpha-chain in parallel with a fivefold increase in cellular hexosaminidase activity using the synthetic substrate 4-methylumbelliferyl beta-N-acetylglucosamine 6-sulfate (MUGS). However, both mutants produced only pro-alpha-chains, although no mature form or detectable hexosaminidase activity towards two different synthetic substrates was observed. These data are consistent with the biochemical phenotype of infantile Tay-Sachs disease. We conclude that the overexpressed mutant pro-alpha-chains were misfolded and could not undergo further proteolytic processing to the active form of the enzyme in the lysosome.

  8. Expanding the spectrum of HEXA mutations in Indian patients with Tay–Sachs disease

    Directory of Open Access Journals (Sweden)

    Jayesh Sheth

    2014-01-01

    Full Text Available Tay–Sachs disease is an autosomal recessive neurodegenerative disorder occurring due to impaired activity of β-hexosaminidase-A (EC 3.2.1.52, resulting from the mutation in HEXA gene. Very little is known about the molecular pathology of TSD in Indian children except for a few mutations identified by us. The present study is aimed to determine additional mutations leading to Tay–Sachs disease in nine patients confirmed by the deficiency of β-hexosaminidase-A (C (D175A and c.805G>C (p.G269R in one case; and one small 1 bp deletion c.426delT (p.F142LfsX57 and one splice site mutation c.459+4A>C in the other two cases respectively. None of these mutations were detected in 100 chromosomes from healthy individuals of the same ethnic group. Three previously reported missense mutations, (i c.532C>T (p.R178C, (ii c.964G>T (p.D322Y, and (iii c.1385A>T (p.E462V; two nonsense mutations (i c.709C>T (p.Q237X and (ii c.1528C>T (p.R510X, one 4 bp insertion c.1277_1278insTATC (p.Y427IfsX5 and one splice site mutation c.459+5G>A were also identified in six cases. We observe from this study that novel mutations are more frequently observed in Indian patients with Tay–Sachs disease with clustering of ~73% of disease causing mutations in exons 5 to 12. This database can be used for a carrier rate screening in the larger population of the country.

  9. WR 143: A Wolf-Rayet Binary

    OpenAIRE

    Varricatt, Watson P.; Ashok, Nagarhalli M.

    2005-01-01

    Near infrared spectroscopy and photometry of the Wolf-Rayet Star WR 143 (HD 195177) were obtained in the $JHK$ photometric bands. High resolution spectra observed in the J and H bands exhibit narrow 1.083-micron He I line and the H I Pa Beta and the Brackett series lines in emission superposed on the broad emission line spectrum of the Wolf-Rayet star, giving strong indications of the presence of a companion. From the narrow emission lines observed, the companion is identified to be an early-...

  10. Analysis of amplitude-phase disturbances of Wolf's numbers rhythmic structure

    International Nuclear Information System (INIS)

    Vojchishin, K.S.

    1978-01-01

    Statistical analysis of Wolf's number rhythmic structure has been carried out. Wolf's number time series is considered as a stochastic signal with irregular disturbances of rhythmic structure appearing because of random variability of single cycle parameters. A method and an algorythm for transforming the signal, to reduce all quasi-eleven-year cycles of mean-monthly Wolf's numbers to a signal mean duration, to find out and to eliminate rhythmic phase disturbances, are proposed. An estimate of the accuracy of the procedure is given. The results of calculations (on the mean duration range of cycles) of estimates of their mathematical expectation, dispersion and correlation function depending on time and its shift are given. The conclusion that Wolf's number time series may be treated as a sequence of stochastic cycles with randomly varying amplitude, duration and phase is grounded. A possibility for reducing the forecast of smoothed mean-monthly Wolf's numbers for one or more cycles ahead to the forecast of only three abovementioned parameters is pointed out

  11. WOLF REXUS EXPERIMENT - European Planetary Science Congress

    Science.gov (United States)

    Buzdugan, A.

    2017-09-01

    WOLF experiment is developing a reaction wheel-based control system, effectively functioning as active nutation damper. One reaction wheel is used to reduce the undesirable lateral rates of spinning cylindrically symmetric free falling units, ejected from a sounding rocket. Once validated in REXUS flight, the concept and the design developed during WOLF experiment can be used for other application which require a flat spin of the free falling units.

  12. A Reduced Dantzig-Wolfe Decomposition for a Suboptimal Linear MPC

    DEFF Research Database (Denmark)

    Standardi, Laura; Poulsen, Niels Kjølstad; Jørgensen, John Bagterp

    2014-01-01

    Linear Model Predictive Control (MPC) is an efficient control technique that repeatedly solves online constrained linear programs. In this work we propose an economic linear MPC strategy for operation of energy systems consisting of multiple and independent power units. These systems cooperate...

  13. ACOG committee opinion. Number 318, October 2005. Screening for Tay-Sachs disease.

    Science.gov (United States)

    2005-10-01

    Tay-Sachs disease (TSD) is a severe progressive neurologic disease that causes death in early childhood. Carrier screening, should be offered before pregnancy to individuals and couples at high-risk, including those of Ashkenazi Jewish, French-Canadian, or Cajun descent and those with a family history consistent with TSD. If both partners are determined to be carriers of TSD, genetic counseling and prenatal diagnosis should be offered.

  14. Doença de Tay-Sachs em criança de raça negra Tay-Sachs disease in a Negro child

    Directory of Open Access Journals (Sweden)

    Sérgio Rosemberg

    1970-12-01

    Full Text Available É relatado um caso de doença de Tay-Sachs em criança da raça negra. O encontro desta entidade em indivíduos não judeus é bastante raro, sendo este o quinto caso descrito em criança negra. Os autores estudam alguns aspectos genéticos, chegando à conclusão de que o cálculo da freqüência do gene mutante é impossível devido ao pequeno número de casos e ao fato de que não há estimativas seguras quanto à taxa de nascimento de indivíduos negros na população, Em que pese o fato de o diagnóstico se assentar em bases puramente clínicas e anatômicas, prescindindo os autores de exames bioquímicos específicos, os dados apresentados são congruentes com os casos clássicos descritos na literatura.A case of Tay-Sachs diseases in a Negro child is reported. The occurrence of this disease in non-Jewish individuals is very rare and this is the fifth case of a Negro-child reported in the litterature. Some genetical aspects are studied and the authors conclude that the frequency calculation of the mutant gene is impossible due to the small number of cases and to the fact that there is reliable information regarding the rate of birth of Negro individuals in the general population. The diagnosis was based on ophtalmological and clinical data. No specific biochemical tests were performed but the data obtained are congruent with other cases reported in the litterature.

  15. Cytogenomic Integrative Network Analysis of the Critical Region Associated with Wolf-Hirschhorn Syndrome

    Directory of Open Access Journals (Sweden)

    Thiago Corrêa

    2018-01-01

    Full Text Available Deletions in the 4p16.3 region are associated with Wolf-Hirschhorn syndrome (WHS, a contiguous gene deletion syndrome involving variable size deletions. In this study, we perform a cytogenomic integrative analysis combining classical cytogenetic methods, fluorescence in situ hybridization (FISH, chromosomal microarray analysis (CMA, and systems biology strategies, to establish the cytogenomic profile involving the 4p16.3 critical region and suggest WHS-related intracellular cell signaling cascades. The cytogenetic and clinical patient profiles were evaluated. We characterized 12 terminal deletions, one interstitial deletion, two ring chromosomes, and one classical translocation 4;8. CMA allowed delineation of the deletions, which ranged from 3.7 to 25.6 Mb with breakpoints from 4p16.3 to 4p15.33. Furthermore, the smallest region of overlapping (SRO encompassed seven genes in a terminal region of 330 kb in the 4p16.3 region, suggesting a region of susceptibility to convulsions and microcephaly. Therefore, molecular interaction networks and topological analysis were performed to understand these WHS-related symptoms. Our results suggest that specific cell signaling pathways including dopamine receptor, NAD+ nucleosidase activity, and fibroblast growth factor-activated receptor activity are associated with the diverse pathological WHS phenotypes and their symptoms. Additionally, we identified 29 hub-bottlenecks (H-B nodes with a major role in WHS.

  16. Unilaterally and rapidly progressing white matter lesion and elevated cytokines in a patient with Tay-Sachs disease.

    Science.gov (United States)

    Hayase, Tomomi; Shimizu, Jun; Goto, Tamako; Nozaki, Yasuyuki; Mori, Masato; Takahashi, Naoto; Namba, Eiji; Yamagata, Takanori; Momoi, Mariko Y

    2010-03-01

    We report the case of a girl with Tay-Sachs disease who had convulsions and deteriorated rapidly after an upper respiratory infection at the age of 11 months. At the age of 16 months, her seizures became intractable and magnetic resonance imaging of the brain showed high signal intensity on T2-weighted images and marked swelling in the white matter and basal nucelei of the right hemisphere. Her seizures and right hemisphere lesion improved with glycerol and dexamethasone treatment. When dexamethasone was discontinued, her symptoms worsened and lesions later appeared in the left hemisphere. Her cerebrospinal fluid showed elevated levels of the cytokines TNF-alpha and IL-5. It is considered that inflammation contributes to disease progression in Tay-Sachs disease.

  17. Long GRBs from Binary Stars: Runaway, Wolf-Rayet Progenitors

    NARCIS (Netherlands)

    Cantiello, M.; Yoon, S.C.; Langer, N.; Livio, M.

    2007-01-01

    The collapsar model for long gamma-ray bursts requires a rapidly rotating Wolf-Rayet star as progenitor. We test the idea of producing rapidly rotating Wolf-Rayet stars in massive close binaries through mass accretion and consecutive quasi-chemically homogeneous evolution - the latter had previously

  18. Affinity for music in Wolf-Hirschhorn syndrome: two case reports.

    Science.gov (United States)

    Arakawa, Chikako; Fujita, Yukihiko; Fuchigami, Tatsuo; Kawamura, Yuki; Ishii, Wakako; Endo, Ayumi; Kohira, Ryutaro; Takahashi, Shori

    2014-10-01

    Wolf-Hirschhorn syndrome is a congenital malformation syndrome resulting from deletion of the short arm of chromosome 4. Individuals with Wolf-Hirschhorn syndrome may have a "Greek warrior helmet" appearance, growth retardation, developmental delay, muscular hypotonia, epilepsy, and difficulty with language including verbal communication. An affinity for music has not previously been reported in these patients. We describe two patients with Wolf-Hirschhorn syndrome who both have a strong affinity for music. One patient is a 20-year-old woman who likes to listen to music all day and can hum many tunes. The other patient is a 9-year-old girl who is calmed by music and received music therapy, with subsequent improvement in her communication skills (eye contact, joint attention, and vocalizations to request music). Individuals with Wolf-Hirschhorn syndrome may have a strong affinity for music and may benefit from music therapy. Additional studies are needed to investigate the interest in music in individuals with Wolf-Hirschhorn syndrome. Copyright © 2014 Elsevier Inc. All rights reserved.

  19. Long GRBs from binary stars: runaway, Wolf-Rayet progenitors

    NARCIS (Netherlands)

    Cantiello, M.; Yoon, S.C.; Langer, N.; Livio, M.

    2007-01-01

    The collapsar model for long gamma-ray bursts requires a rapidly rotating Wolf-Rayet star as progenitor. We test the idea of producing rapidly rotating Wolf-Rayet stars in massive close binaries through mass accretion and consecutive quasi-chemically homogeneous evolution — the latter had previously

  20. Neuro-ophthalmology of late-onset Tay-Sachs disease (LOTS).

    Science.gov (United States)

    Rucker, J C; Shapiro, B E; Han, Y H; Kumar, A N; Garbutt, S; Keller, E L; Leigh, R J

    2004-11-23

    Late-onset Tay-Sachs disease (LOTS) is an adult-onset, autosomal recessive, progressive variant of GM2 gangliosidosis, characterized by involvement of the cerebellum and anterior horn cells. To determine the range of visual and ocular motor abnormalities in LOTS, as a prelude to evaluating the effectiveness of novel therapies. Fourteen patients with biochemically confirmed LOTS (8 men; age range 24 to 53 years; disease duration 5 to 30 years) and 10 age-matched control subjects were studied. Snellen visual acuity, contrast sensitivity, color vision, stereopsis, and visual fields were measured, and optic fundi were photographed. Horizontal and vertical eye movements (search coil) were recorded, and saccades, pursuit, vestibulo-ocular reflex (VOR), vergence, and optokinetic (OK) responses were measured. All patients showed normal visual functions and optic fundi. The main eye movement abnormality concerned saccades, which were "multistep," consisting of a series of small saccades and larger movements that showed transient decelerations. Larger saccades ended earlier and more abruptly (greater peak deceleration) in LOTS patients than in control subjects; these changes can be attributed to premature termination of the saccadic pulse. Smooth-pursuit and slow-phase OK gains were reduced, but VOR, vergence, and gaze holding were normal. Patients with late-onset Tay-Sachs disease (LOTS) show characteristic abnormalities of saccades but normal afferent visual systems. Hypometria, transient decelerations, and premature termination of saccades suggest disruption of a "latch circuit" that normally inhibits pontine omnipause neurons, permitting burst neurons to discharge until the eye movement is completed. These measurable abnormalities of saccades provide a means to evaluate the effects of novel treatments for LOTS.

  1. Higher spin currents in Wolf space. Part I

    Energy Technology Data Exchange (ETDEWEB)

    Ahn, Changhyun [Department of Physics, Kyungpook National University,Taegu 702-701 (Korea, Republic of)

    2014-03-20

    For the N=4 superconformal coset theory described by ((SU(N+2))/(SU(N))) (that contains a Wolf space) with N=3, the N=2 WZW affine current algebra with constraints is obtained. The 16 generators of the large N=4 linear superconformal algebra are described by those WZW affine currents explicitly. By factoring out four spin-(1/2) currents and the spin-1 current from these 16 generators, the remaining 11 generators (spin-2 current, four spin-(3/2) currents, and six spin-1 currents) corresponding to the large N=4 nonlinear superconformal algebra are obtained. Based on the recent work by Gaberdiel and Gopakumar on the large N=4 holography, the extra 16 currents, with spin contents (1,(3/2),(3/2),2), ((3/2),2,2,(5/2)), ((3/2),2,2,(5/2)), and (2,(5/2),(5/2),3) described in terms of N=2 multiplets, are obtained and realized by the WZW affine currents. As a first step towards N=4W algebra (which is NOT known so far), the operator product expansions (OPEs) between the above 11 currents and these extra 16 higher spin currents are found explicitly. It turns out that the composite fields with definite U(1) charges, made of above (11+16) currents (which commute with the Wolf space subgroup SU(N=3)×SU(2)×U(1) currents), occur in the right hand sides of these OPEs.

  2. O stars and Wolf-Rayet stars

    International Nuclear Information System (INIS)

    Conti, P.S.; Underhill, A.B.; Jordan, S.; Thomas, R.

    1988-01-01

    Basic information is given about O and Wolf-Rayet stars indicating how these stars are defined and what their chief observable properties are. Part 2 of the volume discussed four related themes pertaining to the hottest and most luminous stars. Presented are: an observational overview of the spectroscopic classification and extrinsic properties of O and Wolf-Rayet stars; the intrinsic parameters of luminosity, effective temperature, mass, and composition of the stars, and a discussion of their viability; stellar wind properties; and the related issues concerning the efforts of stellar radiation and wind on the immediate interstellar environment are presented

  3. O stars and Wolf-Rayet stars

    Science.gov (United States)

    Conti, Peter S.; Underhill, Anne B.; Jordan, Stuart (Editor); Thomas, Richard (Editor)

    1988-01-01

    Basic information is given about O and Wolf-Rayet stars indicating how these stars are defined and what their chief observable properties are. Part 2 of the volume discussed four related themes pertaining to the hottest and most luminous stars. Presented are: an observational overview of the spectroscopic classification and extrinsic properties of O and Wolf-Rayet stars; the intrinsic parameters of luminosity, effective temperature, mass, and composition of the stars, and a discussion of their viability; stellar wind properties; and the related issues concerning the efforts of stellar radiation and wind on the immediate interstellar environment are presented.

  4. Wolf Search Algorithm for Solving Optimal Reactive Power Dispatch Problem

    Directory of Open Access Journals (Sweden)

    Kanagasabai Lenin

    2015-03-01

    Full Text Available This paper presents a new bio-inspired heuristic optimization algorithm called the Wolf Search Algorithm (WSA for solving the multi-objective reactive power dispatch problem. Wolf Search algorithm is a new bio – inspired heuristic algorithm which based on wolf preying behaviour. The way wolves search for food and survive by avoiding their enemies has been imitated to formulate the algorithm for solving the reactive power dispatches. And the speciality  of wolf is  possessing  both individual local searching ability and autonomous flocking movement and this special property has been utilized to formulate the search algorithm .The proposed (WSA algorithm has been tested on standard IEEE 30 bus test system and simulation results shows clearly about the good performance of the proposed algorithm .

  5. Using radioecological data to determine prey selection by the Alaska wolf

    International Nuclear Information System (INIS)

    Holleman, D.F.; Luick, J.R.

    1978-01-01

    Recently the predation of the Alaska wolf (Canis lupus pambasileus) upon various species of big game has been the subject of considerable controversy between game management specialists and environmentalists. The basis of this controversy centers primarily on the selectivity and extent of prey utilization by the wolf. This report suggests how radioecological data can be used to assess both qualitative and quantitative aspects of wolf predation. Primary prey species of the wolf have distinctly different fallout radiocesium body burdens; e.g., reindeer/caribou have high radiocesium body burdens, whereas moose and small game have low radiocesium body burdens. Consequently, the resulting radiocesium body burden of the wolf depends upon the type and quantity of prey species consumed. Laboratory measurements for this study show a wide variation of radiocesium concentrations of skeletal muscle of wolves within Alaska. Values ranged from 263 to 17,300 pCi 137 Cs/kg of wet muscle. These data relate to known degrees of reindeer/caribou predation by the wolves. A radiocesium kinetic model was constructed from data obtained with wolves and other arctic carnivores and was used to estimate reindeer/caribou intake by wolves. Estimates ranged from 40 to 1650 g of reindeer/caribou muscle per day per wolf. Although the application has limitations, it could yield useful information for evaluating the food habits of wolves, especially in areas of the state where it is important to know the extent of reindeer/caribou utilization by the wolf

  6. Elk migration patterns and human activity influence wolf habitat use in the Greater Yellowstone Ecosystem

    Science.gov (United States)

    Nelson, Abigail; Kauffman, Matthew J.; Middleton, Arthur D.; Jimenez, Mike; McWhirter, Douglas; Barber, Jarrett; Gerow, Ken

    2012-01-01

    Identifying the ecological dynamics underlying human–wildlife conflicts is important for the management and conservation of wildlife populations. In landscapes still occupied by large carnivores, many ungulate prey species migrate seasonally, yet little empirical research has explored the relationship between carnivore distribution and ungulate migration strategy. In this study, we evaluate the influence of elk (Cervus elaphus) distribution and other landscape features on wolf (Canis lupus) habitat use in an area of chronic wolf–livestock conflict in the Greater Yellowstone Ecosystem, USA. Using three years of fine-scale wolf (n = 14) and elk (n = 81) movement data, we compared the seasonal habitat use of wolves in an area dominated by migratory elk with that of wolves in an adjacent area dominated by resident elk. Most migratory elk vacate the associated winter wolf territories each summer via a 40–60 km migration, whereas resident elk remain accessible to wolves year-round. We used a generalized linear model to compare the relative probability of wolf use as a function of GIS-based habitat covariates in the migratory and resident elk areas. Although wolves in both areas used elk-rich habitat all year, elk density in summer had a weaker influence on the habitat use of wolves in the migratory elk area than the resident elk area. Wolves employed a number of alternative strategies to cope with the departure of migratory elk. Wolves in the two areas also differed in their disposition toward roads. In winter, wolves in the migratory elk area used habitat close to roads, while wolves in the resident elk area avoided roads. In summer, wolves in the migratory elk area were indifferent to roads, while wolves in resident elk areas strongly avoided roads, presumably due to the location of dens and summering elk combined with different traffic levels. Study results can help wildlife managers to anticipate the movements and establishment of wolf packs as they expand into

  7. Wolf (Canis lupus) generation time and proportion of current breeding females by age

    Science.gov (United States)

    Mech, L. David; Barber-Meyer, Shannon M.; Erb, John

    2016-01-01

    Information is sparse about aspects of female wolf (Canis lupus) breeding in the wild, including age of first reproduction, mean age of primiparity, generation time, and proportion of each age that breeds in any given year. We studied these subjects in 86 wolves (113 captures) in the Superior National Forest (SNF), Minnesota (MN), during 1972–2013 where wolves were legally protected for most of the period, and in 159 harvested wolves from throughout MN wolf range during 2012–2014. Breeding status of SNF wolves were assessed via nipple measurements, and wolves from throughout MN wolf range, by placental scars. In the SNF, proportions of currently breeding females (those breeding in the year sampled) ranged from 19% at age 2 to 80% at age 5, and from throughout wolf range, from 33% at age 2 to 100% at age 7. Excluding pups and yearlings, only 33% to 36% of SNF females and 58% of females from throughout MN wolf range bred in any given year. Generation time for SNF wolves was 4.3 years and for MN wolf range, 4.7 years. These findings will be useful in modeling wolf population dynamics and in wolf genetic and dog-domestication studies.

  8. Atypical presentation of late-onset Tay-Sachs disease.

    Science.gov (United States)

    Deik, Andres; Saunders-Pullman, Rachel

    2014-05-01

    Late-onset Tay-Sachs disease (LOTS) is a lysosomal storage disease caused by deficient Beta-hexosaminidase A activity. We describe a 53-year-old woman who presented with adult-onset leg weakness, and whose initial diagnosis was progressive muscular atrophy without identifiable etiology. Development of cerebellar ataxia in mid-life prompted reassessment. Beta-hexosaminidase A quantification assay demonstrated absence of the isozyme. Genetic testing identified compound heterozygous mutations in the HEXA gene, confirming the diagnosis of LOTS. The phenotypic spectrum of LOTS includes motor neuronopathy, ataxia, choreoathetosis, neuropathy, and psychiatric symptoms in various combinations. This patient highlights the emergence of different clinical features over many years and emphasizes the need to consider LOTS in the differential diagnosis of progressive muscular atrophy. Copyright © 2013 Wiley Periodicals, Inc.

  9. The challenge of wolf recovery: an ongoing dilemma for state managers

    Science.gov (United States)

    Mech, L. David

    2013-01-01

    “Dave, would you do another legal declaration on the wolf for us?” The weary voice on the phone belonged to Mike Jimenez, Northern Rocky Mountain Wolf Management and Science Coordinator for the U.S. Fish and Wildlife Service (FWS). He was calling from Wyoming to ask me to prepare a document to address a legal challenge to the FWS’s August 2012 delisting of the wolf (Canis lupus) in Wyoming, a highly controversial move. Mike’s tone reflected the reality that — as so many wildlife biologists know and live each day — wildlife management is mainly people management. This contention could not be truer for managing any wildlife species than for managing the wolf. Dubbed “the beast of waste and desolation” by Teddy Roosevelt (The Wilderness Hunter 1893/1900), wolves had been universally hated as prolific predators of valuable livestock and game. Around the turn of the 20th century, members of the U.S. Biological Survey and various state agents, ranchers, cowboys, and other frontiersmen poisoned and persecuted wolves, extirpating them from most of the contiguous United States (Young and Goldman 1944). By 1967, Minnesota and nearby Isle Royale National Park in Michigan held the only remaining wolves in the Lower 48 states, prompting the FWS to place the wolf on the Endangered Species List (established by the Endangered Species Preservation Act of 1966). The wolf then became the list’s poster species, and the timing was ideal: Silent Spring (Carson 1962) had just seeded and fertilized the environmental movement, which blossomed on Earth Day (April 22, 1970) into the environmental revolution. “Save the wolf!” became one of the movement’s rallying cries. And save the wolf we did.

  10. Are luminous and metal-rich Wolf Rayet stars inflated?

    NARCIS (Netherlands)

    Petrovic, J.; Pols, O.; Langer, N.

    2006-01-01

    Aims. We investigate the influence of metallicity and stellar wind mass loss on the radius of Wolf-Rayet stars. Methods. We have calculated chemically homogeneous models of Wolf-Rayet stars of 10 to 200 M for two metallicities (Z = 0.02 and Z = 0.001), without mass loss, using OPAL opacities. We

  11. Wolf's syndrome in a neonatal period: new find neuroradiology

    International Nuclear Information System (INIS)

    Gracia Chapulle, A.; Alvarez Villa, A.; Diaz Diaz, E.; Lopez Suarez, Y.

    1992-01-01

    We report a new born patient with Wolf's syndrome. We contribute with the most frequently clinical, genetics and radiological findings including a radiological discovery linked a malformation of the central nervous system, consistent in a genesia of the corpus callosum , not described so far in the reviewed literature about the Wolf syndrome. (author)

  12. Adaptive Kronrod-Patterson integration of non-linear finite-element matrices

    DEFF Research Database (Denmark)

    Janssen, Hans

    2010-01-01

    inappropriate discretization. In response, this article develops adaptive integration, based on nested Kronrod-Patterson-Gauss integration schemes: basically, the integration order is adapted to the locally observed grade of non-linearity. Adaptive integration is developed based on a standard infiltration...

  13. Generalized Bondi-Sachs equations for characteristic formalism of numerical relativity

    Science.gov (United States)

    Cao, Zhoujian; He, Xiaokai

    2013-11-01

    The Cauchy formalism of numerical relativity has been successfully applied to simulate various dynamical spacetimes without any symmetry assumption. But discovering how to set a mathematically consistent and physically realistic boundary condition is still an open problem for Cauchy formalism. In addition, the numerical truncation error and finite region ambiguity affect the accuracy of gravitational wave form calculation. As to the finite region ambiguity issue, the characteristic extraction method helps much. But it does not solve all of the above issues. Besides the above problems for Cauchy formalism, the computational efficiency is another problem. Although characteristic formalism of numerical relativity suffers the difficulty from caustics in the inner near zone, it has advantages in relation to all of the issues listed above. Cauchy-characteristic matching (CCM) is a possible way to take advantage of characteristic formalism regarding these issues and treat the inner caustics at the same time. CCM has difficulty treating the gauge difference between the Cauchy part and the characteristic part. We propose generalized Bondi-Sachs equations for characteristic formalism for the Cauchy-characteristic matching end. Our proposal gives out a possible same numerical evolution scheme for both the Cauchy part and the characteristic part. And our generalized Bondi-Sachs equations have one adjustable gauge freedom which can be used to relate the gauge used in the Cauchy part. Then these equations can make the Cauchy part and the characteristic part share a consistent gauge condition. So our proposal gives a possible new starting point for Cauchy-characteristic matching.

  14. Extinguishing a learned response in a free-ranging gray wolf (Canis lupus)

    Science.gov (United States)

    Mech, L. David

    2017-01-01

    A free-ranging Gray Wolf (Canis lupus), habituated to human presence (the author) on Ellesmere Island, Canada, learned to anticipate experimental feeding by a human, became impatient, persistent, and bold and exhibited stalking behaviour toward the food source. Only after the author offered the wolf about 90 clumps of dry soil over a period of 45 minutes in three bouts, did the wolf give up this behaviour. To my knowledge, this is the first example of extinguishing a learned response in a free-ranging wolf and provides new insight into the learning behaviour of such animals.

  15. Are luminous and metal-rich Wolf-Rayet stars inflated?

    NARCIS (Netherlands)

    Petrovic, J.; Pols, O.; Langer, N.

    2006-01-01

    Aims.We investigate the influence of metallicity and stellar wind mass loss on the radius of Wolf-Rayet stars.
    Methods: .We have calculated chemically homogeneous models of Wolf-Rayet stars of 10 to 200 Mo for two metallicities (Z=0.02 and Z=0.001), without mass loss, using OPAL

  16. Studies of wolf x coyote hybridization via artificial insemination

    Science.gov (United States)

    Mech, L. David; Asa, Cheryl S.; Callahan, Margaret; Christensen, Bruce W.; Smith, Fran; Young, Julie K.

    2017-01-01

    Following the production of western gray wolf (Canis lupus) x western coyote (Canis latrans) hybrids via artificial insemination (AI), the present article documents that the hybrids survived in captivity for at least 4 years and successfully bred with each other. It further reports that backcrossing one of the hybrids to a male gray wolf by AI also resulted in the birth of live pups that have survived for at least 10 months. All male hybrids (F1 and F2) produced sperm by about 10 months of age, and sperm quality of the F1 males fell within the fertile range for domestic dogs, but sperm motility and morphology, in particular, were low in F2 males at 10 months but improved in samples taken at 22 months of age. These studies are relevant to a long-standing controversy about the identity of the red wolf (Canis rufus), the existence of a proposed new species (Canis lycaon) of gray wolf, and to the role of hybridization in mammalian evolution.

  17. GM2-Gangliosidosis (Sandhoff and Tay Sachs disease): Diagnosis and Neuroimaging Findings (An Iranian Pediatric Case Series).

    Science.gov (United States)

    Karimzadeh, Parvaneh; Jafari, Narjes; Nejad Biglari, Habibeh; Jabbeh Dari, Sayena; Ahmad Abadi, Farzad; Alaee, Mohammad-Reza; Nemati, Hamid; Saket, Sasan; Tonekaboni, Seyed Hasan; Taghdiri, Mohammad-Mahdi; Ghofrani, Mohammad

    2014-01-01

    GM2-Gangliosidosis disease is a rare autosomal recessive genetic disorder that includes two disorders (Tay-Sachs and Sandhoff disease).These disorders cause a progressive deterioration of nerve cells and inherited deficiency in creating hexosaminidases A, B, and AB. Patients who were diagnosed withGM2-Gangliosidosis in the Neurology Department of Mofid Children's Hospital in Tehran, Iran from October 2009 to February 2014were included in our study. The disorder was confirmed by neurometabolic and enzyme level detection of hexosaminidases A, B, and AB in reference to Wagnester Laboratory in Germany. We assessed age, gender, past medical history, developmental status, clinical manifestations, and neuroimaging findings of 9 patients with Sandhoff disease and 9 with Tay Sachs disease. 83% of our patients were the offspring of consanguineous marriages. All of them had a developmental disorder as a chief complaint. 38%of patients had a history of developmental delay or regression and 22% had seizures. The patients with Sandhoff and Tay Sachs disease were followed for approximately 5 years and the follow-up showed all patients were bedridden or had expired due to refractory seizures, pneumonia aspiration, or swallowing disorders. Neuro-imaging findings included bilateral thalamic involvement, brain atrophy, and hypo myelination in near half of our patients (48%). According to the results of this study, we suggest that cherry-red spots, hyperacusis, refractory seizures, and relative parents in children with developmental delay and/or regression should be considered for assessment of GM2-Gangliosidosis disease.

  18. Targeted disruption of the Hexa gene results in mice with biochemical and pathologic features of Tay-Sachs disease

    Energy Technology Data Exchange (ETDEWEB)

    Proia, R.L.; Yamanaka, S.; Johnson, M.D. [and others

    1994-09-01

    Tay-Sachs disease, the prototype of the G{sub M2} gangliosidoses, is a catastrophic neurodegenerative disorder of infancy. The disease is caused by mutations in the HEXA gene resulting in an absence of the lysosomal enzyme, {beta}-hexosaminidase A. As consequence of the enzyme deficiency, G{sub M2} ganglioside accumulates progressively, beginning early in fetal life, to excessive amounts in the central nervous system (CNS). Rapid mental and motor deterioration starting in the first year of life leads to death by 2 to 4 years of age. Through the targeted disruption of the Hexa gene in embryonic stem cells, we have produced mice with biochemical and neuropathologic features of Tay-Sachs disease. The mutant mice exhibited less than 1% of normal {beta}-hexosaminidase A activity and accumulated G{sub M2} ganglioside in their CNS in an age-dependent manner. The accumulated ganglioside was stored in neurons as membranous cytoplasmic bodies characteristically found in the neurons of Tay-Sachs disease patients. At three to five months of age the mutant mice showed no apparent defects in motor or memory function. These {beta}-hexosaminidase A deficient mice should be useful for devising strategies to introduce functional enzymes and genes into the CNS. This model may also be valuable for studying the biochemical and pathologic changes occurring during the course of the disease.

  19. Bucking the trend in wolf-dog hybridization: first evidence from europe of hybridization between female dogs and male wolves.

    Science.gov (United States)

    Hindrikson, Maris; Männil, Peep; Ozolins, Janis; Krzywinski, Andrzej; Saarma, Urmas

    2012-01-01

    Studies on hybridization have proved critical for understanding key evolutionary processes such as speciation and adaptation. However, from the perspective of conservation, hybridization poses a concern, as it can threaten the integrity and fitness of many wild species, including canids. As a result of habitat fragmentation and extensive hunting pressure, gray wolf (Canis lupus) populations have declined dramatically in Europe and elsewhere during recent centuries. Small and fragmented populations have persisted, but often only in the presence of large numbers of dogs, which increase the potential for hybridization and introgression to deleteriously affect wolf populations. Here, we demonstrate hybridization between wolf and dog populations in Estonia and Latvia, and the role of both genders in the hybridization process, using combined analysis of maternal, paternal and biparental genetic markers. Eight animals exhibiting unusual external characteristics for wolves - six from Estonia and two from Latvia - proved to be wolf-dog hybrids. However, one of the hybridization events was extraordinary. Previous field observations and genetic studies have indicated that mating between wolves and dogs is sexually asymmetrical, occurring predominantly between female wolves and male dogs. While this was also the case among the Estonian hybrids, our data revealed the existence of dog mitochondrial genomes in the Latvian hybrids and, together with Y chromosome and autosomal microsatellite data, thus provided the first evidence from Europe of mating between male wolves and female dogs. We discuss patterns of sexual asymmetry in wolf-dog hybridization.

  20. The sixth catalogue of galactic Wolf-Rayet stars, their past and present

    International Nuclear Information System (INIS)

    Hucht, K.A. van der; Conti, P.S.; Lundstroem, I.; Stenholm, B.

    1981-01-01

    This paper presents the Sixth Catalogue of galactic Wolf-Rayet stars (Pop I), a short history on the five earlier WR catalogues, improved spectral classification, finding charts, a discussion on related objects, and a review of the current statur of Wolf-Rayet star research. The appendix presents a bibliography on most of the Wolf-Rayet literature published since 1867. (orig.)

  1. On linearity of pan-integral and pan-integrable functions space

    Czech Academy of Sciences Publication Activity Database

    Ouyang, Y.; Li, J.; Mesiar, Radko

    2017-01-01

    Roč. 90, č. 1 (2017), s. 307-318 ISSN 0888-613X Institutional support: RVO:67985556 Keywords : linearity * monotone measure * Pan-integrable space Subject RIV: BA - General Mathematics OBOR OECD: Pure mathematics Impact factor: 2.845, year: 2016 http://library.utia.cas.cz/separaty/2017/E/mesiar-0477549.pdf

  2. Ecology and conservation of the maned wolf: introduction

    OpenAIRE

    Consorte-McCrea, A.; Santos, Eliana Ferraz

    2013-01-01

    Wolves are considered controversial figures worldwide: mythical creatures as well as troublemakers, their conservation can polarize extreme views and it often is riddled with conflicts. In recent years much effort has focussed on the discussion of how to conserve large carnivores, such as wolves, while addressing public concerns. The maned wolf, however, has mostly eluded the discussion stage. With its solitary habits and unusual fruit-eating diet, the endangered maned wolf roams the grasslan...

  3. Shock absorption of below-knee prostheses : A comparison between the SACH and the Multiflex foot

    NARCIS (Netherlands)

    van Leeuwen, J. L.; Speth, L. A W M; Daanen, H. A M

    1990-01-01

    Shock waves were measured during walking on a treadmill on the metal tube of a below-knee KBM prosthesis, provided either with a SACH foot or with a Multiflex foot. Accelerations were measured in the axial direction and the dorso-ventral direction, about 160 mm proximal to the sole of the shoe. The

  4. Predicting red wolf release success in the southeastern United States

    Science.gov (United States)

    van Manen, Frank T.; Crawford, Barron A.; Clark, Joseph D.

    2000-01-01

    Although the red wolf (Canis rufus) was once found throughout the southeastern United States, indiscriminate killing and habitat destruction reduced its range to a small section of coastal Texas and Louisiana. Wolves trapped from 1973 to 1980 were taken to establish a captive breeding program that was used to repatriate 2 mainland and 3 island red wolf populations. We collected data from 320 red wolf releases in these areas and classified each as a success or failure based on survival and reproductive criteria, and whether recaptures were necessary to resolve conflicts with humans. We evaluated the relations between release success and conditions at the release sites, characteristics of released wolves, and release procedures. Although <44% of the variation in release success was explained, model performance based on jackknife tests indicated a 72-80% correct prediction rate for the 4 operational models we developed. The models indicated that success was associated with human influences on the landscape and the level of wolf habituation to humans prior to release. We applied the models to 31 prospective areas for wolf repatriation and calculated an index of release success for each area. Decision-makers can use these models to objectively rank prospective release areas and compare strengths and weaknesses of each.

  5. Kantowski-Sachs multidimensional cosmological models and dynamical dimensional reduction

    International Nuclear Information System (INIS)

    Demianski, M.; Rome Univ.; Golda, Z.A.; Heller, M.; Szydlowski, M.

    1988-01-01

    Einstein's field equations are solved for a multidimensional spacetime (KS) x Tsup(m), where (KS) is a four-dimensional Kantowski-Sachs spacetime and Tsup(m) is an m-dimensional torus. Among all possible vacuum solutions there is a large class of spacetimes in which the macroscopic space expands and the microscopic space contracts to a finite volume. We also consider a non-vacuum case and we explicitly solve the field equations for the matter satisfying the Zel'dovich equation of state. In non-vacuum models, with matter satisfying an equation of state p = γρ, O ≤ γ < 1, at a sufficiently late stage of evolution the microspace always expands and the dynamical dimensional reduction does not occur. (author)

  6. The theory of radiation driven stellar winds and the Wolf-Rayet phenomenon

    International Nuclear Information System (INIS)

    Abbott, D.C.

    1982-01-01

    The author considers the question of whether the mass loss observed from Wolf-Rayet stars can be explained by a version of wind theory which is scaled to the conditions found in the envelopes of Wolf-Rayet stars. He discusses the following topics: - The calculated radiation pressure in OB stars, and its dependence on temperature, density, and chemical composition. - A comparison between predicted and observed mass loss rates and terminal velocities for OB stars. - The applicability of the standard radiation driven wind models to Wolf-Rayet stars. - Speculations on how Wolf-Rayet stars achieve their enormous mass loss rates within the context of the radiation pressure mechanism. (Auth.)

  7. Classification of Kantowski-Sachs and Bianchi Type III Space-Times According to Their Killing Vector Fields in Teleparallel Theory of Gravitation

    International Nuclear Information System (INIS)

    Shabbir, Ghulam; Khan, Suhail

    2010-01-01

    In this paper we classify Kantowski-Sachs and Bianchi type III space-times according to their teleparallel Killing vector fields using direct integration technique. It turns out that the dimension of the teleparallel Killing vector fields are 4 or 6, which are the same in numbers as in general relativity. In case of 4 the teleparallel Killing vector fields are multiple of the corresponding Killing vector fields in general relativity by some function of t. In the case of 6 Killing vector fields the metric functions become constants and the Killing vector fields in this case are exactly the same as in general relativity. Here we also discuss the Lie algebra in each case. (general)

  8. Path Tortuosity and the Permeability of Roads and Trails to Wolf Movement

    Directory of Open Access Journals (Sweden)

    Jesse Whittington

    2004-06-01

    Full Text Available Few studies have examined the effects of human development on fine-scale movement behavior, yet understanding animal movement through increasingly human-dominated landscapes is essential for the persistence of many wild populations, especially wary species. In mountainous areas, roads and trails may be particularly deserving of study because they are concentrated in the valley bottoms where they can impede animal movement both across and between valleys. In this study, we tracked wolf (Canis lupus movement in the snow for two winters in Jasper National Park, Alberta, Canada to examine how wolves navigate through or around human-use features. We quantified the effects of human development and topography on the tortuosity of wolf paths and then tested the permeability of roads, trails, and a railway line to wolf movement by comparing the frequency with which actual wolf paths and a null model of random paths crossed these features. Wolf path tortuosity increased near high-use trails, within areas of high-trail and road density, near predation sites, and in rugged terrain. Wolves crossed all roads, trails, and the railway line 9.7% less often than expected, but avoided crossing high-use roads more than low-use trails. Surprisingly, trails affected movement behavior of wolves equally, if not more, than roads. These results suggest that although roads and trails in this study were not absolute barriers to wolf movement, they altered wolf movements across their territories.

  9. Molecular characterization of a novel HEXA mutation at the +3 position of intron 8 in a Tay-Sachs disease patient

    Energy Technology Data Exchange (ETDEWEB)

    Richard, M.; Triggs-Raine, B. [Univ. of Manitoba, Winnipeg (Canada); Natowicz, M. [E.K. Shriver Center, Waltham, MA (United States)

    1994-09-01

    Tay-Sachs disease is an autosomal recessive lysosomal storage disorder resulting from mutations in the HEXA gene that cause a deficiency in the activity of that enzyme {beta}-hexosaminidase A (Hex A). This deficiency leads to the build-up of G{sub M2} ganglioside, resulting in neurodegeneration and death. Biochemical analysis of a non-Jewish patient with a late-infantile form of Tay-Sachs disease revealed a substantial level of Hex A activity (38.4%) when 4-MUG was used as the substrate. However, when a substrate (4-MUGS) specific for the {alpha}-subunit of Hex A ({alpha}{beta}) was used, almost no activity was detected in the HEXA gene of the patient using SSCP analysis followed by sequencing. The first mutation, a G533A substitution in exon 5, is previously described and associated with the B1 form of Tay-Sachs disease. The second mutation is a novel a-to-g base change at the +3 position of intron 8. This was confirmed using the AIRS method, whereby a MaeIII site was created in the presence of the mutation. Normal and patient mRNA was reverse transcribed and exons 7 to 9 were PCR-amplified from the cDNA. An abnormally sized amplification product detected only in the patient cDNA was sequenced; exon 8 had been deleted and exons 7 and 9 were spliced together. A substantial level of normally-sized PCR product was also detected in the patient`s cDNA. Experiments are in progress to determine if this is produced from the allele harboring the G533A mutation. Given that previous mutations of this type have been associated with 97-100% abnormal splicing, this mutation is likely to be the cause, together with the G533A mutation, of Tay-Sachs disease in this patient.

  10. Links among impossible differential, integral and zero correlation linear cryptanalysis

    DEFF Research Database (Denmark)

    Sun, Bing; Liu, Zhiqiang; Rijmen, Vincent

    2015-01-01

    is to fix this gap and establish links between impossible differential cryptanalysis and integral cryptanalysis. Firstly, by introducing the concept of structure and dual structure, we prove that a → b is an impossible differential of a structure E if and only if it is a zero correlation linear hull...... linear hull always indicates the existence of an integral distinguisher. With this observation we improve the number of rounds of integral distinguishers of Feistel structures, CAST-256, SMS4 and Camellia. Finally, we conclude that an r-round impossible differential of E always leads to an r...

  11. Wolf--Rayet stars of M33

    International Nuclear Information System (INIS)

    Corso, G.J. Jr.

    1975-01-01

    A comprehensive study of the 54 known Wolf--Rayet stars of M33 is undertaken with the intention of improving our knowledge of the Wolf--Rayet phenomenon, identifying significant features of their distribution in an Sc galaxy, and discerning possible trends in the variation of chemical composition of the interstellar medium from place to place within that galaxy. Thirty-seven of these stars were classified for the first time into carbon and nitrogen sequences on the basis of photographic photometry of image tube plates obtained with the Kitt Peak 84-inch telescope and an ITT magnetically focused image tube equipped with a set of narrow-band interference filters designed to isolate the broad emission features between 4640 A and 4686 A due to N III, C III-IV, and He II. The subclasses WC6-9, missing in the Large Magellanic Cloud, were found in M33, although there is a tendency for the known stars of both sequences to belong to the high-excitation subclasses. The distribution of these stars was compared with the distributions of luminous blue stars, dust, and H II regions. Star counts on the image tube plates indicated that one out of every 75 stars in M33 brighter than M/sub B/ = --4.5 is a Wolf--Rayet star

  12. Integration of differential equations by the pseudo-linear (PL) approximation

    International Nuclear Information System (INIS)

    Bonalumi, Riccardo A.

    1998-01-01

    A new method of integrating differential equations was originated with the technique of approximately calculating the integrals called the pseudo-linear (PL) procedure: this method is A-stable. This article contains the following examples: 1st order ordinary differential equations (ODEs), 2nd order linear ODEs, stiff system of ODEs (neutron kinetics), one-dimensional parabolic (diffusion) partial differential equations. In this latter case, this PL method coincides with the Crank-Nicholson method

  13. Mechanism of interrupted saccades in patients with late-onset Tay-Sachs disease.

    Science.gov (United States)

    Optican, Lance M; Rucker, Janet C; Keller, Edward L; Leigh, R John

    2008-01-01

    In late-onset Tay-Sachs disease (LOTS), saccades are interrupted by one or more transient decelerations. Some saccades reaccelerate and continue on before eye velocity reaches zero, even in darkness. Intervals between successive decelerations are not regularly spaced. Peak decelerations of horizontal and vertical components of oblique saccades in LOTS is more synchronous than those in control subjects. We hypothesize that these decelerations are caused by dysregulation of the fastigial nuclei (FN) of the cerebellum, which fire brain stem inhibitory burst neurons (IBNs).

  14. Fort Peck-Wolf Point transmission line project, Montana

    International Nuclear Information System (INIS)

    1992-01-01

    The primary objective of the project is to replace the existing 36-mile Fort Peck-Wolf Point transmission line which has reached the end of its useful service life. Presently, the overall condition of this existing section of the 47-year-old line is poor. Frequent repairs have been required because of the absence of overhead ground wires. The continued maintenance of the line will become more expensive and customer interruptions will persist because of the damage due to lightning. The expense of replacing shell rotted poles, and the concern for the safety of the maintenance personnel because of hazards caused by severe shell rot are also of primary importance. The operational and maintenance problems coupled with power system simulation studies, demonstrate the need for improvements to the Wolf Point area to serve area loads. Western's Wolf Point Substation is an important point of interconnection for the power output from the Fort Peck Dam to area loads as far away as Williston, North Dakota. The proposed transmission line replacement would assure that there will continue to be reliable transmission capacity available to serve area electrical loads, as well as provide a reliable second high-voltage transmission path from the Fort Peck generation to back-up a loss of the Fort Peck-Wolf Point 115-kV Line No. 1

  15. Tay-Sachs disease: current perspectives from Australia.

    Science.gov (United States)

    Lew, Raelia M; Burnett, Leslie; Proos, Anné L; Delatycki, Martin B

    2015-01-01

    Tay-Sachs disease (TSD) is a fatal, recessively inherited neurodegenerative condition of infancy and early childhood. Although rare in most other populations, the carrier frequency is one in 25 in Ashkenazi Jews. Australian high-school-based TSD preconception genetic screening programs aim to screen, educate, and optimize reproductive choice for participants. These programs have demonstrated high uptake, low psychological morbidity, and have been shown to result in fewer than expected Jewish TSD-affected births over 18 years of operation. The majority of Jewish individuals of reproductive age outside of the high school screening program setting in Australia have not accessed screening. Recent recommendations advocate supplementing the community high school screening programs with general practitioner- and obstetrician-led genetic screening of Ashkenazi Jewish individuals for TSD and other severe recessive diseases for which this group is at risk. Massively parallel DNA sequencing is expected to become the testing modality of choice over the coming years.

  16. Integrability and Linear Stability of Nonlinear Waves

    Science.gov (United States)

    Degasperis, Antonio; Lombardo, Sara; Sommacal, Matteo

    2018-03-01

    It is well known that the linear stability of solutions of 1+1 partial differential equations which are integrable can be very efficiently investigated by means of spectral methods. We present here a direct construction of the eigenmodes of the linearized equation which makes use only of the associated Lax pair with no reference to spectral data and boundary conditions. This local construction is given in the general N× N matrix scheme so as to be applicable to a large class of integrable equations, including the multicomponent nonlinear Schrödinger system and the multiwave resonant interaction system. The analytical and numerical computations involved in this general approach are detailed as an example for N=3 for the particular system of two coupled nonlinear Schrödinger equations in the defocusing, focusing and mixed regimes. The instabilities of the continuous wave solutions are fully discussed in the entire parameter space of their amplitudes and wave numbers. By defining and computing the spectrum in the complex plane of the spectral variable, the eigenfrequencies are explicitly expressed. According to their topological properties, the complete classification of these spectra in the parameter space is presented and graphically displayed. The continuous wave solutions are linearly unstable for a generic choice of the coupling constants.

  17. Time-dependent automorphism-inducing diffeomorphisms, open algebras and the generality of the Kantowski-Sachs vacuum geometry

    Science.gov (United States)

    Christodoulakis, T.; Papadopoulos, G. O.

    2002-10-01

    Following the spirit of a previous work of ours, we investigate the group of those general coordinate transformations (GCTs) which preserve manifest spatial homogeneity. In contrast to the case of Bianchi type models, here we permit an isometry group of motions G4 = SO(3) ⊗ Tr, where Tr is the translations group, along the radial direction, while SO(3) acts multiply transitively on each hypersurface of simultaneity Σt. The basis 1-forms cannot be invariant under the action of the entire isometry group and hence produce an open Lie algebra. In order for these GCTs to exist and have a nontrivial, well-defined action, certain integrability conditions have to be satisfied; their solutions, exhibiting the maximum expected 'gauge' freedom, can be used to simplify the generic, spatially homogeneous, line element. In this way an alternative proof of the generality of the Kantowski-Sachs (KS) vacuum is given, while its most general, manifestly homogeneous, form is explicitly presented.

  18. Hill-Sachs lesion location: does it play a role in engagement?

    Energy Technology Data Exchange (ETDEWEB)

    Gyftopoulos, Soterios; Wang, Annie; Babb, James [NYU Langone Medical Center, Department of Radiology, New York, NY (United States)

    2015-08-15

    To see if there is an association between engagement on physical examination (PE) and the location of the Hill-Sachs lesion (HSL) as assessed by the modified biceps angle. Sixty-two patients with a history of anterior shoulder dislocation, who underwent preoperative MRI and arthroscopy at our institution and were tested for engagement on PE, were collected. Two musculoskeletal radiologists reviewed the MR studies, noting the presence of an HSL and documenting the location of the HSL with the modified biceps angle. Statistical analysis included the Mann-Whitney (MW) test and ROC (receiver-operating characteristic) curve. Of 62 patients, there were 58 males and 4 females with a mean age of 30 (range 18-59 years). Twenty patients demonstrated engagement on PE, while 42 did not. All patients had evidence of an HSL on MRI and arthroscopy. The mean biceps angle for the engaging group was 151.5 ± 13.9 , and 142.4 ± 17.3 for the non-engaging group. The biceps angle was statistically significantly higher among patients who had engagement compared to those who did not (p = 0.027). Overall, diagnostic accuracy was highest for a biceps angle >149 , which resulted in a sensitivity of 70 % and specificity of 67 %. The modified biceps angle, as measured on MRI, was significantly higher in patients who demonstrated engagement on physical examination than in those who did not. This supports the theory that the location of the Hill-Sachs lesion may play a role in engagement and may be its most important characteristic when determining its significance. (orig.)

  19. Gray Wolf (Canis lupus) dyad monthly association rates by demographic group.

    Science.gov (United States)

    Barber-Meyer, Shannon M.; Mech, L. David

    2015-01-01

    Preliminary data from GPS-collared wolves (Canis lupus) in the Superior National Forest of northeastern Minnesota indicated wolves had low association rates with packmates during summer. However, aerial-telemetry locations of very high frequency (VHF)-radioed wolves in this same area showed high associations among packmates during winter. We analyzed aerial-telemetry-location data from VHF-collared wolves in several packs (n=18 dyads) in this same area from 1994-2012 by month, and found lowest association rates occurred during June. While other studies have found low association among wolf packmates during summer, information on differences in association patterns depending on the wolf associates’ demographics is sparse. During May-July, association rates were greatest for breeding pairs, followed by sibling dyads, and lowest for parent– offspring dyads. Our findings improve our understanding of how individual wolf relationships affect monthly association rates. We highlight some important remaining questions regarding wolf packmate associations.

  20. Polarization variablity among Wolf-Rayet stars. IV. A complete lack of circular polarization in the optical continuum

    International Nuclear Information System (INIS)

    Robert, C.; Moffat, A.F.J.

    1989-01-01

    Quasi-simultaneous blue and red, broadband optical monitoring in linear and circular polarization and in intensity has been carried out over an interval of three weeks for several Wolf-Rayet stars that show relatively large Delta P variations in linear polarization. No significant varying Delta V component of circular polarization is detected in any of these stars. The lower upper limit Delta V/Delta P implies that the intrinsic linearly polarized light which does vary cannot be produced by electrons gyrating in a magnetic field, unless they are ultrarelativistic - a rather unlikely situation. The low mean circular polarization typically observed is probably interstellar in origin. Lack of periodicity in the observed variations of linear polarization implies that even weak magnetic field loops are unlikely to be involved in confining pockets of wind plasma. The observed linear polarization variations are related mainly to electron scattering. 25 refs

  1. Numerical method for solving linear Fredholm fuzzy integral equations of the second kind

    Energy Technology Data Exchange (ETDEWEB)

    Abbasbandy, S. [Department of Mathematics, Imam Khomeini International University, P.O. Box 288, Ghazvin 34194 (Iran, Islamic Republic of)]. E-mail: saeid@abbasbandy.com; Babolian, E. [Faculty of Mathematical Sciences and Computer Engineering, Teacher Training University, Tehran 15618 (Iran, Islamic Republic of); Alavi, M. [Department of Mathematics, Arak Branch, Islamic Azad University, Arak 38135 (Iran, Islamic Republic of)

    2007-01-15

    In this paper we use parametric form of fuzzy number and convert a linear fuzzy Fredholm integral equation to two linear system of integral equation of the second kind in crisp case. We can use one of the numerical method such as Nystrom and find the approximation solution of the system and hence obtain an approximation for fuzzy solution of the linear fuzzy Fredholm integral equations of the second kind. The proposed method is illustrated by solving some numerical examples.

  2. HD 193793, a radio-emitting Wolf-Rayet binary star

    International Nuclear Information System (INIS)

    Florkowski, D.R.; Gottesman, S.T.

    1977-01-01

    The Wolf-Rayet binary HD 193793 has been observed as a weak, unresolved radio source. The observed flux densities do not agree with the predictions of the constant-mass-flow model of Wright and Barlow and Panagia and Felli. A variable-mass-flow model is suggested and an observational test is proposed. A comparison with γ 2 Vel is made, and the parameters affecting radio emission from Wolf-Rayet stars are briefly discussed. (author)

  3. Wolf restoration to the Adirondacks: the advantages and disadvantages of public participation in the decision

    Science.gov (United States)

    Mech, L. David; Sharpe, V.A.; Norton, B.; Donnelley, S.

    2000-01-01

    The first time I ever saw a wolf in New York State's Adirondack Mountains was in 1956. It was a brush wolf, or coyote (Canis latrans), not a real wolf, but to an eager young wildlife student this distinction meant little. The presence of this large deer-killing canid let my fresh imagination view the Adirondacks as a real northern wilderness. Since then I have spent the last 40 years studying the real wolf: the gray wolf (Canis lupus). Although inhabiting nearby Quebec and Ontario, the gray wolf still has not made its way back to the Adirondacks as it has to Wisconsin, Michigan, and Montana. Those three states had the critical advantages of a nearby reservoir population of wolves and wilderness corridors through which dispersers from the reservoirs could immigrate. The Adirondacks, on the other hand, are geographically more similar to the greater Yellowstone area in that they are separated from any wolf reservoir by long distances and intensively human-developed areas aversive to wolves from the reservoir populations. If wolves are to return to the Adirondacks, they almost certainly will have to be reintroduced, as they were to Yellowstone National Park. Wolf reintroduction, as distinct from natural recovery, is an especially contentious issue, for it entails dramatic, deliberate action that must be open to public scrutiny, thorough discussion and review, and highly polarized debate. This is as it should be because once a wolf population is reintroduced to an area, it must be managed forever. There is no turning back. The wolf was once eradicated not just from the Adirondacks but from almost all of the 48 contiguous states. That feat was accomplished by a primarily pioneering society that applied itself endlessly to the task, armed with poison. We can never return to those days, so once the wolf is reintroduced successfully, it will almost certainly be here to stay.

  4. Anesthetic considerations for a pediatric patient with Wolf-Hirschhorn syndrome: a case report

    OpenAIRE

    Tsukamoto, Masanori; Yamanaka, Hitoshi; Yokoyama, Takeshi

    2017-01-01

    Wolf-Hirschhorn syndrome is a rare hereditary disease that results from a 4p chromosome deletion. Patients with this syndrome are characterized by craniofacial dysgenesis, seizures, growth delay, intellectual disability, and congenital heart disease. Although several cases have been reported, very little information is available on anesthetic management for patients with Wolf-Hirschhorn syndrome. We encountered a case requiring anesthetic management for a 2-year-old girl with Wolf-Hirschhorn ...

  5. Long-distance dispersal connects Dinaric-Balkan and Alpine grey wolf (Canis lupus) populations

    OpenAIRE

    Ražen, Nina; Kljun, Franc; Kos, Ivan; Krofel, Miha; Luštrik, Roman; Majić Skrbinšek, Aleksandra; Potočnik, Hubert

    2017-01-01

    In the last two centuries, persecution and deforestation caused grey wolf Canis lupus populations in Europe to decline. Recently, their numbers started to recover although most populations still remain isolated from one another. This study presents the first documented evidence of the successful reconnection of the Dinaric-Balkan and the Alpine wolf populations via long distance dispersal and subsequent reproduction. A young male wolf radiocollared in the Dinaric Mountains in July...

  6. System integration of CDC attenuation in the new Opel Astra; Systemintegration der CDC-Daempfung beim neuen Opel Astra

    Energy Technology Data Exchange (ETDEWEB)

    Balandat, W.; Kutsche, T. [ZF Sachs AG, Schweinfurt (Germany)

    2004-08-01

    The optional carriage system IDS Plus of the new Opel Astra was developed in close cooperation between opel, ZF Sachs and other suppliers. This networking approach resulted in a high degree of system integration with the electronic attenuation control system CDC as key element. (orig.) [German] Das optionale Fahrwerksystem IDS Plus im neuen Opel Astra entstand in enger Kooperation zwischen Opel, ZF Sachs und weiteren Zulieferern. Die Arbeit im Netzwerk fuehrte zu einer hohen Systemintegration, in deren Kern die elektronische Daempferregelung CDC steht. (orig.)

  7. The WO Wolf-Rayet stars

    International Nuclear Information System (INIS)

    Barlow, M.J.

    1982-01-01

    Sanduleak (1971) has listed five stars, not apparently associated with planetary nebulae, which show very strong O VI 3811.34 A emission. He pointed out that two of them are in the Magellanic Clouds and have absolute magnitudes comparable to those of classical (Population I) Wolf-Rayet stars. O VI emission is known to occur in some classical Wolf-Rayet stars, but not with the extreme strength shown by the Sanduleak stars. The authors have obtained absolute optical spectrophotometry (3100 - 7400 A) of all five of these stars, using the UCL Image Photon Counting System and RGO Spectrograph on the Anglo-Australian Telescope. Their relative flux distributions are shown. Inspection shows that Sand 1 is very lightly reddened, Sand 2 and 3 have intermediate reddening, and Sand 4 and 5 are heavily reddened. IUE ultraviolet spectrophotometry has been obtained of the first three stars; Sand 4 and 5 are too heavily reddened for IUE spectra to be feasible. (Auth.)

  8. Integrating immunomarking with ecological and behavioural approaches to assess predation of Helicoverpa spp. larvae by wolf spiders in cotton

    Science.gov (United States)

    Wolf spiders (Araneae: Lycosidae) are abundant soil-dwelling predators found in cotton fields and can contribute important pest management services. These spiders can kill and consume larvae of the cotton bollworm Helicoverpa spp. (Lepidoptera: Noctuidae) that survive foraging on Bt cotton and desce...

  9. Genetics Home Reference: Wolf-Hirschhorn syndrome

    Science.gov (United States)

    ... ajmg.c.30187. Citation on PubMed Bergemann AD, Cole F, Hirschhorn K. The etiology of Wolf-Hirschhorn ... medicine? What is newborn screening? New Pages Lyme disease Fibromyalgia White-Sutton syndrome All New & Updated Pages ...

  10. Serial MR imaging and 1H-MR spectroscopy in monozygotic twins with Tay-Sachs disease.

    Science.gov (United States)

    Imamura, A; Miyajima, H; Ito, R; Orii, K O

    2008-10-01

    Four-year-old monozygotic female twins with early onset Tay-Sachs disease are described. The sisters showed similar slowly progressive clinical symptoms and deterioration, however the younger sister also demonstrated intractable myoclonus in the right leg. The serial MR images and (1)H-MR spectroscopy of the brain were obtained in both twins. MR images showed high intensity on T (2)-weighted image in the bilateral white matter, however there were no signal changes in the basal ganglia and thalamus during any of the phases. The ratio of N-acetylaspartate (NAA)/creatine (Cr) was decreased in the both white matter lesions and the corpus striatum, and that of myoinositol (mI)/Cr was increased in the damaged white matter on MR spectroscopy. The elevation of the lactate peak was clearly demonstrated in the left basal ganglia of the younger sister; however it was not shown in cerebral lesions of the elder sister. Changes in metabolites on MR spectroscopy were closely linked to the respective clinical features of each twin. Follow-up examination by (1)H-MR spectroscopy is useful for the evaluation of neuronal changes in children with Tay-Sachs disease.

  11. Hardware Tailored Linear Algebra for Implicit Integrators in Embedded NMPC

    DEFF Research Database (Denmark)

    Frison, Gianluca; Quirynen, Rien; Zanelli, Andrea

    2017-01-01

    . In the case of stiff or implicitly defined dynamics, implicit integration schemes are typically preferred. This paper proposes a tailored implementation of the necessary linear algebra routines (LU factorization and triangular solutions), in order to allow for a considerable computational speedup...... of such integrators. In particular, the open-source BLASFEO framework is presented as a library of efficient linear algebra routines for small to medium-scale embedded optimization applications. Its performance is illustrated on the nonlinear optimal control example of a chain of masses. The proposed library allows...

  12. Improved dark energy detection through the polarization-assisted cross correlation of the cosmic microwave background with radio sources

    International Nuclear Information System (INIS)

    Liu, Guo-Chin; Ng, Kin-Wang; Pen, Ue-Li

    2011-01-01

    Integrated Sachs-Wolfe (ISW) effect can be estimated by cross-correlating the cosmic microwave background (CMB) sky with tracers of the local matter distribution. At late cosmic time, the dark energy-induced decay of gravitation potential generates a cross correlation signal on large angular scales. The dominant noise is the intrinsic CMB anisotropies from the inflationary epoch. In this paper we use CMB polarization to reduce this intrinsic noise. We cross-correlate the microwave sky observed by Wilkinson Microwave Anisotropy Probe (WMAP) with the radio source catalog compiled by NRAO VLA Sky Survey (NVSS) to study the efficiency of the noise suppression. We find that the error bars are reduced by about 4 to 14% and the statistical power in the signal is improved.

  13. Algebraic Properties of First Integrals for Scalar Linear Third-Order ODEs of Maximal Symmetry

    Directory of Open Access Journals (Sweden)

    K. S. Mahomed

    2013-01-01

    Full Text Available By use of the Lie symmetry group methods we analyze the relationship between the first integrals of the simplest linear third-order ordinary differential equations (ODEs and their point symmetries. It is well known that there are three classes of linear third-order ODEs for maximal cases of point symmetries which are 4, 5, and 7. The simplest scalar linear third-order equation has seven-point symmetries. We obtain the classifying relation between the symmetry and the first integral for the simplest equation. It is shown that the maximal Lie algebra of a first integral for the simplest equation y′′′=0 is unique and four-dimensional. Moreover, we show that the Lie algebra of the simplest linear third-order equation is generated by the symmetries of the two basic integrals. We also obtain counting theorems of the symmetry properties of the first integrals for such linear third-order ODEs. Furthermore, we provide insights into the manner in which one can generate the full Lie algebra of higher-order ODEs of maximal symmetry from two of their basic integrals.

  14. Effect of Integral Non-Linearity on Energy Calibration of ...

    African Journals Online (AJOL)

    The integral non-linearity (INL) of four spectroscopy systems, two integrated (A1 and A2) and two classical (B1 and B2) systems was determined using pulses from a random pulse generator. The effect of INL on the system's energy calibration was also determined. The effect is minimal in the classical system at high ...

  15. Social-Natural Landscape Reorganised: Swedish Forest-edge Farmers and Wolf Recovery

    Directory of Open Access Journals (Sweden)

    Sjolander-Lindqvist Annelie

    2009-01-01

    Full Text Available The politics and the underlying reasons behind the recovery of the Scandinavian wolf population are increasingly contested. According to official policy, wolves should be guaranteed a place in the Swedish natural world. However, the conflict over whether Sweden should host a wolf population sets views on biodiversity and sustainable development against the perspective that local traditions and livelihoods are threatened by the return of wolves. These diverging environmental visions can be seen as competing interests and understandings of nature and wildlife. The desire of the state and nature conservation organisations to implement measures to provide conditions fostering wolf survival are counterbalanced by local action groups and community residents struggling to maintain conditions for conserving summer pastures, opportunities for hunting with sporting dogs, and other recreational activities such as mushroom- and berry-picking. Not only are these activities considered to have high natural and cultural value, the European Union (EU has stated that small-scale farming is important for maintaining the landscape and safeguarding the survival of values associated with ′agri-environmental′ habitats. The conflict between the interest groups is essentially about the access to and use of environmental resources. Squeezed between policies safeguarding wolf populations, preventing cruelty to animals and implementing activities required by the EU agricultural programme, farmers in areas with resident wolf populations have come to take part in processes that may reinforce rural identity.

  16. Uurimist toimetavad Wolfe ja Goodwin / Katrin Mõttus

    Index Scriptorium Estoniae

    Mõttus, Katrin

    2003-01-01

    Sari filme - Rex Stout'i detektiivromaanide ekraniseeringud, kus peategelaseks eradetektiiv Nero Wolfe (Maury Chaykin) koos oma noore abilise Archie Goodwiniga (Timothy Hutton) : Ameerika Ühendriigid 2001-2002

  17. Can occupancy-abundance models be used to monitor wolf abundance?

    Directory of Open Access Journals (Sweden)

    M Cecilia Latham

    Full Text Available Estimating the abundance of wild carnivores is of foremost importance for conservation and management. However, given their elusive habits, direct observations of these animals are difficult to obtain, so abundance is more commonly estimated from sign surveys or radio-marked individuals. These methods can be costly and difficult, particularly in large areas with heavy forest cover. As an alternative, recent research has suggested that wolf abundance can be estimated from occupancy-abundance curves derived from "virtual" surveys of simulated wolf track networks. Although potentially more cost-effective, the utility of this approach hinges on its robustness to violations of its assumptions. We assessed the sensitivity of the occupancy-abundance approach to four assumptions: variation in wolf movement rates, changes in pack cohesion, presence of lone wolves, and size of survey units. Our simulations showed that occupancy rates and wolf pack abundances were biased high if track surveys were conducted when wolves made long compared to short movements, wolf packs were moving as multiple hunting units as opposed to a cohesive pack, and lone wolves were moving throughout the surveyed landscape. We also found that larger survey units (400 and 576 km2 were more robust to changes in these factors than smaller survey units (36 and 144 km2. However, occupancy rates derived from large survey units rapidly reached an asymptote at 100% occupancy, suggesting that these large units are inappropriate for areas with moderate to high wolf densities (>15 wolves/1,000 km2. Virtually-derived occupancy-abundance relationships can be a useful method for monitoring wolves and other elusive wildlife if applied within certain constraints, in particular biological knowledge of the surveyed species needs to be incorporated into the design of the occupancy surveys. Further, we suggest that the applicability of this method could be extended by directly incorporating some of its

  18. Wolf, Canis lupus, visits to white-tailed deer, Odocoileus virginianus, summer ranges: Optimal foraging?

    Science.gov (United States)

    Demma, D.J.; Mech, L.D.

    2009-01-01

    We tested whether Wolf (Canis lupus) visits to individual female White-tailed Deer (Odocoileus virginianus) summer ranges during 2003 and 2004 in northeastern Minnesota were in accord with optimal-foraging theory. Using GPS collars with 10- to 30-minute location attempts on four Wolves and five female deer, plus eleven VHF-collared female deer in the Wolves' territory, provided new insights into the frequency of Wolf visits to summer ranges of female deer. Wolves made a mean 0.055 visits/day to summer ranges of deer three years and older, significantly more than their 0.032 mean visits/day to ranges of two-year-old deer, which generally produce fewer fawns, and most Wolf visits to ranges of older deer were much longer than those to ranges of younger deer. Because fawns comprise the major part of the Wolf's summer diet, this Wolf behavior accords with optimal-foraging theory.

  19. [Dr Mavro Sachs (1817-1888): the first lecturer of Zagreb University].

    Science.gov (United States)

    Dugacki, Vladimir

    2010-01-01

    Mavro Sachs (Jánosháza, Hungary, 1817 % emdash; Rijeka, 1888.) was a Zagreb student since 1828. In 1846 he graduated in medicine from the University of Vienna and returned to Zagreb to be the city physician. In 1849, he started to teach forensic medicine at the School of Law of the Royal Academy of Sciences in Zagreb and continued teaching the same subject at the Zagreb University Faculty of Law in the capacity of docent (corresponds to lecturer in the British system). He also taught medical law at pharmaceutical studies of Zagreb University. From 1855 to 1860, he presided over the Jewish Community of Zagreb.

  20. Growth rates and variances of unexploited wolf populations in dynamic equilibria

    Science.gov (United States)

    Mech, L. David; Fieberg, John

    2015-01-01

    Several states have begun harvesting gray wolves (Canis lupus), and these states and various European countries are closely monitoring their wolf populations. To provide appropriate perspective for determining unusual or extreme fluctuations in their managed wolf populations, we analyzed natural, long-term, wolf-population-density trajectories totaling 130 years of data from 3 areas: Isle Royale National Park in Lake Superior, Michigan, USA; the east-central Superior National Forest in northeastern Minnesota, USA; and Denali National Park, Alaska, USA. Ratios between minimum and maximum annual sizes for 2 mainland populations (n = 28 and 46 yr) varied from 2.5–2.8, whereas for Isle Royale (n = 56 yr), the ratio was 6.3. The interquartile range (25th percentile, 75th percentile) for annual growth rates, Nt+1/Nt, was (0.88, 1.14), (0.92, 1.11), and (0.86, 1.12) for Denali, Superior National Forest, and Isle Royale respectively. We fit a density-independent model and a Ricker model to each time series, and in both cases we considered the potential for observation error. Mean growth rates from the density-independent model were close to 0 for all 3 populations, with 95% credible intervals including 0. We view the estimated model parameters, including those describing annual variability or process variance, as providing useful summaries of the trajectories of these populations. The estimates of these natural wolf population parameters can serve as benchmarks for comparison with those of recovering wolf populations. Because our study populations were all from circumscribed areas, fluctuations in them represent fluctuations in densities (i.e., changes in numbers are not confounded by changes in occupied area as would be the case with populations expanding their range, as are wolf populations in many states).

  1. In vivo evaluation of the hypoglycemic effect of wolf-apple flour ...

    African Journals Online (AJOL)

    The prevalence of diabetes has increased at alarming rates worldwide, and has become a serious health problem in modern society, highlighting the need for adjuvants to assist in its treatment. The starch from wolf-apple is a product extracted from the pulp of the unripe wolf-apple (Solanum lycocarpum A. St.-Hil), which ...

  2. Conditions for caribou persistence in the wolf-elk-caribou systems of the Canadian Rockies

    Directory of Open Access Journals (Sweden)

    Mark Hebblewhite

    2007-04-01

    Full Text Available Woodland caribou populations are considered threatened in Alberta and have declined in the Canadian Rocky Mountain National Parks of Banff and Jasper despite protection from factors causing caribou populations to decline outside of parks. Recent research emphasizes the importance of the numeric response of wolves to moose in moose-caribou-wolf systems to caribou persistence. Moose are rare in the Canadian Rockies, where the dominant ungulate prey for wolves is elk. Few studies have explored wolf-elk dynamics and none have examined implications for caribou. We used data collected in Banff to estimate the numeric response of wolves to elk from 1985 to 2005. Because no caribou kill-rate data exist for the Rockies, we explore the consequences of a range of hypothetical kill-rates based on kill-rates of alternate prey collected from 1985 to 2000 in Banff. We then multiplied the numeric response of wolves by the estimated caribou kill-rates to estimate the wolf predation response on caribou as a function of elk density. Caribou predation rates were inversely density dependent because wolf numbers depend on prey species besides caribou in multiple prey species systems. We then combined this simple wolf-elk-caribou model with observed demographic and population estimates for Banff and Jasper caribou from 2003-2004 and solved for the critical kill-rate thresholds above which caribou populations would decline. Using these critical kill-rate thresholds, Jasper caribou are likely to persist when wolf densities are below 2.1 - 4.3 wolves/1000km2 and/or when elk densities are below 0.015- 0.033 elk/km2. Thresholds for Banff caribou persistence are much lower because of inverse density dependence. Future research is needed on some of the necessary assumptions underlying our modeling including multi-prey wolf numeric responses, wolf kill-rates of caribou, caribou mortality by other predators, and spatial aspects of wolf-elk-caribou dynamics.

  3. Peculiarities in the distribution of galactic Wolf-Rayet stars: constraints on evolutionary scenarios

    International Nuclear Information System (INIS)

    Gomez, A.; Lortet, M.-C.; Pitault, A.

    1981-01-01

    The re-examination of the spatial distribution of 158 Wolf-Rayet stars in the Galaxy may help to define different breeds of Wolf-Rayet stars and put constraints on possible evolutionary scenarios. (Auth.)

  4. Multimedia messages in genetics: design, development, and evaluation of a computer-based instructional resource for secondary school students in a Tay Sachs disease carrier screening program.

    Science.gov (United States)

    Gason, Alexandra A; Aitken, MaryAnne; Delatycki, Martin B; Sheffield, Edith; Metcalfe, Sylvia A

    2004-01-01

    Tay Sachs disease is a recessively inherited neurodegenerative disorder, for which carrier screening programs exist worldwide. Education for those offered a screening test is essential in facilitating informed decision-making. In Melbourne, Australia, we have designed, developed, and evaluated a computer-based instructional resource for use in the Tay Sachs disease carrier screening program for secondary school students attending Jewish schools. The resource entitled "Genetics in the Community: Tay Sachs disease" was designed on a platform of educational learning theory. The development of the resource included formative evaluation using qualitative data analysis supported by descriptive quantitative data. The final resource was evaluated within the screening program and compared with the standard oral presentation using a questionnaire. Knowledge outcomes were measured both before and after either of the educational formats. Data from the formative evaluation were used to refine the content and functionality of the final resource. The questionnaire evaluation of 302 students over two years showed the multimedia resource to be equally effective as an oral educational presentation in facilitating participants' knowledge construction. The resource offers a large number of potential benefits, which are not limited to the Tay Sachs disease carrier screening program setting, such as delivery of a consistent educational message, short delivery time, and minimum financial and resource commitment. This article outlines the value of considering educational theory and describes the process of multimedia development providing a framework that may be of value when designing genetics multimedia resources in general.

  5. Regional neuro axonal injury detected by 1H 3 Tesla spectroscopic imaging in late onset Tay sachs

    International Nuclear Information System (INIS)

    Gagoski, Borjan Aleksandar; Eichler, Florian S.

    2010-01-01

    Late-onset Tay Sachs (LOTS) is a rare lysosomal storage disorder resulting from mutations of the subunit of the lysosomal enzyme β-hexosaminidase A, which catalyzes the degradation of GM2 ganglioside. We have applied the fast encoding spectroscopic imaging technique to LOTS patients to further investigate the neuro degenerative consequences of this disease.(Author)

  6. The Woman as Wolf (AT 409: Some Interpretations of a Very Estonian Folk Tale

    Directory of Open Access Journals (Sweden)

    Merili Metsvahi

    2013-12-01

    Full Text Available The article analyses tale type The Woman as Wolf, which is one of the most popular folk tales in the Estonian Folklore Archives and is represented there both in the form of a fairy tale and in the form of a legend. The vast majority of the versions of The Woman as Wolf were written down in the first part of the 20th century within Estonia and where recorded from Estonians. The article introduces the content of the tale, the origin of the first records from the early 19th century, and the dissemination area of the tale, which remains outside Western Europe: apart from the Estonian versions there are Sami, Karelian, Vepsian, Livonian, Latvian, Lithuanian, Russian, Ukrainian and Belarusian versions. While in almost all the Estonian versions the main protagonist is transformed into a wolf, in most of the versions written down in other areas and ethnic groups, another animal or bird replaces the wolf. The author is of the opinion that the Finnic area is central to the distribution of the folk tale The Woman as Wolf. The animal the woman is transformed into in the plot would not have been a wolf in earlier times. The article provides an explanation why the wolf is predominant in Estonian written sources. For that purpose the ways in which the wolf and werewolf were perceived in earlier Estonian folk belief are introduced. At the end of the article interpretation of the folk tale is provided. The author states that the plot and some of the motifs found in this folk tale reflect the difficulties women had in submitting to the norms and values of patriarchal order within their society.

  7. Intensive photometry of southern Wolf-Rayet stars

    International Nuclear Information System (INIS)

    Balona, L.A.; Egan, J.; Marang, F.

    1989-01-01

    Results are presented of an intensive photometric campaign on 17 of the brightest southern Wolf-Rayet stars. We report the detection of multi-periodicity in two stars: HD 50896 and HD 96548. It is likely that these periodicities are not coherent but are manifestations of the quasi-periodic variations seen in a few WR stars. A good example of these variations is given by HD 86161. A new eclipsing binary, HD 92740 has been discovered; other stars show periodic variations which can be explained by phase-dependent scattering of the secondary light as it traverses the Wolf-Rayet wind. An important conclusion of this study is that not a single example was found of short-period variations which can be attributed to pulsation. (author)

  8. The Wolf 630 moving group of stars

    International Nuclear Information System (INIS)

    McDonald, A.R.E.; Hearnshaw, J.B.; Canterbury Univ., Christchurch

    1983-01-01

    An analysis is made of the probability of collective membership of the stars assigned by Eggen to the Wolf 630 moving group. This probability is estimated from the scatter of points in the colour-absolute magnitude diagram when compared to the intrinsic scatter observed for M67. Particular attention is paid to the random errors for all the observed and deduced stellar parameters. Results show that either the observational errors must be about 2.4 times larger than given in the proper motion and radial velocity source catalogues, or the intrinsic scatter in the colour-magnitude diagram for the Wolf 630 group must be much larger than for M67, or many of the stars considered cannot be members. (author)

  9. Evaluating the Effectiveness of Natura 2000 Network for Wolf Conservation: A Case-Study in Greece

    Science.gov (United States)

    Votsi, Nefta-Eleftheria P.; Zomeni, Maria S.; Pantis, J. D.

    2016-02-01

    The wolf ( Canis lupus) is used as a case study to rate Natura 2000 sites in Greece based on preferred wolf habitat characteristics and test whether the network is suitable for their conservation. Road density, agricultural area, site area, connectivity, food availability (i.e., presence of natural prey), and elevation in 237 sites are combined in a logistic regression model. The occurrence of the wolf's natural prey was the most prevalent factor determining wolf presence, followed by agricultural cover. Considering the current status of these features at N2K site level, most sites currently hosting wolves (85.7 %) have good or excellent prospects for the long-term presence of the wolf. On the contrary, 11 sites which now have wolves are predicted to be ineffective in keeping them in the future due to the absence of wild ungulates and their high agricultural coverage. Four sites with no wolf presence currently have excellent prospects to host wolves in the future. Roadless sites are a priority for protection and retaining their current condition is strongly suggested. The proposed approach aims to detect gaps in protection for the wolf and identify priority sites in need of mitigation actions. It can also assist the assessment of conservation policies in Greece and elsewhere toward accomplishing set goals in protected areas. By focusing on wolf protection, we hope to increase agencies' attention to deal with conservation effectiveness, especially in cases like Greece, where a number of sites are insufficiently known and protected and management measures are not properly implemented.

  10. A Differential Monolithically Integrated Inductive Linear Displacement Measurement Microsystem

    Directory of Open Access Journals (Sweden)

    Matija Podhraški

    2016-03-01

    Full Text Available An inductive linear displacement measurement microsystem realized as a monolithic Application-Specific Integrated Circuit (ASIC is presented. The system comprises integrated microtransformers as sensing elements, and analog front-end electronics for signal processing and demodulation, both jointly fabricated in a conventional commercially available four-metal 350-nm CMOS process. The key novelty of the presented system is its full integration, straightforward fabrication, and ease of application, requiring no external light or magnetic field source. Such systems therefore have the possibility of substituting certain conventional position encoder types. The microtransformers are excited by an AC signal in MHz range. The displacement information is modulated into the AC signal by a metal grating scale placed over the microsystem, employing a differential measurement principle. Homodyne mixing is used for the demodulation of the scale displacement information, returned by the ASIC as a DC signal in two quadrature channels allowing the determination of linear position of the target scale. The microsystem design, simulations, and characterization are presented. Various system operating conditions such as frequency, phase, target scale material and distance have been experimentally evaluated. The best results have been achieved at 4 MHz, demonstrating a linear resolution of 20 µm with steel and copper scale, having respective sensitivities of 0.71 V/mm and 0.99 V/mm.

  11. Are Wolf-Rayet Stars Able to Pollute the Interstellar Medium of Galaxies? Results from Integral Field Spectroscopy

    Directory of Open Access Journals (Sweden)

    Enrique Pérez-Montero

    2013-01-01

    Full Text Available We investigate the spatial distribution of chemical abundances in a sample of low metallicity Wolf-Rayet (WR galaxies selected from the SDSS. We used the integral field spectroscopy technique in the optical spectral range (3700 Å–6850 Å with PMAS attached to the CAHA 3.5 m telescope. Our statistical analysis of the spatial distributions of O/H and N/O, as derived using the direct method or strong-line parameters consistent with it, indicates that metallicity is homogeneous in five out of the six analysed objects in scales of the order of several kpc. Only in the object WR404 is a gradient of metallicity found in the direction of the low surface brightness tail. In contrast, we found an overabundance of N/O in spatial scales of the order of hundreds of pc associated with or close to the positions of the WR stars in 4 out of the 6 galaxies. We exclude possible hydrodynamical causes, such as the metal-poor gas inflow, for this local pollution by means of the analysis of the mass-metallicity relation (MZR and mass-nitrogen-to-oxygen relation (MNOR for the WR galaxies catalogued in the SDSS.

  12. A Teacher is Forever: The Legacy of Harry Kirke Wolfe (1858-1918).

    Science.gov (United States)

    Benjamin, Ludy T. Jr.

    1987-01-01

    This article traces the career of Harry Kirke Wolfe, Nebraska educator and one of the earliest U.S. psychologists to earn a doctorate in psychology from Wilhelm Wundt at Leipzig. Emphasis is placed on Wolfe's blending of psychology and pedagogy, and his qualities as a teacher. (Author/JDH)

  13. SS433: the second Wolf-Rayet X-ray binary ?

    OpenAIRE

    Fuchs, Yael; Koch-Miramond, Lydie; Abraham, Peter

    2002-01-01

    We present mid-infrared spectrophotometric observations of SS433 with ISOPHOT. The HeI+HeII lines in both spectra of SS433 and of the Wolf-Rayet star WR147, a wind-colliding WN8+BO5 binary system, closely match. The 2.5-12 micron continuum radiation is due to an expanding wind free-free emission in an intermediate case between optically thick and optically thin regimes. The inferred mass loss rate evaluation gives ~10^{-4} Msun/yr. Our results are consistent with a Wolf-Rayet-like companion t...

  14. Osteometrical and CT examination of the Japanese wolf [Canis hodophilax] skull

    International Nuclear Information System (INIS)

    Endo, H.; Obara, I.; Yoshida, T.; Kurohmaru, M.; Hayshi, Y.; Suzuki, N.

    1997-01-01

    The skulls of Japanese wolf (Canis hodophilax) were osteometrically examined and compared with those of Akita-Inu. The skull total length was not statistically different between two species. However, significant differences were demonstrated between two species in some ratios concerning the frontal bone. CT examination was carried out in the Japanese wolf skull. The data indicated that the frontal sinus is not be largely developed and compressed in the dorso-ventral direction in parasagittal area. The narrow frontal sinus fitted to external shape of the frontal bone. The cribriform plate had a well-developed complicated structure in a caudal part of the ethmoid bone. These data will be useful to examine the respiratory function and the olfactory sense in the Japanese wolf

  15. Yellowstone wolf (Canis lupus) denisty predicted by elk (Cervus elaphus) biomass

    Science.gov (United States)

    Mech, L. David; Barber-Meyer, Shannon

    2015-01-01

    The Northern Range (NR) of Yellowstone National Park (YNP) hosts a higher prey biomass density in the form of elk (Cervus elaphus L., 1758) than any other system of gray wolves (Canis lupus L., 1758) and prey reported. Therefore, it is important to determine whether that wolf–prey system fits a long-standing model relating wolf density to prey biomass. Using data from 2005 to 2012 after elk population fluctuations dampened 10 years subsequent to wolf reintroduction, we found that NR prey biomass predicted wolf density. This finding and the trajectory of the regression extend the validity of the model to prey densities 19% higher than previous data and suggest that the model would apply to wolf–prey systems of even higher prey biomass.

  16. Assessment of attachment behaviour to human caregivers in wolf pups (Canis lupus lupus).

    Science.gov (United States)

    Hall, Nathaniel J; Lord, Kathryn; Arnold, Anne-Marie K; Wynne, Clive D L; Udell, Monique A R

    2015-01-01

    Previous research suggested that 16-week old dog pups, but not wolf pups, show attachment behaviour to a human caregiver. Attachment to a caregiver in dog pups has been demonstrated by differential responding to a caregiver compared to a stranger in the Ainsworth Strange Situation Test. We show here that 3-7 week old wolf pups also show attachment-like behaviour to a human caregiver as measured by preferential proximity seeking, preferential contact, and preferential greeting to a human caregiver over a human stranger in a modified and counterbalanced version of the Ainsworth Strange Situation Test. In addition, our results show that preferential responding to a caregiver over a stranger is only apparent following brief isolation. In initial episodes, wolf pups show no differentiation between the caregiver and the stranger; however, following a 2-min separation, the pups show proximity seeking, more contact, and more greeting to the caregiver than the stranger. These results suggest intensive human socialization of a wolf can lead to attachment--like responding to a human caregiver during the first two months of a wolf pup's life. Published by Elsevier B.V.

  17. The gravitational wave contribution to cosmic microwave background anisotropies and the amplitude of mass fluctuations from COBE results

    Science.gov (United States)

    Lucchin, Francesco; Matarrese, Sabino; Mollerach, Silvia

    1992-01-01

    A stochastic background of primordial gravitational waves may substantially contribute, via the Sachs-Wolfe effect, to the large-scale cosmic microwave background (CMB) anisotropies recently detected by COBE. This implies a bias in any resulting determination of the primordial amplitude of density fluctuations. We consider the constraints imposed on n is less than 1 ('tilted') power-law fluctuation spectra, taking into account the contribution from both scalar and tensor waves, as predicted by power-law inflation. The gravitational wave contribution to CMB anisotropies generally reduces the required rms level of mass fluctuation, thereby increasing the linear bias parameter, even in models where the spectral index is close to the Harrison-Zel'dovich value n = 1. This 'gravitational wave bias' helps to reconcile the predictions of CDM models with observations on pairwise galaxy velocity dispersion on small scales.

  18. Fluctuations from cosmic strings and the microwave background

    International Nuclear Information System (INIS)

    Brandenberger, R.H.; Turok, N.

    1986-01-01

    The spectrum of energy-density perturbations and anisotropies in the microwave background radiation are calculated in models with cosmic strings. The computations are based on a mathematical model of the network of cosmic strings as a combination of a random walk of infinite strings and a distribution of string loops. The energy-density distribution is scale invariant at Hubble radius crossing, but the k dependence of the spectrum is nontrivial and not equal to the result for adiabatic linear perturbations. The anisotropies in the microwave background radiation are smaller than the observational upper bounds on all angular scales for a value μGapprox.2 x 10 -6 obtained from independent astrophysical considerations. We include both the effects due to gravitational lensing from long strings and from local gravitational perturbations due to loops (the Sachs-Wolfe effect)

  19. The use of the J* integral for non-linear fracture mechanics

    International Nuclear Information System (INIS)

    Hellen, T.K.

    1976-09-01

    The Griffith energy balance criterion, first postulated over 50 years ago, is still the basis of linear elastic fracture mechanics. From this, accurate numerical methods for establishing stress intensity factors and energy release rates have been developed. One such method involves path independent contour integrals about the crack tip. An improved contour integral, designated J* is discussed, and shown to have distinct advantages over others in non-linear strain situations. A number of examples are shown including fractures in thermo-plastic and creep situations. (author)

  20. Music Activities for "Little Wolf's Song"

    Science.gov (United States)

    Cardany, Audrey Berger

    2015-01-01

    Drawn from Britta Techentrup's children's book "Little Wolf's Song", the author shares music activities appropriate for preschool and children in primary grades. Children will enjoy Technentrup's tender family story, while exploring vocal and instrumental timbres, as well as reading, writing, and creating with melodic contour.

  1. Tay-Sachs disease: progression of changes on neuroimaging in four cases

    International Nuclear Information System (INIS)

    Fukumizu, M.; Yoshikawa, H.; Kurokawa, T.; Takashima, S.; Sakuragawa, N.

    1992-01-01

    The neuroradiological findings in four patients with Tay-Sachs disease are described in three phases of the clinical course. The basal ganglia and cerebral white matter show low density on computed tomography and high signal intensity on T2-weighted magnetic resonance imaging in the initial phase. The caudate nuclei are characteristically enlarged and protrude into the lateral ventricles in the first and second phases. The cerebral white matter shows low density on the CT which varies in extent from the second to third phases, and the whole brain becomes atrophic in the last phase. Thus, central nervous system involvement in the disease may begin in basal ganglia as well as in cerebral white matter. (orig.)

  2. Tay-Sachs disease: progression of changes on neuroimaging in four cases

    Energy Technology Data Exchange (ETDEWEB)

    Fukumizu, M; Yoshikawa, H; Kurokawa, T [National Center Hospital for Mental, Nervous, and Muscular Disorders, Tokyo (Japan). Div. of Child Neurology; Takashima, S [National Inst. of Neuroscience, National Center of Neurology and Psychiatry, Tokyo (Japan). Div. of Mental Retardation and Birth Defect Research; Sakuragawa, N [National Inst. of Neuroscience, National Center of Neurology and Psychiatry, Tokyo (Japan). Div. of Inherited Metabolic Disease

    1992-11-01

    The neuroradiological findings in four patients with Tay-Sachs disease are described in three phases of the clinical course. The basal ganglia and cerebral white matter show low density on computed tomography and high signal intensity on T2-weighted magnetic resonance imaging in the initial phase. The caudate nuclei are characteristically enlarged and protrude into the lateral ventricles in the first and second phases. The cerebral white matter shows low density on the CT which varies in extent from the second to third phases, and the whole brain becomes atrophic in the last phase. Thus, central nervous system involvement in the disease may begin in basal ganglia as well as in cerebral white matter. (orig.).

  3. Anesthetic considerations for a pediatric patient with Wolf-Hirschhorn syndrome: a case report.

    Science.gov (United States)

    Tsukamoto, Masanori; Yamanaka, Hitoshi; Yokoyama, Takeshi

    2017-09-01

    Wolf-Hirschhorn syndrome is a rare hereditary disease that results from a 4p chromosome deletion. Patients with this syndrome are characterized by craniofacial dysgenesis, seizures, growth delay, intellectual disability, and congenital heart disease. Although several cases have been reported, very little information is available on anesthetic management for patients with Wolf-Hirschhorn syndrome. We encountered a case requiring anesthetic management for a 2-year-old girl with Wolf-Hirschhorn syndrome. The selection of an appropriately sized tracheal tube and maintaining intraoperatively stable hemodynamics might be critical problems for anesthetic management. In patients with short stature, the tracheal tube size may differ from what may be predicted based on age. The appropriate size ( internal diameter ) of tracheal tubes for children has been investigated. Congenital heart disease is frequently associated with Wolf-Hirschhorn syndrome. Depending on the degree and type of heart disease, careful monitoring of hemodynamics is important.

  4. Local linearization methods for the numerical integration of ordinary differential equations: An overview

    International Nuclear Information System (INIS)

    Jimenez, J.C.

    2009-06-01

    Local Linearization (LL) methods conform a class of one-step explicit integrators for ODEs derived from the following primary and common strategy: the vector field of the differential equation is locally (piecewise) approximated through a first-order Taylor expansion at each time step, thus obtaining successive linear equations that are explicitly integrated. Hereafter, the LL approach may include some additional strategies to improve that basic affine approximation. Theoretical and practical results have shown that the LL integrators have a number of convenient properties. These include arbitrary order of convergence, A-stability, linearization preserving, regularity under quite general conditions, preservation of the dynamics of the exact solution around hyperbolic equilibrium points and periodic orbits, integration of stiff and high-dimensional equations, low computational cost, and others. In this paper, a review of the LL methods and their properties is presented. (author)

  5. Application of digital mapping technology to the display of hydrologic information; a proof-of-concept test in the Fox-Wolf River Basin, Wisconsin

    Science.gov (United States)

    Moore, G.K.; Baten, L.G.; Allord, G.J.; Robinove, C.J.

    1983-01-01

    The Fox-Wolf River basin in east-central Wisconsin was selected to test concepts for a water-resources information system using digital mapping technology. This basin of 16,800 sq km is typical of many areas in the country. Fifty digital data sets were included in the Fox-Wolf information system. Many data sets were digitized from 1:500,000 scale maps and overlays. Some thematic data were acquired from WATSTORE and other digital data files. All data were geometrically transformed into a Lambert Conformal Conic map projection and converted to a raster format with a 1-km resolution. The result of this preliminary processing was a group of spatially registered, digital data sets in map form. Parameter evaluation, areal stratification, data merging, and data integration were used to achieve the processing objectives and to obtain analysis results for the Fox-Wolf basin. Parameter evaluation includes the visual interpretation of single data sets and digital processing to obtain new derived data sets. In the areal stratification stage, masks were used to extract from one data set all features that are within a selected area on another data set. Most processing results were obtained by data merging. Merging is the combination of two or more data sets into a composite product, in which the contribution of each original data set is apparent and can be extracted from the composite. One processing result was also obtained by data integration. Integration is the combination of two or more data sets into a single new product, from which the original data cannot be separated or calculated. (USGS)

  6. Using Spatial, Economic, and Ecological Opinion Data to Inform Gray Wolf Conservation.

    Science.gov (United States)

    Berry, Meredith S; Nickerson, Norma P; Metcalf, Elizabeth Covelli

    2016-09-01

    Public opinion can be an influential factor in wildlife management decisions. Evaluating public opinions can help legitimize, or delegitimize, management and facilitate long-term conservation goals. This is especially true for the controversial issues surrounding the management of predators. We surveyed Montana, USA, residents during summer of 2013 to measure public opinion regarding economic and ecological impacts of the gray wolf ( Canis lupus ), and current management of this species. Although opinions were polarized in some areas, a greater percentage of Montanans think that wolves negatively affect the economy, but impact tourism (which contributes to the economy) positively. These differences may reflect the belief that rancher economic losses from wolf predation of cattle is greater than overall tourism gains related to wolves (e.g., wolf-watching), in addition to the perception of wolves negatively affecting big game (e.g., elk [ Cervus canadensis ]). Results also show that a slightly greater percentage of Montanans feel that wolves positively rather than negatively affect the ecosystem. Regarding specific practices, more Montanans than not have a positive opinion of maintaining wolves on the landscape and also support hunting of wolves. More Montanans hold negative rather than positive opinions, however, regarding wolf trapping. This result was most evident in western Montana as assessed by a spatial distribution of opinions by county and has implications for current wolf management and nontarget species. Results of ordinal regression analyses revealed that big game hunters, males, and those who held negative opinions of the effect of wolves on the Montana ecosystem and economy were significantly more likely to support both hunting and trapping practices. Living in western Montana predicted positive opinions of hunting, but alternatively, negative opinions of trapping. These results provide an understanding of public opinion of wolf management by county as

  7. Using Spatial, Economic, and Ecological Opinion Data to Inform Gray Wolf Conservation

    Science.gov (United States)

    Berry, Meredith S.; Nickerson, Norma P.; Metcalf, Elizabeth Covelli

    2018-01-01

    Public opinion can be an influential factor in wildlife management decisions. Evaluating public opinions can help legitimize, or delegitimize, management and facilitate long-term conservation goals. This is especially true for the controversial issues surrounding the management of predators. We surveyed Montana, USA, residents during summer of 2013 to measure public opinion regarding economic and ecological impacts of the gray wolf (Canis lupus), and current management of this species. Although opinions were polarized in some areas, a greater percentage of Montanans think that wolves negatively affect the economy, but impact tourism (which contributes to the economy) positively. These differences may reflect the belief that rancher economic losses from wolf predation of cattle is greater than overall tourism gains related to wolves (e.g., wolf-watching), in addition to the perception of wolves negatively affecting big game (e.g., elk [Cervus canadensis]). Results also show that a slightly greater percentage of Montanans feel that wolves positively rather than negatively affect the ecosystem. Regarding specific practices, more Montanans than not have a positive opinion of maintaining wolves on the landscape and also support hunting of wolves. More Montanans hold negative rather than positive opinions, however, regarding wolf trapping. This result was most evident in western Montana as assessed by a spatial distribution of opinions by county and has implications for current wolf management and nontarget species. Results of ordinal regression analyses revealed that big game hunters, males, and those who held negative opinions of the effect of wolves on the Montana ecosystem and economy were significantly more likely to support both hunting and trapping practices. Living in western Montana predicted positive opinions of hunting, but alternatively, negative opinions of trapping. These results provide an understanding of public opinion of wolf management by county as

  8. The Work-Related Flow Inventory: Construction and Initial Validation of the WOLF

    Science.gov (United States)

    Bakker, Arnold B.

    2008-01-01

    The WOrk-reLated Flow inventory (WOLF) measures flow at work, defined as a short-term peak experience characterized by absorption, work enjoyment, and intrinsic work motivation. Results of Study 1 among 7 samples of employees (total N=1346) from different occupational groups offer support for the factorial validity and reliability of the WOLF.…

  9. Exact Kantowski-Sachs and Bianchi types I and III cosmological models with a conformally invariant scalar field

    International Nuclear Information System (INIS)

    Accioly, A.J.

    1985-01-01

    Exact solutions of the Einstein-Conformally Invariant Scalar Field Equations are obtained for Kantowski-Sachs and Bianchi types I and III cosmologies. The presence of the conformally invariant scalar field is responsible for some interesting features of the solutions. In particular it is found that the Bianchi I model is consistent with the big-bang theory of cosmology. (Author) [pt

  10. Never Cry Wolf

    DEFF Research Database (Denmark)

    Sinding, Mikkel Holger Strander

    of mostly wolves and dogs, to explore and document the origin and genomic history of indigenous Greenland dogs and wolves. During data exploration, several findings were made, including many novel results in surprising parts of the data, overall making 6 structured datasets of focus. 1) When focusing...... in any sled dogs. 6) When focusing on Greenland wolves we find that the original exterminated wolves and the wolves that have re-colonised, are the same distinct Polar wolf population endemic to Axel Heibergs Land, Ellesmere Island and Greenland. The Polar wolves have very little gene flow with other...

  11. Serial CT scans and MRI scans of Tay-Sachs disease

    International Nuclear Information System (INIS)

    Shimoizumi, Hideo; Miyao, Masutomo; Ichihashi, Koh; Sawa, Rituko; Yamamoto, Yoshifumi; Tanaka, Osamu; Yanagisawa, Masayoshi; Kamoshita, Shigehiko.

    1986-01-01

    Serial X-ray CT and MRI were performed on 2 cases of Tay-Sachs disease. Case 1 : a 3-year-10-month-old girl. Her developmental milestones were normal until the age of 6 months. At the age of 10 months, hypotonia and unduly sensitiveness to sounds were noticed. She had cherry red spots in both fundi, and the serum hexosaminidase A activity was low. Significant clinical features during the next 3 years included regression of developmental milestones to the level of one month, and myoclonic and generalised seizures. After 3 years she showed megalencephaly, unstable body temperature, and respiratory disorders. Case 2 : a 2-year-6-month-old boy. His developmental milestones were normal until the age of 5 months. Then he manifested hyperreaction to noises and further developments ceased. He started to deteriorate from the age of 1 year and 2 months, and had no head control at the age of 1 year and 6 months. Cherry red spots were detected and the serum hexsosaminidase A activity was low, too. X-ray CT at the early stage of cases 1 and 2 showed mild cerebral atrophy and high density areas in bilateral thalami and basal ganglia. At the late stage of Case 1, high density areas appeared in occipital and frontal white matters. MRI in both patients were strikingly similar. In their grey matters, linear light areas were demonstrated on IR images. Their white matters showed extensive dark (long T 1 ) areas on IR images, and extensive light (long T 2 ) areas on SE images. Bilateral thalami and basal ganglia were light on IR images and dark on SE images. The findings of MRI in our patients seemed to correlate well with pathological and biochemical changes in their brains. (author)

  12. Definition of path integrals and rules for non-linear transformations

    International Nuclear Information System (INIS)

    Kerler, W.

    1978-01-01

    Functional integrals are defined as the limit of multidimensional integrals based on fundamental generating distributions. The 'lattice choice' is put into a suitable functional form. The independence of the particular choice and the necessity of this fact are shown. Various forms of the path integrals are derived and discussed. The relation to the traditional ordering problem is pointed out. The mechanism of non-linear transformations of variables is investigated and rules are given. In the case of fields it turns out that the path integrals can also be considered for space translations. (Auth.)

  13. Competition between apex predators? Brown bears decrease wolf kill rate on two continents.

    Science.gov (United States)

    Tallian, Aimee; Ordiz, Andrés; Metz, Matthew C; Milleret, Cyril; Wikenros, Camilla; Smith, Douglas W; Stahler, Daniel R; Kindberg, Jonas; MacNulty, Daniel R; Wabakken, Petter; Swenson, Jon E; Sand, Håkan

    2017-02-08

    Trophic interactions are a fundamental topic in ecology, but we know little about how competition between apex predators affects predation, the mechanism driving top-down forcing in ecosystems. We used long-term datasets from Scandinavia (Europe) and Yellowstone National Park (North America) to evaluate how grey wolf ( Canis lupus ) kill rate was affected by a sympatric apex predator, the brown bear ( Ursus arctos ). We used kill interval (i.e. the number of days between consecutive ungulate kills) as a proxy of kill rate. Although brown bears can monopolize wolf kills, we found no support in either study system for the common assumption that they cause wolves to kill more often. On the contrary, our results showed the opposite effect. In Scandinavia, wolf packs sympatric with brown bears killed less often than allopatric packs during both spring (after bear den emergence) and summer. Similarly, the presence of bears at wolf-killed ungulates was associated with wolves killing less often during summer in Yellowstone. The consistency in results between the two systems suggests that brown bear presence actually reduces wolf kill rate. Our results suggest that the influence of predation on lower trophic levels may depend on the composition of predator communities. © 2017 The Authors.

  14. GALAXY COUNTS ON THE COSMIC MICROWAVE BACKGROUND COLD SPOT

    International Nuclear Information System (INIS)

    Granett, Benjamin R.; Szapudi, Istvan; Neyrinck, Mark C.

    2010-01-01

    The cold spot on the cosmic microwave background (CMB) could arise due to a supervoid at low redshift through the integrated Sachs-Wolfe effect. We imaged the region with MegaCam on the Canada-France-Hawaii Telescope and present galaxy counts in photometric redshift bins. We rule out the existence of a 100 Mpc radius spherical supervoid with underdensity δ = -0.3 at 0.5 < z < 0.9 at high significance. The data are consistent with an underdensity at low redshift, but the fluctuations are within the range of cosmic variance and the low-density areas are not contiguous on the sky. Thus, we find no strong evidence for a supervoid. We cannot resolve voids smaller than a 50 Mpc radius; however, these can only make a minor contribution to the CMB temperature decrement.

  15. The interaction between dark energy and dark matter

    International Nuclear Information System (INIS)

    He Jianhua; Wang Bin

    2010-01-01

    In this review we first present a general formalism to study the growth of dark matter perturbations in the presence of interactions between dark matter(DM) and dark energy(DE). We also study the signature of such interaction on the temperature anisotropies of the large scale cosmic microwave background (CMB). We find that the effect of such interaction has significant signature on both the growth of dark matter structure and the late Integrated Sachs Wolfe effect(ISW). We further discuss the potential possibility to detect the coupling by cross-correlating CMB maps with tracers of the large scale structure. We finally confront this interacting model with WMAP 5-year data as well as other data sets. We find that in the 1σ range, the constrained coupling between dark sectors can solve the coincidence problem.

  16. Fear or food - abundance of red fox in relation to occurrence of lynx and wolf.

    Science.gov (United States)

    Wikenros, Camilla; Aronsson, Malin; Liberg, Olof; Jarnemo, Anders; Hansson, Jessica; Wallgren, Märtha; Sand, Håkan; Bergström, Roger

    2017-08-22

    Apex predators may affect mesopredators through intraguild predation and/or supply of carrion from their prey, causing a trade-off between avoidance and attractiveness. We used wildlife triangle snow-tracking data to investigate the abundance of red fox (Vulpes vulpes) in relation to lynx (Lynx lynx) and wolf (Canis lupus) occurrence as well as land composition and vole (Microtus spp.) density. Data from the Swedish wolf-monitoring system and VHF/GPS-collared wolves were used to study the effect of wolf pack size and time since wolf territory establishment on fox abundance. Bottom-up processes were more influential than top-down effects as the proportion of arable land was the key indicator of fox abundance at the landscape level. At this spatial scale, there was no effect of wolf abundance on fox abundance, whereas lynx abundance had a positive effect. In contrast, at the wolf territory level there was a negative effect of wolves on fox abundance when including detailed information of pack size and time since territory establishment, whereas there was no effect of lynx abundance. This study shows that different apex predator species may affect mesopredator abundance in different ways and that the results may be dependent on the spatiotemporal scale and resolution of the data.

  17. Late-onset Tay-Sachs disease: phenotypic characterization and genotypic correlations in 21 affected patients.

    Science.gov (United States)

    Neudorfer, Orit; Pastores, Gregory M; Zeng, Bai J; Gianutsos, John; Zaroff, Charles M; Kolodny, Edwin H

    2005-02-01

    The purpose of this study was to describe the phenotype (and corresponding genotype) of adult patients with late-onset Tay-Sachs disease, a clinical variant of the GM2-gangliosidoses. A comprehensive physical examination, including neurological assessments, was performed to establish the current disease pattern and severity. In addition, the patients' past medical histories were reviewed. The patients' alpha-subunit mutations (beta-Hexosaminidase A genotype) were determined and correlated with their corresponding clinical findings and disease course. Twenty-one patients (current mean age: 27.0 years; range: 14-47 years) were identified. The pedigree revealed a relative with the "classic" infantile or late-onset form of Tay-Sachs disease in four (out of 18) unrelated families. The patients were predominantly male (15/21 individuals) and of Ashkenazi Jewish ancestry (15/18 families). Mean age at onset was 18.1 years; balance problems and difficulty climbing stairs were the most frequent presenting complaints. In several cases, the diagnosis was delayed (mean age at diagnosis: 27.0 years). Analysis of the beta-hex A gene revealed the G269S mutation as the most common disease allele; found in homozygosity (N = 1) or heterozygosity (N = 18; including 2 sib pairs). Disease onset (age 36 years) was delayed and progression relatively slower in the homozygous G269S patient. Two siblings (ages 28 and 31 years), of non-Jewish ancestry, were compound heterozygotes (TATC1278/W474C); their clinical course is dominated by psychiatric problems. Brain imaging studies revealed marked cerebellar atrophy in all patients (N = 18) tested, regardless of disease stage. Late-onset Tay-Sachs disease is an infrequent disorder and the diagnosis is often missed or delayed (by approximately 8 years). Early on, the majority of patients develop signs of either cerebellar or anterior motor neuron involvement. Affected individuals may also develop psychotic episodes. In most cases, the later

  18. Alternative model for the atmospheres of Wolf-Rayet and O stars

    International Nuclear Information System (INIS)

    Underhill, A.B.

    1983-01-01

    It is noted that postulating dense spherically symmetric outflow in order to account for the observed radio fluxes from Wolf-Rayet stars requires that much more momentum must be deposited in the wind of a Wolf-Rayet star than is available from the radiation field of the star, and it is shown that the deduced massive winds become opaque in visible wavelengths at stellar radii where the velocity of outflow is several hundred km s - 1 . To attain such a state of motion in the wind requires the action of a strong, unknown source of outward directed momentum in optically thick layers of the star. This is an unreasonable demand. An alternative model for the mantle of a Wolf-Rayet star (the part of the atmosphere in which nonradiative energy and momentum are deposited) is proposed and shown to be feasible

  19. Audit of Wolf Creek Generating Station, Unit 1 technical specifications. Final technical evaluation report

    International Nuclear Information System (INIS)

    Stromberg, H.M.

    1985-07-01

    This document was prepared for the Nuclear Regulatory Commission (NRC) to assist them in determining whether the Wolf Creek Generating Station Unit 1 Technical Specifications (T/S), which govern plant systems configurations and operations, are in conformance with the assumptions of the Final Safety Analysis Report (FSAR) as amended, the requirements of the Safety Evaluation Report (SER) as supplemented, and the Comments and Responses to the Wolf Creek Technical Specification Draft Inspection Report. A comparative audit of the FSAR as amended, the SER as supplemented, and the Draft Inspection Report was performed with the Wolf Creek T/S. Several discrepancies were identified and subsequently resolved through discussions with the cognizant NRC reviewer, NRC staff reviewers and/or utility representatives. The Wolf Creek Generating Station Unit 1 T/S, to the extent reviewed, are in conformance with the FSAR, SER, and Draft Inspection Report

  20. Haematopoietic Stem Cell Transplantation Arrests the Progression of Neurodegenerative Disease in Late-Onset Tay-Sachs Disease.

    Science.gov (United States)

    Stepien, Karolina M; Lum, Su Han; Wraith, J Edmond; Hendriksz, Christian J; Church, Heather J; Priestman, David; Platt, Frances M; Jones, Simon; Jovanovic, Ana; Wynn, Robert

    2017-12-07

    Tay-Sachs disease is a rare metabolic disease caused by a deficiency of hexosaminidase A that leads to accumulation of GM2 gangliosides predominantly in neural tissue. Late-onset Tay-Sachs disease variant is associated with a higher level of residual HexA activity. Treatment options are limited, and there are a few described cases who have undergone haematopoietic stem cell transplantation (HSCT) with variable outcome.We describe a case of a 23-year-old male patient who presented with a long-standing tremor since 7 years of age. He had gait ataxia, a speech stammer and swallowing problems. His condition had had a static course apart from his tremor that had been gradually deteriorating. Because of the deterioration in his neurological function, the patient had an uneventful, matched-sibling donor bone marrow transplant at the age of 15 years. Eight years post-HSCT, at the age of 23, he retains full donor engraftment, and his white cell beta-HexA of 191 nmol/mg/h is comparable to normal controls (in-assay control = 187). He continues to experience some intentional tremor that is tolerable for daily life and nonprogressive since HSCT. HSCT is a potential treatment option which might arrest neurodegeneration in patients with LOTS.

  1. Localization of the eigenvalues of linear integral equations with applications to linear ordinary differential equations.

    Science.gov (United States)

    Sloss, J. M.; Kranzler, S. K.

    1972-01-01

    The equivalence of a considered integral equation form with an infinite system of linear equations is proved, and the localization of the eigenvalues of the infinite system is expressed. Error estimates are derived, and the problems of finding upper bounds and lower bounds for the eigenvalues are solved simultaneously.

  2. Problems with the claim of ecotype and taxon status of the wolf in the Great Lakes region

    Science.gov (United States)

    Cronin, Matthew A.; Mech, L. David

    2009-01-01

    Koblmuller et al. (2009) analysed molecular genetic data of the wolf in the Great Lakes (GL) region of the USA and concluded that the animal was a unique ecotype of grey wolf and that genetic data supported the population as a discrete wolf taxon. However, some of the literature that the researchers used to support their position actually did not, and additional confusion arises from indefinite use of terminology. Herein, we discuss the problems with designation of a wolf population as a taxon or ecotype without proper definition and assessment of criteria.

  3. Extraosseous osteosarcoma in a maned wolf (Chrysocyon brachyurus).

    Science.gov (United States)

    Reid, Heather L; Deem, Sharon L; Citino, Scott B

    2005-09-01

    A 6-yr-old maned wolf (Chrysocyon brachyurus) was diagnosed with an extraosseous osteosarcoma on the lateral aspect of the right thigh. Antemortem radiography revealed a calcified mass with no skeletal involvement. The mass was excised, but visible regrowth of the tumor was evident within 5 wk. Histologic examination and immunohistochemistry, including staining for p53 tumor suppression gene protein, were performed on the excised mass. The maned wolf was euthanized 13 wk after the initial diagnosis. The neoplasm was located in a site commonly used for the delivery of intramuscular injections, including vaccinations. Although no definitive association can be made, it is worth noting this relationship, as vaccine-site neoplasias have been observed in other species, most notably the domestic cat (Felis domesticus).

  4. Ecosystem scale declines in elk recruitment and population growth with wolf colonization: a before-after-control-impact approach.

    Science.gov (United States)

    Christianson, David; Creel, Scott

    2014-01-01

    The reintroduction of wolves (Canis lupus) to Yellowstone provided the unusual opportunity for a quasi-experimental test of the effects of wolf predation on their primary prey (elk--Cervus elaphus) in a system where top-down, bottom-up, and abiotic forces on prey population dynamics were closely and consistently monitored before and after reintroduction. Here, we examined data from 33 years for 12 elk population segments spread across southwestern Montana and northwestern Wyoming in a large scale before-after-control-impact analysis of the effects of wolves on elk recruitment and population dynamics. Recruitment, as measured by the midwinter juvenile∶female ratio, was a strong determinant of elk dynamics, and declined by 35% in elk herds colonized by wolves as annual population growth shifted from increasing to decreasing. Negative effects of population density and winter severity on recruitment, long recognized as important for elk dynamics, were detected in uncolonized elk herds and in wolf-colonized elk herds prior to wolf colonization, but not after wolf colonization. Growing season precipitation and harvest had no detectable effect on recruitment in either wolf treatment or colonization period, although harvest rates of juveniles∶females declined by 37% in wolf-colonized herds. Even if it is assumed that mortality due to predation is completely additive, liberal estimates of wolf predation rates on juvenile elk could explain no more than 52% of the total decline in juvenile∶female ratios in wolf-colonized herds, after accounting for the effects of other limiting factors. Collectively, these long-term, large-scale patterns align well with prior studies that have reported substantial decrease in elk numbers immediately after wolf recolonization, relatively weak additive effects of direct wolf predation on elk survival, and decreased reproduction and recruitment with exposure to predation risk from wolves.

  5. Ecosystem scale declines in elk recruitment and population growth with wolf colonization: a before-after-control-impact approach.

    Directory of Open Access Journals (Sweden)

    David Christianson

    Full Text Available The reintroduction of wolves (Canis lupus to Yellowstone provided the unusual opportunity for a quasi-experimental test of the effects of wolf predation on their primary prey (elk--Cervus elaphus in a system where top-down, bottom-up, and abiotic forces on prey population dynamics were closely and consistently monitored before and after reintroduction. Here, we examined data from 33 years for 12 elk population segments spread across southwestern Montana and northwestern Wyoming in a large scale before-after-control-impact analysis of the effects of wolves on elk recruitment and population dynamics. Recruitment, as measured by the midwinter juvenile∶female ratio, was a strong determinant of elk dynamics, and declined by 35% in elk herds colonized by wolves as annual population growth shifted from increasing to decreasing. Negative effects of population density and winter severity on recruitment, long recognized as important for elk dynamics, were detected in uncolonized elk herds and in wolf-colonized elk herds prior to wolf colonization, but not after wolf colonization. Growing season precipitation and harvest had no detectable effect on recruitment in either wolf treatment or colonization period, although harvest rates of juveniles∶females declined by 37% in wolf-colonized herds. Even if it is assumed that mortality due to predation is completely additive, liberal estimates of wolf predation rates on juvenile elk could explain no more than 52% of the total decline in juvenile∶female ratios in wolf-colonized herds, after accounting for the effects of other limiting factors. Collectively, these long-term, large-scale patterns align well with prior studies that have reported substantial decrease in elk numbers immediately after wolf recolonization, relatively weak additive effects of direct wolf predation on elk survival, and decreased reproduction and recruitment with exposure to predation risk from wolves.

  6. Wolf population genetics in Europe: a systematic review, meta-analysis and suggestions for conservation and management.

    Science.gov (United States)

    Hindrikson, Maris; Remm, Jaanus; Pilot, Malgorzata; Godinho, Raquel; Stronen, Astrid Vik; Baltrūnaité, Laima; Czarnomska, Sylwia D; Leonard, Jennifer A; Randi, Ettore; Nowak, Carsten; Åkesson, Mikael; López-Bao, José Vicente; Álvares, Francisco; Llaneza, Luis; Echegaray, Jorge; Vilà, Carles; Ozolins, Janis; Rungis, Dainis; Aspi, Jouni; Paule, Ladislav; Skrbinšek, Tomaž; Saarma, Urmas

    2017-08-01

    The grey wolf (Canis lupus) is an iconic large carnivore that has increasingly been recognized as an apex predator with intrinsic value and a keystone species. However, wolves have also long represented a primary source of human-carnivore conflict, which has led to long-term persecution of wolves, resulting in a significant decrease in their numbers, genetic diversity and gene flow between populations. For more effective protection and management of wolf populations in Europe, robust scientific evidence is crucial. This review serves as an analytical summary of the main findings from wolf population genetic studies in Europe, covering major studies from the 'pre-genomic era' and the first insights of the 'genomics era'. We analyse, summarize and discuss findings derived from analyses of three compartments of the mammalian genome with different inheritance modes: maternal (mitochondrial DNA), paternal (Y chromosome) and biparental [autosomal microsatellites and single nucleotide polymorphisms (SNPs)]. To describe large-scale trends and patterns of genetic variation in European wolf populations, we conducted a meta-analysis based on the results of previous microsatellite studies and also included new data, covering all 19 European countries for which wolf genetic information is available: Norway, Sweden, Finland, Estonia, Latvia, Lithuania, Poland, Czech Republic, Slovakia, Germany, Belarus, Russia, Italy, Croatia, Bulgaria, Bosnia and Herzegovina, Greece, Spain and Portugal. We compared different indices of genetic diversity in wolf populations and found a significant spatial trend in heterozygosity across Europe from south-west (lowest genetic diversity) to north-east (highest). The range of spatial autocorrelation calculated on the basis of three characteristics of genetic diversity was 650-850 km, suggesting that the genetic diversity of a given wolf population can be influenced by populations up to 850 km away. As an important outcome of this synthesis, we

  7. Adjustable Permanent Quadrupoles Using Rotating Magnet Material Rods for the Next Linear Collider

    International Nuclear Information System (INIS)

    Spencer, Cherrill M

    2002-01-01

    The proposed Next Linear Collider (NLC) will require over 1400 adjustable quadrupoles between the main linacs' accelerator structures. These 12.7 mm bore quadrupoles will have a range of integrated strength from 0.6 to 132 Tesla, with a maximum gradient of 135 Tesla per meter, an adjustment range of +0 -20% and effective lengths from 324 mm to 972 mm. The magnetic center must remain stable to within 1 micrometer during the 20% adjustment. In an effort to reduce estimated costs and increase reliability, several designs using hybrid permanent magnets have been developed. All magnets have iron poles and use either Samarium Cobalt or Neodymium Iron to provide the magnetic fields. Two prototypes use rotating rods containing permanent magnetic material to vary the gradient. Gradient changes of 20% and center shifts of less than 20 microns have been measured. These data are compared to an equivalent electromagnet prototype. See High Reliability Prototype Quadrupole for the Next Linear Collider by C.E Rago, C.M SPENCER, Z. Wolf submitted to this conference

  8. Cosmological constraints on the amplitude of relic gravitational waves

    International Nuclear Information System (INIS)

    Novosyadlij, B.; Apunevich, S.

    2005-01-01

    The evolution of the amplitude of relic gravitational waves (RGW) generated in early Universe has been analyzed. The analytical approximation is presented for angular power spectrum of cosmic microwave background anisotropies caused by gravitational waves through Sachs-Wolfe effect. The estimate of the most probable value for this amplitude was obtained on the basis of observation data on cosmic microwave background anisotropies from COBE, WMAP and BOOMERanG experiments along with large-scale structure observations

  9. How cosmic microwave background correlations at large angles relate to mass autocorrelations in space

    Science.gov (United States)

    Blumenthal, George R.; Johnston, Kathryn V.

    1994-01-01

    The Sachs-Wolfe effect is known to produce large angular scale fluctuations in the cosmic microwave background radiation (CMBR) due to gravitational potential fluctuations. We show how the angular correlation function of the CMBR can be expressed explicitly in terms of the mass autocorrelation function xi(r) in the universe. We derive analytic expressions for the angular correlation function and its multipole moments in terms of integrals over xi(r) or its second moment, J(sub 3)(r), which does not need to satisfy the sort of integral constraint that xi(r) must. We derive similar expressions for bulk flow velocity in terms of xi and J(sub 3). One interesting result that emerges directly from this analysis is that, for all angles theta, there is a substantial contribution to the correlation function from a wide range of distance r and that radial shape of this contribution does not vary greatly with angle.

  10. The strong 3.3 micron emission line in Wolf-Rayet stars

    International Nuclear Information System (INIS)

    Williams, P.M.

    1982-01-01

    A number of Wolf-Rayet stars have been found to show in their spectra a strong emission feature at 3.28 μm, the wavelength of the 'unidentified' feature observed in some nebular spectra. From comparison of the strength of this line from stars of different spectral type and excitation, it is identified with the CIV (11-10) transition group and shown not to be connected with the circumstellar dust associated with some Wolf-Rayet stars. (author)

  11. Assessment of prey vulnerability through analysis of wolf movements and kill sites.

    Science.gov (United States)

    Bergman, Eric J; Garrott, Robert A; Creel, Scott; Borkowski, John J; Jaffe, Rosemary; Watson, E G R

    2006-02-01

    Within predator-prey systems behavior can heavily influence spatial dynamics, and accordingly, the theoretical study of how spatial dynamics relate to stability within these systems has a rich history. However, our understanding of these behaviors in large mammalian systems is poorly developed. To address the relationship between predator selection patterns, prey density, and prey vulnerability, we quantified selection patterns for two fine-scale behaviors of a recovering wolf (Canis lupus) population in Yellowstone National Park, Wyoming, USA. Wolf spatial data were collected between November and May from 1998-1999 until 2001-2002. Over four winters, 244 aerial locations, 522 ground-based telemetry locations, 1287 km of movement data from snow tracking, and the locations of 279 wolf kill sites were recorded. There was evidence that elk (Cervus elaphus) and bison (Bison bison) densities had a weak effect on the sites where wolves traveled and made kills. Wolf movements showed a strong selection for geothermal areas, meadows, and areas near various types of habitat edges. Proximity to edge and habitat class also had a strong influence on the locations where elk were most vulnerable to predation. There was little evidence that wolf kill sites differed from the places where wolves traveled, indicating that elk vulnerability influenced where wolves selected to travel. Our results indicate that elk are more vulnerable to wolves under certain conditions and that wolves are capable of selecting for these conditions. As such, vulnerability plays a central role in predator-prey behavioral games and can potentially impact the systems to which they relate.

  12. Impact of wolf (Canis lupus on animal husbandry in an Apennine province

    Directory of Open Access Journals (Sweden)

    Claudia Russo

    2014-07-01

    Full Text Available Predation has always been an important problem in extensive sheep farms, causing serious economic losses to the farmers. In the Province of Lucca, the presence of reproductive wolf packs has already been confirmed in natural reserves, but occasional signs of presence of the predator have been reported also in neighbouring areas. The present research has been carried out in this Province (between the Orecchiella Natural Reserve and the medium Serchio Valley, in order to obtain more complete information on the location of the wolf (with transects, wolfhowling and snow-tracking, and to verify the real impact and risk factors of predation on livestock (by means of on-farm surveys carried out in 42 semi-extensive farms in this area. The presence of wolf was confirmed in the study area with a minimum of four adult individuals and at least one pup: this pack lives around the peaks of the Apennines in the municipalities covered by this investigation. A growing conflict between the wolf and the sheep and goat farms was observed: since 2007 there have been 25 attacks and three farms can be considered subject to chronic predation. The major risk factors are high altitude, large flock size and lack of fences and of guardian dogs. An accurate knowledge of wolf presence and the identification of the farms mostly at risk can be useful for future planning of interventions aimed at prevention and support of farmers, in order to mitigate the conflict caused by predation.

  13. Science, economics, and rhetoric: environmental advocacy and the wolf reintroduction debate, 1987-1999

    Science.gov (United States)

    Dayle C. Hardy-Short; C. Brant Short

    2000-01-01

    This paper examines the arguments employed in the debate over reintroduction of wolves into Idaho, Montana, and the Yellowstone National Park Ecosystem; and in Arizona and New Mexico. The study reviews common rhetorical themes used by advocates and opponents of wolf reintroduction and identifies a significant rhetorical shift in the debate. Advocates opposed to wolf...

  14. 76 FR 61781 - Endangered and Threatened Wildlife and Plants; Removal of the Gray Wolf in Wyoming From the...

    Science.gov (United States)

    2011-10-05

    ... gray wolf reintroductions in central Idaho and in Yellowstone National Park (YNP). The Yellowstone... Wolves to Yellowstone National Park and Central Idaho (EIS) reviewed wolf recovery in the NRM region and... Service 50 CFR Part 17 Endangered and Threatened Wildlife and Plants; Removal of the Gray Wolf in Wyoming...

  15. Anti-symmetrically fused model and non-linear integral equations in the three-state Uimin-Sutherland model

    International Nuclear Information System (INIS)

    Fujii, Akira; Kluemper, Andreas

    1999-01-01

    We derive the non-linear integral equations determining the free energy of the three-state pure bosonic Uimin-Sutherland model. In order to find a complete set of auxiliary functions, the anti-symmetric fusion procedure is utilized. We solve the non-linear integral equations numerically and see that the low-temperature behavior coincides with that predicted by conformal field theory. The magnetization and magnetic susceptibility are also calculated by means of the non-linear integral equation

  16. Spatial genetic analyses reveal cryptic population structure and migration patterns in a continuously harvested grey wolf (Canis lupus population in north-eastern Europe.

    Directory of Open Access Journals (Sweden)

    Maris Hindrikson

    Full Text Available Spatial genetics is a relatively new field in wildlife and conservation biology that is becoming an essential tool for unravelling the complexities of animal population processes, and for designing effective strategies for conservation and management. Conceptual and methodological developments in this field are therefore critical. Here we present two novel methodological approaches that further the analytical possibilities of STRUCTURE and DResD. Using these approaches we analyse structure and migrations in a grey wolf (Canislupus population in north-eastern Europe. We genotyped 16 microsatellite loci in 166 individuals sampled from the wolf population in Estonia and Latvia that has been under strong and continuous hunting pressure for decades. Our analysis demonstrated that this relatively small wolf population is represented by four genetic groups. We also used a novel methodological approach that uses linear interpolation to statistically test the spatial separation of genetic groups. The new method, which is capable of using program STRUCTURE output, can be applied widely in population genetics to reveal both core areas and areas of low significance for genetic groups. We also used a recently developed spatially explicit individual-based method DResD, and applied it for the first time to microsatellite data, revealing a migration corridor and barriers, and several contact zones.

  17. On the structure and applications of the Bondi-Metzner-Sachs group

    Science.gov (United States)

    Alessio, Francesco; Esposito, Giampiero

    This work is a pedagogical review dedicated to a modern description of the Bondi-Metzner-Sachs (BMS) group. Minkowski space-time has an interesting and useful group of isometries, but, for a generic space-time, the isometry group is simply the identity and hence provides no significant informations. Yet symmetry groups have important role to play in physics; in particular, the Poincaré group describing the isometries of Minkowski space-time plays a role in the standard definitions of energy-momentum and angular-momentum. For this reason alone it would seem to be important to look for a generalization of the concept of isometry group that can apply in a useful way to suitable curved space-times. The curved space-times that will be taken into account are the ones that suitably approach, at infinity, Minkowski space-time. In particular we will focus on asymptotically flat space-times. In this work, the concept of asymptotic symmetry group of those space-times will be studied. In the first two sections we derive the asymptotic group following the classical approach which was basically developed by Bondi, van den Burg, Metzner and Sachs. This is essentially the group of transformations between coordinate systems of a certain type in asymptotically flat space-times. In the third section the conformal method and the notion of “asymptotic simplicity” are introduced, following mainly the works of Penrose. This section prepares us for another derivation of the BMS group which will involve the conformal structure, and is thus more geometrical and fundamental. In the subsequent sections we discuss the properties of the BMS group, e.g. its algebra and the possibility to obtain as its subgroup the Poincaré group, as we may expect. The paper ends with a review of the BMS invariance properties of classical gravitational scattering discovered by Strominger, that are finding application to black hole physics and quantum gravity in the literature.

  18. Patterns of wolf pack movements prior to kills as read from tracks in Algonquin Provincial Park, Ont., Canada

    NARCIS (Netherlands)

    Frijlink, Jan Hilco

    1977-01-01

    From data, gathered by the author and his students, during a wolf study in Algonquin Provincial Park, Canada, six kills of white-tailed deer by wolf packs are described. Case histories are reconstructed by means of interpreting tracks. In one case a wolf was also killed, this animal turned out to

  19. A chaos wolf optimization algorithm with self-adaptive variable step-size

    Science.gov (United States)

    Zhu, Yong; Jiang, Wanlu; Kong, Xiangdong; Quan, Lingxiao; Zhang, Yongshun

    2017-10-01

    To explore the problem of parameter optimization for complex nonlinear function, a chaos wolf optimization algorithm (CWOA) with self-adaptive variable step-size was proposed. The algorithm was based on the swarm intelligence of wolf pack, which fully simulated the predation behavior and prey distribution way of wolves. It possessed three intelligent behaviors such as migration, summons and siege. And the competition rule as "winner-take-all" and the update mechanism as "survival of the fittest" were also the characteristics of the algorithm. Moreover, it combined the strategies of self-adaptive variable step-size search and chaos optimization. The CWOA was utilized in parameter optimization of twelve typical and complex nonlinear functions. And the obtained results were compared with many existing algorithms, including the classical genetic algorithm, the particle swarm optimization algorithm and the leader wolf pack search algorithm. The investigation results indicate that CWOA possess preferable optimization ability. There are advantages in optimization accuracy and convergence rate. Furthermore, it demonstrates high robustness and global searching ability.

  20. Severe maxillary osteomyelitis in a Gray Wolf (Canis lupus)

    Science.gov (United States)

    Barber-Meyer, Shannon

    2012-01-01

    Dental injuries to or abnormalities in functionally important teeth and associated bones in predators may significantly reduce the ability to kill and consume prey (Lazar et al. 2009). This impairment is likely exacerbated in coursing predators, such as Gray Wolves, that bite and hold onto fleeing and kicking prey with their teeth. Damage to carnassials (upper fourth premolar, P4, and lower first molar, M1) and associated bones in Gray Wolves may especially inhibit the consumption of prey because these teeth slice meat and crush bone. Here I report maxillary osteomyelitis involving the carnassials in a wild Gray Wolf from northeastern Minnesota of such severity that I hypothesize it ultimately caused the Gray Wolf to starve to death.

  1. The Baldwin-effect in Wolf-Rayet stars

    NARCIS (Netherlands)

    Gent, Jeroen Ivar van

    2000-01-01

    This thesis investigates the Baldwin-effect in Wolf-Rayet (WR) stars. The Baldwin-effect is a correlation with negative slope between the equivalent width of spectral emission lines and the monochromatic luminosity of the underlying continuum at the line wavelength. This effect has been known to

  2. The radii of the Wolf-Rayet stars and the extent of their chromosphere-corona formation

    Energy Technology Data Exchange (ETDEWEB)

    Sahade, J [Instituto de Astronomia y Fisica del Espacio, Buenos Aires, Argentina; Zorec, J [College de France, Paris, France

    1981-03-01

    The radii of 14 Wolf-Rayet stars are computed on the basis of previously reported absolute fluxes in the region from 4150 to 8000 A for 10 WN stars and from 3650 to 8000 A for four WC stars. For comparison, the radii of 11 Of stars are also calculated. The Wolf-Rayet radii are found to range from about 10 to 35 solar radii, values that do not appear to provide supporting evidence for the hypothesis that Of stars evolve into late WN stars. Available UV observations of Gamma-2 Vel are used to investigate the extent of the chromosphere-corona structure in Wolf-Rayet stars. It is suggested that the second electron-temperature maximum in a recently proposed model for the extended envelopes of Wolf-Rayet stars should be further than about 300 solar radii from the center of a star.

  3. Fractional order differentiation by integration: An application to fractional linear systems

    KAUST Repository

    Liu, Dayan

    2013-02-04

    In this article, we propose a robust method to compute the output of a fractional linear system defined through a linear fractional differential equation (FDE) with time-varying coefficients, where the input can be noisy. We firstly introduce an estimator of the fractional derivative of an unknown signal, which is defined by an integral formula obtained by calculating the fractional derivative of a truncated Jacobi polynomial series expansion. We then approximate the FDE by applying to each fractional derivative this formal algebraic integral estimator. Consequently, the fractional derivatives of the solution are applied on the used Jacobi polynomials and then we need to identify the unknown coefficients of the truncated series expansion of the solution. Modulating functions method is used to estimate these coefficients by solving a linear system issued from the approximated FDE and some initial conditions. A numerical result is given to confirm the reliability of the proposed method. © 2013 IFAC.

  4. Wolf population dynamics in the U.S. Northern Rocky Mountains are affected by recruitment and human-caused mortality

    Science.gov (United States)

    Gude, J.A.; Mitchell, M.S.; Russell, R.E.; Sime, C.A.; Bangs, E.E.; Mech, L.D.; Ream, R.R.

    2012-01-01

    Reliable analyses can help wildlife managers make good decisions, which are particularly critical for controversial decisions such as wolf (Canis lupus) harvest. Creel and Rotella (2010) recently predicted substantial population declines in Montana wolf populations due to harvest, in contrast to predictions made by Montana Fish, Wildlife and Parks (MFWP). We replicated their analyses considering only those years in which field monitoring was consistent, and we considered the effect of annual variation in recruitment on wolf population growth. Rather than assuming constant rates, we used model selection methods to evaluate and incorporate models of factors driving recruitment and human-caused mortality rates in wolf populations in the Northern Rocky Mountains. Using data from 27 area-years of intensive wolf monitoring, we show that variation in both recruitment and human-caused mortality affect annual wolf population growth rates and that human-caused mortality rates have increased with the sizes of wolf populations. We document that recruitment rates have decreased over time, and we speculate that rates have decreased with increasing population sizes and/or that the ability of current field resources to document recruitment rates has recently become less successful as the number of wolves in the region has increased. Estimates of positive wolf population growth in Montana from our top models are consistent with field observations and estimates previously made by MFWP for 2008-2010, whereas the predictions for declining wolf populations of Creel and Rotella (2010) are not. Familiarity with limitations of raw data, obtained first-hand or through consultation with scientists who collected the data, helps generate more reliable inferences and conclusions in analyses of publicly available datasets. Additionally, development of efficient monitoring methods for wolves is a pressing need, so that analyses such as ours will be possible in future years when fewer resources

  5. Wolf-Rayet stars

    Energy Technology Data Exchange (ETDEWEB)

    Sahade, J

    1981-12-01

    Aspects of the problems of the Wolf-Rayet stars related to their chemical composition, their evolutionary status, and their apparent dichotomy in two spectral sequences are discussed. Dogmas concerning WR stars are critically discussed, including the belief that WR stars lack hydrogen, that they are helium stars evolved from massive close binaries, and the existence of a second WR stage in which the star is a short-period single-lined binary. The relationship of WR stars with planetary nebulae is addressed, as is the membership of these stars in clusters and associations. The division of WR stars into WN and WC sequences is considered, questioning the reasonability of accounting for WR line formation in terms of abundance differences.

  6. Wolf-Rayet phenomena

    International Nuclear Information System (INIS)

    Conti, P.S.

    1982-01-01

    The author reviews in broad terms the concept of Wolf-Rayet (W-R) phenomena, outlines what we currently know about the properties of stars showing such phenomena and indicates the directions in which future work is leading. He begins by listing the characteristics of W-R spectra and then considers the following specific problems: the absolute visual magnitudes; the heterogeneity of WN spectra; the existence of transition type spectra and compositions; the mass loss rates; the existence of very luminous and possibly very massive W-R stars. He discusses briefly our current understanding of the theoretical aspects of stellar evolution and stellar winds and the various scenarios that have been proposed to understand W-R stars. (Auth.)

  7. The 2015 Wolf volcano (Galápagos) eruption studied using Sentinel-1 and ALOS-2 data

    KAUST Repository

    Xu, Wenbin; Jonsson, Sigurjon; Ruch, Joel; Aoki, Yosuke

    2016-01-01

    An energetic eruption started on 25 May 2015 from a circumferential fissure at the summit of Wolf volcano on Isabela Island, western Galápagos. Further eruptive activity within the Wolf caldera followed in mid-June 2015. As no geodetic observations of earlier eruptions at Wolf exist, this eruption provides an opportunity to study the volcano's magmatic plumbing system for the first time. Here we use interferometric synthetic aperture radar (InSAR) data from both the Sentinel-1A and ALOS-2 satellites to map and analyze the surface deformation at four time periods during the activity. These data allow us to identify the two eruption phases and reveal strong coeruptive subsidence within the Wolf caldera that is superimposed on a larger volcano-wide subsidence signal. Modeling of the surface displacements shows that two shallow magma reservoirs located under Wolf at ~1 km and ~5 km below sea level explain the subsidence and that these reservoirs appear to be hydraulically connected. We also suggest that the transition from the circumferential to the intracaldera eruption may have involved ring fault activity.

  8. The 2015 Wolf volcano (Galápagos) eruption studied using Sentinel-1 and ALOS-2 data

    KAUST Repository

    Xu, Wenbin

    2016-09-28

    An energetic eruption started on 25 May 2015 from a circumferential fissure at the summit of Wolf volcano on Isabela Island, western Galápagos. Further eruptive activity within the Wolf caldera followed in mid-June 2015. As no geodetic observations of earlier eruptions at Wolf exist, this eruption provides an opportunity to study the volcano\\'s magmatic plumbing system for the first time. Here we use interferometric synthetic aperture radar (InSAR) data from both the Sentinel-1A and ALOS-2 satellites to map and analyze the surface deformation at four time periods during the activity. These data allow us to identify the two eruption phases and reveal strong coeruptive subsidence within the Wolf caldera that is superimposed on a larger volcano-wide subsidence signal. Modeling of the surface displacements shows that two shallow magma reservoirs located under Wolf at ~1 km and ~5 km below sea level explain the subsidence and that these reservoirs appear to be hydraulically connected. We also suggest that the transition from the circumferential to the intracaldera eruption may have involved ring fault activity.

  9. Developing metapopulation connectivity criteria from genetic and habitat data to recover the endangered Mexican wolf.

    Science.gov (United States)

    Carroll, Carlos; Fredrickson, Richard J; Lacy, Robert C

    2014-02-01

    Restoring connectivity between fragmented populations is an important tool for alleviating genetic threats to endangered species. Yet recovery plans typically lack quantitative criteria for ensuring such population connectivity. We demonstrate how models that integrate habitat, genetic, and demographic data can be used to develop connectivity criteria for the endangered Mexican wolf (Canis lupus baileyi), which is currently being restored to the wild from a captive population descended from 7 founders. We used population viability analysis that incorporated pedigree data to evaluate the relation between connectivity and persistence for a restored Mexican wolf metapopulation of 3 populations of equal size. Decreasing dispersal rates greatly increased extinction risk for small populations (0.5 genetically effective migrants per generation may be achievable via natural dispersal under current landscape conditions. When sufficient data are available, these methods allow planners to move beyond general aspirational connectivity goals or rules of thumb to develop objective and measurable connectivity criteria that more effectively support species recovery. The shift from simple connectivity rules of thumb to species-specific analyses parallels the previous shift from general minimum-viable-population thresholds to detailed viability modeling in endangered species recovery planning. © 2013 Society for Conservation Biology.

  10. Near infrared spectra of Wolf-Rayet stars

    International Nuclear Information System (INIS)

    Bappu, M.K.V.; Ganesh, K.S.; Scaria, K.K.

    1977-01-01

    The near-infrared spectra of three Wolf-Rayet stars of the carbon sequence and five of the nitrogen sequence have been studied. Wavelength identifications and intensity scans are presented to show the emission line characteristics of these objects in the 6800 A to 8200 A domain of the spectrum. (author)

  11. Dynamics and bifurcations of a three-dimensional piecewise-linear integrable map

    International Nuclear Information System (INIS)

    Tuwankotta, J M; Quispel, G R W; Tamizhmani, K M

    2004-01-01

    In this paper, we consider a four-parameter family of piecewise-linear ordinary difference equations (OΔEs) in R 3 . This system is obtained as a limit of another family of three-dimensional integrable systems of OΔEs. We prove that the limiting procedure sends integrals of the original system to integrals of the limiting system. We derive some results for the solutions such as boundedness of solutions and the existence of periodic solutions. We describe all topologically different shapes of the integral manifolds and present all possible scenarios of transitions as we vary the natural parameters in the system, i.e. the values of the integrals

  12. MASTICATORY MUSCLE MYOSITIS IN A GRAY WOLF (CANIS LUPUS).

    Science.gov (United States)

    Kent, Marc; Glass, Eric N; Castro, Fernando A; Miller, Andrew D; de Lahunta, Alexander

    2017-03-01

    A 10-yr-old male, neutered gray wolf ( Canis lupus ) was presented for atrophy of the temporalis and masseter muscles. Clinical signs and magnetic resonance imaging were consistent with a myopathy. Positive serology for antibody titers directed against Type 2M myofibers, and the observation of a mixed mononuclear inflammatory cell infiltrate along with eosinophils and neutrophils within the temporalis muscle, were diagnostic for masticatory muscle myositis. Importantly, protozoal myositis was excluded based on other clinicopathologic data. The case highlights the potential for immune-mediated polymyositis in canids other than the domesticated dog ( Canis lupus familaris). Additionally, awareness of a diet in which raw meat is used should prompt a thorough investigation for an underlying infectious myositis in the gray wolf.

  13. A dust shell around the early-type Wolf-Ryate star WR 19

    International Nuclear Information System (INIS)

    Williams, P.M.; Hucht, K.A. van der; Bouchet, P.

    1990-01-01

    Infrared photometry of the WC4-type Wolf-Rayet star WR 19 (LS 3) in 1988-90 shows evidence for an expanding dust shell in its wind, similar to those observed from late-type WR stars like WR 48a (WC8), WR 140 (WC7+04) and WR 137 (WC7+). This demonstrates that dust formation by Wolf-Rayet stars is not restricted to later WC subtypes and is more common than hitherto supposed. (author)

  14. 75 FR 24741 - Endangered and Threatened Wildlife and Plants; Mexican Wolf (Canis lupus baileyi) Conservation...

    Science.gov (United States)

    2010-05-05

    ...] Endangered and Threatened Wildlife and Plants; Mexican Wolf (Canis lupus baileyi) Conservation Assessment... Mexico Ecological Services Field Office, 2105 Osuna NE, Albuquerque, NM 87113; by telephone at 505-761... guided by the 1982 Mexican Wolf Recovery Plan (U.S. Fish and Wildlife Service 1982) (recovery plan...

  15. An historical overview and update of wolf-moose interactions in northeastern Minnesota

    Science.gov (United States)

    Mech, L. David; Fieberg, John; Barber-Meyer, Shannon

    2018-01-01

    Wolf (Canis lupus) and moose (Alces americanus) populations in northeastern Minnesota, USA, have fluctuated for decades and, based on helicopter counts, moose numbers declined to a new low from 2006 to about 2012. Other steep declines were found in 1991 and 1998 during periods when moose counts were done with ®xed-wing aircraft; these declines also appeared to be real. Winter wolf numbers, monitored in part of the moose range, had been increasing since about 2002 to the highest population in decades in 2009. However, from 2009 to 2016, wolves decreased precipitously, and the moose-population decline leveled off from 2012 to 2017. Calf:population ratios from 1985 to 1997 and from 2005 to 2016 were inversely related to wolf numbers in the wolf-study area the previous winter both as wolves increased and decreased in abundance. Similarly, log annual growth rates of moose numbers were negatively correlated with counts of wolves in the prior year. Other factors such as nutrition and parasites, and possibly climate change, likely have been involved in the recent moose decline. However, wolves, as in other areas, appear to have contributed to the decline in the northeastern Minnesota moose population at least in part through predation on calves, supporting earlier reports. Published 2018. This article is a U.S. Government work and is in the public domain in the USA.

  16. William L. Wolfe, 1989 President of SPIE, encourages scientists from Eastern Europe

    Science.gov (United States)

    Schmit, Joanna

    2012-10-01

    In 1990 Professor Wolfe after his SPIE presidency trekked the world, even making it as far as post-communist Poland, to see (in the visible and maybe in infrared - who knows) the work of optical scientists hidden behind the iron curtain. I am not sure if he was ready for how different that world was at this time, but for sure he was very inquisitive and eager to learn about the nuances of Poland right after the fall of communism. He met, visited with and encouraged young and old scientists from Poland, Russia, Hungary and Lithuania to add their expertise to the scientific conversations happening in the West. His mission in Poland was to invite us all, and he was ready to help us achieve our dreams. I was one of those he encouraged. This talk is my personal reflection of Professor Wolfe as an encouraging and sometimes brave SPIE pioneer - a stranger in a strange land - and as an energetic, caring SPIE president, Optical Sciences professor and human being. Disclaimer: Professor Bill Wolfe's contributions to the field of radiometry are well known and very well recognized. This conference is a tribute to him. However, my paper is not on radiometry; rather, I wish to illustrate the adventurous, caring and positive Bill Wolfe that helped me find my way to the American desert Southwest.

  17. Phenomenological dynamics of loop quantum cosmology in Kantowski-Sachs spacetime

    International Nuclear Information System (INIS)

    Chiou, D.-W.

    2008-01-01

    The fundamental theory and the semiclassical description of loop quantum cosmology (LQC) have been studied in the Friedmann-Robertson-Walker and Bianchi I models. As an extension to include both anisotropy and intrinsic curvature, this paper investigates the cosmological model of Kantowski-Sachs spacetime with a free massless scalar field at the level of phenomenological dynamics with the LQC discreteness corrections. The LQC corrections are implemented in two different improved quantization schemes. In both schemes, the big bang and big crunch singularities of the classical solution are resolved and replaced by the big bounces when the area or volume scale factor approaches the critical values in the Planck regime measured by the reference of the scalar field momentum. Symmetries of scaling are also noted and suggest that the fundamental spatial scale (area gap) may give rise to a temporal scale. The bouncing scenarios are in an analogous fashion of the Bianchi I model, naturally extending the observations obtained previously.

  18. A chaos wolf optimization algorithm with self-adaptive variable step-size

    Directory of Open Access Journals (Sweden)

    Yong Zhu

    2017-10-01

    Full Text Available To explore the problem of parameter optimization for complex nonlinear function, a chaos wolf optimization algorithm (CWOA with self-adaptive variable step-size was proposed. The algorithm was based on the swarm intelligence of wolf pack, which fully simulated the predation behavior and prey distribution way of wolves. It possessed three intelligent behaviors such as migration, summons and siege. And the competition rule as “winner-take-all” and the update mechanism as “survival of the fittest” were also the characteristics of the algorithm. Moreover, it combined the strategies of self-adaptive variable step-size search and chaos optimization. The CWOA was utilized in parameter optimization of twelve typical and complex nonlinear functions. And the obtained results were compared with many existing algorithms, including the classical genetic algorithm, the particle swarm optimization algorithm and the leader wolf pack search algorithm. The investigation results indicate that CWOA possess preferable optimization ability. There are advantages in optimization accuracy and convergence rate. Furthermore, it demonstrates high robustness and global searching ability.

  19. Relationship between snow depth and gray wolf predation on white-tailed deer

    Science.gov (United States)

    Nelson, M.E.; Mech, L.D.

    1986-01-01

    Survival of 203 yearling and adult white-tailed deer (Odocoileus virginianus) was monitored for 23,441 deer days from January through April 1975-85 in northeastern Minnesota. Gray wolf (Canis lupus) predation was the primary mortality cause, and from year to year during this period, the mean predation rate ranged from 0.00 to 0.29. The sum of weekly snow depths/month explained 51% of the variation in annual wolf predation rate, with the highest predation during the deepest snow.

  20. Local hyperspectral data multisharpening based on linear/linear-quadratic nonnegative matrix factorization by integrating lidar data

    Science.gov (United States)

    Benhalouche, Fatima Zohra; Karoui, Moussa Sofiane; Deville, Yannick; Ouamri, Abdelaziz

    2015-10-01

    In this paper, a new Spectral-Unmixing-based approach, using Nonnegative Matrix Factorization (NMF), is proposed to locally multi-sharpen hyperspectral data by integrating a Digital Surface Model (DSM) obtained from LIDAR data. In this new approach, the nature of the local mixing model is detected by using the local variance of the object elevations. The hyper/multispectral images are explored using small zones. In each zone, the variance of the object elevations is calculated from the DSM data in this zone. This variance is compared to a threshold value and the adequate linear/linearquadratic spectral unmixing technique is used in the considered zone to independently unmix hyperspectral and multispectral data, using an adequate linear/linear-quadratic NMF-based approach. The obtained spectral and spatial information thus respectively extracted from the hyper/multispectral images are then recombined in the considered zone, according to the selected mixing model. Experiments based on synthetic hyper/multispectral data are carried out to evaluate the performance of the proposed multi-sharpening approach and literature linear/linear-quadratic approaches used on the whole hyper/multispectral data. In these experiments, real DSM data are used to generate synthetic data containing linear and linear-quadratic mixed pixel zones. The DSM data are also used for locally detecting the nature of the mixing model in the proposed approach. Globally, the proposed approach yields good spatial and spectral fidelities for the multi-sharpened data and significantly outperforms the used literature methods.

  1. Tay-Sachs disease in Jacob sheep.

    Science.gov (United States)

    Torres, Paola A; Zeng, Bai Jin; Porter, Brian F; Alroy, Joseph; Horak, Fred; Horak, Joan; Kolodny, Edwin H

    2010-12-01

    Autopsy studies of four Jacob sheep dying within their first 6-8 months of a progressive neurodegenerative disorder suggested the presence of a neuronal storage disease. Lysosomal enzyme studies of brain and liver from an affected animal revealed diminished activity of hexosaminidase A (Hex A) measured with an artificial substrate specific for this component of β-hexosaminidase. Absence of Hex A activity was confirmed by cellulose acetate electrophoresis. Brain lipid analyses demonstrated the presence of increased concentrations of G(M2)-ganglioside and asialo-G(M2)-ganglioside. The hexa cDNA of Jacob sheep was cloned and sequenced revealing an identical number of nucleotides and exons as in human HexA and 86% homology in nucleotide sequence. A missense mutation was found in the hexa cDNA of the affected sheep caused by a single nucleotide change at the end of exon 11 resulting in skipping of exon 11. Transfection of normal sheep hexa cDNA into COS1 cells and human Hex A-deficient cells led to expression of Hex S but no increase in Hex A indicating absence of cross-species dimerization of sheep Hex α-subunit with human Hex β-subunits. Using restriction site analysis, the heterozygote frequency of this mutation in Jacob sheep was determined in three geographically separate flocks to average 14%. This large naturally occurring animal model of Tay-Sachs disease is the first to offer promise as a means for trials of gene therapy applicable to human infants. Copyright © 2010 Elsevier Inc. All rights reserved.

  2. The spectrum of HM Sagittae: a planetary nebula excited by a Wolf--Rayet star

    International Nuclear Information System (INIS)

    Brown, L.W.; Feibelman, W.A.; Hobbs, R.W.; Mccracken, C.W.

    1977-10-01

    Image tube spectrograms of HM Sagittae were obtained. More than 70 emission lines, including several broad emission features, were identified. An analysis of the spectra indicates that HM Sagittae is a planetary nebula excited by a Wolf-Rayet star. The most conspicuous Wolf-Rayet feature is that attributed to a blend of C III at 4650 A and He II at 4686 A

  3. Crystal Structure of Human [Beta]-Hexosaminidase B: Understanding the Molecular Basis of Sandhoff and Tay-Sachs Disease

    Energy Technology Data Exchange (ETDEWEB)

    Mark, Brian L.; Mahuran, Don J.; Cherney, Maia M.; Zhao, Dalian; Knapp, Spencer; James, Michael N.G.

    2010-12-01

    In humans, two major {beta}-hexosaminidase isoenzymes exist: Hex A and Hex B. Hex A is a heterodimer of subunits {alpha} and {beta} (60% identity), whereas Hex B is a homodimer of {beta}-subunits. Interest in human {beta}-hexosaminidase stems from its association with Tay-Sachs and Sandhoff disease; these are prototypical lysosomal storage disorders resulting from the abnormal accumulation of G{sub M2}-ganglioside (G{sub M2}). Hex A degrades G{sub M2} by removing a terminal N-acetyl-D-galactosamine ({beta}-GalNAc) residue, and this activity requires the G{sub M2}-activator, a protein which solubilizes the ganglioside for presentation to Hex A. We present here the crystal structure of human Hex B, alone (2.4 {angstrom}) and in complex with the mechanistic inhibitors GalNAc-isofagomine (2.2 {angstrom}) or NAG-thiazoline (2.5 {angstrom}). From these, and the known X-ray structure of the G{sub M2}-activator, we have modeled Hex A in complex with the activator and ganglioside. Together, our crystallographic and modeling data demonstrate how {alpha} and {beta}-subunits dimerize to form either Hex A or Hex B, how these isoenzymes hydrolyze diverse substrates, and how many documented point mutations cause Sandhoff disease ({beta}-subunit mutations) and Tay-Sachs disease ({alpha}-subunit mutations).

  4. Efficient decomposition and linearization methods for the stochastic transportation problem

    International Nuclear Information System (INIS)

    Holmberg, K.

    1993-01-01

    The stochastic transportation problem can be formulated as a convex transportation problem with nonlinear objective function and linear constraints. We compare several different methods based on decomposition techniques and linearization techniques for this problem, trying to find the most efficient method or combination of methods. We discuss and test a separable programming approach, the Frank-Wolfe method with and without modifications, the new technique of mean value cross decomposition and the more well known Lagrangian relaxation with subgradient optimization, as well as combinations of these approaches. Computational tests are presented, indicating that some new combination methods are quite efficient for large scale problems. (authors) (27 refs.)

  5. Parasite species of the endangered Iberian wolf (Canis lupus signatus and a sympatric widespread carnivore

    Directory of Open Access Journals (Sweden)

    Ana Figueiredo

    2016-08-01

    Full Text Available Parasites have a profound impact on wildlife population dynamics. However, until some years ago, studies on the occurrence and prevalence of wildlife parasites were neglected comparatively with the studies on humans and domestic animals. In this study, we determined the parasite prevalence of two sympatric wild canids: the endangered Iberian wolf (Canis lupus signatus and the widespread red fox (Vulpes vulpes, in central Portugal. From November 2014 to July 2015, fresh fecal samples from both species were collected monthly in several transects distributed throughout the study area. All samples were submitted to several coprological techniques. In total, 6 helminth parasites (Crenosoma vulpis, Angiostrongylus vasorum, Toxocara canis, Trichuris vulpis, Ancylostomatidae, Toxascaris leonina, and a protozoa (Balantidium coli were identified based on size and morphology. The red fox was infected by seven different parasites while the Iberian wolf was infected by four. All parasites present in wolf were also present in the red fox. C. vulpis had the higher prevalence in red fox, while Ancylostomatidae were the most prevalent parasites in wolf. To our knowledge, this is the first study in this isolated subpopulation of the Iberian wolf. Our results show that both carnivores carry parasites that are of concern as they are pathogenic to humans and other wild and domestic animals. We suggest that surveillance programs must also include monitoring protocols of wildlife; particularly endangered species.

  6. Area density of localization-entropy II: double cone-localization and quantum origin of the Bondi-Metzner-Sachs symmetry

    Energy Technology Data Exchange (ETDEWEB)

    Schroer, Bert

    2006-04-15

    The holographic encoding is generalized to subalgebras of QFT localized in double cones. It is shown that as a result of this radically different spacetime encoding the modular group acts geometrically on the holographic image. As a result we obtain a formula for localization entropy which is identical to the previously derived formula for the wedge-localized subalgebra. The symmetry group in the holographic encoding turns out to be the Bondi-Metzner-Sachs group. (author)

  7. A mixed integer linear program for an integrated fishery | Hasan ...

    African Journals Online (AJOL)

    ... and labour allocation of quota based integrated fisheries. We demonstrate the workability of our model with a numerical example and sensitivity analysis based on data obtained from one of the major fisheries in New Zealand. Keywords: mixed integer linear program, fishing, trawler scheduling, processing, quotas ORiON: ...

  8. Pathological alterations typical of human Tay-Sachs disease, in the retina of a deep-sea fish

    Science.gov (United States)

    Fishelson, L.; Delarea, Yacov; Galil, Bella S.

    Micrographs of retinas from the deep-sea fish Cataetyx laticeps revealed visual cells containing membranous whorls in the ellipsoids of the inner segments resulting from stretching and modifications of the mitochondria membranes and their cristae. These pathological structures seem to be homologous to the whorls observed in retinas of human carriers of Tay-Sachs disease. This disease, a genetic disorder, is found in humans and some mammals. Our findings in fish suggest that the gene responsible can be found throughout the vertebrate evolutionary tree, possibly dormant in most taxa.

  9. Quasi-Linear Algebras and Integrability (the Heisenberg Picture

    Directory of Open Access Journals (Sweden)

    Alexei Zhedanov

    2008-02-01

    Full Text Available We study Poisson and operator algebras with the ''quasi-linear property'' from the Heisenberg picture point of view. This means that there exists a set of one-parameter groups yielding an explicit expression of dynamical variables (operators as functions of ''time'' t. We show that many algebras with nonlinear commutation relations such as the Askey-Wilson, q-Dolan-Grady and others satisfy this property. This provides one more (explicit Heisenberg evolution interpretation of the corresponding integrable systems.

  10. A hybrid Dantzig-Wolfe, Benders decomposition and column generation procedure for multiple diet production planning under uncertainties

    Science.gov (United States)

    Udomsungworagul, A.; Charnsethikul, P.

    2018-03-01

    This article introduces methodology to solve large scale two-phase linear programming with a case of multiple time period animal diet problems under both nutrients in raw materials and finished product demand uncertainties. Assumption of allowing to manufacture multiple product formulas in the same time period and assumption of allowing to hold raw materials and finished products inventory have been added. Dantzig-Wolfe decompositions, Benders decomposition and Column generations technique has been combined and applied to solve the problem. The proposed procedure was programmed using VBA and Solver tool in Microsoft Excel. A case study was used and tested in term of efficiency and effectiveness trade-offs.

  11. Predicting wolf (Canis lupus)-cattle (Bos Taurus) encounters and consequential effects on cattle resource selection patterns

    Science.gov (United States)

    The gray wolf population in Idaho has grown dramatically from the original 35 reintroduced individuals in 1995-1996 to 94 documented packs and a minimum population of 835 individuals in 2009. Wolf depredation on livestock has also increased dramatically with this population growth. Substantial spa...

  12. The clustering of galaxies in the completed SDSS-III Baryon Oscillation Spectroscopic Survey: cosmic flows and cosmic web from luminous red galaxies

    Science.gov (United States)

    Ata, Metin; Kitaura, Francisco-Shu; Chuang, Chia-Hsun; Rodríguez-Torres, Sergio; Angulo, Raul E.; Ferraro, Simone; Gil-Marín, Hector; McDonald, Patrick; Hernández Monteagudo, Carlos; Müller, Volker; Yepes, Gustavo; Autefage, Mathieu; Baumgarten, Falk; Beutler, Florian; Brownstein, Joel R.; Burden, Angela; Eisenstein, Daniel J.; Guo, Hong; Ho, Shirley; McBride, Cameron; Neyrinck, Mark; Olmstead, Matthew D.; Padmanabhan, Nikhil; Percival, Will J.; Prada, Francisco; Rossi, Graziano; Sánchez, Ariel G.; Schlegel, David; Schneider, Donald P.; Seo, Hee-Jong; Streblyanska, Alina; Tinker, Jeremy; Tojeiro, Rita; Vargas-Magana, Mariana

    2017-06-01

    We present a Bayesian phase-space reconstruction of the cosmic large-scale matter density and velocity fields from the Sloan Digital Sky Survey-III Baryon Oscillations Spectroscopic Survey Data Release 12 CMASS galaxy clustering catalogue. We rely on a given Λ cold dark matter cosmology, a mesh resolution in the range of 6-10 h-1 Mpc, and a lognormal-Poisson model with a redshift-dependent non-linear bias. The bias parameters are derived from the data and a general renormalized perturbation theory approach. We use combined Gibbs and Hamiltonian sampling, implemented in the argo code, to iteratively reconstruct the dark matter density field and the coherent peculiar velocities of individual galaxies, correcting hereby for coherent redshift space distortions. Our tests relying on accurate N-body-based mock galaxy catalogues show unbiased real space power spectra of the non-linear density field up to k ˜ 0.2 h Mpc-1, and vanishing quadrupoles down to r ˜ 20 h-1 Mpc. We also demonstrate that the non-linear cosmic web can be obtained from the tidal field tensor based on the Gaussian component of the reconstructed density field. We find that the reconstructed velocities have a statistical correlation coefficient compared to the true velocities of each individual light-cone mock galaxy of r ˜ 0.68 including about 10 per cent of satellite galaxies with virial motions (about r = 0.75 without satellites). The power spectra of the velocity divergence agree well with theoretical predictions up to k ˜ 0.2 h Mpc-1. This work will be especially useful to improve, for example, baryon acoustic oscillation reconstructions, kinematic Sunyaev-Zeldovich, integrated Sachs-Wolfe measurements or environmental studies.

  13. Study of Piezoelectric Vibration Energy Harvester with non-linear conditioning circuit using an integrated model

    Science.gov (United States)

    Manzoor, Ali; Rafique, Sajid; Usman Iftikhar, Muhammad; Mahmood Ul Hassan, Khalid; Nasir, Ali

    2017-08-01

    Piezoelectric vibration energy harvester (PVEH) consists of a cantilever bimorph with piezoelectric layers pasted on its top and bottom, which can harvest power from vibrations and feed to low power wireless sensor nodes through some power conditioning circuit. In this paper, a non-linear conditioning circuit, consisting of a full-bridge rectifier followed by a buck-boost converter, is employed to investigate the issues of electrical side of the energy harvesting system. An integrated mathematical model of complete electromechanical system has been developed. Previously, researchers have studied PVEH with sophisticated piezo-beam models but employed simplistic linear circuits, such as resistor, as electrical load. In contrast, other researchers have worked on more complex non-linear circuits but with over-simplified piezo-beam models. Such models neglect different aspects of the system which result from complex interactions of its electrical and mechanical subsystems. In this work, authors have integrated the distributed parameter-based model of piezo-beam presented in literature with a real world non-linear electrical load. Then, the developed integrated model is employed to analyse the stability of complete energy harvesting system. This work provides a more realistic and useful electromechanical model having realistic non-linear electrical load unlike the simplistic linear circuit elements employed by many researchers.

  14. The origins of the enigmatic Falkland Islands wolf.

    Science.gov (United States)

    Austin, Jeremy J; Soubrier, Julien; Prevosti, Francisco J; Prates, Luciano; Trejo, Valentina; Mena, Francisco; Cooper, Alan

    2013-01-01

    The origins of the extinct Falkland Islands wolf (FIW), Dusicyon australis, have remained a mystery since it was first recorded by Europeans in the seventeenth century. It is the only terrestrial mammal on the Falkland Islands (also known as the Malvinas Islands), which lie ~460 km from Argentina, leading to suggestions of either human-mediated transport or overwater dispersal. Previous studies used ancient DNA from museum specimens to suggest that the FIW diverged from its closest living relative, the South American maned wolf (Chrysocyon brachyurus) around 7 Ma, and colonized the islands ~330 ka by unknown means. Here we retrieve ancient DNA from subfossils of an extinct mainland relative, Dusicyon avus, and reveal the FIW lineage became isolated only 16 ka (8-31 ka), during the last glacial phase. Submarine terraces, formed on the Argentine coastal shelf by low sea-stands during this period, suggest that the FIW colonized via a narrow, shallow marine strait, potentially while it was frozen over.

  15. Preventing Lone Wolf Terrorism: some CT Approaches Addressed

    Directory of Open Access Journals (Sweden)

    Edwin Bakker

    2011-12-01

    Full Text Available After a brief discussion of the epistemological and phenomenological difficulties associated with the concept of lone wolf terrorism, a number of possible counter-terrorist approaches are discussed. Lone operator terrorist acts should be considered ‘black swan’ occurrences that are almost impossible to categorize or systematize, let alone forecast. Thus, not the profile of the perpetrator, but the modus operandi offer clues for a better response to this particular threat. Furthermore, almost all lone operators do display a degree of commitment to, and identification with, extremist movements – providing leads for preventing new rounds of radicalization within this potential group of sympathizers or followers. With the apparent increase of Islamist lone wolf terrorism and fears for right-wing extremists wanting to follow the example of the Norwegian mass murderer A.B. Breivik, new questions need to be posed, addressing the role of virtual communities with which lone operators identify themselves. 

  16. Death of a wild wolf from canine parvovirus enteritis

    Science.gov (United States)

    Mech, L.D.; Kurtz, H.J.; Goyal, S.

    1997-01-01

    A 9-mo-old female wolf (Canis lupus) in the Superior National Forest of Minnesota (USA) died from a canine parvovirus (CPV) infection. This is the first direct evidence that this infection effects free-ranging wild wolves.

  17. Spotted black snake (Pseudechis guttatus) envenomation in a maned wolf (Chrysocyon brachyurus).

    Science.gov (United States)

    Portas, Timothy J; Montali, Richard J

    2007-09-01

    Envenomation by a spotted black snake (Pseudechis guttatus), following multiple bites on the buccal mucosa of a captive maned wolf (Chrysocyon brachyurus), caused the animal's collapse, hemolysis, rhabdomyolysis, local tissue necrosis, hepatic and renal failure, and subsequent death. The wolf died despite intensive supportive care including antivenom administration, fluid support, and a blood transfusion. Gross necropsy findings included myocardial and intestinal hemorrhage, pulmonary congestion, hepatomegaly, and splenomegaly. Microscopic examination of formalin-fixed tissues demonstrated pulmonary and abdominal visceral hemorrhage, acute nephrosis with casts, multifocal hepatic necrosis, and splenic congestion.

  18. Cystinuria in a maned wolf.

    Science.gov (United States)

    Bush, M; Bovee, K C

    1978-11-01

    A renal calculus composed principally of the amino acid, cystine, was found in an 8-year-old male maned wolf (Chrysocyon brachyurus). Cystine crystals were found in the urine sediment. The renal clearance of 10 amino acids was abnormal, whereas reabsorption of others was normal. The renal clearance of cystine, lysine, ornithine, and arginine exceeded the filtered load. The renal tubular handling of glucose, phosphate, sodium, potassium, and uric acid was identical to that for the clinically normal dog. These findings indicated an isolated renal tubular defect for cystine and other amino acids.

  19. Structure formation in inhomogeneous Early Dark Energy models

    International Nuclear Information System (INIS)

    Batista, R.C.; Pace, F.

    2013-01-01

    We study the impact of Early Dark Energy fluctuations in the linear and non-linear regimes of structure formation. In these models the energy density of dark energy is non-negligible at high redshifts and the fluctuations in the dark energy component can have the same order of magnitude of dark matter fluctuations. Since two basic approximations usually taken in the standard scenario of quintessence models, that both dark energy density during the matter dominated period and dark energy fluctuations on small scales are negligible, are not valid in such models, we first study approximate analytical solutions for dark matter and dark energy perturbations in the linear regime. This study is helpful to find consistent initial conditions for the system of equations and to analytically understand the effects of Early Dark Energy and its fluctuations, which are also verified numerically. In the linear regime we compute the matter growth and variation of the gravitational potential associated with the Integrated Sachs-Wolf effect, showing that these observables present important modifications due to Early Dark Energy fluctuations, though making them more similar to the ΛCDM model. We also make use of the Spherical Collapse model to study the influence of Early Dark Energy fluctuations in the nonlinear regime of structure formation, especially on δ c parameter, and their contribution to the halo mass, which we show can be of the order of 10%. We finally compute how the number density of halos is modified in comparison to the ΛCDM model and address the problem of how to correct the mass function in order to take into account the contribution of clustered dark energy. We conclude that the inhomogeneous Early Dark Energy models are more similar to the ΛCDM model than its homogeneous counterparts

  20. Efficient EBE treatment of the dynamic far-field in non-linear FE soil-structure interaction analyses

    NARCIS (Netherlands)

    Crouch, R.S.; Bennett, T.

    2000-01-01

    This paper presents results and observations from the use of a rigorous method of treating the dynamic far-field as part of a non-linear FE analysis. The technique de-veloped by Wolf and Song (referred to as the Scaled Boundary Finite-Element Method) is incorporated into a 3-D time-domain analysis

  1. On x radiation of double systems containing Wolf-Rayet type stars

    International Nuclear Information System (INIS)

    Prilutskij, O.F.; Usov, V.V.

    1976-01-01

    It is shown that the close binary systems must be rather intensive sources of X radiation one or both components of which are young massive stars with strong outflow of matter from them (Wolf-Rayet type stars and OB supergiants). X-radiation of such binary systems is stimulated by gas heating behind the front of shock waves formed as a result of collision of gas outflowing from one component either with the second star surface or with its magnetosphere or with gas outflowing from the second star. The most possible candidates of X-ray sources among double Wolf-Rayet stars are γ 2 Vel and V 444 Cyg

  2. The Wolf-Rayet nebula NGC 3199 - an interstellar snow plough?

    Science.gov (United States)

    Dyson, J. E.; Ghanbari, J.

    1989-12-01

    The Wolf-Rayet nebula NGC 3199 has a highly asymmetric morphology, with a very bright hemisphere near the exciting star HD 89358 and a much fainter and more extended other hemisphere. This nebula is modeled in terms of the distorted bubble produced by a moving star blowing a strong stellar wind into a surrounding uniform interstellar medium; this model is fitted to the morphology and observed kinematic data. The exciting star appears to be moving at about 60 km/s into local interstellar gas of density of about 10/cu cm, and has a mass-loss rate of about 0.000027 solar mass/yr. This latter mass-loss rate is in excellent agreement with observed mass-loss rates from Wolf-Rayet stars.

  3. Optimal Homotopy Asymptotic Method for Solving the Linear Fredholm Integral Equations of the First Kind

    Directory of Open Access Journals (Sweden)

    Mohammad Almousa

    2013-01-01

    Full Text Available The aim of this study is to present the use of a semi analytical method called the optimal homotopy asymptotic method (OHAM for solving the linear Fredholm integral equations of the first kind. Three examples are discussed to show the ability of the method to solve the linear Fredholm integral equations of the first kind. The results indicated that the method is very effective and simple.

  4. Weak lensing in generalized gravity theories

    International Nuclear Information System (INIS)

    Acquaviva, Viviana; Baccigalupi, Carlo; Perrotta, Francesca

    2004-01-01

    We extend the theory of weak gravitational lensing to cosmologies with generalized gravity, described in the Lagrangian by a generic function depending on the Ricci scalar and a nonminimal coupled scalar field. We work out the generalized Poisson equations relating the dynamics of the fluctuating components to the two gauge-invariant scalar gravitational potentials, fixing the contributions from the modified background expansion and fluctuations. We show how the lensing equation gets modified by the cosmic expansion as well as by the presence of anisotropic stress, which is non-null at the linear level both in scalar-tensor gravity and in theories where the gravitational Lagrangian term features a nonminimal dependence on the Ricci scalar. Starting from the geodesic deviation, we derive the generalized expressions for the shear tensor and projected lensing potential, encoding the spacetime variation of the effective gravitational constant and isolating the contribution of the anisotropic stress, which introduces a correction due to the spatial correlation between the gravitational potentials. Finally, we work out the expressions of the lensing convergence power spectrum as well as the correlation between the lensing potential and the integrated Sachs-Wolfe effect affecting cosmic microwave background total intensity and polarization anisotropies. To illustrate phenomenologically the effects, we work out approximate expressions for the quantities above in extended quintessence scenarios where the scalar field coupled to gravity plays the role of the dark energy

  5. Red Wolf (Canis rufus Recovery: A Review with Suggestions for Future Research

    Directory of Open Access Journals (Sweden)

    Michael J. Chamberlain

    2013-08-01

    Full Text Available By the 1970s, government-supported eradication campaigns reduced red wolves to a remnant population of less than 100 individuals on the southern border of Texas and Louisiana. Restoration efforts in the region were deemed unpromising because of predator-control programs and hybridization with coyotes. The U.S. Fish and Wildlife Service (USFWS removed the last remaining red wolves from the wild and placed them in a captive-breeding program. In 1980, the USFWS declared red wolves extinct in the wild. During 1987, the USFWS, through the Red Wolf Recovery Program, reintroduced red wolves into northeastern North Carolina. Although restoration efforts have established a population of approximately 70–80 red wolves in the wild, issues of hybridization with coyotes, inbreeding, and human-caused mortality continue to hamper red wolf recovery. We explore these three challenges and, within each challenge, we illustrate how research can be used to resolve problems associated with red wolf-coyote interactions, effects of inbreeding, and demographic responses to human-caused mortality. We hope this illustrates the utility of research to advance restoration of red wolves.

  6. A Conceptual Analysis of Mass Loss and Frequency Redistribution in Wolf-Rayet Winds

    OpenAIRE

    Onifer, A. J.; Gayley, K. G.

    2007-01-01

    To better understand Wolf-Rayet stars as progenitors of gamma-ray bursts, an understanding of the effect metallicity has on Wolf-Rayet mass loss is needed. Using simple analytic models, we study the Mdot - Z relation of a WN star and compare the results to similar models. We find that Mdot roughly follows a power law in Z with index 0.88 from -2.5 < log Z/Z_sun < -1 and appears to flatten by log Z/Z_sun ~ -0.5.

  7. Wolf pack hunting strategy for automatic generation control of an islanding smart distribution network

    International Nuclear Information System (INIS)

    Xi, Lei; Zhang, Zeyu; Yang, Bo; Huang, Linni; Yu, Tao

    2016-01-01

    Highlights: • A mixed homogeneous and heterogeneous multi-agent based wolf pack hunting (WPH) method is proposed. • WPH can effectively handle the ever-increasing penetration of renewable energy in smart grid. • An AGC power dispatch, coordinated control, and electric power autonomy of an ISDN is achieved. - Abstract: As the conventional centralized automatic generation control (AGC) is inadequate to handle the ever-increasing penetration of renewable energy and the requirement of plug-and-play of smart grid, this paper proposes a mixed homogeneous and heterogeneous multi-agent based wolf pack hunting (WPH) strategy to achieve a fast AGC power dispatch, optimal coordinated control, and electric power autonomy of an islanding smart distribution network (ISDN). A virtual consensus variable is employed to deal with the topology variation resulted from the excess of power limits and to achieve the plug-and-play of AGC units. Then an integrated objective of frequency deviation and short-term economic dispatch is developed, such that all units can maintain an optimal operation in the presence of load disturbances. Four case studies are undertaken to an ISDN with various distributed generations and microgrids. Simulation results demonstrate that WPH has a greater robustness and a faster dynamic optimization than that of conventional approaches, which can increase the utilization rate of the renewable energy and effectively resolve the coordination and electric power autonomy of ISDN.

  8. New classical r-matrices from integrable non-linear sigma-models

    International Nuclear Information System (INIS)

    Laartz, J.; Bordemann, M.; Forger, M.; Schaper, U.

    1993-01-01

    Non-linear sigma models on Riemannian symmetric spaces constitute the most general class of classical non-linear sigma models which are known to be integrable. Using the current algebra structure of these models their canonical structure is analyzed and it is shown that their non-ultralocal fundamental Poisson bracket relation is governed by a field dependent non antisymmetric r-matrix obeying a dynamical Yang Baxter equation. The fundamental Poisson bracket relations and the r-matrix are derived explicitly and a new kind of algebra is found that is supposed to replace the classical Yang Baxter algebra governing the canonical structure of ultralocal models. (Author) 9 refs

  9. Cross-correlation of the cosmic microwave background with the 2MASS galaxy survey: Signatures of dark energy, hot gas, and point sources

    International Nuclear Information System (INIS)

    Afshordi, Niayesh; Loh, Yeong-Shang; Strauss, Michael A.

    2004-01-01

    We cross-correlate the cosmic microwave background temperature anisotropies observed by the Wilkinson Microwave Anisotropy Probe (WMAP) with the projected distribution of extended sources in the Two Micron All Sky Survey (2MASS). By modeling the theoretical expectation for this signal, we extract the signatures of dark energy [integrated Sachs-Wolfe effect (ISW)], hot gas [thermal Sunyaev-Zeldovich (SZ) effect], and microwave point sources in the cross-correlation. Our strongest signal is the thermal SZ, at the 3.1-3.7σ level, which is consistent with the theoretical prediction based on observations of x-ray clusters. We also see the ISW signal at the 2.5σ level, which is consistent with the expected value for the concordance ΛCDM cosmology, and is an independent signature of the presence of dark energy in the Universe. Finally, we see the signature of microwave point sources at the 2.7σ level

  10. Integrated all optical transmodulator circuits with non-linear gain elements and tunable optical fibers

    NARCIS (Netherlands)

    Kuindersma, P.I.; Leijtens, X.J.M.; Zantvoort, van J.H.C.; Waardt, de H.

    2012-01-01

    We characterize integrated InP circuits for high speed ‘all-optical’ signal processing. Single chip circuits act as optical transistors. Transmodulation is performed by non-linear gain sections. Integrated tunable filters give signal equalization in time domain.

  11. Gemini/GNIRS infrared spectroscopy of the Wolf-Rayet stellar wind in Cygnus X-3

    Science.gov (United States)

    Koljonen, K. I. I.; Maccarone, T. J.

    2017-12-01

    The microquasar Cygnus X-3 was observed several times with the Gemini North Infrared Spectrograph while the source was in the hard X-ray state. We describe the observed 1.0-2.4 μm spectra as arising from the stellar wind of the companion star and suggest its classification as a WN 4-6 Wolf-Rayet star. We attribute the orbital variations of the emission line profiles to the variations in the ionization structure of the stellar wind caused by the intense X-ray emission from the compact object. The strong variability observed in the line profiles will affect the mass function determination. We are unable to reproduce earlier results, from which the mass function for the Wolf-Rayet star was derived. Instead, we suggest that the system parameters are difficult to obtain from the infrared spectra. We find that the near-infrared continuum and the line spectra can be represented with non-LTE Wolf-Rayet atmosphere models if taking into account the effects arising from the peculiar ionization structure of the stellar wind in an approximative manner. From the representative models we infer the properties of the Wolf-Rayet star and discuss possible mass ranges for the binary components.

  12. THE PROPAGATION OF NEUTRINO-DRIVEN JETS IN WOLF-RAYET STARS

    Energy Technology Data Exchange (ETDEWEB)

    Nagakura, Hiroki, E-mail: hiroki@heap.phys.waseda.ac.jp [Yukawa Institute for Theoretical Physics, Kyoto University, Kyoto 606-8502, JapanAND (Japan); Advanced Research Institute for Science and Engineering, Waseda University, 3-4-1 Okubo, Shinjuku, Tokyo 169-8555 (Japan)

    2013-02-20

    We numerically investigate the jet propagation through a rotating collapsing Wolf-Rayet star with detailed central engine physics constructed based on the neutrino-driven collapsar model. The collapsing star determines the evolution of the mass accretion rate, black hole mass, and spin, all of which are important ingredients for determining the jet luminosity. We reveal that neutrino-driven jets in rapidly spinning Wolf-Rayet stars are capable of breaking out from the stellar envelope, while those propagating in slower rotating progenitors fail to break out due to insufficient kinetic power. For progenitor models with successful jet breakouts, the kinetic energy accumulated in the cocoon could be as large as {approx}10{sup 51} erg and might significantly contribute to the luminosity of the afterglow emission or to the kinetic energy of the accompanying supernova if nickel production takes place. We further analyze the post-breakout phase using a simple analytical prescription and conclude that the relativistic jet component could produce events with an isotropic luminosity L {sub p(iso)} {approx} 10{sup 52} erg s{sup -1} and isotropic energy E {sub j(iso)} {approx} 10{sup 54} erg. Our findings support the idea of rapidly rotating Wolf-Rayet stars as plausible progenitors of GRBs, while slowly rotational ones could be responsible for low-luminosity or failed GRBs.

  13. The Gender of Authorship: Heiner Müller and Christa Wolf

    Directory of Open Access Journals (Sweden)

    Helen Fehervary

    1980-09-01

    Full Text Available The relationship between sexuality and politics has always been an underlying assumption of the avant-garde. In recent East German avant-garde literature, the notion of authorship as production has become associated with technological rationality and the patriarchal socialist state. The ensuing crisis of the traditional male author has thus led necessarily to a radicalization of subjectivity and to the politics of gender. A comparison of two contemporary texts, one by a female author, one by a male, shows that the crisis of authorship assumes two distinctly different forms when differences in gender are taken into account. The East German authors Heiner Müller and Christa Wolf have exhibited remarkably similar literary and political developments. Two of their most recent texts, Mülller's Hamletmachine and Wolf's No Place. Nowhere , both address the problematic of traditional male authorship and the disintegration of a preconceived literary gender identity. Yet, these two texts exemplify very different assumptions about the relationship between authorship and the literary tradition. Müller's text suggests the imprisonment of the male author within a petrified system of tradition and images, and hence the necessity of deconstruction. Wolf's text manifests a process of creating a new form of female-identified authorship and the possibility of redefining the tradition of literature and its future.

  14. Three novel mutations in Iranian patients with Tay-Sachs disease.

    Science.gov (United States)

    Jamali, Solmaz; Eskandari, Nasim; Aryani, Omid; Salehpour, Shadab; Zaman, Talieh; Kamalidehghan, Behnam; Houshmand, Massoud

    2014-01-01

    Tay-Sachs disease (TSD), or GM2 gangliosidosis, is a lethal autosomal recessive neurodegenerative disorder, which is caused by a deficiency of beta-hexosaminidase A (HEXA), resulting in lysosomal accumulation of GM2 ganglioside. The aim of this study was to identify the TSD-causing mutations in an Iranian population. In this study, we examined 31 patients for TSD-causing mutations using PCR, followed by restriction enzyme digestion. Molecular genetics analysis of DNA from 23 patients of TSD revealed mutations that has been previously reported, including four-base duplications c.1274_1277dupTATC in exon 11 and IVS2+1G>A, deletion TTAGGCAAGGGC in exon 10 as well as a few novel mutations, including C331G, which altered Gln>Glu in HEXB, A>G, T>C, and p.R510X in exon 14, which predicted a termination codon or nonsense mutation. In conclusion, with the discovery of these novel mutations, the genotypic spectrum of Iranian patients with TSD disease has been extended and could facilitate definition of disease-related mutations.

  15. The Lurking Wolf

    DEFF Research Database (Denmark)

    Larsen, Janni Lisander; Jacobsen, Søren; Hall, Elisabeth

    of existential experiences over time in female patients suffering from Lupus. Method: Three 3 qualitative indept interviews with 15 women is planned during 1½ year. First and second round is performed, and third is planned during spring 2015. Interviews are guided by Van Manens life world existentials (time...... to withdraw their consent, and to choose time and place for the interviewing. Results: Interpretation on the existential meaning is in progress. Preliminary results document that the chaotic time of the diagnosis gradually changes over the years, leaving a mark on their existential life. Experiencing......THE LURKING OF THE WOLF- QUALITATIVE RESARCH OF EXISTENTIAL EXPERIENCES WITH LUPUS IN FEMALE PATIENTS. J. Lisander Larsen (1, 2), S. Jacobsen (2), E.O. C. Hall (1), R. Birkelund(3) (1) Department of Public Health, Aarhus University, Section for Nursing, Denmark. (2) University Hospital...

  16. Sarcocystis arctica (Apicomplexa: Sarcocystidae): ultrastructural description and its new host record, the Alaskan wolf (Canis lupus

    Science.gov (United States)

    Sarcocystis sarcocysts are common in muscles of herbivores but are rare in muscles of carnivores. Here, we report sarcocysts in muscle of an Alaskan wolf (Canis lupus) from Alaska, USA for the first time. Sarcocysts extracted from tongue of the wolf were up to 900 µm long, slender, and appeared to h...

  17. The Wolf-Rayet stars in 30 Doradus

    International Nuclear Information System (INIS)

    Melnick, J.

    1982-01-01

    The second brightest giant HII region in the sky is the 30 Doradus nebula in the LMC. This cluster contains many WR stars and may be one of the best objects where general ideas about the origin and evolution of WR stars can be tested. The author briefly describes observations of WR stars in 30 Doradus and discusses the implications for Wolf-Rayet evolutionary theories. (Auth.)

  18. Emission - line theoretical profiles for Wolf- Rayet stars with low-mass companions

    International Nuclear Information System (INIS)

    Antokhin, I.I.

    1986-01-01

    Profiles of the resonant line λ 765 A and the subordinate line λ 4058 of N4 have been calculated for a binary system medel consisting of the Wolf-Rayet star and the low-mass companion (possibly, a relativistic object) by means of Sobolev approximation. The equations of statistical equilibrium have been solved for the first 32 levels of N4. Two cases have been considered: 1) detached zone of N5 surrounding the Wolf-Rayet star and the companion; 2) common zone of N5. The criteria for detection of presence of a companion in line profile observations have been formulated

  19. Identification of two HEXA mutations causing infantile-onset Tay-Sachs disease in the Persian population.

    Science.gov (United States)

    Haghighi, Alireza; Rezazadeh, Jamileh; Shadmehri, Azam Ahmadi; Haghighi, Amirreza; Kornreich, Ruth; Desnick, Robert J

    2011-09-01

    The β-hexosaminidase A (HEXA) mutations in the first reported cases of infantile Tay-Sachs disease in the Persian population were identified in two unrelated consanguineous families. The clinical diagnoses of the affected infants were confirmed by their markedly deficient levels of HEXA activity in plasma or peripheral leukocytes. The specific causative mutation in each family was determined by sequencing the HEXA alleles in both sets of related parents. Two mutations were identified: c.1A>G (p.MIV), which obliterated the initiating methionine in codon 1, and c.1177C>T (p.R393X), which predicted a termination codon or nonsense mutation.

  20. The MAT/TOCO Measurement of the Angular Power Spectrum of the Cosmic Microwave Background at 30 and 40 GHz

    Science.gov (United States)

    Nolta, M. R.; Devlin, M. J.; Dorwart, W. B.; Miller, A. D.; Page, L. A.; Puchalla, J.; Torbet, E.; Tran, H. T.

    2003-11-01

    We present a measurement of the angular spectrum of the cosmic microwave background from l=26 to 225 from the 30 and 40 GHz channels of the MAT/TOCO experiment based on two seasons of observations. At comparable frequencies, the data extend to a lower l than the recent Very Small Array and DASI results. After accounting for known foreground emission in a self-consistent analysis, a rise from the Sachs-Wolfe plateau to a peak of δTl~80 μK near l~200 is observed.

  1. Largest global shark biomass found in the northern Galápagos Islands of Darwin and Wolf

    Directory of Open Access Journals (Sweden)

    Pelayo Salinas de León

    2016-05-01

    Full Text Available Overfishing has dramatically depleted sharks and other large predatory fishes worldwide except for a few remote and/or well-protected areas. The islands of Darwin and Wolf in the far north of the Galapagos Marine Reserve (GMR are known for their large shark abundance, making them a global scuba diving and conservation hotspot. Here we report quantitative estimates of fish abundance at Darwin and Wolf over two consecutive years using stereo-video surveys, which reveal the largest reef fish biomass ever reported (17.5 t ${\\mathrm{ha}}^{-1}$ ha − 1 on average, consisting largely of sharks. Despite this, the abundance of reef fishes around the GMR, such as groupers, has been severely reduced because of unsustainable fishing practices. Although Darwin and Wolf are within the GMR, they were not fully protected from fishing until March 2016. Given the ecological value and the economic importance of Darwin and Wolf for the dive tourism industry, the current protection should ensure the long-term conservation of this hotspot of unique global value.

  2. WOLF RAYETS: INTERFEROMETRY OF HOT DUST

    OpenAIRE

    J. Rajagopal

    2010-01-01

    Las Wolf Rayets (WRs) son estrellas masivas calientes en sus ltimas etapas evolutivas. Son indicadores prominentes de la formaci n de estrellas de gran masa y sus vientos masivos tienen in uencia signi cativa en el medio interestelar. Es sabido que un peque o pero signi cativo n mero de estas estrellas en la galaxia producen copiosas cantidades de polvo. Dado el hostil ambiente circunestelar, esto plantea preguntas interesantes. Las observaciones interferom tricas y de abertura de m scara in...

  3. Painlevйe analysis and integrability of two-coupled non-linear ...

    Indian Academy of Sciences (India)

    the Painlevйe property. In this case the system is expected to be integrable. In recent years more attention is paid to the study of coupled non-linear oscilla- ... Painlevйe analysis. To be self-contained, in §2 we briefly outline the salient features.

  4. Theories for the winds from Wolf Rayet stars

    International Nuclear Information System (INIS)

    Cassinelli, J.P.

    1982-01-01

    The massive and fast winds of Wolf Rayet stars present a serious momentum problem for the line-driven wind theories that are commonly used to explain the fast winds of early type stars. It is perhaps possible for the winds to be driven by lines, if multiple scattering occurs and if there are a sufficient number of lines in the spectrum so that large fraction of the continuum is blocked by line opacity in the winds. Several other mechanisms are discussed, in particular two that rely on strong magnetic fields: a) Alfven wave driven wind models like those recently developed by Hartmann and MacGregor for late type supergiants and b) the ''Fast Magnetic Rotator'' model that was developed by Belcher and MacGregor for the winds from pre-main sequence stars. In either model, large magnetic fields (approximately equal to 10 4 gauss) are required to drive the massive and fast winds of Wolf Rayet stars. Smaller fields might be possible if the multiple scattering line radiation force can be relied on to provide a final acceleration to terminal velocity. (Auth.)

  5. The Lie-Poisson structure of integrable classical non-linear sigma models

    International Nuclear Information System (INIS)

    Bordemann, M.; Forger, M.; Schaeper, U.; Laartz, J.

    1993-01-01

    The canonical structure of classical non-linear sigma models on Riemannian symmetric spaces, which constitute the most general class of classical non-linear sigma models known to be integrable, is shown to be governed by a fundamental Poisson bracket relation that fits into the r-s-matrix formalism for non-ultralocal integrable models first discussed by Maillet. The matrices r and s are computed explicitly and, being field dependent, satisfy fundamental Poisson bracket relations of their own, which can be expressed in terms of a new numerical matrix c. It is proposed that all these Poisson brackets taken together are representation conditions for a new kind of algebra which, for this class of models, replaces the classical Yang-Baxter algebra governing the canonical structure of ultralocal models. The Poisson brackets for the transition matrices are also computed, and the notorious regularization problem associated with the definition of the Poisson brackets for the monodromy matrices is discussed. (orig.)

  6. Finding the Wolves in Sheeps Clothing: Ways to Distinguish and Deter Lone-Wolf Terrorists

    Science.gov (United States)

    2015-03-01

    Mark Hamm, “Lone-wolf Terrorism in America: Forging a New Way of Looking at an Old Problem,” YouTube video, 7:32, posted by the National Institute...that factors that influence most lone-wolf terrorists include, “to varying degrees and in variable combinations: personal aversion or depression ...Russia.”97 Also discovered after the bombings, “Tamerlan had a YouTube account that he used to post and watch violent Islamic extremist videos starting

  7. How massive the Wolf-Rayet stars are

    International Nuclear Information System (INIS)

    Niemela, V.S.

    1981-01-01

    If the Wolf-Rayet stars are produced by the evolution of massive stars with mass loss (Paczynski 1967, Conti 1976) from O stars to WN stars and thereafter to WC stars, then we may expect to observe a correlation of decreasing mean masses in the same sense as the evolution. Information about the masses of WR stars are obtained from studies of binary systems with WR components. (Auth.)

  8. Wolf-Rayet stars featured in emission-line galaxies

    International Nuclear Information System (INIS)

    Kunth, D.

    1982-01-01

    In the galaxy Tololo 3 (NGC 3125) recent observations by the author and Sargent (1981) have revealed the presence of an unusual strong and broad He II 4686 emission. The origin of this line together with some nitrogen lines (e.g. N V 4620 and N III 4638) is attributed to Wolf-Rayet stars, mostly of WN types. (Auth.)

  9. Traumatic glenohumeral bone defects and their relationship to failure of arthroscopic Bankart repairs: significance of the inverted-pear glenoid and the humeral engaging Hill-Sachs lesion.

    Science.gov (United States)

    Burkhart, S S; De Beer, J F

    2000-10-01

    Our goal was to analyze the results of 194 consecutive arthroscopic Bankart repairs (performed by 2 surgeons with an identical suture anchor technique) in order to identify specific factors related to recurrence of instability. Case series. We analyzed 194 consecutive arthroscopic Bankart repairs by suture anchor technique performed for traumatic anterior-inferior instability. The average follow-up was 27 months (range, 14 to 79 months). There were 101 contact athletes (96 South African rugby players and 5 American football players). We identified significant bone defects on either the humerus or the glenoid as (1) "inverted-pear" glenoid, in which the normally pear-shaped glenoid had lost enough anterior-inferior bone to assume the shape of an inverted pear; or (2) "engaging" Hill-Sachs lesion of the humerus, in which the orientation of the Hill-Sachs lesion was such that it engaged the anterior glenoid with the shoulder in abduction and external rotation. There were 21 recurrent dislocations and subluxations (14 dislocations, 7 subluxations). Of those 21 shoulders with recurrent instability, 14 had significant bone defects (3 engaging Hill-Sachs and 11 inverted-pear Bankart lesions). For the group of patients without significant bone defects (173 shoulders), there were 7 recurrences (4% recurrence rate). For the group with significant bone defects (21 patients), there were 14 recurrences (67% recurrence rate). For contact athletes without significant bone defects, there was a 6.5% recurrence rate, whereas for contact athletes with significant bone defects, there was an 89% recurrence rate. (1) Arthroscopic Bankart repairs give results equal to open Bankart repairs if there are no significant structural bone deficits (engaging Hill-Sachs or inverted-pear Bankart lesions). (2) Patients with significant bone deficits as defined in this study are not candidates for arthroscopic Bankart repair. (3) Contact athletes without structural bone deficits may be treated by

  10. Key Issues and Research Agendas in Lone Wolf Terrorism

    NARCIS (Netherlands)

    Spaaij, R.; Hamm, M.S.

    2015-01-01

    This article builds on recent contributions to the academic literature on lone wolf terrorism to critically examine key issues that are germane to the current state of play in this field of study. It finds that, overall, the recent academic literature still suffers from considerable problems

  11. Chemical composition and origin of the Wolf-Rayet ring Nebula NGC 6888

    International Nuclear Information System (INIS)

    Kwitter, K.B.

    1981-01-01

    We have obtained spectrophotometric observations of NGC 6888, a ring nebula surrounding the Population I Wolf-Rayet star HD 192163, in order to determine the physical conditions and chemical abundances in this object. We conclude that NGC 6888 is enriched in nitrogen and helium by factors of about 9 and 2, respectively, compared with the Orion Nebula. This enrichment is a result of contamination of the ambient abundances by the nigrogen-rich and helium-rich wind from the central Wolf-rayet star. We have interpreted NGC 6888 as an interstellar bubble, according to recent theory, and calculate an approximate age for the nebula of 18,000 years. The fraction of nebular mass contributed by the central star can be estimated from published stellar abundances; we calculate that the stellar wind has provided approx.10% of the observed nebular mass. If this nebula is representative, then the total mass contributed to a ring nebula by stellar wind is small (< or approx. =1M/sub sun/). This suggests that mass loss from stars in the Wolf-Rayet phase does not play a significant role in the nitrogen and helium enrichment of the interstellar medium

  12. Treatment of fibrosarcoma in a maned wolf (Chrysocyon brachyurus) by rostral maxillectomy.

    Science.gov (United States)

    McNulty, E E; Gilson, S D; Houser, B S; Ouse, A

    2000-09-01

    A 12-yr-old captive intact male maned wolf (Chrysocyon brachyurus) was diagnosed with a fibrosarcoma of the incisive bones. The mass was excised by rostral maxillectomy, and the wolf remained normal and on display with good function and cosmetics for 7 mo. Subsequently, it became weak, ataxic, and dyspneic and was euthanatized. At necropsy, there was a small regrowth of the maxillary tumor, a metastatic mediastinal mass, and multiple metastatic lung masses, suggesting that oral fibrosarcoma in maned wolves behaves similarly to oral fibrosarcoma in domestic canines. Aggressive surgical treatment of oral fibrosarcoma in this species can achieve good functional and cosmetic results.

  13. Generation of HEXA-deficient hiPSCs from fibroblasts of a Tay-Sachs disease patient

    Directory of Open Access Journals (Sweden)

    Zhong Liu

    2016-09-01

    Full Text Available Human iPSC line TSD-01-hiPSC was generated from fibroblasts of a patient with infantile Tay-Sachs disease (TSD. The patient is compound heterozygous at the HEXA gene by carrying a 1278insTATC allele and an IVS12+1G>C allele. STEMCCA lentivirus, which expresses OCT4, SOX2, KLF4, and c-MYC from a polycistronic transcript, were used for reprogramming. TSD-01-hiPSC express pluripotency markers such as OCT4, SOX2, NANOG, Tra-1-60, and alkaline phosphatase, and can differentiate into tissues from all the three embryonic germ layers. This TSD patient-derived hiPSC line may serve as a valuable in vitro tool for disease modeling and drug test.

  14. Gray wolf exposure to emerging vector-borne diseases in Wisconsin with comparison to domestic dogs and humans

    Science.gov (United States)

    Jara, Rocio F.; Wydeven, Adrian P.; Samuel, Michael D.

    2016-01-01

    World-wide concern over emerging vector-borne diseases has increased in recent years for both animal and human health. In the United Sates, concern about vector-borne diseases in canines has focused on Lyme disease, anaplasmosis, ehrlichiosis, and heartworm which infect domestic and wild canids. Of these diseases, Lyme and anaplasmosis are also frequently diagnosed in humans. Gray wolves (Canis lupus) recolonized Wisconsin in the 1970s, and we evaluated their temporal and geographic patterns of exposure to these four vector-borne diseases in Wisconsin as the population expanded between 1985 and 2011. A high proportion of the Wisconsin wolves were exposed to the agents that cause Lyme (65.6%) and anaplasma (47.7%), and a smaller proportion to ehrlichiosis (5.7%) and infected with heartworm (9.2%). Wolf exposure to tick borne diseases was consistently higher in older animals. Wolf exposure was markedly higher than domestic dog (Canis familiaris) exposure for all 4 disease agents during 2001–2013. We found a cluster of wolf exposure to Borrelia burgdorferi in northwestern Wisconsin, which overlaps human and domestic dog clusters for the same pathogen. In addition, wolf exposure to Lyme disease in Wisconsin has increased, corresponding with the increasing human incidence of Lyme disease in a similar time period. Despite generally high prevalence of exposure none of these diseases appear to have slowed the growth of the Wisconsin wolf population.

  15. Genome-wide analysis of SNPs is consistent with no domestic dog ancestry in the endangered Mexican Wolf (Canis lupus baileyi)

    Science.gov (United States)

    Fitak, Robert R.; Rinkevich, Sarah E.; Culver, Melanie

    2018-01-01

    The Mexican gray wolf (Canis lupus baileyi) was historically distributed throughout the southwestern United States and northern Mexico. Extensive predator removal campaigns during the early 20th century, however, resulted in its eventual extirpation by the mid 1980s. At this time, the Mexican wolf existed only in 3 separate captive lineages (McBride, Ghost Ranch, and Aragón) descended from 3, 2, and 2 founders, respectively. These lineages were merged in 1995 to increase the available genetic variation, and Mexican wolves were reintroduced into Arizona and New Mexico in 1998. Despite the ongoing management of the Mexican wolf population, it has been suggested that a proportion of the Mexican wolf ancestry may be recently derived from hybridization with domestic dogs. In this study, we genotyped 87 Mexican wolves, including individuals from all 3 captive lineages and cross-lineage wolves, for more than 172000 single nucleotide polymorphisms. We identified levels of genetic variation consistent with the pedigree record and effects of genetic rescue. To identify the potential to detect hybridization with domestic dogs, we compared our Mexican wolf genotypes with those from studies of domestic dogs and other gray wolves. The proportion of Mexican wolf ancestry assigned to domestic dogs was only between 0.06% (SD 0.23%) and 7.8% (SD 1.0%) for global and local ancestry estimates, respectively; and was consistent with simulated levels of incomplete lineage sorting. Overall, our results suggested that Mexican wolves lack biologically significant ancestry with dogs and have useful implications for the conservation and management of this endangered wolf subspecies.

  16. Characterization of the Wolf 1061 Planetary System

    Energy Technology Data Exchange (ETDEWEB)

    Kane, Stephen R.; Waters, Miranda A. [Department of Physics and Astronomy, San Francisco State University, 1600 Holloway Avenue, San Francisco, CA 94132 (United States); Von Braun, Kaspar [Lowell Observatory, 1400 West Mars Hill Road, Flagstaff, AZ 86001 (United States); Henry, Gregory W. [Center of Excellence in Information Systems, Tennessee State University, 3500 John A. Merritt Blvd., Box 9501, Nashville, TN 37209 (United States); Boyajian, Tabetha S. [Department of Physics and Astronomy, Louisiana State University, Baton Rouge, LA 70803 (United States); Mann, Andrew W., E-mail: skane@sfsu.edu [Department of Astronomy, University of Texas at Austin, Austin, TX 78712 (United States)

    2017-02-01

    A critical component of exoplanetary studies is an exhaustive characterization of the host star, from which the planetary properties are frequently derived. Of particular value are the radius, temperature, and luminosity, which are key stellar parameters for studies of transit and habitability science. Here we present the results of new observations of Wolf 1061, known to host three super-Earths. Our observations from the Center for High Angular Resolution Astronomy interferometric array provide a direct stellar radius measurement of 0.3207±0.0088 R{sub ⊙}, from which we calculate the effective temperature and luminosity using spectral energy distribution models. We obtained 7 yr of precise, automated photometry that reveals the correct stellar rotation period of 89.3±1.8 days, finds no evidence of photometric transits, and confirms that the radial velocity signals are not due to stellar activity. Finally, our stellar properties are used to calculate the extent of the Habitable Zone (HZ) for the Wolf 1061 system, for which the optimistic boundaries are 0.09–0.23 au. Our simulations of the planetary orbital dynamics show that the eccentricity of the HZ planet oscillates to values as high as ∼0.15 as it exchanges angular momentum with the other planets in the system.

  17. Population genomics of the inbred Scandinavian wolf

    OpenAIRE

    Hagenblad, Jenny; Olsson, Maria; Parker, Heidi G.; Ostrander, Elaine A.; Ellegren, Hans

    2009-01-01

    The Scandinavian wolf population represents one of the genetically most well characterized examples of a severely bottlenecked natural population (with only two founders), and of how the addition of new genetic material (one immigrant) can at least temporarily provide a “genetic rescue”. However, inbreeding depression has been observed in this population and in the absence of additional immigrants, its long-term viability is questioned. To study the effects of inbreeding and selection on geno...

  18. Habitat suitability and movement corridors of grey wolf (Canis lupus) in Northern Pakistan

    Science.gov (United States)

    Kabir, Muhammad; Hameed, Shoaib; Ali, Hussain; Bosso, Luciano; Din, Jaffar Ud; Bischof, Richard; Redpath, Steve

    2017-01-01

    Habitat suitability models are useful to understand species distribution and to guide management and conservation strategies. The grey wolf (Canis lupus) has been extirpated from most of its historic range in Pakistan primarily due to its impact on livestock and livelihoods. We used non-invasive survey data from camera traps and genetic sampling to develop a habitat suitability model for C. lupus in northern Pakistan and to explore the extent of connectivity among populations. We detected suitable habitat of grey wolf using a maximum entropy approach (Maxent ver. 3.4.0) and identified suitable movement corridors using the Circuitscape 4.0 tool. Our model showed high levels of predictive performances, as seen from the values of area under curve (0.971±0.002) and true skill statistics (0.886±0.021). The main predictors for habitat suitability for C. lupus were distances to road, mean temperature of the wettest quarter and distance to river. The model predicted ca. 23,129 km2 of suitable areas for wolf in Pakistan, with much of suitable habitat in remote and inaccessible areas that appeared to be well connected through vulnerable movement corridors. These movement corridors suggest that potentially the wolf range can expand in Pakistan’s Northern Areas. However, managing protected areas with stringent restrictions is challenging in northern Pakistan, in part due to heavy dependence of people on natural resources. The habitat suitability map provided by this study can inform future management strategies by helping authorities to identify key conservation areas. PMID:29121089

  19. Treatment of a forelimb fracture and rehabilitation of a free-ranging Iberian Wolf (Canis lupus signatus

    Directory of Open Access Journals (Sweden)

    Filipe C. Silva

    2016-05-01

    Full Text Available Abstract: The surgical treatment of an exposed compounded comminuted fracture of the right radius and ulna in a free-ranging adult female Iberian Wolf (Canis lupus signatus with an osteosynthesis plate and screws and subsequent post-operative care are described. The evolution of the fracture healing was very similar to those expected in a dog of the same size. The prompt surgical intervention and a proper housing, feeding and wound management adapted to a free-ranging wolf, in view to reduce manipulation and post-operative complications, allowed the subsequent rehabilitation and release of the animal. After 10th post-operative weeks the wolf was fitted with a Global Positioning System (GPS for wildlife tracking collar and released in the same area where it has been caught. GPS telemetry data showed that the animal covered increasingly large distances confirming a complete functionality of the right thoracic limb and its successfully return to the wild. This report could constitute the first detailed report of a long bone fracture treatment in a free-ranging wolf and its successfully rehabilitation, release and adaptation to the wild.

  20. AHP 6: The Brag 'go Wolf Begging Ritual

    Directory of Open Access Journals (Sweden)

    Mgon po tshe ring

    2010-06-01

    Full Text Available A ritual performed in 1999 in Dge rtse (Genzhi 更知 Township, Brag 'go (Luhuo 炉霍 County, Dkar mdzes (Ganzi 甘孜 Tibetan Autonomous Prefecture, Sichuan (四川 Province is described. The ritual involved a man, his son, and his nephew taking a wolf skin, visiting nine villages, and asking for donations to appease the 'owner of the wolves'.

  1. Methods of measurement of integral and differential linearity distortions of spectrometry sets

    International Nuclear Information System (INIS)

    Fuan, Jacques; Grimont, Bernard; Marin, Roland; Richard, Jean-Pierre

    1969-05-01

    The objective of this document is to describe different measurement methods, and more particularly to present a software for the processing of obtained results in order to avoid interpretation by the investigator. In a first part, the authors define the parameters of integral and differential linearity, outlines their importance in measurements performed by spectrometry, and describe the use of these parameters. In the second part, they propose various methods of measurement of these linearity parameters, report experimental applications of these methods and compare the obtained results

  2. Thrust Augmentation by Airframe-Integrated Linear-Spike Nozzle Concept for High-Speed Aircraft

    Directory of Open Access Journals (Sweden)

    Hidemi Takahashi

    2018-02-01

    Full Text Available The airframe-integrated linear-spike nozzle concept applied to an external nozzle for high-speed aircraft was evaluated with regard to the thrust augmentation capability and the trim balance. The main focus was on the vehicle aftbody. The baseline airframe geometry was first premised to be a hypersonic waverider design. The baseline aftbody case had an external nozzle comprised of a simple divergent nozzle and was hypothetically replaced with linear-spike external nozzle configurations. Performance evaluation was mainly conducted by considering the nozzle thrust generated by the pressure distribution on the external nozzle surface at the aftbody portion calculated by computer simulation at a given cruise condition with zero angle of attack. The thrust performance showed that the proposed linear-spike external nozzle concept was beneficial in thrust enhancement compared to the baseline geometry because the design of the proposed concept had a compression wall for the exhaust flow, which resulted in increasing the wall pressure. The configuration with the boattail and the angled inner nozzle exhibited further improvement in thrust performance. The trim balance evaluation showed that the aerodynamic center location appeared as acceptable. Thus, benefits were obtained by employing the airframe-integrated linear-spike external nozzle concept.

  3. Wolf-Rayet nebulae

    International Nuclear Information System (INIS)

    Chu, You-Hua

    2016-01-01

    Since the discovery of nebulae around Wolf-Rayet (WR) stars in the 1960s, it has been established that WR stars are massive stars at advanced evolutionary stages and that their surrounding nebulae result from the interactions between the stellar mass loss and the ambient interstellar medium. Surveys of WR nebulae have been made in the Galaxy, Magellanic Clouds, and other nearby galaxies in the Local Group. Some WR nebulae exhibit He II λ4686 line emission, indicating stellar effective temperatures of 90 — 100 x 10 3 K. The shocked fast stellar winds from WR nebulae have been detected in soft X-rays, but theoretical models have not been able to reproduce the observed X-ray spectral properties. Elemental abundances of WR nebulae consisting of synthesized stellar material can constrain stellar evolution models, but high-dispersion spectra are needed to kinematically separate the expanding shell of a WR nebula and the background interstellar medium for accurate abundance analyses. (paper)

  4. Constraints on gamma-ray burst and supernova progenitors through circumstellar absorption lines : II. Post-LBV Wolf-Rayet stars

    NARCIS (Netherlands)

    Marle, A.J.; Langer, N.; Garcia-Segura, G.

    2007-01-01

    Van Marle et al. (2005) showed that circumstellar absorption lines in early Type Ib/c supernova and gamma-ray burst afterglow spectra may reveal the progenitor evolution of the exploding Wolf-Rayet star. While the quoted paper deals with Wolf-Rayet stars which evolved through a red supergiant stage,

  5. 77 FR 55529 - Endangered and Threatened Wildlife and Plants; Removal of the Gray Wolf in Wyoming From the...

    Science.gov (United States)

    2012-09-10

    ... background information is also available online at http://www.fws.gov/mountain-prairie/species/mammals/wolf... allowing the Wyoming Game and Fish Commission (WGFC) to diminish Wyoming's Wolf Trophy Game Management Area... minimum levels. In early 2011, we began discussions with Wyoming seeking to develop a strategy to address...

  6. Some problems on non-linear semigroups and the blow-up of integral solutions

    International Nuclear Information System (INIS)

    Pavel, N.H.

    1983-07-01

    After some introductory remarks, this highly mathematical document considers a unifying approach in the theory of non-linear semigroups. Then a brief survey is given on blow-up of mild solutions from the semilinear case. Finally, the global behavior of solutions to non-linear evolution equations is addressed; it is found that classical results on the behavior of the maximal solution u as t up-arrow tsub(max) hold also for integral solutions

  7. Fear or food ? abundance of red fox in relation to occurrence of lynx and wolf

    OpenAIRE

    Wikenros, Camilla; Aronsson, Malin; Liberg, Olof; Jarnemo, Anders; Hansson, Jessica; Wallgren, M?rtha; Sand, H?kan; Bergstr?m, Roger

    2017-01-01

    Apex predators may affect mesopredators through intraguild predation and/or supply of carrion from their prey, causing a trade-off between avoidance and attractiveness. We used wildlife triangle snow-tracking data to investigate the abundance of red fox (Vulpes vulpes) in relation to lynx (Lynx lynx) and wolf (Canis lupus) occurrence as well as land composition and vole (Microtus spp.)?density. Data from the Swedish wolf-monitoring system and VHF/GPS-collared wolves were used to study the eff...

  8. Gray Wolf Exposure to Emerging Vector-Borne Diseases in Wisconsin with Comparison to Domestic Dogs and Humans.

    Directory of Open Access Journals (Sweden)

    Rocio F Jara

    Full Text Available World-wide concern over emerging vector-borne diseases has increased in recent years for both animal and human health. In the United Sates, concern about vector-borne diseases in canines has focused on Lyme disease, anaplasmosis, ehrlichiosis, and heartworm which infect domestic and wild canids. Of these diseases, Lyme and anaplasmosis are also frequently diagnosed in humans. Gray wolves (Canis lupus recolonized Wisconsin in the 1970s, and we evaluated their temporal and geographic patterns of exposure to these four vector-borne diseases in Wisconsin as the population expanded between 1985 and 2011. A high proportion of the Wisconsin wolves were exposed to the agents that cause Lyme (65.6% and anaplasma (47.7%, and a smaller proportion to ehrlichiosis (5.7% and infected with heartworm (9.2%. Wolf exposure to tick borne diseases was consistently higher in older animals. Wolf exposure was markedly higher than domestic dog (Canis familiaris exposure for all 4 disease agents during 2001-2013. We found a cluster of wolf exposure to Borrelia burgdorferi in northwestern Wisconsin, which overlaps human and domestic dog clusters for the same pathogen. In addition, wolf exposure to Lyme disease in Wisconsin has increased, corresponding with the increasing human incidence of Lyme disease in a similar time period. Despite generally high prevalence of exposure none of these diseases appear to have slowed the growth of the Wisconsin wolf population.

  9. Effective Theory of Dark Energy at Redshift Survey Scales

    CERN Document Server

    Gleyzes, Jérôme; Mancarella, Michele; Vernizzi, Filippo

    2016-01-01

    We explore the phenomenological consequences of general late-time modifications of gravity in the quasi-static approximation, in the case where cold dark matter is non-minimally coupled to the gravitational sector. Assuming spectroscopic and photometric surveys with configuration parameters similar to those of the Euclid mission, we derive constraints on our effective description from three observables: the galaxy power spectrum in redshift space, tomographic weak-lensing shear power spectrum and the correlation spectrum between the integrated Sachs-Wolfe effect and the galaxy distribution. In particular, with $\\Lambda$CDM as fiducial model and a specific choice for the time dependence of our effective functions, we perform a Fisher matrix analysis and find that the unmarginalized $68\\%$ CL errors on the parameters describing the modifications of gravity are of order $\\sigma\\sim10^{-2}$--$10^{-3}$. We also consider two other fiducial models. A nonminimal coupling of CDM enhances the effects of modified gravit...

  10. Use of linkage mapping and centrality analysis across habitat gradients to conserve connectivity of gray wolf populations in western North America.

    Science.gov (United States)

    Carroll, Carlos; McRae, Brad H; Brookes, Allen

    2012-02-01

    Centrality metrics evaluate paths between all possible pairwise combinations of sites on a landscape to rank the contribution of each site to facilitating ecological flows across the network of sites. Computational advances now allow application of centrality metrics to landscapes represented as continuous gradients of habitat quality. This avoids the binary classification of landscapes into patch and matrix required by patch-based graph analyses of connectivity. It also avoids the focus on delineating paths between individual pairs of core areas characteristic of most corridor- or linkage-mapping methods of connectivity analysis. Conservation of regional habitat connectivity has the potential to facilitate recovery of the gray wolf (Canis lupus), a species currently recolonizing portions of its historic range in the western United States. We applied 3 contrasting linkage-mapping methods (shortest path, current flow, and minimum-cost-maximum-flow) to spatial data representing wolf habitat to analyze connectivity between wolf populations in central Idaho and Yellowstone National Park (Wyoming). We then applied 3 analogous betweenness centrality metrics to analyze connectivity of wolf habitat throughout the northwestern United States and southwestern Canada to determine where it might be possible to facilitate range expansion and interpopulation dispersal. We developed software to facilitate application of centrality metrics. Shortest-path betweenness centrality identified a minimal network of linkages analogous to those identified by least-cost-path corridor mapping. Current flow and minimum-cost-maximum-flow betweenness centrality identified diffuse networks that included alternative linkages, which will allow greater flexibility in planning. Minimum-cost-maximum-flow betweenness centrality, by integrating both land cost and habitat capacity, allows connectivity to be considered within planning processes that seek to maximize species protection at minimum cost

  11. Zosteriform impetigo: Wolf's isotopic response in a cutaneous immunocompromised district.

    Science.gov (United States)

    Cohen, Philip R

    2015-07-01

    Impetigo can result from Staphylococcus aureus (S. aureus). Wolf's isotopic response is the occurrence of a new cutaneous disorder at the site of a previously healed disease. A cutaneous immunocompromised district is an area of skin that is more vulnerable than the rest of the individual's body. To describe a man with impetigo localized to a unilateral dermatome and review the clinical features of other patients with zosteriform Staphylococcus aureus cutaneous infection. PubMed was used to search the following terms, separately and in combination: cutaneous, dermatome, dermatomal, district, herpes, immunocompromised, impetigo, infection, isotopic, response, skin, staphylococcal, Staphylococcus aureus, Wolf, zoster, zosteriform. All papers were reviewed and relevant manuscripts, along with their reference citations, were evaluated. Crusted, eroded and intact, erythematous papules and nodules acutely presented localized to the mandibular branch of the left trigeminal nerve on the face of a 66-year-old man; he did not recall a prior episode of varicella-zoster virus infection in that area. A bacterial culture isolated methicillin-susceptible S. aureus. Viral cultures and direct fluorescent absorption studies were negative for herpes simplex and herpes zoster virus. All of the lesions resolved after oral treatment with cefdinir. Impetigo and/or furunculosis in a zosteriform distribution have also been described in 3 additional patients. The bacterial culture showed either methicillin-susceptible or methicillin-resistant S. aureus; the skin infection resolved after treatment with oral antibiotics; however one man experienced 2 recurrences in the same area. Zosteriform cutaneous staphylococcal impetigo may be an example of Wolf's isotopic response in a cutaneous immunocompromised district.

  12. Generation of HEXA-deficient hiPSCs from fibroblasts of a Tay-Sachs disease patient.

    Science.gov (United States)

    Liu, Zhong; Zhao, Rui

    2016-09-01

    Human iPSC line TSD-01-hiPSC was generated from fibroblasts of a patient with infantile Tay-Sachs disease (TSD). The patient is compound heterozygous at the HEXA gene by carrying a 1278insTATC allele and an IVS12+1G>C allele. STEMCCA lentivirus, which expresses OCT4, SOX2, KLF4, and c-MYC from a polycistronic transcript, were used for reprogramming. TSD-01-hiPSC express pluripotency markers such as OCT4, SOX2, NANOG, Tra-1-60, and alkaline phosphatase, and can differentiate into tissues from all the three embryonic germ layers. This TSD patient-derived hiPSC line may serve as a valuable in vitro tool for disease modeling and drug test. Copyright © 2016 The Authors. Published by Elsevier B.V. All rights reserved.

  13. Late-onset Tay-Sachs disease: the spectrum of peripheral neuropathy in 30 affected patients.

    Science.gov (United States)

    Shapiro, Barbara E; Logigian, Eric L; Kolodny, Edwin H; Pastores, Gregory M

    2008-08-01

    Late-onset Tay-Sachs (LOTS) disease is a chronic, progressive, lysosomal storage disorder caused by a partial deficiency of beta-hexosaminidase A (HEXA) activity. Deficient levels of HEXA result in the intracellular accumulation of GM2-ganglioside, resulting in toxicity to nerve cells. Clinical manifestations primarily involve the central nervous system (CNS) and lower motor neurons, and include ataxia, weakness, spasticity, dysarthria, dysphagia, dystonia, seizures, psychosis, mania, depression, and cognitive decline. The prevalence of peripheral nervous system (PNS) involvement in LOTS has not been well documented, but it has traditionally been thought to be very low. We examined a cohort of 30 patients with LOTS who underwent clinical and electrophysiologic examination, and found evidence of a predominantly axon loss polyneuropathy affecting distal nerve segments in the lower and upper extremities in eight patients (27%).

  14. Wolf presence and increased willow consumption by Yellowstone elk: implications for trophic cascades.

    Science.gov (United States)

    Creel, Scott; Christianson, David

    2009-09-01

    Recent increases in the height and growth ring width of willow (Salix spp.) and other woody plants in the Greater Yellowstone Ecosystem (GYE) have been attributed to a behaviorally mediated trophic cascade from wolves (Canis lupus) to elk (Cervus elaphus) to willows. This hypothesis predicts that individual elk consume less willow in response to the presence of wolves, but this prediction has not been directly tested with data from elk. We collected 727 fecal samples from elk in the Gallatin Canyon portion of the GYE over three winters and used microhistological methods to quantify the proportion of willow in each sample. We then tested the effect of wolf presence on willow consumption by elk, controlling for the effects of snow conditions, sex, and habitat type. During the period of study, 8-17 wolves occupied the study area, and wolves were locally present on 49% of 260 sampling days, stratified at two-week intervals across three drainages. Over the three years combined, willow consumption was related to snow conditions, wolf presence, and a wolf X sex interaction. As expected, willow consumption increased with deeper and less penetrable snow, and this effect was strong. Contrary to expectation, willow consumption increased in the presence of wolves. As with other aspects of antipredator behavior, wolves had different effects on willow consumption by males and females. Finally, we aggregated the data to estimate winter-long mean willow consumption within each drainage; at this broader scale, willow consumption again increased as predation risk increased. In summary, willow consumption was more strongly affected by snow conditions than by the presence of wolves. Interactions between elk and willow were affected by wolves, but not as predicted by the hypothesis that wolf presence favors willow release through a reduction in the selection of willow by individual elk. If a trophic cascade is operating, our results suggest that a decline in the size of the elk

  15. An integrated model of water resources optimization allocation based on projection pursuit model - Grey wolf optimization method in a transboundary river basin

    Science.gov (United States)

    Yu, Sen; Lu, Hongwei

    2018-04-01

    Under the effects of global change, water crisis ranks as the top global risk in the future decade, and water conflict in transboundary river basins as well as the geostrategic competition led by it is most concerned. This study presents an innovative integrated PPMGWO model of water resources optimization allocation in a transboundary river basin, which is integrated through the projection pursuit model (PPM) and Grey wolf optimization (GWO) method. This study uses the Songhua River basin and 25 control units as examples, adopting the PPMGWO model proposed in this study to allocate the water quantity. Using water consumption in all control units in the Songhua River basin in 2015 as reference to compare with optimization allocation results of firefly algorithm (FA) and Particle Swarm Optimization (PSO) algorithms as well as the PPMGWO model, results indicate that the average difference between corresponding allocation results and reference values are 0.195 bil m3, 0.151 bil m3, and 0.085 bil m3, respectively. Obviously, the average difference of the PPMGWO model is the lowest and its optimization allocation result is closer to reality, which further confirms the reasonability, feasibility, and accuracy of the PPMGWO model. And then the PPMGWO model is adopted to simulate allocation of available water quantity in Songhua River basin in 2018, 2020, and 2030. The simulation results show water quantity which could be allocated in all controls demonstrates an overall increasing trend with reasonable and equal exploitation and utilization of water resources in the Songhua River basin in future. In addition, this study has a certain reference value and application meaning to comprehensive management and water resources allocation in other transboundary river basins.

  16. Killing and caching of an adult White-tailed deer, Odocoileus virginianus, by a single Gray Wolf, Canis lupus

    Science.gov (United States)

    Nelson, Michael E.

    2011-01-01

    A single Gray Wolf (Canis lupus) killed an adult male White-tailed Deer (Odocoileus virginianus) and cached the intact carcass in 76 cm of snow. The carcass was revisited and entirely consumed between four and seven days later. This is the first recorded observation of a Gray Wolf caching an entire adult deer.

  17. Miglustat in late-onset Tay-Sachs disease: a 12-month, randomized, controlled clinical study with 24 months of extended treatment.

    Science.gov (United States)

    Shapiro, Barbara E; Pastores, Gregory M; Gianutsos, John; Luzy, Cécile; Kolodny, Edwin H

    2009-06-01

    To evaluate the safety and efficacy of miglustat in patients with GM2 gangliosidosis. A randomized, multicenter, open-label, 12-month study involving patients aged 18 years or older, randomized 2:1 to miglustat (200 mg TID) or "no miglustat treatment." This study was followed by 24 months of extended treatment during which all patients received miglustat. Primary efficacy endpoints were change in eight measures of isometric muscle strength in the limbs and isometric grip strength, evaluated at baseline, and months 12 and 36. Secondary efficacy endpoints included gait, balance, disability, and other neurological assessments. Safety evaluations included adverse event reporting. Thirty patients (67% male, age range 18-56 years) with late-onset Tay-Sachs disease were enrolled; 20 were randomized to miglustat and 10 to "no miglustat treatment." Muscle and grip strength generally decreased over the study period. No differences were observed between the two groups in any efficacy measure, either during the 12-month randomized phase or the full 36 months. The most common treatment-related adverse events were decrease in weight and diarrhea. Miglustat treatment was not shown to lead to measurable benefits in this cohort of patients with late-onset Tay-Sachs disease. The observed safety profile was consistent with that of the approved dose (100 mg TID) in type 1 Gaucher disease.

  18. Enchanced total dose damage in junction field effect transistors and related linear integrated circuits

    International Nuclear Information System (INIS)

    Flament, O.; Autran, J.L.; Roche, P.; Leray, J.L.; Musseau, O.

    1996-01-01

    Enhanced total dose damage of Junction Field-effect Transistors (JFETs) due to low dose rate and/or elevated temperature has been investigated for elementary p-channel structures fabricated on bulk and SOI substrates as well as for related linear integrated circuits. All these devices were fabricated with conventional junction isolation (field oxide). Large increases in damage have been revealed by performing high temperature and/or low dose rate irradiations. These results are consistent with previous studies concerning bipolar field oxides under low-field conditions. They suggest that the transport of radiation-induced holes through the oxide is the underlying mechanism. Such an enhanced degradation must be taken into account for low dose rate effects on linear integrated circuits

  19. Spermatozoa from the maned wolf (Chrysocyon brachyurus) display typical canid hyper-sensitivity to osmotic and freezing-induced injury, but respond favorably to dimethyl sulfoxide.

    Science.gov (United States)

    Johnson, Amy E M; Freeman, Elizabeth W; Wildt, David E; Songsasen, Nucharin

    2014-06-01

    We assessed the influences of medium osmolality, cryoprotectant and cooling and warming rate on maned wolf (Chrysocyon brachyurus) spermatozoa. Ejaculates were exposed to Ham's F10 medium (isotonic control) or to this medium plus NaCl (350-1000mOsm), sucrose (369 and 479mOsm), 1M glycerol (1086mOsm) or dimethyl sulfoxide (Me2SO, 1151mOsm) for 10 min. Each sample then was diluted back into Ham's medium and assessed for sperm motility and plasma membrane integrity. Although glycerol and Me2SO had no influence (P>0.05), NaCl and sucrose solutions affected sperm motility (P0.05) to the control. As osmolality of the NaCl solution increased, motility decreased to maned wolf spermatozoa are similar to domestic dog sperm in their sensitivity to osmotic-induced motility damage, the plasma membranes tolerate dehydration, and the cells respond favorably to Me2SO as a cryoprotectant. Published by Elsevier Inc.

  20. Ring nebulae associated with Wolf-Rayet stars

    International Nuclear Information System (INIS)

    Chu, Y.-H.

    1982-01-01

    Using strict selection criteria, the author and colleagues have searched for ring nebulae associated with Wolf-Rayet stars in the Galaxy and the Magellanic Clouds. 15 WR ring nebulae are identified in the Galaxy, 9 in the Large Magellanic Cloud, and none in the small Magellanic Cloud. The morphology and kinematics of these 24 nebulae have subsequently been observed to study their nature. These nebulae and their references are listed and a correlation between spectral and nebular types is presented. (Auth.)

  1. Community Resource Uses and Ethiopian Wolf Conservation in Mount Abune Yosef

    Science.gov (United States)

    Eshete, Girma; Tesfay, Girmay; Bauer, Hans; Ashenafi, Zelealem Tefera; de Iongh, Hans; Marino, Jorgelina

    2015-09-01

    People who perceive economic benefits and enjoy unrestricted access to natural resources tend to support ecosystem conservation efforts. Our study explores whether this remains true in remnant patches of Afroalpine ecosystem in North Ethiopia, where communal land provides valuable natural resources for the local communities and also sustain small populations of the endangered Ethiopian wolf ( Canis simensis). Questionnaires were designed to assess ecological and socio-economic characteristics of the livelihoods of the Amhara people living in Mount Abune Yosef and their attitudes toward Afroalpine and Ethiopian wolf conservation. Of the 120 households interviewed, selected randomly from across eight villages, 80 % benefited from natural resources by grazing their livestock and harvesting firewood and grasses. The majority (90 %) also suffered from livestock predation by Ethiopian wolves and common jackals (Canis aureus) and crop raiding by geladas ( Theropithecus gelada), birds, and rodents, yet more than half reported a positive attitudes toward Ethiopian wolves (66 %). People with positive attitudes tended to live close to the communal land, to own more livestock, and to be unaffected by conflict. Many also recognized the need to protect the Afroalpine habitats of Abune Yosef (71 %), and this attitude predominated among the literate, households that owned land, had smaller herds and were further away. We discussed how people's attitudes were modulated by human-wildlife conflicts and by the benefits derived from the access to natural resources in communal land, and the implications for the conservation of Afroalpine ecosystem and the flagship Ethiopian wolf.

  2. Parsing demographic effects of canine pParvovirus on a Minnesota wolf population

    Science.gov (United States)

    Mech, L. David; Goyal, Sagar M.

    2011-01-01

    We examined 35 years of relationships among wolf (Canis lupus) pup survival, population change and canine parvovirus (CPV) seroprevalence in Northeastern Minnesota to determine when CPV exerted its strongest effects. Using correlation analysis of data from five periods of 7-years each from 1973 through 2007, we learned that the strongest effect of CPV on pup survival (r = -0.73) and on wolf population change (r = -0.92) was during 1987 to 1993. After that, little effect was documented despite a mean CPV seroprevalence from 1994 of 2007 of 70.8% compared with 52.6% during 1987 to 1993. We conclude that after CPV became endemic and produced its peak effect on the study population, that population developed enough immunity to withstand the disease.

  3. Noninvasive molecular tracking of colonizing wolf (Canis lupus) packs in the western Italian Alps.

    Science.gov (United States)

    Lucchini, V; Fabbri, E; Marucco, F; Ricci, S; Boitani, L; Randi, E

    2002-05-01

    We used noninvasive methods to obtain genetic and demographic data on the wolf packs (Canis lupus), which are now recolonizing the Alps, a century after their eradication. DNA samples, extracted from presumed wolf scats collected in the western Italian Alps (Piemonte), were genotyped to determine species and sex by sequencing parts of the mitochondrial DNA (mtDNA) control-region and ZFX/ZFY genes. Individual genotypes were identified by multilocus microsatellite analyses using a multiple tubes polymerase chain reaction (PCR). The performance of the laboratory protocols was affected by the age of samples. The quality of excremental DNA extracts was higher in samples freshly collected on snow in winter than in samples that were older or collected during summer. Preliminary mtDNA screening of all samples allowed species identification and was a good predictor of further PCR performances. Wolf, and not prey, DNA targets were preferentially amplified. Allelic dropout occurred more frequently than false alleles, but the probability of false homozygote determinations was always wolf genotypes, also whether related, with a probability of identity of < 0.015. Genealogical relationships among individuals could be determined reliably if the number of candidate parents was 6-8, and most of them had been sampled and correctly genotyped. Genetic data indicate that colonizing Alpine wolves originate exclusively from the Italian source population and retain a high proportion of its genetic diversity. Spatial and temporal locations of individual genotypes, and kinship analyses, suggest that two distinct packs of closely related wolves, plus some unrelated individuals, ranged in the study areas. This is in agreement with field observations.

  4. Preventing Lone Wolf Terrorism: some CT Approaches Addressed

    OpenAIRE

    Edwin Bakker; Beatrice de Graaf

    2011-01-01

    After a brief discussion of the epistemological and phenomenological difficulties associated with the concept of lone wolf terrorism, a number of possible counter-terrorist approaches are discussed. Lone operator terrorist acts should be considered ‘black swan’ occurrences that are almost impossible to categorize or systematize, let alone forecast. Thus, not the profile of the perpetrator, but the modus operandi offer clues for a better response to this particular threat. Furtherm...

  5. Path integral solution of linear second order partial differential equations I: the general construction

    International Nuclear Information System (INIS)

    LaChapelle, J.

    2004-01-01

    A path integral is presented that solves a general class of linear second order partial differential equations with Dirichlet/Neumann boundary conditions. Elementary kernels are constructed for both Dirichlet and Neumann boundary conditions. The general solution can be specialized to solve elliptic, parabolic, and hyperbolic partial differential equations with boundary conditions. This extends the well-known path integral solution of the Schroedinger/diffusion equation in unbounded space. The construction is based on a framework for functional integration introduced by Cartier/DeWitt-Morette

  6. Complete Genomic Sequence of Canine Distemper Virus from an Ethiopian Wolf.

    Science.gov (United States)

    Marston, Denise A; Watson, Jemma; Wise, Emma L; Ellis, Richard J; Bedin, Eric; Ayalew, Girma; Abute, Muktar; de Lamballerie, Xavier; Fooks, Anthony R; Sillero-Zubiri, Claudio; Banyard, Ashley C

    2017-07-20

    Canine distemper virus (CDV) has been implicated in population declines of wildlife, including many threatened species. Here we present the full genome of CDV from an Ethiopian wolf, Canis simensis , the world's rarest and most endangered canid. © Crown copyright 2017.

  7. Using Pop-II models to predict effects of wolf predation and hunter harvests on elk, mule deer, and moose on the northern range

    Science.gov (United States)

    Mack, John A.; Singer, Francis J.

    1993-01-01

    The effects of establishing a gray wolf (Canis lupus) population in Yellowstone National Park were predicted for three ungulate species—elk (Cervus elaphus), mule deer (Odocoileus hemionus), and moose (Alces alces)—using previously developed POP-II population models. We developed models for 78 and 100 wolves. For each wolf population, we ran scenarios using wolf predation rates of 9, 12, and 15 ungulates/wolf/year. With 78 wolves and the antlerless elk harvest reduced 27%, our modeled elk population estimated were 5-18% smaller than the model estimate without wolves. With 100 wolves and the antlerless elk harvest reduced 27%, our elk population estimated were 11-30% smaller than the population estimates without wolves. Wolf predation effects were greater on the modeled mule deer population than on elk. With 78 wolves and no antlerless deer harvest, we predicted the mule deer population could be 13-44% larger than without wolves. With 100 wolves and no antlerless deer harvest, the mule deer population was 0-36% larger than without wolves. After wolf recovery, our POP-II models suggested moose harvests would have to be reduced at least 50% to maintain moose numbers at the levels predicted when wolves were not present. Mule deer and moose population data are limited, and these wolf predation effects may be overestimated if population sizes or male-female ratios were underestimated in our population models. We recommend additional mule deer and moose population data be obtained.

  8. How linear features alter predator movement and the functional response.

    KAUST Repository

    McKenzie, Hannah W

    2012-01-18

    In areas of oil and gas exploration, seismic lines have been reported to alter the movement patterns of wolves (Canis lupus). We developed a mechanistic first passage time model, based on an anisotropic elliptic partial differential equation, and used this to explore how wolf movement responses to seismic lines influence the encounter rate of the wolves with their prey. The model was parametrized using 5 min GPS location data. These data showed that wolves travelled faster on seismic lines and had a higher probability of staying on a seismic line once they were on it. We simulated wolf movement on a range of seismic line densities and drew implications for the rate of predator-prey interactions as described by the functional response. The functional response exhibited a more than linear increase with respect to prey density (type III) as well as interactions with seismic line density. Encounter rates were significantly higher in landscapes with high seismic line density and were most pronounced at low prey densities. This suggests that prey at low population densities are at higher risk in environments with a high seismic line density unless they learn to avoid them.

  9. How linear features alter predator movement and the functional response.

    KAUST Repository

    McKenzie, Hannah W; Merrill, Evelyn H; Spiteri, Raymond J; Lewis, Mark A

    2012-01-01

    In areas of oil and gas exploration, seismic lines have been reported to alter the movement patterns of wolves (Canis lupus). We developed a mechanistic first passage time model, based on an anisotropic elliptic partial differential equation, and used this to explore how wolf movement responses to seismic lines influence the encounter rate of the wolves with their prey. The model was parametrized using 5 min GPS location data. These data showed that wolves travelled faster on seismic lines and had a higher probability of staying on a seismic line once they were on it. We simulated wolf movement on a range of seismic line densities and drew implications for the rate of predator-prey interactions as described by the functional response. The functional response exhibited a more than linear increase with respect to prey density (type III) as well as interactions with seismic line density. Encounter rates were significantly higher in landscapes with high seismic line density and were most pronounced at low prey densities. This suggests that prey at low population densities are at higher risk in environments with a high seismic line density unless they learn to avoid them.

  10. Dioctophyma renale in maned wolf in the geoeconomic region of Jatai, GO, Brazil - Case report

    Directory of Open Access Journals (Sweden)

    Valcinir Aloisio Scalla Vulcani

    2015-06-01

    Full Text Available ABSTRACT. Vulcani V.A.S., Franzo V.S., de Araújo D.P., Vicentin F.R., da Costa O.M., Rangel A.S. & Gomes L.A. [Dioctophyma renale in maned wolf in the geoeconomic region of Jatai, GO, Brazil - Case report.] Dioctophyma renale em Lobo-Guará na região geoeconômica de Jataí, GO, Brasil - Relato de caso. Revista Brasileira de Medicina Veterinária, 37(2:149-152, 2015. Laboratório de Anatomia, Curso de Medicina Veterinária, Universidade Federal de Goiás, Campus de Jataí, BR 364 Km 192, 3800, Setor Industrial, Jataí, GO 75801-615, Brasil. E-mail: aloisiosv@hotmail.com The maned Wolf (Chrysocyon brachyurus (Illiger, 1815 is the largest animal that represents the canidae species at the South America. The Dioctophyme renale (Goeze, 1782 known as the giant kidney-worm, because it is used to lodges mostly this organs, and it has been also responsible for the majority of captive animals deaths. A maned wolf necropsy was done at the animal anatomy laboratory of the Universidade Federal de Goiás (UFG - Campus Jataí and showed a nematode in the right kidney of the wild dog. This is the second confirmed case report of maned wolf that have free life in the city of Jataí and in State of Goiás, that suggests that is a common parasitism in this region of the country.

  11. False alerts in air traffic control conflict alerting system: is there a "cry wolf" effect?

    Science.gov (United States)

    Wickens, Christopher D; Rice, Stephen; Keller, David; Hutchins, Shaun; Hughes, Jamie; Clayton, Krisstal

    2009-08-01

    The aim is to establish the extent to which the high false-alarm rate of air traffic control midair conflict alerts is responsible for a "cry wolf' effect-where true alerts are not responded to and all alerts are delayed in their response. Some aircraft collisions have been partly attributed to the cry wolf effect, and in other domains (health care and systems monitoring), there is a causal connection between false-alarm rate and cry wolf behavior. We hypothesized that a corresponding relationship exists in air traffic control (ATC). Aircraft track and alert system behavior data surrounding 495 conflict alerts were analyzed to identify true and false alerts, trajectory type, and controller behavior. Forty-five percent of the alerts were false, ranging from 0.28 to 0.58. Although centers with more false alerts contributed to more nonresponses, there was no evidence that these were nonresponses to true alerts or that response times were delayed in those centers. Instead, controllers showed desirable anticipatory behavior by issuing trajectory changes prior to the alert. Those trajectory pairs whose conflicts were more difficult to visualize induced more reliance on, and less compliance with, the alerting system. The high false-alarm rate does not appear to induce cry wolf behavior in the context of en route ATC conflict alerts. There is no need to substantially modify conflict alert algorithms, but the conflict alert system may be modified to address difficult-to-visualize conflicts.

  12. Light curve of the CX Cep eclipsing binary system and characteristics of a Wolf-Rayet star

    International Nuclear Information System (INIS)

    Lipunova, N.A.; Cherepashchuk, A.M.

    1982-01-01

    The photoelectric B, V, R observations of the eclipsing Wolf-Rayet binary CX Cep (WN 5 + 08V, V approximately equal to 12sup(m),1, p approximately equal to 2sup(d),127) have been carried out. The physical characteristics of the WN 5 star, the core radius r 0 =(4.5+-2.5) Rsub(S) (Rsub(S) is the Sun radius) and the brightness temperature of the core Tsub(b)>50 000 K, are determined from the analysis of the light curve lambdasub(eff) approximately equal to 6 000 A. These characteristics are close to those of the WN 5 star in the eclipsing Wolf-Rayet binary V 444 Cyg. The results of the interpretation of the light curves of two eclipsing Wolf-Rayet binaries (V 444 Cyg and CX Cep) confirm the conclusions of the modern theory of evolution of massive close binary systems [ru

  13. Spectrophotometry of ring nebulae around Wolf-Rayet stars

    International Nuclear Information System (INIS)

    Kwitter, K.B.

    1979-01-01

    Spectrophotometric observations of four ring nebulae surrounding population I Wolf-Rayet (WN) stars have been obtained, and four additional filamentary nebulae in order to determine the physical conditions and chemical abundances in these objects. It was concluded that the ring nebulae are enriched in nitrogen and helium as a result of contamination of the ambient interstellar medium by the helium- and nitrogen-rich wind from the central Wolf-Rayet star. Of the additional nebulae studied, two were found to be Peimbert Type I planetary nebulae, overabundant in nitrogen and helium due to mixing of CNO processed material into the parent envelope prior to ejection. One of the remaining objects, a shell around an Oef star, is found to have normal abundances; the other, a small H II region around an early Be star, also exhibits normal abundances. It was attempted to interpret the ring nebulae and the Oef shell as interstellar bubbles, according to recent theory; it met with varying degrees of success. For two of the ring nebulae, the fraction of nebular mass contributed by the central star can be estimated from published stellar abundances. It was found that in these two cases, the stellar wind has provided less than 10% of the observed nebular mass

  14. The effective temperatures of early Wolf-Rayet stars

    International Nuclear Information System (INIS)

    Schmutz, W.

    1982-01-01

    Nussbaumer et al. (1981) (NSSW) have calculated temperatures of 15 Wolf-Rayet stars, which cover most subtypes in the WN and WC sequences. Some Wolf-Rayet stars are found to have a blackbody-like energy distribution and therefore their effective temperature can be well determined, others show deviation from the blackbody shape. This deviation is probably due to an extended continuum emitting region. In view of these later cases NSSW adopted a temperature which they derived from a Zanstra analysis of the He II recombination lines. According to their analysis there should be a continuum jump at 2050 A. New IUE observations of the WN 5 star HD 50896 however did not show any trace of such an absorption edge. This implies that the effective temperature of this WN 5 star and probably of all WR stars with a non blackbody energy distribution is not yet known accurately. Therefore the author started a detailed analysis with a spherically symmetric atmosphere model. Though the model has not yet reached its final shape he has obtained as a first result that the location of HD 50896 in the HR-diagram is to the left of the ZAMS and that the Zanstra analysis is indeed not valid for this star. (Auth.)

  15. MANDIBULAR MORPHOMETRY APPLIED TO ANESTHETIC BLOCKAGE IN THE MANED WOLF (CHRYSOCYON BRACHYURUS).

    Science.gov (United States)

    de Souza Junior, Paulo; de Moraes, Flavio Machado; de Carvalho, Natan da Cruz; Canelo, Evandro Alves; Thiesen, Roberto; Santos, André Luiz Quagliatto

    2016-03-01

    Chrysocyon brachyurus (maned wolf) is the biggest South American canid and has a high frequency of dental injuries, both in the wild and in captivity. Thus, veterinary procedures are necessary to preserve the feeding capacity of hundreds of captive specimens worldwide. The aim of this study was to investigate the mandibular morphometry of the maned wolf with emphasis on the establishment of anatomic references for anesthetic block of the inferior alveolar and mental nerves. Therefore, 16 measurements in 22 mandibles of C. brachyurus adults were taken. For extraoral block of the inferior alveolar nerve at the level of the mandibular foramen, the needle should be advanced close to the medial face of the mandibular ramus for 11.4 mm perpendicular to the palpable concavity. In another extraoral approach, the needle may be introduced for 30.4 mm from the angular process at a 20-25° angle to the ventral margin. For blocking only the mental nerve, the needle should be inserted for 10 mm from ventral border, close to the labial surface of the mandibular body, at the level of the lower first premolar. The mandibular foramen showed similar position, size, and symmetry in the maned wolf specimens examined. Comparison of the data observed here with those available for other carnivores indicates the need to determine these anatomic references specifically for each species.

  16. Microbiota and anthropic interference on antimicrobial resistance profile of bacteria isolated from Brazilian maned-wolf (Chrysocyon brachyurus)

    OpenAIRE

    Vieira-da-Motta, Olney; Eckhardt-de-Pontes, Luiz Antonio; Petrucci, Melissa Paes; dos Santos, Israel Pereira; da Cunha, Isabel Candia Nunes; Morato, Ronaldo Gonçalves

    2014-01-01

    Both the study of Brazilian wild mammal fauna and the conditions that foster the preservation of endangered species, such as Brazilian Maned-wolf (Chrysocyon brachyurus), in wild life are of extreme importance. In order to study the resistance profile of microbiota bacterial colonizing Brazilian Maned-wolf, this work investigated samples from eight male captive and free roaming animals originating from different Brazilian geographical regions. Samples for microbiological purposes were collect...

  17. Unusual motions in the Wolf-Rayet nebula NGC 6888

    International Nuclear Information System (INIS)

    Johnson, P.G.; Songsathaporn, R.

    1981-01-01

    A systematic survey of the velocity structure within the Wolf-Rayet ring nebula NGC 6888 has been undertaken by making observations of the [N II] line profiles. They reveal a hitherto undetected and particularly unusual velocity structure with three of the brightest portions of the circumference of this ring exhibiting triple line components. Possible models to explain these observations are discussed. (author)

  18. Weak lensing probes of modified gravity

    International Nuclear Information System (INIS)

    Schmidt, Fabian

    2008-01-01

    We study the effect of modifications to general relativity on large-scale weak lensing observables. In particular, we consider three modified gravity scenarios: f(R) gravity, the Dvali-Gabadadze-Porrati model, and tensor-vector-scalar theory. Weak lensing is sensitive to the growth of structure and the relation between matter and gravitational potentials, both of which will in general be affected by modified gravity. Restricting ourselves to linear scales, we compare the predictions for galaxy-shear and shear-shear correlations of each modified gravity cosmology to those of an effective dark energy cosmology with the same expansion history. In this way, the effects of modified gravity on the growth of perturbations are separated from the expansion history. We also propose a test which isolates the matter-potential relation from the growth factor and matter power spectrum. For all three modified gravity models, the predictions for galaxy and shear correlations will be discernible from those of dark energy with very high significance in future weak lensing surveys. Furthermore, each model predicts a measurably distinct scale dependence and redshift evolution of galaxy and shear correlations, which can be traced back to the physical foundations of each model. We show that the signal-to-noise for detecting signatures of modified gravity is much higher for weak lensing observables as compared to the integrated Sachs-Wolfe effect, measured via the galaxy-cosmic microwave background cross-correlation.

  19. A highly linear fully integrated powerline filter for biopotential acquisition systems.

    Science.gov (United States)

    Alzaher, Hussain A; Tasadduq, Noman; Mahnashi, Yaqub

    2013-10-01

    Powerline interference is one of the most dominant problems in detection and processing of biopotential signals. This work presents a new fully integrated notch filter exhibiting high linearity and low power consumption. High filter linearity is preserved utilizing active-RC approach while IC implementation is achieved through replacing passive resistors by R-2R ladders achieving area saving of approximately 120 times. The filter design is optimized for low power operation using an efficient circuit topology and an ultra-low power operational amplifier. Fully differential implementation of the proposed filter shows notch depth of 43 dB (78 dB for 4th-order) with THD of better than -70 dB while consuming about 150 nW from 1.5 V supply.

  20. Simultaneous preimplantation genetic diagnosis for Tay-Sachs and Gaucher disease.

    Science.gov (United States)

    Altarescu, Gheona; Brooks, Barry; Margalioth, Ehud; Eldar Geva, Talia; Levy-Lahad, Ephrat; Renbaum, Paul

    2007-07-01

    Preimplantation genetic diagnosis (PGD) for single gene defects is described for a family in which each parent is a carrier of both Tay-Sachs (TS) and Gaucher disease (GD). A multiplex fluorescent polymerase chain reaction protocol was developed that simultaneously amplified all four familial mutations and 10 informative microsatellite markers. In one PGD cycle, seven blastomeres were analysed, reaching a conclusive diagnosis in six out of seven embryos for TS and in five out of seven embryos for GD. Of the six diagnosed embryos, one was wild type for both TS and GD, and three were wild type for GD and carriers of TS. Two remaining embryos were compound heterozygotes for TS. Two transferable embryos developed into blastocysts (wt/wt and wt GD/carrier TS) and both were transferred on day 5. This single cycle of PGD resulted in a healthy live child. Allele drop-out (ADO) was observed in three of 34 reactions, yielding an 8% ADO rate. The occurrence of ADO in single cell analysis and undetected recombination events are primary causes of misdiagnosis in PGD and emphasize the need to use multiple polymorphic markers. So far as is known, this is the first report of concomitant PGD for two frequent Ashkenazi Jewish recessive disorders.

  1. The Tibetan Wolf Canis lupus chanco Gray (Mammalia: Carnivora: Canidae in northeastern India with a recent sighting from northern Sikkim, India

    Directory of Open Access Journals (Sweden)

    Anwaruddin Choudhury

    2015-06-01

    Full Text Available The Tibetan Wolf Canis lupus chanco which is a relatively rarer subspecies of the wolf C. lupus occurs in Sikkim in North-east India with unconfirmed reports from Arunachal Pradesh. Its range in Sikkim is in the high elevation areas. There are very few sighting records from the state. This article reports an observation made recently between Thangu and Gyagong or Gogong in North Sikkim district at 4,250m elevation on 17 April 2014. Presence of feral dogs are threats to the Wolf as well as its prey base.  

  2. Active packaging for fresh-cut broccoli using 1-methylcyclopropene in biodegradable sachet/ Embalagem ativa para brócolis minimamente processado utilizando 1-metilciclopropeno em sachê biodegradável

    Directory of Open Access Journals (Sweden)

    Marta de Toledo Benassi

    2006-07-01

    Full Text Available Fresh-cut broccoli florets were packed in polypropylene pots containing a sachet with 1- methylcyclopropene (1-MCP and sealed with biodegradable starch-based film. Broccoli was stored for 8 days at 12oC and after this time the color and the texture of the product were similar of the fresh broccoli, with no off-flavor development or decay. Active packaging with 1-MCP in a sachet was efficient to extent shelf life of broccoli florets retarding yellowing and vitamin C losses. It is an alternative of 1-MCP treatment for fresh-cut products and besides, the sachet can absorbs condensed water reducing spoilage and off-odor development.Brócolis minimamente processados foram embalados em bandejas de polipropileno com sachê contendo 1-metilciclopropeno (1-MCP e selado com filme biodegradável de amido. Os brócolis foram armazenados por 8 dias a 12oC e após este tempo a cor e a textura mantiveram-se similares às do produto fresco, sem desenvolvimento de odor não característico ou podridão. A embalagem ativa contendo 1-MCP na forma de sachê foi eficiente no aumento da vida de prateleira de brócolis, retardando o amarelecimento e a perda de vitamina C. Esta é uma alternativa para o tratamento com 1-MCP para produtos minimamente processados e além disso, o sachê pode absorver a água condensada, reduzindo a deterioração e o desenvolvimento de odor não característico.

  3. Gray Wolf (Canis lupus) movements and behavior around a kill site and implications for GPS collar studies

    Science.gov (United States)

    Mech, L. David

    2011-01-01

    Global Positioning System (GPS) radio-collars are increasingly used to estimate Gray Wolf (Canis lupus) kill rates. In interpreting results from this technology, researchers make various assumptions about wolf behavior around kills, yet no detailed description of this behavior has been published. This article describes the behavior of six wolves in an area of constant daylight during 30 hours, from when the pack killed a Muskox (Ovibos moschatus) calf and yearling on Ellesmere Island, Nunavut, Canada, to when they abandoned the kill remains. Although this is only a single incident, it demonstrates one possible scenario of pack behavior around a kill. Combined with the literature, this observation supports placing a radio-collar on the breeding male to maximize finding kills via GPS collars and qualifying results depending on whatever other information is available about the collared wolf's pack.

  4. Optical structures, algebraically special spacetimes, and the Goldberg-Sachs theorem in five dimensions

    International Nuclear Information System (INIS)

    Taghavi-Chabert, Arman

    2011-01-01

    Optical (or Robinson) structures are one generalization of four-dimensional shearfree congruences of null geodesics to higher dimensions. They are Lorentzian analogues of complex and CR structures. In this context, we extend the Goldberg-Sachs theorem to five dimensions. To be precise, we find a new algebraic condition on the Weyl tensor, which generalizes the Petrov type II condition, in the sense that it ensures the existence of such congruences on a five-dimensional spacetime, vacuum or under weaker assumptions on the Ricci tensor. This results in a significant simplification of the field equations. We discuss possible degenerate cases, including a five-dimensional generalization of the Petrov type D condition. We also show that the vacuum black ring solution is endowed with optical structures, yet fails to be algebraically special with respect to them. We finally explain the generalization of these ideas to higher dimensions, which has been checked in six and seven dimensions.

  5. Genetic variability in maned wolf based on heterologous short-tandem repeat markers from domestic dog.

    Science.gov (United States)

    Salim, D C; Akimoto, A A; Carvalho, C B; Oliveira, S F; Grisolia, C K; Moreira, J R; Klautau-Guimarães, M N

    2007-06-20

    The maned wolf (Chrysocyon brachyurus) is the largest South American canid. Habitat loss and fragmentation, due to agricultural expansion and predatory hunting, are the main threats to this species. It is included in the official list of threatened wildlife species in Brazil, and is also protected by IUCN and CITES. Highly variable genetic markers such as microsatellites have the potential to resolve genetic relationships at all levels of the population structure (among individuals, demes or metapopulations) and also to identify the evolutionary unit for strategies for the conservation of the species. Tests were carried out to verify whether a class of highly polymorphic tetranucleotide repeats described for the domestic dog effectively amplifies DNA in the maned wolf. All five loci studied were amplified; however, one of these, was shown to be monomorphic in 69 maned wolf samples. The average allele number and estimated heterozygosity per polymorphic locus were 4.3 and 67%, respectively. The genetic variability found for this species, which is considered threatened with extinction, showed similar results when compared to studies of other canids.

  6. Hybrid Algorithm of Particle Swarm Optimization and Grey Wolf Optimizer for Improving Convergence Performance

    Directory of Open Access Journals (Sweden)

    Narinder Singh

    2017-01-01

    Full Text Available A newly hybrid nature inspired algorithm called HPSOGWO is presented with the combination of Particle Swarm Optimization (PSO and Grey Wolf Optimizer (GWO. The main idea is to improve the ability of exploitation in Particle Swarm Optimization with the ability of exploration in Grey Wolf Optimizer to produce both variants’ strength. Some unimodal, multimodal, and fixed-dimension multimodal test functions are used to check the solution quality and performance of HPSOGWO variant. The numerical and statistical solutions show that the hybrid variant outperforms significantly the PSO and GWO variants in terms of solution quality, solution stability, convergence speed, and ability to find the global optimum.

  7. Estimating integrated variance in the presence of microstructure noise using linear regression

    Science.gov (United States)

    Holý, Vladimír

    2017-07-01

    Using financial high-frequency data for estimation of integrated variance of asset prices is beneficial but with increasing number of observations so-called microstructure noise occurs. This noise can significantly bias the realized variance estimator. We propose a method for estimation of the integrated variance robust to microstructure noise as well as for testing the presence of the noise. Our method utilizes linear regression in which realized variances estimated from different data subsamples act as dependent variable while the number of observations act as explanatory variable. We compare proposed estimator with other methods on simulated data for several microstructure noise structures.

  8. Murine Sialidase Neu3 facilitates GM2 degradation and bypass in mouse model of Tay-Sachs disease.

    Science.gov (United States)

    Seyrantepe, Volkan; Demir, Secil Akyildiz; Timur, Zehra Kevser; Von Gerichten, Johanna; Marsching, Christian; Erdemli, Esra; Oztas, Emin; Takahashi, Kohta; Yamaguchi, Kazunori; Ates, Nurselin; Dönmez Demir, Buket; Dalkara, Turgay; Erich, Katrin; Hopf, Carsten; Sandhoff, Roger; Miyagi, Taeko

    2018-01-01

    Tay-Sachs disease is a severe lysosomal storage disorder caused by mutations in Hexa, the gene that encodes for the α subunit of lysosomal β-hexosaminidase A (HEXA), which converts GM2 to GM3 ganglioside. Unexpectedly, Hexa -/- mice have a normal lifespan and show no obvious neurological impairment until at least one year of age. These mice catabolize stored GM2 ganglioside using sialidase(s) to remove sialic acid and form the glycolipid GA2, which is further processed by β-hexosaminidase B. Therefore, the presence of the sialidase (s) allows the consequences of the Hexa defect to be bypassed. To determine if the sialidase NEU3 contributes to GM2 ganglioside degradation, we generated a mouse model with combined deficiencies of HEXA and NEU3. The Hexa -/- Neu3 -/- mice were healthy at birth, but died at 1.5 to 4.5months of age. Thin-layer chromatography and mass spectrometric analysis of the brains of Hexa -/- Neu3 -/- mice revealed the abnormal accumulation of GM2 ganglioside. Histological and immunohistochemical analysis demonstrated cytoplasmic vacuolation in the neurons. Electron microscopic examination of the brain, kidneys and testes revealed pleomorphic inclusions of many small vesicles and complex lamellar structures. The Hexa -/- Neu3 -/- mice exhibited progressive neurodegeneration with neuronal loss, Purkinje cell depletion, and astrogliosis. Slow movement, ataxia, and tremors were the prominent neurological abnormalities observed in these mice. Furthermore, radiographs revealed abnormalities in the skeletal bones of the Hexa -/- Neu3 -/- mice. Thus, the Hexa -/- Neu3 -/- mice mimic the neuropathological and clinical abnormalities of the classical early-onset Tay-Sachs patients, and provide a suitable model for the future pre-clinical testing of potential treatments for this condition. Copyright © 2017 Elsevier Inc. All rights reserved.

  9. Observations of mass loss from OB and Wolf-Rayet stars

    International Nuclear Information System (INIS)

    Barlow, M.J.

    1982-01-01

    In this review, three observationally accessible parameters of the winds of OB and Wolf-Rayet stars are discussed: (1) Terminal velocities, (2) Velocity laws, (3) Mass loss rates. In addition, some discussion of the ionisation structure of the winds is included. In general, only the most recent results for OB stars are mentioned. (Auth.)

  10. The wolf reference genome sequence (Canis lupus lupus) and its implications for Canis spp. population genomics

    DEFF Research Database (Denmark)

    Gopalakrishnan, Shyam; Samaniego Castruita, Jose Alfredo; Sinding, Mikkel Holger Strander

    2017-01-01

    Background An increasing number of studies are addressing the evolutionary genomics of dog domestication, principally through resequencing dog, wolf and related canid genomes. There is, however, only one de novo assembled canid genome currently available against which to map such data - that of a......Background An increasing number of studies are addressing the evolutionary genomics of dog domestication, principally through resequencing dog, wolf and related canid genomes. There is, however, only one de novo assembled canid genome currently available against which to map such data...

  11. The Val{sup 192}Leu mutation in the {alpha}-subunit of {beta}-hexosaminidase A is not associated with the B1-variant form of Tay-Sachs disease

    Energy Technology Data Exchange (ETDEWEB)

    Hou, Y.; Vavougios, G.; Hinek, A. [Univ. of Toronto (Canada)] [and others

    1996-07-01

    Substitution mutations adversely affecting the {alpha}-subunit of {beta}-hexosaminidase A ({alpha}{beta}) (EC 3.2.1.52) result in Tay-Sachs disease. The majority affect the initial folding of the pro-{alpha} chain in the endoplasmic reticulum, resulting in its retention and degradation. A much less common occurrence is a mutation that specifically affects an {open_quotes}active-site{close_quotes} residue necessary for substrate binding and/or catalysis. In this case, hexosaminidase A is present in the lysosome, but it lacks all {alpha}-specific activity. This biochemical phenotype is referred to as the {open_quotes}B1-variant form{close_quotes} of Tay-Sachs disease. Kinetic analysis of suspected B1-variant mutations is complex because hexosaminidase A is heterodimeric and both subunits possess similar active sites. In this report, we examine a previously identified B1-variant mutation, {alpha}-Val{sup 192}Leu. Chinese hamster ovary cells were permanently cotransfected with an {alpha}-cDNA-construct encoding the substitution and a mutant {beta}-cDNA ({beta}-Arg{sup 211}Lys), encoding a {beta}-subunit that is inactive but normal in all other respects. We were surprised to find that the Val{sup 192}Leu substitution produced a pro-{alpha} chain that did not form {alpha}-{beta} dimers and was not transported to the lysosome. Finally, we reexamined the hexosaminidase activity and protein levels in the fibroblasts from the original patient. These data were also not consistent with the biochemical phenotype of the B1 variant of Tay-Sachs disease previously reported to be present. Thus, we conclude that the Val{sup 192}Leu substitution does not specifically affect the {alpha}-active site. 23 refs., 4 figs., 2 tabs.

  12. A CMOS 128-APS linear array integrated with a LVOF for highsensitivity and high-resolution micro-spectrophotometry

    NARCIS (Netherlands)

    Liu, C.; Emadi, A.; Wu, H.; De Graaf, G.; Wolffenbuttel, R.F.

    2010-01-01

    A linear array of 128 Active Pixel Sensors has been developed in standard CMOS technology and a Linear Variable Optical Filter (LVOF) is added using CMOS-compatible post-process, resulting in a single chip highly-integrated highresolution microspectrometer. The optical requirements imposed by the

  13. To Find a Treasure: The Nuu-chah-nulth Wolf Mask

    Science.gov (United States)

    Kruger, Arnold

    2003-01-01

    The Wolf Ritual, or Tlukwana, with its associated regalia of masks, dances, costumes, and musical instruments, was a major feature of the Nuu-chah-nulth Winter Ceremonies. In common with other Northwest Coast Native nations, the lives of the Nuu-chah-nulth people were controlled by the seasons, and following a summer and autumn of gathering and…

  14. The Wolf-Rayet hydrogen puzzle -- an observational point of view

    OpenAIRE

    Foellmi, C.; Marchenko, S. V.; Moffat, A. F. J.

    2005-01-01

    Significant amounts of hydrogen were found in very hot early-type single WN stars in the SMC and the LMC. Recently, we found similar evidence in the Wolf-Rayet star of a short-period LMC binary. We discuss here the relevance of hydrogen for WR star classification, models, the relation to metallicity, and the GRB progenitors.

  15. Epilepsy in a child with Wolf-Hirschhorn syndrome

    Directory of Open Access Journals (Sweden)

    Radlović Nedeljko

    2011-01-01

    Full Text Available Introduction. Wolf-Hirschhorn syndrome (WHS is a rare chromosomal disorder characterized by facial dismorphy, multiple congenital anomalies, delayed psychomotor development and pharmaco-resistant epilepsy. Case Outline. We present a 5-year-old girl with severe delay in growth and development, microcephaly, mild facial dismorphy and epilepsy. The pregnancy was complicated by intrauterine growth retardation. Generalized muscle hypotonia was observed at birth. First seizures started at age of 9 months as unilateral convulsive status epilepticus (SE, sometimes with bilateral generalization. Seizures were often triggered by fever and were resistant to antiepileptic treatment. Introduction of lamotrigine and valproate therapy led to complete seizure control at the age of 33 months. Electroencephalographic (EEG finding was typical at the beginning. After transitory improvement between age four and five years, epileptiform EEG activity appeared again at the age of five years, without observed clinical seizures. Magnetic resonance imaging showed diffuse brain atrophy and delay in myelination. Using Multiplex ligation-dependent probe amplification (MLPA method, we disclosed heterozygote microdeletation of the distal part of the short arm of chromosome 4 (4p16. Conclusion. We present a clinical course of epilepsy in a patient with Wolf-Hirschhorn syndrome. The diagnosis was verified by modern molecular technique. This is the first molecular characterization of a patient with WHS performed in our country.

  16. A unified framework for testing in the linear regression model under unknown order of fractional integration

    DEFF Research Database (Denmark)

    Christensen, Bent Jesper; Kruse, Robinson; Sibbertsen, Philipp

    We consider hypothesis testing in a general linear time series regression framework when the possibly fractional order of integration of the error term is unknown. We show that the approach suggested by Vogelsang (1998a) for the case of integer integration does not apply to the case of fractional...

  17. Subluminous Wolf-Rayet stars: Observations

    International Nuclear Information System (INIS)

    Heap, S.R.

    1982-01-01

    The author has used the fact that some central stars are WR stars and others are say, O stars, as a focal point for his presentation. In attempting to answer this question he has considered how the properties of WR-type central stars differ from those of O-type stars. The study begins with the classification and calibration of WR spectra, then goes on to the physical properties of WR-type central stars, and at the end returns to the question of what distinguishes a Wolf-Rayet star. The observational data for central stars are neither complete nor precise. Nevertheless, they suggest that what distinguishes a WR central star is not so much its present physical properties (e.g. temperature, gravity), but rather, its fundamental properties (initial and evolutionary history). (Auth.)

  18. Supernovae from Wolf-Rayet stars

    International Nuclear Information System (INIS)

    Schaeffer, R.

    1986-01-01

    Wolf-Rayet stars are known to originate from the most massive stars. Under the assumption that these stripped stars explode at the end of their evolution through the same instability mechanism as type II supernovae, we calculate their light curve. The latter is found to be quite similar to the typical SN I light curves but is fainter by about 2 magnitudes. A detailed study of its shape leads to identify the WR supernovae with the SNIp (or SNIb) subclass. The more massive WR stars should explode via the e + e - pair production mechanism, with negligible 56 Ni formation. Their rather dim light curve is predicted to have a ∼ 2 month plateau and afterwards a very sharp decline. A delayed manifestation of such an event might be the Cas A remnant

  19. The Klynac: An integrated klystron and linear accelerator

    International Nuclear Information System (INIS)

    Potter, James M.; Schwellenbach, David; Meidinger, Alfred

    2013-01-01

    The Klynac concept integrates an electron gun, a radio frequency (RF) power source, and a coupled-cavity linear accelerator into a single resonant system. The klystron is essentially a conventional klystron structure with an input cavity, some number of intermediate cavities and an output cavity. The accelerator structure is, likewise, a conventional on-axis coupled structure. The uniqueness is the means of coupling the klystron output cavity to the accelerator. The coupler is a resonant coupler rather than an ordinary transmission line. The geometry of such a system need not be coaxial. However, if the klystron and accelerator are coaxial we can eliminate the need for a separate cathode for the accelerator by injecting some of the klystron beam into the accelerator. Such a device can be made cylindrical which is ideal for some applications.

  20. Differential wolf-pack-size persistence and the role of risk when hunting dangerous prey

    Science.gov (United States)

    Barber-Meyer, Shannon M.; Mech, L. David; Newton, Wesley E.; Borg, Bridget

    2016-01-01

    Risk to predators hunting dangerous prey is an emerging area of research and could account for possible persistent differences in gray wolf (Canis lupus) pack sizes. We documented significant differences in long-term wolf-pack-size averages and variation in the Superior National Forest (SNF), Denali National Park and Preserve, Yellowstone National Park, and Yukon, Canada (pwolves’ risk when hunting primary prey, for those packs (N=3) hunting moose (Alces americanus) were significantly larger than those (N=10) hunting white-tailed deer (Odocoileus virginianus) (F1,8=16.50, p=0.004). Our data support the hypothesis that differential pack-size persistence may be perpetuated by differences in primary prey riskiness to wolves, and we highlight two important extensions of this idea: (1) the potential for wolves to provision and defend injured packmates from other wolves and (2) the importance of less-risky, buffer prey to pack-size persistence and year-to-year variation. Risk to predators hunting dangerous prey is an emerging area of research and could account for possible persistent differences in gray wolf (Canis lupus) pack sizes. We documented significant differences in long-term wolf-pack-size averages and variation in the Superior National Forest (SNF), Denali National Park and Preserve, Yellowstone National Park, and Yukon, Canada (pwolves’ risk when hunting primary prey, for those packs (N=3) hunting moose (Alces americanus) were significantly larger than those (N=10) hunting white-tailed deer (Odocoileus virginianus) (F1,8=16.50, p=0.004). Our data support the hypothesis that differential pack-size persistence may be perpetuated by differences in primary prey riskiness to wolves, and we highlight two important extensions of this idea: (1) the potential for wolves to provision and defend injured packmates from other wolves and (2) the importance of less-risky, buffer prey to pack-size persistence and year-to-year variation.

  1. Medea in the past and in the present: (Reconstructing the identity of a woman and a foreigner in the novel Medea by Christa Wolf

    Directory of Open Access Journals (Sweden)

    Stevanović Lada

    2010-01-01

    Full Text Available One of the most shocking, the most difficult to understand and at the same time the most inspiring tragedy from the Greek antiquity is Euripeides' Medea. This drama has a rich and a long history of adaptations that have always represented an interface between the ancient text on the one hand, and the author's reading on the other, which always depended on cultural and political context of the period that influenced the perspective of the author. Most of these adaptations belong to theatrical production. However my interest in this paper is not oriented towards theatre, but towards literature, a novel Medea: A Modern Retelling by Christa Wolf. First part of the paper deals with the reconstruction of the ancient context and meaning of this drama in the antiquity from the perspective of contemporary study of classics, while the second part is devoted to the questions of Wolf's adaptation: the ways in which Christa Wolf rewrites Medea, tracing of Wolf's reasons to approach the text in such a way, the main relation of Wolf's version to former version(s, and intellectual and social climate in which Wolf's Medea was created. The focus of the research will be on the reading of the construction of the Other, of gender and gender roles, also through the analysis of their transgression - in the Greek antiquity when Euripides drama was staged, as well in the Germen (European context of the end of the twentieth century in which Medea was rewritten by Christa Wolf.

  2. Wolf-Rayet stars and O-star runaways with HIPPARCOS - I. Kinematics

    NARCIS (Netherlands)

    Moffat, AFJ; Marchenko, SV; Seggewiss, W; van der Hucht, KA; Schrijver, H; Stenholm, B; Lundstrom, [No Value; Gunawan, DYAS; Sutantyo, W; van den Heuvel, EPJ; De Cuyper, JP; Gomez, AE

    Reliable systemic radial velocities are almost impossible to secure for Wolf-Rayet stars, difficult for O stars. Therefore, to study the motions - both systematic in the Galaxy and peculiar - of these two related types of hot, luminous star, we have examined the Hipparcos proper motions of some 70

  3. Wolf-Rayet stars and O-star runaways with HIPPARCOS - II. Photometry

    NARCIS (Netherlands)

    Marchenko, SV; Moffat, AFJ; van der Hucht, KA; Seggewiss, W; Schrijver, H; Stenholm, B; Lundstrom, [No Value; Setia Gunawan, DYA; Sutantyo, W; van den Heuvel, EPJ; De Cuyper, JP; Gomez, AE

    Abundant HIPPARCOS photometry over 3 years of 141 O and Wolf-Rayet stars, including 8 massive X-ray binaries, provides a magnificent variety of light curves at the sigma similar to 1-5% level. Among the most interesting results, we mention: optical outbursts in HD 102567 (MXRB), coinciding with

  4. Transfer of the uranium decay products, polonium-210 and lead-210, through the lichen-cariboo-wolf food chain in northern Canada. Manuscript report No. MR18-91

    Energy Technology Data Exchange (ETDEWEB)

    Thomas, P A

    1991-01-01

    Study to investigate the accumulation and transfer of polonium-210 and lead-210 in the Arctic food chain lichen-caribou-wolf in the Northwest Territories. With the participation of the hunters of Baker Lake, caribou and wolf samples were collected and analyzed for polonium. The level of polonium-210 was determined in lichen, several caribou tissues, and several wolf tissues. Transfer coefficients were derived between trophic levels in the food chain and the Po-210:PB-210 ratios for lichens and selected tissues in caribou and wolf were determined.

  5. Demographic effects of canine parvovirus on a free-ranging wolf population over 30 years

    Science.gov (United States)

    Mech, L.D.; Goyal, S.M.; Paul, W.J.; Newton, W.E.

    2008-01-01

    We followed the course of canine parvovinis (CPV) antibody prevalence in a subpopulation of wolves (Canis 1upus) in northeastern Minnesota from 1973, when antibodies were first detected, through 2004. Annual early pup survival was reduced by 70%, and wolf population change was related to CPV antibody prevalence. In the greater Minnesota population of 3,000 wolves, pup survival was reduced by 40-60%. This reduction limited the Minnesota wolf population rate of increase to about 4% per year compared with increases of 16-58% in other populations. Because it is young wolves that disperse, reduced pup survival may have caused reduced dispersal and reduced recolonization of new range in Minnesota. ?? Wildlife Disease Association 2008.

  6. The close binary frequency of Wolf-Rayet stars as a function of metallicity in M31 and M33

    Energy Technology Data Exchange (ETDEWEB)

    Neugent, Kathryn F.; Massey, Philip, E-mail: kneugent@lowell.edu, E-mail: phil.massey@lowell.edu [Lowell Observatory, 1400 West Mars Hill Road, Flagstaff, AZ 86001 (United States)

    2014-07-01

    Massive star evolutionary models generally predict the correct ratio of WC-type and WN-type Wolf-Rayet stars at low metallicities, but underestimate the ratio at higher (solar and above) metallicities. One possible explanation for this failure is perhaps single-star models are not sufficient and Roche-lobe overflow in close binaries is necessary to produce the 'extra' WC stars at higher metallicities. However, this would require the frequency of close massive binaries to be metallicity dependent. Here we test this hypothesis by searching for close Wolf-Rayet binaries in the high metallicity environments of M31 and the center of M33 as well as in the lower metallicity environments of the middle and outer regions of M33. After identifying ∼100 Wolf-Rayet binaries based on radial velocity variations, we conclude that the close binary frequency of Wolf-Rayets is not metallicity dependent and thus other factors must be responsible for the overabundance of WC stars at high metallicities. However, our initial identifications and observations of these close binaries have already been put to good use as we are currently observing additional epochs for eventual orbit and mass determinations.

  7. A fresh look at the wolf-pack theory of companion-animal dog social behavior.

    Science.gov (United States)

    van Kerkhove, Wendy

    2004-01-01

    A popular perspective on the social behavior of dogs in multiple-dog households sees the dogs' behavior as reflecting the sociobiological laws of the rigidly structured dominance hierarchy that has been described for wolf packs. This view suggests that aggression problems among dogs are natural expressions of conflict that arise whenever dominance status is in contention. One recommended solution has been for the owner to endorse and enforce a particular dominance hierarchy because, on the wolf pack model, aggression is minimized when the structure of the hierarchy is clear, strong, and stable. This article questions the validity of this perspective on 2 principal grounds. First, because it does not seem to occur in the wild, this article suggests the strong dominance hierarchy that has been described for wolves may be a by-product of captivity. If true, it implies that social behavior--even in wolves--may be a product more of environmental circumstances and contingencies than an instinctive directive. Second, because feral dogs do not exhibit the classic wolf-pack structure, the validity of the canid, social dominance hierarchy again comes into question. This article suggests that behavioral learning theory offers another perspective regarding the behavior of dogs and wolves in the wild or in captivity and offers an effective intervention for aggression problems.

  8. Efficacy Quotient Tindakan ESWL Piezolith Richard Wolf 3000 pada Penderita Batu Ureter di RSUPN Dr. Cipto Mangunkusumo, 2008–2011

    Directory of Open Access Journals (Sweden)

    Vinny Verdini

    2017-01-01

    Full Text Available Extracorporeal shockwave lithotripsy (ESWL saat ini telah menjadi metode yang paling umum dalam tatalaksana aktif batu ureter. Sejak Maret 2008, RSCM telah menggunakan mesin ESWL piezolith 3000 richard wolf dan belum diketahui nilai efficacy quotient (EQ. Penelitian ini bertujuan untuk menentukan nilai EQ dari tindakan ESWL menggunakan mesin piezolith richard wolf 3000 pada batu ureter dan hubungan angka bebas batu dengan lokasi batu, jumlah batu, beban batu, opasitas batu, obstruksi, dan fungsi ginjal. Studi cross sectional ini dilakukan pada bulan Januari 2008-Desember 2011 dan data dianalisis dengan statistik multivariat. Terdapat 113 (95 % dari 119 pasien yang dinyatakan bebas batu setelah tindakan ESWL pertama. Didapatkan nilai EQ 0,89. Hanya ukuran batu yang mempengaruhi angka bebas batu dalam penelitian ini (p<0,05. Disimpulkan bahwa prosedur ESWL menggunakan mesin richard wolf piezolith 3000 memiliki nilai EQ dan angka bebas batu yang lebih baik daripada mesin-mesin sebelumnya dan mesin lain yang sejenis. Faktor yang mempengaruhi keberhasilan adalah ukuran batu ureter yang ditatalaksana.Kata Kunci: batu ureter, ESWL, efficacy quotient, angka bebas batu. Efficacy Quotient of ESWL Piezolith Richard Wolf 3000 Machine in Patientswith Ureteral Stones in Dr. Cipto MangunkusumoNational Hospital 2008 - 2011AbstractExtracorporeal shockwave lithotripsy (ESWL is the most common method of ureteral stone management. Since 2008, RSCM has ben using ESWL piezolith 3000 richard wolf and efficacy quotient (EQ value have not yet studied. The study aims was to determine the efficacy quotient (EQ of ESWL using piezolith richard wolf 3000 machine for ureteral stone by analyzing free-stone rate with location of stones, number of stones, stone burden, stone opacity, obstruction and kidney function. This cross sectional study was carried out in January 2008-December 2011, with multivariate analytical study. Ninety five percent (n=113 of 119 patients were

  9. Quantum density fluctuations in liquid neon from linearized path-integral calculations

    International Nuclear Information System (INIS)

    Poulsen, Jens Aage; Scheers, Johan; Nyman, Gunnar; Rossky, Peter J.

    2007-01-01

    The Feynman-Kleinert linearized path-integral [J. A. Poulsen et al., J. Chem. Phys. 119, 12179 (2003)] representation of quantum correlation functions is applied to compute the spectrum of density fluctuations for liquid neon at T=27.6 K, p=1.4 bar, and Q vector 1.55 Aa -1 . The calculated spectrum as well as the kinetic energy of the liquid are in excellent agreement with the experiment of Cunsolo et al. [Phys. Rev. B 67, 024507 (2003)

  10. Identification of novel mutations in HEXA gene in children affected with Tay Sachs disease from India.

    Directory of Open Access Journals (Sweden)

    Mehul Mistri

    Full Text Available Tay Sachs disease (TSD is a neurodegenerative disorder due to β-hexosaminidase A deficiency caused by mutations in the HEXA gene. The mutations leading to Tay Sachs disease in India are yet unknown. We aimed to determine mutations leading to TSD in India by complete sequencing of the HEXA gene. The clinical inclusion criteria included neuroregression, seizures, exaggerated startle reflex, macrocephaly, cherry red spot on fundus examination and spasticity. Neuroimaging criteria included thalamic hyperdensities on CT scan/T1W images of MRI of the brain. Biochemical criteria included deficiency of hexosaminidase A (less than 2% of total hexosaminidase activity for infantile patients. Total leukocyte hexosaminidase activity was assayed by 4-methylumbelliferyl-N-acetyl-β-D-glucosamine lysis and hexosaminidase A activity was assayed by heat inactivation method and 4-methylumbelliferyl-N-acetyl-β-D-glucosamine-6-sulphate lysis method. The exons and exon-intron boundaries of the HEXA gene were bidirectionally sequenced using an automated sequencer. Mutations were confirmed in parents and looked up in public databases. In silico analysis for mutations was carried out using SIFT, Polyphen2, MutationT@ster and Accelrys Discovery Studio softwares. Fifteen families were included in the study. We identified six novel missense mutations, c.340 G>A (p.E114K, c.964 G>A (p.D322N, c.964 G>T (p.D322Y, c.1178C>G (p.R393P and c.1385A>T (p.E462V, c.1432 G>A (p.G478R and two previously reported mutations. c.1277_1278insTATC and c.508C>T (p.R170W. The mutation p.E462V was found in six unrelated families from Gujarat indicating a founder effect. A previously known splice site mutation c.805+1 G>C and another intronic mutation c.672+30 T>G of unknown significance were also identified. Mutations could not be identified in one family. We conclude that TSD patients from Gujarat should be screened for the common mutation p.E462V.

  11. Identification of novel mutations in HEXA gene in children affected with Tay Sachs disease from India.

    Science.gov (United States)

    Mistri, Mehul; Tamhankar, Parag M; Sheth, Frenny; Sanghavi, Daksha; Kondurkar, Pratima; Patil, Swapnil; Idicula-Thomas, Susan; Gupta, Sarita; Sheth, Jayesh

    2012-01-01

    Tay Sachs disease (TSD) is a neurodegenerative disorder due to β-hexosaminidase A deficiency caused by mutations in the HEXA gene. The mutations leading to Tay Sachs disease in India are yet unknown. We aimed to determine mutations leading to TSD in India by complete sequencing of the HEXA gene. The clinical inclusion criteria included neuroregression, seizures, exaggerated startle reflex, macrocephaly, cherry red spot on fundus examination and spasticity. Neuroimaging criteria included thalamic hyperdensities on CT scan/T1W images of MRI of the brain. Biochemical criteria included deficiency of hexosaminidase A (less than 2% of total hexosaminidase activity for infantile patients). Total leukocyte hexosaminidase activity was assayed by 4-methylumbelliferyl-N-acetyl-β-D-glucosamine lysis and hexosaminidase A activity was assayed by heat inactivation method and 4-methylumbelliferyl-N-acetyl-β-D-glucosamine-6-sulphate lysis method. The exons and exon-intron boundaries of the HEXA gene were bidirectionally sequenced using an automated sequencer. Mutations were confirmed in parents and looked up in public databases. In silico analysis for mutations was carried out using SIFT, Polyphen2, MutationT@ster and Accelrys Discovery Studio softwares. Fifteen families were included in the study. We identified six novel missense mutations, c.340 G>A (p.E114K), c.964 G>A (p.D322N), c.964 G>T (p.D322Y), c.1178C>G (p.R393P) and c.1385A>T (p.E462V), c.1432 G>A (p.G478R) and two previously reported mutations. c.1277_1278insTATC and c.508C>T (p.R170W). The mutation p.E462V was found in six unrelated families from Gujarat indicating a founder effect. A previously known splice site mutation c.805+1 G>C and another intronic mutation c.672+30 T>G of unknown significance were also identified. Mutations could not be identified in one family. We conclude that TSD patients from Gujarat should be screened for the common mutation p.E462V.

  12. Spontaneous appearance of Tay-Sachs disease in an animal model.

    Science.gov (United States)

    Zeng, B J; Torres, P A; Viner, T C; Wang, Z H; Raghavan, S S; Alroy, J; Pastores, G M; Kolodny, E H

    2008-01-01

    Tay-Sachs disease (TSD) is a progressive neurodegenerative disorder due to an autosomal recessively inherited deficiency of beta-hexosaminidase A (Hex A). Deficiency of Hex A in TSD is caused by a defect of the alpha-subunit resulting from mutations of the HEXA gene. To date, there is no effective treatment for TSD. Animal models of genetic diseases, similar to those known to exist in humans, are valuable and essential research tools for the study of potentially effective therapies. However, there is no ideal animal model of TSD available for use in therapeutic trials. In the present study, we report an animal model (American flamingo; Phoenicopterus ruber) of TSD with Hex A deficiency occurring spontaneously in nature, with accumulation of G(M2)-ganglioside, deficiency of Hex A enzymatic activity, and a homozygous P469L mutation in exon 12 of the hexa gene. In addition, we have isolated the full-length cDNA sequence of the flamingo, which consists of 1581 nucleotides encoding a protein of 527 amino acids. Its coding sequence indicates approximately 71% identity at the nucleotide level and about 72.5% identity at the amino acid level with the encoding region of the human HEXA gene. This animal model, with many of the same features as TSD in humans, could represent a valuable resource for investigating therapy of TSD.

  13. Hemiterpenoids and Pyrazines in the Odoriferous Urine of the Maned Wolf

    NARCIS (Netherlands)

    Goodwin, T.E.; Songsasen, N.; Broederdorf, L.J.; Burkert, B.A.; Joi Chen, C.; Jackson, S.R.; Keplinger, K.B.; Rountree, M.E.; Waldrip, Z.J.; Wedell, M.E.; Desrochers, L.P.; Baker, W.K.; Helsper, J.P.F.G.

    2013-01-01

    Maned wolves ( Chrysocyon brachyurus ) are endemic to South America, monogamous, solitary, and threatened in the wild. Maned wolf urine has a pungent and powerful odour and is used for scent marking. There is evidence to suggest that the presence of a male may be required to initiate oestrus and/or

  14. Winter wolf predation in a multiple ungulate prey system, Gates of the Arctic National Park, Alaska

    Science.gov (United States)

    Dale, Bruce W.; Adams, Layne G.; Bowyer, R. Terry; Carbyn, Ludwig N.; Fritts, Steven H.; Seip, Dale R.

    1995-01-01

    We investigated patterns of winter wolf predation, including prey selection, prey switching, kill rates, carcass utilization, and consumption rates for four wolf packs during three different study periods (March 1989, March 1990, and November 1990) in Gates of the Arctic National Park and Preserve, Alaska. Wolves killed predominantly caribou (165 caribou, seven moose, and five Dall sheep) even when moose and sheep were more abundant. Prey selection varied between study periods. More moose were killed in march 1989, a particularly deep snow year, and more sheep were killed in November 1990 than during other periods. Overall kill rates ranged from 0-8 days/ungulate killed (x̅ = 2.0, SD = 1.6) and did not vary between study periods.  Pack size and species killed explained significant variation in the length of time intervals between kills. Although caribou density varied nearly 40-fold between pack territories, it had little influence on predation characteristics except at low densities, when kill rates may have declined. Caribou distribution had marked effects on wolf predation rate.

  15. Estimation of non-linear continuous time models for the heat exchange dynamics of building integrated photovoltaic modules

    DEFF Research Database (Denmark)

    Jimenez, M.J.; Madsen, Henrik; Bloem, J.J.

    2008-01-01

    This paper focuses on a method for linear or non-linear continuous time modelling of physical systems using discrete time data. This approach facilitates a more appropriate modelling of more realistic non-linear systems. Particularly concerning advanced building components, convective and radiati...... that a description of the non-linear heat transfer is essential. The resulting model is a non-linear first order stochastic differential equation for the heat transfer of the PV component....... heat interchanges are non-linear effects and represent significant contributions in a variety of components such as photovoltaic integrated facades or roofs and those using these effects as passive cooling strategies, etc. Since models are approximations of the physical system and data is encumbered...

  16. Wolf-Hirschhorn syndrome (WHS): a history in pictures.

    Science.gov (United States)

    Battaglia, A; Carey, J C; Viskochil, D H; Cederholm, P; Opitz, J M

    2000-01-01

    The Wolf-Hirschhorn syndrome (WHS) is a well known chromosomal disorder, due to a deletion of distal chromosome 4p. The classical gestalt is striking and poses few diagnostic problems. However, due to the difficulty of detecting very small deletions by standard cytogenetics, diagnosis can be sometimes very difficult, particularly in older patients. In this paper we show the changes, occurring over time, in facial appearance of affected individuals, to improve insight into the evolution of the phenotype, and to increase its diagnostic potential.

  17. A new ejecta shell surrounding a Wolf-Rayet star in the LMC

    Science.gov (United States)

    Garnett, Donald R.; Chu, You-Hua

    1994-01-01

    We have obtained CCD spectra of newly discovered shell-like nebulae around the WN4 star Breysacher 13 and the WN1 star Breysacher 2 in the Large Magellanic Cloud (LMC). The shell around Br 13 shows definite signs of enrichment in both nitrogen and helium, having much stronger (N II) and He I emission lines than are seen in typical LMC H II regions. From the measured electron temperature of about 17,000 K in the shell, we derive He/H and N/O abundance ratios which are factors of 2 and more than 10 higher, respectively, than the average LMC interstellar values. The derived oxygen abundance in the Br 13 shell is down by a factor of 8 compared to the local LMC interstellar medium (ISM); however, the derived electron temperature is affected by the presence of an incomplete shock arising from the interaction of the stellar wind with photoionized material. This uncertainty does not affect the basic conclusion that the Br 13 shell is enriched by processed material from the Wolf-Rayet star. In contrast, the shell around Br 2 shows no clear evidence of enrichment. The nebular spectrum is characterized by extremely strong (O III) and He II emission and very weak (N II). We derive normal He, O, and N abundances from our spectrum. This object therefore appears to be simply a wind-blown structure associated with a relatively dense cloud near the Wolf-Rayet star, although the very high-ionization state of the gas is unusual for a nebula associated with a Wolf-Rayet star.

  18. Corrections for hydrostatic atmospheric models: radii and effective temperatures of Wolf Rayet stars

    International Nuclear Information System (INIS)

    Loore, C. de; Hellings, P.; Lamers, H.J.G.L.M.

    1982-01-01

    With the assumption of plane-parallel hydrostatic atmospheres, used generally for the computation of evolutionary models, the radii of WR stars are seriously underestimated. The true atmospheres may be very extended, due to the effect of the stellar wind. Instead of these hydrostatic atmospheres the authors consider dynamical atmospheres adopting a velocity law. The equation of the optical depth is integrated outwards using the equation of continuity. The ''hydrostatic'' radii are to be multiplied with a factor 2 to 8, and the effective temperatures with a factor 0.8 to 0.35 when Wolf Rayet characteristics for the wind are considered, and WR mass loss rates are used. With these corrections the effective temperatures of the theoretical models, which are helium burning Roche lobe overflow remnants, range between 30,000 K and 50,000 K. Effective temperatures calculated in the hydrostatic hypothesis can be as high as 150,000 K for helium burning RLOF-remnants with WR mass loss rates. (Auth.)

  19. Preserving Simplecticity in the Numerical Integration of Linear Beam Optics

    Energy Technology Data Exchange (ETDEWEB)

    Allen, Christopher K. [Oak Ridge National Lab. (ORNL), Oak Ridge, TN (United States)

    2017-07-01

    Presented are mathematical tools and methods for the development of numerical integration techniques that preserve the symplectic condition inherent to mechanics. The intended audience is for beam physicists with backgrounds in numerical modeling and simulation with particular attention to beam optics applications. The paper focuses on Lie methods that are inherently symplectic regardless of the integration accuracy order. Section 2 provides the mathematically tools used in the sequel and necessary for the reader to extend the covered techniques. Section 3 places those tools in the context of charged-particle beam optics; in particular linear beam optics is presented in terms of a Lie algebraic matrix representation. Section 4 presents numerical stepping techniques with particular emphasis on a third-order leapfrog method. Section 5 discusses the modeling of field imperfections with particular attention to the fringe fields of quadrupole focusing magnets. The direct computation of a third order transfer matrix for a fringe field is shown.

  20. Hungry Unlike the Wolf: Ecology, Posthumanism, Narratology in Fred Vargas’s Seeking Whom He May Devour

    Directory of Open Access Journals (Sweden)

    John Parham

    2012-01-01

    Full Text Available This paper examines posthumanism as a philosophical position equipped to inform ecocriticism and the potential of popular fiction to articulate ecological complexity. Posthumanism will be reappraised as a dialectical model that decentres the human in relation to ‘evolutionary, ecological, or technological coordinates’ (Wolfe 2010: xvi while nevertheless retaining a sense of the integrity of, and boundaries between, human and nonhuman species or phenomena. It will be argued that a novelistic emphasis on human being, agency, and action, coupled with devices of genre, plot, atnd narrative – are consistent with the process of human self‐examinaion engendered by posthumanism. The essay will, thereafter, illustrate and examine this approach through the French crime writer Fred Vargas’s1999 novel Seeking Whom He May Devour. Identifying two human protagonists – the Canadian conservationist Johnstone and his girlfriend Camille – an initial decentring of the human subject will be examined in relation to two equivalent, nonhuman protagonists, the French Alps and the wolf. Utilising newspaper interviews that highlight Vargas’s own posthumanist perspective (grounded in her profession as an archaeologist, I will examine a how the novel explores appropriate relationships between human and nonhuman animals; b how Vargas utilises both the generic features of the crime novel – e.g. the resolution of a ‘crime’ – and the subtle narrative manipulations of character focalisation to construct (via the preferred ‘point of iew’ offered by Camille a posthumanist position on human/animal relations which argas explicitly opposes to the inhumanism represented by Johnstone.

  1. Modified Discrete Grey Wolf Optimizer Algorithm for Multilevel Image Thresholding

    Directory of Open Access Journals (Sweden)

    Linguo Li

    2017-01-01

    Full Text Available The computation of image segmentation has become more complicated with the increasing number of thresholds, and the option and application of the thresholds in image thresholding fields have become an NP problem at the same time. The paper puts forward the modified discrete grey wolf optimizer algorithm (MDGWO, which improves on the optimal solution updating mechanism of the search agent by the weights. Taking Kapur’s entropy as the optimized function and based on the discreteness of threshold in image segmentation, the paper firstly discretizes the grey wolf optimizer (GWO and then proposes a new attack strategy by using the weight coefficient to replace the search formula for optimal solution used in the original algorithm. The experimental results show that MDGWO can search out the optimal thresholds efficiently and precisely, which are very close to the result examined by exhaustive searches. In comparison with the electromagnetism optimization (EMO, the differential evolution (DE, the Artifical Bee Colony (ABC, and the classical GWO, it is concluded that MDGWO has advantages over the latter four in terms of image segmentation quality and objective function values and their stability.

  2. Tubulopapillary carcinoma of the mammary gland in a maned wolf (Chrysocyon brachyurus: histopathological and immunophenotypical analysis

    Directory of Open Access Journals (Sweden)

    C.O. Gamba

    2011-12-01

    Full Text Available A maned female wolf (Chrysocyon brachyurus showed nodules in the inguinal and left abdominal cranial mammary glands. The mammary gland was surgically excised, and microscopic analysis revealed epithelial cell proliferation in a tubular and papillary pattern; delicate fibrovascular stalks presenting numerous layers of moderately pleomorfic epithelial cells were observed. This histologic appearance was compatible with a diagnosis of mammary tubulopapillary carcinoma. The immunohistochemical profile revealed nuclear positivity for estrogen (70% and progesterone (at least 90% of the neoplastic cells. The myoepithelium-associated with neoplastic cells lacked integrity, as evidenced by failed smooth muscle alpha actin reactivity in microinvasive areas. A low proliferation index was observed (3.4%. To the authors' knowledge, the present case represents the first finding of female tubulopapillary carcinoma in a mammary gland in this species.

  3. Direct linearizing transform for three-dimensional discrete integrable systems: the lattice AKP, BKP and CKP equations.

    Science.gov (United States)

    Fu, Wei; Nijhoff, Frank W

    2017-07-01

    A unified framework is presented for the solution structure of three-dimensional discrete integrable systems, including the lattice AKP, BKP and CKP equations. This is done through the so-called direct linearizing transform, which establishes a general class of integral transforms between solutions. As a particular application, novel soliton-type solutions for the lattice CKP equation are obtained.

  4. The use of a path independent integral in non-linear fracture mechanics

    International Nuclear Information System (INIS)

    Hellen, T.K.

    1977-01-01

    A computer program for calculating the J and J* integrals has been developed as an extension to the BERSAFE finite element system. A full analysis of the cracked structure including plasticity, creep and thermal strains is conducted and the results are stored on a permanent data set. The integral values may then be calculated using the post-processor program for any number of contours and load or time steps, without recourse to further expensive computations. Numerical examples are presented comparing the J and J* integrals for a number of cracked plates under thermal, plastic and creep environments. To demonstrate the accuracy for a simple thermo-elastic case, a centre cracked plate subject to a symmetric quadratic gradient is included. Here, the J integral is shown to be path dependent whereas good independence is seen for the J* integral. The case of an elastic-plastic plate is invetigated to demonstrate path independence for both integrals in non-linear elasticity, and the effects of unloading are discussed. An alternative method for obtaining the change of potential energy over a small crack extension is briefly mentioned and compared to the J and J* results in this case. An axisymmetric bar with an internal penny-shaped crack subjected to tension is discussed under elastic-plastic materials behavior

  5. Hemorrhagic and necrotizing hepatitis associated with administration of a modified live canine adenovirus-2 vaccine in a maned wolf (Chrysocyon brachyurus).

    Science.gov (United States)

    Swenson, Julie; Orr, Kathryn; Bradley, Gregory A

    2012-06-01

    A 15-yr-old, female, maned wolf (Chrysocyon brachyurus) was euthanized after presenting semicomatose with severe, uncontrolled frank hemorrhage from her rectum 6 days following a routine physical examination and vaccination. Histopathology indicated severe hemorrhagic and necrotizing hepatitis with intranuclear basophilic inclusion bodies in the liver that were thought to be consistent with adenoviral infection. Further classification by polymerase chain reaction, immunohistochemical staining, virus isolation, and electron microscopy confirmed the etiologic agent to be canine adenovirus-2. A representative sample of the vaccine that had been used was submitted and sequenced along with the virus isolated from the maned wolf. The sequencing of the etiologic agent that had been isolated from the maned wolf was determined to be the same as the strain of virus used in the production of the modified live vaccine that had been administered 6 days prior to death. From this information, the diagnosis of vaccine-induced adenoviral hepatitis was made. This is the first confirmed case of vaccine-induced canine adenoviral hepatitis in a maned wolf.

  6. GMRT Low Radio Frequency Study of the Wolf Rayet Galaxy NGC ...

    Indian Academy of Sciences (India)

    In this paper, we present the first low frequency (< 1.4 GHz) radio continuum study of a Wolf Rayet galaxy NGC 4214 using the Giant Meterwave Radio Telescope (GMRT). We detect diffuse extended emission from the galaxy disk at 325 MHz and find that the radio emission closely follows the ultraviolet emission mapped by ...

  7. GMRT Low Radio Frequency Study of the Wolf Rayet Galaxy NGC ...

    Indian Academy of Sciences (India)

    Abstract. In this paper, we present the first low frequency (< 1.4 GHz) radio continuum study of a Wolf Rayet galaxy NGC 4214 using the. Giant Meterwave Radio Telescope (GMRT). We detect diffuse extended emission from the galaxy disk at 325 MHz and find that the radio emis- sion closely follows the ultraviolet emission ...

  8. ISO-SWS spectrophotometry of galactic Wolf-Rayet stars: preliminary results.

    NARCIS (Netherlands)

    van der Hucht, K. A.; Morris, P. W.; Williams, P. M.; Setia Gunawan, D. Y. A.; Beintema, D. A.; Boxhoorn, D. R.; de Graauw, T.; Heras, A.; Kester, D. J. M.; Lahuis, F.; Leech, K. J.; Roelfsema, P. R.; Salama, A.; Valentijn, E. A.; Vandenbussche, B.

    1996-01-01

    ISO-SWS spectra of seven late-type galactic Wolf-Rayet stars are discussed. A high resolution spectrum (2.3-29.6μm, λ/{DELTA}λ=~820-1700) of the WC8 star WR11 (γ^2^ Vel) is shown and its Ne abundance is discussed. Medium resolution spectra (λ/{DELTA}λ=~250-600) of the WC8-9 stars WR48a, WR98a,

  9. Survey of selected pathogens and blood parameters of northern yellowstone elk: Wolf sanitation effect implications

    Science.gov (United States)

    Barber-Meyer, S. M.; White, P.J.; Mech, L.D.

    2007-01-01

    The restoration or conservation of predators could reduce seroprevalences of certain diseases in prey if predation selectively removes animals exhibiting clinical signs. We assessed disease seroprevalences and blood parameters of 115 adult female elk (Cervus elaphus) wintering on the northern range of Yellowstone National Park [YNP] during 2000-2005 and compared them to data collected prior to wolf (Canis lupus) restoration (WR) in 1995 and to two other herds in Montana to assess this prediction. Blood parameters were generally within two standard deviations of the means observed in other Montana herds (Gravelly-Snowcrest [GS] and Garnet Mountain [GM]), but Yellowstone elk had higher seroprevalences of parainfluenza-3 virus (95% CI YNP = 61.1-78.6, GS = 30.3-46.5) and bovine-virus-diarrhea virus type 1 (95% CI YNP = 15.9-31.9, GM = 0). In comparisons between pre-wolf restoration [pre-WR] (i.e., prior to 1995) seroprevalences with those post-wolf restoration [post-WR] in Yellowstone, we found lower seroprevalences for some disease-causing agents post-wolf restoration (e.g., bovine-virus-diarrhea virus type-1 [95% CI pre-WR = 73.1-86.3, post-WR = 15.9-31.9] and bovine-respiratory syncytial virus [95% CI pre-WR = 70.0-83.8, post-WR = 0]), but similar (e.g., Brucella abortus [95% CI pre-WR = 0-4.45, post-WR = 0-4.74] and epizootic hemorrhagic disease virus [95% CI pre-WR = 0, post-WR = 0]) or higher for others (e.g., Anaplasma marginale [95% CI pre-WR = 0, post-WR = 18.5-38.7] and Leptospira spp. [95% CI pre-WR = 0.5-6.5, post-WR = 9.5-23.5]). Though we did not detect an overall strong predation effect through reduced disease seroprevalence using retrospective comparisons with sparse data, our reference values will facilitate future assessments of this issue.

  10. The first family with Tay-Sachs disease in Cyprus: Genetic analysis reveals a nonsense (c.78G>A) and a silent (c.1305C>T) mutation and allows preimplantation genetic diagnosis.

    Science.gov (United States)

    Georgiou, Theodoros; Christopoulos, George; Anastasiadou, Violetta; Hadjiloizou, Stavros; Cregeen, David; Jackson, Marie; Mavrikiou, Gavriella; Kleanthous, Marina; Drousiotou, Anthi

    2014-12-01

    Tay-Sachs disease (TSD) is a recessively inherited neurodegenerative disorder caused by mutations in the HEXA gene resulting in β-hexosaminidase A (HEX A) deficiency and neuronal accumulation of GM2 ganglioside. We describe the first patient with Tay-Sachs disease in the Cypriot population, a juvenile case which presented with developmental regression at the age of five. The diagnosis was confirmed by measurement of HEXA activity in plasma, peripheral leucocytes and fibroblasts. Sequencing the HEXA gene resulted in the identification of two previously described mutations: the nonsense mutation c.78G>A (p.Trp26X) and the silent mutation c.1305C>T (p.=). The silent mutation was reported once before in a juvenile TSD patient of West Indian origin with an unusually mild phenotype. The presence of this mutation in another juvenile TSD patient provides further evidence that it is a disease-causing mutation. Successful preimplantation genetic diagnosis (PGD) and prenatal follow-up were provided to the couple.

  11. The origin of Wolf-Rayet stars

    International Nuclear Information System (INIS)

    Doom, C.

    1987-01-01

    The paper reviews the origin of Wolf-Rayet (WR) stars, with emphasis on the so-called Population I WR stars which are associated with the young and luminous stellar population. A description is given of the observational characteristics i.e. classification, luminosities composition, etc. of WR stars. The origin and evolution of WR stars is described, including the single, binary, subtypes and ratio WR/O. The interaction of the WR stars with their environment is discussed with respect to the energy deposition and composition anomalies. A brief account of the discovery of WR stars in other galaxies is given. Finally, some of the main issues in the research into the structure and evolution of WR stars are outlined. (U.K.)

  12. The COBE normalization for standard cold dark matter

    Science.gov (United States)

    Bunn, Emory F.; Scott, Douglas; White, Martin

    1995-01-01

    The Cosmic Background Explorer Satellite (COBE) detection of microwave anisotropies provides the best way of fixing the amplitude of cosmological fluctuations on the largest scales. This normalization is usually given for an n = 1 spectrum, including only the anisotropy caused by the Sachs-Wolfe effect. This is certainly not a good approximation for a model containing any reasonable amount of baryonic matter. In fact, even tilted Sachs-Wolfe spectra are not a good fit to models like cold dark matter (CDM). Here, we normalize standard CDM (sCDM) to the two-year COBE data and quote the best amplitude in terms of the conventionally used measures of power. We also give normalizations for some specific variants of this standard model, and we indicate how the normalization depends on the assumed values on n, Omega(sub B) and H(sub 0). For sCDM we find the mean value of Q = 19.9 +/- 1.5 micro-K, corresponding to sigma(sub 8) = 1.34 +/- 0.10, with the normalization at large scales being B = (8.16 +/- 1.04) x 10(exp 5)(Mpc/h)(exp 4), and other numbers given in the table. The measured rms temperature fluctuation smoothed on 10 deg is a little low relative to this normalization. This is mainly due to the low quadrupole in the data: when the quadrupole is removed, the measured value of sigma(10 deg) is quite consistent with the best-fitting the mean value of Q. The use of the mean value of Q should be preferred over sigma(10 deg), when its value can be determined for a particular theory, since it makes full use of the data.

  13. DISCOVERY OF A WOLF-RAYET STAR THROUGH DETECTION OF ITS PHOTOMETRIC VARIABILITY

    Energy Technology Data Exchange (ETDEWEB)

    Littlefield, Colin [Law School, University of Notre Dame, Notre Dame, IN 46556 (United States); Garnavich, Peter; McClelland, Colin; Rettig, Terrence [Physics Department, University of Notre Dame, Notre Dame, IN 46556 (United States); Marion, G. H. [Harvard-Smithsonian Center for Astrophysics, Cambridge, MA 02138 (United States); Vinko, Jozsef [Department of Optics, University of Szeged (Hungary); Wheeler, J. Craig [Astronomy Department, University of Texas, Austin, TX 78712 (United States)

    2012-06-15

    We report the serendipitous discovery of a heavily reddened Wolf-Rayet star that we name WR 142b. While photometrically monitoring a cataclysmic variable, we detected weak variability in a nearby field star. Low-resolution spectroscopy revealed a strong emission line at 7100 A, suggesting an unusual object and prompting further study. A spectrum taken with the Hobby-Eberly Telescope confirms strong He II emission and an N IV 7112 A line consistent with a nitrogen-rich Wolf-Rayet star of spectral class WN6. Analysis of the He II line strengths reveals no detectable hydrogen in WR 142b. A blue-sensitive spectrum obtained with the Large Binocular Telescope shows no evidence for a hot companion star. The continuum shape and emission line ratios imply a reddening of E(B - V) = 2.2-2.6 mag. We estimate that the distance to WR 142b is 1.4 {+-} 0.3 kpc.

  14. DISCOVERY OF A WOLF-RAYET STAR THROUGH DETECTION OF ITS PHOTOMETRIC VARIABILITY

    International Nuclear Information System (INIS)

    Littlefield, Colin; Garnavich, Peter; McClelland, Colin; Rettig, Terrence; Marion, G. H.; Vinkó, József; Wheeler, J. Craig

    2012-01-01

    We report the serendipitous discovery of a heavily reddened Wolf-Rayet star that we name WR 142b. While photometrically monitoring a cataclysmic variable, we detected weak variability in a nearby field star. Low-resolution spectroscopy revealed a strong emission line at 7100 Å, suggesting an unusual object and prompting further study. A spectrum taken with the Hobby-Eberly Telescope confirms strong He II emission and an N IV 7112 Å line consistent with a nitrogen-rich Wolf-Rayet star of spectral class WN6. Analysis of the He II line strengths reveals no detectable hydrogen in WR 142b. A blue-sensitive spectrum obtained with the Large Binocular Telescope shows no evidence for a hot companion star. The continuum shape and emission line ratios imply a reddening of E(B – V) = 2.2-2.6 mag. We estimate that the distance to WR 142b is 1.4 ± 0.3 kpc.

  15. Lechery, lycanthropy and Little Red Riding Hood in Type O Negative’s ‘Wolf Moon (Including Zoanthropic Paranoia)’

    OpenAIRE

    Digioia, A.

    2016-01-01

    Type O Negative’s ‘Wolf Moon (including Zoanthropic Paranoia)’ seems to be a melodic ode to lascivious werewolves or to sexual intercourse during menstruation, which is transformative, allowing participation to channel animalistic instincts. Subject to more critical examination, ‘Wolf Moon (including Zoanthropic Paranoia)’ can also be presented as a contemporary incarnation of the ‘Little Red Riding Hood’ myth. Both contain the same themes: the stigmatization of eroticism, reclamation of agen...

  16. Consequences of a refuge for the predator-prey dynamics of a wolf-elk system in Banff National Park, Alberta, Canada.

    Science.gov (United States)

    Goldberg, Joshua F; Hebblewhite, Mark; Bardsley, John

    2014-01-01

    Refugia can affect predator-prey dynamics via movements between refuge and non-refuge areas. We examine the influence of a refuge on population dynamics in a large mammal predator-prey system. Wolves (Canis lupus) have recolonized much of their former range in North America, and as a result, ungulate prey have exploited refugia to reduce predation risk with unknown impacts on wolf-prey dynamics. We examined the influence of a refuge on elk (Cervus elaphus) and wolf population dynamics in Banff National Park. Elk occupy the Banff townsite with little predation, whereas elk in the adjoining Bow Valley experience higher wolf predation. The Banff refuge may influence Bow Valley predator-prey dynamics through source-sink movements. To test this hypothesis, we used 26 years of wolf and elk population counts and the Delayed Rejection Adaptive Metropolis Markov chain Monte Carlo method to fit five predator-prey models: 1) with no source-sink movements, 2) with elk density-dependent dispersal from the refuge to the non-refuge, 3) with elk predation risk avoidance movements from the non-refuge to the refuge, 4) with differential movement rates between refuge and non-refuge, and 5) with short-term, source-sink wolf movements. Model 1 provided the best fit of the data, as measured by Akaike Information Criterion (AIC). In the top model, Banff and Bow Valley elk had median growth rates of 0.08 and 0.03 (95% credibility intervals [CIs]: 0.027-0.186 and 0.001-0.143), respectively, Banff had a median carrying capacity of 630 elk (95% CI: 471.9-2676.9), Bow Valley elk had a median wolf encounter rate of 0.02 (95% CI: 0.013-0.030), and wolves had a median death rate of 0.23 (95% CI: 0.146-0.335) and a median conversion efficiency of 0.07 (95% CI: 0.031-0.124). We found little evidence for potential source-sink movements influencing the predator-prey dynamics of this system. This result suggests that the refuge was isolated from the non-refuge.

  17. Nucleosynthesis in Wolf-Rayet stars and galactic cosmic-ray isotopic composition

    International Nuclear Information System (INIS)

    Prantzos, N.

    1984-01-01

    An explanation of the isotopic composition of galactic cosmic rays could provide some clues to the mystery of their origin. It seems now that the strong stellar winds of Wolf-Rayet stars could account for most of the isotopic anomalies that have been observed in cosmic rays. Some results are presented, obtained by detailed nucleosynthesis computations. 25 references

  18. Neon Abundances from a Spitzer/IRS Survey of Wolf-Rayet Stars

    NARCIS (Netherlands)

    Ignace, R.; Cassinelli, J.P.; Tracy, G.; Churchwell, E.B.; Lamers, H.J.G.L.M.

    2007-01-01

    We report on neon abundances derived from Spitzer high resolution spectral data of eight Wolf-Rayet (WR) stars using the forbidden line of [Ne III] 15.56 μm. Our targets include four WN stars of subtypes 4-7, and four WC stars of subtypes 4-7. We derive ion fraction abundances γ of Ne2+ for the

  19. Planetary nebulae and Wolf-Rayet stars in the galactic-centre field

    Energy Technology Data Exchange (ETDEWEB)

    Allen, D A [Anglo-Australian Observatory, Epping (Australia)

    1979-06-01

    A UK Schmidt objective-prism plate of the Galactic-centre field has been examined. Of the 74 objects in the field which have been catalogued as planetary nebulae, only half appear correctly classified; the others include Be stars, symbiotic stars, and stars without emission lines. A further 19 planetary nebulae and two Wolf-Rayet stars have been discovered.

  20. On the use of small integrating spheres to improve the linearity range of RASNIKS systems

    International Nuclear Information System (INIS)

    Alberdi, J.; Burgos, C.; Ferrando, A.; Molinero, A.; Schvachkin, V.; Figueroa, C.F.; Matorras, F.; Rodrigo, T.; Ruiz, A.; Vila, I.

    1997-10-01

    Rasniks elements will be used in the CMS alignment system. The large displacements of the different sub detectors expected in the CMS experiment demands large linearity response of this system. By the use of a small integrating sphere we have optimized the source definition such that a factor three improvement in the linearity range with respect to conventional Rasniks configurations is obtained. The response range reached coincides with the maximum one can get with the components used in the test

  1. Effects of Headcutting on the Bottomland Hardwood Wetlands Adjacent to the Wolf River, Tennessee

    National Research Council Canada - National Science Library

    Weins, Karen

    2003-01-01

    The Wolf River in western Tennessee has experienced severe channel erosion in the form of headcutting and downcutting that has extended 17 kilometers upstream from the location at which channelization ceased in 1964...

  2. Weak lensing of the cosmic microwave background: Power spectrum covariance

    International Nuclear Information System (INIS)

    Cooray, Asantha

    2002-01-01

    We discuss the non-Gaussian contribution to the power spectrum covariance of cosmic microwave background (CMB) anisotropies resulting through weak gravitational lensing angular deflections and the correlation of deflections with secondary sources of temperature fluctuations generated by the large scale structure, such as the integrated Sachs-Wolfe effect and the Sunyaev-Zel'dovich effect. This additional contribution to the covariance of binned angular power spectrum, beyond the well known cosmic variance and any associated instrumental noise, results from a trispectrum, or a four point correlation function, in temperature anisotropy data. With substantially wide bins in multipole space, the resulting non-Gaussian contribution from lensing to the binned power spectrum variance is insignificant out to multipoles of a few thousand and is not likely to affect the cosmological parameter estimation with acoustic peaks and the damping tail. The non-Gaussian contribution to covariance, however, should be considered when interpreting binned CMB power spectrum measurements at multipoles of a few thousand corresponding to angular scales of few arcminutes and less

  3. Using NCAP to predict RFI effects in linear bipolar integrated circuits

    Science.gov (United States)

    Fang, T.-F.; Whalen, J. J.; Chen, G. K. C.

    1980-11-01

    Applications of the Nonlinear Circuit Analysis Program (NCAP) to calculate RFI effects in electronic circuits containing discrete semiconductor devices have been reported upon previously. The objective of this paper is to demonstrate that the computer program NCAP also can be used to calcuate RFI effects in linear bipolar integrated circuits (IC's). The IC's reported upon are the microA741 operational amplifier (op amp) which is one of the most widely used IC's, and a differential pair which is a basic building block in many linear IC's. The microA741 op amp was used as the active component in a unity-gain buffer amplifier. The differential pair was used in a broad-band cascode amplifier circuit. The computer program NCAP was used to predict how amplitude-modulated RF signals are demodulated in the IC's to cause undesired low-frequency responses. The predicted and measured results for radio frequencies in the 0.050-60-MHz range are in good agreement.

  4. Gastrointestinal parasites of maned wolf (Chrysocyon brachyurus, Illiger 1815) in a suburban area in southeastern Brazil.

    Science.gov (United States)

    Massara, R L; Paschoal, A M O; Chiarello, A G

    2015-08-01

    We examined 42 maned wolf scats in an unprotected and disturbed area of Cerrado in southeastern Brazil. We identified six helminth endoparasite taxa, being Phylum Acantocephala and Family Trichuridae the most prevalent. The high prevalence of the Family Ancylostomatidae indicates a possible transmission via domestic dogs, which are abundant in the study area. Nevertheless, our results indicate that the endoparasite species found are not different from those observed in protected or least disturbed areas, suggesting a high resilience of maned wolf and their parasites to human impacts, or a common scenario of disease transmission from domestic dogs to wild canid whether in protected or unprotected areas of southeastern Brazil.

  5. Conservation implications for the Himalayan wolf Canis (lupus) himalayensis based on observations of packs and home sites in Nepal

    OpenAIRE

    Werhahn, G; Kusi, N; Sillero-Zubiri, C; Macdonald, DW

    2017-01-01

    We provide insights into pack composition and den site parameters of the Himalayan wolf Canis (lupus) himalayensis based on observations of free-ranging wolves in three study areas in Nepal. We combine this with a social survey of the local Buddhist communities regarding human–carnivore conflict, to draw inferences for conservation practice in the Nepalese Himalayas. We recorded eight wolf packs (with an average composition of two adults and three pups), and found five home sites in high-alti...

  6. Linking Community Communication to Conservation of the Maned Wolf in Central Brazil

    Science.gov (United States)

    Bizerril, Marcelo Ximenes A.; Soares, Carla Cruz; Santos, Jean Pierre

    2011-01-01

    This article describes the environmental education (EE) program developed in the neighboring community of Serra da Canastra National Park based on a research project focused on the maned wolf conservation. The article assesses three tools used to foster the community's participation in discussing local issues: (1) communal production of a book…

  7. Gray wolf (Canis lupus) is a natural definitive host for Neospora caninum

    Science.gov (United States)

    The gray wolf (Canis lupus) was found to be a new natural definitive host for Neospora caninum. This finding is based on the recovery of Neospora-like oocysts from the feces of 3 of 73 wolves from Minnesota examined at necropsy, and on successful amplification of N. caninum-specific sequences from ...

  8. Spectra of the Wolf-Rayet stars in 30 Doradus

    International Nuclear Information System (INIS)

    Conti, P.S.

    1982-01-01

    The central cluster of the 30 Doradus complex in the Large Magellanic Cloud (LMC) contains at least a dozen Wolf-Rayet (W-R) stars of the nitrogen sequence according to Melnick (1978). In addition to the six stars previously identified by Feast, Thackeray and Wesselinck (1960), Melnick identified an additional six using an Echele spectrograph on the 1.52 m telescope at La Silla. The author has obtained high resolution spectra of ten of these stars and discusses the spectral types. (Auth.)

  9. The use of a path independent integral in non-linear fracture mechanics

    International Nuclear Information System (INIS)

    Hellen, T.K.

    1977-01-01

    The use of the Rice J-intergral to assess conditions at a crack tip in an elastic or non-linear elastic body is well known. The integral equals the energy release rate and is path independent for any contour surrounding the crack tip provided no other singularities are encompassed. The path independence propertiy breaks down, however, in more general situations such as in three dimensional stress systems, plasticity unloading, thermal or creep states. Hence the required crack tip characteristics represented by the value of the integral round a contour whose radius about the tip tends to zero, is not reproduced along contours away from the tip. Consequently, an alternative integral, designated J*, has been proposed which equals J for elastic cases and in the other cases cited above remains path independent. A computer program for calculating the J and J* integrals has been developed as an extension to the BERSAFE finite element system. A full analysis of the cracked structure including plasticity, creep and thermal strains is conducted and the results are stored on a permanent data set. The integral values may then be calculated using the post-processor program for any number of contours and load or time steps, without recourse to further expensive computations. (Auth. )

  10. Crystallographic Structure of Human β-Hexosaminidase A: Interpretation of Tay-Sachs Mutations and Loss of GM2 Ganglioside Hydrolysis

    Science.gov (United States)

    Lemieux, M. Joanne; Mark, Brian L.; Cherney, Maia M.; Withers, Stephen G.; Mahuran, Don J.; James, Michael N. G.

    2010-01-01

    Lysosomal β-hexosaminidase A (Hex A) is essential for the degradation of GM2 gangliosides in the central and peripheral nervous system. Accumulation of GM2 leads to severely debilitating neurodegeneration associated with Tay-Sachs disease (TSD), Sandoff disease (SD) and AB variant. Here, we present the X-ray crystallographic structure of Hex A to 2.8 Å resolution and the structure of Hex A in complex with NAG-thiazoline, (NGT) to 3.25 Å resolution. NGT, a mechanism-based inhibitor, has been shown to act as a chemical chaperone that, to some extent, prevents misfolding of a Hex A mutant associated with adult onset Tay Sachs disease and, as a result, increases the residual activity of Hex A to a level above the critical threshold for disease. The crystal structure of Hex A reveals an αβ heterodimer, with each subunit having a functional active site. Only the α-subunit active site can hydrolyze GM2 gangliosides due to a flexible loop structure that is removed post-translationally from β, and to the presence of αAsn423 and αArg424. The loop structure is involved in binding the GM2 activator protein, while αArg424 is critical for binding the carboxylate group of the N-acetyl-neuraminic acid residue of GM2. The β-subunit lacks these key residues and has βAsp452 and βLeu453 in their place; the β-subunit therefore cleaves only neutral substrates efficiently. Mutations in the α-subunit, associated with TSD, and those in the β-subunit, associated with SD are discussed. The effect of NGT binding in the active site of a mutant Hex A and its effect on protein function is discussed. PMID:16698036

  11. Carnivore specific bone bioapatite and collagen carbon isotope fractionations: Case studies of modern and fossil grey wolf populations

    Science.gov (United States)

    Fox-Dobbs, K.; Wheatley, P. V.; Koch, P. L.

    2006-12-01

    Stable isotope analyses of modern and fossil biogenic tissues are routinely used to reconstruct present and past vertebrate foodwebs. Accurate isotopic dietary reconstructions require a consumer and tissue specific understanding of how isotopes are sorted, or fractionated, between trophic levels. In this project we address the need for carnivore specific isotope variables derived from populations that are ecologically well- characterized. Specifically, we investigate the trophic difference in carbon isotope values between mammalian carnivore (wolf) bone bioapatite and herbivore (prey) bone bioapatite. We also compare bone bioapatite and collagen carbon isotope values collected from the same individuals. We analyzed bone specimens from two modern North American grey wolf (Canis lupus) populations (Isle Royale National Park, Michigan and Yellowstone National Park, Wyoming), and the ungulate herbivores that are their primary prey (moose and elk, respectively). Because the diets of both wolf populations are essentially restricted to a single prey species, there were no confounding effects due to carnivore diet variability. We measured a trophic difference of approximately -1.3 permil between carnivore (lower value) and herbivore (higher value) bone bioapatite carbon isotope values, and an average inter-tissue difference of 5.1 permil between carnivore bone collagen (lower value) and bioapatite (higher value) carbon isotope values. Both of these isotopic differences differ from previous estimates derived from a suite of African carnivores; our carnivore-herbivore bone bioapatite carbon isotope spacing is smaller (-1.3 vs. -4.0 permil), and our carnivore collagen-bioapatite carbon difference is larger (5.1 vs. 3.0 permil). These discrepancies likely result from comparing values measured from a single hypercarnivore (wolf) to average values calculated from several carnivore species, some of which are insectivorous or partly omnivorous. The trophic and inter

  12. Características clínicas y citogenéticas en el síndrome de Wolf-Hirschhorn. Serie de casos

    Directory of Open Access Journals (Sweden)

    Francisco Cammarata-Scalisi

    2015-10-01

    Full Text Available Resumen (español El síndrome de Wolf-Hirschhorn es una entidad genética producida por una deleción parcial que abarca la región distal del brazo corto del cromosoma 4 (4p16.3. Las manifestaciones más frecuentes son anomalías craneofaciales, retraso psicomotor y alteraciones neurológicas. La incidencia estimada es de 1 en 50.000 nacimientos, aunque hay un subdiagnóstico de esta entidad. El objetivo de este estudio es describir cuatro casos clínicos del síndrome de Wolf-Hirschhorn con descripción específica de los hallazgos clínicos y citogenéticos Abstract (english Wolf-Hirschhorn syndrome is a genetic entity produced by a partial deletion spanning the distal short arm of chromosome 4 (4p16.3. The most common manifestations are craniofacial anomalies, psychomotor retardation and neurological disorders. The estimated incidence is 1 in 50,000 births, although there is an underdiagnosis of this entity. The aim of this study is to describe four clinical cases of Wolf-Hirschhorn syndrome with specific description of clinical and cytogenetic findings

  13. Electron density in the emission-line region of Wolf-Rayet stars

    International Nuclear Information System (INIS)

    Varshni, Y.P.

    1978-01-01

    The Inglis-Teller relation, generalized for a hydrogen-like or alkali-like ion with an arbitrary core charge, is used to estimate the electron density in the emission-like region of Wolf-Rayet stars. It is found that the electron density in the region which gives rise to He II emission lines is approximately = 4 x 10 14 cm -3 . (Auth.)

  14. Observational tests of modified gravity

    International Nuclear Information System (INIS)

    Jain, Bhuvnesh; Zhang Pengjie

    2008-01-01

    Modifications of general relativity provide an alternative explanation to dark energy for the observed acceleration of the Universe. Modified gravity theories have richer observational consequences for large-scale structures than conventional dark energy models, in that different observables are not described by a single growth factor even in the linear regime. We examine the relationships between perturbations in the metric potentials, density and velocity fields, and discuss strategies for measuring them using gravitational lensing, galaxy cluster abundances, galaxy clustering/dynamics, and the integrated Sachs-Wolfe effect. We show how a broad class of gravity theories can be tested by combining these probes. A robust way to interpret observations is by constraining two key functions: the ratio of the two metric potentials, and the ratio of the gravitational 'constant' in the Poisson equation to Newton's constant. We also discuss quasilinear effects that carry signatures of gravity, such as through induced three-point correlations. Clustering of dark energy can mimic features of modified gravity theories and thus confuse the search for distinct signatures of such theories. It can produce pressure perturbations and anisotropic stresses, which break the equality between the two metric potentials even in general relativity. With these two extra degrees of freedom, can a clustered dark energy model mimic modified gravity models in all observational tests? We show with specific examples that observational constraints on both the metric potentials and density perturbations can in principle distinguish modifications of gravity from dark energy models. We compare our result with other recent studies that have slightly different assumptions (and apparently contradictory conclusions).

  15. Initial-boundary-value problem of the self-gravitating scalar field in the Bondi-Sachs gauge

    International Nuclear Information System (INIS)

    Frittelli, Simonetta; Gomez, Roberto

    2007-01-01

    It is shown that, in the Bondi-Sachs gauge that fixes the speed of incoming light rays to the value 1, the Einstein equations coupled to a scalar field in spherical symmetry are cast into a symmetric-hyperbolic system of equations for the scalar field, lapse and shift as fundamental variables. In this system of equations, the lapse and shift are incoming characteristic fields, and the scalar field has three components: incoming, outgoing and static. A constraint-preserving boundary condition is prescribed by imposing the projection of the Einstein equation normal to the boundary at the outer value of the radial coordinate. The boundary condition specifies one of the two incoming metric fields. The remaining incoming metric field and the incoming scalar field component need to be specified arbitrarily. Numerical simulations of the scattering of the scalar field by a black hole in the nonlinear regime are presented that illustrate interesting facts about black-hole physics and the behavior of the characteristic variables of the problem

  16. On the removal of boundary errors caused by Runge-Kutta integration of non-linear partial differential equations

    Science.gov (United States)

    Abarbanel, Saul; Gottlieb, David; Carpenter, Mark H.

    1994-01-01

    It has been previously shown that the temporal integration of hyperbolic partial differential equations (PDE's) may, because of boundary conditions, lead to deterioration of accuracy of the solution. A procedure for removal of this error in the linear case has been established previously. In the present paper we consider hyperbolic (PDE's) (linear and non-linear) whose boundary treatment is done via the SAT-procedure. A methodology is present for recovery of the full order of accuracy, and has been applied to the case of a 4th order explicit finite difference scheme.

  17. Wolf-Hirschhorn (4p-) syndrome with West syndrome.

    Science.gov (United States)

    Motoi, Hirotaka; Okanishi, Tohru; Kanai, Sotaro; Yokota, Takuya; Yamazoe, Tomohiro; Nishimura, Mitsuyo; Fujimoto, Ayataka; Yamamoto, Takamichi; Enoki, Hideo

    2016-01-01

    Wolf-Hirschhorn syndrome (WHS) is a chromosome disorder (4p-syndrome) which is characterized by craniofacial features and epileptic seizures. Here, we report a case of WHS with West syndrome, in whom the seizures were refractory to several antiepileptic drugs but were responsive to the addition of lamotrigine. The patient had epileptic spasms at age seven months. The interictal electroencephalogram was hypsarrhythmic. After adding lamotrigine, seizures decreased remarkably, and spasms disappeared. We have identified and described the very rare case of a girl with WHS who also developed West syndrome. In this case, adding lamotrigine to her medications effectively treated the spasms.

  18. Wolf-Rayet stars in the Andromeda Galaxy

    International Nuclear Information System (INIS)

    Moffat, A.F.J.; Shara, M.M.; Space Telescope Science Institute, Baltimore, MD)

    1987-01-01

    A survey of M31 for strong-line Wolf-Rayet (W-R) stars has been completed, confirming the trends found previously, that (1) M31 is at present about an order of magnitude less active in star formation than the Galaxy, as reflected in the total number of W-R stars, assumed to have evolved from massive progenitors; (2) the number ratio of late to early WC stars, WCL/WCE, varies systematically with galactocentric radius as in the Galaxy, possibly a consequence of the metallicity gradient in the disk; and (3) most W-R stars lie in the prominent ring of active star formation at R = 7-12 kpc from the center of M31. 19 references

  19. Decoding Group Vocalizations: The Acoustic Energy Distribution of Chorus Howls Is Useful to Determine Wolf Reproduction

    Science.gov (United States)

    López-Bao, José Vicente; Llaneza, Luis; Fernández, Carlos; Font, Enrique

    2016-01-01

    Population monitoring is crucial for wildlife management and conservation. In the last few decades, wildlife researchers have increasingly applied bioacoustics tools to obtain information on several essential ecological parameters, such as distribution and abundance. One such application involves wolves (Canis lupus). These canids respond to simulated howls by emitting group vocalizations known as chorus howls. These responses to simulated howls reveal the presence of wolf litters during the breeding period and are therefore often used to determine the status of wolf populations. However, the acoustic structure of chorus howls is complex and discriminating the presence of pups in a chorus is sometimes difficult, even for experienced observers. In this study, we evaluate the usefulness of analyses of the acoustic energy distribution in chorus howls to identify the presence of pups in a chorus. We analysed 110 Iberian wolf chorus howls with known pack composition and found that the acoustic energy distribution is concentrated at higher frequencies when there are pups vocalizing. We built predictive models using acoustic energy distribution features to determine the presence of pups in a chorus, concluding that the acoustic energy distribution in chorus howls can be used to determine the presence of wolf pups in a pack. The method we outline here is objective, accurate, easily implemented, and independent of the observer's experience. These advantages are especially relevant in the case of broad scale surveys or when many observers are involved. Furthermore, the analysis of the acoustic energy distribution can be implemented for monitoring other social canids that emit chorus howls such as jackals or coyotes, provides an easy way to obtain information on ecological parameters such as reproductive success, and could be useful to study other group vocalizations. PMID:27144887

  20. Decoding Group Vocalizations: The Acoustic Energy Distribution of Chorus Howls Is Useful to Determine Wolf Reproduction.

    Directory of Open Access Journals (Sweden)

    Vicente Palacios

    Full Text Available Population monitoring is crucial for wildlife management and conservation. In the last few decades, wildlife researchers have increasingly applied bioacoustics tools to obtain information on several essential ecological parameters, such as distribution and abundance. One such application involves wolves (Canis lupus. These canids respond to simulated howls by emitting group vocalizations known as chorus howls. These responses to simulated howls reveal the presence of wolf litters during the breeding period and are therefore often used to determine the status of wolf populations. However, the acoustic structure of chorus howls is complex and discriminating the presence of pups in a chorus is sometimes difficult, even for experienced observers. In this study, we evaluate the usefulness of analyses of the acoustic energy distribution in chorus howls to identify the presence of pups in a chorus. We analysed 110 Iberian wolf chorus howls with known pack composition and found that the acoustic energy distribution is concentrated at higher frequencies when there are pups vocalizing. We built predictive models using acoustic energy distribution features to determine the presence of pups in a chorus, concluding that the acoustic energy distribution in chorus howls can be used to determine the presence of wolf pups in a pack. The method we outline here is objective, accurate, easily implemented, and independent of the observer's experience. These advantages are especially relevant in the case of broad scale surveys or when many observers are involved. Furthermore, the analysis of the acoustic energy distribution can be implemented for monitoring other social canids that emit chorus howls such as jackals or coyotes, provides an easy way to obtain information on ecological parameters such as reproductive success, and could be useful to study other group vocalizations.

  1. Implications of teenagers' attitudes toward maned wolf conservation in Brazil

    OpenAIRE

    Consorte-McCrea, A.; Nigbur, D.; Bath, A.

    2017-01-01

    The relationships between people and wild canids are a widespread concern for the conservation of species and habitats. The maned wolf Chrysocyon brachyurus is a Near Threatened species inhabiting South America. Strategies to conserve this keystone species may benefit the also-declining Cerrado biome. The attitudes of teenagers toward wild carnivores are also of worldwide interest as these youth are the future decision makers. We investigated selected attitudes, beliefs and knowledge in relat...

  2. Airway Management in a Patient with Wolf-Hirschhorn Syndrome.

    Science.gov (United States)

    Gamble, John F; Kurian, Dinesh J; Udani, Andrea G; Greene, Nathaniel H

    2016-01-01

    We present a case of a 3-month-old female with Wolf-Hirschhorn syndrome (WHS) undergoing general anesthesia for laparoscopic gastrostomy tube placement with a focus on airway management. WHS is a rare 4p microdeletion syndrome resulting in multiple congenital abnormalities, including craniofacial deformities. Microcephaly, micrognathia, and glossoptosis are common features in WHS patients and risk factors for a pediatric airway that is potentially difficult to intubate. We discuss anesthesia strategies for airway preparation and management in a WHS patient requiring general anesthesia with endotracheal intubation.

  3. NOVEL RADIOGRAPHIC TECHNIQUE FOR PREGNANCY DETECTION IN THE MANED WOLF (CHRYSOCYON BRACHYURUS) WITHOUT ANESTHESIA.

    Science.gov (United States)

    Aitken-Palmer, Copper; A C Z M, Dipl; Ware, Lisa H; Braun, Lacey; Lang, Kenneth; Joyner, Priscilla H

    2017-03-01

    Maned wolves ( Chrysocyon brachyurus ) maintained in ex situ populations challenge veterinarians and managers with high neonatal mortality and parental incompetence. These challenges led to the development of a novel diagnostic approach for pregnancy detection using radiographic imaging without anesthesia or sedation. To do this, a specialized crate was constructed to easily contain a single maned wolf, allowing the capture of lateral projection radiographic images of the abdomen prior to and throughout a 66-day pregnancy (days 20, 34, 48, and 55 of 66). Radiographs taken at days 48 and 55 postbreeding showed evidence of neonatal skeleton mineralization, confirming pregnancy with two pups. The dam gave birth at day 66 to two pups. This technical report describes a novel approach without anesthesia for successful radiographic pregnancy detection and determination of litter size in the maned wolf, a midsize carnivore, using a specially constructed crate.

  4. Gastrointestinal parasites of maned wolf (Chrysocyon brachyurus, Illiger 1815 in a suburban area in southeastern Brazil

    Directory of Open Access Journals (Sweden)

    RL Massara

    Full Text Available AbstractWe examined 42 maned wolf scats in an unprotected and disturbed area of Cerrado in southeastern Brazil. We identified six helminth endoparasite taxa, being Phylum Acantocephala and Family Trichuridae the most prevalent. The high prevalence of the Family Ancylostomatidae indicates a possible transmission via domestic dogs, which are abundant in the study area. Nevertheless, our results indicate that the endoparasite species found are not different from those observed in protected or least disturbed areas, suggesting a high resilience of maned wolf and their parasites to human impacts, or a common scenario of disease transmission from domestic dogs to wild canid whether in protected or unprotected areas of southeastern Brazil.

  5. Spectrum and light curve of a supernova shock breakout through a thick Wolf-Rayet wind

    Energy Technology Data Exchange (ETDEWEB)

    Svirski, Gilad; Nakar, Ehud, E-mail: swirskig@post.tau.ac.il [Raymond and Beverly Sackler School of Physics and Astronomy, Tel Aviv University, Tel Aviv 69978 (Israel)

    2014-06-20

    Wolf-Rayet stars are known to eject winds. Thus, when a Wolf-Rayet star explodes as a supernova, a fast (≳ 40, 000 km s{sup –1}) shock is expected to be driven through a wind. We study the signal expected from a fast supernova shock propagating through an optically thick wind and find that the electrons behind the shock driven into the wind are efficiently cooled by inverse Compton over soft photons that were deposited by the radiation-mediated shock that crossed the star. Therefore, the bolometric luminosity is comparable to the kinetic energy flux through the shock, and the spectrum is found to be a power law, whose slope and frequency range depend on the number flux of soft photons available for cooling. Wolf-Rayet supernovae that explode through a thick wind have a high flux of soft photons, producing a flat spectrum, νF {sub ν} = Const, in the X-ray range of 0.1 ≲ T ≲ 50 keV. As the shock expands into an optically thin wind, the soft photons are no longer able to cool the shock that plows through the wind, and the bulk of the emission takes the form of a standard core-collapse supernova (without a wind). However, a small fraction of the soft photons is upscattered by the shocked wind and produces a transient unique X-ray signature.

  6. Spectrum and light curve of a supernova shock breakout through a thick Wolf-Rayet wind

    International Nuclear Information System (INIS)

    Svirski, Gilad; Nakar, Ehud

    2014-01-01

    Wolf-Rayet stars are known to eject winds. Thus, when a Wolf-Rayet star explodes as a supernova, a fast (≳ 40, 000 km s –1 ) shock is expected to be driven through a wind. We study the signal expected from a fast supernova shock propagating through an optically thick wind and find that the electrons behind the shock driven into the wind are efficiently cooled by inverse Compton over soft photons that were deposited by the radiation-mediated shock that crossed the star. Therefore, the bolometric luminosity is comparable to the kinetic energy flux through the shock, and the spectrum is found to be a power law, whose slope and frequency range depend on the number flux of soft photons available for cooling. Wolf-Rayet supernovae that explode through a thick wind have a high flux of soft photons, producing a flat spectrum, νF ν = Const, in the X-ray range of 0.1 ≲ T ≲ 50 keV. As the shock expands into an optically thin wind, the soft photons are no longer able to cool the shock that plows through the wind, and the bulk of the emission takes the form of a standard core-collapse supernova (without a wind). However, a small fraction of the soft photons is upscattered by the shocked wind and produces a transient unique X-ray signature.

  7. Let's stay together? Intrinsic and extrinsic factors involved in pair bond dissolution in a recolonizing wolf population.

    Science.gov (United States)

    Milleret, Cyril; Wabakken, Petter; Liberg, Olof; Åkesson, Mikael; Flagstad, Øystein; Andreassen, Harry Peter; Sand, Håkan

    2017-01-01

    For socially monogamous species, breeder bond dissolution has important consequences for population dynamics, but the extent to which extrinsic or intrinsic population factors causes pair dissolution remain poorly understood, especially among carnivores. Using an extensive life-history data set, a survival analysis and competing risks framework, we examined the fate of 153 different wolf (Canis lupus) pairs in the recolonizing Scandinavian wolf population, during 14 winters of snow tracking and DNA monitoring. Wolf pair dissolution was generally linked to a mortality event and was strongly affected by extrinsic (i.e. anthropogenic) causes. No divorce was observed, and among the pair dissolution where causes have been identified, death of one or both wolves was always involved. Median time from pair formation to pair dissolution was three consecutive winters (i.e. approximately 2 years). Pair dissolution was mostly human-related, primarily caused by legal control actions (36·7%), verified poaching (9·2%) and traffic-related causes (2·1%). Intrinsic factors, such as disease and age, accounted for only 7·7% of pair dissolutions. The remaining 44·3% of dissolution events were from unknown causes, but we argue that a large portion could be explained by an additional source of human-caused mortality, cryptic poaching. Extrinsic population factors, such as variables describing the geographical location of the pair, had a stronger effect on risk of pair dissolution compared to anthropogenic landscape characteristics. Population intrinsic factors, such as the inbreeding coefficient of the male pair member, had a negative effect on pair bond duration. The mechanism behind this result remains unknown, but might be explained by lower survival of inbred males or more complex inbreeding effects mediated by behaviour. Our study provides quantitative estimates of breeder bond duration in a social carnivore and highlights the effect of extrinsic (i.e. anthropogenic) and

  8. Foraging and feeding ecology of the gray wolf (Canis lupus): lessons from Yellowstone National Park, Wyoming, USA.

    Science.gov (United States)

    Stahler, Daniel R; Smith, Douglas W; Guernsey, Debra S

    2006-07-01

    The foraging and feeding ecology of gray wolves is an essential component to understanding the role that top carnivores play in shaping the structure and function of terrestrial ecosystems. In Yellowstone National Park (YNP), predation studies on a highly visible, reintroduced population of wolves are increasing our understanding of this aspect of wolf ecology. Wolves in YNP feed primarily on elk, despite the presence of other ungulate species. Patterns of prey selection and kill rates in winter have varied seasonally each year from 1995 to 2004 and changed in recent years as the wolf population has become established. Wolves select elk based on their vulnerability as a result of age, sex, and season and therefore kill primarily calves, old cows, and bulls that have been weakened by winter. Summer scat analysis reveals an increased variety in diet compared with observed winter diets, including other ungulate species, rodents, and vegetation. Wolves in YNP hunt in packs and, upon a successful kill, share in the evisceration and consumption of highly nutritious organs first, followed by major muscle tissue, and eventually bone and hide. Wolves are adapted to a feast-or-famine foraging pattern, and YNP packs typically kill and consume an elk every 2-3 d. However, wolves in YNP have gone without fresh meat for several weeks by scavenging off old carcasses that consist mostly of bone and hide. As patterns of wolf density, prey density, weather, and vulnerability of prey change, in comparison with the conditions of the study period described here, we predict that there will also be significant changes in wolf predation patterns and feeding behavior.

  9. Optimal distribution of integration time for intensity measurements in degree of linear polarization polarimetry.

    Science.gov (United States)

    Li, Xiaobo; Hu, Haofeng; Liu, Tiegen; Huang, Bingjing; Song, Zhanjie

    2016-04-04

    We consider the degree of linear polarization (DOLP) polarimetry system, which performs two intensity measurements at orthogonal polarization states to estimate DOLP. We show that if the total integration time of intensity measurements is fixed, the variance of the DOLP estimator depends on the distribution of integration time for two intensity measurements. Therefore, by optimizing the distribution of integration time, the variance of the DOLP estimator can be decreased. In this paper, we obtain the closed-form solution of the optimal distribution of integration time in an approximate way by employing Delta method and Lagrange multiplier method. According to the theoretical analyses and real-world experiments, it is shown that the variance of the DOLP estimator can be decreased for any value of DOLP. The method proposed in this paper can effectively decrease the measurement variance and thus statistically improve the measurement accuracy of the polarimetry system.

  10. In silico analyses of essential interactions of iminosugars with the Hex A active site and evaluation of their pharmacological chaperone effects for Tay-Sachs disease.

    Science.gov (United States)

    Kato, Atsushi; Nakagome, Izumi; Nakagawa, Shinpei; Kinami, Kyoko; Adachi, Isao; Jenkinson, Sarah F; Désiré, Jérôme; Blériot, Yves; Nash, Robert J; Fleet, George W J; Hirono, Shuichi

    2017-11-15

    The affinity of a series of iminosugar-based inhibitors exhibiting various ring sizes toward Hex A and their essential interactions with the enzyme active site were investigated. All the Hex A-inhibiting iminosugars tested formed hydrogen bonds with Arg178, Asp322, Tyr421 and Glu462 and had the favorable cation-π interaction with Trp460. Among them, DMDP amide (6) proved to be the most potent competitive inhibitor with a K i value of 0.041 μM. We analyzed the dynamic properties of both DMDP amide (6) and DNJNAc (1) in aqueous solution using molecular dynamics (MD) calculations; the distance of the interaction between Asp322 and 3-OH and Glu323 and 6-OH was important for stable interactions with Hex A, reducing fluctuations in the plasticity of the active site. DMDP amide (6) dose-dependently increased intracellular Hex A activity in the G269S mutant cells and restored Hex A activity up to approximately 43% of the wild type level; this effect clearly exceeded the border line treatment for Tay-Sachs disease, which is regarded as 10-15% of the wild type level. This is a significantly greater effect than that of pyrimethamine, which is currently in Phase 2 clinical trials. DMDP amide (6), therefore, represents a new promising pharmacological chaperone candidate for the treatment of Tay-Sachs disease.

  11. Integration of Attributes from Non-Linear Characterization of Cardiovascular Time-Series for Prediction of Defibrillation Outcomes.

    Directory of Open Access Journals (Sweden)

    Sharad Shandilya

    Full Text Available The timing of defibrillation is mostly at arbitrary intervals during cardio-pulmonary resuscitation (CPR, rather than during intervals when the out-of-hospital cardiac arrest (OOH-CA patient is physiologically primed for successful countershock. Interruptions to CPR may negatively impact defibrillation success. Multiple defibrillations can be associated with decreased post-resuscitation myocardial function. We hypothesize that a more complete picture of the cardiovascular system can be gained through non-linear dynamics and integration of multiple physiologic measures from biomedical signals.Retrospective analysis of 153 anonymized OOH-CA patients who received at least one defibrillation for ventricular fibrillation (VF was undertaken. A machine learning model, termed Multiple Domain Integrative (MDI model, was developed to predict defibrillation success. We explore the rationale for non-linear dynamics and statistically validate heuristics involved in feature extraction for model development. Performance of MDI is then compared to the amplitude spectrum area (AMSA technique.358 defibrillations were evaluated (218 unsuccessful and 140 successful. Non-linear properties (Lyapunov exponent > 0 of the ECG signals indicate a chaotic nature and validate the use of novel non-linear dynamic methods for feature extraction. Classification using MDI yielded ROC-AUC of 83.2% and accuracy of 78.8%, for the model built with ECG data only. Utilizing 10-fold cross-validation, at 80% specificity level, MDI (74% sensitivity outperformed AMSA (53.6% sensitivity. At 90% specificity level, MDI had 68.4% sensitivity while AMSA had 43.3% sensitivity. Integrating available end-tidal carbon dioxide features into MDI, for the available 48 defibrillations, boosted ROC-AUC to 93.8% and accuracy to 83.3% at 80% sensitivity.At clinically relevant sensitivity thresholds, the MDI provides improved performance as compared to AMSA, yielding fewer unsuccessful defibrillations

  12. Do really all wolf spiders carry spiderlings on their opisthosomas? The case of Hygrolycosa rubrofasciata (Araneae: Lycosidae

    Directory of Open Access Journals (Sweden)

    Dolejš, Petr

    2013-06-01

    Full Text Available Wolf spider females are characterised by carrying cocoons attached to their spinnerets. Emerged spiderlings are carried on the females’ opisthosomas, with the exception of three Japanese lycosid species who carry spiderlings on empty cocoons. Here, the same behaviour is recorded in a European spider: the drumming wolf spider Hygrolycosa rubrofasciata. Spiderlings of this species do not try to climb on the female’s opisthosoma, even when they are adopted by a female of a species with a normal pulli-carrying behaviour. This behaviour occurs in Trechaleidae and four unrelated species of Lycosidae inhabiting wet habitats and is therefore regarded as an adaptation to the unsuitable environment.

  13. On planetary nebulae and Wolf-Rayet stars in the galactic-centre field

    International Nuclear Information System (INIS)

    Allen, D.A.

    1979-01-01

    A UK Schmidt objective-prism plate of the Galactic-centre field has been examined. Of the 74 objects in the field which have been catalogued as planetary nebulae, only half appear correctly classified; the others include Be stars, symbiotic stars, and stars without emission lines. A further 19 planetary nebulae and two Wolf-Rayet stars have been discovered. (author)

  14. Isolation and genetic characterization of Toxoplasma gondii from the gray wolf Canis lupus

    Science.gov (United States)

    Little is known of the genetic diversity of Toxoplasma gondii circulating in wildlife. In the present study feral gray wolf (Canis lupus) from Minnesota were examined for T. gondii infection. Antibodies to T. gondii were detected in 130 (52.4%) of 248 wolves tested by the modified agglutination test...

  15. First record of entodiniomorph ciliates in a carnivore, the maned wolf (Chrysocyon brachyurus), from Brazil.

    Science.gov (United States)

    Vynne, Carly; Kinsella, John M

    2009-06-01

    The entodiniomorph ciliates (Ciliophora: Entodiniomorphida) are endosymbiotes widely found in the intestines of herbivorous mammals. These commensals commonly occur in the Artiodactyla and Perissodactyla and have also been described in the Proboscidea, Primates, Rodentia, and Diprotodontia. This study reports the first finding of a ciliate in a member of order Carnivora, the maned wolf (Chrysocyon brachyurus). Fecal samples from wild and captive maned wolves were screened using ethyl acetate sedimentation. Prevalence in fecal samples collected from free-ranging maned wolves in Brazil was 40% (6 of 15). Fecal samples from two of four captive individuals from the St. Louis Zoo also had the same species of ciliate. The largely frugivorous diet of the maned wolf likely explains the occurrence of these normally herbivore-associated endosymbiotes in a carnivore.

  16. Canine parvovirus effect on wolf population change and pup survival

    Science.gov (United States)

    Mech, L.D.; Goyal, S.M.

    1993-01-01

    Canine parvovirus infected wild canids more than a decade ago, but no population effect has been documented. In wild Minnesota wolves (Canis lupus) over a 12-yr period, the annual percent population increase and proportion of pups each were inversely related to the percentage of wolves serologically positive to the disease. Although these effects did not seem to retard this large extant population, similar relationships in more isolated wolf populations might hinder recovery of this endangered and threatened species.

  17. Airway Management in a Patient with Wolf-Hirschhorn Syndrome

    Directory of Open Access Journals (Sweden)

    John F. Gamble

    2016-01-01

    Full Text Available We present a case of a 3-month-old female with Wolf-Hirschhorn syndrome (WHS undergoing general anesthesia for laparoscopic gastrostomy tube placement with a focus on airway management. WHS is a rare 4p microdeletion syndrome resulting in multiple congenital abnormalities, including craniofacial deformities. Microcephaly, micrognathia, and glossoptosis are common features in WHS patients and risk factors for a pediatric airway that is potentially difficult to intubate. We discuss anesthesia strategies for airway preparation and management in a WHS patient requiring general anesthesia with endotracheal intubation.

  18. GM2 gangliosidoses in Spain: analysis of the HEXA and HEXB genes in 34 Tay-Sachs and 14 Sandhoff patients.

    Science.gov (United States)

    Gort, Laura; de Olano, Natalia; Macías-Vidal, Judit; Coll, M A Josep

    2012-09-10

    The GM2 gangliosidoses are autosomal recessive lysosomal storage diseases caused by a deficiency of the β-hexosaminidase A enzyme. This enzyme is composed of two polypeptide chains designated the α- and β- subunits and it interacts with the GM2 activator protein. The HEXA and HEXB genes encode the α-subunit and the β-subunit, respectively. Mutations in these genes are causative of Tay-Sachs disease (HEXA) and Sandhoff disease (HEXB). We analyzed the complete HEXA gene in 34 Spanish patients with Tay-Sachs disease and the HEXB gene in 14 Spanish patients with Sandhoff disease. We identified 27 different mutations, 14 of which were novel, in the HEXA gene and 14 different mutations, 8 of which unreported until now, in the HEXB gene, and we attempted to correlate these mutations with the clinical presentation of the patients. We found a high frequency of c.459+5G>A (IVS4+5G>A) mutation in HEXA affected patients, 22 of 68 alleles, which represent the 32.4%. This is the highest percentage found of this mutation in a population. All patients homozygous for mutation c.459+5G>A presented with the infantile form of the disease and, as previously reported, patients carrying mutation p.R178H in at least one of the alleles presented with a milder form. In HEXB affected patients, the novel deletion c.171delG accounts for 21.4% of the mutant alleles (6/28). All patients with this deletion showed the infantile form of the disease. The Spanish GM2 gangliosidoses affected patients show a great mutational heterogeneity as seen in other inherited lisosomal diseases in this country. Copyright © 2012. Published by Elsevier B.V.

  19. Characterization of inducible models of Tay-Sachs and related disease.

    Science.gov (United States)

    Sargeant, Timothy J; Drage, Deborah J; Wang, Susan; Apostolakis, Apostolos A; Cox, Timothy M; Cachón-González, M Begoña

    2012-09-01

    Tay-Sachs and Sandhoff diseases are lethal inborn errors of acid β-N-acetylhexosaminidase activity, characterized by lysosomal storage of GM2 ganglioside and related glycoconjugates in the nervous system. The molecular events that lead to irreversible neuronal injury accompanied by gliosis are unknown; but gene transfer, when undertaken before neurological signs are manifest, effectively rescues the acute neurodegenerative illness in Hexb-/- (Sandhoff) mice that lack β-hexosaminidases A and B. To define determinants of therapeutic efficacy and establish a dynamic experimental platform to systematically investigate cellular pathogenesis of GM2 gangliosidosis, we generated two inducible experimental models. Reversible transgenic expression of β-hexosaminidase directed by two promoters, mouse Hexb and human Synapsin 1 promoters, permitted progression of GM2 gangliosidosis in Sandhoff mice to be modified at pre-defined ages. A single auto-regulatory tetracycline-sensitive expression cassette controlled expression of transgenic Hexb in the brain of Hexb-/- mice and provided long-term rescue from the acute neuronopathic disorder, as well as the accompanying pathological storage of glycoconjugates and gliosis in most parts of the brain. Ultimately, late-onset brainstem and ventral spinal cord pathology occurred and was associated with increased tone in the limbs. Silencing transgenic Hexb expression in five-week-old mice induced stereotypic signs and progression of Sandhoff disease, including tremor, bradykinesia, and hind-limb paralysis. As in germline Hexb-/- mice, these neurodegenerative manifestations advanced rapidly, indicating that the pathogenesis and progression of GM2 gangliosidosis is not influenced by developmental events in the maturing nervous system.

  20. Characterization of inducible models of Tay-Sachs and related disease.

    Directory of Open Access Journals (Sweden)

    Timothy J Sargeant

    2012-09-01

    Full Text Available Tay-Sachs and Sandhoff diseases are lethal inborn errors of acid β-N-acetylhexosaminidase activity, characterized by lysosomal storage of GM2 ganglioside and related glycoconjugates in the nervous system. The molecular events that lead to irreversible neuronal injury accompanied by gliosis are unknown; but gene transfer, when undertaken before neurological signs are manifest, effectively rescues the acute neurodegenerative illness in Hexb-/- (Sandhoff mice that lack β-hexosaminidases A and B. To define determinants of therapeutic efficacy and establish a dynamic experimental platform to systematically investigate cellular pathogenesis of GM2 gangliosidosis, we generated two inducible experimental models. Reversible transgenic expression of β-hexosaminidase directed by two promoters, mouse Hexb and human Synapsin 1 promoters, permitted progression of GM2 gangliosidosis in Sandhoff mice to be modified at pre-defined ages. A single auto-regulatory tetracycline-sensitive expression cassette controlled expression of transgenic Hexb in the brain of Hexb-/- mice and provided long-term rescue from the acute neuronopathic disorder, as well as the accompanying pathological storage of glycoconjugates and gliosis in most parts of the brain. Ultimately, late-onset brainstem and ventral spinal cord pathology occurred and was associated with increased tone in the limbs. Silencing transgenic Hexb expression in five-week-old mice induced stereotypic signs and progression of Sandhoff disease, including tremor, bradykinesia, and hind-limb paralysis. As in germline Hexb-/- mice, these neurodegenerative manifestations advanced rapidly, indicating that the pathogenesis and progression of GM2 gangliosidosis is not influenced by developmental events in the maturing nervous system.