Equatorial E Region Electric Fields and Sporadic E Layer Responses to the Recovery Phase of the November 2004 Geomagnetic Storm

Science.gov (United States)

Moro, J.; Resende, L. C. A.; Denardini, C. M.; Xu, J.; Batista, I. S.; Andrioli, V. F.; Carrasco, A. J.; Batista, P. P.; Schuch, N. J.

2017-12-01

Equatorial E region electric fields (EEFs) inferred from coherent radar data, sporadic-E (Es) layers observed from a digital ionosonde data, and modeling results are used to study the responses of the equatorial E region over São Luís (SLZ, 2.3°S, 44.2°W, -7° dip angle), Brazil, during the super storm of November 2004. The EEF is presented in terms of the zonal (Ey) and vertical (Ez) components in order to analyze the corresponding characteristics of different types of Es seen in ionograms and simulated with the E region ionospheric model. We bring out the variabilities of Ey and Ez components with storm time changes in the equatorial E region. In addition, some aspects of the electric fields and Es behavior in three cases of weak, very weak, and strong Type II occurrences during the recovery phase of the geomagnetic storm are discussed. The connection between the enhanced occurrence and suppressions of the Type II irregularities and the q-type Es (Esq) controlled by electric fields, with the development or disruption of the blanketing sporadic E (Esb) layers produced by wind shear mechanism, is also presented. The mutual presence of Esq along with the Esb occurrences is a clear indicator of the secular drift of the magnetic equator and hence that of the equatorial electrojet (EEJ) over SLZ. The results show evidence about the EEJ and Es layer electrodynamics and coupling during geomagnetic disturbance time electric fields.

Searching for effects caused by thunderstorms in midlatitude sporadic E layers

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Barta, Veronika; Haldoupis, Christos; Sátori, Gabriella; Buresova, Dalia; Chum, Jaroslav; Pozoga, Mariusz; Berényi, Kitti A.; Bór, József; Popek, Martin; Kis, Árpád; Bencze, Pál

2017-08-01

Possible thunderstorm - sporadic E (Es) layer coupling effects are investigated during two measurement periods, one in 2013 and one in 2014. The analysis was based on ionospheric observations obtained from a Digisonde at Pruhonice, the Czech Republic, an ionosonde at Nagycenk, Hungary, and a 3.59 MHz five-point continuous HF Doppler system located in the western part of the Czech Republic. The latter is capable of detecting ionospheric wave-like variations caused by neutral atmospheric waves generated by thunderstorms. The present study searches for possible impacts on Es layers caused by the presence of two active thunderstorms: one passing across the Czech Republic on June 20, 2013 (19:00-01:00 LT), and one through Hungary on July 30, 2014 (11:00-01:00 LT). During these two time periods, presence and parameters of Es layer were inferred from ionograms, recorded every minute at Pruhonice and every two minutes at Nagycenk, whereas concurrent lightning activity was monitored by the LINET detection network. In addition, transient luminous events (TLEs) were also observed during both nights from Sopron, Hungary and from Nýdek, the Czech Republic. A noticeable fact was the reduction and disappearance of the ongoing Es layer activity during part of the time in both of the traversing thunderstorms. The analysis indicated that the critical frequency foEs dropped below ionosonde detection levels in both cases, possibly because of thunderstorm activity effects. This option, however, needs more case studies in order to be further substantiated.

Searching for effects caused by thunderstorms in midlatitude sporadic E layers

Czech Academy of Sciences Publication Activity Database

Barta, V.; Haldoupis, C.; Sátori, G.; Burešová, Dalia; Chum, Jaroslav; Pozoga, M.; Berényi, K. A.; Bór, J.; Popek, Martin; Kis, Á.; Bencze, P.

2017-01-01

Roč. 161, August (2017), s. 150-159 ISSN 1364-6826 R&D Projects: GA ČR(CZ) GC15-07281J; GA ČR(CZ) GAP209/12/2440; GA ČR(CZ) GA14-31899S Institutional support: RVO:68378289 Keywords : atmospheric gravity waves * ionosphere coupling * lightning * sporadic E layer * sprites * thunderstorm Subject RIV: DG - Athmosphere Sciences, Meteorology OBOR OECD: Meteorology and atmospheric sciences Impact factor: 1.326, year: 2016 https://arxiv.org/abs/1708.00270

Case study of inclined sporadic E layers in the Earth's ionosphere observed by CHAMP/GPS radio occultations: Coupling between the tilted plasma layers and internal waves

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Gubenko, Vladimir N.; Pavelyev, A. G.; Kirillovich, I. A.; Liou, Y.-A.

2018-04-01

We have used the radio occultation (RO) satellite data CHAMP/GPS (Challenging Minisatellite Payload/Global Positioning System) for studying the ionosphere of the Earth. A method for deriving the parameters of ionospheric structures is based upon an analysis of the RO signal variations in the phase path and intensity. This method allows one to estimate the spatial displacement of a plasma layer with respect to the ray perigee, and to determine the layer inclination and height correction values. In this paper, we focus on the case study of inclined sporadic E (Es) layers in the high-latitude ionosphere based on available CHAMP RO data. Assuming that the internal gravity waves (IGWs) with the phase-fronts parallel to the ionization layer surfaces are responsible for the tilt angles of sporadic plasma layers, we have developed a new technique for determining the parameters of IGWs linked with the inclined Es structures. A small-scale internal wave may be modulating initially horizontal Es layer in height and causing a direction of the plasma density gradient to be rotated and aligned with that of the wave propagation vector k. The results of determination of the intrinsic wave frequency and period, vertical and horizontal wavelengths, intrinsic vertical and horizontal phase speeds, and other characteristics of IGWs under study are presented and discussed.

Simultaneous lidar observation of peculiar sporadic K and Na layers at São José dos Campos (23.1°S, 45.9°W), Brazil

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Jiao, Jing; Yang, Guotao; Cheng, Xuewu; Liu, Zhengkuan; Wang, Jihong; Yan, Zhenzhong; Wang, Chi; Batista, Paulo; Pimenta, Alexandre; Andrioli, Vânia; Denardini, C. M.

2018-04-01

We present the first simultaneous observation of mesopause sodium (Na) and potassium (K) layer by a lidar which has Na and K channel simultaneously at the South Hemisphere site, São José dos Campos (23.1°S, 45.9°W). Measurements reported here were conducted on two nights with 3.5 and 8 h of observations in November 2016. On 20 November 2016, sporadic layers in both Na and K layer occurred above 100 km, and the higher layers corresponded well with sporadic E (Es) layer. And the density of Na at 100-105 km is higher than that at main layer around 90 km, but K density around 100 km is at least 3 times smaller compared with the K density around 90 km for the similar period. On 21 November 2016, both sporadic layers occurred in main layer height with obvious descending variations with time, which seems like tidal induced. Notably, the peak K/Na ratio slowly increased with time. And Na layer and K layer showed different processes along with time with K density reaching its maximum 1 h later than that of Na. Correlations of Na/K density, Es, and winds were also discussed.

Studies of sporadic E (Es) associated with the main ionospheric trough

International Nuclear Information System (INIS)

Rodger, A.S.; Morrell, C.; Dudeney, J.R.

1983-01-01

Sporadic E, or E(s) events under the main F region trough have been confirmed on the basis of ionograms from a vertical incidence ionosonde at Halley Bay, Antarctica. Analyses indicate that E(s) is frequently observable under both the equatorward and the poleward edges of the trough, as well as poleward of it. Before magnetic midnight, E(s) layers whose semithickness resembles those of the normal E layer are common, in contrast to layers seen after magnetic midnight which show the characteristics of thin E(s) layers. A possible explanation of the observed change in the E(s) layer characteristics at magnetic midnight is related to differences in the type and spectra of the precipitating particles. It is shown that the redistribution of ionization by the convection electric field may be important. 40 references

Analysis of wave-like oscillations in parameters of sporadic E layer and neutral atmosphere

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Mošna, Z.; Koucká Knížová, P.

2012-12-01

The present study mainly concerns the wave-like activity in the ionospheric sporadic E layer (Es) and in the lower lying stratosphere. The proposed analysis involves parameters describing the state of plasma in the sporadic E layer. Critical frequencies foEs and layer heights hEs were measured at the Pruhonice station (50°N, 14.5°E) during summer campaigns 2004, 2006 and 2008. Further, we use neutral atmosphere (temperature data at 10 hPa) data from the same time interval. The analysis concentrates on vertically propagating wave-like structures within distant atmospheric regions. By means of continuous wavelet transform (CWT) we have detected significant wave-like oscillation at periods covering tidal and planetary oscillation domains both in the Es layer parameters (some of them were reported earlier, for instance in works of Abdu et al., 2003; Pancheva and Mitchel, 2004; Pancheva et al., 2003; Šauli and Bourdillon, 2008) and in stratospheric temperature variations. Further analyses using cross wavelet transform (XWT) and wavelet coherence analysis (WTC) show that despite high wave-like activity in a wide period range, there are only limited coherent wave-like bursts present in both spectra. Such common coherent wave bursts occur on periods close to eigen-periods of the terrestrial atmosphere. We suppose that vertical coupling between atmospheric regions realized by vertically propagating planetary waves occurs predominantly on periods close to those of Rossby modes. Analysis of the phase shift between data from distant atmospheric regions reveals high variability and very likely supports the non-linear scenario of the vertical coupling provided by planetary waves.

Study of sporadic E layers based on GPS radio occultation measurements and digisonde data over the Brazilian region

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Resende, Laysa C. A.; Arras, Christina; Batista, Inez S.; Denardini, Clezio M.; Bertollotto, Thainá O.; Moro, Juliano

2018-04-01

This work presents new results about sporadic E-layers (Es layers) using GPS (global positioning system) radio occultation (RO) measurements obtained from the FORMOSAT-3/COSMIC satellites and digisonde data. The RO profiles are used to study the Es layer occurrence as well as its intensity of the signal-to-noise ratio (SNR) of the 50 Hz GPS L1 signal. The methodology was applied to identify the Es layer on RO measurements over Cachoeira Paulista, a low-latitude station in the Brazilian region, in which the Es layer development is not driven tidal winds only as it is at middle latitudes. The coincident events were analyzed using the RO technique and ionosonde observations during the year 2014 to 2016. We used the electron density obtained using the blanketing frequency parameter (fbEs) and the Es layer height (h'Es) acquired from the ionograms to validate the satellite measurements. The comparative results show that the Es layer characteristics extracted from the RO measurements are in good agreement with the Es layer parameters from the digisonde.

Observations of neutral winds, wind shears, and wave structure during a sporadic-E/QP event

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M. F. Larsen

2005-10-01

Full Text Available The second Sporadic E Experiment over Kyushu (SEEK-2 was carried out on 3 August 2002, during an active sporadic-E event that also showed quasi-periodic (QP echoes. Two rockets were launched into the event from Kagoshima Space Center in southern Japan 15 min apart. Both carried a suite of instruments, but the second rocket also released a trimethyl aluminum (TMA trail to measure the neutral winds and turbulence structure. In a number of earlier measurements in similar conditions, large winds and shears that were either unstable or close to instability were observed in the altitude range where the ionization layer occurred. The SEEK-2 wind measurements showed similar vertical structure, but unlike earlier experiments, there was a significant difference between the up-leg and down-leg wind profiles. In addition, wave or billow-like fluctuations were evident in the up-leg portion of the trail, while the lower portion of the down-leg trail was found to have extremely strong turbulence that led to a rapid break-up of the trail. The large east-west gradient in the winds and the strong turbulence have not been observed before. The wind profiles and shears, as well as the qualitative characteristics of the strong turbulence are presented, along with a discussion of the implications of the dynamical features. Keywords. Ionosphere (Mid-latitude ionosphere; Ionospheric irregularities; Electric field and currents

Energetics and structure of the lower E region associated with sporadic E layer

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K.-I. Oyama

2008-09-01

Full Text Available The electron temperature (T_e, electron density (N_e, and two components of the electric field were measured from the height of 90 km to 150 km by one of the sounding rockets launched during the SEEK-2 campaign. The rocket went through sporadic E layer (E_s at the height of 102 km–109 km during ascent and 99 km–108 km during decent, respectively. The energy density of thermal electrons calculated from N_e and T_e shows the broad maximum in the height range of 100–110 km, and it decreases towards the lower and higher altitudes, which implies that a heat source exists in the height region of 100 km–110 km. A 3-D picture of E_s, that was drawn by using T_e, N_e, and the electric field data, corresponded to the computer simulation; the main structure of E_s is projected to a higher altitude along the magnetic line of force, thus producing irregular structures of T_e, N_e and electric field in higher altitude.

Sporadic-E and spread-F in high latitude region

International Nuclear Information System (INIS)

Tao, Kazuhiko

1974-01-01

The heretofore made morphological studies of sporadic-E and spread-F as the typical irregularities of electron density are reviewed. These phenomena have close correlation with other geophysical phenomena which occur in the atmosphere of superhigh altitude in high latitude region. Many of these phenomena occur from same causes. Although the quantitative data are insufficient, the sporadic-E and spread-F in high latitude region are supposed to be caused by the precipitating charged particles falling from magnetosphere. A system, which can observe such phenomena simultaneously using the measuring instruments carried by satellites in the atmosphere of high altitude over high latitude region, is desirable to solve such problems. In detail, the morphological study on sporadic-E obtained from the observation of vertically projected ionosphere and the morphological study on sporadic-E from the observation of forward scattering and slanting entrance are reviewed. The correlation of the occurrence frequency of sporadic-E with solar activity, geomagnetic activity and other phenomena was studied. The morphological study on spread-F occurrence is reviewed. The observation of the spread-F in high latitude region by the application of top side sounding is reviewed. The correlation of the sporadic-E and spread-F in high latitude region with other geophysical phenomena is discussed. Finally, the discrete phenomenon and the diffuse phenomenon are discussed too. (Iwakiri, K.)

On the influence of solar activity on the mid-latitude sporadic E layer

Science.gov (United States)

Pezzopane, Michael; Pignalberi, Alessio; Pietrella, Marco

2015-09-01

To investigate the influence of solar cycle variability on the sporadic E layer (Es), hourly measurements of the critical frequency of the Es ordinary mode of propagation, foEs, and of the blanketing frequency of the Es layer, fbEs, recorded from January 1976 to December 2009 at the Rome (Italy) ionospheric station (41.8° N, 12.5° E), were examined. The results are: (1) a high positive correlation between the F10.7 solar index and foEs as well as between F10.7 and fbEs, both for the whole data set and for each solar cycle separately, the correlation between F10.7 and fbEs being much higher than the one between F10.7 and foEs; (2) a decreasing long-term trend of the F10.7, foEs and fbEs time series, with foEs decreasing more rapidly than F10.7 and fbEs; (3) clear and statistically significant peaks at 11 years in the foEs and fbEs time series, inferred from Lomb-Scargle periodograms.

Energetics and structure of the lower E region associated with sporadic E layer

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K.-I. Oyama

2008-09-01

Full Text Available The electron temperature (Te, electron density (Ne, and two components of the electric field were measured from the height of 90 km to 150 km by one of the sounding rockets launched during the SEEK-2 campaign. The rocket went through sporadic E layer (Es at the height of 102 km–109 km during ascent and 99 km–108 km during decent, respectively. The energy density of thermal electrons calculated from Ne and Te shows the broad maximum in the height range of 100–110 km, and it decreases towards the lower and higher altitudes, which implies that a heat source exists in the height region of 100 km–110 km. A 3-D picture of Es, that was drawn by using Te, Ne, and the electric field data, corresponded to the computer simulation; the main structure of Es is projected to a higher altitude along the magnetic line of force, thus producing irregular structures of Te, Ne and electric field in higher altitude.

Sporadic-E associated with the Leonid meteor shower event of November 1998 over low and equatorial latitudes

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H. Chandra

2001-01-01

Full Text Available Rapid radio soundings were made over Ahmedabad, a low latitude station during the period 16–20 November 1998 to study the sporadic-E layer associated with the Leonid shower activity using the KEL Aerospace digital ionosonde. Hourly ionograms for the period 11 November to 24 November were also examined during the years from 1994 to 1998. A distinct increase in sporadic-E layer occurrence is noticed on 17, 18 and 19 November from 1996 to 1998. The diurnal variations  of  f0Es and fbEs also show significantly enhanced values for the morning hours of 18 and 19 November 1998. The ionograms clearly show strong sporadic-E reflections at times of peak shower activity with multiple traces in the altitude range of 100–140 km in few ionograms. Sporadic-E layers with multiple structures in altitude are also seen in some of the ionograms (quarter hourly at Thumba, situated near the magnetic equator. Few of ionograms recorded at Kodaikanal, another equatorial station, also show sporadic- E reflections in spite of the transmitter power being significantly lower. These new results highlighting the effect of intense meteor showers in the equatorial and low latitude E-region are presented.Key words. Ionosphere (equatorial ionosphere – Radio science (ionospheric physics

Strong Sporadic E Occurrence Detected by Ground-Based GNSS

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Sun, Wenjie; Ning, Baiqi; Yue, Xinan; Li, Guozhu; Hu, Lianhuan; Chang, Shoumin; Lan, Jiaping; Zhu, Zhengping; Zhao, Biqiang; Lin, Jian

2018-04-01

The ionospheric sporadic E (Es) layer has significant impact on radio wave propagation. The traditional techniques employed for Es layer observation, for example, ionosondes, are not dense enough to resolve the morphology and dynamics of Es layer in spatial distribution. The ground-based Global Navigation Satellite Systems (GNSS) technique is expected to shed light on the understanding of regional strong Es occurrence, owing to the facts that the critical frequency (foEs) of strong Es structure is usually high enough to cause pulse-like disturbances in GNSS total electron content (TEC), and a large number of GNSS receivers have been deployed all over the world. Based on the Chinese ground-based GNSS networks, including the Crustal Movement Observation Network of China and the Beidou Ionospheric Observation Network, a large-scale strong Es event was observed in the middle latitude of China. The strong Es shown as a band-like structure in the southwest-northeast direction extended more than 1,000 km. By making a comparative analysis of Es occurrences identified from the simultaneous observations by ionosondes and GNSS TEC receivers over China middle latitude statistically, we found that GNSS TEC can be well employed to observe strong Es occurrence with a threshold value of foEs, 14 MHz.

Sporadic-E associated with the Leonid meteor shower event of November 1998 over low and equatorial latitudes

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H. Chandra

Full Text Available Rapid radio soundings were made over Ahmedabad, a low latitude station during the period 16–20 November 1998 to study the sporadic-E layer associated with the Leonid shower activity using the KEL Aerospace digital ionosonde. Hourly ionograms for the period 11 November to 24 November were also examined during the years from 1994 to 1998. A distinct increase in sporadic-E layer occurrence is noticed on 17, 18 and 19 November from 1996 to 1998. The diurnal variations 
of  f₀E_s and f_bE_s also show significantly enhanced values for the morning hours of 18 and 19 November 1998. The ionograms clearly show strong sporadic-E reflections at times of peak shower activity with multiple traces in the altitude range of 100–140 km in few ionograms. Sporadic-E layers with multiple structures in altitude are also seen in some of the ionograms (quarter hourly at Thumba, situated near the magnetic equator. Few of ionograms recorded at Kodaikanal, another equatorial station, also show sporadic- E reflections in spite of the transmitter power being significantly lower. These new results highlighting the effect of intense meteor showers in the equatorial and low latitude E-region are presented.

Key words. Ionosphere (equatorial ionosphere – Radio science (ionospheric physics

SEEK-2 (Sporadic-E Experiment over Kyushu 2 − Project Outline, and Significance

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R. Pfaff

2005-10-01

Full Text Available SEEK-2 (Sporadic-E Experiment over Kyushu 2 is an observation campaign to study the spatial structure of the field-aligned irregularity (FAI and sporadic-E(Es-layer by means of two sounding rockets and a ground-based observation network with radars and optical instruments. The experiment was successfully conducted on 3 August 2002, with successive launches of two sounding rockets from the Uchinoura Space Center (USC of the Japan Aerospace Exploration Agency (JAXA. The timing of the experiment was carefully selected, while intense quasi-periodic (QP echoes were observed with two radars in Tanegashima. The main Es-layer, with its double-layered structure, was observed at altitudes of 103–105 km, the presence of which was well accounted for by the ion accumulation due to neutral-wind shear. Several minor peaks were detected in the electron density profiles at altitudes of up to 130 km. The intensity of the electric field was 5–10 mV/m and showed intense fluctuations below 110 km. Wave-like variation of the electric field was seen above 110 km. From radar experiments, we found that QP echoes appeared around 105 km, which agreed well with the main Es-layer height. The QP echoes propagated to the west-northwest, with frontal structures elongated from north-northeast to south-southwest. Radar observations conduced throughout the SEEK-2 period, on the other hand, showed that frontal structures of the QP echoes were most frequently propagated to the southeast. This result was consistent with the direction of gravity-wave propagation observed with the OH imager during the same period. The rocket beacon experiment with the Es-layers revealed the spatial structure of the plasma densities. On the basis of these results and those from SEEK-1 in 1996, we examined the structures of the nighttime mid-latitude E-region. We concluded that the QP echoes reflect the horizontal structures of the main Es-layers. The source of the structures was not clearly

Temporal evolution of the HF-enhanced plasma line in sporadic E

International Nuclear Information System (INIS)

Djuth, F.T.; Gonzales, C.A.

1988-01-01

The high-power, high-frequency (HF) facility at Arecibo, Puerto Rico, has been used to study the excitation of Langmuir waves in mid-latitude sporadic E. Measurements of the temporal evolution of so-called HF-enhanced plasma line (HFPL) were made using the Arecibo 430-MHz radar. After HF turn-on in the plasma the HFPL exhibits a rapid growth phase followed by a quick overshoot. During periods of strong HFPL excitation the e-folding growth time of the HFPL power is typically approx-lt 20 μs, and the total overshoot period is ∼1 ms. On the basis of the current observations, mode conversion of the HF wave into Langmuir waves near HF reflection appears to be a promising mechanism for the production of Langmuir waves in sporadic E. Caviton formation at the critical layer is expected to accompany this process, and there is some evidence that the 430-MHz radar is probing the plasma in a region where density cavities of this nature form. While no specific explanation is offered for the HFPL overshoot, it appears that this phenomenon is fundamental to the Langmuir wave excitation process

Height and critical frequency variations of the sporadic-E layer at midlatitudes

Czech Academy of Sciences Publication Activity Database

Šauli, Petra; Bourdillon, A.

2008-01-01

Roč. 70, č. 15 (2008), s. 1904-1910 ISSN 1364-6826 R&D Projects: GA AV ČR IAA300420704 Grant - others:European Union(XE) COST 296 Institutional research plan: CEZ:AV0Z30420517 Keywords : Sporadic E * Planetary waves * Tidal waves * Mid-latitude ionosphere * Wavelet transform Subject RIV: DG - Athmosphere Sciences, Meteorology Impact factor: 1.667, year: 2008

A case study on generation mechanisms of a sporadic sodium layer above Tromsø (69.6° N during a night of high auroral activity

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T. Takahashi

2015-08-01

Full Text Available We have quantitatively evaluated generation mechanisms of a sporadic sodium layer (SSL based on observational data obtained by multiple instruments at a high-latitude station: Ramfjordmoen, Tromsø, Norway (69.6° N, 19.2° E. The sodium lidar observed an SSL at 21:18 UT on 22 January 2012. The SSL was observed for 18 min, with a maximum sodium density of about 1.9 × 1010 m−3 at 93 km with a 1.1 km thickness. The European Incoherent Scatter (EISCAT UHF radar observed a sporadic E layer (Es layer above 90 km from 20:00 to 23:00 UT. After 20:00 UT, the Es layer gradually descended and reached 94 km at 21:18 UT when the SSL appeared at the same altitude. In this event, considering the abundance of sodium ions (10 % or less, the Es layer could provide only about 37 % or less of the sodium atoms to the SSL. We have investigated a temporal development of the normal sodium ion layer with a consideration of chemical reactions and the effect of the (southwestward electric field using observational values of the neutral temperature, electron density, horizontal neutral wind, and electric field. This calculation has shown that those processes, including contributions of the Es layer, would provide about 88 % of sodium atoms of the SSL. The effects of meteor absorption and auroral particle sputtering appear to be less important. Therefore, we have concluded that the major source of the SSL was sodium ions in a normal sodium ion layer. Two processes – namely the downward transportation of sodium ions from a normal sodium ion layer due to the electric field and the additional supply of sodium ions from the Es layer under relatively high electron density conditions (i.e., in the Es layer – played a major role in generating the SSL in this event. Furthermore, we have found that the SSL was located in a lower-temperature region and that the temperature inside the SSL did not show any remarkable temperature enhancements.

Morphological study of the field-aligned E-layer irregularities observed by the Gadanki VHF radar

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C. J. Pan

2004-11-01

Full Text Available We report on the field-aligned irregularities observed in the low-latitude sporadic E-layer (Es with the Gadanki (13.5° N, 79.2° E; geomagnetic latitude 6.3° N VHF radar. The radar was operated intermittently for 15 days during the summer months in 1998 and 1999, for both daytime and nighttime observation. The total observation periods are 161h for the nighttime and 68h for the daytime. The observations were used to study the percentage of occurrence of the E-region echoes for both daytime and nighttime. The statistical characteristics of the mean radial velocity and spectral width are presented for three cases based on the echo occurrence characteristics and the altitude of observations (from 90 to 140km ranges, namely, the lower E-region daytime (90-110km, the lower E-region nighttime (90-105km and the upper E-region nighttime (105-140km echoes. The results are compared with that of Piura, a low-latitude station located at about the same geomagnetic latitude, but to the south of the equator. By comparing the behaviors of the lower E-region radar echoes of the summer months between Gadanki and Piura, we find that the lower altitude echoes below about 100km are rarely reported in Piura but commonly seen in Gadanki. Features of the nighttime echoes observed by these two radars are quite similar but daytime FAI echoes are again seldom detected by Piura.

The influence of tidal winds in the formation of blanketing sporadic e-layer over equatorial Brazilian region

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Resende, Laysa Cristina Araujo; Batista, Inez Staciarini; Denardini, Clezio Marcos; Batista, Paulo Prado; Carrasco, Alexander José; Andrioli, Vânia Fátima; Moro, Juliano

2018-06-01

This work analysis the blanketing sporadic layers (Esb) behavior over São Luís, Brazil (2° 31‧ S, 44° 16‧ W, dip: -4.80) which is classified as a transition region between equatorial and low-latitude. Hence, some peculiarities can appear as Esb occurrence instead of the common Esq, which is a non-blanketing irregularity layer. The analysis presented here was obtained using a modified version of a theoretical model for the E region (MIRE), which computes the densities of the metallic ions (Fe+ and Mg+) and the densities of the main molecular ions (NO+, O2+, N2+) by solving the continuity and momentum equations for each one of them. In that model, the Es layer physics driven by both diurnal and semidiurnal tidal winds are taken into account and it was extended in height coverage by adding a novel neutral wind model derived from the all-sky meteor radar measurements. Thus, we provide more trustworthy results related to the Es layer formation in the equatorial region. We verified the contribution of each tidal wind component to the Esb layer formation in this equatorial region. Additionally, we compared the Es layer electron density computed by MIRE with the data obtained by using the blanketing frequency parameter (fbEs) deduced from ionograms. The results show that the diurnal component of the tidal wind is more important in the Esb layer formation whereas the semidiurnal component has a little contribution in our simulations. Finally, it was verified that the modified MIRE presented here can be used to study the Esb layers occurrence over the equatorial region in the Brazilian sector.

Sporadic E S Layers at High Latitudes During a Magnetic Storm of March 17, 2015 According to the Vertical and Oblique Ionospheric Sounding Data

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Blagoveshchensky, D. V.; Maltseva, O. A.; Anishin, M. M.; Rogov, D. D.

2017-11-01

We consider the behavior of the parameters of the ionospheric E s layers according to the vertical sounding at the Sodankylä observatory and oblique sounding at the Lovozero (Murmansk region)—Gor'kovskaya station (Leningrad region) path during a superstorm of March 17, 2015. Temporal and spatial behavior of these parameters is compared. It was found that the storm significantly distorted the normal course of variations of the sporadic E s layer characteristics. Specific behavior of the layers during a storm at points separated by about 300 km was detected. With the help of ray tracing calculations using the IRI model, oblique sounding ionograms were constructed for the radio path analyzed. Primary attention is given to the maximum usable frequency of the F 2 layer—MUF- F 2. Additionally, for the disturbed conditions where there is only a high-power E s layer on the experimental ionograms, the values of MUF- E s and the ratio K =MUF- E s/ f o E s for various cutoff frequencies f o E s of the E s layer and its altitudes {h}_{E_s} are calculated within the framework of the well-known approximations. Calculations for the case of weak disturbance and semitransparent E s layers are carried out with the IRI model adapted to the current diagnostics parameters. It was found that the calculated and experimental values of MUF- F 2 are close to each other or coincide, while this cannot be said about MUF- E s. The calculated and experimental values of MUF- E s can be matched in the model of mirror reflection from a flat layer for intense layers and the model of the E layer for thick E s layers of low intensity.

Analysis of wave-like oscillations in parameters of sporadic E layer and neutral atmosphere

Czech Academy of Sciences Publication Activity Database

Mošna, Zbyšek; Koucká Knížová, Petra

90-91, SI (2012), s. 172-178 ISSN 1364-6826. [IAGA/ICMA/CAWSES-II TG4 Workshop on Vertical Coupling in the Atmosphere-Ionosphere System /4./. Prague, 14.02.2011-18.02.2011] R&D Projects: GA AV ČR IAA300420704 Institutional support: RVO:68378289 Keywords : Sporadic E * Planetary waves * Tidal waves * Mid-latitude ionosphere Subject RIV: DG - Athmosphere Sciences, Meteorology Impact factor: 1.417, year: 2012 http://www.sciencedirect.com/science/article/pii/S1364682612001186

The lunar tide in sporadic E

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R. J. Stening

1999-10-01

Full Text Available It seems that the wind shear theory is accepted for the explanation of sporadic E at mid and low latitudes. Some examples from Arecibo are displayed to show this. The effect of lunar tides should then modify the wind-shear theory in a manner that yields the observed features of the lunar tide in the critical frequency foEs and the height h'Es of the sporadic E. This is shown to imply that the phase of the lunar tide in h'Es should be the same as the phase of the lunar tide in the eastward wind and that the phase of the lunar tide in foEs is three hours later. Hourly values of foEs, f bEs (the blanketing critical frequency and h'Es from several observatories are analysed for the lunar semidiurnal tide. It is found that the phase of the tide in foEs is often about 3 hours later than for h'Es in agreement with the theory. Seasonal variations in the tide are also examined with the statistically most significant results (largest amplitudes usually occurring in summer. After reviewing the many difficulties associated with determining the lunar tide in Es, both experimentally and theoretically, the analysed phase results are compared with what might be expected from Hagan's global scale wave model. Agreement is only fair (a success rate of 69% among the cases examined but probably as good as might be expected.Key words. Ionosphere (ionosphere – atmosphere interactions – ionospheric irregularities, Meteorology and atmosphere dynamics (waves and tides

Effects of sporadic E-layer characteristics on spread-F generation in the nighttime ionosphere near a northern equatorial anomaly crest during solar minimum

Science.gov (United States)

Lee, C. C.; Chen, W. S.

2015-06-01

This study is to know how the characteristics of sporadic E-layer (Es-layer) affect the generation of spread-F in the nighttime ionosphere near the crest of equatorial ionization anomaly during solar minimum. The data of Es-layer parameters and spread-F are obtained from the Chungli ionograms of 1996. The Es-layer parameters include foEs (critical frequency of Es-layer), fbEs (blanketing frequency of Es-layer), and Δf (≡foEs-fbEs). Results show that the nighttime variations of foEs and fbEs medians (Δf medians) are different from (similar to) that of the occurrence probabilities of spread-F. Because the total number of Es-layer events is greater than that of spread-F events, the comparison between the medians of Es-layer parameters and the occurrence probabilities of spread-F might have a shortfall. Further, we categorize the Es-layer and spread-F events into each frequency interval of Es-layer parameters. For the occurrence probabilities of spread-F versus foEs, an increasing trend is found in post-midnight of all three seasons. The increasing trend also exists in pre-midnight of the J-months and in post-midnight of all seasons, for the occurrence probabilities of spread-F versus Δf. These demonstrate that the spread-F occurrence increases with increasing foEs and/or Δf. Moreover, the increasing trends indicate that polarization electric fields generated in Es-layer assist to produce spread-F, through the electrodynamical coupling of Es-layer and F-region. Regarding the occurrence probabilities of spread-F versus fbEs, the significant trend only appears in post-midnight of the E-months. This implies that fbEs might not be a major factor for the spread-F formation.

Solar eclipse effects of 22 July 2009 on Sporadic-E

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G. Chen

2010-02-01

Full Text Available The total solar eclipse of 22 July 2009, was visible from some regions of China and the intense sporadic-E (Es that broke out during the solar eclipse period over the eastern China provided a unique chance to study solar eclipse effects on the Es-layer. The ground based high-frequency (HF vertical-incidence and oblique-incidence backscatter radio systems in Wuhan and an HF oblique receivers located in Suzhou were operated to detect the Es-layer. The vertical, oblique and backscatter ionograms of 22 and 23 July were recorded, processed and analyzed. The analyzing results show that the critical frequency of Es, the hop number and power of the rays transmitted from Wuhan to Suzhou as well as the Doppler frequency shift of the one-hop oblique-incidence waves reflected by the Es-layer all increased during the solar eclipse period. These variations are displayed in the paper and explained to be induced by the wind-field, which is produced by the powerful meridional air flows from the sunshine region to the moon's shadow.

Solar eclipse effects of 22 July 2009 on Sporadic-E

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G. Chen

2010-02-01

Full Text Available The total solar eclipse of 22 July 2009, was visible from some regions of China and the intense sporadic-E (Es that broke out during the solar eclipse period over the eastern China provided a unique chance to study solar eclipse effects on the Es-layer. The ground based high-frequency (HF vertical-incidence and oblique-incidence backscatter radio systems in Wuhan and an HF oblique receivers located in Suzhou were operated to detect the Es-layer. The vertical, oblique and backscatter ionograms of 22 and 23 July were recorded, processed and analyzed. The analyzing results show that the critical frequency of Es, the hop number and power of the rays transmitted from Wuhan to Suzhou as well as the Doppler frequency shift of the one-hop oblique-incidence waves reflected by the Es-layer all increased during the solar eclipse period. These variations are displayed in the paper and explained to be induced by the wind-field, which is produced by the powerful meridional air flows from the sunshine region to the moon's shadow.

Observations of peculiar sporadic sodium structures and their relation with wind variations

Science.gov (United States)

Sridharan, S.; Prasanth, P. Vishnu; Kumar, Y. Bhavani; Ramkumar, Geetha; Sathishkumar, S.; Raghunath, K.

2009-04-01

Resonance lidar observations of sodium density in the upper mesosphere region over Gadanki (13.5°N, 79.2°E) rarely show complex structures with rapid enhancements of sodium density, completely different from normal sporadic sodium structures. The hourly averaged meteor radar zonal winds over Trivandrum (8.5°N, 76.5°E) show an eastward shear with altitude during the nights, when these events are formed. As suggested by Kane et al. [2001. Joint observations of sodium enhancements and field-aligned ionospheric irregularities. Geophysical Research Letters 28, 1375-1378], our observations show that the complex structures may be formed due to Kelvin-Helmholtz instability, which can occur in the region of strong wind shear.

More evidence for a planetary wave link with midlatitude E region coherent backscatter and sporadic E layers

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K. Schlegel

Full Text Available Measurements of midlatitude E region coherent backscatter obtained during four summers with SESCAT, a 50 MHz Doppler system operating in Crete, Greece, and concurrent ionosonde recordings from the same ionospheric volume obtained with a CADI for one of these summers, are used to analyse the long-term variability in echo and Es occurrence. Echo and Es layer occurrences, computed in percent of time over a 12-h nighttime interval, take the form of time sequences. Linear power spectrum analysis shows that there are dominant spectral peaks in the range of 2–9 days, the most commonly observed periods appearing in two preferential bands, of 2–3 days and 4–7 days. No connection with geomagnetic activity was found. The characteristics of these periodicities compare well with similar properties of planetary waves, which suggests the possibility that planetary waves are responsible for the observed long-term periodicities. These findings indicate also a likely close relation between planetary wave (PW activity and the well known but not well understood seasonal Es dependence. To test the PW postulation, we used simultaneous neutral wind data from the mesopause region around 95 km, measured from Collm, Germany. Direct comparison of the long-term periodicities in echo and Es layer occurrence with those in the neutral wind show some reasonable agreement. This new evidence, although not fully conclusive, is the first direct indication in favour of a planetary wave role on the unstable midlatitude E region ionosphere. Our results suggest that planetary waves observation is a viable option and a new element into the physics of midlatitude Es layers that needs to be considered and investigated.Key words: Ionosphere (ionosphere irregularities; mid-latitude ionosphere – Meteorology and atmospheric dynamics (waves and tides

A spectral study of the mid-latitude sporadic E layer characteristic oscillations comparable to those of the tidal and the planetary waves

Science.gov (United States)

Pignalberi, A.; Pezzopane, M.; Zuccheretti, E.

2015-01-01

In this paper different spectral analyses are employed to investigate the tidal and planetary wave periodicities imprinted in the following two main characteristics of the sporadic E (Es) layer: the top frequency (ftEs) and the lowest virtual height (h‧Es). The study is based on ionograms recorded during the summertime of 2013, and precisely in June, July, August and September, by the Advanced Ionospheric Sounder by Istituto Nazionale di Geofisica e Vulcanologia (AIS-INGV) ionosondes installed at Rome (41.8°N, 12.5°E) and Gibilmanna (37.9°N, 14.0°E), Italy. It was confirmed that the diurnal and semidiurnal atmospheric tides play a fundamental role in the formation of the mid-latitude Es layers, acting through their vertical wind-shear forcing of the long-living metallic ions in the lower thermosphere, and at the same time it was found that the planetary atmospheric waves might affect the Es layers acting through their horizontal wind-shear forcing with periods close to the normal Rossby modes, that is 2, 5, 10 and 16 days. The wavelet analysis shows also that the ftEs and h‧Es tidal oscillations undergo a strong amplitude modulation with periods of several days and with important differences between the two parameters. This amplitude modulation, characterizing markedly the first thirty days of the ftEs spectrogram, suggests that Es layers are affected indirectly by planetary waves through their nonlinear interaction with the atmospheric tides at lower altitudes. This study wants to be a continuation of the Haldoupis et al. (2004) work in order to verify their results for the foEs characteristic and on the other hand to extend the study also to the h‧Es characteristic not yet shown so far. Anyhow, the study confirms that ionosonde data, especially those registered in summertime, represent a powerful tool for studying tidal and planetary waves properties and their climatology in the mesosphere-low-thermosphere region.

The interaction between stratospheric monthly mean regional winds and sporadic-E

International Nuclear Information System (INIS)

Çetin, Kenan; Korlaelçi, Serhat; Özcan, Osman

2017-01-01

In the present study, a statistical investigation is carried out to explore whether there is a relationship between the critical frequency (foEs) of the sporadic-E layer that is occasionally seen on the E region of the ionosphere and the quasi-biennial oscillation (QBO) that flows in the east–west direction in the equatorial stratosphere. Multiple regression model as a statistical tool was used to determine the relationship between variables. In this model, the stationarity of the variables (foEs and QBO) was firstly analyzed for each station (Cocos Island, Gibilmanna, Niue Island, and Tahiti). Then, a co-integration test was made to determine the existence of a long-term relationship between QBO and foEs. After verifying the presence of a long-term relationship between the variables, the magnitude of the relationship between variables was further determined using the multiple regression model. As a result, it is concluded that the variations in foEs were explainable with QBO measured at 10 hPa altitude at the rate of 69%, 94%, 79%, and 58% for Cocos Island, Gibilmanna, Niue Island, and Tahiti stations, respectively. It is observed that the variations in foEs were explainable with QBO measured at 70 hPa altitude at the rate of 66%, 69%, 53%, and 47% for Cocos Island, Gibilmanna, Niue Island, and Tahiti stations, respectively. (paper)

Daytime dependence of disturbances of ionospheric Es-layers connected to earthquakes

Science.gov (United States)

Liperovskaya, E. V.; Liperovsky, A. V.; Meister, C.-V.; Silina, A. S.

2012-04-01

In the present work variations of the semi-transparency of the sporadic E-layer of the ionosphere due to seismic activities are studied. The semi-transparency Q is determined by the blanketing frequency fbEs and the characteristic frequency foEs, Q = (foEs - fbEs)/fbEs. At low values of the blanketing frequency fbEs, the critical frequency foEs does not describe the maximum ionisation density of the Es-layer, as the critical frequencies of regular ionospheric layers (e.g. foF2) do, but it describes the occurrence of small-scall (tenths of meters) inhomogeneities of the ionisation density along the vertical in the layer. The maximum ionisation density of the sporadic layer is proportional to the square of fbEs. In the case of vertical ionospheric sounding, the sporadic layer becomes transparent for signals with frequencies larger than fbEs. Investigations showed that about three days before an earthquake an increase of the semi-transparency interval is observed during sunset and sunrise. In the present work, analogous results are found for data of the vertical sounding stations "Tokyo" and "Petropavlovsk-Kamchatsky". Using the method of superposition of epoches, more than 50 earthquakes with magnitudes M > 5, depths h < 40 km, and distances between the station and the epicenter R < 300 km are considered in case of the vertical sounding station "Tokyo". More than 20 earthquakes with such parameters were analysed in case of the station "Petropavlovsk-Kamchatsky". Days with strong geomagnetic activity were excluded from the analysis. According to the station "Petropavlovsk-Kamchatsky" about 1-3 days before earthquakes, an increase of Es-spread is observed a few hours before midnight. This increase is a sign of large-scale inhomogeneities in the sporadic layers.

Allele doses of apolipoprotein E type {epsilon}4 in sporadic late-onset Alzheimer`s disease

Energy Technology Data Exchange (ETDEWEB)

Lucotte, G.; Aouizerate, A.; Gerard, N. [Regional Center of Neurogenetics, Paris (France)] [and others

1995-12-18

Apoliprotein E, type {epsilon}4 allele (ApoE-{epsilon}4) is associated with late-onset sporadic Alzheimer`s disease (AD). We have found that the cumulative probability of remaining unaffected over time decreases for each dose of ApoE-{epsilon}4 in sporadic, late-onset French AD. The effect of genotypes on age at onset of AD was analyzed using the product limit method, to compare unaffected groups during aging. 26 refs., 2 figs., 1 tab.

Numerical simulation of mid-latitude ionospheric E-region based on SEEK and SEEK-2 observations

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T. Yokoyama

2005-10-01

Full Text Available Observational campaigns of the mid-latitude ionospheric E-region with sounding rockets and ground-based instruments were conducted in 1996 (SEEK and 2002 (SEEK-2. Both of them were successfully conducted to bring important findings about the mid-latitude E-region and quasi-periodic (QP VHF radar echoes. The observational results in the SEEK and the SEEK-2 are compared with numerical simulations and discussed in this paper. While sporadic-E (Es-layers are actually formed by the observed neutral wind, it is difficult for the constant wind shear to produce the sharp Es-layer gradient. However, once they are formed in the lower E-region, they cannot easily be dissipated by the simple diffusive motion. The polarization electric field, calculated under the condition at the rocket launch time, shows similar amplitude and structure to the measurement around the Es-layer altitude. The structure of the plasma density and the electric field above the Es-layer observed in the SEEK-2 showed a wave-like pattern up to an altitude of 150 km. Considering a mapping of the polarization electric field generated within the Es-layers, gravity waves are the possible source of the wave-like structure of the measured electric fields and sub-peaks of the electron density above the main Es-layers. Fluctuation of the measured magnetic field is reproduced by Hall or field-aligned current driven by the polarization electric field. The current theoretical models for QP echoes and the polarization electric field are basically verified by the discussion in this paper. Keywords. Ionospheric irregularities – Mid-latitude ionosphere – Numerical simulation studies

Evidence of the Dampening Effect of Dense E-region Structures on E-F Coupling

Science.gov (United States)

Helmboldt, J.

2012-12-01

Results from a combination of instruments including ionosondes, GPS receivers, the Very Large Array (VLA), and the Long Wavelength Array (LWA) are used to demonstrate the role structure within the E-region plays in coupling between instabilities within the E and F regions at midlatitudes. VLA observations of cosmic sources at 74 MHz during summer nighttime in 2002 detected northwest-to-southeast aligned wavefronts, consistent with medium-scale traveling ionospheric disturbances (MSTIDs). These waves were only found when contemporaneous observations from nearby ionosondes detected echoes from sporadic-E layers. However, when the peak density of these layers was high (foEs> 3 MHz), there were no MSTIDs detected. Similar results are presented using the first station of the LWA, LWA1, to perform all-sky imaging of dense E-region structures (sporadic-E "clouds") via coherent scattering of distant analog TV broadcasts at 55 MHz. These observations were conducted during summer/autumn 2012 and include simultaneous GPS-based observations of F-region disturbances.Left: LWA1 all-sky image of ionospheric echoes of analog TV transmissions at 55.25 MHz. Right: Doppler speed maps for the brightest echoes.

A multi-platform investigation of midlatitude sporadic E and its ties to E–F coupling and meteor activity

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J. Helmboldt

2016-05-01

Full Text Available This paper describes the results of a multi-platform observing campaign aimed at studying midlatitude sporadic E (Es and associated ionospheric phenomena. The assets used were the digisonde in Boulder, Colorado; the first station of the Long Wavelength Array, LWA1, in New Mexico; the transmitters of the radio station WWV in Colorado; and 61 continuously operating GPS receivers between LWA1 and WWV. The results show that southwestward-directed medium-scale traveling ionospheric disturbances (MSTIDs were substantially more prevalent when Es was detected. The amplitudes of these correlate with a plasma frequency up to about 4.5 MHz. For fp ≳ 5 MHz, the MSTIDs become significantly weaker and basically vanish above  ∼ 6.5 MHz. The prevalence of meteor trail reflections observed with LWA1 also correlates with fp up to about 4.5 MHz; above this limit, the relationship exhibits a significant turnover. The observed intensity of coherent backscatter from Es field-aligned irregularities (FAIs also correlates with inferred plasma frequency. However, this trend continues to higher frequencies with a peak near 6 MHz, followed by a much more subtle turnover. The reflected power from Es structures observed with LWA1 is significantly more correlated on spatial scales between 10 and 40 km. The magnitude of this correlation increases with fp up to  ∼  6 MHz, above which it drops. These results are consistent with the following: (1 southwestward-directed MSTIDs are produced via E–F coupling; (2 this coupling is stronger when the Es layer, seeded by meteor ablation, is more dense; (3 the coupling is substantially diminished for Es layers harboring extremely dense structures (fp ≳ 5 MHz.

Seizures in E200K familial and sporadic Creutzfeldt-Jakob disease.

Science.gov (United States)

Appel, S; Chapman, J; Cohen, O S; Rosenmann, H; Nitsan, Z; Blatt, I

2015-03-01

Although seizures (other than myoclonus) are frequently reported in Creutzfeldt-Jakob disease (CJD), their frequency, clinical manifestations, and effect on the disease course is unknown. To characterize the frequency of seizures in E200K familial and sporadic CJD, to describe its semiology, EEG and MRI findings. In this retrospective study, we reviewed all patients with CJD who were seen in the Sheba Medical Center between the years 2003-2012 and underwent clinical evaluation, genetic testing, EEG and MRI studies. The diagnosis of seizures was carried out based on documentation of episodes consistent with seizures or episode of unresponsiveness correlated with ictal activity in EEG. Sixty-four probable patients with CJD were included in the study, 57 (89%) with E200K familial (fCJD) and 7 (11%) with sporadic (sCJD). Seizures occurred in 8 patients: 3 of 7 (43%) in patients with sCJD compared to 5/57 (9%) in patients with E200K fCJD (P = 0.04, chi-square test). Two of E200K fCJD patients with seizures had other non-prion etiologies for seizures (brain metastasis, known history of temporal lobe epilepsy which started 44 years before the diagnosis of CJD). Seizures occurred late in the course of the disease with an average of 12 days between the onset of seizures and death. Seizures in E200K fCJD were infrequent and occurred late in the disease course. This difference suggests that E200K fCJD represents a separate subtype of the disease with distinct clinical characteristics. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Association of apolipoprotein E allele {epsilon}4 with late-onset sporadic Alzheimer`s disease

Energy Technology Data Exchange (ETDEWEB)

Lucotte, G.; David, F.; Berriche, S. [Regional Center of Neurogenetics, Reims (France)] [and others

1994-09-15

Apolipoprotein E, type {epsilon}4 allele (ApoE {epsilon}4), is associated with late-onset sporadic Alzheimer`s disease (AD) in French patients. The association is highly significant (0.45 AD versus 0.12 controls for {epsilon}4 allele frequencies). These data support the involvement of ApoE {epsilon}4 allele as a very important risk factor for the clinical expression of AD. 22 refs., 1 fig., 3 tabs.

Midlatitude ionospheric F2-layer response to eruptive solar events-caused geomagnetic disturbances over Hungary during the maximum of the solar cycle 24: A case study

Science.gov (United States)

Berényi, K. A.; Barta, V.; Kis, Á.

2018-03-01

In our study we analyze and compare the response and behavior of the ionospheric F2 and of the sporadic E-layer during three strong (i.e., Dst art digital ionosonde of the Széchenyi István Geophysical Observatory located at midlatitude, Nagycenk, Hungary (IAGA code: NCK, geomagnetic latitude: 46.17° geomagnetic longitude: 98.85°). The local time of the sudden commencement (SC) was used to characterize the type of the ionospheric storm (after Mendillo and Narvaez, 2010). This way two regular positive phase (RPP) ionospheric storms and one no-positive phase (NPP) storm have been analyzed. In all three cases a significant increase in electron density of the foF2 layer can be observed at dawn/early morning (around 6:00 UT, 07:00 LT). Also we can observe the fade-out of the ionospheric layers at night during the geomagnetically disturbed time periods. Our results suggest that the fade-out effect is not connected to the occurrence of the sporadic E-layers.

Artificial E-region field-aligned plasma irregularities generated at pump frequencies near the second electron gyroharmonic

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D. L. Hysell

2009-07-01

Full Text Available E region ionospheric modification experiments have been performed at HAARP using pump frequencies about 50 kHz above and below the second electron gyroharmonic frequency. Artificial E region field-aligned plasma density irregularities (FAIs were created and observed using the imaging coherent scatter radar near Homer, Alaska. Echoes from FAIs generated with pump frequencies above and below 2Ωe did not appear to differ significantly in experiments conducted on summer afternoons in 2008, and the resonance instability seemed to be at work in either case. We argue that upper hybrid wave trapping and resonance instability at pump frequencies below the second electron gyroharmonic frequency are permitted theoretically when the effects of finite parallel wavenumbers are considered. Echoes from a sporadic E layer were observed to be somewhat weaker when the pump frequency was 50 kHz below the second electron gyroharmonic frequency. This may indicate that finite parallel wavenumbers are inconsistent with wave trapping in thin sporadic E ionization layers.

Airborne observations of newly formed boundary layer aerosol particles under cloudy conditions

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B. Altstädter

2018-06-01

Full Text Available This study describes the appearance of ultrafine boundary layer aerosol particles under classical non-favourable conditions at the research site of TROPOS (Leibniz Institute for Tropospheric Research. Airborne measurements of meteorological and aerosol properties of the atmospheric boundary layer (ABL were repeatedly performed with the unmanned aerial system ALADINA (Application of Light-weight Aircraft for Detecting IN-situ Aerosol during three seasons between October 2013 and July 2015. More than 100 measurement flights were conducted on 23 different days with a total flight duration of 53 h. In 26 % of the cases, maxima of ultrafine particles were observed close to the inversion layer at altitudes between 400 and 600 m and the particles were rapidly mixed vertically and mainly transported downwards during short time intervals of cloud gaps. This study focuses on two measurement days affected by low-level stratocumulus clouds, but different wind directions (NE, SW and minimal concentrations (< 4.6 µg m−3 of SO2, as a common indicator for precursor gases at ground. Taken from vertical profiles, the onset of clouds led to a non-linearity of humidity that resulted in an increased turbulence at the local-scale and caused fast nucleation e.g., but in relation to rapid dilution of surrounding air, seen in sporadic clusters of ground data, so that ultrafine particles disappeared in the verticality. The typical banana shape of new particle formation (NPF and growth was not seen at ground and thus these days might not have been classified as NPF event days by pure surface studies.

Artificial E-region field-aligned plasma irregularities generated at pump frequencies near the second electron gyroharmonic

Directory of Open Access Journals (Sweden)

D. L. Hysell

2009-07-01

Full Text Available E region ionospheric modification experiments have been performed at HAARP using pump frequencies about 50 kHz above and below the second electron gyroharmonic frequency. Artificial E region field-aligned plasma density irregularities (FAIs were created and observed using the imaging coherent scatter radar near Homer, Alaska. Echoes from FAIs generated with pump frequencies above and below 2Ω_e did not appear to differ significantly in experiments conducted on summer afternoons in 2008, and the resonance instability seemed to be at work in either case. We argue that upper hybrid wave trapping and resonance instability at pump frequencies below the second electron gyroharmonic frequency are permitted theoretically when the effects of finite parallel wavenumbers are considered. Echoes from a sporadic E layer were observed to be somewhat weaker when the pump frequency was 50 kHz below the second electron gyroharmonic frequency. This may indicate that finite parallel wavenumbers are inconsistent with wave trapping in thin sporadic E ionization layers.

Effects of mid-latitude ionosphere observed from ground-based ionosonde data obtained at Alma-Ata station during strong geomagnetic storms

International Nuclear Information System (INIS)

Gordienko, G.I.; Vodynnikov, V.V.; Yakovets, A.E.

2006-01-01

The ionospheric effects of fourteen great geomagnetic storms occurred in the 1986-2005 time period observed over Alma-Ata (43.25 N , 76.92 E ) were studied experimentally using ground-based ionosonde. The observations showed a number of unusual (for the Alma-Ata location) ionospheric phenomena during the active phase of geomagnetic storms, along with a negative phase in the ionospheric F2-layer disturbance an anomalous formation of the E, E2, and F1 layers at nighttime, and the appearance of aurora-type sporadic E layers were found. Processes of interaction of energetic neutrals with the upper atmosphere modeled by Bauske et al. (1997) for magnetically distributed condition seem to explain the phenomena of ionization of F1 and E region at night. (author)

Study of midlatitude ionospheric irregularities and E- and F-region coupling based on rocket and radar observations from Japan

Science.gov (United States)

Yamamoto, M.

2015-12-01

We have been studying ionspheric irregularities in mid-latitude region by using radars, sounding rockets, etc. The mid-latitude ionosphere was considered much stable than those in the equatorial or polar region in the past, but our studies for years have revealed that there are much active variabilities. We found variety of wave-like structures that are specific in the mid-latitudes. One of the phenomena is quasi-periodic echoes (QP echoes) first observed by the MU radar that reflects horizontal plasma-density structures associated to sporadic-E layers. Another phenomenon is medium-scale traveling ionospheric disturbance (MSTID) in the F-region. In the generation mechanism we think that Ionospheric E- and F-region coupling process is important. In this presentation, we will discuss nature of mid-latitude ionosphere based on our observations; the MU radar, sounding rocket campaigns of SEEK-1/2, and recent MSTID rocket experiment from JAXA Uchinoura Space Center in July 2013.

Electrondynamics of the auroral E region

International Nuclear Information System (INIS)

Kirkwood, Sheila.

1990-10-01

This study concentrates on the signatures of the interaction process seen closest to the Earth - i.e. in the region where the visible aurora is produced. A number of experimental investigations are presented which look at details of the dynamics and electrodynamics of auroral ionisation features in the ionospheric E region. The studies are based primarily on measurement with the EISCAT UHF incoherent scatter radar which is located in northern Scandinavia. Initial calibration of the radar, and some measurements of the properties of the background atmosphere are described first. These are a necessary basis for the detailed studies which follow, which are of two aspects of auroral electrodynamics - substorms and aurorally-associated sporadic E layers. For the substorm studies, all-sky camera observations of the aurora over Scandinavia and ultra-violet images from the Viking satellite are used to identify the auroral forms corresponding to EISCAT measurements. This allows the determination of representative conductivities for the different auroral features which are characteristic of the substorm development. These conductivities are an important input for modelling studies of the electric current systems and have not previously been measured directly. Particle measurements from the Viking satellite are used together with EISCAT to determine the origin of the particles precipitated into the ionosphere during the energy-storage phase prior to the substorm. The sporadic E layers studies address the question of how ion layers and associated neutral sodium layers can be produced by some process which is common in association with auroral activity. Two candidates are considered - gravity waves and electric fields. (author)

Pontine hyperperfusion in sporadic hyperekplexia.

Science.gov (United States)

Vetrugno, Roberto; Mascalchi, Mario; Vella, Alessandra; Della Nave, Riccardo; Guerrini, Laura; Vattimo, Angelo; del Giudice, Emanuele Miraglia; Plazzi, Giuseppe; D'Angelo, Roberto; Greco, Giovanni; Montagna, Pasquale

2007-09-01

To explore with neuroimaging techniques the anatomical and functional correlates of sporadic hyperekplexia. Two elderly women with sporadic hyperekplexia underwent neurophysiological assessment, MRI of the brain and proton magnetic resonance spectroscopy (1H-MRS) of the brainstem and frontal lobes. Regional cerebral blood flow was investigated with single photon emission tomography (SPECT) during evoked startles and at rest. Both patients showed excessively large and non-habituating startle responses. In both patients, MRI showed impingement of the brainstem by the vertebrobasilar artery, lack of frontal or brainstem abnormalities on 1H-MRS and hyperperfusion in the dorsal pons and cingulate cortex, and superior frontal gyrus at SPECT during evoked startles. In our patients with hyperekplexia, the vertebrobasilar arteries were found to impinge on the brainstem. Neurophysiological findings and neurofunctional imaging of evoked startles indicated a pontine origin of the movement disorder modulated by activation in cortical, especially frontal, areas. The neurofunctional correlates of evoked startles in human sporadic hyperekplexia are similar to those observed for the startle circuit in animals.

An Experiment to Study Sporadic Atom Layers in the Earth's Mesosphere and Lower Thermosphere (SAL)

Science.gov (United States)

Kelley, Michael C.

1999-01-01

The Sudden Atom Layer (SAL) Rocket was successfully launched in February 1998. All instruments worked well except those supplied by NASA Goddard Space Flight Center. (A dummy weight was launched for the neutral mass spectrometer and the ion version died shortly after lift-off.) A paper has already been published in GRL concerning the dust layer detected by an on board instrument and compared to ground-based observations made at the Arecibo Observatory by Cornell graduate student S. Collins (lidar) and Q. Zhou (radar). Collins presented a comparison of the sodium lidar data and onboard observations with a theoretical model by Plane and Cox at the Fan AGU Meeting. In addition Gelinas and Kelley presented a review paper dealing with the entire SAL instrument complement at the same meeting. An unexpected new explanation for the outer scale of E region plasma irregularities has come out of the data set. We anticipate at least a total of four papers will be published within a year of launch.

Germline Mutations in Cancer Predisposition Genes are Frequent in Sporadic Sarcomas

OpenAIRE

Chan, Sock Hoai; Lim, Weng Khong; Ishak, Nur Diana Binte; Li, Shao-Tzu; Goh, Wei Lin; Tan, Gek San; Lim, Kiat Hon; Teo, Melissa; Young, Cedric Ng Chuan; Malik, Simeen; Tan, Mann Hong; Teh, Jonathan Yi Hui; Chin, Francis Kuok Choon; Kesavan, Sittampalam; Selvarajan, Sathiyamoorthy

2017-01-01

Associations of sarcoma with inherited cancer syndromes implicate genetic predisposition in sarcoma development. However, due to the apparently sporadic nature of sarcomas, little attention has been paid to the role genetic susceptibility in sporadic sarcoma. To address this, we performed targeted-genomic sequencing to investigate the prevalence of germline mutations in known cancer-associated genes within an Asian cohort of sporadic sarcoma patients younger than 50 years old. We observed 13....

Aspect sensitive E- and F-region SPEAR-enhanced incoherent backscatter observed by the EISCAT Svalbard radar

Directory of Open Access Journals (Sweden)

R. S. Dhillon

2009-01-01

Full Text Available Previous studies of the aspect sensitivity of heater-enhanced incoherent radar backscatter in the high-latitude ionosphere have demonstrated the directional dependence of incoherent scatter signatures corresponding to artificially excited electrostatic waves, together with consistent field-aligned signatures that may be related to the presence of artificial field-aligned irregularities. These earlier high-latitude results have provided motivation for repeating the investigation in the different geophysical conditions that obtain in the polar cap ionosphere. The Space Plasma Exploration by Active Radar (SPEAR facility is located within the polar cap and has provided observations of RF-enhanced ion and plasma line spectra recorded by the EISCAT Svalbard UHF incoherent scatter radar system (ESR, which is collocated with SPEAR. In this paper, we present observations of aspect sensitive E- and F-region SPEAR-induced ion and plasma line enhancements that indicate excitation of both the purely growing mode and the parametric decay instability, together with sporadic E-layer results that may indicate the presence of cavitons. We note consistent enhancements from field-aligned, vertical and also from 5° south of field-aligned. We attribute the prevalence of vertical scatter to the importance of the Spitze region, and of that from field-aligned to possible wave/irregularity coupling.

New global electron density observations from GPS-RO in the D- and E-Region ionosphere

Science.gov (United States)

Wu, Dong L.

2018-06-01

A novel retrieval technique is developed for electron density (Ne) in the D- and E-region (80-120 km) using the high-quality 50-Hz GPS radio occultation (GPS-RO) phase measurements. The new algorithm assumes a slow, linear variation in the F-region background when the GPS-RO passes through the D- and E-region, and extracts the Ne profiles at 80-130 km from the phase advance signal caused by Ne. Unlike the conventional Abel function, the new approach produces a sharp Ne weighting function in the lower ionosphere, and the Ne retrievals are in good agreement with the IRI (International Reference Ionosphere) model in terms of monthly maps, zonal means and diurnal variations. The daytime GPS-RO Ne profiles can be well characterized by the α-Chapman function of three parameters (NmE, hmE and H), showing that the bottom of E-region is deepening and sharpening towards the summer pole. At high latitudes the monthly GPS-RO Ne maps at 80-120 km reveal clear enhancement in the auroral zones, more prominent at night, as a result of energetic electron precipitation (EEP) from the outer radiation belt. The D-/E-region auroral Ne is strongly correlated with Kp on a daily basis. The new Ne data allow further comprehensive analyses of the sporadic E (Es) phenomena in connection with the background Ne in the E-region. The layered (2-10 km) and fluctuated (Layer than Ne_Pert, are extracted with respect to the background Ne_Region on a profile-by-profile basis. The Ne_Layer component has a strong but highly-refined peak at ∼105 km, with an amplitude smaller than Ne_Region approximately by an order of magnitude. The Ne_Pert component, which was studied extensively in the past, is ∼2 orders of magnitude weaker than Ne_Layer. Both Ne_Layer and Ne_Pert are subject to significant diurnal and semidiurnal variations, showing downward progression with local time in amplitude. The 11-year solar cycle dominates the Ne interannual variations, showing larger Ne_Region and Ne_Layer but smaller

The enhancement of neutral metal Na layer above thunderstorms

Science.gov (United States)

Yu, Bingkun; Xue, Xianghui; Lu, Gaopeng; Kuo, Chengling; Dou, Xiankang; Gao, Qi; Qie, Xiushu; Wu, Jianfei; Tang, Yihuan

2017-04-01

Na (sodium) exists as layers of atoms in the mesosphere/lower thermosphere (MLT) at altitudes between 80 and 105 km. It has lower ionization potential of 5.139 eV than atmospheric species, such as O2 (12.06 eV). Tropospheric thunderstorms affect the lower ionosphere and the ionospheric sporadic E (Es) at 100 km can also be influenced by lightning. The mechanism is expected to be associated with transient luminous events (TLE) as red sprites and gigantic jets at upper atmosphere. However, measurements of ionospheric electric fields of 20mV·m-1 above thunderstorms are less than estimated value (>48 0mV·m-1) to excite ionization in the lower ionosphere. We found an enhancement of Na layer above thunderstorms. The increase of Na density in the statistical result can be as much as 500 cm-3 and it will have an impact on ionospheric chemistry and modify the conductivity properties of the MLT region. The ionospheric observations made with two digisondes near the Na lidar, the thunderstorm model, ionosphere model, and Na chemistry model are all used to discuss the possible mechanisms responsible for the enhancement of Na layer after thunderstorms.

Genetics Home Reference: sporadic hemiplegic migraine

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... Home Health Conditions Sporadic hemiplegic migraine Sporadic hemiplegic migraine Printable PDF Open All Close All Enable Javascript ... view the expand/collapse boxes. Description Sporadic hemiplegic migraine is a rare form of migraine headache. Migraines ...

Polimorfismo da apolipoproteína e nos familiares em primeiro grau de pacientes com doença de Alzheimer familial ou esporádica Apolipoprotein e polymorphism in first-degree relatives of patients with familial or sporadic Alzheimer's disease

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João de Castilho Cação

2007-06-01

Full Text Available INTRODUÇÃO: A apolipoproteína E (apo E é reconhecida como fator de risco para doença de Alzheimer (DA. OBJETIVO: Analisar o polimorfismo da apo E nos familiares em primeiro grau de pacientes com DA familial ou esporádica do tipo tardio, comparando a famílias sem DA. MÉTODO: Foram estudados 40 pacientes com DA familial ou esporádica do tipo tardio, sendo os grupos classificados como provável, segundo critérios da NINCS-ADRDA. RESULTADO: O alelo épsilon3 foi o mais freqüente em todos os grupos. Observou-se freqüência mais elevada de épsilon4 comparando os familiares dos probandos aos do grupo controle (pINTRODUCTION: Apolipoproteín E (apo E has been recognized as a risk factor for Alzheimer disease (AD. OBJECTIVE: To analyze apo E polymorphism in first-degree relatives of patients with familial or sporadic late-onset AD comparing with families without AD. METHOD: Forty patients with familial or sporadic late-onset of AD, being both groups classified as probable, according of NINCS-ADRDA’s criteria. RESULTS: Allele epsilon3 was the most frequent in all of these groups. Higher frequency of epsilon4 when comparing the relatives of the probands with the relatives of the control group (p<0,0001 was observed. Allele epsilon2 showed significant difference only between relatives of familial AD and relatives of control group (p=0,026. CONCLUSION: Apo E polymorphism has not differentiated familial from sporadic AD. The study of families allows to amplify the alelles epsilon2 and epsilon4 representativity, revealing, their value as protecting factor and of risk for AD, respectively.

Comparison Between Sporadic and Misdiagnosed Sporadic Creutzfeldt-Jakob Disease: A Report of Two Cases.

Science.gov (United States)

Zhao, Xiongfei; Yu, Yingxin; Zhao, Zhiru; Xu, Jiaping

2015-06-01

Definite accurate diagnosis for Creutzfeldt-Jakob disease (CJD) depends on neuropathologic examination of brain biopsy or autopsy. However, transmissible nature makes the invasive examination dangerous. This study was set to determine that the clinical features are for the diagnosis of CJD through a comparison study. We compared clinical features of two cases with initial diagnosis of sporadic CJD. One case was finally diagnosed as definite sporadic CJD. According to World Health Organization diagnosis criteria, the other one, which had been diagnosed as probable sporadic CJD, was confirmed as limbic encephalitis after long-term follow-up. Compared with the case of definite sporadic CJD, the misdiagnosed case did not present typical electroencephalogram (EEG) and diffusion-weighted in magnetic resonance images (DWI) of CJD. However, cerebrospinal fluid in the misdiagnosed patient showed 14-3-3 protein positivity. The patient conditions improved after treatment. Through this case comparison, we conclude that EEG and DWI are necessary for accurate diagnosis of sporadic CJD. Further, long-term follow-up is crucial to diagnosis and treatment of CJD.

Introduction to Sporadic Groups

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Luis J. Boya

2011-01-01

Full Text Available This is an introduction to finite simple groups, in particular sporadic groups, intended for physicists. After a short review of group theory, we enumerate the 1+1+16=18 families of finite simple groups, as an introduction to the sporadic groups. These are described next, in three levels of increasing complexity, plus the six isolated ''pariah'' groups. The (old five Mathieu groups make up the first, smallest order level. The seven groups related to the Leech lattice, including the three Conway groups, constitute the second level. The third and highest level contains the Monster group M, plus seven other related groups. Next a brief mention is made of the remaining six pariah groups, thus completing the 5+7+8+6=26 sporadic groups. The review ends up with a brief discussion of a few of physical applications of finite groups in physics, including a couple of recent examples which use sporadic groups.

Selenofuranoside Ameliorates Memory Loss in Alzheimer-Like Sporadic Dementia: AChE Activity, Oxidative Stress, and Inflammation Involvement

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Cristiano Chiapinotto Spiazzi

2015-01-01

Full Text Available Alzheimer’s disease (AD is becoming more common due to the increase in life expectancy. This study evaluated the effect of selenofuranoside (Se in an Alzheimer-like sporadic dementia animal model. Male mice were divided into 4 groups: control, Aβ, Se, and Aβ + Se. Single administration of Aβ peptide (fragments 25–35; 3 nmol/3 μL or distilled water was administered via intracerebroventricular (i.c.v. injection. Selenofuranoside (5 mg/kg or vehicle (canola oil was administered orally 30 min before Aβ and for 7 subsequent days. Memory was tested through the Morris water maze (MWM and step-down passive-avoidance (SDPA tests. Antioxidant defenses along with reactive species (RS were assessed. Inflammatory cytokines levels and AChE activity were measured. SOD activity was inhibited in the Aβ group whereas RS were increased. AChE activity, GSH, and IL-6 levels were increased in the Aβ group. These changes were reflected in impaired cognition and memory loss, observed in both behavioral tests. Se compound was able to protect against memory loss in mice in both behavioral tests. SOD and AChE activities as well as RS and IL-6 levels were also protected by Se administration. Therefore, Se is promising for further studies.

Multistation digisonde observations of equatorial spread F in South America

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B. W. Reinisch

2004-09-01

Full Text Available Directional ionogram and F-region drift observations were conducted at seven digisonde stations in South America during the COPEX campaign from October to December 2002. Five stations in Brazil, one in Argentina, and one in Peru, monitored the ionosphere across the continent to study the onset and development of F-region density depletions that cause equatorial spread F (ESF. New ionosonde techniques quantitatively describe the prereversal uplifting of the F layer at the magnetic equator and the eastward motion of the depletions over the stations. Three of the Brazilian stations were located along a field line with a 350-km apex over the equator to investigate the relation of the occurrence of ESF and the presence of sporadic E-layers at the two E-region intersections of the field line. No simple correlation was found.

Genomic Alterations Observed in Colitis-Associated Cancers Are Distinct From Those Found in Sporadic Colorectal Cancers and Vary by Type of Inflammatory Bowel Disease.

Science.gov (United States)

Yaeger, Rona; Shah, Manish A; Miller, Vincent A; Kelsen, Judith R; Wang, Kai; Heins, Zachary J; Ross, Jeffrey S; He, Yuting; Sanford, Eric; Yantiss, Rhonda K; Balasubramanian, Sohail; Stephens, Philip J; Schultz, Nikolaus; Oren, Moshe; Tang, Laura; Kelsen, David

2016-08-01

Patients with inflammatory bowel diseases, such as Crohn's disease (CD) and ulcerative colitis (UC), are at increased risk for small bowel or colorectal cancers (colitis-associated cancers [CACs]). We compared the spectrum of genomic alterations in CACs with those of sporadic colorectal cancers (CRCs) and investigated differences between CACs from patients with CD vs UC. We studied tumor tissues from patients with CACs treated at Memorial Sloan Kettering Cancer Center or Weill Cornell Medical College from 2003 through 2015. We performed hybrid capture-based next-generation sequencing analysis of >300 cancer-related genes to comprehensively characterize genomic alterations. We performed genomic analyses of 47 CACs (from 29 patients with UC and 18 with CD; 43 primary tumors and 4 metastases). Primary tumors developed in the ileum (n = 2), right colon (n = 18), left colon (n = 6), and rectosigmoid or rectum (n = 21). We found genomic alterations in TP53, IDH1, and MYC to be significantly more frequent, and mutations in APC to be significantly less frequent, than those reported in sporadic CRCs by The Cancer Genome Atlas or Foundation Medicine. We identified genomic alterations that might be targeted by a therapeutic agent in 17 of 47 (36%) CACs. These included the mutation encoding IDH1 R132; amplification of FGFR1, FGFR2, and ERBB2; and mutations encoding BRAF V600E and an EML4-ALK fusion protein. Alterations in IDH1 and APC were significantly more common in CACs from patients with CD than UC. In an analysis of CACs from 47 patients, we found significant differences in the spectrum of genomic alterations in CACs compared with sporadic CRCs. We observed a high frequency of IDH1 R132 mutations in patients with CD but not UC, as well as a high frequency of MYC amplification in CACs. Many genetic alterations observed in CACs could serve as therapeutic targets. Copyright © 2016 AGA Institute. Published by Elsevier Inc. All rights reserved.

Wavelength dependence of the linear growth rate of the Es layer instability

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R. B. Cosgrove

2007-06-01

Full Text Available It has recently been shown, by computation of the linear growth rate, that midlatitude sporadic-E (Es layers are subject to a large scale electrodynamic instability. This instability is a logical candidate to explain certain frontal structuring events, and polarization electric fields, which have been observed in Es layers by ionosondes, by coherent scatter radars, and by rockets. However, the original growth rate derivation assumed an infinitely thin Es layer, and therefore did not address the short wavelength cutoff. Also, the same derivation ignored the effects of F region loading, which is a significant wavelength dependent effect. Herein is given a generalized derivation that remedies both these short comings, and thereby allows a computation of the wavelength dependence of the linear growth rate, as well as computations of various threshold conditions. The wavelength dependence of the linear growth rate is compared with observed periodicities, and the role of the zeroth order meridional wind is explored. A three-dimensional paper model is used to explain the instability geometry, which has been defined formally in previous works.

Multi-Layer E-Textile Circuits

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Dunne, Lucy E.; Bibeau, Kaila; Mulligan, Lucie; Frith, Ashton; Simon, Cory

2012-01-01

Stitched e-textile circuits facilitate wearable, flexible, comfortable wearable technology. However, while stitched methods of e-textile circuits are common, multi-layer circuit creation remains a challenge. Here, we present methods of stitched multi-layer circuit creation using accessible tools and techniques.

Sporadic Fatal Insomnia in an Adolescent

Science.gov (United States)

Blase, Jennifer L.; Cracco, Laura; Schonberger, Lawrence B.; Maddox, Ryan A.; Cohen, Yvonne; Cali, Ignazio

2014-01-01

The occurrence of sporadic prion disease among adolescents is extremely rare. A prion disease was confirmed in an adolescent with disease onset at 13 years of age. Genetic, neuropathologic, and biochemical analyses of the patient’s autopsy brain tissue were consistent with sporadic fatal insomnia, a type of sporadic prion disease. There was no evidence of an environmental source of infection, and this patient represents the youngest documented case of sporadic prion disease. Although rare, a prion disease diagnosis should not be discounted in adolescents exhibiting neurologic signs. Brain tissue testing is necessary for disease confirmation and is particularly beneficial in cases with an unusual clinical presentation. PMID:24488737

Adult onset sporadic ataxias: a diagnostic challenge

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Orlando Graziani Povoas Barsottini

2014-03-01

Full Text Available Patients with adult onset non-familial progressive ataxia are classified in sporadic ataxia group. There are several disease categories that may manifest with sporadic ataxia: toxic causes, immune-mediated ataxias, vitamin deficiency, infectious diseases, degenerative disorders and even genetic conditions. Considering heterogeneity in the clinical spectrum of sporadic ataxias, the correct diagnosis remains a clinical challenge. In this review, the different disease categories that lead to sporadic ataxia with adult onset are discussed with special emphasis on their clinical and neuroimaging features, and diagnostic criteria.

Sporadic wind wave horse-shoe patterns

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S. Yu. Annenkov

1999-01-01

Full Text Available The work considers three-dimensional crescent-shaped patterns often seen on water surface in natural basins and observed in wave tank experiments. The most common of these 'horse-shoe-like' patterns appear to be sporadic, i.e., emerging and disappearing spontaneously even under steady wind conditions. The paper suggests a qualitative model of these structures aimed at explaining their sporadic nature, physical mechanisms of their selection and their specific asymmetric form. First, the phenomenon of sporadic horse-shoe patterns is studied numerically using the novel algorithm of water waves simulation recently developed by the authors (Annenkov and Shrira, 1999. The simulations show that a steep gravity wave embedded into widespectrum primordial noise and subjected to small nonconservative effects typically follows the simple evolution scenario: most of the time the system can be considered as consisting of a basic wave and a single pair of oblique satellites, although the choice of this pair tends to be different at different instants. Despite the effective low-dimensionality of the multimodal system dynamics at relatively sho ' rt time spans, the role of small satellites is important: in particular, they enlarge the maxima of the developed satellites. The presence of Benjamin-Feir satellites appears to be of no qualitative importance at the timescales under consideration. The selection mechanism has been linked to the quartic resonant interactions among the oblique satellites lying in the domain of five-wave (McLean's class II instability of the basic wave: the satellites tend to push each other out of the resonance zone due to the frequency shifts caused by the quartic interactions. Since the instability domain is narrow (of order of cube of the basic wave steepness, eventually in a generic situation only a single pair survives and attains considerable amplitude. The specific front asymmetry is found to result from the interplay of quartic

Characteristics of the magnetospheric boundary layer and magnetopause layer as observed by Imp 6

International Nuclear Information System (INIS)

Eastman, T.E.; Hones, E.W. Jr.

1979-01-01

Imp 6 observations of the low-latitude magnetospheric boundary layer indicate that the plasma within it is supplied primarily by direct entry of magnetosheath plasma across the magnetopause layer. We define the magnetopause layer as the current layer (separating the magnetosheath from the boundary layer) through which the magnetic field shifts in direction. High temporal resolution (3-s average) data reveal that in a majority of Imp 6 magnetopause crossing, no distinct changes in electron density or energry spectra are observed at the magne opause layer. In all Imp 6 crossings, some magnetosheathlike plasma is observed earthward of the magnetopause layer, implying the existence of a boundary layer. Boundary layer electron energy spectra are often virtually indistinguishable from the adjacent magnetosheath spectra. Low-latitude boundary layer bulk plasma flow as observed by Imp 6 almost always has an antisunward component and often has a significant cross-field component. The boundary layer thickness is highly variable and is generally much larger than the magnetopause layer thickness. Energetic electron pitch angle distributions indicate that the low-latitude boundary layers is normally on closed field lines. We conclude that diffusive as well as nondiffusive processes probably contribute to the entry of magnetosheath plasma into the boundary layer

Effects of sporadic E-layer characteristics on spread-F generation in the nighttime midlatitude ionosphere: A climatological study

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Lee, C. C.; Chen, W. S.

2018-04-01

The aim of this study is to examine the effects of Es-layer characteristics on spread-F generation in the nighttime midlatitude ionosphere. The Es-layer parameters and spread-F appearance of the 23rd solar cycle (1996-2008) are recorded by the Kokubunji ionosonde. The Es-layer parameters are foEs (critical frequency of Es-layer), fbEs (blanketing frequency of Es-layer), and Δf (≡foEs-fbEs). In order to completely explore the effects, the pre-midnight and post-midnight data are classified by seasons, solar activities, and geomagnetic conditions. Results show that the spread-F occurs more frequently in post-midnight and in summer. And, the occurrence probabilities of spread-F are greater, when the solar activity is lower. For the occurrence probabilities of spread-F versus foEs and Δf under geomagnetic quiet-conditions, the trend is increasing, when the associated probabilities are significant. These indicate that the spread-F occurrence increases with increasing foEs and/or Δf. Further, the increasing trends demonstrate that polarization electric fields generated in Es-layer would be helpful to generate spread-F, through the electrodynamical coupling of Es-layer and F-region. Moreover, this electrodynamical coupling is efficient not only under quiet-conditions but under disturbed-conditions, since the significant increasing trend can also be found under disturbed-conditions. Regarding the occurrence probabilities of spread-F versus fbEs, the evident trends are not in the majority. This implies that fbEs might not be a major factor for the spread-F formation.

Coherent structures in the Es layer and neutral middle atmosphere

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Mošna, Zbyšek; Knížová, Petra Koucká; Potužníková, Kateřina

2015-12-01

The present paper shows results from the summer campaign performed during geomagnetically quiet period from June 1 to August 31, 2009. Within time-series of stratospheric and mesospheric temperatures at pressure levels 10-0.1 hPa, mesospheric winds measured in Collm, Germany, and the sporadic E-layer parameters foEs and hEs measured at the Pruhonice station we detected specific coherent wave-bursts in planetary wave domain. Permanent wave-like activity is observed in all analyzed data sets. However, the number of wave-like structures persistent in large range of height from the stratosphere to lower ionosphere is limited. The only coherent modes that are detected on consequent levels of the atmosphere are those corresponding to eigenmodes of planetary waves.

The atmospheric boundary layer in the CSIRO global climate model: simulations versus observations

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Garratt, J. R.; Rotstayn, L. D.; Krummel, P. B.

2002-07-01

A 5-year simulation of the atmospheric boundary layer in the CSIRO global climate model (GCM) is compared with detailed boundary-layer observations at six locations, two over the ocean and four over land. Field observations, in the form of surface fluxes and vertical profiles of wind, temperature and humidity, are generally available for each hour over periods of one month or more in a single year. GCM simulations are for specific months corresponding to the field observations, for each of five years. At three of the four land sites (two in Australia, one in south-eastern France), modelled rainfall was close to the observed climatological values, but was significantly in deficit at the fourth (Kansas, USA). Observed rainfall during the field expeditions was close to climatology at all four sites. At the Kansas site, modelled screen temperatures (Tsc), diurnal temperature amplitude and sensible heat flux (H) were significantly higher than observed, with modelled evaporation (E) much lower. At the other three land sites, there is excellent correspondence between the diurnal amplitude and phase and absolute values of each variable (Tsc, H, E). Mean monthly vertical profiles for specific times of the day show strong similarities: over land and ocean in vertical shape and absolute values of variables, and in the mixed-layer and nocturnal-inversion depths (over land) and the height of the elevated inversion or height of the cloud layer (over the sea). Of special interest is the presence climatologically of early morning humidity inversions related to dewfall and of nocturnal low-level jets; such features are found in the GCM simulations. The observed day-to-day variability in vertical structure is captured well in the model for most sites, including, over a whole month, the temperature range at all levels in the boundary layer, and the mix of shallow and deep mixed layers. Weaknesses or unrealistic structure include the following, (a) unrealistic model mixed-layer

Screening of hypoxia-inducible genes in sporadic ALS.

LENUS (Irish Health Repository)

Cronin, Simon

2008-10-01

Genetic variations in two hypoxia-inducible angiogenic genes, VEGF and ANG, have been linked with sporadic amyotrophic lateral sclerosis (SALS). Common variations in these genes may reduce the levels or functioning of their products. VEGF and ANG belong to a larger group of angiogenic genes that are up-regulated under hypoxic conditions. We hypothesized that common genetic variation across other members of this group may also predispose to sporadic ALS. To screen other hypoxia-inducible angiogenic genes for association with SALS, we selected 112 tagging single nucleotide polymorphisms (tgSNPs) that captured the common genetic variation across 16 VEGF-like and eight ANG-like hypoxia-inducible genes. Screening for association was performed in 270 Irish individuals with typical SALS and 272 ethnically matched unrelated controls. SNPs showing association in the Irish phase were genotyped in a replication sample of 281 Swedish sporadic ALS patients and 286 Swedish controls. Seven markers showed association in the Irish. The one modest replication signal observed in the Swedish replication sample, at rs3801158 in the gene inhibin beta A, was for the opposite allele vs. the Irish cohort. We failed to detect association of common variation across 24 candidate hypoxia-inducible angiogenic genes with SALS.

Radar observations of field-aligned plasma irregularities in the SEEK-2 campaign

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S. Saito

2005-10-01

Full Text Available During the Sporadic E Experiment over Kyushu 2 (SEEK-2 campaign, field-aligned irregularities (FAIs associated with midlatitude sporadic-E (E_s layers were observed with two backscatter radars, the Lower Thermosphere Profiler Radar (LTPR and the Frequency Agile Radar (FAR, which were located 40 km apart in Tanegashima, Japan. We conducted observations of FAI echoes from 31 July to 24 August 2002, and the radar data were used to determine launch timing of two sounding rockets on 3 August 2002. Our comparison of echoes obtained by the LTPR and the FAR revealed that echoes often appeared at the FAR about 10min earlier than they did at the LTPR and were well correlated. This indicates that echoing regions drift with a southward velocity component that maintains the spatial shape. Interferometry observations that were conducted with the LTPR from 3 to 8 August 2002, revealed that the quasi-periodic (QP striations in the Range-Time-Intensity (RTI plots were due to the apparent motion of echoing regions across the radar beam including both main and side lobes. In most cases, the echo moved to the east-southeast at an almost constant altitude of 100–110 km, which was along the locus of perpendicularity of the radar line-of-sight to the geomagnetic field line. We found that the QP pattern on the RTI plot reflects the horizontal structure and motion of the (E_s layer, and that echoing regions seemed to be in one-dimensionally elongated shapes or in chains of patches. Neutral wind velocities from 75 to 105 km altitude were simultaneously derived with meteor echoes from the LTPR. This is the first time-continuous simultaneous observation FAIs and neutral wind with interferometry measurements. Assuming that the echoing regions were drifting with an ambient neutral wind, we found that the echoing region was aligned east-northeast-west-southwest in eight out of ten QP echo events during the SEEK-2 campaign. A range rate was

Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease

Science.gov (United States)

Kallenberg, K.; Summers, D. M.; Romero, C.; Taratuto, A.; Heinemann, U.; Breithaupt, M.; Varges, D.; Meissner, B.; Ladogana, A.; Schuur, M.; Haik, S.; Collins, S. J.; Jansen, Gerard H.; Stokin, G. B.; Pimentel, J.; Hewer, E.; Collie, D.; Smith, P.; Roberts, H.; Brandel, J. P.; van Duijn, C.; Pocchiari, M.; Begue, C.; Cras, P.; Will, R. G.; Sanchez-Juan, P.

2009-01-01

Several molecular subtypes of sporadic Creutzfeldt–Jakob disease have been identified and electroencephalogram and cerebrospinal fluid biomarkers have been reported to support clinical diagnosis but with variable utility according to subtype. In recent years, a series of publications have demonstrated a potentially important role for magnetic resonance imaging in the pre-mortem diagnosis of sporadic Creutzfeldt–Jakob disease. Magnetic resonance imaging signal alterations correlate with distinct sporadic Creutzfeldt–Jakob disease molecular subtypes and thus might contribute to the earlier identification of the whole spectrum of sporadic Creutzfeldt–Jakob disease cases. This multi-centre international study aimed to provide a rationale for the amendment of the clinical diagnostic criteria for sporadic Creutzfeldt–Jakob disease. Patients with sporadic Creutzfeldt–Jakob disease and fluid attenuated inversion recovery or diffusion-weight imaging were recruited from 12 countries. Patients referred as ‘suspected sporadic Creutzfeldt–Jakob disease’ but with an alternative diagnosis after thorough follow up, were analysed as controls. All magnetic resonance imaging scans were assessed for signal changes according to a standard protocol encompassing seven cortical regions, basal ganglia, thalamus and cerebellum. Magnetic resonance imaging scans were evaluated in 436 sporadic Creutzfeldt–Jakob disease patients and 141 controls. The pattern of high signal intensity with the best sensitivity and specificity in the differential diagnosis of sporadic Creutzfeldt–Jakob disease was identified. The optimum diagnostic accuracy in the differential diagnosis of rapid progressive dementia was obtained when either at least two cortical regions (temporal, parietal or occipital) or both caudate nucleus and putamen displayed a high signal in fluid attenuated inversion recovery or diffusion-weight imaging magnetic resonance imaging. Based on our analyses, magnetic

Fatores de risco na gagueira desenvolvimental familial e isolada Risk factors in the familial and sporadic developmental stuttering

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Cristiane Moço Canhetti de Oliveira

2011-04-01

Full Text Available OBJETIVO: investigar e comparar os achados dos fatores de risco para a cronicidade da gagueira em crianças com gagueira desenvolvimental familial e isolada. MÉTODOS: participaram 60 crianças de ambos os gêneros, divididas em dois grupos: GI - 30 crianças com gagueira desenvolvimental familial; GII - 30 crianças com gagueira desenvolvimental isolada. A coleta de dados foi realizada por meio do Protocolo de Risco para a Gagueira do Desenvolvimento - PRGD (Andrade, 2006, que considera os seguintes fatores de risco: idade, gênero, tipo de surgimento e tempo de duração das disfluências, tipologia das disfluências, fatores comunicativos e qualitativos associados, histórico mórbido pré, peri e pós natal, fatores estressantes que ocorreram próximo ao surgimento do distúrbio, histórico familial, reação pessoal, familiar e social e atitudes familiares. RESULTADOS: quando o grupo I (GI foi comparado com o grupo II (GII, a única diferença estatisticamente significante foi com relação aos fatores estressantes que ocorreram próximo ao surgimento do distúrbio. CONCLUSÃO: os resultados confirmam a natureza complexa da gagueira, bem como a necessidade de se investigar os vários fatores considerados como de risco para o distúrbio, com intuito de melhorar a compreensão de suas possíveis etiologias.PURPOSE: to investigate and compare the risk factors for stuttering between children with familial developmental stuttering and children with sporadic developmental stuttering. METHODS: 60 children of both genders with stuttering took part, divided in two groups: GI - 30 children with familial developmental stuttering; GII - 30 children with sporadic developmental stuttering. Data were gathered through the Protocol of Risk for the Developmental Stuttering - PRGD (Andrade, 2006, which considers the following factors: age; gender; manner of onset and time of duration for the disfluencies; typology of the disfluencies; associated communicative

Ground observations of magnetospheric boundary layer phenomena

International Nuclear Information System (INIS)

McHenry, M.A.; Clauer, C.R.; Friis-Christensen, E.; Newell, P.T.; Kelly, J.D.

1990-01-01

Several classes of traveling vortices in the dayside ionospheric convection have been detected and tracked using the Greenland magnetometer chain (Friis-Christensen et al., 1988, McHenry et al., 1989). One class observed during quiet times consists of a continuous series of vortices moving generally anti-sunward for several hours at a time. The vortices strength is seen to be approximately steady and neighboring vortices rotate in opposite directions. Sondrestrom radar observations show that the vortices are located at the ionospheric convection reversal boundary. Low altitude DMSP observations indicate the vortices are on field lines which map to the inner edge of the low latitude boundary layer. Because the vortices are conjugate to the boundary layer, repeat in a regular fashion and travel antisunward, the authors argue that this class of vortices is caused by the Kelvin-Helmholtz instability of the inner edge of the magnetospheric boundary layer

Can the TLR-4-Mediated Signaling Pathway Be “A Key Inflammatory Promoter for Sporadic TAA”?

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Giovanni Ruvolo

2014-01-01

Full Text Available Thoracic aorta shows with advancing age various changes and a progressive deterioration in structure and function. As a result, vascular remodeling (VR and medial degeneration (MD occur as pathological entities responsible principally for the sporadic TAA onset. Little is known about their genetic, molecular, and cellular mechanisms. Recent evidence is proposing the strong role of a chronic immune/inflammatory process in their evocation and progression. Thus, we evaluated the potential role of Toll like receptor- (TLR- 4-mediated signaling pathway and its polymorphisms in sporadic TAA. Genetic, immunohistochemical, and biochemical analyses were assessed. Interestingly, the rs4986790 TLR4 polymorphism confers a higher susceptibility for sporadic TAA (OR=14.4, P=0.0008 and it represents, together with rs1799752 ACE, rs3918242 MMP-9, and rs2285053 MMP-2 SNPs, an independent sporadic TAA risk factor. In consistency with these data, a significant association was observed between their combined risk genotype and sporadic TAA. Cases bearing this risk genotype showed higher systemic inflammatory mediator levels, significant inflammatory/immune infiltrate, a typical MD phenotype, lower telomere length, and positive correlations with histopatological abnormalities, hypertension, smoking, and ageing. Thus, TLR4 pathway should seem to have a key role in sporadic TAA. It might represent a potential useful tool for preventing and monitoring sporadic TAA and developing personalized treatments.

Association between rs6812193 polymorphism and sporadic Parkinson's disease susceptibility.

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Huo, Qiang; Li, Tao; Zhao, Peiqing; Wang, Lianqing

2015-08-01

Recently, the association of a single nucleotide polymorphism rs6812193 C/T with sporadic Parkinson's disease (PD) susceptibility has been widely evaluated, but the results remained inconsistent. This association should be clarified because of the importance of it on human health and quality of life. We performed a comprehensive meta-analysis to evaluate the association between the rs6812193 polymorphism and sporadic PD. PubMed was used to retrieve articles published up to June 2014 for all studies evaluating the rs6812193 polymorphism and PD in humans. Ethnicity-specific subgroup analysis was also performed based on ethnicity susceptibility. A total of 17 independent study samples (15 Caucasians and 2 Asians) including 17,956 cases and 52,751 controls were used in the presented study. The MAFT (minor allele T frequency) in PD patients of European descent is obviously higher than Asian cases (p susceptibility among overall samples (OR 0.882, 95 % CI 0.856-0.908) and Caucasian population (OR 0.881, 95 % CI 0.856-0.907), but not in Asian samples (OR 0.918, 95 % CI 0.721-1.168). No evidence of publication bias was observed. Throughout our analysis, the rs6812193 polymorphism is significantly associated with sporadic PD susceptibility in Caucasian samples, and ethnicity might be the key point of inconsistency in rs6812193 studies. Further studies are warranted to re-examine the observed associations, especially in different ethnicities.

Daytime descending intermediate layers observed over a sub-tropical Indian station Waltair during low-solar activity period

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K. Niranjan

2010-03-01

Full Text Available Study on daytime descending intermediate layer over subtropical Indian station Waltair (17.7° N, 83.3° E geographic, 6.4° N, 10° E geomagnetic, 20° N dip located in the equatorial anomaly transition region, using an IPS 42 Digital Ionosonde during the low solar activity year 2004 showed that the layers occur in the altitude range of 140–160 km with maximum occurrence during winter solstice. The layers observed during daytime occur with a double peak variation throughout the year with less occurrence probability and shorter duration presence during forenoon hours. The morning layer descent was associated with a density increase where as during afternoon hours a decrease in density was observed. The downward drift velocity was about 8 km/h during morning hours and between 7–11 km/h during afternoon hours, with a low descent rate of around 4.5 km/h during summer morning hours. The results indicate the presence of a 6 h tide at this location as observed from the characteristics of the descending layers, unlike at majority of locations where a significant semi diurnal trend is observed. The study brings out the complex nature of the tidal interaction at different locations.

Hypermethylation of the FANCC and FANCL Promoter Regions in Sporadic Acute Leukaemia

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C. J. Hess

2008-01-01

Full Text Available Objective: Inactivation of the FA-BRCA pathway results in chromosomal instability. Fanconi anaemia (FA patients have an inherited defect in this pathway and are strongly predisposed to the development of acute myeloid leukaemia (AML. Studies in sporadic cancers have shown promoter methylation of the FANCF gene in a significant proportion of various solid tumours. However, only a single leukaemic case with methylation of one of the FA-BRCA genes has been described to date, i.e. methylation of FANCF in cell line CHRF-288. We investigated the presence of aberrant methylation in 11 FA-BRCA genes in sporadic cases of leukaemia.

Oestrogen receptor beta isoform expression in sporadic colorectal cancer, familial adenomatous polyposis and progressive stages of colorectal cancer

DEFF Research Database (Denmark)

Stevanato Filho, Paulo Roberto; Aguiar Júnior, Samuel; Begnami, Maria Dirlei

2017-01-01

BACKGROUND: Among the sex hormones, oestrogen may play a role in colorectal cancer, particularly in conjunction with oestrogen receptor-β (ERβ). The expression of ERβ isoform variants and their correlations with familial adenomatous polyposis (FAP) syndrome and sporadic colorectal carcinomas...... was identified in sporadic polyps and in sporadic colorectal cancer as well as in polyps from FAP syndrome patients compared with normal tissues (p expression in polyps (p ..., no differences were observed when sporadic colorectal carcinomas were compared to normal mucosa tissues. These findings suggest an association of the ERβ isoform variants in individuals affected by germline mutations of the APC gene. Progressively decreased expression of ERβ was found in polyps at early stages...

Dual infection with hepatitis A and E viruses in outbreaks and in sporadic clinical cases: Cuba 1998-2003.

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Rodríguez Lay, Licel de los Angeles; Quintana, Ariel; Villalba, María Caridad Montalvo; Lemos, Gilda; Corredor, Marité Bello; Moreno, Aidonis Gutiérrez; Prieto, Pablo Aguiar; Guzmán, María G; Anderson, David

2008-05-01

Viral hepatitis ranks as the fifth cause of morbidity for infectious diseases in Cuba. Epidemics are observed frequently in the population, the hepatitis A virus being the main agent responsible for such epidemics. Previous reports also confirmed the circulation of the hepatitis E virus. From 1998 to 2003, 258 serum samples were collected by the Reference Laboratory on Viral Hepatitis during 33 outbreaks of acute viral hepatitis as well as from 39 sporadic clinical cases. Sera were tested for anti-HAV and anti-HEV IgM by EIA. Overall of the 33 outbreaks studied sera from 12 (36.4%) were positive for anti-HAV IgM only, from 7 (21.2%) were positive for anti-HEV IgM only, and from 14 (42.4%) were positive for antibodies to both viruses. Individually of the 258 sera collected, 137 (53.1%) were positives for anti-HAV IgM, 20 (7.8%) were positives for anti-HEV IgM, 33 (12.8%) were positives for both markers and 68 (26.4%) were negative to both. Of the clinical cases, 4 (10.3%) were positives for anti-HAV IgM, 13 (33.3%) were positives for anti-HEV IgM and 5 (12.8%) were positives for both markers. Seventeen (43.6%) sera were negatives for all viral hepatitis markers available (A-E). A high positivity for HEV was found in outbreaks tested with the kit produced by CIGB. In particular HEV seems to infect individuals of all ages. The results demonstrate the co-circulation of and co-infection with two enterically transmitted viruses; however a higher positivity was observed for anti-HAV than to anti-HEV (53.1% vs. 7.8%) in outbreaks.

Observational description of the atmospheric and oceanic boundary layers over the Atlantic Ocean

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Marcelo Dourado

2001-01-01

Full Text Available Time evolution of atmospheric and oceanic boundary layers are described for an upwelling region in the Atlantic Ocean located in Cabo Frio, Brazil (23°00'S, 42°08'W. The observations were obtained during a field campaign carried out by the "Instituto de Estudos do Mar Almirante Paulo Moreira", on board of the oceanographic ship Antares of the Brazilian Navy, between July 7 and 10 of 1992. The analysis shown here was based on 19 simultaneous vertical soundings of atmosphere and ocean, carried out consecutively every 4 hours. The period of observation was characterized by a passage of a cold front that penetrated in Cabo Frio on July 6. During the cold front passage the vertical extension of atmospheric (and oceanic mixed layer varied from 200 m (and 13 m to 1000 m (and 59 m. These changes occurred in the first day of observation and were followed by an increase of 1.2°C in the oceanic mixed layer temperature and by a decrease of 6 K and 6 g/kg in the virtual potential temperature and specific humidity of the atmospheric mixed layer. The short time scale variations in the ocean can be explained in terms of the substitution of cold upwelling water by warm downwelling water regime, as the surface winds shift from pre-frontal NE to post-frontal SSW during the cold front passage in Cabo Frio. The large vertical extent of the atmospheric mixed layer can be explained in terms of an intensification of the thermal mixing induced by the warming of the oceanic upper layers combined with the cooling of the lower atmospheric layers during the cold front passage. An intensification of the mechanical mixing, observed during the cold front passage, may also be contributing to the observed variations in the vertical extent of both layers.A evolução temporal das camadas limites atmosféricas e oceânicas são descritas para a região de ressurgência do Oceano Atlântico localizada em Cabo Frio. As observações foram obtidas durante a campanha de medidas

Ground-Based Observations and Modeling of the Visibility and Radar Reflectivity in a Radiation Fog Layer

NARCIS (Netherlands)

Boers, R.; Baltink, K.H.; Hemink, H.J.; Bosveld, F.C.; Moerman, M.

2013-01-01

The development of a radiation fog layer at the Cabauw Experimental Site for Atmospheric Research(51.97°N, 4.93°E) on 23 March 2011 was observed with ground-based in situ and remote sensing observationsto investigate the relationship between visibility and radar reflectivity. The fog layer thickness

Molecular Cytogenetic Characterization Identified the Murine B-Cell Lymphoma Cell Line A-20 as a Model for Sporadic Burkitt's Lymphoma.

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Guja, Karolina; Liehr, Thomas; Rincic, Martina; Kosyakova, Nadezda; Hussein Azawi, Shaymaa S

2017-11-01

Here, we report the first molecular cytogenetic characterization of the BALB/cAnN mouse derived B-cell non-Hodgkin lymphoma (B-cell NHL) cell lines A-20. Even though previously used as a model for testing of, for example, dexametason, up to present, no data in the genetic properties of A-20 were available. The present study closed this gap and provides evidence that A-20 is a model for B-cell NHL subgroup sporadic Burkitt's lymphoma. C-myc oncogene is involved in a translocation and copy number alterations as gain of murine 14q material could be observed. Interestingly, the cell line showed the karyotype 39,X,-X or -Y,t(2;15)(qE5;qD2),del(6)(qB3qC3),del(9)(qA3qA4),dup(14)(qE1qE4) in ~95% of the cells, being exceptionally stable for cell lines being established 38 years ago. Still, ~5% of the cells showed polyploidization followed by chromothripsis. It remains to be determined if this can be observed also in other cell lines, just has not been reported yet, and/or if it is a unique feature of A-20. Overall, finally here, the necessary genetic data to identify A-20 as a model for human sporadic Burkitt's lymphoma are provided.

Inheritable and sporadic non-autoimmune hyperthyroidism.

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Ferraz, Carolina; Paschke, Ralf

2017-03-01

Hyperthyroidism is a clinical state that results from high thyroid hormone levels which has multiple etiologies, manifestations, and potential therapies. Excluding the autoimmune Graves disease, autonomic adenomas account for the most import cause of non-autoimmune hyperthyroidism. Activating germline mutations of the TSH receptor are rare etiologies for hyperthyroidism. They can be inherited in an autosomal dominant manner (familial or hereditary, FNAH), or may occur sporadically as a de novo condition, also called: persistent sporadic congenital non-autoimmune hyperthyroidism (PSNAH). These three conditions: autonomic adenoma, FNAH and PSNAH constitute the inheritable and sporadic non-autoimmune hyperthyroidism. Particularities in epidemiology, etiology, molecular and clinical aspects of these three entities will be discussed in this review in order to guide to an accurate diagnosis allowing among others genetic counseling and presymptomatic diagnosis for the affected families. The optimal treatment based on the right diagnosis will avoid consequences of a persistent or relapsing hyperthyroidism. Crown Copyright © 2017. Published by Elsevier Ltd. All rights reserved.

BRCA1 and BRCA2 Gene Mutations Screening In Sporadic Breast Cancer Patients In Kazakhstan.

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Ainur R. Akilzhanova

2013-05-01

Full Text Available Background: A large number of distinct mutations in the BRCA1 and BRCA2 genes have been reported worldwide, but little is known regarding the role of these inherited susceptibility genes in breast cancer risk among Kazakhstan women. Aim: To evaluate the role of BRCA1/2 mutations in Kazakhstan women presenting with sporadic breast cancer. Methods: We investigated the distribution and nature of polymorphisms in BRCA1 and BRCA2 entire coding regions in 156 Kazakhstan sporadic breast cancer cases and 112 age-matched controls using automatic direct sequencing. Results: We identified 22 distinct variants, including 16 missense mutations and 6 polymorphisms in BRCA1/2 genes. In BRCA1, 9 missense mutations and 3 synonymous polymorphisms were observed. In BRCA2, 7 missense mutations and 3 polymorphisms were detected. There was a higher prevalence of observed mutations in Caucasian breast cancer cases compared to Asian cases (p<0.05; higher frequencies of sequence variants were observed in Asian controls. No recurrent or founder mutations were observed in BRCA1/2 genes. There were no statistically significant differences in age at diagnosis, tumor histology, size of tumor, and lymph node involvement between women with breast cancer with or without the BRCA sequence alterations. Conclusions: Considering the majority of breast cancer cases are sporadic, the present study will be helpful in the evaluation of the need for the genetic screening of BRCA1/2 mutations and reliable genetic counseling for Kazakhstan sporadic breast cancer patients. Evaluation of common polymorphisms and mutations and breast cancer risk in families with genetic predisposition to breast cancer is ongoing in another current investigation. 

Observational study of atmospheric surface layer and coastal weather in northern Qatar

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Samanta, Dhrubajyoti; Sadr, Reza

2016-04-01

Atmospheric surface layer is the interaction medium between atmosphere and Earth's surface. Better understanding of its turbulence nature is essential in characterizing the local weather, climate variability and modeling of turbulent exchange processes. The importance of Middle East region, with its unique geographical, economical and weather condition is well recognized. However, high quality micrometeorological observational studies are rare in this region. Here we show experimental results from micrometeorological observations from an experimental site in the coastal region of Qatar during August-December 2015. Measurements of winds are obtained from three sonic anemometers installed on a 9 m tower placed at Al Ghariyah beach in northern Qatar (26.08 °N, 51.36 °E). Different surface layer characteristics is analyzed and compared with earlier studies in equivalent weather conditions. Monthly statistics of wind speed, wind direction, temperature, humidity and heat index are made from concurrent observations from sonic anemometer and weather station to explore variations with surface layer characteristics. The results also highlights potential impact of sea breeze circulation on local weather and atmospheric turbulence. The observed daily maximum temperature and heat index during morning period may be related to sea breeze circulations. Along with the operational micrometeorological observation system, a camera system and ultrasonic wave measurement system are installed recently in the site to study coastline development and nearshore wave dynamics. Overall, the complete observational set up is going to provide new insights about nearshore wind dynamics and wind-wave interaction in Qatar.

An emerging role for misfolded wild-type SOD1 in sporadic ALS pathogenesis

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Melissa S Rotunno

2013-12-01

Full Text Available Amyotrophic lateral sclerosis (ALS is a fatal neurodegenerative disorder that targets motor neurons, leading to paralysis and death within a few years of disease onset. While several genes have been linked to the inheritable, or familial, form of ALS, much less is known about the cause(s of sporadic ALS, which accounts for approximately 90% of ALS cases. Due to the clinical similarities between familial and sporadic ALS, it is plausible that both forms of the disease converge on a common pathway and, therefore, involve common factors. Recent evidence suggests the Cu,Zn-superoxide dismutase (SOD1 protein to be one such factor that is common to both sporadic and familial ALS. In 1993, mutations were uncovered in SOD1 that represent the first known genetic cause of familial ALS. While the exact mechanism of mutant-SOD1 toxicity is still not known today, most evidence points to a gain of toxic function that stems, at least in part, from the propensity of this protein to misfold. In the wild-type SOD1 protein, non-genetic perturbations such as metal depletion, disruption of the quaternary structure, and oxidation, can also induce SOD1 to misfold. In fact, these aforementioned post-translational modifications cause wild-type SOD1 to adopt a toxic conformation that is similar to familial ALS-linked SOD1 variants. These observations, together with the detection of misfolded wild-type SOD1 within human post-mortem sporadic ALS samples, have been used to support the controversial hypothesis that misfolded forms of wild-type SOD1 contribute to sporadic ALS pathogenesis. In this review, we present data from the literature that both support and contradict this hypothesis. We also discuss SOD1 as a potential therapeutic target for both familial and sporadic ALS.

The P413L chromogranin B variation in French patients with sporadic amyotrophic lateral sclerosis.

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Blasco, Hélène; Corcia, Philippe; Veyrat-Durebex, Charlotte; Coutadeur, Cathleen; Fournier, Clémentine; Camu, William; Gordon, Paul; Praline, Julien; Andres, Christian R; Vourc'h, Patrick

2011-05-01

Chromogranins interact with mutant forms of superoxide dismutase 1 (SOD1) responsible for a portion of familial amyotrophic lateral sclerosis (ALS). A particular variation (P413L) in the chromogranin B gene, CHGB, has been recently associated with an earlier age at onset in both familial and sporadic ALS. The aim of our study was to evaluate the P413L chromogranin variation in French patients with sporadic amyotrophic lateral sclerosis. We developed a High Resolution DNA Melting (HRM) protocol to analyse the P413L variation in the CHGB gene in 540 French patients with sporadic ALS and 504 controls. The clinical characteristics of patients were analysed in relation to their genotype. Results showed that our study on a large cohort of French-Caucasian patients with SALS and controls failed to confirm an increased frequency of the 413L variant in SALS patients. This frequency was 5.3% in the SALS population and 5.5% in the control group. Moreover, we did not observe a previous observation of a difference of age at onset between T-allele carriers and non-carriers (median age of onset 60.4 vs. 62.0 years of age, respectively). Thus, our findings do not support the 413L variant of rs742710 as a risk factor for sporadic ALS in the French population.

Microsatellite D21D210 (GT-12) allele frequencies in sporadic Alzheimer's disease

International Nuclear Information System (INIS)

Lannfelt, L.; Lilius, L.; Viitanen, M.; Winblad, B.; Basun, H.; Houlden, H.; Rossor, M.; Hardy, J.

1995-01-01

Four disease-causing mutations have so far been described in the amyloid precursor protein gene on chromosome 21 in familial early-onset Alzheimer's disease. Linkage analysis with a fourteen-allele microsatellite at D21S210 named GT-12 has proven useful in the elucidation of amyloid presursor protein gene involvement in Alzheimer's disease families, as it is closely linked to the gene. Most cases of Alzheimer's disease are thought to be sporadic and not familial. However, evidence from earlier studies suggests an important genetic contribution also in sporadic cases, where gene-environment interaction may contribute to the disease. We have determined frequencies of the GT-12 alleles in 78 Swedish and 49 British sporadic Alzheimer's disease cases and 104 healthy elderly control subjects, to investigate if the disease associates with a particular genotype in GT-12. However, no differences in allele frequencies were observed between any of the groups. (au) (26 refs.)

Generation of mesoscale F layer structure and electric fields by the combined Perkins and Es layer instabilities, in simulations

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R. B. Cosgrove

2007-07-01

Full Text Available The generic equilibrium configuration of the nighttime midlatitude ionosphere consists of an F layer held up against gravity by winds and/or electric fields, and a sporadic E (Es layer located by a sheared wind field, which experiences the same electric fields as the F layer. This configuration is subject to two large-scale (e.g. >10 km "layer instabilities": one of the F layer known as the Perkins instability, and another of the Es layer which has been called the Es layer instability. Electric fields on scales larger than (about 10 km map very efficiently between the Es and F layers, and the two instabilities have a similar geometry, allowing them to interact with one another. As shown through a linear growth rate analysis, the two most important parameters governing the interaction are the relative horizontal velocity between the Es and F layers, and the integrated conductivity ratio ΣH/ΣPF, where ΣH and ΣPF are the field line integrated Hall conductivity of the Es layer, and the field line integrated Pedersen conductivity of the F layer, respectively. For both large and small relative velocities the growth rate was found to be more than double that of the Perkins instability alone, when ΣHΣPF=1.8. However, the characteristic eigenmode varies considerably with relative velocity, and different nonlinear behavior is expected in these two cases. As a follow up to the linear growth rate analysis, we explore in this article the nonlinear evolution of the unstable coupled system subject to a 200 km wavelength initial perturbation of the F layer, using a two-dimensional numerical solution of the two-fluid equations, as a function of relative horizontal velocity and ΣHΣPF. We find that when ΣHΣPF⪝0.5 the Perkins instability is able to control the dynamics and modulate the F layer altitude in 2 to 3 h time. However, the electric fields remain small until the altitude modulation is extremely large, and even then they are not large enough to

Neuropathological and biochemical criteria to identify acquired Creutzfeldt-Jakob disease among presumed sporadic cases.

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Kobayashi, Atsushi; Parchi, Piero; Yamada, Masahito; Mohri, Shirou; Kitamoto, Tetsuyuki

2016-06-01

As an experimental model of acquired Creutzfeldt-Jakob disease (CJD), we performed transmission studies of sporadic CJD using knock-in mice expressing human prion protein (PrP). In this model, the inoculation of the sporadic CJD strain V2 into animals homozygous for methionine at polymorphic codon 129 (129 M/M) of the PRNP gene produced quite distinctive neuropathological and biochemical features, that is, widespread kuru plaques and intermediate type abnormal PrP (PrP(Sc) ). Interestingly, this distinctive combination of molecular and pathological features has been, to date, observed in acquired CJD but not in sporadic CJD. Assuming that these distinctive phenotypic traits are specific for acquired CJD, we revisited the literature and found two cases showing widespread kuru plaques despite the 129 M/M genotype, in a neurosurgeon and in a patient with a medical history of neurosurgery without dura mater grafting. By Western blot analysis of brain homogenates, we revealed the intermediate type of PrP(Sc) in both cases. Furthermore, transmission properties of brain extracts from these two cases were indistinguishable from those of a subgroup of dura mater graft-associated iatrogenic CJD caused by infection with the sporadic CJD strain V2. These data strongly suggest that the two atypical CJD cases, previously thought to represent sporadic CJD, very likely acquired the disease through exposure to prion-contaminated brain tissues. Thus, we propose that the distinctive combination of 129 M/M genotype, kuru plaques, and intermediate type PrP(Sc) , represents a reliable criterion for the identification of acquired CJD cases among presumed sporadic cases. © 2015 Japanese Society of Neuropathology.

Modeling the Observed Microwave Emission from Shallow Multi-Layer Tundra Snow Using DMRT-ML

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Nastaran Saberi

2017-12-01

Full Text Available The observed brightness temperatures (Tb at 37 GHz from typical moderate density dry snow in mid-latitudes decreases with increasing snow water equivalent (SWE due to volume scattering of the ground emissions by the overlying snow. At a certain point, however, as SWE increases, the emission from the snowpack offsets the scattering of the sub-nivean emission. In tundra snow, the Tb slope reversal occurs at shallower snow thicknesses. While it has been postulated that the inflection point in the seasonal time series of observed Tb V 37 GHz of tundra snow is controlled by the formation of a thick wind slab layer, the simulation of this effect has yet to be confirmed. Therefore, the Dense Media Radiative Transfer Theory for Multi Layered (DMRT-ML snowpack is used to predict the passive microwave response from airborne observations over shallow, dense, slab-layered tundra snow. Airborne radiometer observations coordinated with ground-based in situ snow measurements were acquired in the Canadian high Arctic near Eureka, NT, in April 2011. The DMRT-ML was parameterized with the in situ snow measurements using a two-layer snowpack and run in two configurations: a depth hoar and a wind slab dominated pack. With these two configurations, the calibrated DMRT-ML successfully predicted the Tb V 37 GHz response (R correlation of 0.83 when compared with the observed airborne Tb footprints containing snow pits measurements. Using this calibrated model, the DMRT-ML was applied to the whole study region. At the satellite observation scale, observations from the Advanced Microwave Scanning Radiometer-Earth Observing System (AMSR-E over the study area reflected seasonal differences between Tb V 37 GHz and Tb V 19 GHz that supports the hypothesis of the development of an early season volume scattering depth hoar layer, followed by the growth of the late season emission-dominated wind slab layer. This research highlights the necessity to consider the two

Atmospheric aerosol layers over Bangkok Metropolitan Region from CALIPSO observations

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Bridhikitti, Arika

2013-06-01

Previous studies suggested that aerosol optical depth (AOD) from the Earth Observing System satellite retrievals could be used for inference of ground-level air quality in various locations. This application may be appropriate if pollution in elevated atmospheric layers is insignificant. This study investigated the significance of elevated air pollution layers over the Bangkok Metropolitan Region (BMR) from all available aerosol layer scenes taken from Cloud-Aerosol Lidar and Infrared Pathfinder Satellite Observations (CALIPSO) for years 2007 to 2011. The results show that biomass burning smoke layers alone were the most frequently observed. The smoke layers accounted for high AOD variations and increased AOD levels. In the dry seasons, the smoke layers alone with high AOD levels were likely brought to the BMR via northeasterly to easterly prevailing winds and found at altitudes above the typical BMR mixing heights of approximately 0.7 to 1.5 km. The smoke should be attributed to biomass burning emissions outside the BMR.

Composition of the low seismic velocity E' layer at the top of Earth's core

Science.gov (United States)

Badro, J.; Brodholt, J. P.

2017-12-01

Evidence for a layer (E') at the top of the outer core has been available since the '90s and while different studies suggest slightly different velocity contrasts and thicknesses, the common observation is that the layer has lower velocities than the bulk outer core (PREM). Although there are no direct measurements on the density of this layer, dynamic stability requires it to be less dense than the bulk outer core under those same pressure and temperature conditions. Using ab initio simulations on Fe-Ni-S-C-O-Si liquids we constrain the origin and composition of the low-velocity layer E' at the top of Earth's outer core. We find that increasing the concentration of any light-element always increases velocity and so a low-velocity and low-density layer (for stability) cannot be made by simply increasing light element concentration. This rules out barodiffusion or upwards sedimentation of a light phase for its origin. However, exchanging elements can—depending on the elements exchanged—produce such a layer. We evaluate three possibilities. Firstly, crystallization of a light phase from a core containing more than one light element may make such a layer, but only if the crystalizing phase is very Fe-rich, which is at odds with available phase diagrams at CMB conditions. Secondly, the E' layer may result from incomplete mixing of an early Earth core with a late impactor, depending on the light element compositions of the impactor and Earth's core, but such a primordial stratification is neither supported by dynamical models of the core nor thermodynamic models of core merger after the giant impact. The last and most plausible scenario is core-mantle chemical interaction; using thermodynamic models for metal-silicate partitioning of silicon and oxygen at CMB conditions, we show that a reaction between the core and an FeO-rich basal magma ocean can enrich the core in oxygen while depleting it in silicon, in relative amounts that produce a light and slow layer

Study of medium-scale traveling ionospheric disturbances (MSTID) with sounding rockets and ground observations

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Yamamoto, Mamoru; Abe, Takumi; Kumamoto, Atsushi; Yokoyama, Tatsuhiro; Bernhardt, Paul; Watanabe, Shigeto; Yamamoto, Masa-yuki; Larsen, Miguel; Saito, Susumu; Tsugawa, Takuya; Ishisaka, Keigo; Iwagami, Naomoto; Nishioka, Michi; Kato, Tomohiro; Takahashi, Takao; Tanaka, Makoto; Mr

Medium-scale traveling ionospheric disturbance (MSTID) is an interesting phenomenon in the F-region. The MSTID is frequent in summer nighttime over Japan, showing wave structures with wavelengths of 100-200 km, periodicity of about 1 hour, and propagation toward the southwest. The phenomena are observed by the total electron content (TEC) from GEONET, Japanese dense network of GPS receivers, and 630 nm airglow imagers as horizontal pattern. It was also measured as Spread-F events of ionograms or as field-aligned echoes of the MU radar. MSTID was, in the past, explained by Perkins instability (Perkins, 1973) while its low growth rate was a problem. Recently 3D simulation study by Yokoyama et al (2009) hypothesized a generation mechanism of the MSTID, which stands on electromagnetic E/F-region coupling of the ionosphere. The hypothesis is that the MSTID first grows with polarization electric fields from sporadic-E, then show spatial structures resembling to the Perkins instability. We recently conducted a observation campaign to check this hypothesis. We launched JASA ISAS sounding rockets S-310-42 and S-520-27 at 23:00 JST and 23:57JST on July 20, 2013 while an MSTID event was monitored in real-time by the GPS-TEC from GEONET. We found 1-5mV/m northeastward/eastward electric fields during the flight. Variation of electric fileds were associated with horizontal distribution of plasma density. Wind velocity was measured by the TME and Lithium releases from S-310-42 and S-520-27 rockets, respectively, showing southward wind near the sporadic-E layer heights. These results are consistent to the expected generation mechanism shown above. In the presentation we will discuss electric-field results and its relationship with plasma density variability together with preliminary results from the neutral-wind observations.

Intermediate layer observed by the impedance probe on board the S-310-3 sounding rocket

Energy Technology Data Exchange (ETDEWEB)

Watanabe, Y; Obayashi, T [Tokyo Univ. (Japan). Inst. of Space and Aeronautical Science

1977-08-01

The intermediate layer (or M layer) was detected at the height of 150-170 km in the nighttime ionospheric electron density profile measured by impedance probe on board the S-310-3 sounding rocket. This M layer was interpreted to be generated by the convergence effect of the ionization due to the west-east component of the solar tidal wind as suggested by K.Fujitaka. The altitude variation of the M layer during the course of a night is studied at three other locations with different latitudes. At Boulder (40/sup 0/N, 105/sup 0/W) and Wallops Island (38/sup 0/N, 75/sup 0/W) which have higher latitude than that of KSC(31/sup 0/N, 131/sup 0/E), the altitude of the observed M layers seems to be determined by the north-south component of the wind above about 150 km, by the west-east component of the wind below about 130 km in agreement with the drift theory. The altitude of the observed M layers at Arecibo (19/sup 0/N, 67/sup 0/W) located at lower latitude than that of KSC also coincides with the theoretical estimate when the direction of the north-south wind is assumed to be opposite to that prevailing in middle latitudes. Thus, M layer observations at these stations are consistent with the view that around the latitude range of KSC the north-south wind reverses direction and the west-east component of the wind has the dominant effect on the formation of the M layer.

Microsatellite D21D210 (GT-12) allele frequencies in sporadic Alzheimer`s disease

Energy Technology Data Exchange (ETDEWEB)

Lannfelt, L; Lilius, L; Viitanen, M; Winblad, B; Basun, H [Huddinge Hospital, Karolinska Institute, Dept. of Geriatric Medicine, (Sweden); Houlden, H; Rossor, M [St. Mary` s Hospital, Dept. of Neurology, Medical School, London (United Kingdom); Hardy, J [University of South Florida, Suncoast Alzheimer` s Disease Research Labs, Department of Psychiatry, Tampa (United States)

1995-02-01

Four disease-causing mutations have so far been described in the amyloid precursor protein gene on chromosome 21 in familial early-onset Alzheimer`s disease. Linkage analysis with a fourteen-allele microsatellite at D21S210 named GT-12 has proven useful in the elucidation of amyloid presursor protein gene involvement in Alzheimer`s disease families, as it is closely linked to the gene. Most cases of Alzheimer`s disease are thought to be sporadic and not familial. However, evidence from earlier studies suggests an important genetic contribution also in sporadic cases, where gene-environment interaction may contribute to the disease. We have determined frequencies of the GT-12 alleles in 78 Swedish and 49 British sporadic Alzheimer`s disease cases and 104 healthy elderly control subjects, to investigate if the disease associates with a particular genotype in GT-12. However, no differences in allele frequencies were observed between any of the groups. (au) (26 refs.).

The Prion Protein Preference of Sporadic Creutzfeldt-Jakob Disease Subtypes*

Science.gov (United States)

Klemm, Helen M. J.; Welton, Jeremy M.; Masters, Colin L.; Klug, Genevieve M.; Boyd, Alison; Hill, Andrew F.; Collins, Steven J.; Lawson, Victoria A.

2012-01-01

Sporadic Creutzfeldt-Jakob disease (CJD) is the most prevalent manifestation of the transmissible spongiform encephalopathies or prion diseases affecting humans. The disease encompasses a spectrum of clinical phenotypes that have been correlated with molecular subtypes that are characterized by the molecular mass of the protease-resistant fragment of the disease-related conformation of the prion protein and a polymorphism at codon 129 of the gene encoding the prion protein. A cell-free assay of prion protein misfolding was used to investigate the ability of these sporadic CJD molecular subtypes to propagate using brain-derived sources of the cellular prion protein (PrPC). This study confirmed the presence of three distinct sporadic CJD molecular subtypes with PrPC substrate requirements that reflected their codon 129 associations in vivo. However, the ability of a sporadic CJD molecular subtype to use a specific PrPC substrate was not determined solely by codon 129 as the efficiency of prion propagation was also influenced by the composition of the brain tissue from which the PrPC substrate was sourced, thus indicating that nuances in PrPC or additional factors may determine sporadic CJD subtype. The results of this study will aid in the design of diagnostic assays that can detect prion disease across the diversity of sporadic CJD subtypes. PMID:22930754

Multiple imputation by chained equations for systematically and sporadically missing multilevel data.

Science.gov (United States)

Resche-Rigon, Matthieu; White, Ian R

2018-06-01

In multilevel settings such as individual participant data meta-analysis, a variable is 'systematically missing' if it is wholly missing in some clusters and 'sporadically missing' if it is partly missing in some clusters. Previously proposed methods to impute incomplete multilevel data handle either systematically or sporadically missing data, but frequently both patterns are observed. We describe a new multiple imputation by chained equations (MICE) algorithm for multilevel data with arbitrary patterns of systematically and sporadically missing variables. The algorithm is described for multilevel normal data but can easily be extended for other variable types. We first propose two methods for imputing a single incomplete variable: an extension of an existing method and a new two-stage method which conveniently allows for heteroscedastic data. We then discuss the difficulties of imputing missing values in several variables in multilevel data using MICE, and show that even the simplest joint multilevel model implies conditional models which involve cluster means and heteroscedasticity. However, a simulation study finds that the proposed methods can be successfully combined in a multilevel MICE procedure, even when cluster means are not included in the imputation models.

Serum uric acid and lipid profiles in sporadic Creutzfeldt-Jakob disease.

Science.gov (United States)

Chen, Shuai; He, Shuang; Shang, Jun-Kui; Ma, Ming-Ming; Xu, Chang-Shui; Shi, Xiao-Hong; Zhang, Jie-Wen

2016-02-01

Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive, and fatal neurodegenerative disease affecting the central nervous system. Brain lipid homeostasis and oxidative stress seem to play an important role in the disease pathogenesis. But little was known whether serum lipids and uric acid (a natural antioxidant) levels changed in patients with prion disease. Here we retrospectively reviewed and compared the serum lipids and uric acid levels of 19 probable sporadic CJD patients and 26 healthy control subjects. We found that the serum uric acid levels in sporadic CJD patients were significantly lower than that in controls (P=0.01). Serum triglycerides, cholesterol, low-density lipoprotein (LDL), high-density lipoprotein (HDL), and apolipoprotein A1 (ApoA1) were similar in sporadic CJD patients and controls. However, LDL/HDL ratio was lower in sporadic CJD patients (P=0.003). The low serum uric acid and LDL/HDL ratio levels in sporadic CJD indicate that dysfunction in the lipid homeostasis and oxidative stress is associated with sporadic prion disease. Copyright © 2015 The Canadian Society of Clinical Chemists. Published by Elsevier Inc. All rights reserved.

The first coordinated observations of mid-latitude E-region quasi-periodic radar echoes and lower thermospheric 557.7-nm airglow

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T. Ogawa

2005-10-01

Full Text Available We present the first coordinated observations of quasi-periodic (QP radar echoes from sporadic-E (Es field-aligned irregularities (FAIs, OI 557.7-nm airglow, and neutral winds in a common volume over Shigaraki, Japan (34.9° N, 136.1° E on the night of 5 August 2002 during the SEEK-2 campaign. QP echo altitudes of 90-110 km were lower than usual by 10 km, enabling us to make a detailed comparison among QP echoes, airglow intensity, and neutral wind at around 96 km altitude. Eastward movement of the QP echo regions is consistent with the motions of neutral winds, airglow structures, and FAIs, suggesting that the electrodynamics of Es-layers is fundamentally controlled by the neutral atmospheric dynamics. During the QP echo event, the echo altitudes clearly went up (down in harmony with an airglow enhancement (subsidence that also moved to the east. This fact suggests that the eastward-moving enhanced airglow region included an upward (downward component of neutral winds to raise (lower the altitude of the wind-shear node responsible for the Es formation. The airglow intensity, echo intensity, and Doppler velocity of FAIs at around 96 km altitude fluctuated with periods from 10 min to 1h, indicating that these parameters were modulated with short-period atmospheric disturbances. Some QP echo regions below 100km altitude contained small-scale QP structures in which very strong neutral winds exceeding 100 m/s existed. The results are compared with recent observations, theories, and simulations of QP echoes. Keywords. Ionosphere (Ionosphere-atmosphere interactions; Ionospheric irregularities; Mid-latitude ionosphere

Impaired proteasome function in sporadic amyotrophic lateral sclerosis.

Science.gov (United States)

Kabashi, Edor; Agar, Jeffrey N; Strong, Michael J; Durham, Heather D

2012-06-01

Abstract The ubiquitin-proteasome system, important for maintaining protein quality control, is compromised in experimental models of familial ALS. The objective of this study was to determine if proteasome function is impaired in sporadic ALS. Proteasomal activities and subunit composition were evaluated in homogenates of spinal cord samples obtained at autopsy from sporadic ALS and non-neurological control cases, compared to cerebellum as a clinically spared tissue. The level of 20S α structural proteasome subunits was assessed in motor neurons by immunohistochemistry. Catalysis of peptide substrates of the three major proteasomal activities was substantially reduced in ALS thoracic spinal cord, but not in cerebellum, accompanied by alterations in the constitutive proteasome machinery. Chymotrypsin-like activity was decreased to 60% and 65% of control in ventral and dorsal spinal cord, respectively, concomitant with reduction in the β5 subunit with this catalytic activity. Caspase- and trypsin-like activities were reduced to a similar extent (46% - 68% of control). Proteasome levels, although generally maintained, appeared reduced specifically in motor neurons by immunolabelling. In conclusion, there are commonalities of findings in sporadic ALS patients and presymptomatic SOD1-G93A transgenic mice and these implicate inadequate proteasome function in the pathogenesis of both familial and sporadic ALS.

Dietary factors and microsatellite instability in sporadic colon carcinomas

NARCIS (Netherlands)

Diergaarde, B.; Braam, H.; Muijen, van G.N.P.; Ligtenberg, M.J.L.; Kok, F.J.; Kampman, E.

2003-01-01

Microsatellite instability (MSI) occurs in 10-20% of the sporadic colon carcinomas and appears to be primarily due to alterations in hMLH1 and hMSH2. Little is known about the role of diet in MSI-related colon carcinogenesis. We used data from a Dutch population-based case-control study on sporadic

Dietary factors and microsatellite instability in sporadic colon carcinomas.

NARCIS (Netherlands)

Diergaarde, B.; Braam, H.; Muijen, G.N.P. van; Ligtenberg, M.J.L.; Kok, F.J.; Kampman, E.

2003-01-01

Microsatellite instability (MSI) occurs in 10-20% of the sporadic colon carcinomas and appears to be primarily due to alterations in hMLH1 and hMSH2. Little is known about the role of diet in MSI-related colon carcinogenesis. We used data from a Dutch population-based case-control study on sporadic

Visual art therapy in sporadic Creutzfeldt-Jakob disease: a case study.

Science.gov (United States)

Shrestha, Rajeet; Trauger-Querry, Barbara; Loughrin, Athena; Appleby, Brian S

2016-01-01

This paper describes the diagnostic and treatment utility of visual art therapy in a case of sporadic Creutzfeldt-Jakob disease. Visual art therapy was compared longitudinally with clinical and neuroimaging data over five-month period in an autopsy-confirmed case of sporadic Creutzfeldt-Jakob disease of MM2-cortical subtype. Art therapy sessions and content were useful in ascertaining neuropsychiatric symptoms during the course of her illness. Art therapy offered a unique emotional and cognitive outlet as illness progressed. Patients and families affected by sporadic Creutzfeldt-Jakob disease may benefit from art therapy despite the rapidly progressive nature of the illness. Art therapy can also be useful for assessment of patients with sporadic Creutzfeldt-Jakob disease by healthcare professionals.

Case-Control Studies of Sporadic Enteric Infections: A Review and Discussion of Studies Conducted Internationally from 1990 to 2009

Science.gov (United States)

Fullerton, Kathleen E.; Scallan, Elaine; Kirk, Martyn D.; Mahon, Barbara E.; Angulo, Frederick J.; de Valk, Henriette; van Pelt, Wilfrid; Gauci, Charmaine; Hauri, Anja M.; Majowicz, Shannon; O’Brien, Sarah J.

2015-01-01

Epidemiologists have used case-control studies to investigate enteric disease outbreaks for many decades. Increasingly, case-control studies are also used to investigate risk factors for sporadic (not outbreak-associated) disease. While the same basic approach is used, there are important differences between outbreak and sporadic disease settings that need to be considered in the design and implementation of the case-control study for sporadic disease. Through the International Collaboration on Enteric Disease “Burden of Illness” Studies (the International Collaboration), we reviewed 79 case-control studies of sporadic enteric infections caused by nine pathogens that were conducted in 22 countries and published from 1990 through to 2009. We highlight important methodological and study design issues (including case definition, control selection, and exposure assessment) and discuss how approaches to the study of sporadic enteric disease have changed over the last 20 years (e.g., making use of more sensitive case definitions, databases of controls, and computer-assisted interviewing). As our understanding of sporadic enteric infections grows, methods and topics for case-control studies are expected to continue to evolve; for example, advances in understanding of the role of immunity can be used to improve control selection, the apparent protective effects of certain foods can be further explored, and case-control studies can be used to provide population-based measures of the burden of disease. PMID:22443481

Origin of the 2.45 eV luminescence band observed in ZnO epitaxial layers grown on c-plane sapphire by chemical vapour deposition

International Nuclear Information System (INIS)

Saroj, R K; Dhar, S

2014-01-01

Zinc oxide epitaxial layers have been grown on c-plane sapphire substrates by the chemical vapour deposition (CVD) technique. A structural study shows (0001)-oriented films with good crystalline quality. The temperature and excitation power dependence of the photoluminescence (PL) characteristics of these layers is studied as a function of various growth parameters, such as the growth temperature, oxygen flow rate and Zn flux, which suggest that the origin of the broad visible luminescence (VL), which peaks at 2.45 eV, is the transition between the conduction band and the Zn vacancy acceptor states. A bound excitonic transition observed at 3.32 eV in low temperature PL has been identified as an exciton bound to the neutral Zn vacancy. Our study also reveals the involvement of two activation processes in the dynamics of VL, which has been explained in terms of the fluctuation of the capture barrier height for the holes trapped in Zn vacancy acceptors. The fluctuation, which might be a result of the inhomogeneous distribution of Zn vacancies, is found to be associated with an average height of 7 and 90 meV, respectively, for the local and global maxima. (paper)

Familial or Sporadic Idiopathic Scoliosis – classification based on artificial neural network and GAPDH and ACTB transcription profile

Science.gov (United States)

2013-01-01

Background Importance of hereditary factors in the etiology of Idiopathic Scoliosis is widely accepted. In clinical practice some of the IS patients present with positive familial history of the deformity and some do not. Traditionally about 90% of patients have been considered as sporadic cases without familial recurrence. However the exact proportion of Familial and Sporadic Idiopathic Scoliosis is still unknown. Housekeeping genes encode proteins that are usually essential for the maintenance of basic cellular functions. ACTB and GAPDH are two housekeeping genes encoding respectively a cytoskeletal protein β-actin, and glyceraldehyde-3-phosphate dehydrogenase, an enzyme of glycolysis. Although their expression levels can fluctuate between different tissues and persons, human housekeeping genes seem to exhibit a preserved tissue-wide expression ranking order. It was hypothesized that expression ranking order of two representative housekeeping genes ACTB and GAPDH might be disturbed in the tissues of patients with Familial Idiopathic Scoliosis (with positive family history of idiopathic scoliosis) opposed to the patients with no family members affected (Sporadic Idiopathic Scoliosis). An artificial neural network (ANN) was developed that could serve to differentiate between familial and sporadic cases of idiopathic scoliosis based on the expression levels of ACTB and GAPDH in different tissues of scoliotic patients. The aim of the study was to investigate whether the expression levels of ACTB and GAPDH in different tissues of idiopathic scoliosis patients could be used as a source of data for specially developed artificial neural network in order to predict the positive family history of index patient. Results The comparison of developed models showed, that the most satisfactory classification accuracy was achieved for ANN model with 18 nodes in the first hidden layer and 16 nodes in the second hidden layer. The classification accuracy for positive Idiopathic

Legionnaires’ Disease: Clinicoradiological Comparison of Sporadic Versus Outbreak Cases

Directory of Open Access Journals (Sweden)

Hafiz Rizwan Talib Hashmi

2017-06-01

Full Text Available Background: In 2015, New York City experienced the worst outbreak of Legionnaires’ disease in the history of the city. We compare patients seen during the 2015 outbreak with sporadic cases of Legionella during the past 5 years. Methods: We conducted a retrospective chart review of 90 patients with Legionnaires’ disease, including sporadic cases of Legionella infection admitted from 2010 to 2015 (n = 55 and cases admitted during the 2015 outbreak (n = 35. Results: We saw no significant differences between the 2 groups regarding demographics, smoking habits, alcohol intake, underlying medical disease, or residence type. Univariate and multivariate analyses showed that patients with sporadic case of Legionella had a longer stay in the hospital and intensive care unit as well as an increased stay in mechanical ventilation. Short-term mortality, discharge disposition, and most clinical parameters did not differ significantly between the 2 groups. Conclusions: We found no specific clinicoradiological characteristics that could differentiate sporadic from epidemic cases of Legionella . Early recognition and high suspicion for Legionnaires’ disease are critical to provide appropriate treatment. Cluster of cases should increase suspicion for an outbreak.

Quiet Time Depression of the Equatorial Electrojet and Dynamics of the F-layer Ionosphere

Science.gov (United States)

Khadka, S.; Valladares, C. E.; Doherty, P.

2017-12-01

The depression of the equatorial electrojet (EEJ) is marked by a westward current due to streaming movement of laterally limited (±3°) charged particles in the ionospheric E region during the day along the magnetic equator. It is a complex low-latitude phenomenon and driven by various sources of electric fields associated with global neutral wind, solar tidal force, Interplanetary magnetic Field (IMF), etc. This unique physical property of the equatorial ionosphere holds a great promise for sorting out the governing mechanism of the dayside ionospheric electrodynamics and the onset of the enigmatic plasma structures in the ionospheric layers. Present study provides an overview of the special sequence of the longitudinal, seasonal, and occurrence rate variability of the depression of the EEJ, including its temporal variation, using data from an excellent chain of magnetic and ionospheric observatories along the low-latitude regions. A case and statistical study of the geomagnetically quiet time depression of EEJ strengths is presented using a pair of magnetometers, one located at the dip equator and another off the dip equator (±6° to ±9° away) in the American low-latitude regions. The significance of the variability of the depression of the EEJ current observed in the scenario of vertical drifts, sporadic E-layer, the equatorial F region plasma fountain, and height of the peak ionization in the F-layer, as well as GPS-TEC distributions, will be investigated.

SARC006: Phase II Trial of Chemotherapy in Sporadic and Neurofibromatosis Type 1 Associated Chemotherapy-Naive Malignant Peripheral Nerve Sheath Tumors

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Christine S. Higham

2017-01-01

Full Text Available Background. Worse chemotherapy response for neurofibromatosis type 1- (NF1- associated compared to sporadic malignant peripheral nerve sheath tumors (MPNST has been reported. Methods. We evaluated the objective response (OR rate of patients with AJCC Stage III/IV chemotherapy-naive NF1 MPNST versus sporadic MPNST after 4 cycles of neoadjuvant chemotherapy, 2 cycles of ifosfamide/doxorubicin, and 2 cycles of ifosfamide/etoposide. A Simon optimal two-stage design was used (target response rate 40%. Results. 34 NF1 (median age 33 years and 14 sporadic (median age 40 years MPNST patients enrolled. Five of 28 (17.9% evaluable NF1 MPNST patients had a partial response (PR, as did 4 of 9 (44.4% patients with sporadic MPNST. Stable disease (SD was achieved in 22 NF1 and 4 sporadic MPNST patients. In both strata, results in the initial stages met criteria for expansion of enrollment. Only 1 additional PR was observed in the expanded NF1 stratum. Enrollment was slower than expected and the trial closed before full accrual. Conclusions. This trial was not powered to detect differences in response rates between NF1 and sporadic MPNST. While the OR rate was lower in NF1 compared to sporadic MPNST, qualitative responses were similar, and disease stabilization was achieved in most patients.

Sporadic manipulation in money markets with central bank standing facilities

OpenAIRE

Ewerhart, Christian; Cassola, Nuno; Ejerskov, Steen; Valla, Natacha

2004-01-01

In certain market environments, a large investor may benefit from building up a futures position first and trading subsequently in the spot market (Kumar and Seppi, 1992). The present paper identifies a variation of this type of manipulation that might occur in money markets with an interest rate corridor. We show that manipulation involving the use of central bank facilities would be observable only sporadically. The probability of manipulation decreases when the central bank uses an active ...

Ion layers, tides, gravity waves, and electric fields in the upper atmosphere, inferred from Arecibo incoherent scatter radar measurements

International Nuclear Information System (INIS)

Morton, Y.T.

1991-01-01

This thesis uses data accumulated during 1980-1989 by the Arecibo incoherent scatter radar to study the behavior and physics of ionization irregularities. Low latitude ionization irregularities, known as sporadic-E and intermediate layers, undergo a regular daily descent, convergence, and dumping of ion layers controlled by the neutral tidal wind. A useful way of studying ion layers and their motion is by ion layer trajectory maps which consist of points representing the altitude and time of ionization layers. Two types of maps were used which assigned either a uniform layer intensity or a gray level/pseudo-color to indicate different layer intensities. Important aspects of layer formation are revealed by map analysis. During January, intermediate layers consistently appeared four times per day instead of the normal twice per day pattern. Simulation of ion trajectories based on the ion momentum equation, which includes both Lorentzian and collisional forces, shows that a combination of diurnal, semidiurnal, and six-hour tides is necessary for such a feature to exist, whereas only diurnal and semidiurnal tides are needed to create the normal pattern. The six-hour period tide has not been previously reported. Extra or irregular layers appear frequently in layer trajectory maps, which can be simulated by the addition of gravity waves to the regular tidal wind system. Electric field effects are normally not a factor in low latitude ion layer formation because they are relatively weak and not commonly observed. Layer configurations during a geomagnetic storm, however, indicate that the electric field played an important role in controlling ion motion

Sporadic colorectal polyps and mismatch repair proteins

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Mahsa Molaei

2011-01-01

Full Text Available Background: Colorectal cancers often arise from benign polyps. Adenomatous polyps and serrated polyps progress step by step to adenocarcinoma and change into malignant cancers. Genetic and epigenetic changes have correlation with specific stages of polyp-adenocarcinoma progression and colorectal cancer histopathological changes. Aims: In this study we used immunohistochemistry (IHC staining in sporadic colorectal polyps to assay functional status of MLH1, MSH2, MSH6, and PMS2 proteins, to track genetic/epigenetic roles of this issue in our patients. Materials and Methods: In this cross-sectional study we assessed all patients who were admitted with sporadic colorectal polyps and underwent polypectomy in endoscopy department during 2004-2008. Result: IHC results were abnormal in 6.8% cases for MLH1, in 4.5% cases for MSH2, in 3% for MSH6, and in 4.8% for PMS2. In all cases with abnormal PMS2, MLH1 was also reported as abnormal. Same results were reported for abnormal MSH2, which is accompanied with abnormal MSH6 in all cases (P values < 0.001. There is no significant difference between IHC staining results, gender, dysplasia grade, adenomatous type, and invasion. On the other hand, there was significant difference between IHC staining results, polyp location, and mean age of patients. The same significant difference was between adenomatous polyps and serrated adenoma polyps by MLH1 and PMS2 (P values < 0.05. Conclusion: According to our findings, maybe MMR dysfunction is the cause of sporadic colorectal polyps in younger age and its increasing risk of dysplasia progression and malignancy progression is only in serrated adenoma. Sporadic polyps in left colon had a higher risk to progress to malignancies, and abnormal IHC staining for MLH1 and PMS2 in serrated polyps is much more than in other adenomatous polyps.

Observations of the atmospheric boundary layer height over Abu Dhabi, United Arab Emirates: Investigating boundary layer climatology in arid regions

Science.gov (United States)

Marzooqi, Mohamed Al; Basha, Ghouse; Ouarda, Taha B. M. J.; Armstrong, Peter; Molini, Annalisa

2014-05-01

Strong sensible heat fluxes and deep turbulent mixing - together with marked dustiness and a low substrate water content - represent a characteristic signature in the boundary layer over hot deserts, resulting in "thicker" mixing layers and peculiar optical properties. Beside these main features however, desert ABLs present extremely complex local structures that have been scarcely addressed in the literature, and whose understanding is essential in modeling processes such as the transport of dust and pollutants, and turbulent fluxes of momentum, heat and water vapor in hyper-arid regions. In this study, we analyze a continuous record of observations of the atmospheric boundary layer (ABL) height from a single lens LiDAR ceilometer operated at Masdar Institute Field Station (24.4oN, 54.6o E, Abu Dhabi, United Arab Emirates), starting March 2013. We compare different methods for the estimation of the ABL height from Ceilometer data such as, classic variance-, gradient-, log gradient- and second derivation-methods as well as recently developed techniques such as the Bayesian Method and Wavelet covariance transform. Our goal is to select the most suited technique for describing the climatology of the ABL in desert environments. Comparison of our results with radiosonde observations collected at the nearby airport of Abu Dhabi indicate that the WCT and the Bayesian method are the most suitable tools to accurately identify the ABL height in all weather conditions. These two methods are used for the definition of diurnal and seasonal climatologies of the boundary layer conditional to different atmospheric stability classes.

Radar observations of artificial E-region field-aligned irregularities

Directory of Open Access Journals (Sweden)

E. Nossa

2009-07-01

Full Text Available Artificial E region field aligned plasma density irregularities (FAIs were generated using HAARP in four different experimental modes and observed with a coherent scatter radar imager located 450 km to the southwest where it could detect field-aligned backscatter. The experiments were conducted in July of 2008, during the Polar Aeronomy and Radio Science Summer School (PARS, during quiet conditions in the daytime when the E layer was dense and absorption was modest. The echoes observed during zenith and magnetic zenith heating experiments were deflected from their nominally anticipated horizontal positions toward the midpoint position. The occurrence of hysteresis when heating with amplitude modulated pulses implied the development of the resonance instability, although the threshold for the onset of instability appeared to be higher than what has been predicted theoretically. Heating experiments involving pump frequencies slightly above and below the second electron gyroharmonic frequency produced no significant differences in the observed echoes. Finally, heating with a pump frequency slightly above the E region critical frequency appears to have produced FAIs at two distinct altitudes where the upper-hybrid resonance condition could be satisfied.

Mutations in fibroblast growth-factor receptor 3 in sporadic cases of achondroplasia occur exclusively on the paternally derived chromosome.

Science.gov (United States)

Wilkin, D J; Szabo, J K; Cameron, R; Henderson, S; Bellus, G A; Mack, M L; Kaitila, I; Loughlin, J; Munnich, A; Sykes, B; Bonaventure, J; Francomano, C A

1998-01-01

More than 97% of achondroplasia cases are caused by one of two mutations (G1138A and G1138C) in the fibroblast growth factor receptor 3 (FGFR3) gene, which results in a specific amino acid substitution, G380R. Sporadic cases of achondroplasia have been associated with advanced paternal age, suggesting that these mutations occur preferentially during spermatogenesis. We have determined the parental origin of the achondroplasia mutation in 40 sporadic cases. Three distinct 1-bp polymorphisms were identified in the FGFR3 gene, within close proximity to the achondroplasia mutation site. Ninety-nine families, each with a sporadic case of achondroplasia in a child, were analyzed in this study. In this population, the achondroplasia mutation occurred on the paternal chromosome in all 40 cases in which parental origin was unambiguous. This observation is consistent with the clinical observation of advanced paternal age resulting in new cases of achondroplasia and suggests that factors influencing DNA replication or repair during spermatogenesis, but not during oogenesis, may predispose to the occurrence of the G1138 FGFR3 mutations. PMID:9718331

Moored surface buoy observations of the diurnal warm layer

KAUST Repository

Prytherch, J.

2013-09-01

An extensive data set is used to examine the dynamics of diurnal warming in the upper ocean. The data set comprises more than 4700 days of measurements at five sites in the tropics and subtropics, obtained from surface moorings equipped to make comprehensive meteorological, incoming solar and infrared radiation, and high-resolution subsurface temperature (and, in some cases, velocity) measurements. The observations, which include surface warmings of up to 3.4°C, are compared with a selection of existing models of the diurnal warm layer (DWL). A simple one-layer physical model is shown to give a reasonable estimate of both the magnitude of diurnal surface warming (model-observation correlation 0.88) and the structure and temporal evolution of the DWL. Novel observations of velocity shear obtained during 346 days at one site, incorporating high-resolution (1 m) upper ocean (5-15 m) acoustic Doppler current profile measurements, are also shown to be in reasonable agreement with estimates from the physical model (daily maximum shear model-observation correlation 0.77). Physics-based improvements to the one-layer model (incorporation of rotation and freshwater terms) are discussed, though they do not provide significant improvements against the observations reported here. The simplicity and limitations of the physical model are used to discuss DWL dynamics. The physical model is shown to give better model performance under the range of forcing conditions experienced across the five sites than the more empirical models. ©2013. American Geophysical Union. All Rights Reserved.

Moored surface buoy observations of the diurnal warm layer

KAUST Repository

Prytherch, J.; Farrar, J. T.; Weller, R. A.

2013-01-01

An extensive data set is used to examine the dynamics of diurnal warming in the upper ocean. The data set comprises more than 4700 days of measurements at five sites in the tropics and subtropics, obtained from surface moorings equipped to make comprehensive meteorological, incoming solar and infrared radiation, and high-resolution subsurface temperature (and, in some cases, velocity) measurements. The observations, which include surface warmings of up to 3.4°C, are compared with a selection of existing models of the diurnal warm layer (DWL). A simple one-layer physical model is shown to give a reasonable estimate of both the magnitude of diurnal surface warming (model-observation correlation 0.88) and the structure and temporal evolution of the DWL. Novel observations of velocity shear obtained during 346 days at one site, incorporating high-resolution (1 m) upper ocean (5-15 m) acoustic Doppler current profile measurements, are also shown to be in reasonable agreement with estimates from the physical model (daily maximum shear model-observation correlation 0.77). Physics-based improvements to the one-layer model (incorporation of rotation and freshwater terms) are discussed, though they do not provide significant improvements against the observations reported here. The simplicity and limitations of the physical model are used to discuss DWL dynamics. The physical model is shown to give better model performance under the range of forcing conditions experienced across the five sites than the more empirical models. ©2013. American Geophysical Union. All Rights Reserved.

White matter involvement in sporadic Creutzfeldt-Jakob disease.

Science.gov (United States)

Caverzasi, Eduardo; Mandelli, Maria Luisa; DeArmond, Stephen J; Hess, Christopher P; Vitali, Paolo; Papinutto, Nico; Oehler, Abby; Miller, Bruce L; Lobach, Irina V; Bastianello, Stefano; Geschwind, Michael D; Henry, Roland G

2014-12-01

Sporadic Creutzfeldt-Jakob disease is considered primarily a disease of grey matter, although the extent of white matter involvement has not been well described. We used diffusion tensor imaging to study the white matter in sporadic Creutzfeldt-Jakob disease compared to healthy control subjects and to correlated magnetic resonance imaging findings with histopathology. Twenty-six patients with sporadic Creutzfeldt-Jakob disease and nine age- and gender-matched healthy control subjects underwent volumetric T1-weighted and diffusion tensor imaging. Six patients had post-mortem brain analysis available for assessment of neuropathological findings associated with prion disease. Parcellation of the subcortical white matter was performed on 3D T1-weighted volumes using Freesurfer. Diffusion tensor imaging maps were calculated and transformed to the 3D-T1 space; the average value for each diffusion metric was calculated in the total white matter and in regional volumes of interest. Tract-based spatial statistics analysis was also performed to investigate the deeper white matter tracts. There was a significant reduction of mean (P=0.002), axial (P=0.0003) and radial (P=0.0134) diffusivities in the total white matter in sporadic Creutzfeldt-Jakob disease. Mean diffusivity was significantly lower in most white matter volumes of interest (PCreutzfeldt-Jakob disease. Mean diffusivity reduction reflected concomitant decrease of both axial and radial diffusivity, without appreciable changes in white matter anisotropy. Tract-based spatial statistics analysis showed significant reductions of mean diffusivity within the white matter of patients with sporadic Creutzfeldt-Jakob disease, mainly in the left hemisphere, with a strong trend (P=0.06) towards reduced mean diffusivity in most of the white matter bilaterally. In contrast, by visual assessment there was no white matter abnormality either on T2-weighted or diffusion-weighted images. Widespread reduction in white matter mean

STS-39 Earth observation of Earth's limb at sunset shows atmospheric layers

Science.gov (United States)

1991-01-01

STS-39 Earth observation taken aboard Discovery, Orbiter Vehicle (OV) 103, shows the Earth's limb at sunset with numerous atmospheric scattering layers highlighted. The layers consist of fine particles suspended in very stable layers of the atmosphere. The layers act as a prism for the sunlight.

Modelling the effects of penetrance and family size on rates of sporadic and familial disease.

Science.gov (United States)

Al-Chalabi, Ammar; Lewis, Cathryn M

2011-01-01

Many complex diseases show a diversity of inheritance patterns ranging from familial disease, manifesting with autosomal dominant inheritance, through to simplex families in which only one person is affected, manifesting as apparently sporadic disease. The role of ascertainment bias in generating apparent patterns of inheritance is often overlooked. We therefore explored the role of two key parameters that influence ascertainment, penetrance and family size, in rates of observed familiality. We develop a mathematical model of familiality of disease, with parameters for penetrance, mutation frequency and family size, and test this in a complex disease: amyotrophic lateral sclerosis. Monogenic, high-penetrance variants can explain patterns of inheritance in complex diseases and account for a large proportion of those with no apparent family history. With current demographic trends, rates of familiality will drop further. For example, a variant with penetrance 0.5 will cause apparently sporadic disease in 12% of families of size 10, but 80% of families of size 1. A variant with penetrance 0.9 has only an 11% chance of appearing sporadic in families of a size similar to those of Ireland in the past, compared with 57% in one-child families like many in China. These findings have implications for genetic counselling, disease classification and the design of gene-hunting studies. The distinction between familial and apparently sporadic disease should be considered artificial. Copyright © 2011 S. Karger AG, Basel.

Observation of an unusual mid-stratospheric aerosol layer in the Arctic: possible sources and implications for polar vortex dynamics

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M. Gerding

Full Text Available By the beginning of winter 2000/2001, a mysterious stratospheric aerosol layer had been detected by four different Arctic lidar stations. The aerosol layer was observed first on 16 November 2000, at an altitude of about 38 km near Søndre Strømfjord, Greenland (67° N, 51° W and on 19 November 2000, near Andenes, Norway (69°  N, 16°  E. Subsequently, in early December 2000, the aerosol layer was observed near Kiruna, Sweden (68°  N, 21°  E and Ny-Ålesund, Spitsbergen (79°  N, 12°  E. No mid-latitude lidar station observed the presence of aerosols in this altitude region. The layer persisted throughout the winter 2000/2001, at least up to 12 February 2001. In November 2000, the backscatter ratio at a wavelength of 532 nm was up to 1.1, with a FWHM of about 2.5 km. By early February 2001, the layer had sedimented from an altitude of 38 km to about 26 km. Measurements at several wavelengths by the ALOMAR and Koldewey lidars indicate the particle size was between 30 and 50 nm. Depolarisation measurements reveal that the particles in the layer are aspherical, hence solid. In the mid-stratosphere, the ambient atmospheric temperature was too high to support in situ formation or existence of cloud particles consisting of ice or an acid-water solution. Furthermore, in the year 2000 there was no volcanic eruption, which could have injected aerosols into the upper stratosphere. Therefore, other origins of the aerosol, such as meteoroid debris, condensed rocket fuel, or aerosols produced under the influence of charged solar particles, will be discussed in the paper. Trajectory calculations illustrate the path of the aerosol cloud within the polar vortex and are used to link the observations at the different lidar sites. From the descent rate of  the layer and particle sedimentation rates, the mean down-ward motion of air within the polar vortex was estimated to be about 124 m/d between 35 and 30 km, with higher values at the edge of the

Observation of an unusual mid-stratospheric aerosol layer in the Arctic: possible sources and implications for polar vortex dynamics

Directory of Open Access Journals (Sweden)

M. Gerding

2003-04-01

Full Text Available By the beginning of winter 2000/2001, a mysterious stratospheric aerosol layer had been detected by four different Arctic lidar stations. The aerosol layer was observed first on 16 November 2000, at an altitude of about 38 km near Søndre Strømfjord, Greenland (67° N, 51° W and on 19 November 2000, near Andenes, Norway (69°  N, 16°  E. Subsequently, in early December 2000, the aerosol layer was observed near Kiruna, Sweden (68°  N, 21°  E and Ny-Ålesund, Spitsbergen (79°  N, 12°  E. No mid-latitude lidar station observed the presence of aerosols in this altitude region. The layer persisted throughout the winter 2000/2001, at least up to 12 February 2001. In November 2000, the backscatter ratio at a wavelength of 532 nm was up to 1.1, with a FWHM of about 2.5 km. By early February 2001, the layer had sedimented from an altitude of 38 km to about 26 km. Measurements at several wavelengths by the ALOMAR and Koldewey lidars indicate the particle size was between 30 and 50 nm. Depolarisation measurements reveal that the particles in the layer are aspherical, hence solid. In the mid-stratosphere, the ambient atmospheric temperature was too high to support in situ formation or existence of cloud particles consisting of ice or an acid-water solution. Furthermore, in the year 2000 there was no volcanic eruption, which could have injected aerosols into the upper stratosphere. Therefore, other origins of the aerosol, such as meteoroid debris, condensed rocket fuel, or aerosols produced under the influence of charged solar particles, will be discussed in the paper. Trajectory calculations illustrate the path of the aerosol cloud within the polar vortex and are used to link the observations at the different lidar sites. From the descent rate of  the layer and particle sedimentation rates, the mean down-ward motion of air within the polar vortex was estimated to be about 124 m/d between 35 and 30 km, with higher values at the edge of the

Remote Sensing Observations and Numerical Simulation for Martian Layered Ejecta Craters

Science.gov (United States)

Li, L.; Yue, Z.; Zhang, C.; Li, D.

2018-04-01

To understand past Martian climates, it is important to know the distribution and nature of water ice on Mars. Impact craters are widely used ubiquitous indicators for the presence of subsurface water or ice on Mars. Remote sensing observations and numerical simulation are powerful tools for investigating morphological and topographic features on planetary surfaces, and we can use the morphology of layered ejecta craters and hydrocode modeling to constrain possible layering and impact environments. The approach of this work consists of three stages. Firstly, the morphological characteristics of the Martian layered ejecta craters are performed based on Martian images and DEM data. Secondly, numerical modeling layered ejecta are performed through the hydrocode iSALE (impact-SALE). We present hydrocode modeling of impacts onto targets with a single icy layer within an otherwise uniform basalt crust to quantify the effects of subsurface H2O on observable layered ejecta morphologies. The model setup is based on a layered target made up of a regolithic layer (described by the basalt ANEOS), on top an ice layer (described by ANEOS equation of H2O ice), in turn on top of an underlying basaltic crust. The bolide is a 0.8 km diameter basaltic asteroid hitting the Martian surface vertically at a velocity of 12.8 km/s. Finally, the numerical results are compared with the MOLA DEM profile in order to analyze the formation mechanism of Martian layered ejecta craters. Our simulations suggest that the presence of an icy layer significantly modifies the cratering mechanics, and many of the unusual features of SLE craters may be explained by the presence of icy layers. Impact cratering on icy satellites is significantly affected by the presence of subsurface H2O.

Sporadic simple groups and quotient singularities

International Nuclear Information System (INIS)

Cheltsov, I A; Shramov, C A

2013-01-01

We show that if a faithful irreducible representation of a central extension of a sporadic simple group with centre contained in the commutator subgroup gives rise to an exceptional (resp. weakly exceptional but not exceptional) quotient singularity, then that simple group is the Hall-Janko group (resp. the Suzuki group)

Effects of Alclad Layer and Anodizing Time on Sulfuric Acid Anodizing and Film Properties of 2E12 Aluminum Alloy

OpenAIRE

CHEN Gao-hong; HU Yuan-sen; YU Mei; LIU Jian-hua; LI Guo-ai

2017-01-01

Alclad and unclad 2E12 aerospace aluminum alloy were treated by sulfuric acid anodic oxidation. The effects of alclad layer and anodizing time on the anodization behaviour and corrosion resistance of anodic oxide layer on 2E12 aluminum alloy were studied. Surface and cross-section morphology of anodic oxide films were observed by scanning electron microscopy. The electrochemical properties of anodic oxide films were analyzed by potentiodynamic polarization curve and electrochemical impedance ...

Deletion and reduced expression of the Fanconi anemia FANCA gene in sporadic acute myeloid leukemia.

Science.gov (United States)

Tischkowitz, M D; Morgan, N V; Grimwade, D; Eddy, C; Ball, S; Vorechovsky, I; Langabeer, S; Stöger, R; Hodgson, S V; Mathew, C G

2004-03-01

Fanconi anemia (FA) is an autosomal recessive chromosomal instability disorder caused by mutations in one of seven known genes (FANCA,C,D2,E,F,G and BRCA2). Mutations in the FANCA gene are the most prevalent, accounting for two-thirds of FA cases. Affected individuals have greatly increased risks of acute myeloid leukemia (AML). This raises the question as to whether inherited or acquired mutations in FA genes might be involved in the development of sporadic AML. Quantitative fluorescent PCR was used to screen archival DNA from sporadic AML cases for FANCA deletions, which account for 40% of FANCA mutations in FA homozygotes. Four heterozygous deletions were found in 101 samples screened, which is 35-fold higher than the expected population frequency for germline FANCA deletions (PFANCA in the AML samples with FANCA deletions did not detect mutations in the second allele and there was no evidence of epigenetic silencing by hypermethylation. However, real-time quantitative PCR analysis in these samples showed reduced expression of FANCA compared to nondeleted AML samples and to controls. These findings suggest that gene deletions and reduced expression of FANCA may be involved in the promotion of genetic instability in a subset of cases of sporadic AML.

The vertical structure of the Saharan boundary layer: Observations and modelling

Science.gov (United States)

Garcia-Carreras, L.; Parker, D. J.; Marsham, J. H.; Rosenberg, P.; Marenco, F.; Mcquaid, J.

2012-04-01

The vertical structure of the Saharan atmospheric boundary layer (SABL) is investigated with the use of aircraft data from the Fennec observational campaign, and high-resolution large-eddy model (LEM) simulations. The SABL is one of the deepest on Earth, and crucial in controlling the vertical redistribution and long-range transport of dust in the Sahara. The SABL is typically made up of an actively growing convective region driven by high sensible heating at the surface, with a deep, near-neutrally stratified Saharan residual layer (SRL) above it, which is mostly well mixed in humidity and temperature and reaches a height of ~500hPa. These two layers are usually separated by a weak (≤1K) temperature inversion, making the vertical structure very sensitive to the surface fluxes. Large-eddy model (LEM) simulations initialized with radiosonde data from Bordj Bardji Mokhtar (BBM), southern Algeria, are used to improve our understanding of the turbulence structure of the stratification of the SABL, and any mixing or exchanges between the different layers. The model can reproduce the typical SABL structure from observations, and a tracer is used to illustrate the growth of the convective boundary layer into the residual layer above. The heat fluxes show a deep entrainment zone between the convective region and the SRL, potentially enhanced by the combination of a weak lid and a neutral layer above. The horizontal variability in the depth of the convective layer was also significant even with homogeneous surface fluxes. Aircraft observations from a number of flights are used to validate the model results, and to highlight the variability present in a more realistic setting, where conditions are rarely homogeneous in space. Stacked legs were performed to get an estimate of the mean flux profile of the boundary layer, as well as the variations in the vertical structure of the SABL with heterogeneous atmospheric and surface conditions. Regular radiosondes from BBM put

Some aspects of metallic ion chemistry and dynamics in the mesosphere and thermosphere

Science.gov (United States)

Mathews, J. D.

1987-01-01

The relationship between the formation of sporadic layers of metallic ion and the dumping of these ions into the upper mesosphere is discussed in terms of the tidal wind, classical (i.e., windshear) and other more complex, perhaps highly nonlinear layer formation mechanisms, and a possible circulation mechanism for these ions. Optical, incoherent scatter radar, rocket, and satellite derived evidence for various layer formation mechanisms and for the metallic ion circulation system is reviewed. The results of simple one dimensional numerical model calculations of sporadic E and intermediate layer formation are presented along with suggestions for more advanced models of intense or blanketing sporadic E. The flux of metallic ions dumped by the tidal wind system into the mesosphere is estimated and compared with estimates of total particle flux of meteoric origin. Possible effects of the metallic ion flux and of meteoric dust on D region ion chemistry are discussed.

Tracking atmospheric boundary layer dynamics with water vapor D-excess observations

KAUST Repository

Parkes, Stephen

2015-04-01

Stable isotope water vapor observations present a history of hydrological processes that have impacted on an air mass. Consequently, there is scope to improve our knowledge of how different processes impact on humidity budgets by determining the isotopic end members of these processes and combining them with in-situ water vapor measurements. These in-situ datasets are still rare and cover a limited geographical expanse, so expanding the available data can improve our ability to define isotopic end members and knowledge about atmospheric humidity dynamics. Using data collected from an intensive field campaign across a semi-arid grassland site in eastern Australia, we combine multiple methods including in-situ stable isotope observations to study humidity dynamics associated with the growth and decay of the atmospheric boundary layer and the stable nocturnal boundary layer. The deuterium-excess (D-excess) in water vapor is traditionally thought to reflect the sea surface temperature and relative humidity at the point of evaporation over the oceans. However, a number of recent studies suggest that land-atmosphere interactions are also important in setting the D-excess of water vapor. These studies have shown a highly robust diurnal cycle for the D-excess over a range of sites that could be exploited to better understand variations in atmospheric humidity associated with boundary layer dynamics. In this study we use surface radon concentrations as a tracer of surface layer dynamics and combine these with the D-excess observations. The radon concentrations showed an overall trend that was inversely proportional to the D-excess, with early morning entrainment of air from the residual layer of the previous day both diluting the radon concentration and increasing the D-excess, followed by accumulation of radon at the surface and a decrease in the D-excess as the stable nocturnal layer developed in the late afternoon and early evening. The stable nocturnal boundary layer

Transarterial ethanol ablation for sporadic and non-hemorrhaging angiomyolipoma in the kidney

International Nuclear Information System (INIS)

Takebayashi, Shigeo; Horikawa, Ayumi; Arai, Mito; Iso, Shinichiroh; Noguchi, Kazumi

2009-01-01

Purpose: We evaluated the efficacy and side effects of transarterial ethanol ablation in sporadic and non-hemorrhaging angiomyolipomas (AMLs) in the kidney. Material and Methods: A total of 10 patients with solitary and sporadic AMLs underwent selective transarterial absolute ethanol ablation for prophylaxis against hemorrhage. We confirmed the ratio areas of tumor vessel on angiogram, those of infraction on post-ablation computed tomography (CT) and those of tumor reduction in a 3-, 6- and 12-month follow-up CT. Results: Once or twice a single infusion of 1 or 2 ml absolute ethanol achieved in a total occlusion of 22 feeding arteries which consisted of 7 proximal interlobar arteries, 12 distal interlobar arteries and 3 renal capsular arteries. Nontarget occlusion did not occur by ethanol reflux in any cases but occurred causing spasms provoked by repeated inflation and deflation of the balloon in one case. Total occlusion of tumor vessels was observed in 7 patients and 92-95% occlusion in 3. Ethanol ablation produced 1.8-22.5% (mean 8.4 ± 6.8%) areas of infarctions but the outcome was not serious in all cases. Mean percentage areas of tumor reduction were 29.4 ± 10.6% in a 3-month follow-up, 45.7 ± 11.9% in a 6-month and 59.3 ± 11.5% in a 12-month follow-up. Conclusions: Absolute ethanol ablation for sporadic and non-hemorrhaging AML is safe and effective in reducing majority of tumor area in a 1-year follow-up.

Electromagnetic internal gravity waves in the Earth's ionospheric E-layer

International Nuclear Information System (INIS)

Kaladze, T.D.; Tsamalashvili, L.V.; Kaladze, D.T.

2011-01-01

In the Earth's ionospheric E-layer existence of the new waves connecting with the electromagnetic nature of internal gravity waves is shown. They represent the mixture of the ordinary internal gravity waves and the new type of dispersive Alfven waves. -- Highlights: ► Existence of electromagnetic internal gravity waves in the ionospheric E-layer is shown. ► Electromagnetic nature of internal gravity waves is described. ► Appearance of the new dispersive Alfven waves is shown.

Effects of Alclad Layer and Anodizing Time on Sulfuric Acid Anodizing and Film Properties of 2E12 Aluminum Alloy

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CHEN Gao-hong

2017-07-01

Full Text Available Alclad and unclad 2E12 aerospace aluminum alloy were treated by sulfuric acid anodic oxidation. The effects of alclad layer and anodizing time on the anodization behaviour and corrosion resistance of anodic oxide layer on 2E12 aluminum alloy were studied. Surface and cross-section morphology of anodic oxide films were observed by scanning electron microscopy. The electrochemical properties of anodic oxide films were analyzed by potentiodynamic polarization curve and electrochemical impedance spectroscopy. The results show that the protective anodic oxide layers are formed on alclad and unclad 2E12 aluminum alloy. The film thickness increases with anodizing time extending. The copper rich second phase particles lead to more cavity defects and even micro cracks on anodic oxide films of unclad 2E12 aluminum alloy. The anodic oxide films on alclad 2E12 aluminum alloy are thicker and have fewer cavity defects, resulting in better corrosion resistance. The films obtained after 30min and 45min anodic oxidation treatment exhibit lower corrosion current and higher impedance of the porous layer than other anodizing time.

Generation of an integration-free induced pluripotent stem cell line (CSC-43 from a patient with sporadic Parkinson's disease

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Ana Marote

2018-03-01

Full Text Available An induced pluripotent stem cell (iPSC line was generated from a 36-year-old patient with sporadic Parkinson's disease (PD. Skin fibroblasts were reprogrammed using the non-integrating Sendai virus technology to deliver OCT3/4, SOX2, c-MYC and KLF4 factors. The generated cell line (CSC-43 exhibits expression of common pluripotency markers, in vitro differentiation into three germ layers and normal karyotype. This iPSC line can be used to study the mechanisms underlying the development of PD.

Early Detection of Sporadic Pancreatic Cancer

Science.gov (United States)

Kenner, Barbara J.; Chari, Suresh T.; Cleeter, Deborah F.; Go, Vay Liang W.

2015-01-01

Abstract Innovation leading to significant advances in research and subsequent translation to clinical practice is urgently necessary in early detection of sporadic pancreatic cancer. Addressing this need, the Early Detection of Sporadic Pancreatic Cancer Summit Conference was conducted by Kenner Family Research Fund in conjunction with the 2014 American Pancreatic Association and Japan Pancreas Society Meeting. International interdisciplinary scientific representatives engaged in strategic facilitated conversations based on distinct areas of inquiry: Case for Early Detection: Definitions, Detection, Survival, and Challenges; Biomarkers for Early Detection; Imaging; and Collaborative Studies. Ideas generated from the summit have led to the development of a Strategic Map for Innovation built upon 3 components: formation of an international collaborative effort, design of an actionable strategic plan, and implementation of operational standards, research priorities, and first-phase initiatives. Through invested and committed efforts of leading researchers and institutions, philanthropic partners, government agencies, and supportive business entities, this endeavor will change the future of the field and consequently the survival rate of those diagnosed with pancreatic cancer. PMID:25938853

Magnetic resonance spectroscopic abnormalities in sporadic and variant Creutzfeldt-Jakob disease

International Nuclear Information System (INIS)

Pandya, H.G.; Coley, S.C.; Wilkinson, I.D.; Griffiths, P.D.

2003-01-01

AIM: To study the proton MR spectroscopic findings in Creutzfeldt-Jakob disease (CJD) (sporadic and variant). MATERIALS AND METHODS: MR imaging and proton MR spectra were acquired in two patients with sporadic CJD (biopsy proven) and one patient with variant CJD. RESULTS: The two patients with sporadic CJD demonstrated MR signal change within the basal ganglia and thalami and reduced N-acetylaspartate (NAA):creatine ratios. The patient with variant CJD showed characteristic signal change within the pulvinar of the thalami and a markedly reduced N-acetylaspartate:creatine ratio. CONCLUSION: All three patients with CJD demonstrated evidence of reduced N-acetylaspartate: creatine ratios on MR spectroscopy. These changes imply that neuronal loss and/or dysfunction is a consistent finding in established CJD. Pandya H. G., et al (2003) Clinical Radiology58, 148--153

Magnetic resonance spectroscopic abnormalities in sporadic and variant Creutzfeldt-Jakob disease

Energy Technology Data Exchange (ETDEWEB)

Pandya, H.G.; Coley, S.C.; Wilkinson, I.D.; Griffiths, P.D

2003-02-01

AIM: To study the proton MR spectroscopic findings in Creutzfeldt-Jakob disease (CJD) (sporadic and variant). MATERIALS AND METHODS: MR imaging and proton MR spectra were acquired in two patients with sporadic CJD (biopsy proven) and one patient with variant CJD. RESULTS: The two patients with sporadic CJD demonstrated MR signal change within the basal ganglia and thalami and reduced N-acetylaspartate (NAA):creatine ratios. The patient with variant CJD showed characteristic signal change within the pulvinar of the thalami and a markedly reduced N-acetylaspartate:creatine ratio. CONCLUSION: All three patients with CJD demonstrated evidence of reduced N-acetylaspartate: creatine ratios on MR spectroscopy. These changes imply that neuronal loss and/or dysfunction is a consistent finding in established CJD. Pandya H. G., et al (2003) Clinical Radiology58, 148--153.

The intermediate layers and associated tidal motions observed by a digisonde in the equatorial anomaly region

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C.-C. Lee

2003-04-01

Full Text Available This investigation presents an initial attempt to analyze a full year of daily ionosonde observations relevant to the determination of plasma densities, tidal structures, and ion transports in the equatorial anomaly region of the lower ionosphere. Particular focus is on the intermediate layers, their seasonal and diurnal variations, and cause-effect relationships. The ionogram database was recorded using a digisonde portable sounder (DPS at National Central University (NCU, 24 °58' N, 121°11' E during 1996. Statistical results indicate that the intermediate layers appear primarily during the daytime of the spring/winter months. The monthly median height characteristics reveal that the layers descend from high to low altitudes and different tidal motions control the layers in different months. Generally, the semi-diurnal and quarter-diurnal tides, which cause ionization convergence, are mainly in the spring/winter and summer/autumn months, respectively. Variations in the electron densities of the layers also indicate that the density increases could result from a great number of molecular and metallic ions. Furthermore, a novel approach to ionogram presentation is introduced to investigate the intermediate layers. This approach allows the DPS to characterize the detailed daily information of the intermediate layers.Key words. Ionosphere (equatorial ionosphere; ionosphere-atmosphere interactions; ionospheric irregularities

Multi-Instrument Observations of Prolonged Stratified Wind Layers at Iqaluit, Nunavut

Science.gov (United States)

Mariani, Zen; Dehghan, Armin; Gascon, Gabrielle; Joe, Paul; Hudak, David; Strawbridge, Kevin; Corriveau, Julien

2018-02-01

Data collected between October 2015 and May 2016 at Environment and Climate Change Canada's Iqaluit research site (64°N, 69°W) have revealed a high frequency (40% of all days for which observations were available) of stratified wind layer events that occur from near the surface up to about 7.2 km above sea level. These stratified wind layers are clearly visible as wind shifts (90 to 180°) with height in range-height indicator scans from the Doppler lidar and Ka-band radar and in wind direction profiles from the Doppler lidar and radiosonde. During these events, the vertical structure of the flow appears to be a stack of 4 to 10 layers ranging in vertical width from 0.1 to 4.4 km. The stratification events that were observed occurred predominantly (81%) during light precipitation and lasted up to 27.5 h. The integrated measurement platforms at Iqaluit permitted continuous observations of the evolution of stratification events in different meteorological conditions.

Cervical dystonia: about familial and sporadic cases in 88 patients

Directory of Open Access Journals (Sweden)

Carlos Henrique F. Camargo

2014-02-01

Full Text Available Cervical dystonia (CD affects the musculature of the neck in a focal way or associated to other parts of the body. The aim of this study was to identify clinical differences between patients with dystonia patients without family history and with family history (sporadic. Eighty-eight patients with CD were recruited in a Movement Disorders Clinic between June of 2008 and June of 2009. Only patients with no etiological diagnosis were accepted for analysis. The age of onset of symptoms was later in patients with focal and segmental dystonia than in patients with generalized dystonia (p<0.001. The severity of symptoms was higher in patients with sporadic dystonia than in familial patients (p<0.01. Generalized cases were more severe in patients with a family history (p<0.01. Sporadic patients had higher levels of pain than familial cases (p<0.05. We expect soon to present the results of genetic analyzes of these patients.

Spray layer-by-layer films based on phospholipid vesicles aiming sensing application via e-tongue system

International Nuclear Information System (INIS)

Aoki, P.H.B.; Volpati, D.; Cabrera, F.C.; Trombini, V.L.; Riul, A.; Constantino, C.J.L.

2012-01-01

The Layer-by-Layer (LbL) technique via spraying (spray-LbL) has been applied as new and alternative methodology to fabricate ultrathin films due to its versatility in relation to the conventional dipping-LbL method, mainly in terms of faster layer deposition and larger coated area. In this work, the possibility of immobilizing vesicles of dipalmitoyl phosphatidyl glycerol (DPPG) phospholipid onto alternating layers of the polyelectrolyte poly(allylamine hydrochloride) (PAH) using the spray-LbL method was investigated, being the results compared to the conventional dipping-LbL method. The growth of (PAH/DPPG) n spray-LbL films was systematically monitored by quartz crystal microbalance (QCM) and ultraviolet–visible (UV–vis) absorption spectroscopy, revealing a linear increase of the absorbance vs deposited layers. In relation to a possible electrostatic interaction between the groups PO 4 − (DPPG) and NH 3 + (PAH), it was observed through Fourier transform infrared (FTIR) absorption spectroscopy that the spectrum recorded for the spray-LbL film is basically a simple superposition of the FTIR spectra from PAH and DPPG casting films. The latter indicates a weak interaction between both materials, differently of the trend observed for (PAH/DPPG) n grown via dipping-LbL method. Atomic force microscopy (AFM) images of spray-LbL films showed evidences that the DPPG vesicles present in the aqueous dispersion are not destroyed when submitted to pressure conditions during the spray deposition. However, comparing to dipping-LbL, the DPPG vesicles do not cover completely the PAH layer for the spray-LbL film, which was further confirmed by surface-enhanced Raman scattering (SERS) measurements. Moreover, the AFM analysis showed that the spray-LbL deposition led to thicker PAH/DPPG bilayers in average than via dipping-LbL for the same concentrations of PAH solution and DPPG dispersion, which is consistent with QCM and UV–vis absorption results. PAH/DPPG films deposited by

Using long-term ARM observations to evaluate Arctic mixed-phased cloud representation in the GISS ModelE GCM

Science.gov (United States)

Lamer, K.; Fridlind, A. M.; Luke, E. P.; Tselioudis, G.; Ackerman, A. S.; Kollias, P.; Clothiaux, E. E.

2016-12-01

The presence of supercooled liquid in clouds affects surface radiative and hydrological budgets, especially at high latitudes. Capturing these effects is crucial to properly quantifying climate sensitivity. Currently, a number of CGMs disagree on the distribution of cloud phase. Adding to the challenge is a general lack of observations on the continuum of clouds, from high to low-level and from warm to cold. In the current study, continuous observations from 2011 to 2014 are used to evaluate all clouds produced by the GISS ModelE GCM over the ARM North Slope of Alaska site. The International Satellite Cloud Climatology Project (ISCCP) Global Weather State (GWS) approach reveals that fair-weather (GWS 7, 32% occurrence rate), as well as mid-level storm related (GWS 5, 28%) and polar (GWS 4, 14%) clouds, dominate the large-scale cloud patterns at this high latitude site. At higher spatial and temporal resolutions, ground-based cloud radar observations reveal a majority of single layer cloud vertical structures (CVS). While clear sky and low-level clouds dominate (each with 30% occurrence rate) a fair amount of shallow ( 10%) to deep ( 5%) convection are observed. Cloud radar Doppler spectra are used along with depolarization lidar observations in a neural network approach to detect the presence, layering and inhomogeneity of supercooled liquid layers. Preliminary analyses indicate that most of the low-level clouds sampled contain one or more supercooled liquid layers. Furthermore, the relationship between CVS and the presence of supercooled liquid is established, as is the relationship between the presence of supercool liquid and precipitation susceptibility. Two approaches are explored to bridge the gap between large footprint GCM simulations and high-resolution ground-based observations. The first approach consists of comparing model output and ground-based observations that exhibit the same column CVS type (i.e. same cloud depth, height and layering

New application of intelligent agents in sporadic amyotrophic lateral sclerosis identifies unexpected specific genetic background

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Marocchi Alessandro

2008-05-01

Full Text Available Abstract Background Few genetic factors predisposing to the sporadic form of amyotrophic lateral sclerosis (ALS have been identified, but the pathology itself seems to be a true multifactorial disease in which complex interactions between environmental and genetic susceptibility factors take place. The purpose of this study was to approach genetic data with an innovative statistical method such as artificial neural networks to identify a possible genetic background predisposing to the disease. A DNA multiarray panel was applied to genotype more than 60 polymorphisms within 35 genes selected from pathways of lipid and homocysteine metabolism, regulation of blood pressure, coagulation, inflammation, cellular adhesion and matrix integrity, in 54 sporadic ALS patients and 208 controls. Advanced intelligent systems based on novel coupling of artificial neural networks and evolutionary algorithms have been applied. The results obtained have been compared with those derived from the use of standard neural networks and classical statistical analysis Results Advanced intelligent systems based on novel coupling of artificial neural networks and evolutionary algorithms have been applied. The results obtained have been compared with those derived from the use of standard neural networks and classical statistical analysis. An unexpected discovery of a strong genetic background in sporadic ALS using a DNA multiarray panel and analytical processing of the data with advanced artificial neural networks was found. The predictive accuracy obtained with Linear Discriminant Analysis and Standard Artificial Neural Networks ranged from 70% to 79% (average 75.31% and from 69.1 to 86.2% (average 76.6% respectively. The corresponding value obtained with Advanced Intelligent Systems reached an average of 96.0% (range 94.4 to 97.6%. This latter approach allowed the identification of seven genetic variants essential to differentiate cases from controls: apolipoprotein E arg

New application of intelligent agents in sporadic amyotrophic lateral sclerosis identifies unexpected specific genetic background.

Science.gov (United States)

Penco, Silvana; Buscema, Massimo; Patrosso, Maria Cristina; Marocchi, Alessandro; Grossi, Enzo

2008-05-30

Few genetic factors predisposing to the sporadic form of amyotrophic lateral sclerosis (ALS) have been identified, but the pathology itself seems to be a true multifactorial disease in which complex interactions between environmental and genetic susceptibility factors take place. The purpose of this study was to approach genetic data with an innovative statistical method such as artificial neural networks to identify a possible genetic background predisposing to the disease. A DNA multiarray panel was applied to genotype more than 60 polymorphisms within 35 genes selected from pathways of lipid and homocysteine metabolism, regulation of blood pressure, coagulation, inflammation, cellular adhesion and matrix integrity, in 54 sporadic ALS patients and 208 controls. Advanced intelligent systems based on novel coupling of artificial neural networks and evolutionary algorithms have been applied. The results obtained have been compared with those derived from the use of standard neural networks and classical statistical analysis Advanced intelligent systems based on novel coupling of artificial neural networks and evolutionary algorithms have been applied. The results obtained have been compared with those derived from the use of standard neural networks and classical statistical analysis. An unexpected discovery of a strong genetic background in sporadic ALS using a DNA multiarray panel and analytical processing of the data with advanced artificial neural networks was found. The predictive accuracy obtained with Linear Discriminant Analysis and Standard Artificial Neural Networks ranged from 70% to 79% (average 75.31%) and from 69.1 to 86.2% (average 76.6%) respectively. The corresponding value obtained with Advanced Intelligent Systems reached an average of 96.0% (range 94.4 to 97.6%). This latter approach allowed the identification of seven genetic variants essential to differentiate cases from controls: apolipoprotein E arg158cys; hepatic lipase -480 C/T; endothelial

Genetic Relatedness among Nontypeable Pneumococci Implicated in Sporadic Cases of Conjunctivitis

OpenAIRE

Barker, Jason H.; Musher, Daniel M.; Silberman, Ronald; Phan, Hoang M.; Watson, David A.

1999-01-01

Nontypeable Streptococcus pneumoniae is a common cause of epidemic conjunctivitis. A previous molecular fingerprinting study identified a clone of nontypeable pneumococcus that was responsible for a recent outbreak of conjunctivitis. In the present study, we examined the extent to which pneumococci that cause sporadic cases of conjunctivitis are related to this epidemic strain. Using arbitrarily primed BOX-PCR, we have determined that, of 10 nontypeable pneumococci causing sporadic conjunctiv...

Difference in aneurysm characteristics between patients with familial and sporadic aneurysmal subarachnoid haemorrhage

NARCIS (Netherlands)

Mensing, Liselore A.; Rinkel, Gabriel J E; Vlak, Monique H M; Van Der Schaaf, Irene C.; Ruigrok, Ynte M.

2016-01-01

Object Patients with familial intracranial aneurysms (IA) have a higher risk of rupture than patients with sporadic IA. We compared geometric and morphological risk factors for aneurysmal rupture between patients with familial and sporadic aneurysmal subarachnoid hemorrhage (aSAH) to analyse if

Analysis of euv limb-brightening observations from ATM. I. Model for the transition layer and the corona

Energy Technology Data Exchange (ETDEWEB)

Mariska, J T; Withbroe, G L [Harvard Coll. Observatory, Cambridge, Mass. (USA)

1975-09-01

Limb-brightening curves for euv resonance lines of O VI and Mg X have been constructed from spectroheliograms (5 sec resolution) of quiet limb regions observed with the Harvard experiment on Skylab. The observations are interpreted with a simple model for the transition layer and the corona. A comparison of theoretical and observed limb-brightening curves indicates that the lower boundary of the corona, where T/sub e/ = 10/sup 6/K, is at a height of about 8000 km in typical quiet areas. For 1.01 R(sun) approximately = to or < r < 1.25(sun), the corona can be represented by a homogeneous model in hydrostatic equilibrium with a temperature of 10/sup 6/K for 1.01 R(sun) approximately = to or < r < 1.1 R(sun) and 1.1x10/sup 6/K for r > approximately = to 1.1 R(sun). The model for the transition layer is inhomogeneous, with the temperature gradient a factor of 3 shallower in the network than in the intranetwork regions. It appears that spicules should be included in the model in order to account for the penetration into the corona of cool (T/sub e/ < 10/sup 6/K) euv-emitting material to heights up to 20000 km above the limb.

Molecular pathogenesis of sporadic prion diseases in man

Science.gov (United States)

Safar, Jiri G.

2012-01-01

The yeast, fungal and mammalian prions determine heritable and infectious traits that are encoded in alternative conformations of proteins. They cause lethal sporadic, familial and infectious neurodegenerative conditions in man, including Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome (GSS), kuru, sporadic fatal insomnia (SFI) and likely variable protease-sensitive prionopathy (VPSPr). The most prevalent of human prion diseases is sporadic (s)CJD. Recent advances in amplification and detection of prions led to considerable optimism that early and possibly preclinical diagnosis and therapy might become a reality. Although several drugs have already been tested in small numbers of sCJD patients, there is no clear evidence of any agent’s efficacy. Therefore, it remains crucial to determine the full spectrum of sCJD prion strains and the conformational features in the pathogenic human prion protein governing replication of sCJD prions. Research in this direction is essential for the rational development of diagnostic as well as therapeutic strategies. Moreover, there is growing recognition that fundamental processes involved in human prion propagation – intercellular induction of protein misfolding and seeded aggregation of misfolded host proteins – are of far wider significance. This insight leads to new avenues of research in the ever-widening spectrum of age-related human neurodegenerative diseases that are caused by protein misfolding and that pose a major challenge for healthcare. PMID:22421210

Generation of a human induced pluripotent stem cell line (CSC-42 from a patient with sporadic form of Parkinson's disease

Directory of Open Access Journals (Sweden)

Ekaterina Savchenko

2018-03-01

Full Text Available Skin fibroblasts were collected from a 44-year-old patient with sporadic case of Parkinson's disease (PD. The non-integrating Sendai virus vector encoding OCT3/4, SOX2, c-MYC and KLF4 was used to reprogram fibroblasts into induced pluripotent stem cells (iPSCs. Generated iPSCs had normal karyotypes, expressed common stem cell markers, and were capable of differentiating into all three germ layers. Generated line could be used for PD modeling to understand the mechanisms that influence the disorder.

An Algorithm to Generate Deep-Layer Temperatures from Microwave Satellite Observations for the Purpose of Monitoring Climate Change. Revised

Science.gov (United States)

Goldberg, Mitchell D.; Fleming, Henry E.

1994-01-01

An algorithm for generating deep-layer mean temperatures from satellite-observed microwave observations is presented. Unlike traditional temperature retrieval methods, this algorithm does not require a first guess temperature of the ambient atmosphere. By eliminating the first guess a potentially systematic source of error has been removed. The algorithm is expected to yield long-term records that are suitable for detecting small changes in climate. The atmospheric contribution to the deep-layer mean temperature is given by the averaging kernel. The algorithm computes the coefficients that will best approximate a desired averaging kernel from a linear combination of the satellite radiometer's weighting functions. The coefficients are then applied to the measurements to yield the deep-layer mean temperature. Three constraints were used in deriving the algorithm: (1) the sum of the coefficients must be one, (2) the noise of the product is minimized, and (3) the shape of the approximated averaging kernel is well-behaved. Note that a trade-off between constraints 2 and 3 is unavoidable. The algorithm can also be used to combine measurements from a future sensor (i.e., the 20-channel Advanced Microwave Sounding Unit (AMSU)) to yield the same averaging kernel as that based on an earlier sensor (i.e., the 4-channel Microwave Sounding Unit (MSU)). This will allow a time series of deep-layer mean temperatures based on MSU measurements to be continued with AMSU measurements. The AMSU is expected to replace the MSU in 1996.

RNF43 is mutated less frequently in Lynch Syndrome compared with sporadic microsatellite unstable colorectal cancers.

Science.gov (United States)

Fennell, Lochlan J; Clendenning, Mark; McKeone, Diane M; Jamieson, Saara H; Balachandran, Samanthy; Borowsky, Jennifer; Liu, John; Kawamata, Futoshi; Bond, Catherine E; Rosty, Christophe; Burge, Matthew E; Buchanan, Daniel D; Leggett, Barbara A; Whitehall, Vicki L J

2018-01-01

The WNT signaling pathway is commonly altered during colorectal cancer development. The E3 ubiquitin ligase, RNF43, negatively regulates the WNT signal through increased ubiquitination and subsequent degradation of the Frizzled receptor. RNF43 has recently been reported to harbor frequent truncating frameshift mutations in sporadic microsatellite unstable (MSI) colorectal cancers. This study assesses the relative frequency of RNF43 mutations in hereditary colorectal cancers arising in the setting of Lynch syndrome. The entire coding region of RNF43 was Sanger sequenced in 24 colorectal cancers from 23 patients who either (i) carried a germline mutation in one of the DNA mismatch repair genes (MLH1, MSH6, MSH2, PMS2), or (ii) showed immunohistochemical loss of expression of one or more of the DNA mismatch repair proteins, was BRAF wild type at V600E, were under 60 years of age at diagnosis, and demonstrated no promoter region methylation for MLH1 in tumor DNA. A validation cohort of 44 colorectal cancers from mismatch repair germline mutation carriers from the Australasian Colorectal Cancer Family Registry (ACCFR) were sequenced for the most common truncating mutation hotspots (X117 and X659). RNF43 mutations were found in 9 of 24 (37.5%) Lynch syndrome colorectal cancers. The majority of mutations were frameshift deletions in the G659 G7 repeat tract (29%); 2 cancers (2/24, 8%) from the one patient harbored frameshift mutations at codon R117 (C6 repeat tract) within exon 3. In the ACCFR validation cohort, RNF43 hotspot mutations were identified in 19/44 (43.2%) of samples, which was not significantly different to the initial series. The proportion of mutant RNF43 in Lynch syndrome related colorectal cancers is significantly lower than the previously reported mutation rate found in sporadic MSI colorectal cancers. These findings identify further genetic differences between sporadic and hereditary colorectal cancers. This may be because Lynch Syndrome cancers

UAV-borne coherent doppler lidar for marine atmospheric boundary layer observations

Science.gov (United States)

Wu, Songhua; Wang, Qichao; Liu, Bingyi; Liu, Jintao; Zhang, Kailin; Song, Xiaoquan

2018-04-01

A compact UAV-borne Coherent Doppler Lidar (UCDL) has been developed at the Ocean University of China for the observation of wind profile and boundary layer structure in Marine Atmospheric Boundary Layer (MABL). The design, specifications and motion-correction methodology of the UCDL are presented. Preliminary results of the first flight campaign in Hailing Island in December 2016 is discussed.

PRKAG3 polymorphisms associated with sporadic Wolff–Parkinson–White syndrome among a Taiwanese population

Directory of Open Access Journals (Sweden)

Ken-Pen Weng

2016-12-01

Conclusion: This study shows that PRKAG3-230 may be associated with sporadic WPW syndrome among a Taiwanese population. Further studies are warranted to elucidate the role of mutations in AMPK subunit genes other than PRKAG3-230 in sporadic WPW syndrome.

Case Studies in e-RPL and e-PR

Science.gov (United States)

Cameron, Roslyn; Miller, Allison

2014-01-01

The use of ePortfolios for recognition of prior learning (e-RPL) and for professional recognition (e-PR) is slowly gaining in popularity in the VET sector however their use is sporadic across educational sectors, disciplines, educational institutions and professions. Added to this is an array of purposes and types of e-RPL and e-PR models and…

The genetics of radiation-induced and sporadic osteosarcoma: a unifying theory?

International Nuclear Information System (INIS)

Rosemann, Michael; Kuosaite, Virginija; Nathrath, Michaela; Atkinson, Michael J.

2002-01-01

Cancer is a disease of the genome, with the neoplastic phenotype being passed from one cell generation to the other. Radiation-induced cancer has often been considered to represent a unique entity amongst neoplasia, with the energy deposition being held responsible for both direct (gene mutations) and indirect (bystander effects, induced instability etc) alterations to the cellular genome. However, radiogenic tumours in man and experimental animals appear to be physiologically and genetically indistinguishable from their sporadic counterparts, suggesting that the aetiologies of these two tumour types are in fact closely related. We have conducted a general screen of the genetic alterations in radiation-induced mouse osteosarcoma, a tumour that is histopathologically indistinguishable from human sporadic osteosarcoma. Comparison of the two tumour types indicates the existence of a common set of genetic changes, providing additional evidence to support the concept that the molecular pathology of radiation-induced malignancy is no different to that of sporadic cancers. (author)

Equilibrium and stability properties of relativistic electron rings and E-layers

International Nuclear Information System (INIS)

Uhm, H.

1976-01-01

Equilibrium and stability properties of magnetically confined partially-neutralized thin electron ring and E-layer are investigated using the Vlasov-Maxwell equations. The analysis is carried out within the context of the assumption that the minor dimensions (a,b) of the system are much less than the collisionless skin depth (c/antiω/sub p/). The equilibrium configuration of the E-layer is assumed to be an infinitely long, azimuthally symmetric hollow electron beam which is aligned parallel to a uniform axial magnetic field. On the other hand, the electron ring is located at the midplane of an externally imposed mirror field which acts to confine the ring both axially and radially. The equilibrium properties of the E-layer and electron ring are obtained self-consistently for several choices of equilibrium electron distribution function. The negative-mass instability analysis is carried out for the relativistic E-layer equilibrium in which all of the electrons have the same transverse energy and a spread in canonical angular momentum, assuming a fixed ion background. The ion resonance instability properties are investigated for a relativistic nonneutral E-layer aligned parallel to a uniform magnetic field and located between two ground coaxial cylindrical conductors. The stability properties of a nonrelativistic electron ring is investigated within the framework of the linearized Vlasov-Poisson equations. The dispersion relation is obtained for the self-consistent electron distribution function in which all electrons have the same value of energy an the same value of canonical angular momentum. The positive ions in the electron ring are assumed to form an immobile partially neutralizing background. The stability criteria as well as the instability growth rates are derived and discussed including the effect of geometrical configuration of the system. Equilibrium space-charge effects play a significant role in stability behavior

Comparing Sporadic and Outbreak-associated Foodborne Illness

Centers for Disease Control (CDC) Podcasts

2016-11-04

Dr. Eric Ebel, a veterinarian and risk analyst with USDA’s Food Safety and Inspection Service, discusses his article on sporadic and outbreak-associated cases of foodborne illness.  Created: 11/4/2016 by National Center for Emerging and Zoonotic Infectious Diseases (NCEZID).   Date Released: 11/4/2016.

Brain sonography in African infants with complicated sporadic ...

African Journals Online (AJOL)

Background: To determine the structural findings in brain sonography of African infants with complicated sporadic bacterial meningitis. Materials and Methods: Retrospective assessment of medical records of patients who underwent brain sonography on account of complicated bacterial meningitis. The brain sonography ...

Insights into the nature of northwest-to-southeast aligned ionospheric wavefronts from contemporaneous Very Large Array and ionosonde observations

Science.gov (United States)

Helmboldt, J. F.

2012-07-01

The results of contemporaneous summer nighttime observations of midlatitude medium scale traveling ionospheric disturbances (MSTIDs) with the Very Large Array (VLA) in New Mexico and nearby ionosondes in Texas and Colorado are presented. Using 132, 20-minute observations, several instances of MSTIDs were detected, all having wavefronts aligned northwest to southeast and mostly propagating toward the southwest, consistent with previous studies of MSTIDs. However, some were also found to move toward the northeast. It was found that both classes of MSTIDs were only found when sporadic-E (Es) layers of moderate peak density (1.5​ foEs​ foEs > ​3 MHz that was not present when 1.5​ foEs < ​3 MHz. No MSTIDs were observed either before midnight or when the F-region height was increasing at a relatively high rate, even when these Es layers were observed. Combining this result with AE indices which were relatively high at the time (an average of about 300 nT and maximum of nearly 700 nT), it is inferred that both the lack of MSTIDs and the increase in F-region height are due to substorm-induced electric fields. The northeastward-directed MSTIDs were strongest post-midnight during times when the F-region was observed to be collapsing relatively quickly. This implies that these two occurrences are related and likely both caused by rare shifts in F-region neutral wind direction from southwest to northwest.

Specific deficit of colour-colour short-term memory binding in sporadic and familial Alzheimer's disease.

Science.gov (United States)

Parra, Mario A; Sala, Sergio Della; Abrahams, Sharon; Logie, Robert H; Méndez, Luis Guillermo; Lopera, Francisco

2011-06-01

Short-term memory binding of visual features which are processed across different dimensions (shape-colour) is impaired in sporadic Alzheimer's disease, familial Alzheimer's disease, and in asymptomatic carriers of familial Alzheimer's disease. This study investigated whether Alzheimer's disease also impacts on within-dimension binding processes. The study specifically explored whether visual short-term memory binding of features of the same type (colour-colour) is sensitive to Alzheimer's disease. We used a neuropsychological battery and a short-term memory binding task to assess patients with sporadic Alzheimer's disease (Experiment 1), familial Alzheimer's disease (Experiment 2) due to the mutation E280A of the Presenilin-1 gene and asymptomatic carriers of the mutation. The binding task assessed change detection within arrays of unicoloured objects (Colour Only) or bicoloured objects the colours of which had to be remembered separately (Unbound Colours) or together (Bound Colours). Performance on the Bound Colours condition (1) explained the largest proportion of variance between patients (sporadic and familial Alzheimer's disease), (2) combined more sensitivity and specificity for the disease than other more traditional neuropsychological tasks, (3) identified asymptomatic carriers of the mutation even when traditional neuropsychological measures and other measures of short-term memory did not and, (4) contrary to shape-colour binding, correlated with measures of hippocampal functions. Colour-colour binding and shape-colour binding both appear to be sensitive to AD even though they seem to rely on different brain mechanisms. Copyright © 2011 Elsevier Ltd. All rights reserved.

Three male patients with sporadic acute hepatitis E in Sendai, Japan, who were domestically infected with hepatitis E virus of genotype III or IV.

Science.gov (United States)

Yamamoto, Takeshi; Suzuki, Hiroshi; Toyota, Takayoshi; Takahashi, Masaharu; Okamoto, Hiroaki

2004-01-01

Recent studies indicate that hepatitis E virus (HEV) infection occurs not only in developing countries but also in industrialized nations. However, the characteristics of domestic infections of hepatitis E in Japan are not fully understood. We analyzed serum samples from 34 patients who were seen at a city hospital in Sendai, Japan, between January 1997 and December 2002, and who had been given the diagnosis of sporadic acute hepatitis of non-A, non-B, non-C etiology. Among these 34 patients, 3 (9%; all men; aged 54, 59, and 61 years) were positive for both IgG and IgM anti-HEV antibodies and for HEV RNA. The HEV isolates (HE-JAS1 and HE-JAS3) obtained from case 1 and case 3, respectively, segregated into genotype III; they had the highest nucleotide sequence identity, of 99.5% and 99.0%, with HE-JA7 and HE-JA8, respectively, both of which had been isolated in Iwate, a neighboring prefecture of Sendai. In contrast, the remaining HEV isolate (HE-JAS2), obtained from case 2, segregated into genotype IV; it had the highest nucleotide sequence identity, of 99.8% and 99.3%, with JKK-Sap and HE-JA3, respectively, both of which had been isolated in Hokkaido, Japan, although case 2 had never been to Hokkaido. Our three patients with hepatitis E had not traveled abroad in the preceding 1 year, had had no contact with pigs, and no history of blood transfusion. These results indicate that HEV should be considered as an etiological agent of acute hepatitis of non-A, non-B, non-C etiology in Japan. The risk factor(s) for acquiring domestic HEV infection in Japan needs to be clarified in future studies.

Left frontal hub connectivity delays cognitive impairment in autosomal-dominant and sporadic Alzheimer’s disease

Science.gov (United States)

Franzmeier, Nicolai; Düzel, Emrah; Jessen, Frank; Buerger, Katharina; Levin, Johannes; Duering, Marco; Dichgans, Martin; Haass, Christian; Suárez-Calvet, Marc; Fagan, Anne M; Paumier, Katrina; Benzinger, Tammie; Masters, Colin L; Morris, John C; Perneczky, Robert; Janowitz, Daniel; Catak, Cihan; Wolfsgruber, Steffen; Wagner, Michael; Teipel, Stefan; Kilimann, Ingo; Ramirez, Alfredo; Rossor, Martin; Jucker, Mathias; Chhatwal, Jasmeer; Spottke, Annika; Boecker, Henning; Brosseron, Frederic; Falkai, Peter; Fliessbach, Klaus; Heneka, Michael T; Laske, Christoph; Nestor, Peter; Peters, Oliver; Fuentes, Manuel; Menne, Felix; Priller, Josef; Spruth, Eike J; Franke, Christiana; Schneider, Anja; Kofler, Barbara; Westerteicher, Christine; Speck, Oliver; Wiltfang, Jens; Bartels, Claudia; Araque Caballero, Miguel Ángel; Metzger, Coraline; Bittner, Daniel; Weiner, Michael; Lee, Jae-Hong; Salloway, Stephen; Danek, Adrian; Goate, Alison; Schofield, Peter R; Bateman, Randall J; Ewers, Michael

2018-01-01

Abstract Patients with Alzheimer’s disease vary in their ability to sustain cognitive abilities in the presence of brain pathology. A major open question is which brain mechanisms may support higher reserve capacity, i.e. relatively high cognitive performance at a given level of Alzheimer’s pathology. Higher functional MRI-assessed functional connectivity of a hub in the left frontal cortex is a core candidate brain mechanism underlying reserve as it is associated with education (i.e. a protective factor often associated with higher reserve) and attenuated cognitive impairment in prodromal Alzheimer’s disease. However, no study has yet assessed whether such hub connectivity of the left frontal cortex supports reserve throughout the evolution of pathological brain changes in Alzheimer’s disease, including the presymptomatic stage when cognitive decline is subtle. To address this research gap, we obtained cross-sectional resting state functional MRI in 74 participants with autosomal dominant Alzheimer’s disease, 55 controls from the Dominantly Inherited Alzheimer’s Network and 75 amyloid-positive elderly participants, as well as 41 amyloid-negative cognitively normal elderly subjects from the German Center of Neurodegenerative Diseases multicentre study on biomarkers in sporadic Alzheimer’s disease. For each participant, global left frontal cortex connectivity was computed as the average resting state functional connectivity between the left frontal cortex (seed) and each voxel in the grey matter. As a marker of disease stage, we applied estimated years from symptom onset in autosomal dominantly inherited Alzheimer’s disease and cerebrospinal fluid tau levels in sporadic Alzheimer’s disease cases. In both autosomal dominant and sporadic Alzheimer’s disease patients, higher levels of left frontal cortex connectivity were correlated with greater education. For autosomal dominant Alzheimer’s disease, a significant left frontal cortex connectivity �

Martian Ionospheric Observation and Modeling

Science.gov (United States)

González-Galindo, Francisco

2018-02-01

The Martian ionosphere is a plasma embedded within the neutral upper atmosphere of the planet. Its main source is the ionization of the CO2-dominated Martian mesosphere and thermosphere by the energetic EUV solar radiation. The ionosphere of Mars is subject to an important variability induced by changes in its forcing mechanisms (e.g., the UV solar flux) and by variations in the neutral atmosphere (e.g., the presence of global dust storms, atmospheric waves and tides, changes in atmospheric composition, etc.). Its vertical structure is dominated by a maximum in the electron concentration placed at about 120–140 km of altitude, coincident with the peak of the ionization rate. Below, a secondary peak produced by solar X-rays and photoelectron-impact ionization is observed. A sporadic third layer, possibly of meteoric origin, has been also detected below. The most abundant ion in the Martian ionosphere is O2+, although O+ can become more abundant in the upper ionospheric layers. While below about 180–200 km the Martian ionosphere is dominated by photochemical processes, above those altitudes the dynamics of the plasma become more important. The ionosphere is also an important source of escaping particles via processes such as dissociative recombination of ions or ion pickup. So, characterization of the ionosphere provides or can provide information about such disparate systems and processes as the solar radiation getting to the planet, the neutral atmosphere, the meteoric influx, the atmospheric escape to space, or the interaction of the planet with the solar wind. It is thus not surprising that the interest about this region dates from the beginning of the space era. From the first measurements provided by the Mariner 4 mission in the 1960s to the contemporaneous observations, still ongoing, by the Mars Express and MAVEN orbiters, our current knowledge of this atmospheric region is the consequence of the accumulation of more than 50 years of discontinuous

Genetic overlap between apparently sporadic motor neuron diseases

NARCIS (Netherlands)

van Blitterswijk, Marka; Vlam, Lotte; van Es, Michael A.; van der Pol, W.-Ludo; Hennekam, Eric A. M.; Dooijes, Dennis; Schelhaas, Helenius J.; van der Kooi, Anneke J.; de Visser, Marianne; Veldink, Jan H.; van den Berg, Leonard H.

2012-01-01

Progressive muscular atrophy (PMA) and amyotrophic lateral sclerosis (ALS) are devastating motor neuron diseases (MNDs), which result in muscle weakness and/or spasticity. We compared mutation frequencies in genes known to be associated with MNDs between patients with apparently sporadic PMA and

A virtual remote sensing observation network for continuous, near-real-time monitoring of atmospheric instability

Science.gov (United States)

Toporov, Maria; Löhnert, Ulrich; Potthast, Roland; Cimini, Domenico; De Angelis, Francesco

2017-04-01

Short-term forecasts of current high-resolution numerical weather prediction models still have large deficits in forecasting the exact temporal and spatial location of severe, locally influenced weather such as summer-time convective storms or cool season lifted stratus or ground fog. Often, the thermodynamic instability - especially in the boundary layer - plays an essential role in the evolution of weather events. While the thermodynamic state of the atmosphere is well measured close to the surface (i.e. 2 m) by in-situ sensors and in the upper troposphere by satellite sounders, the planetary boundary layer remains a largely under-sampled region of the atmosphere where only sporadic information from radiosondes or aircraft observations is available. The major objective of the presented DWD-funded project ARON (Extramural Research Programme) is to overcome this observational gap and to design an optimized network of ground based microwave radiometers (MWR) and compact Differential Absorption Lidars (DIAL) for a continuous, near-real-time monitoring of temperature and humidity in the atmospheric boundary layer in order to monitor thermodynamic (in)stability. Previous studies showed, that microwave profilers are well suited for continuously monitoring the temporal development of atmospheric stability (i.e. Cimini et al., 2015) before the initiation of deep convection, especially in the atmospheric boundary layer. However, the vertical resolution of microwave temperature profiles is best in the lowest kilometer above the surface, decreasing rapidly with increasing height. In addition, humidity profile retrievals typically cannot be resolved with more than two degrees of freedom for signal, resulting in a rather poor vertical resolution throughout the troposphere. Typical stability indices used to assess the potential of convection rely on temperature and humidity values not only in the region of the boundary layer but also in the layers above. Therefore, satellite

Rayleigh lidar observation of tropical mesospheric inversion layer: a comparison between dynamics and chemistry

Directory of Open Access Journals (Sweden)

Ramesh K.

2018-01-01

Full Text Available The Rayleigh lidar at National Atmospheric Research Laboratory, Gadanki (13.5°N, 79.2°E, India operates at 532 nm green laser with ~600 mJ/pulse since 2007. The vertical temperature profiles are derived above ~30 km by assuming the atmosphere is in hydrostatic equilibrium and obeys ideal gas law. A large mesospheric inversion layer (MIL is observed at ~77.4-84.6 km on the night of 22 March 2007 over Gadanki. Although dynamics and chemistry play vital role, both the mechanisms are compared for the occurrence of the MIL in the present study.

Rayleigh lidar observation of tropical mesospheric inversion layer: a comparison between dynamics and chemistry

Science.gov (United States)

Ramesh, K.; Sridharan, S.; Raghunath, K.

2018-04-01

The Rayleigh lidar at National Atmospheric Research Laboratory, Gadanki (13.5°N, 79.2°E), India operates at 532 nm green laser with 600 mJ/pulse since 2007. The vertical temperature profiles are derived above 30 km by assuming the atmosphere is in hydrostatic equilibrium and obeys ideal gas law. A large mesospheric inversion layer (MIL) is observed at 77.4-84.6 km on the night of 22 March 2007 over Gadanki. Although dynamics and chemistry play vital role, both the mechanisms are compared for the occurrence of the MIL in the present study.

Neuroendocrine Differentiation in Sporadic CRC and Hereditary Nonpolyosis Colorectal Cancer

Directory of Open Access Journals (Sweden)

M. H. Sun

2004-01-01

Full Text Available Extent neuroendocrine differentiation can be encountered in many human neoplasm derived from different organs and systems using immunohistochemistry and ultrastructural techniques. The tumor cells' behaviors resemble those of neurons and neuroendocrine cells. The presence of neuroendocrine differentiation reputedly appears to be associated with a poorer prognosis than the adenocarcinoma counterparts in sporadic human neoplasm. In this review the neuroendocrine carcinoma and the adenocarcinoma with neuroendocrine differentiation of colon and rectum both in sporadic colorectal carcinoma and the hereditary nonpolyposis colorectal cancer, the relationship of neuroendocrine differentiation and some possible molecular pathways in tumorogenesis of colorectal cancer will be discussed. Possible treatment strategy will also be addressed.

Early Detection of Sporadic Pancreatic Cancer

Science.gov (United States)

Chari, Suresh T.; Kelly, Kimberly; Hollingsworth, Michael A.; Thayer, Sarah P.; Ahlquist, David A.; Andersen, Dana K.; Batra, Surinder K.; Brentnall, Teresa A.; Canto, Marcia; Cleeter, Deborah F.; Firpo, Matthew A.; Gambhir, Sanjiv Sam; Go, Vay Liang W.; Hines, O. Joe; Kenner, Barbara J.; Klimstra, David S.; Lerch, Markus M.; Levy, Michael J.; Maitra, Anirban; Mulvihill, Sean J.; Petersen, Gloria M.; Rhim, Andrew D.; Simeone, Diane M.; Srivastava, Sudhir; Tanaka, Masao; Vinik, Aaron I.; Wong, David

2015-01-01

Abstract Pancreatic cancer (PC) is estimated to become the second leading cause of cancer death in the United States by 2020. Early detection is the key to improving survival in PC. Addressing this urgent need, the Kenner Family Research Fund conducted the inaugural Early Detection of Sporadic Pancreatic Cancer Summit Conference in 2014 in conjunction with the 45th Anniversary Meeting of the American Pancreatic Association and Japan Pancreas Society. This seminal convening of international representatives from science, practice, and clinical research was designed to facilitate challenging interdisciplinary conversations to generate innovative ideas leading to the creation of a defined collaborative strategic pathway for the future of the field. An in-depth summary of current efforts in the field, analysis of gaps in specific areas of expertise, and challenges that exist in early detection is presented within distinct areas of inquiry: Case for Early Detection: Definitions, Detection, Survival, and Challenges; Biomarkers for Early Detection; Imaging; and Collaborative Studies. In addition, an overview of efforts in familial PC is presented in an addendum to this article. It is clear from the summit deliberations that only strategically designed collaboration among investigators, institutions, and funders will lead to significant progress in early detection of sporadic PC. PMID:25931254

Genetic Relatedness among Nontypeable Pneumococci Implicated in Sporadic Cases of Conjunctivitis

Science.gov (United States)

Barker, Jason H.; Musher, Daniel M.; Silberman, Ronald; Phan, Hoang M.; Watson, David A.

1999-01-01

Nontypeable Streptococcus pneumoniae is a common cause of epidemic conjunctivitis. A previous molecular fingerprinting study identified a clone of nontypeable pneumococcus that was responsible for a recent outbreak of conjunctivitis. In the present study, we examined the extent to which pneumococci that cause sporadic cases of conjunctivitis are related to this epidemic strain. Using arbitrarily primed BOX-PCR, we have determined that, of 10 nontypeable pneumococci causing sporadic conjunctivitis, 5 were clonal and closely related to a previous outbreak strain, whereas 5 others were genetically diverse. PMID:10565927

Mutation analysis for DJ-1 in sporadic and familial parkinsonism: screening strategy in parkinsonism.

Science.gov (United States)

Tomiyama, Hiroyuki; Li, Yuanzhe; Yoshino, Hiroyo; Mizuno, Yoshikuni; Kubo, Shin-Ichiro; Toda, Tatsushi; Hattori, Nobutaka

2009-05-22

DJ-1 mutations cause autosomal recessive parkinsonism (ARP). Although some reports of DJ-1 mutations have been published, there is lack of information on the prevalence of these mutations in large-scale studies of both familial and sporadic parkinsonism. In this genetic screening study, we analyzed the distribution and frequency of DJ-1 mutations by direct nucleotide sequencing of coding exons and exon-intron boundaries of DJ-1, in 386 parkin-negative parkinsonism patients (371 index cases: 67 probands of autosomal recessive parkinsonism families, 90 probands of autosomal dominant parkinsonism families, 201 patients with sporadic parkinsonism, and 13 with unknown family histories) from 12 countries (Japan 283, China 27, Taiwan 22, Korea 22, Israel 16, Turkey 5, Philippines 2, Bulgaria 2, Greece 2, Tunisia 1, USA 2, Ukraine 1, unknown 1). None had causative mutation in DJ-1, suggesting DJ-1 mutation is very rare among patients with familial and sporadic parkinsonism from Asian countries and those with other ethnic background. This is in contrast to the higher frequencies and worldwide distribution of parkin- and PINK1-related parkinsonism in ARP and sporadic parkinsonism. Thus, after obtaining clinical information, screening for mutations in (1) parkin, (2) PINK1, (3) DJ-1, (4) ATP13A2 should be conducted in that order, in ARP and sporadic parkinsonism, based on their reported frequencies. In addition, haplotype analysis should be employed to check for homozygosity of 1p36, which harbors a cluster of causative genes for ARP such as DJ-1, PINK1 and ATP13A2 in ARP and sporadic parkinsonism, especially in parkinsonism with consanguinity.

Observations on the scattering layers over the continental shelf off Konkan coast (India)

Digital Repository Service at National Institute of Oceanography (India)

RamaRaju, V.S.

The echograms obtained by R.V. "varuna" using asdic during the September, 1963 cruise are analysed. Scattering layers observed over the shelf area, off Ratnagiri, are examined and studied in relation to the thermocline layer present. Comparison...

HEOS-2 observations of the boundary layer from the magnetopause to the ionosphere

International Nuclear Information System (INIS)

Formisano, V.

1980-01-01

HEOS-2 low energy electron data (10 eV - 3.7 keV) from the LPS Frascati plasma experiment have been used to identify three different magnetospheric electron populations. Magnetosheath-like electron energy spectra (35-50 eV) are characteristic of the plasma mantle, entry layer and cusps from the magnetopause down to 2-3 Rsub(E). Plasma sheet electrons (energy > 1 keV) are found at all local times, with strong intensities in the early morning quadrant and weaker intensities in the afternoon quadrant. The plasma sheet shows a well defined inner edge at all local times and latitudes, the inner edge coinciding probably with the plasmapause. The plasma sheet does not reach the magnetopause, but it is separated from it by a boundary layer electron population that is very distinct from the other two electron populations, most electrons having energies 100-300 eV. These three electron populations are mapped from the magnetopause down to the high latitude near earth regions. The boundary layer extends along the magnetopause up to 5-7 Rsub(E) above the equator; at higher latitudes it follows the magnetic lines of force and it is found closer and closer to the earth. (author)

Contribution of TARDBP mutations to sporadic amyotrophic lateral sclerosis.

Science.gov (United States)

Daoud, H; Valdmanis, P N; Kabashi, E; Dion, P; Dupré, N; Camu, W; Meininger, V; Rouleau, G A

2009-02-01

Mutations in the TARDBP gene, which encodes the TAR DNA binding protein (TDP-43), have been described in individuals with familial and sporadic amyotrophic lateral sclerosis (ALS). We screened the TARDBP gene in 285 French sporadic ALS patients to assess the frequency of TARDBP mutations in ALS. Six individuals had potentially deleterious mutations of which three were novel including a Y374X truncating mutation and P363A and A382P missense mutations. This suggests that TARDBP mutations may predispose to ALS in approximately 2% of the individuals followed in this study. Our findings, combined with those from other collections, brings the total number of mutations in unrelated ALS patients to 17, further suggesting that mutations in the TARDBP gene have an important role in the pathogenesis of ALS.

Prevalence of Abnormal Cervical Smears from Sporadic Screening ...

African Journals Online (AJOL)

The aim of the study was to find the prevalence of abnormal smears in an unscreened population of sexually active women attending a gynaecological clinic. “Pap” smears were taken sporadically for cytological examination from sexually active women attending gynaecological clinics at the Federal Medical Centre Gombe.

MAJOR MOLECULAR GENETIC DRIVERS IN SPORADIC PRIMARY HYPERPARATHYROIDISM.

Science.gov (United States)

Arnold, Andrew

2016-01-01

Primary hyperparathyroidism is primarily due to a solitary parathyroid adenoma but multi-gland disease, parathyroid carcinoma, and ectopic parathyroid hormone production can occur. Although primary hyperparathyroidism mostly presents sporadically, strong familial predispositions also exist. Much is known about heritable genetic mutations responsible for these syndromes, including multiple endocrine neoplasia types 1 and 2A, hyperparathyroidism-jaw tumor syndrome, and familial hypocalciuric hypercalcemia. Acquired mutations in common sporadic hyperparathyroidism have also been discovered. Here we focus on the most common and well-established genetic drivers: 1) involvement of the oncogene cyclin D1 in human neoplasia was first established in parathyroid adenomas, followed by recognition of its importance in other tumor types including breast cancer and B-lymphoid malignancy; and 2) somatic mutation of the MEN1 gene, first identified as the source of pathogenic germline mutations in patients with familial endocrinopathies, is found in a substantial fraction of non-familial parathyroid adenomas.

COL11A1 in FAP polyps and in sporadic colorectal tumors

International Nuclear Information System (INIS)

Fischer, Heléne; Salahshor, Sima; Stenling, Roger; Björk, Jan; Lindmark, Gudrun; Iselius, Lennart; Rubio, Carlos; Lindblom, Annika

2001-01-01

We previously reported that the α-1 chain of type 11 collagen (COL11A1), not normally expressed in the colon, was up-regulated in stromal fibroblasts in most sporadic colorectal carcinomas. Patients with germline mutations in the APC gene show, besides colonic polyposis, symptoms of stromal fibroblast involvement, which could be related to COL11A1 expression. Most colorectal carcinomas are suggested to be a result of an activated Wnt- pathway, most often involving an inactivation of the APC gene or activation of β-catenin. We used normal and polyp tissue samples from one FAP patient and a set of 37 sporadic colorectal carcinomas to find out if the up-regulation of COL11A1 was associated with an active APC/β-catenin pathway. In this study we found a statistically significant difference in COL11A1 expression between normal tissue and adenomas from one FAP patient, and all adenomas gave evidence for an active APC/β-catenin pathway. An active Wnt pathway has been suggested to involve stromal expression of WISP-1. We found a strong correlation between WISP-1 and COL11A1 expression in sporadic carcinomas. Our results suggest that expression of COL11A1 in colorectal tumors could be associated with the APC/β-catenin pathway in FAP and sporadic colorectal cancer

Clinicopathologic factors identify sporadic mismatch repair-defective colon cancers

DEFF Research Database (Denmark)

Halvarsson, Britta; Anderson, Harald; Domanska, Katarina

2008-01-01

Identification of sporadic mismatch repair (MMR)-defective colon cancers is increasingly demanded for decisions on adjuvant therapies. We evaluated clinicopathologic factors for the identification of these prognostically favorable tumors. Histopathologic features in 238 consecutive colon cancers...

30-year lidar observations of the stratospheric aerosol layer state over Tomsk (Western Siberia, Russia)

Science.gov (United States)

Zuev, Vladimir V.; Burlakov, Vladimir D.; Nevzorov, Aleksei V.; Pravdin, Vladimir L.; Savelieva, Ekaterina S.; Gerasimov, Vladislav V.

2017-02-01

There are only four lidar stations in the world which have almost continuously performed observations of the stratospheric aerosol layer (SAL) state over the last 30 years. The longest time series of the SAL lidar measurements have been accumulated at the Mauna Loa Observatory (Hawaii) since 1973, the NASA Langley Research Center (Hampton, Virginia) since 1974, and Garmisch-Partenkirchen (Germany) since 1976. The fourth lidar station we present started to perform routine observations of the SAL parameters in Tomsk (56.48° N, 85.05° E, Western Siberia, Russia) in 1986. In this paper, we mainly focus on and discuss the stratospheric background period from 2000 to 2005 and the causes of the SAL perturbations over Tomsk in the 2006-2015 period. During the last decade, volcanic aerosol plumes from tropical Mt. Manam, Soufrière Hills, Rabaul, Merapi, Nabro, and Kelut and extratropical (northern) Mt. Okmok, Kasatochi, Redoubt, Sarychev Peak, Eyjafjallajökull, and Grímsvötn were detected in the stratosphere over Tomsk. When it was possible, we used the NOAA HYSPLIT trajectory model to assign aerosol layers observed over Tomsk to the corresponding volcanic eruptions. The trajectory analysis highlighted some surprising results. For example, in the cases of the Okmok, Kasatochi, and Eyjafjallajökull eruptions, the HYSPLIT air mass backward trajectories, started from altitudes of aerosol layers detected over Tomsk with a lidar, passed over these volcanoes on their eruption days at altitudes higher than the maximum plume altitudes given by the Smithsonian Institution Global Volcanism Program. An explanation of these facts is suggested. The role of both tropical and northern volcanic eruptions in volcanogenic aerosol loading of the midlatitude stratosphere is also discussed. In addition to volcanoes, we considered other possible causes of the SAL perturbations over Tomsk, i.e., the polar stratospheric cloud (PSC) events and smoke plumes from strong forest fires. At least

The lobe to plasma sheet boundary layer transition: Theory and observations

International Nuclear Information System (INIS)

Schriver, D.; Ashour-Abdalla, M.; Treumann, R.; Nakamura, M.; Kistler, L.M.

1990-01-01

The lobe and the plasma sheet boundary layer in the Earth's magnetotail are regions of different plasma conditions and share a common interface. The transition from the lobe to the plasma sheet boundary layer is examined here using AMPTE/IRM data. When the satellite crossed from the lobe to the plasma sheet boundary layer, intense narrow banded wave bursts at 1 kHz were observed an d then broadband electrostatic noise (BEN) immediately followed. Simultaneous with the onset of BEN, high energy earthward streaming proton beams at > 40 keV (> 2,700 km/s) were detected. These results are used as input into a numerical simulation to study ion beam instabilities in the PSBL

Observations and model calculations of an additional layer in the topside ionosphere above Fortaleza, Brazil

Directory of Open Access Journals (Sweden)

B. Jenkins

1997-06-01

Full Text Available Calculations using the Sheffield University plasmasphere ionosphere model have shown that under certain conditions an additional layer can form in the low latitude topside ionosphere. This layer (the F3 layer has subsequently been observed in ionograms recorded at Fortaleza in Brazil. It has not been observed in ionograms recorded at the neighbouring station São Luis. Model calculations have shown that the F3 layer is most likely to form in summer at Fortaleza due to a combination of the neutral wind and the E×B drift acting to raise the plasma. At the location of São Luis, almost on the geomagnetic equator, the neutral wind has a smaller vertical component so the F3 layer does not form.

Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease

NARCIS (Netherlands)

I. Zerr; K. Kallenberg; D.M. Summers; C. Romero; A. Taratuto; U. Heinemann; M. Breithaupt; D. Varges; B. Meissner; A. Ladogana (Anna); M. Schuur (Maaike); S. Haik; S.J. Collins (Steven); G.H. Jansen (Gerard); G.B. Stokin; J. Pimentel; E. Hewer; D. Collie; P. Smith; H. Roberts; J.P. Brandel; P. Tikka-Kleemola (Päivi); M. Pocchiari (Maurizio); C. Begue; P. Cras (Patrick); R.G. Will; P. Sanchez-Juan (Pascual)

2009-01-01

textabstractSeveral molecular subtypes of sporadic Creutzfeldt-Jakob disease have been identified and electroencephalogram and cerebrospinal fluid biomarkers have been reported to support clinical diagnosis but with variable utility according to subtype. In recent years, a series of publications

Double-layer structure in polar mesospheric clouds observed from SOFIE/AIM

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H. Gao

2017-02-01

Full Text Available Double-layer structures in polar mesospheric clouds (PMCs are observed by using Solar Occultation for Ice Experiment (SOFIE data between 2007 and 2014. We find 816 and 301 events of double-layer structure with percentages of 10.32 and 7.25 % compared to total PMC events, and the mean distances between two peaks are 3.06 and 2.73 km for the Northern Hemisphere (NH and Southern Hemisphere (SH respectively. Double-layer PMCs almost always have less mean ice water content (IWC than daily IWC during the core of the season, but they are close to each other at the beginning and the end. The result by averaging over all events shows that the particle concentration has obvious double peaks, while the particle radius exhibits an unexpected monotonic increase with decreasing altitude. By further analysis of the background temperature and water vapour residual profiles, we conclude that the lower layer is a reproduced one formed at the bottom of the upper layer. 56.00 and 47.51 % of all double-layer events for the NH and SH respectively have temperature enhancements larger than 2 K locating between their double peaks. The longitudinal anti-correlation between the gravity waves' (GWs' potential energies and occurrence frequencies of double-layer PMCs suggests that the double-layer PMCs tend to form in an environment where the GWs have weaker intensities.

Observations of propagating double layers in a high current discharge

International Nuclear Information System (INIS)

Lindberg, L.

1988-01-01

Observations of current disruptions and strong electric fields along the magnetic field in a high-density (2 x 10 19 m - 3 , highly-ionized, moving, and expanding plasma column are reported. The electric field is interpreted in terms of propagating, strong electric double layers (3-5kV). An initial plasma column is formed in an axial magnetic field (0.1T) by means of a conical theta-pinch plasma gun. When an axial current (max 5kA, 3-5 kV) is drawn through the column spontaneous disruptions and double-layer formation occur within a few microseconds. Floating, secondary emitting Langmuir probes are used. They often indicate very high positive potential peaks (1-2 kV above the anode potential during a few μs) in the plasma on the positive side of the double layer. Short, intense bursts of HF radiation are detected at the disruptions

A joint study of the lower ionosphere by radar, lidar, and spectrometer

International Nuclear Information System (INIS)

Zhou, Qihou.

1991-01-01

The dynamics and associated phenomena occurring in the lower ionospheric-E region, especially the mesopause region between 80 km to 110 km at low latitude, are studied. In particular, incoherent scatter radar (ISR), sodium lidar and airglow spectrometry are used to study the ionospheric structure and neutral sodium structure. The simultaneous study of the ionospheric plasma and neutral atomic sodium is unprecedented in scope and detail. The joint study of the mesopause region reveals that plasma, neutral densities and temperature are interconnected through the same atmospheric dynamics. The theme of the thesis is to explain the formation of the controversial sporadic sodium layer (SSL) events. Strong correlation is established between the average total ion and sodium concentrations, and between sporadic-E and SSL events. The mechanism proposed in the thesis, which invokes temperature fluctuations induced by tides and gravity waves, finds good agreement with observations. Tides and gravity waves can converge ions into thin layers through the windshear mechanisms and can influence the concentration of atomic sodium through temperature fluctuations. Sodium abundance is shown to augment rapidly when the temperature is increased. Gravity wave theory states that the ion convergence node coincides with a temperature maximum for a westward propagating gravity wave, and coincides with a temperature minimum for an eastward propagating wave. Because tidal winds propagate westward, the ion layer coincides with the temperature maximum which consequently induces higher sodium concentration. This can account for the general correlation between sodium and total ion concentration and is supported by the O2(0-1) rotational temperature. Gravity waves and their interaction with tidal winds are believed to be responsible for the close association between sudden sodium layers and sporadic-E layers

The Prognostic Impact of p53 Expression on Sporadic Colorectal Cancer Is Dependent on p21 Status

International Nuclear Information System (INIS)

Kruschewski, Martin; Mueller, Kathrin; Lipka, Sybille; Budczies, Jan; Noske, Aurelia; Buhr, Heinz Johannes; Elezkurtaj, Sefer

2011-01-01

The prognostic value of p53 and p21 expression in colorectal cancer is still under debate. We hypothesize that the prognostic impact of p53 expression is dependent on p21 status. The expression of p53 and p21 was immunohistochemically investigated in a prospective cohort of 116 patients with UICC stage II and III sporadic colorectal cancer. The results were correlated with overall and recurrence-free survival. The mean observation period was 51.8 ± 2.5 months. Expression of p53 was observed in 72 tumors (63%). Overall survival was significantly better in patients with p53-positive carcinomas than in those without p53 expression (p = 0.048). No differences were found in recurrence-free survival (p = 0.161). The p53+/p21− combination was seen in 68% (n = 49), the p53+/p21+ combination in 32% (n = 23). Patients with p53+/p21− carcinomas had significantly better overall and recurrence-free survival than those with p53+/p21+ (p < 0.0001 resp. p = 0.003). Our data suggest that the prognostic impact of p53 expression on sporadic colorectal cancer is dependent on p21 status

Imaging movement-related activity in medicated Parkin-associated and sporadic Parkinson's disease

DEFF Research Database (Denmark)

van Eimeren, Thilo; Binkofski, Ferdinand; Buhmann, Carsten

2010-01-01

Treatment-related motor complications such as dyskinesias are a major problem in the long-term management of Parkinson's disease (PD). In sporadic PD, a relatively early onset of the disease is known to be associated with an early development of dyskinesias. Although linked with early onset...... selected movements. Patients with Parkin-associated and sporadic PD showed no difference in movement-related activation patterns. Moreover, the covariates 'age' and 'disease duration' similarly influenced brain activation in both patient groups. The present finding suggests that a stable long-term motor...

Mutations in the HFE gene and sporadic amyotrophic lateral sclerosis risk: a meta-analysis of observational studies

Directory of Open Access Journals (Sweden)

M. Li

2014-03-01

Full Text Available Iron homeostasis dysregulation has been regarded as an important mechanism in neurodegenerative diseases. The H63D and C282Y polymorphisms in the HFE gene may be involved in the development of sporadic amyotrophic lateral sclerosis (ALS through the disruption of iron homeostasis. However, studies investigating the relationship between ALS and these two polymorphisms have yielded contradictory outcomes. We performed a meta-analysis to assess the roles of the H63D and C282Y polymorphisms of HFE in ALS susceptibility. PubMed, MEDLINE, EMBASE, and Cochrane Library databases were systematically searched to identify relevant studies. Strict selection criteria and exclusion criteria were applied. Odds ratios (ORs with 95% confidence intervals (CIs were used to assess the strength of associations. A fixed- or random-effect model was selected, depending on the results of the heterogeneity test. Fourteen studies were included in the meta-analysis (six studies with 1692 cases and 8359 controls for C282Y; 14 studies with 5849 cases and 13,710 controls for H63D. For the C282Y polymorphism, significant associations were observed in the allele model (Y vs C: OR=0.76, 95%CI=0.62-0.92, P=0.005 and the dominant model (YY+CY vs CC: OR=0.75, 95%CI=0.61-0.92, P=0.006. No associations were found for any genetic model for the H63D polymorphism. The C282Y polymorphism in HFE could be a potential protective factor for ALS in Caucasians. However, the H63D polymorphism does not appear to be associated with ALS.

Cluster observations of bounday layer structure and a flux transfer event near the cusp

Directory of Open Access Journals (Sweden)

R. C. Fear

2005-10-01

Full Text Available On the 25th January 2002 between 10:00 and 12:00 UT, the four Cluster spacecraft passed through the northern high-latitude cusp, the dayside magnetosphere and into the magnetosheath in a linear formation. In the magnetosphere the PEACE electron spectrometers on the four spacecraft all observed a series of transient bursts of magnetosheath-like plasma, but without bipolar magnetic signatures in the magnetopause normal component as might be expected if the plasma had been injected by transient reconnection (flux transfer events – FTEs. Reordering the data using the magnetopause transition parameter reveals that these plasma observations, the related variations in the magnetic field and the balance of magnetic and thermal gas pressures are consistent with transient entries into a stable high-latitude boundary layer structure. However, once some of the spacecraft entered the magnetosheath, FTE signatures were observed outside the magnetopause at the same time as some of the boundary layer entries occurred at the other spacecraft inside. Thus, (a the lack of a bipolar BN signature is inconsistent with the traditional picture of a magnetospheric FTE, and (b the cause of the observed entry of the spacecraft into the boundary layer (pressure pulse or passing magnetosheath FTE can only be determined by spacecraft observations in the magnetosheath. Keywords. Magnetospheric physics (Magnetopause, cusp and bondary layers; Solar wind- magnetosphere interactions; Magnetosheath

Application of quantitative DTI metrics in sporadic CJD

Directory of Open Access Journals (Sweden)

E. Caverzasi

2014-01-01

Full Text Available Diffusion Weighted Imaging is extremely important for the diagnosis of probable sporadic Jakob–Creutzfeldt disease, the most common human prion disease. Although visual assessment of DWI MRI is critical diagnostically, a more objective, quantifiable approach might more precisely identify the precise pattern of brain involvement. Furthermore, a quantitative, systematic tracking of MRI changes occurring over time might provide insights regarding the underlying histopathological mechanisms of human prion disease and provide information useful for clinical trials. The purposes of this study were: 1 to describe quantitatively the average cross-sectional pattern of reduced mean diffusivity, fractional anisotropy, atrophy and T1 relaxation in the gray matter (GM in sporadic Jakob–Creutzfeldt disease, 2 to study changes in mean diffusivity and atrophy over time and 3 to explore their relationship with clinical scales. Twenty-six sporadic Jakob–Creutzfeldt disease and nine control subjects had MRIs on the same scanner; seven sCJD subjects had a second scan after approximately two months. Cortical and subcortical gray matter regions were parcellated with Freesurfer. Average cortical thickness (or subcortical volume, T1-relaxiation and mean diffusivity from co-registered diffusion maps were calculated in each region for each subject. Quantitatively on cross-sectional analysis, certain brain regions were preferentially affected by reduced mean diffusivity (parietal, temporal lobes, posterior cingulate, thalamus and deep nuclei, but with relative sparing of the frontal and occipital lobes. Serial imaging, surprisingly showed that mean diffusivity did not have a linear or unidirectional reduction over time, but tended to decrease initially and then reverse and increase towards normalization. Furthermore, there was a strong correlation between worsening of patient clinical function (based on modified Barthel score and increasing mean diffusivity.

Transmission of sporadic Creutzfeldt-Jakob disease by blood transfusion: risk factor or possible biases.

Science.gov (United States)

Puopolo, Maria; Ladogana, Anna; Vetrugno, Vito; Pocchiari, Maurizio

2011-07-01

The occurrence of transfusion transmissions of variant Creutzfeldt-Jakob disease (CJD) cases has reawakened attention to the possible similar risk posed by other forms of CJD. CJD with a definite or probable diagnosis (sporadic CJD, n = 741; genetic CJD, n = 175) and no-CJD patients with definite alternative diagnosis (n = 482) with available blood transfusion history were included in the study. The risk of exposure to blood transfusion occurring more than 10 years before disease onset and for some possible confounding factors was evaluated by calculating crude odds ratios (ORs). Variables with significant ORs in univariate analyses were included in multivariate logistic regression analyses. In the univariate model, blood transfusion occurring more than 10 years before clinical onset is 4.1-fold more frequent in sporadic CJD than in other neurologic disorders. This significance is lost when the 10-year lag time was not considered. Multivariate analyses show that the risk of developing sporadic CJD after transfusion increases (OR, 5.05) after adjusting for possible confounding factors. Analysis conducted on patients with genetic CJD did not reveal any significant risk factor associated with transfusion. This is the first case-control study showing a significant risk of transfusion occurring more than 10 years before clinical onset in sporadic CJD patients. It remains questionable whether the significance of these data is biologically plausible or the consequence of biases in the design of the study, but they counterbalance previous epidemiologic negative reports that might have overestimated the assessment of blood safety in sporadic CJD. © 2010 American Association of Blood Banks.

Auroral lights created by high-power radiowaves in the ionospheric E region

International Nuclear Information System (INIS)

Kagan, L M

2008-01-01

Artificial auroral lights are optical emissions induced by high-power radiowaves in a manner similar to the creation of natural auroral lights due to precipitation of superthermal electrons. Here the Earth's atmosphere itself plays the role of a plasma laboratory. A high-power radio-transmitter creates superthermal electrons in situ due to radiowave interactions with the ionospheric plasma. Low-altitude (85-125 km) artificial aurora is a relatively rare phenomenon observed for the first time about 10 years ago. Analysis of available observations shows that its intensity and structure, as well as the very possibility of its generation at these altitudes, depend largely on the presence and features of so-called sporadic ionization, best known for its effect on navigation and communication. Thus, all other parameters unchanged, a 20 times increase in the height-extent of sporadic ionization clouds could under certain circumstances result in a 27-fold increase in the intensity of the low-altitude artificial aurora. Understanding the morphology and typical features of sporadic ionization clouds for a given location would allow proper planning of experimental campaigns and noticeably extend our knowledge of the atmosphere and its use for human needs. Equally important is that low-altitude artificial auroras give information about the horizontal structure and dynamics of E-region sporadic ionization, the airglow-source region, the energy of superthermal electrons and perhaps the local atmospheric temperature and water vapor content at 80-90 km

Coherent structures in the Es layer and neutral middle atmosphere

Czech Academy of Sciences Publication Activity Database

Mošna, Zbyšek; Koucká Knížová, Petra; Potužníková, Kateřina

136 B, December (2015), s. 155-162 ISSN 1364-6826 R&D Projects: GA ČR(CZ) GA15-24688S; GA ČR(CZ) GAP209/12/2440 Institutional support: RVO:68378289 Keywords : critical frequency foEs * mesospheric temperature * mesospheric winds * planetary waves * Rossby mode * sporadic layer * stratospheric temperature Subject RIV: DG - Athmosphere Sciences, Meteorology Impact factor: 1.463, year: 2015 http://www.sciencedirect.com/science/article/pii/S1364682615001273

Gastric Medullary Carcinoma with Sporadic Mismatch Repair Deficiency and a TP53 R273C Mutation: An Unusual Case with Wild-Type BRAF

Directory of Open Access Journals (Sweden)

Brett M. Lowenthal

2017-01-01

Full Text Available Medullary carcinoma has long been recognized as a subtype of colorectal cancer associated with microsatellite instability and Lynch syndrome. Gastric medullary carcinoma is a very rare neoplasm. We report a 67-year-old male who presented with a solitary gastric mass. Total gastrectomy revealed a well-demarcated, poorly differentiated carcinoma with an organoid growth pattern, pushing borders, and abundant peritumoral lymphocytic response. The prior cytology was cellular with immunohistochemical panel consistent with upper gastrointestinal/pancreaticobiliary origin. Overall, the histopathologic findings were consistent with gastric medullary carcinoma. A mismatch repair panel revealed a mismatch repair protein deficient tumor with loss of MLH1 and PMS2 expression. BRAF V600E immunostain (VE1 and BRAF molecular testing were negative, indicating a wild-type gene. Tumor sequencing of MLH1 demonstrated a wild-type gene, while our molecular panel identified TP53 c.817C>T (p.R273C mutation. These findings were compatible with a sporadic tumor. Given that morphologically identical medullary tumors often occur in Lynch syndrome, it is possible that mismatch repair loss is an early event in sporadic tumors with p53 mutation being a late event. Despite having wild-type BRAF, this tumor is sporadic and unrelated to Lynch syndrome. This case report demonstrates that coordinate ancillary studies are needed to resolve sporadic versus hereditary rare tumors.

Observation of layered antiferromagnetism in self-assembled parallel NiSi nanowire arrays on Si(110) by spin-polarized scanning tunneling spectromicroscopy

Science.gov (United States)

Hong, Ie-Hong; Hsu, Hsin-Zan

2018-03-01

The layered antiferromagnetism of parallel nanowire (NW) arrays self-assembled on Si(110) have been observed at room temperature by direct imaging of both the topographies and magnetic domains using spin-polarized scanning tunneling microscopy/spectroscopy (SP-STM/STS). The topographic STM images reveal that the self-assembled unidirectional and parallel NiSi NWs grow into the Si(110) substrate along the [\\bar{1}10] direction (i.e. the endotaxial growth) and exhibit multiple-layer growth. The spatially-resolved SP-STS maps show that these parallel NiSi NWs of different heights produce two opposite magnetic domains, depending on the heights of either even or odd layers in the layer stack of the NiSi NWs. This layer-wise antiferromagnetic structure can be attributed to an antiferromagnetic interlayer exchange coupling between the adjacent layers in the multiple-layer NiSi NW with a B2 (CsCl-type) crystal structure. Such an endotaxial heterostructure of parallel magnetic NiSi NW arrays with a layered antiferromagnetic ordering in Si(110) provides a new and important perspective for the development of novel Si-based spintronic nanodevices.

Near Earth space sporadic radio emission busts occurring during sunrise

Science.gov (United States)

Dudnik, A. V.; Zaljubovsky, I. I.; Kartashev, V. M.; Lasarev, A. V.; Shmatko, E. S.

1985-01-01

During the period of low solar activity at sunrise the effect of sporadic high frequency near Earth space radio emission was experimentally discovered at middle latitudes. The possible mechanism of its origin is discussed.

Boundary layer transition observations on a body of revolution with surface heating and cooling in water

Science.gov (United States)

Arakeri, V. H.

1980-04-01

Boundary layer flow visualization in water with surface heat transfer was carried out on a body of revolution which had the predicted possibility of laminar separation under isothermal conditions. Flow visualization was by in-line holographic technique. Boundary layer stabilization, including elimination of laminar separation, was observed to take place on surface heating. Conversely, boundary layer destabilization was observed on surface cooling. These findings are consistent with the theoretical predictions of Wazzan et al. (1970).

Influence of Coding Variability in APP-Aβ Metabolism Genes in Sporadic Alzheimer's Disease.

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Celeste Sassi

Full Text Available The cerebral deposition of Aβ42, a neurotoxic proteolytic derivate of amyloid precursor protein (APP, is a central event in Alzheimer's disease (AD(Amyloid hypothesis. Given the key role of APP-Aβ metabolism in AD pathogenesis, we selected 29 genes involved in APP processing, Aβ degradation and clearance. We then used exome and genome sequencing to investigate the single independent (single-variant association test and cumulative (gene-based association test effect of coding variants in these genes as potential susceptibility factors for AD, in a cohort composed of 332 sporadic and mainly late-onset AD cases and 676 elderly controls from North America and the UK. Our study shows that common coding variability in these genes does not play a major role for the disease development. In the single-variant association analysis, the main hits, none of which statistically significant after multiple testing correction (1.9e-4e-3 sporadic AD.

Cluster observations of bounday layer structure and a flux transfer event near the cusp

Directory of Open Access Journals (Sweden)

R. C. Fear

2005-10-01

Full Text Available On the 25th January 2002 between 10:00 and 12:00 UT, the four Cluster spacecraft passed through the northern high-latitude cusp, the dayside magnetosphere and into the magnetosheath in a linear formation. In the magnetosphere the PEACE electron spectrometers on the four spacecraft all observed a series of transient bursts of magnetosheath-like plasma, but without bipolar magnetic signatures in the magnetopause normal component as might be expected if the plasma had been injected by transient reconnection (flux transfer events – FTEs. Reordering the data using the magnetopause transition parameter reveals that these plasma observations, the related variations in the magnetic field and the balance of magnetic and thermal gas pressures are consistent with transient entries into a stable high-latitude boundary layer structure. However, once some of the spacecraft entered the magnetosheath, FTE signatures were observed outside the magnetopause at the same time as some of the boundary layer entries occurred at the other spacecraft inside. Thus, (a the lack of a bipolar B_N signature is inconsistent with the traditional picture of a magnetospheric FTE, and (b the cause of the observed entry of the spacecraft into the boundary layer (pressure pulse or passing magnetosheath FTE can only be determined by spacecraft observations in the magnetosheath.

Keywords. Magnetospheric physics (Magnetopause, cusp and bondary layers; Solar wind- magnetosphere interactions; Magnetosheath

Long-term Auditory Symptoms in Patients With Sporadic Vestibular Schwannoma: An International Cross-Sectional Study.

Science.gov (United States)

Tveiten, Oystein Vesterli; Carlson, Matthew L; Goplen, Frederik; Vassbotn, Flemming; Link, Michael J; Lund-Johansen, Morten

2015-08-01

There are limited data on the long-term auditory symptoms in patients with sporadic small- and medium-sized vestibular schwannoma (VS). The initial treatment strategy for VS is controversial. To characterize auditory symptoms in a large cohort of patients with VS. Patients with ≤3 cm VS who underwent primary microsurgery, gamma knife surgery, or observation between 1998 and 2008 at 2 independent hospitals were identified. Clinical data were extracted from existing VS databases. At a mean time of 7.7 years after initial treatment, patients were surveyed via mail with the use of the Hearing Handicap Inventory for Adults (HHIA) and the Tinnitus Handicap Inventory. The response rate was 79%; a total of 539 respondents were analyzed. Overall, the hearing prognosis was poor, because more than 75% of all patients had nonserviceable hearing at the last clinical follow-up. Good baseline hearing proved to be a strong predictor for maintained serviceable hearing. Treatment modality was independently associated with both audiometric outcome and HHIA results. Active treatment with microsurgery or gamma knife surgery did not appear to be protective, because patients who were observed had the greatest probability of durable hearing. Patients in the surgical series had the greatest hearing loss. Tinnitus Handicap Inventory results were less predictable. The only predictors of tinnitus handicap were age and HHIA score. The overall prognosis for hearing in sporadic VS is poor regardless of treatment strategy. Treatment modality was an independent predictor of hearing status; observation was associated with the highest rate of hearing preservation. .

Mutation analysis of SDHB and SDHC: novel germline mutations in sporadic head and neck paraganglioma and familial paraganglioma and/or pheochromocytoma

Directory of Open Access Journals (Sweden)

Wong Nora

2006-01-01

Full Text Available Abstract Background Germline mutations of the SDHD, SDHB and SDHC genes, encoding three of the four subunits of succinate dehydrogenase, are a major cause of hereditary paraganglioma and pheochromocytoma, and demonstrate that these genes are classic tumor suppressors. Succinate dehydrogenase is a heterotetrameric protein complex and a component of both the Krebs cycle and the mitochondrial respiratory chain (succinate:ubiquinone oxidoreductase or complex II. Methods Using conformation sensitive gel electrophoresis (CSGE and direct DNA sequencing to analyse genomic DNA from peripheral blood lymphocytes, here we describe the mutation analysis of the SDHB and SDHC genes in 37 patients with sporadic (i.e. no known family history head and neck paraganglioma and five pheochromocytoma and/or paraganglioma families. Results Two sporadic patients were found to have a SDHB splice site mutation in intron 4, c.423+1G>A, which produces a mis-spliced transcript with a 54 nucleotide deletion, resulting in an 18 amino acid in-frame deletion. A third patient was found to carry the c.214C>T (p.Arg72Cys missense mutation in exon 4 of SDHC, which is situated in a highly conserved protein motif that constitutes the quinone-binding site of the succinate: ubiquinone oxidoreductase (SQR complex in E. coli. Together with our previous results, we found 27 germline mutations of SDH genes in 95 cases (28% of sporadic head and neck paraganglioma. In addition all index patients of five families showing hereditary pheochromocytoma-paraganglioma were found to carry germline mutations of SDHB: four of which were novel, c.343C>T (p.Arg115X, c.141G>A (p.Trp47X, c.281G>A (p.Arg94Lys, and c.653G>C (p.Trp218Ser, and one reported previously, c.136C>T, p.Arg46X. Conclusion In conclusion, these data indicate that germline mutations of SDHB and SDHC play a minor role in sporadic head and neck paraganglioma and further underline the importance of germline SDHB mutations in cases of

Coexistence of protease sensitive and resistant prion protein in 129VV homozygous sporadic Creutzfeldt–Jakob disease: a case report

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Rodríguez-Martínez Ana B

2012-10-01

Full Text Available Abstract Introduction The coexistence of different molecular types of classical protease-resistant prion protein in the same individual have been described, however, the simultaneous finding of these with the recently described protease-sensitive variant or variably protease-sensitive prionopathy has, to the best of our knowledge, not yet been reported. Case presentation A 74-year-old Caucasian woman showed a sporadic Creutzfeldt–Jakob disease clinical phenotype with reactive depression, followed by cognitive impairment, akinetic-rigid Parkinsonism with pseudobulbar syndrome and gait impairment with motor apraxia, visuospatial disorientation, and evident frontal dysfunction features such as grasping, palmomental reflex and brisk perioral reflexes. She died at age 77. Neuropathological findings showed: spongiform change in the patient’s cerebral cortex, striatum, thalamus and molecular layer of the cerebellum with proteinase K-sensitive synaptic-like, dot-like or target-like prion protein deposition in the cortex, thalamus and striatum; proteinase K-resistant prion protein in the same regions; and elongated plaque-like proteinase K-resistant prion protein in the molecular layer of the cerebellum. Molecular analysis of prion protein after proteinase K digestion revealed decreased signal intensity in immunoblot, a ladder-like protein pattern, and a 71% reduction of PrPSc signal relative to non-digested material. Her cerebellum showed a 2A prion protein type largely resistant to proteinase K. Genotype of polymorphism at codon 129 was valine homozygous. Conclusion Molecular typing of prion protein along with clinical and neuropathological data revealed, to the best of our knowledge, the first case of the coexistence of different protease-sensitive prion proteins in the same patient in a rare case that did not fulfill the current clinical diagnostic criteria for either probable or possible sporadic Creutzfeldt–Jakob disease. This highlights the

The localization of facial motor impairment in sporadic Möbius syndrome.

Science.gov (United States)

Cattaneo, L; Chierici, E; Bianchi, B; Sesenna, E; Pavesi, G

2006-06-27

To investigate the neurophysiologic aspects of facial motor control in patients with sporadic Möbius syndrome defined as nonprogressive congenital facial and abducens palsy. The authors assessed 24 patients with sporadic Möbius syndrome by performing a complete clinical examination and neurophysiologic tests including facial nerve conduction studies, needle electromyography examination of facial muscles, and recording of the blink reflex and of the trigeminofacial inhibitory reflex. Two distinct groups of patients were identified according to neurophysiologic testing. The first group was characterized by increased facial distal motor latencies (DMLs) and poor recruitment of small and polyphasic motor unit action potentials (MUAPs). The second group was characterized by normal facial DMLs and neuropathic MUAPs. It is hypothesized that in the first group, the disorder is due to a rhombencephalic maldevelopment with selective sparing of small-size MUs, and in the second group, the disorder is related to an acquired nervous injury during intrauterine life, with subsequent neurogenic remodeling of MUs. The trigeminofacial reflexes showed that in most subjects of both groups, the functional impairment of facial movements was caused by a nuclear or peripheral site of lesion, with little evidence of brainstem interneuronal involvement. Two different neurophysiologically defined phenotypes can be distinguished in sporadic Möbius syndrome, with different pathogenetic implications.

Mapping of gene expression reveals CYP27A1 as a susceptibility gene for sporadic ALS.

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Frank P Diekstra

Full Text Available Amyotrophic lateral sclerosis (ALS is a progressive, neurodegenerative disease characterized by loss of upper and lower motor neurons. ALS is considered to be a complex trait and genome-wide association studies (GWAS have implicated a few susceptibility loci. However, many more causal loci remain to be discovered. Since it has been shown that genetic variants associated with complex traits are more likely to be eQTLs than frequency-matched variants from GWAS platforms, we conducted a two-stage genome-wide screening for eQTLs associated with ALS. In addition, we applied an eQTL analysis to finemap association loci. Expression profiles using peripheral blood of 323 sporadic ALS patients and 413 controls were mapped to genome-wide genotyping data. Subsequently, data from a two-stage GWAS (3,568 patients and 10,163 controls were used to prioritize eQTLs identified in the first stage (162 ALS, 207 controls. These prioritized eQTLs were carried forward to the second sample with both gene-expression and genotyping data (161 ALS, 206 controls. Replicated eQTL SNPs were then tested for association in the second-stage GWAS data to find SNPs associated with disease, that survived correction for multiple testing. We thus identified twelve cis eQTLs with nominally significant associations in the second-stage GWAS data. Eight SNP-transcript pairs of highest significance (lowest p = 1.27 × 10(-51 withstood multiple-testing correction in the second stage and modulated CYP27A1 gene expression. Additionally, we show that C9orf72 appears to be the only gene in the 9p21.2 locus that is regulated in cis, showing the potential of this approach in identifying causative genes in association loci in ALS. This study has identified candidate genes for sporadic ALS, most notably CYP27A1. Mutations in CYP27A1 are causal to cerebrotendinous xanthomatosis which can present as a clinical mimic of ALS with progressive upper motor neuron loss, making it a plausible

Reactive GTS Allocation Protocol for Sporadic Events Using the IEEE 802.15.4

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Mukhtar Azeem

2014-01-01

by the IEEE 802.15.4 standard. The proposed control protocol ensures that a given offline sporadic schedule can be adapted online in a timely manner such that the static periodic schedule has not been disturbed and the IEEE 802.15.4 standard compliance remains intact. The proposed protocol is simulated in OPNET. The simulation results are analyzed and presented in this paper to prove the correctness of the proposed protocol regarding the efficient real-time sporadic event delivery along with the periodic event propagation.

Observed seasonal variability of barrier layer in the Bay of Bengal

Digital Repository Service at National Institute of Oceanography (India)

Pankajakshan, T.; Muraleedharan, P.M.; Rao, R.R.; Somayajulu, Y.K.; Reddy, G.V.; Revichandran, C.

The observed formation of Barrier Layer (BL) and the seasonal variability of BL thickness (BLT) in the Bay of Bengal are examined utilizing the most comprehensive data set. Thick BL (~50m) first appears in the coastal region of the northeastern bay...

TWO-LAYER SECURE PREVENTION MECHANISM FOR REDUCING E-COMMERCE SECURITY RISKS

OpenAIRE

Sen-Tarng Lai

2015-01-01

E-commerce is an important information system in the network and digital age. However, the network intrusion, malicious users, virus attack and system security vulnerabilities have continued to threaten the operation of the e-commerce, making e-commerce security encounter serious test. How to improve ecommerce security has become a topic worthy of further exploration. Combining routine security test and security event detection procedures, this paper proposes the Two-Layer Secure ...

Atomic-scale observation of structural and electronic orders in the layered compound α-RuCl3

Science.gov (United States)

Ziatdinov, M.; Banerjee, A.; Maksov, A.; Berlijn, T.; Zhou, W.; Cao, H. B.; Yan, J.-Q.; Bridges, C. A.; Mandrus, D. G.; Nagler, S. E.; Baddorf, A. P.; Kalinin, S. V.

2016-12-01

A pseudospin-1/2 Mott phase on a honeycomb lattice is proposed to host the celebrated two-dimensional Kitaev model which has an elusive quantum spin liquid ground state, and fascinating physics relevant to the development of future templates towards topological quantum bits. Here we report a comprehensive, atomically resolved real-space study by scanning transmission electron and scanning tunnelling microscopies on a novel layered material displaying Kitaev physics, α-RuCl3. Our local crystallography analysis reveals considerable variations in the geometry of the ligand sublattice in thin films of α-RuCl3 that opens a way to realization of a spatially inhomogeneous magnetic ground state at the nanometre length scale. Using scanning tunnelling techniques, we observe the electronic energy gap of ~0.25 eV and intra-unit cell symmetry breaking of charge distribution in individual α-RuCl3 surface layer. The corresponding charge-ordered pattern has a fine structure associated with two different types of charge disproportionation at Cl-terminated surface.

Higher cytoplasmic and nuclear poly(ADP-ribose) polymerase expression in familial than in sporadic breast cancer

NARCIS (Netherlands)

Klauke, M.L.; Hoogerbrugge-van der Linden, N.; Budczies, J.; Bult, P.; Prinzler, J.; Radke, C.; van Krieken, J.H.; Dietel, M.; Denkert, C.; Muller, B.M.

2012-01-01

Poly(ADP-ribose) polymerase 1 (PARP) is a key element of the single-base excision pathway for repair of DNA single-strand breaks. To compare the cytoplasmic and nuclear poly(ADP-ribose) expression between familial (BRCA1, BRCA2, or non BRCA1/2) and sporadic breast cancer, we investigated 39 sporadic

Observations of Flaking of Co-deposited Layers in TFTR

International Nuclear Information System (INIS)

Gentile, C.A.; Skinner, C.H.; Young, K.M.

1999-01-01

Flaking of co-deposited layers in the Tokamak Fusion Test Reactor (TFTR) has been observed after the termination of plasma operations. This unexpected flaking affects approximately 15% of the tiles and appears on isotropic graphite tiles but not on carbon fiber composite tiles. Samples of tiles, flakes and dust were recently collected from the inside of the vacuum vessel and will be analyzed to better characterize the behavior of tritium on plasma facing components in DT fusion devices

Structure of the low-latitude boundary layer

International Nuclear Information System (INIS)

Sckopke, N.; Paschmann, G.; Haerendel, G.; Sonnerup, B.U.O.; Bame, S.J.; Forbes, T.G.; Hones, E.W. Jr.; Russell, C.T.

1981-01-01

Observations at high temporal resolution of the frontside magnetopause and plasma boundary layer, made with the Los Alamos Scientific Laboratory/Max-Planck-Institut, Institut fuer Extraterrestrische Physik, fast plasma analyzer on board the Isee 1 and 2 spacecraft, have revealed a complex quasi-periodic structure of some of the observed boundary layers: cool tailward streaming boundary layer plasma is seen intermittently, with intervening periods of hot tenuous plasma which has properties similar to the magnetospheric population. While individual encounters with the boundary layer plasma last only a few minutes, the total observation time may extend over 1 hour or more. One such crossing, at 0800 hours local time and 40 0 northern GSM latitude, is examined in detail, including a quantitative comparison of the boundary layer entry and exit times of the two spacecraft. The data are found to be compatible with a boundary layer that is always attached to the magnetopause but where the layer thickness has a large-scale spatial modulation pattern which travels tailward past the spacecraft. Included are periods when the thickness is essentially zero and others when it is of the order of 1 R/sub E/. The duration of these periods is highly variable but is typically in the range of 2--5 min, corresponding to a distance along the magnetopause of the order of 3--8 R/sub E/. The observed boundary layer features include a steep density gradient at the magnetopause, with an approximately constant boundary layer plasma density amounting to about 25% of the magnetosheath density, and a second abrupt density decrease at the inner edge of the layer. It also appears that the purely magnetospheric plasma is ocassionally separated from the boundary layer by a halo region in which the plasma density is somewhat higher, and the temperature somewhat lower, than in the magnetosphere. A tentative model is proposed

High prevalence of exon 8 G533C mutation in apparently sporadic medullary thyroid carcinoma in Greece.

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Sarika, H L; Papathoma, A; Garofalaki, M; Vasileiou, V; Vlassopoulou, B; Anastasiou, E; Alevizaki, M

2012-12-01

Genetic screening for ret mutation has become routine practice in the evaluation of medullary thyroid carcinoma (MTC). Approximately 25% of these tumours are familial, and they occur as components of the multiple endocrine neoplasia type 2 syndromes (MEN 2A and 2B) or familial MTC. In familial cases, the majority of mutations are found in exons 10, 11, 13, 14 or 15 of the ret gene. A rare mutation involving exon 8 (G533C) has recently been reported in familial cases of MTC in Brazil and Greece; some of these cases were originally thought to be sporadic. The aim of this study was to re-evaluate a series of sporadic cases of MTC, with negative family history, and screen them for germline mutations in exon 8. Genomic DNA was extracted from peripheral lymphocytes in 129 unrelated individuals who had previously been characterized as 'sporadic' based on the negative family history and negative screening for ret gene mutations. Samples were analysed in Applied Biosystems 7500 real-time PCR and confirmed by sequencing. The G533C exon 8 mutation was identified in 10 of 129 patients with sporadic MTC. Asymptomatic gene carriers were subsequently identified in other family members. In our study, we found that 7·75% patients with apparently sporadic MTC do carry G533C mutation involving exon 8 of ret. We feel that there is now a need to include exon 8 mutation screening in all patients diagnosed as sporadic MTC, in Greece. © 2012 Blackwell Publishing Ltd.

Chang'e 3 and Jade Rabbit's: observations and the landing zone

Science.gov (United States)

Ping, Jinsong

Chang’E-3 was launched and landed on the near side of the Moon in December 2013. It is realizing the 2nd phase of Chinese lunar scientific exploration projects. Together with the various in-situ optical observations around the landing sites, the mission carried 4 kinds of radio science experiments, cover the various lunar scientific disciplines as well as lunar surface radio astronomy studies. The key payloads onboard the lander and rover include the near ultraviolet telescope, extreme ultraviolet cameras, ground penetrating radar, very low frequency radio spectrum analyzer, which have not been used in earlier lunar landing missions. Optical spectrometer, Alpha Paticle X-ray spectrometer and Gama Ray spectrometer is also used. The mission is using extreme ultraviolet camera to observe the sun activity and geomagnetic disturbances on geo-space plasma layer of extreme ultraviolet radiation, studying space weather in the plasma layer role in the process; the mission also carries the first time lunar base optical astronomical observations. Most importantly, the topography, landforms and geological structure has been explored in detail. Additionally, the very precise Earth-Moon radio phase ranging technique was firstly tested and realized in this mission. It may increase the study of lunar dyanmics together with LLR technique. Similar to Luna-Glob landers, together with the VLBI radio beacons, the radio transponders are also set on the Chang’E-3. Transponder will receive the uplink X band radio wave transmitted from the two newly constructed Chinese deep space stations, where the high quality hydrogen maser atomic clocks have been used as local time and frequency standard. Radio science receivers have been developed by updating the multi-channel open loop Doppler receiver developed for VLBI and Doppler tracking in Yinghuo-1 and Phobos-Glob Martian missions. This experiment will improve the study of lunar dynamics, by means of measuring the lunar physical liberations

[The practice guideline 'Dermatomyositis, polymyositis and sporadic inclusion body myositis'

NARCIS (Netherlands)

Hoogendijk, J.E.; Bijlsma, J.W.J.; Engelen, B.G.M. van; Lindeman, E.J.M.; Royen-Kerkhof, A. van; Rie, M.A. de; Visser, M. de; Jennekens, F.G.I.

2005-01-01

This guideline presents recommendations for the diagnosis and treatment of dermatomyositis, polymyositis and sporadic inclusion body myositis (sIBM) according to the best available evidence. Characteristic skin abnormalities can be sufficient for the diagnosis of dermatomyositis. In case of doubt, a

Molecular genetic analysis of 103 sporadic colorectal tumours in Czech patients.

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Peter Vasovcak

Full Text Available The Czech Republic has one of the highest incidences of colorectal cancer (CRC in Europe. To evaluate whether sporadic CRCs in Czech patients have specific mutational profiles we analysed somatic genetic changes in known CRC genes (APC, KRAS, TP53, CTNNB1, MUTYH and BRAF, loss of heterozygosity (LOH at the APC locus, microsatellite instability (MSI, and methylation of the MLH1 promoter in 103 tumours from 102 individuals. The most frequently mutated gene was APC (68.9% of tumours, followed by KRAS (31.1%, TP53 (27.2%, BRAF (8.7% and CTNNB1 (1.9%. Heterozygous germline MUTYH mutations in 2 patients were unlikely to contribute to the development of their CRCs. LOH at the APC locus was found in 34.3% of tumours, MSI in 24.3% and MLH1 methylation in 12.7%. Seven tumours (6.9% were without any changes in the genes tested. The analysis yielded several findings possibly specific for the Czech cohort. Somatic APC mutations did not cluster in the mutation cluster region (MCR. Tumours with MSI but no MLH1 methylation showed earlier onset and more severe mutational profiles compared to MSI tumours with MLH1 methylation. TP53 mutations were predominantly located outside the hot spots, and transitions were underrepresented. Our analysis supports the observation that germline MUTYH mutations are rare in Czech individuals with sporadic CRCs. Our findings suggest the influence of specific ethnic genetic factors and/or lifestyle and dietary habits typical for the Czech population on the development of these cancers.

When does ALS start? ADAR2-GluA2 hypothesis for the etiology of sporadic ALS

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Takuto eHideyama

2011-11-01

Full Text Available Amyotrophic lateral sclerosis (ALS is the most common adult-onset motor neuron disease. More than 90% of ALS cases are sporadic, and the majority of sporadic ALS patients do not carry mutations in genes causative of familial ALS; therefore, investigation specifically targeting sporadic ALS is needed to discover the pathogenesis. The motor neurons of sporadic ALS patients express unedited GluA2 mRNA at the Q/R site in a disease-specific and motor neuron-selective manner. GluA2 is a subunit of the AMPA receptor, and it has a regulatory role in the Ca2+-permeability of the AMPA receptor after the genomic Q codon is replaced with the R codon in mRNA by adenosine-inosine conversion, which is mediated by adenosine deaminase acting on RNA 2 (ADAR2. Therefore, ADAR2 activity may not be sufficient to edit all GluA2 mRNA expressed in the motor neurons of ALS patients. To investigate whether deficient ADAR2 activity plays pathogenic roles in sporadic ALS, we generated genetically modified mice (AR2 in which the ADAR2 gene was conditionally knocked out in the motor neurons. AR2 mice showed an ALS-like phenotype with the death of ADAR2-lacking motor neurons. Notably, the motor neurons deficient in ADAR2 survived when they expressed only edited GluA2 in AR2/GluR-BR/R (AR2res mice, in which the endogenous GluA2 alleles were replaced by the GluR-BR allele that encoded edited GluA2. In heterozygous AR2 mice with only one ADAR2 allele, approximately 20% of the spinal motor neurons expressed unedited GluA2 and underwent degeneration, indicating that half-normal ADAR2 activity is not sufficient to edit all GluA2 expressed in motor neurons. It is likely therefore that the expression of unedited GluA2 causes the death of motor neurons in sporadic ALS. We hypothesize that a progressive downregulation of ADAR2 activity plays a critical role in the pathogenesis of sporadic ALS and that the pathological process commences when motor neurons express unedited GluA2.

Lidar observation of Eyjafjallajoekull ash layer evolution above the Swiss Plateau

Science.gov (United States)

Simeonov, Valentin; Dinoev, Todor; Parlange, Mark; Serikov, Ilya; Calpini, Bertrand; Wienhold, F.; Engel, I.; Brabec, M.; Crisian, A.; Peter, T.; Mitev, Valentin; Matthey, R.

2010-05-01

The Iceland volcano Eyjafjallajökull started to emit significant amounts of volcanic ash and SO2 on 15th April 2010, following the initial eruption on 20th March 2010. In the next days, the ash was dispersed over large parts of Europe resulting in the closure of the major part of the European airspace. Information about spatial and temporal evolution of the cloud was needed urgently to define the conditions for opening the airspace. Satellite, airborne and ground observations together with meteorological models were used to evaluate the cloud propagation and evolution. While the horizontal extents of the volcanic cloud were accurately captured by satellite images, it remained difficult to obtain accurate information about the cloud base and top height, density and dynamics. During this event lidars demonstrated that they were the only ground based instruments allowing monitoring of the vertical distribution of the volcanic ash. Here we present observational results showing the evolution of the volcanic layer over the Swiss plateau. The measurements were carried out by one Raman lidar located in Payerne, two elastic lidars located in Neuchatel and Zurich, and a backscatter sonde launched from Zurich. The observations by the lidars have shown very similar time evolution, coherent with the backscatter sonde profiles and characterized by the appearance of the ash layer on the evening of 16th, followed by descend to 2-3 km during the next day and final mixing with the ABL on 19th. Simultaneous water vapor data from the Payerne lidar show low water content of the ash layer. The CSEM and EPFL gratefully acknowledge the financial support by the European Commission under grant RICA-025991.

Slower Dynamics and Aged Mitochondria in Sporadic Alzheimer's Disease

Science.gov (United States)

Gargini, Ricardo; García, Esther; Perry, George

2017-01-01

Sporadic Alzheimer's disease corresponds to 95% of cases whose origin is multifactorial and elusive. Mitochondrial dysfunction is a major feature of Alzheimer's pathology, which might be one of the early events that trigger downstream principal events. Here, we show that multiple genes that control mitochondrial homeostasis, including fission and fusion, are downregulated in Alzheimer's patients. Additionally, we demonstrate that some of these dysregulations, such as diminished DLP1 levels and its mitochondrial localization, as well as reduced STOML2 and MFN2 fusion protein levels, take place in fibroblasts from sporadic Alzheimer's disease patients. The analysis of mitochondrial network disruption using CCCP indicates that the patients' fibroblasts exhibit slower dynamics and mitochondrial membrane potential recovery. These defects lead to strong accumulation of aged mitochondria in Alzheimer's fibroblasts. Accordingly, the analysis of autophagy and mitophagy involved genes in the patients demonstrates a downregulation indicating that the recycling mechanism of these aged mitochondria might be impaired. Our data reinforce the idea that mitochondrial dysfunction is one of the key early events of the disease intimately related with aging. PMID:29201274

Molecular profile and copy number analysis of sporadic colorectal cancer in Taiwan

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Li Ling-Hui

2011-06-01

Full Text Available Abstract Background Colorectal cancer (CRC is a major health concern worldwide, and recently becomes the most common cancer in Asia. The case collection of this study is one of the largest sets of CRC in Asia, and serves as representative data for investigating genomic differences between ethnic populations. We took comprehensive and high-resolution approaches to compare the clinicopathologic and genomic profiles of microsatellite instability (MSI vs. microsatellite stability (MSS in Taiwanese sporadic CRCs. Methods 1,173 CRC tumors were collected from the Taiwan population, and sequencing-based microsatellite typing assay was used to determine MSI and MSS. Genome-wide SNP array was used to detect CN alterations in 16 MSI-H and 13 MSS CRCs and CN variations in 424 general controls. Gene expression array was used to evaluate the effects of CN alterations, and quantitative PCR methods were used to replicate the findings in independent clinical samples. Results These 1,173 CRC tumors can be classified into 75 high-frequency MSI (MSI-H (6.4%, 96 low-frequency MSI (8.2% and 1,002 MSS (85.4%. Of the 75 MSI-H tumors, 22 had a BRAF mutation and 51 showed MLH1 promoter hypermethylation. There were distinctive differences in the extent of CN alterations between CRC MSS and MSI-H subtypes (300 Mb vs. 42 Mb per genome, p-value Conclusions Sporadic CRCs with MSI-H displayed distinguishable clinicopathologic features, which differ from those of MSS. Genomic profiling of the two types of sporadic CRCs revealed significant differences in the extent and distribution of CN alterations in the cancer genome. More than half of expressed genes showing CN differences can directly contribute to their expressional diversities, and the biological functions of the genes associated with CN changes in sporadic CRCs warrant further investigation to establish their possible clinical implications.

A genome-wide investigation of copy number variation in patients with sporadic brain arteriovenous malformation.

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Nasrine Bendjilali

Full Text Available Brain arteriovenous malformations (BAVM are clusters of abnormal blood vessels, with shunting of blood from the arterial to venous circulation and a high risk of rupture and intracranial hemorrhage. Most BAVMs are sporadic, but also occur in patients with Hereditary Hemorrhagic Telangiectasia, a Mendelian disorder caused by mutations in genes in the transforming growth factor beta (TGFβ signaling pathway.To investigate whether copy number variations (CNVs contribute to risk of sporadic BAVM, we performed a genome-wide association study in 371 sporadic BAVM cases and 563 healthy controls, all Caucasian. Cases and controls were genotyped using the Affymetrix 6.0 array. CNVs were called using the PennCNV and Birdsuite algorithms and analyzed via segment-based and gene-based approaches. Common and rare CNVs were evaluated for association with BAVM.A CNV region on 1p36.13, containing the neuroblastoma breakpoint family, member 1 gene (NBPF1, was significantly enriched with duplications in BAVM cases compared to controls (P = 2.2×10(-9; NBPF1 was also significantly associated with BAVM in gene-based analysis using both PennCNV and Birdsuite. We experimentally validated the 1p36.13 duplication; however, the association did not replicate in an independent cohort of 184 sporadic BAVM cases and 182 controls (OR = 0.81, P = 0.8. Rare CNV analysis did not identify genes significantly associated with BAVM.We did not identify common CNVs associated with sporadic BAVM that replicated in an independent cohort. Replication in larger cohorts is required to elucidate the possible role of common or rare CNVs in BAVM pathogenesis.

TP53 mutations in ovarian carcinomas from sporadic cases and carriers of two distinct BRCA1 founder mutations; relation to age at diagnosis and survival

International Nuclear Information System (INIS)

Kringen, Pedro; Wang, Yun; Dumeaux, Vanessa; Kristensen, Gunnar; Borresen-Dale, Anne-Lise; Dorum, Anne

2005-01-01

Ovarian carcinomas from 30 BRCA1 germ-line carriers of two distinct high penetrant founder mutations, 20 carrying the 1675delA and 10 the 1135insA, and 100 sporadic cases were characterized for somatic mutations in the TP53 gene. We analyzed differences in relation to BRCA1 germline status, TP53 status, survival and age at diagnosis, as previous studies have not been conclusive. DNA was extracted from paraffin embedded formalin fixed tissues for the familial cases, and from fresh frozen specimen from the sporadic cases. All cases were treated at our hospital according to protocol. Mutation analyses of exon 2 – 11 were performed using TTGE, followed by sequencing. Survival rates for BRCA1-familial cases with TP53 mutations were not significantly lower than for familial cases without TP53 mutations (p = 0.25, RR = 1.64, 95% CI [0.71–3.78]). Median age at diagnosis for sporadic (59 years) and familial (49 years) cases differed significantly (p < 0.001) with or without TP53 mutations. Age at diagnosis between the two types of familial carriers were not significantly different, with median age of 47 for 1675delA and 52.5 for 1135insA carriers (p = 0.245). For cases ≥50 years at diagnosis, a trend toward longer survival for sporadic over familial cases was observed (p = 0.08). The opposite trend was observed for cases <50 years at diagnosis. There do not seem to be a protective advantage for familial BRCA1 carriers without TP53 mutations over familial cases with TP53 mutations. However, there seem to be a trend towards initial advantage in survival for familial cases compared to sporadic cases diagnosed before the age of 50 both with and without TP53 mutations. However, this trend diminishes over time and for cases diagnosed ≥50 years the sporadic cases show a trend towards an advantage in survival over familial cases. Although this data set is small, if confirmed, this may be a link in the evidence that the differences in ovarian cancer survival reported, are

Cortical restricted diffusion as the predominant MRI finding in sporadic Creutzfeldt-Jakob disease

Energy Technology Data Exchange (ETDEWEB)

Talbott, Sabrina D.; Sattenberg, Ronald J.; Heidenreich, Jens O. (Dept. of Radiology, Univ. of Louisville, Louisville (United States)), e-mail: sdtalb02@gwise.louisville.edu; Plato, Brian M (Dept. of Neurology, Univ. of Louisville, Louisville (United States)); Parker, John (Dept. of Pathology and Laboratory Medicine, Univ. of Louisville, Louisville (United States))

2011-04-15

Creutzfeldt-Jakob disease is a rare and fatal neurodegenerative disorder with MR findings predominantly limited to the grey matter of the cortex and the basal ganglia. Sporadic Creutzfeldt-Jakob disease can produce a spectrum of MR imaging findings of the brain, most notably on DWI and FLAIR sequences. Involvement of the basal ganglia and neocortex is the most common finding, but isolated involvement of the cortex can also be seen. We describe the clinical history and MRI findings of three patients with sporadic Creutzfeldt-Jakob disease confirmed by brain biopsy or autopsy and review the literature of imaging manifestations of this disease

Cortical restricted diffusion as the predominant MRI finding in sporadic Creutzfeldt-Jakob disease

International Nuclear Information System (INIS)

Talbott, Sabrina D.; Sattenberg, Ronald J.; Heidenreich, Jens O.; Plato, Brian M; Parker, John

2011-01-01

Creutzfeldt-Jakob disease is a rare and fatal neurodegenerative disorder with MR findings predominantly limited to the grey matter of the cortex and the basal ganglia. Sporadic Creutzfeldt-Jakob disease can produce a spectrum of MR imaging findings of the brain, most notably on DWI and FLAIR sequences. Involvement of the basal ganglia and neocortex is the most common finding, but isolated involvement of the cortex can also be seen. We describe the clinical history and MRI findings of three patients with sporadic Creutzfeldt-Jakob disease confirmed by brain biopsy or autopsy and review the literature of imaging manifestations of this disease

The Southern Argentina Agile Meteor Radar Orbital System (SAAMER-OS): An Initial Sporadic Meteoroid Orbital Survey in the Southern Sky

Science.gov (United States)

Janches, D.; Close, S.; Hormaechea, J. L.; Swarnalingam, N.; Murphy, A.; O'Connor, D.; Vandepeer, B.; Fuller, B.; Fritts, D. C.; Brunini, C.

2015-01-01

We present an initial survey in the southern sky of the sporadic meteoroid orbital environment obtained with the Southern Argentina Agile MEteor Radar (SAAMER) Orbital System (OS), in which over three-quarters of a million orbits of dust particles were determined from 2012 January through 2015 April. SAAMER-OS is located at the southernmost tip of Argentina and is currently the only operational radar with orbit determination capability providing continuous observations of the southern hemisphere. Distributions of the observed meteoroid speed, radiant, and heliocentric orbital parameters are presented, as well as those corrected by the observational biases associated with the SAAMER-OS operating parameters. The results are compared with those reported by three previous surveys performed with the Harvard Radio Meteor Project, the Advanced Meteor Orbit Radar, and the Canadian Meteor Orbit Radar, and they are in agreement with these previous studies. Weighted distributions for meteoroids above the thresholds for meteor trail electron line density, meteoroid mass, and meteoroid kinetic energy are also considered. Finally, the minimum line density and kinetic energy weighting factors are found to be very suitable for meteoroid applications. The outcomes of this work show that, given SAAMERs location, the system is ideal for providing crucial data to continuously study the South Toroidal and South Apex sporadic meteoroid apparent sources.

The detection of the Earth rotation and revolution effect using the daily and annual variation of sporadic meteor echo by HRO

OpenAIRE

OHNISHI, Kouji; HATTORI, Shinobu; NISHIMURA, Osamu; ISHIKAWA, Toshiyuki; AOKI, Yoshie; IIJIMA, Yukiko; KOBAYASHI, Aya; MAEGAWA, Kimio; ABE, Shinsuke

2001-01-01

Ham-band Radio Observation (HRO) is one of the observational techniques for the forward scatter observation of meteors. We started the observation of the daily and annual variation of sporadic meteor echoes with paired two-element loop antennas (F/B ratio is 10 dB) at the Nagano National College of Technology (Nagano, JAPAN) using the beacon signals at 53.750MHz, 50W from Fukui National College of Technology (Sabae, Fukui, JAPAN) from Aug.2000. The direction of one of this paired antenna was ...

An observational study of the evolution of the atmospheric boundary-layer over Cabo Frio, Brazil

Directory of Open Access Journals (Sweden)

S. H. Franchito

2007-08-01

Full Text Available The effect of coastal upwelling on the evolution of the atmospheric boundary layer (ABL in Cabo Frio (Brazil is investigated. For this purpose, radiosounding data collected in two experiments made during the austral summer (upwelling case and austral winter (no upwelling case are analysed. The results show that during the austral summer, cold waters that crop up near the Cabo Frio coast favour the formation of an atmospheric stable layer, which persists during the upwelling episode. Due to the low SSTs, the descending branch of the sea-breeze circulation is located close to the coast, inhibiting the development of a mixed layer mainly during the day. At night, with the reduction of the land-sea thermal contrast the descending motion is weaker, allowing a vertical mixing. The stable ABL favours the formation of a low level jet, which may also contribute to the development of a nocturnal atmospheric mixed layer. During the austral winter, due to the higher SSTs observed near the coast, the ABL is less stable compared with that in the austral summer. Due to warming, a mixed layer is observed during the day. The observed vertical profiles of the zonal winds show that the easterlies at low levels are stronger in the austral summer, indicating that the upwelling modulates the sea-breeze signal, thus confirming model simulations.

Novel Genetic Variants of Sporadic Atrial Septal Defect (ASD) in a Chinese Population Identified by Whole-Exome Sequencing (WES).

Science.gov (United States)

Liu, Yong; Cao, Yu; Li, Yaxiong; Lei, Dongyun; Li, Lin; Hou, Zong Liu; Han, Shen; Meng, Mingyao; Shi, Jianlin; Zhang, Yayong; Wang, Yi; Niu, Zhaoyi; Xie, Yanhua; Xiao, Benshan; Wang, Yuanfei; Li, Xiao; Yang, Lirong; Wang, Wenju; Jiang, Lihong

2018-03-05

BACKGROUND Recently, mutations in several genes have been described to be associated with sporadic ASD, but some genetic variants remain to be identified. The aim of this study was to use whole-exome sequencing (WES) combined with bioinformatics analysis to identify novel genetic variants in cases of sporadic congenital ASD, followed by validation by Sanger sequencing. MATERIAL AND METHODS Five Han patients with secundum ASD were recruited, and their tissue samples were analyzed by WES, followed by verification by Sanger sequencing of tissue and blood samples. Further evaluation using blood samples included 452 additional patients with sporadic secundum ASD (212 male and 240 female patients) and 519 healthy subjects (252 male and 267 female subjects) for further verification by a multiplexed MassARRAY system. Bioinformatic analyses were performed to identify novel genetic variants associated with sporadic ASD. RESULTS From five patients with sporadic ASD, a total of 181,762 genomic variants in 33 exon loci, validated by Sanger sequencing, were selected and underwent MassARRAY analysis in 452 patients with ASD and 519 healthy subjects. Three loci with high mutation frequencies, the 138665410 FOXL2 gene variant, the 23862952 MYH6 gene variant, and the 71098693 HYDIN gene variant were found to be significantly associated with sporadic ASD (PASD (PASD, and supported the use of WES and bioinformatics analysis to identify disease-associated mutations.

Relationship between blood harmane and harmine concentrations in familial essential tremor, sporadic essential tremor and controls.

Science.gov (United States)

Louis, Elan D; Jiang, Wendy; Gerbin, Marina; Mullaney, Mary M; Zheng, Wei

2010-12-01

Harmane, a potent tremor-producing β-carboline alkaloid, may play a role in the etiology of essential tremor (ET). Blood harmane concentrations are elevated in ET cases compared with controls yet the basis for this elevation remains unknown. Decreased metabolic conversion (harmane to harmine) is one possible explanation. Using a sample of >500 individuals, we hypothesized that defective metabolic conversion of harmane to harmine might underlie the observed elevated harmane concentration in ET, and therefore expected to find a higher harmane to harmine ratio in familial ET than in sporadic ET or controls. Blood harmane and harmine concentrations were quantified by high performance liquid chromatography. There were 78 familial ET cases, 187 sporadic ET cases, and 276 controls. Blood harmane and harmine concentrations were correlated with one another (Spearman's r=0.24, p<0.001). The mean (±SD) harmane/harmine ratio=23.4±90.9 (range=0.1-987.5). The harmane/harmine ratio was highest in familial ET (46.7±140.4), intermediate in sporadic ET (28.3±108.1), and lowest in controls (13.5±50.3) (p=0.03). In familial ET cases, there was no association between this ratio and tremor severity (Spearman's r=0.08, p=0.48) or tremor duration (Spearman's r=0.14, p=0.24). The basis for the elevated blood harmane concentration, particularly in familial ET, is not known, although the current findings (highest harmane/harmine ratio in familial ET cases) lends support to the possibility that it could be the result of a genetically-driven reduction in harmane metabolism. Copyright © 2010 Elsevier Inc. All rights reserved.

Clinicopathologic factors identify sporadic mismatch repair-defective colon cancers

DEFF Research Database (Denmark)

Halvarsson, Britta; Anderson, Harald; Domanska, Katarina

2008-01-01

Identification of sporadic mismatch repair (MMR)-defective colon cancers is increasingly demanded for decisions on adjuvant therapies. We evaluated clinicopathologic factors for the identification of these prognostically favorable tumors. Histopathologic features in 238 consecutive colon cancers...... and excluded 61.5% of the tumors from MMR testing. This clinicopathologic index thus successfully selects MMR-defective colon cancers. Udgivelsesdato: 2008-Feb...

Differences in histological features and PD-L1 expression between sporadic microsatellite instability and Lynch-syndrome-associated disease in Japanese patients with colorectal cancer.

Science.gov (United States)

Yamada, Rin; Yamaguchi, Tatsuro; Iijima, Takeru; Wakaume, Rika; Takao, Misato; Koizumi, Koichi; Hishima, Tsunekazu; Horiguchi, Shin-Ichiro

2018-06-01

The field of immunotherapy has recently focused on cancers with microsatellite instability (MSI). These cancers include both Lynch-syndrome-associated tumors, which are caused by mismatch repair (MMR) germline mutations, and sporadic MSI tumors, which are mainly attributed to MLH1 promoter methylation. The present study aimed to clarify differences in the histological and PD-L1 expression profiles between these two types of MSI cancers in Japanese patients. Among 908 cases of colorectal cancer treated via surgical resection from 2008 to 2014, we identified 64 MSI cancers, including 36 sporadic MSI and 28 Lynch-syndrome-associated cancers, using a BRAF V600E mutation analysis and MLH1 methylation analysis. Of the latter subgroup, 21 (75%) harbored MMR germline mutations. The following were more frequent with sporadic MSI than with Lynch syndrome associated cancers: poor differentiation (50.0 vs. 7.1%, P = 0.0002), especially solid type (30.6 vs. 3.6%, P = 0.0061); medullary morphology (19.4 and 0%, P = 0.015), Crohn-like lymphoid reaction (50.0 vs. 25.0%, P = 0.042), and PD-L1 expression (25.0 vs. 3.6%, P = 0.034). However, the groups did not differ in terms of the mean invasive front and intratumoral CD8-positive cell densities. In a logistic regression analysis, PD-L1 expression correlated with poor differentiation (odds ratio: 7.65, 95% confidence interval: 1.55-37.7, P = 0.012), but not with the difference between sporadic MSI cancer and Lynch-syndrome-associated cancer (odds ratio: 4.74, 95% confidence interval: 0.50-45.0, P = 0.176). Therefore, compared with Lynch-syndrome-associated cancers, sporadic MSI cancers are more frequently solid, poorly differentiated medullary cancers that express PD-L1.

An Experiment to Study Sporadic Sodium Layers in the Earth's Mesosphere and Lower Thermosphere

Science.gov (United States)

Swenson, Charles M.

2002-01-01

The Utah State University / Space Dynamics Lab was funded under a NASA Grant. This investigation has been part of Rockwell Universities Sudden Atom Layer Investigation (SAL). USU/SDL provided an electron density measurement instrument, the plasma frequency probe, which was launched on the vehicle 21.117 from Puerto-Rico in February of 1998. The instrument successfully measured electron density as designed and measurement techniques included in this version of the Plasma Frequency probe provided valuable insight into the electron density structures associated with sudden sodium layers in a collisional plasma. Electron density data was furnished to Rockwell University but no science meetings were held by Rockwell Data from the instrument was presented to the scientific community at the URSI General Session in 1999. A paper is in preparation for publication in Geophysical Research Letters. The following document provides a summary of the experiment and data obtained as a final report on this grant.

Measuring sporadic gastrointestinal illness associated with drinking water - an overview of methodologies.

Science.gov (United States)

Bylund, John; Toljander, Jonas; Lysén, Maria; Rasti, Niloofar; Engqvist, Jannes; Simonsson, Magnus

2017-06-01

There is an increasing awareness that drinking water contributes to sporadic gastrointestinal illness (GI) in high income countries of the northern hemisphere. A literature search was conducted in order to review: (1) methods used for investigating the effects of public drinking water on GI; (2) evidence of possible dose-response relationship between sporadic GI and drinking water consumption; and (3) association between sporadic GI and factors affecting drinking water quality. Seventy-four articles were selected, key findings and information gaps were identified. In-home intervention studies have only been conducted in areas using surface water sources and intervention studies in communities supplied by ground water are therefore needed. Community-wide intervention studies may constitute a cost-effective alternative to in-home intervention studies. Proxy data that correlate with GI in the community can be used for detecting changes in the incidence of GI. Proxy data can, however, not be used for measuring the prevalence of illness. Local conditions affecting water safety may vary greatly, making direct comparisons between studies difficult unless sufficient knowledge about these conditions is acquired. Drinking water in high-income countries contributes to endemic levels of GI and there are public health benefits for further improvements of drinking water safety.

Observation of η′ →ωe+e-

NARCIS (Netherlands)

Ablikim, M.; Achasov, M. N.; Ai, X. C.; Albayrak, O.; Albrecht, M.; Ambrose, D. J.; Amoroso, A.; An, F. F.; An, Q.; Bai, J. Z.; Ferroli, R. Baldini; Ban, Y.; Bennett, D. W.; Bennett, J. V.; Bertani, M.; Bettoni, D.; Bian, J. M.; Bianchi, F.; Boger, E.; Boyko, I.; Briere, R. A.; Cai, H.; Cai, X.; Cakir, O.; Calcaterra, A.; Cao, G. F.; Cetin, S. A.; Chang, J. F.; Chelkov, G.; Chen, G.; Chen, H. S.; Chen, H. Y.; Chen, J. C.; Chen, M. L.; Chen, S. J.; Chen, X.; Chen, X. R.; Chen, Y. B.; Cheng, H. P.; Chu, X. K.; Cibinetto, G.; Dai, H. L.; Dai, J. P.; Dbeyssi, A.; Dedovich, D.; Deng, Z. Y.; Denig, A.; Denysenko, I.; Destefanis, M.; De Mori, F.; Ding, Y.; Dong, C.; Dong, J.; Dong, L. Y.; Dong, M. Y.; Du, S. X.; Duan, P. F.; Eren, E. E.; Fan, J. Z.; Fang, J.; Fang, S. S.; Fang, X.; Fang, Y.; Fava, L.; Feldbauer, F.; Felici, G.; Feng, C. Q.; Fioravanti, E.; Fritsch, M.; Fu, C. D.; Gao, Q.; Gao, X. Y.; Gao, Y.; Gao, Z.; Garzia, I.; Goetzen, K.; Gong, W. X.; Gradl, W.; Greco, M.; Gu, M. H.; Gu, Y. T.; Guan, Y. H.; Guo, A. Q.; Guo, L. B.; Guo, Y.; Guo, Y. P.; Haddadi, Z.; Hafner, A.; Han, S.; Hao, X. Q.; Harris, F. A.; He, K. L.; He, X. Q.; Held, T.; Heng, Y. K.; Hou, Z. L.; Hu, C.; Hu, H. M.; Hu, J. F.; Hu, T.; Hu, Y.; Huang, G. M.; Huang, G. S.; Huang, J. S.; Huang, X. T.; Huang, Y.; Hussain, T.; Ji, Q.; Ji, Q. P.; Ji, X. B.; Ji, X. L.; Jiang, L. W.; Jiang, X. S.; Jiang, X. Y.; Jiao, J. B.; Jiao, Z.; Jin, D. P.; Jin, S.; Johansson, T.; Julin, A.; Kalantar-Nayestanaki, N.; Kang, X. L.; Kang, X. S.; Kavatsyuk, M.; Ke, B. C.; Kiese, P.; Kliemt, R.; Kloss, B.; Kolcu, O. B.; Kopf, B.; Kornicer, M.; Kühn, W.; Kupsc, A.; Lange, J. S.; Lara, M.; Larin, P.; Leng, C.; Li, C.; Li, Cheng; Li, D. M.; Li, F.; Li, F. Y.; Li, G.; Li, H. B.; Li, J. C.; Li, Jin; Li, K.; Li, K.; Li, Lei; Li, P. R.; Li, T.; Li, W. D.; Li, W. G.; Li, X. L.; Li, X. M.; Li, X. N.; Li, X. Q.; Li, Z. B.; Liang, H.; Liang, Y. F.; Liang, Y. T.; Liao, G. R.; Lin, D. X.; Liu, B. J.; Liu, C. X.; Liu, F. H.; Liu, Fang; Liu, Feng; Liu, H. B.; Liu, H. H.; Liu, H. H.; Liu, H. M.; Liu, J.; Liu, J. B.; Liu, J. P.; Liu, J. Y.; Liu, K.; Liu, K. Y.; Liu, L. D.; Liu, P. L.; Liu, Q.; Liu, S. B.; Liu, X.; Liu, Y. B.; Liu, Z. A.; Liu, Zhiqing; Loehner, Herbert; Lou, X. C.; Lu, H. J.; Lu, J. G.; Lu, Y.; Lu, Y. P.; Luo, C. L.; Luo, M. X.; Luo, T.; Luo, X. L.; Lyu, X. R.; Ma, F. C.; Ma, H. L.; Ma, L. L.; Ma, Q. M.; Ma, T.; Ma, X. N.; Ma, X. Y.; Maas, F. E.; Maggiora, M.; Mao, Y. J.; Mao, Z. P.; Marcello, S.; Messchendorp, J. G.; Min, J.; Mitchell, R. E.; Mo, X. H.; Mo, Y. J.; Morales, C. Morales; Moriya, K.; Muchnoi, N. Yu; Muramatsu, H.; Nefedov, Y.; Nerling, F.; Nikolaev, I. B.; Ning, Z.; Nisar, S.; Niu, S. L.; Niu, X. Y.; Olsen, S. L.; Ouyang, Q.; Pacetti, S.; Patteri, P.; Pelizaeus, M.; Peng, H. P.; Peters, K.; Pettersson, J.; Ping, J. L.; Ping, R. G.; Poling, R.; Prasad, V.; Qi, M.; Qian, S.; Qiao, C. F.; Qin, L. Q.; Qin, N.; Qin, X. S.; Qin, Z. H.; Qiu, J. F.; Rashid, K. H.; Redmer, C. F.; Ripka, M.; Rong, G.; Rosner, Ch; Ruan, X. D.; Santoro, V.; Sarantsev, A.; Savrié, M.; Schoenning, K.; Schumann, S.; Shan, W.; Shao, M.; Shen, C. P.; Shen, P. X.; Shen, X. Y.; Sheng, H. Y.; Song, W. M.; Song, X. Y.; Sosio, S.; Spataro, S.; Sun, G. X.; Sun, J. F.; Sun, S. S.; Sun, Y. J.; Sun, Y. Z.; Sun, Z. J.; Sun, Z. T.; Tang, C. J.; Tang, X.; Tapan, I.; Thorndike, E. H.; Tiemens, M.; Ullrich, M.; Uman, I.; Varner, G. S.; Wang, B.; Wang, D.; Wang, D. Y.; Wang, K.; Wang, L. L.; Wang, L. S.; Wang, M.; Wang, P.; Wang, P. L.; Wang, S. G.; Wang, W.; Wang, X. F.; Wang, Y. D.; Wang, Y. F.; Wang, Y. Q.; Wang, Z.; Wang, Z. G.; Wang, Z. H.; Wang, Z. Y.; Weber, T.; Wei, D. H.; Wei, J. B.; Weidenkaff, P.; Wen, S. P.; Wiedner, U.; Wolke, M.; Wu, L. H.; Wu, Z.; Xia, L. G.; Xia, Y.; Xiao, D.; Xiao, H.; Xiao, Z. J.; Xie, Y. G.; Xiu, Q. L.; Xu, G. F.; Xu, L.; Xu, Q. J.; Xu, X. P.; Yan, L.; Yan, W. B.; Yan, W. C.; Yan, Y. H.; Yang, H. J.; Yang, H. X.; Yang, L.; Yang, Y.; Yang, Y. X.; Ye, M.; Ye, M. H.; Yin, J. H.; Yu, B. X.; Yu, C. X.; Yu, J. S.; Yuan, C. Z.; Yuan, W. L.; Yuan, Y.; Yuncu, A.; Zafar, A. A.; Zallo, A.; Zeng, Y.; Zhang, B. X.; Zhang, B. Y.; Zhang, C.; Zhang, C. C.; Zhang, D. H.; Zhang, H. H.; Zhang, H. Y.; Zhang, J. J.; Zhang, J. L.; Zhang, J. Q.; Zhang, J. W.; Zhang, J. Y.; Zhang, J. Z.; Zhang, K.; Zhang, L.; Zhang, X. Y.; Zhang, Y.; Zhang, Y. N.; Zhang, Y. H.; Zhang, Y. T.; Zhang, Yu; Zhang, Z. H.; Zhang, Z. P.; Zhang, Z. Y.; Zhao, G.; Zhao, J. W.; Zhao, J. Y.; Zhao, J. Z.; Zhao, Lei; Zhao, Ling; Zhao, M. G.; Zhao, Q.; Zhao, Q. W.; Zhao, S. J.; Zhao, T. C.; Zhao, Y. B.; Zhao, Z. G.; Zhemchugov, A.; Zheng, B.; Zheng, J. P.; Zheng, W. J.; Zheng, Y. H.; Zhong, B.; Zhou, L.; Zhou, X.; Zhou, X. K.; Zhou, X. R.; Zhou, X. Y.; Zhu, K.; Zhu, K. J.; Zhu, S.; Zhu, S. H.; Zhu, X. L.; Zhu, Y. C.; Zhu, Y. S.; Zhu, Z. A.; Zhuang, J.; Zotti, L.; Zou, B. S.; Zou, J. H.

2015-01-01

Based on a sample of η′ mesons produced in the radiative decay J/ψ→γη′ in 1.31×109 J/ψ events collected with the BESIII detector, the decay η′→ωe+e- is observed for the first time, with a statistical significance of 8σ. The branching fraction is measured to be

Computed tomographic features of 23 sporadic cases with Legionella pneumophila pneumonia

International Nuclear Information System (INIS)

Yu Hui; Higa, Futoshi; Hibiya, Kenji; Furugen, Makoto; Sato, Yoko; Shinzato, Takashi; Haranaga, Shusaku; Yara, Satomi; Tateyama, Masao; Fujita, Jiro; Li, Huiping

2010-01-01

Objective: To describe the chest computed tomographic (CT) findings of Legionella pneumophila pneumonia. Methods: CT scans obtained from 23 sporadic cases of L. pneumophila pneumonia were retrospectively reviewed. Chest CT findings were analyzed with regard to the patterns and distributions of pulmonary abnormalities. We also analyzed the histopathology of lungs from guinea pigs with experimentally induced L. pneumophila pneumonia. Results: Consolidation and ground-glass opacity (GGO) were the main findings of CT scans in L. pneumophila pneumonia. The distribution of opacities was categorized as non-segmental (n = 20) and segmental (n = 4). Non-segmental distribution may follow an onset of segmental distribution. Pleural effusion was observed in 14 (58.3%) patients, of which 13 were accompanied with non-segmental distribution. Abscess formation was observed in only one immunocompromised patient. In the animal pneumonia model, the lesions comprised of terminal bronchioles, alveolar spaces, and interstitia. Small bacilli were observed to be contained by many macrophages within the alveoli. Conclusion: Non-segmental distribution was significantly more frequent than segmental distribution in L. pneumophila pneumonia. It is possible that L. pneumophila infection initially results in segmental pneumonia, which progresses to typical non-segmental distribution.

Computed tomographic features of 23 sporadic cases with Legionella pneumophila pneumonia

Energy Technology Data Exchange (ETDEWEB)

Yu Hui [Department of Respiratory Diseases, Shanghai Pneumology Hospital, Tongji University, Shanghai (China); Higa, Futoshi; Hibiya, Kenji; Furugen, Makoto [Department of Medicine and Therapeutics, Control and Prevention of Infectious Diseases (First Department of Internal Medicine), Faculty of Medicine, University of the Ryukyus, Okinawa (Japan); Sato, Yoko [Tomishiro Chuo Hospital, Okinawa (Japan); Shinzato, Takashi [Nakagami General Hospital, Okinawa (Japan); Haranaga, Shusaku; Yara, Satomi; Tateyama, Masao [Department of Medicine and Therapeutics, Control and Prevention of Infectious Diseases (First Department of Internal Medicine), Faculty of Medicine, University of the Ryukyus, Okinawa (Japan); Fujita, Jiro, E-mail: fujita@med.u-ryukyu.ac.j [Department of Medicine and Therapeutics, Control and Prevention of Infectious Diseases (First Department of Internal Medicine), Faculty of Medicine, University of the Ryukyus, Okinawa (Japan); Li, Huiping [Department of Respiratory Diseases, Shanghai Pneumology Hospital, Tongji University, Shanghai (China)

2010-06-15

Objective: To describe the chest computed tomographic (CT) findings of Legionella pneumophila pneumonia. Methods: CT scans obtained from 23 sporadic cases of L. pneumophila pneumonia were retrospectively reviewed. Chest CT findings were analyzed with regard to the patterns and distributions of pulmonary abnormalities. We also analyzed the histopathology of lungs from guinea pigs with experimentally induced L. pneumophila pneumonia. Results: Consolidation and ground-glass opacity (GGO) were the main findings of CT scans in L. pneumophila pneumonia. The distribution of opacities was categorized as non-segmental (n = 20) and segmental (n = 4). Non-segmental distribution may follow an onset of segmental distribution. Pleural effusion was observed in 14 (58.3%) patients, of which 13 were accompanied with non-segmental distribution. Abscess formation was observed in only one immunocompromised patient. In the animal pneumonia model, the lesions comprised of terminal bronchioles, alveolar spaces, and interstitia. Small bacilli were observed to be contained by many macrophages within the alveoli. Conclusion: Non-segmental distribution was significantly more frequent than segmental distribution in L. pneumophila pneumonia. It is possible that L. pneumophila infection initially results in segmental pneumonia, which progresses to typical non-segmental distribution.

CP violating observables in e$^{-}$e$^{+}$ --> W$^{-}$W$^{+}$

CERN Document Server

Chang, D; Phillips, I

1993-01-01

We consider various integrated lepton charge-energy asymmetries and azimuthal asymmetries as tests of CP violation in the process $e^-e^+ \\to W^-W^+$. These asymmetries are sensitive to different linear combinations of the CP violating form factors in the three gauge boson $W^-W^+$ production vertex, and can distinguish dispersive and absorptive parts of the form factors. It makes use of purely hadronic and purely leptonic modes of $W$'s decays as well as the mixed modes. The techniques of using the kinematics of jets or missing momentum to construct CP--odd observables are also employed. These CP violating observables are illustrated in the generalized Left-Right Model and the Charged Higgs Model.

MRI of sporadic Creutzfeldt-Jakob disease

International Nuclear Information System (INIS)

Kong, A.; Vliet, A. Van der.

2008-01-01

Full text: The key MRI findings in five cases of sporadic Creutzfeldt-Jakob disease (CJD) are illustrated with four 'definite' and one 'probable' according to World Health Organization criteria. Close attention to fluid-attenuation inversion recovery and diffusion-weighted imaging sequences are important for diagnosis, noting especially restricted diffusion in cortical and deep grey matter. Our study and those of others show predominant cortical, caudate and thalamic involvement. This pattern is highly sensitive and specific for the diagnosis. Fluid-attenuation inversion recovery and diffusion-weighted imaging signal abnormality becomes progressively more extensive and bilateral as disease progresses, but may become less pronounced in end-stage disease because of atrophy.

Observation of anisotropic interlayer Raman modes in few-layer ReS{sub 2}

Energy Technology Data Exchange (ETDEWEB)

Nagler, Philipp; Plechinger, Gerd; Schueller, Christian; Korn, Tobias [Institut fuer Experimentelle und Angewandte Physik, Universitaet Regensburg, 93040, Regensburg (Germany)

2016-02-15

ReS{sub 2} has recently emerged as a new member in the rapidly growing family of two-dimensional materials. Unlike MoS{sub 2} or WSe{sub 2}, the optical and electrical properties of ReS{sub 2} are not isotropic due to the reduced symmetry of the crystal. Here, we present layer-dependent Raman measurements of ReS{sub 2} samples ranging from monolayers to ten layers in the ultralow frequency regime. We observe layer breathing and shear modes which allow for easy assignment of the number of layers. Polarization-dependent measurements give further insight into the crystal structure and reveal an energetic shift of the shear mode which stems from the in-plane anisotropy of the shear modulus in this material. (copyright 2016 WILEY-VCH Verlag GmbH and Co. KGaA, Weinheim)

Sporadic Endolymphatic Sac Tumor-A Very Rare Cause of Hearing Loss, Tinnitus, and Dizziness

DEFF Research Database (Denmark)

Schnack, Didde Trærup; Kiss, Katalin; Hansen, Søren

2017-01-01

Sporadic endolymphatic sac tumor is a very rare neoplasm. It is low malignant, locally destructive and expansive, but non-metastasizing. The tumor is very rare in the sporadic form, but more often associated with Von Hippel-Lindau disease. A 65-year old man with left sided tinnitus and hearing loss......-operative freeze-microscopy showed inflammation tissue, whereas subsequent microscopy showed papillary-cystic endolymphatic sac tumor. Endolymphatic sac tumor is a rare neoplasm. The tumor may present with asymmetrically sensory neural hearing loss with or without tinnitus, dizziness and facial nerve paresis...

Haplotype analysis of common variants in the BRCA1 gene and risk of sporadic breast cancer

International Nuclear Information System (INIS)

Cox, David G; Kraft, Peter; Hankinson, Susan E; Hunter, David J

2005-01-01

Truncation mutations in the BRCA1 gene cause a substantial increase in risk of breast cancer. However, these mutations are rare in the general population and account for little of the overall incidence of sporadic breast cancer. We used whole-gene resequencing data to select haplotype tagging single nucleotide polymorphisms, and examined the association between common haplotypes of BRCA1 and breast cancer in a nested case-control study in the Nurses' Health Study (1323 cases and 1910 controls). One haplotype was associated with a slight increase in risk (odds ratio 1.18, 95% confidence interval 1.02–1.37). A significant interaction (P = 0.05) was seen between this haplotype, positive family history of breast cancer, and breast cancer risk. Although not statistically significant, similar interactions were observed with age at diagnosis and with menopausal status at diagnosis; risk tended to be higher among younger, pre-menopausal women. We have described a haplotype in the BRCA1 gene that was associated with an approximately 20% increase in risk of sporadic breast cancer in the general population. However, the functional variant(s) responsible for the association are unclear

The first report of RPSA polymorphisms, also called 37/67 kDa LRP/LR gene, in sporadic Creutzfeldt-Jakob disease (CJD

Directory of Open Access Journals (Sweden)

Jeong Byung-Hoon

2011-08-01

Full Text Available Abstract Background Although polymorphisms of PRNP, the gene encoding prion protein, are known as a determinant affecting prion disease susceptibility, other genes also influence prion incubation time. This finding offers the opportunity to identify other genetic or environmental factor (s modulating susceptibility to prion disease. Ribosomal protein SA (RPSA, also called 37 kDa laminin receptor precursor (LRP/67 kDa laminin receptor (LR, acts as a receptor for laminin, viruses and prion proteins. The binding/internalization of prion protein is dependent for LRP/LR. Methods To identify other susceptibility genes involved in prion disease, we performed genetic analysis of RPSA. For this case-control study, we included 180 sporadic Creutzfeldt-Jakob disease (CJD patients and 189 healthy Koreans. We investigated genotype and allele frequencies of polymorphism on RPSA by direct sequencing or restriction fragment length polymorphism (RFLP analysis. Results We observed four single nucleotide polymorphisms (SNPs, including -8T>C (rs1803893 in the 5'-untranslated region (UTR of exon 2, 134-32C>T (rs3772138 in the intron, 519G>A (rs2269350 in the intron and 793+58C>T (rs2723 in the intron on the RPSA. The 519G>A (at codon 173 is located in the direct PrP binding site. The genotypes and allele frequencies of the RPSA polymorphisms showed no significant differences between the controls and sporadic CJD patients. Conclusion These results suggest that these RPSA polymorphisms have no direct influence on the susceptibility to sporadic CJD. This was the first genetic association study of the polymorphisms of RPSA gene with sporadic CJD.

Physical function and muscle strength in sporadic inclusion body myositis

DEFF Research Database (Denmark)

Jørgensen, Anders N; Aagaard, Per; Nielsen, Jakob L

2017-01-01

INTRODUCTION: In this study, self-reported physical function, functional capacity, and isolated muscle function were investigated in sporadic inclusion body myositis (sIBM) patients. METHODS: The 36-item Short Form (SF-36) Health Survey and 2-min walk test (2MWT), timed up & go test (TUG), and 30-s...

Sporadic adult onset primary torsion dystonia is a genetic disorder by the temporal discrimination test.

LENUS (Irish Health Repository)

Kimmich, Okka

2012-02-01

Adult-onset primary torsion dystonia is an autosomal dominant disorder with markedly reduced penetrance; patients with sporadic adult-onset primary torsion dystonia are much more prevalent than familial. The temporal discrimination threshold is the shortest time interval at which two stimuli are detected to be asynchronous and has been shown to be abnormal in adult-onset primary torsion dystonia. The aim was to determine the frequency of abnormal temporal discrimination thresholds in patients with sporadic adult-onset primary torsion dystonia and their first-degree relatives. We hypothesized that abnormal temporal discrimination thresholds in first relatives would be compatible with an autosomal dominant endophenotype. Temporal discrimination thresholds were examined in 61 control subjects (39 subjects <50 years of age; 22 subjects >50 years of age), 32 patients with sporadic adult-onset primary torsion dystonia (cervical dystonia n = 30, spasmodic dysphonia n = 1 and Meige\\'s syndrome n = 1) and 73 unaffected first-degree relatives (36 siblings, 36 offspring and one parent) using visual and tactile stimuli. Z-scores were calculated for all subjects; a Z > 2.5 was considered abnormal. Abnormal temporal discrimination thresholds were found in 1\\/61 (2%) control subjects, 27\\/32 (84%) patients with adult-onset primary torsion dystonia and 32\\/73 (44%) unaffected relatives [siblings (20\\/36; 56%), offspring (11\\/36; 31%) and one parent]. When two or more relatives were tested in any one family, 22 of 24 families had at least one first-degree relative with an abnormal temporal discrimination threshold. The frequency of abnormal temporal discrimination thresholds in first-degree relatives of patients with sporadic adult-onset primary torsion dystonia is compatible with an autosomal dominant disorder and supports the hypothesis that apparently sporadic adult-onset primary torsion dystonia is genetic in origin.

Sporadic adult onset primary torsion dystonia is a genetic disorder by the temporal discrimination test.

Science.gov (United States)

Kimmich, Okka; Bradley, David; Whelan, Robert; Mulrooney, Nicola; Reilly, Richard B; Hutchinson, Siobhan; O'Riordan, Sean; Hutchinson, Michael

2011-09-01

Adult-onset primary torsion dystonia is an autosomal dominant disorder with markedly reduced penetrance; patients with sporadic adult-onset primary torsion dystonia are much more prevalent than familial. The temporal discrimination threshold is the shortest time interval at which two stimuli are detected to be asynchronous and has been shown to be abnormal in adult-onset primary torsion dystonia. The aim was to determine the frequency of abnormal temporal discrimination thresholds in patients with sporadic adult-onset primary torsion dystonia and their first-degree relatives. We hypothesized that abnormal temporal discrimination thresholds in first relatives would be compatible with an autosomal dominant endophenotype. Temporal discrimination thresholds were examined in 61 control subjects (39 subjects 50 years of age), 32 patients with sporadic adult-onset primary torsion dystonia (cervical dystonia n = 30, spasmodic dysphonia n = 1 and Meige's syndrome n = 1) and 73 unaffected first-degree relatives (36 siblings, 36 offspring and one parent) using visual and tactile stimuli. Z-scores were calculated for all subjects; a Z > 2.5 was considered abnormal. Abnormal temporal discrimination thresholds were found in 1/61 (2%) control subjects, 27/32 (84%) patients with adult-onset primary torsion dystonia and 32/73 (44%) unaffected relatives [siblings (20/36; 56%), offspring (11/36; 31%) and one parent]. When two or more relatives were tested in any one family, 22 of 24 families had at least one first-degree relative with an abnormal temporal discrimination threshold. The frequency of abnormal temporal discrimination thresholds in first-degree relatives of patients with sporadic adult-onset primary torsion dystonia is compatible with an autosomal dominant disorder and supports the hypothesis that apparently sporadic adult-onset primary torsion dystonia is genetic in origin.

CpG methylation of APC promoter 1A in sporadic and familial breast cancer patients.

Science.gov (United States)

Debouki-Joudi, Saoussen; Trifa, Fatma; Khabir, Abdelmajid; Sellami-Boudawara, Tahia; Frikha, Mounir; Daoud, Jamel; Mokdad-Gargouri, Raja

2017-01-01

Tumour suppressor gene (TSG) silencing through promoter hypermethylation plays an important role in cancer initiation. The aim of this study was to assess the extent of methylation of APC gene promoter in 91 sporadic and 44 familial cases of Tunisian patients with breast cancer (BC) in. The frequency of APC promoter methylation is somewhat similar for sporadic and familial breast cancer cases, (52.1%, and 54.5% respectively). For sporadic breast cancer patients, there was a significant correlation of APC promoter hypermethylation with TNM stage (p = 0.024) and 3-year survival (p = 0.025). Regarding the hormonal status (HR), we found significant association between negativity to PR and unmethylated APC (p= 0.005) while ER and Her2/neu are not correlated. Moreover, unmethylated APC promoter is more frequent in tumours expressing at least one out the 3 proteins compared to triple negative cases (p= 0.053). On the other hand, aberrant methylation of APC was associated with tumour size (p = 0.036), lymph node (p = 0.028), distant metastasis (p = 0.031), and 3-year survival (p = 0.046) in the group of patients with familial breast cancer. Moreover, patients with sporadic breast cancer displaying the unmethylated profile have a significant prolonged overall survival compared to those with the methylated pattern of APC promoter (p log rank = 0.008). Epigenetic change at the CpG islands in the APC promoter was associated with the silence of its transcript and the loss of protein expression suggesting that this event is the main mechanism regulating the APC expression in breast cancer. In conclusion, our data showed that the loss of APC through aberrant methylation is associated with the aggressive behavior of both sporadic and familial breast cancer in Tunisian patients.

Theory and observations of upward field-aligned currents at the magnetopause boundary layer.

Science.gov (United States)

Wing, Simon; Johnson, Jay R

2015-11-16

The dependence of the upward field-aligned current density ( J ‖ ) at the dayside magnetopause boundary layer is well described by a simple analytic model based on a velocity shear generator. A previous observational survey confirmed that the scaling properties predicted by the analytical model are applicable between 11 and 17 MLT. We utilize the analytic model to predict field-aligned currents using solar wind and ionospheric parameters and compare with direct observations. The calculated and observed parallel currents are in excellent agreement, suggesting that the model may be useful to infer boundary layer structures. However, near noon, where velocity shear is small, the kinetic pressure gradients and thermal currents, which are not included in the model, could make a small but significant contribution to J ‖ . Excluding data from noon, our least squares fit returns log( J ‖,max_cal ) = (0.96 ± 0.04) log( J ‖_obs ) + (0.03 ± 0.01) where J ‖,max_cal = calculated J ‖,max and J ‖_obs = observed J ‖ .

CT and MRI in iatrogenic and sporadic Creutzfeldt-Jakob disease: as far as imaging perseives

Energy Technology Data Exchange (ETDEWEB)

Garcia Santos, J.M. [Servicio de Radiodiagnostico, HU Dr. Morales Meseguer, Murcia (Spain)]|[Section of Neuroradiology, HU Virgen de la Arrixaca, Murcia (Spain); Lopez Corbalan, J.A. [Section of Neuroradiology, HU Virgen de la Arrixaca, Murcia (Spain); Martinez-Lage, J.F. [Service of Neurosurgery, HU Virgen de la Arrixaca, Murcia (Spain); Sicilis Guillen, J. [Service of Neurology, HU Virgen de la Arrixaca, Murcia (Spain)

1996-04-01

Creutzfeldt-Jakob Disease (CJD), an invariably fatal dementing illness, affects patients in middle and old age (sporadic form). However, the association of CJD with certain treatments (iatrogenic form) has been described in younger patients. The clinical onset of the two forms seems to differ; in the iatrogenic form a high frequency of the ataxic CJD variant has been reported. Nowadays, a definitive diagnosis of CJD is exclusively histological. We present five cases of CJD, one sporadic and the others iatrogenic, following dura mater grafts and analyse their CT and MRI features. CT typically demonstrates brain atrophy, generally progressive, but in sporadic CJD midfield MRI also showed abnormal signal, with predominant deep grey matter involvement. The use of narrow windows with proton-density sequences may reveal subtle cortical signal abnormalities not clearly visible with conventional windows. The early demonstration of these changes, in the appropriate clinical context, may suggest CJD and this supports the use of mid- or high magnetic fields in the diagnosis of CJD and other forms of dementia. In our cases of iatrogenic CJD, low-field MRI did not reveal more than the progressive atrophy displayed by CT, and raises the question on the one hand of possible differences, based on imaging, from the sporadic form, and on the other of the lack of sensitivity of low-field magnets to signal changes in CJD. (orig.)

CT and MRI in iatrogenic and sporadic Creutzfeldt-Jakob disease: as far as imaging perseives

International Nuclear Information System (INIS)

Garcia Santos, J.M.; Lopez Corbalan, J.A.; Martinez-Lage, J.F.; Sicilis Guillen, J.

1996-01-01

Creutzfeldt-Jakob Disease (CJD), an invariably fatal dementing illness, affects patients in middle and old age (sporadic form). However, the association of CJD with certain treatments (iatrogenic form) has been described in younger patients. The clinical onset of the two forms seems to differ; in the iatrogenic form a high frequency of the ataxic CJD variant has been reported. Nowadays, a definitive diagnosis of CJD is exclusively histological. We present five cases of CJD, one sporadic and the others iatrogenic, following dura mater grafts and analyse their CT and MRI features. CT typically demonstrates brain atrophy, generally progressive, but in sporadic CJD midfield MRI also showed abnormal signal, with predominant deep grey matter involvement. The use of narrow windows with proton-density sequences may reveal subtle cortical signal abnormalities not clearly visible with conventional windows. The early demonstration of these changes, in the appropriate clinical context, may suggest CJD and this supports the use of mid- or high magnetic fields in the diagnosis of CJD and other forms of dementia. In our cases of iatrogenic CJD, low-field MRI did not reveal more than the progressive atrophy displayed by CT, and raises the question on the one hand of possible differences, based on imaging, from the sporadic form, and on the other of the lack of sensitivity of low-field magnets to signal changes in CJD. (orig.)

Radiative Impact of Observed and Simulated Aerosol Layers Over the East Coast of North America

Science.gov (United States)

Berg, L. K.; Fast, J. D.; Burton, S. P.; Chand, D.; Comstock, J. M.; Ferrare, R. A.; Hair, J. W.; Hostetler, C. A.; Hubbe, J. M.; Kassianov, E.; Rogers, R. R.; Sedlacek, A. J., III; Shilling, J. E.; Tomlinson, J. M.; Wilson, J. M.; Zelenyuk, A.

2014-12-01

The vertical distribution of particles in the atmospheric column can have a large impact on the radiative forcing and cloud microphysics. A recent climatology constructed using data collected by the Cloud-Aerosol Lidar and Infrared Pathfinder Satellite Observations (CALIPSO) suggests elevated layers of aerosol are quite common near the North American east coast during both winter and summer. The Two-Column Aerosol Project (TCAP), conducted from June 2012 through June 2013, was a unique study utilizing both in situ and remotely sensed measurements designed to provide a comprehensive data set that can be used to investigate science questions related to aerosol radiative forcing and the vertical distribution of aerosol. The study sampled the atmosphere at a number of altitudes within two atmospheric columns; one located near the coast of North America (over Cape Cod, MA) and a second over the Atlantic Ocean several hundred kilometers from the coast. TCAP included the yearlong deployment of the U.S. Department of Energy's Atmospheric Radiation Measurement (ARM) Mobile Facility (AMF) located at the base of the Cape Cod column, as well as summer and winter aircraft intensive observation periods (IOPs) using the ARM Aerial Facility. One important finding from the TCAP summer IOP is the relatively common occurrence (during four of the six nearly cloud-free flights) of elevated aerosol layers in both the Cape Cod and maritime columns that were detected using the nadir pointing second-generation NASA Langley Research Center High-Spectral Resolution Lidar (HSRL-2). These elevated layers contributed up to 60% of the total observed aerosol optical depth (AOD). Many of these layers were also intercepted by the aircraft configured for in situ sampling, and the aerosol in the layers was found to have increased amounts of biomass burning material and nitrate compared to aerosol found near the surface. Both the in situ and remote sensing observations have been compared to

Fabrication of Crack-Free Barium Titanate Thin Film with High Dielectric Constant Using Sub-Micrometric Scale Layer-by-Layer E-Jet Deposition

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Junsheng Liang

2016-01-01

Full Text Available Dense and crack-free barium titanate (BaTiO3, BTO thin films with a thickness of less than 4 μm were prepared by using sub-micrometric scale, layer-by-layer electrohydrodynamic jet (E-jet deposition of the suspension ink which is composed of BTO nanopowder and BTO sol. Impacts of the jet height and line-to-line pitch of the deposition on the micro-structure of BTO thin films were investigated. Results show that crack-free BTO thin films can be prepared with 4 mm jet height and 300 μm line-to-line pitch in this work. Dielectric constant of the prepared BTO thin film was recorded as high as 2940 at 1 kHz at room temperature. Meanwhile, low dissipation factor of the BTO thin film of about 8.6% at 1 kHz was also obtained. The layer-by-layer E-jet deposition technique developed in this work has been proved to be a cost-effective, flexible and easy to control approach for the preparation of high-quality solid thin film.

Herpes Simplex Virus Type 1 and Other Pathogens are Key Causative Factors in Sporadic Alzheimer’s Disease

Science.gov (United States)

Harris, Steven A.; Harris, Elizabeth A.

2015-01-01

Abstract This review focuses on research in epidemiology, neuropathology, molecular biology, and genetics regarding the hypothesis that pathogens interact with susceptibility genes and are causative in sporadic Alzheimer’s disease (AD). Sporadic AD is a complex multifactorial neurodegenerative disease with evidence indicating coexisting multi-pathogen and inflammatory etiologies. There are significant associations between AD and various pathogens, including Herpes simplex virus type 1 (HSV-1), Cytomegalovirus, and other Herpesviridae, Chlamydophila pneumoniae, spirochetes, Helicobacter pylori, and various periodontal pathogens. These pathogens are able to evade destruction by the host immune system, leading to persistent infection. Bacterial and viral DNA and RNA and bacterial ligands increase the expression of pro-inflammatory molecules and activate the innate and adaptive immune systems. Evidence demonstrates that pathogens directly and indirectly induce AD pathology, including amyloid-β (Aβ) accumulation, phosphorylation of tau protein, neuronal injury, and apoptosis. Chronic brain infection with HSV-1, Chlamydophila pneumoniae, and spirochetes results in complex processes that interact to cause a vicious cycle of uncontrolled neuroinflammation and neurodegeneration. Infections such as Cytomegalovirus, Helicobacter pylori, and periodontal pathogens induce production of systemic pro-inflammatory cytokines that may cross the blood-brain barrier to promote neurodegeneration. Pathogen-induced inflammation and central nervous system accumulation of Aβ damages the blood-brain barrier, which contributes to the pathophysiology of AD. Apolipoprotein E4 (ApoE4) enhances brain infiltration by pathogens including HSV-1 and Chlamydophila pneumoniae. ApoE4 is also associated with an increased pro-inflammatory response by the immune system. Potential antimicrobial treatments for AD are discussed, including the rationale for antiviral and antibiotic clinical trials. PMID

Congenital terminal transverse deformity of upper limb: clinical and radiological findings in a sporadic care.

Science.gov (United States)

Malik, Sajid; Afzal, Muhammad

2013-03-01

Congenital transverse limb anomalies are rare, which affect upper and/or lower limbs and may accompany several syndromic malformations. We present a sporadic male subject with congenital, unilateral transverse arrest of the left hand. The affected arm was observed to be short with reduced zeugopod and truncated palm. Fingers were represented by five bead-like nubbins. Roentgenographic examination revealed short radius and ulna with hypoplastic distal heads, absent carpals/metacarpals, and a hypoplastic bony island in each nubbin. Consanguinity was denied, and the subject had no symptoms in the orofacial, neurological and skeletal systems. Detailed clinical data with literature survey is presented.

The survival of patients with Stage III Colon Cancer is improved in HNPCC compared with sporadic cases. A Danish registry based study

DEFF Research Database (Denmark)

Brixen, Line Merrild; Bernstein, Inge Thomsen; Bülow, Steffen

2013-01-01

AIM: Patients with hereditary non-polyposis colorectal cancer (HNPCC) seem to have a better prognosis than those with sporadic colon cancer (CC)s. The aim was to compare survival after stage III CC in patients with HNPCC with those having sporadic CC. METHOD: 230 patients with hereditary cancer...... from The Danish HNPCC-Register and 3557 patients with sporadic CC from The Danish Colorectal Cancer Database, diagnosed during May 2001-December 2008 were included. HNPCC patients were classified according to Mismatch Repair mutation status and family pedigree. Sporadic cases had no known family...... history of cancer. Patient characteristics, geographic differences and survival data were analyzed. RESULTS: The overall survival (OS) was better in HNPCC patients compared to sporadic CC after stratification for sex and age (p=0.02; CI 1.04-1.7). The 5-year survival was 70% in HNPCC patients compared...

Oestrogen receptor beta isoform expression in sporadic colorectal cancer, familial adenomatous polyposis and progressive stages of colorectal cancer.

Science.gov (United States)

Stevanato Filho, Paulo Roberto; Aguiar Júnior, Samuel; Begnami, Maria Dirlei; Kuasne, Hellen; Spencer, Ranyell Matheus; Nakagawa, Wilson Toshihiko; Bezerra, Tiago Santoro; Kupper, Bruna Catin; Takahashi, Renata Maymi; Barros Filho, Mateus; Rogatto, Silvia Regina; Lopes, Ademar

2017-11-13

Among the sex hormones, oestrogen may play a role in colorectal cancer, particularly in conjunction with oestrogen receptor-β (ERβ). The expression of ERβ isoform variants and their correlations with familial adenomatous polyposis (FAP) syndrome and sporadic colorectal carcinomas are poorly described. This study aimed to investigate the expression levels of the ERβ1, ERβ2, ERβ4 and ERβ5 isoform variants using quantitative RT-PCR (921 analyses) in FAP, normal mucosa, adenomatous polyps and sporadic colorectal carcinomas. Decreased expression of ERβ isoforms was identified in sporadic polyps and in sporadic colorectal cancer as well as in polyps from FAP syndrome patients compared with normal tissues (p colorectal carcinomas were compared to normal mucosa tissues. These findings suggest an association of the ERβ isoform variants in individuals affected by germline mutations of the APC gene. Progressively decreased expression of ERβ was found in polyps at early stages of low-grade dysplasia, followed by T1-T2 and T3-T4 tumours (p colorectal cancer, the loss of expression was an independent predictor of recurrence, and ERβ1 and ERβ5 expression levels were associated with better disease-free survival (p = 0.002). These findings may provide a better understanding of oestrogens and their potential preventive and therapeutic effects on sporadic colorectal cancer and cancers associated with FAP syndrome.

Lidar observations of marine boundary-layer winds and heights: a preliminary study

DEFF Research Database (Denmark)

Peña, Alfredo; Gryning, Sven-Erik; Floors, Rogier Ralph

2015-01-01

the highest data availability (among the three sites) and a very good agreement with the observations of wind speed and direction from cup anemometers and vanes from the platform's tower. The wind lidar was also able to perform measurements under a winter storm where 10-s gusts were observed above 60 m s 1......Here we describe a nearly 1-yr meteorological campaign, which was carried out at the FINO3 marine research platform on the German North Sea, where a pulsed wind lidar and a ceilometer were installed besides the platform's 105-m tower and measured winds and the aerosol backscatter in the entire...... marine atmospheric boundary layer. The campaign was the last phase of a research project, in which the vertical wind profile in the atmospheric boundary layer was firstly investigated on a coastal and a semi-urban site. At FINO3 the wind lidar, which measures the wind speed up to 2000 m, shows...

Evidence for presumable feline origin of sporadic G6P[9] rotaviruses in humans.

Science.gov (United States)

Pietsch, Corinna; Liebert, Uwe G

2018-05-31

Species A rotaviruses are highly diverse and impose a substantial burden to human and animal health. Interspecies transmission between livestock, domestic animals and humans is commonly observed, but spread of animal-like rotaviruses within the human population is limited. During the continued monitoring of rotavirus strains in Germany, an unusual G6P[9] rotavirus strain was detected in feces of a child. The complete rotavirus coding sequences revealed a unique G6-P[9]-I2-R2-C2-M2-A3-N2-T3-E2-H3 genotype constellation. The virus was phylogenetically related to feline G3P[9] strains and other human G6P[9] rotaviruses of presumable zoonotic origin. Analysis of primer binding sites of G6 specific genotyping revealed further evidence of a G6P[9] feline reservoir. Moreover, substantial deficits of conventional semi-nested PCR genotyping approaches in detecting contemporary G6P[9] were revealed. Rotavirus strain GER29-14 most likely resulted from a direct or recent interspecies transmission from a cat to human. Further studies could assess nucleic acid sequences and genotype constellations of feline rotavirus to confirm the likely feline origin of sporadic human G6P[9] strains. Copyright © 2018 Elsevier B.V. All rights reserved.

Observations of high droplet number concentrations in Southern Ocean boundary layer clouds

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T. Chubb

2016-01-01

Full Text Available Cloud physics data collected during the NSF/NCAR High-performance Instrumented Airborne Platform for Environmental Research (HIAPER Pole-to-Pole Observations (HIPPO campaigns provide a snapshot of unusual wintertime microphysical conditions in the boundary layer over the Southern Ocean. On 29 June 2011, the HIAPER sampled the boundary layer in a region of pre-frontal warm air advection between 58 and 48° S to the south of Tasmania. Cloud droplet number concentrations were consistent with climatological values in the northernmost profiles but were exceptionally high for wintertime in the Southern Ocean at 100–200 cm−3 in the southernmost profiles. Sub-micron (0.06  < D <  1 µm aerosol concentrations for the southern profiles were up to 400 cm−3. Analysis of back trajectories and atmospheric chemistry observations revealed that while conditions in the troposphere were more typical of a clean remote ocean airmass, there was some evidence of continental or anthropogenic influence. However, the hypothesis of long-range transport of continental aerosol fails to explain the magnitude of the aerosol and cloud droplet concentration in the boundary layer. Instead, the gale force surface winds in this case (wind speed at 167 m above sea level was  > 25 m s−1 were most likely responsible for production of sea spray aerosol which influenced the microphysical properties of the boundary layer clouds. The smaller size and higher number concentration of cloud droplets is inferred to increase the albedo of these clouds, and these conditions occur regularly, and are expected to increase in frequency, over windy parts of the Southern Ocean.

Novel multiple endocrine neoplasia type 1 variations in patients with sporadic primary hyperparathyroidism

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S Birla

2016-01-01

Full Text Available Background and Objectives: Primary hyperparathyroidism (PHPT can occur either as a sporadic case or in association with syndromes such as multiple endocrine neoplasia. Multiple endocrine neoplasia type 1 (MEN1 is a rare autosomal-dominant disease resulting from mutations in MEN1 gene encoding a 621 amino acid long tumor suppressor protein “menin.” We report here the results of MEN1 screening in 31 patients diagnosed with sporadic PHPT. Materials and Methods: Diagnosis of sporadic PHPT was made when blood urea and serum creatinine were normal, serum parathyroid hormone was high, and parathyroid enlargement could be localized on ultrasound and/or parathyroid scan. A total of 31 patients and 50 healthy volunteers were recruited for molecular analysis after taking informed consent. Results: Major symptoms at presentation were bone pain, fatigue, muscle weakness, and renal stones. Molecular genetic analysis revealed the presence of two novel intronic variations, c. 913-79T>A and c. 784-129T>A which by human splicing finder are predicted to cause potential alteration of splicing by either activating an intronic cryptic acceptor site or converting a conserved exonic splicing silencer sequence to an exonic splicing enhancer site. Apart from these, two reported polymorphisms rs144677807 and rs669976 were seen only in patients and none of the controls. Other reported polymorphisms rs2071313 and rs654440 were identified both in controls and patients. Conclusions: This is the first study of MEN1 gene screening in sporadic PHPT in India reporting on the clinical and genetic findings, wherein two novel intronic variations c. 913-79T>A and c. 784-129T>A were identified showing their possible role in disease causation.

Earth's influx of different populations of sporadic meteoroids from photographic and television data

International Nuclear Information System (INIS)

Ceplecha, Z.

1988-01-01

Precise photographic and television double- and multi-station data on 3624 sporadic meteors in the mass range from 2 x 10 -5 grams to 2 x 10 7 grams form the basis of this paper. The applied classification criteria and procedures are defined and described. A survey of 7 different populations of sporadic meteoroids known so far is presented. The total numbers and masses of meteoroids as a function of mass are given for individual groups and for all sporadic meteors. The absolute calibration of the influx to the Earth was carried out by comparison with the results of Halliday et al. (1984). The comparison with the visual and cratering data revealed good agreement in the narrow ''visual'' interval of masses, and disagreement in the extrapolated parts of the visual and cratering flux curves. The slope of the cumulative number curve for the meteorite-dropping fireballs (type I) with masses larger than 1 kg was found as -0.69 in perfect agreement with the results of Halliday et al. (1984). The final mass scale derived in this paper is situated between the scale of McCrosky and the scale of Halliday. The relative significance of the different groups of meteoroids changes with the mass quite dramatically. The total influx of sporadic meteoroids in the mass interval of 12 orders from 2 x 10 7 to 2 x 10 -5 grams resulted in 5 x 10 9 grams per year for the entire Earth's surface. Most of this mass comes in the form of larger meteoroids. Bulk densities and ablation coefficient are presented for the individual meteor groups depending on different ablation models of several authors and some extreme concepts of this problem are discussed. (author). 3 figs., 6 tabs., 38 refs

Observation of low-frequency acoustic surface waves in the nocturnal boundary layer.

Science.gov (United States)

Talmadge, Carrick L; Waxler, Roger; Di, Xiao; Gilbert, Kenneth E; Kulichkov, Sergey

2008-10-01

A natural terrain surface, because of its porosity, can support an acoustic surface wave that is a mechanical analog of the familiar vertically polarized surface wave in AM radio transmission. At frequencies of several hundred hertz, the acoustic surface wave is attenuated over distances of a few hundred meters. At lower frequencies (e.g., below approximately 200 Hz) the attenuation is much less, allowing surface waves to propagate thousands of meters. At night, a low-frequency surface wave is generally present at long ranges even when downward refraction is weak. Thus, surface waves represent a ubiquitous nighttime transmission mode that exists even when other transmission modes are weak or absent. Data from recent nighttime field experiments and theoretical calculations are presented, demonstrating the persistence of the surface wave under different meteorological conditions. The low-frequency surface wave described here is the "quasiharmonical" tail observed previously in nighttime measurements but not identified by S. Kulichkov and his colleagues (Chunchuzov, I. P. et al. 1990. "On acoustical impulse propagation in a moving inhomogeneous atmospheric layer," J. Acoust. Soc. Am. 88, 455-461).

THE SOUTHERN ARGENTINA AGILE METEOR RADAR ORBITAL SYSTEM (SAAMER-OS): AN INITIAL SPORADIC METEOROID ORBITAL SURVEY IN THE SOUTHERN SKY

Energy Technology Data Exchange (ETDEWEB)

Janches, D.; Swarnalingam, N. [Space Weather Laboratory, Mail Code 674, NASA Goddard Space Flight Center, Greenbelt, MD 20771 (United States); Close, S. [Space Environment and Satellite Systems Laboratory, Department of Aeronautics and Astronautics, Stanford University, Palo Alto, CA (United States); Hormaechea, J. L. [Estacion Astronomica Rio Grande, Rio Grande, Tierra del Fuego (Argentina); Murphy, A.; O’Connor, D.; Vandepeer, B.; Fuller, B. [Genesis Software Pty Ltd, Adelaide (Australia); Fritts, D. C. [GATS Inc., Boulder CO (United States); Brunini, C., E-mail: diego.janches@nasa.gov, E-mail: nimalan.swarnalingam@nasa.gov, E-mail: sigridc@stanford.edu, E-mail: jlhormaechea@untdf.edu.ar, E-mail: amurphy@gsoft.com.au, E-mail: doconnor@gsoft.com.au, E-mail: bvandepe@gsoft.com.au, E-mail: bfuller@gsoft.com.au, E-mail: dave@gats-inc.com, E-mail: claudiobrunini@yahoo.com [Departmento de Astronomia y Geofísica, Universidad Nacional de La Plata, La Plata (Argentina)

2015-08-10

We present an initial survey in the southern sky of the sporadic meteoroid orbital environment obtained with the Southern Argentina Agile MEteor Radar (SAAMER) Orbital System (OS), in which over three-quarters of a million orbits of dust particles were determined from 2012 January through 2015 April. SAAMER-OS is located at the southernmost tip of Argentina and is currently the only operational radar with orbit determination capability providing continuous observations of the southern hemisphere. Distributions of the observed meteoroid speed, radiant, and heliocentric orbital parameters are presented, as well as those corrected by the observational biases associated with the SAAMER-OS operating parameters. The results are compared with those reported by three previous surveys performed with the Harvard Radio Meteor Project, the Advanced Meteor Orbit Radar, and the Canadian Meteor Orbit Radar, and they are in agreement with these previous studies. Weighted distributions for meteoroids above the thresholds for meteor trail electron line density, meteoroid mass, and meteoroid kinetic energy are also considered. Finally, the minimum line density and kinetic energy weighting factors are found to be very suitable for meteroid applications. The outcomes of this work show that, given SAAMER’s location, the system is ideal for providing crucial data to continuously study the South Toroidal and South Apex sporadic meteoroid apparent sources.

Observations of mesoscale and boundary-layer scale circulations affecting dust transport and uplift over the Sahara

Directory of Open Access Journals (Sweden)

J. H. Marsham

2008-12-01

Full Text Available Observations of the Saharan boundary layer, made during the GERBILS field campaign, show that mesoscale land surface temperature variations (which were related to albedo variations induced mesoscale circulations. With weak winds along the aircraft track, land surface temperature anomalies with scales of greater than 10 km are shown to significantly affect boundary-layer temperatures and winds. Such anomalies are expected to affect the vertical mixing of the dusty and weakly stratified Saharan Residual Layer (SRL. Mesoscale variations in winds are also shown to affect dust loadings in the boundary layer.

Using the aircraft observations and data from the COSMO model, a region of local dust uplift, with strong along-track winds, was identified in one low-level flight. Large eddy model (LEM simulations based on this location showed linearly organised boundary-layer convection. Calculating dust uplift rates from the LEM wind field showed that the boundary-layer convection increased uplift by approximately 30%, compared with the uplift rate calculated neglecting the convection. The modelled effects of boundary-layer convection on uplift are shown to be larger when the boundary-layer wind is decreased, and most significant when the mean wind is below the threshold for dust uplift and the boundary-layer convection leads to uplift which would not otherwise occur.

Both the coupling of albedo features to the atmosphere on the mesoscale, and the enhancement of dust uplift by boundary-layer convection are unrepresented in many climate models, but may have significant impacts on the vertical transport and uplift of desert dust. Mesoscale effects in particular tend to be difficult to parametrise.

Calcium dysregulation, functional calpainopathy, and endoplasmic reticulum stress in sporadic inclusion body myositis.

Science.gov (United States)

Amici, David R; Pinal-Fernandez, Iago; Mázala, Davi A G; Lloyd, Thomas E; Corse, Andrea M; Christopher-Stine, Lisa; Mammen, Andrew L; Chin, Eva R

2017-03-22

Sporadic inclusion body myositis (IBM) is the most common primary myopathy in the elderly, but its pathoetiology is still unclear. Perturbed myocellular calcium (Ca 2+ ) homeostasis can exacerbate many of the factors proposed to mediate muscle degeneration in IBM, such as mitochondrial dysfunction, protein aggregation, and endoplasmic reticulum stress. Ca 2+ dysregulation may plausibly be initiated in IBM by immune-mediated membrane damage and/or abnormally accumulating proteins, but no studies to date have investigated Ca 2+ regulation in IBM patients. We first investigated protein expression via immunoblot in muscle biopsies from IBM, dermatomyositis, and non-myositis control patients, identifying several differentially expressed Ca 2+ -regulatory proteins in IBM. Next, we investigated the Ca 2+ -signaling transcriptome by RNA-seq, finding 54 of 183 (29.5%) genes from an unbiased list differentially expressed in IBM vs. controls. Using an established statistical approach to relate genes with causal transcription networks, Ca 2+ abundance was considered a significant upstream regulator of observed whole-transcriptome changes. Post-hoc analyses of Ca 2+ -regulatory mRNA and protein data indicated a lower protein to transcript ratio in IBM vs. controls, which we hypothesized may relate to increased Ca 2+ -dependent proteolysis and decreased protein translation. Supporting this hypothesis, we observed robust (4-fold) elevation in the autolytic activation of a Ca 2+ -activated protease, calpain-1, as well as increased signaling for translational attenuation (eIF2a phosphorylation) downstream of the unfolded protein response. Finally, in IBM samples we observed mRNA and protein under-expression of calpain-3, the skeletal muscle-specific calpain, which broadly supports proper Ca 2+ homeostasis. Together, these data provide novel insight into mechanisms by which intracellular Ca 2+ regulation is perturbed in IBM and offer evidence of pathological downstream effects.

Observation-based estimation of aerosol-induced reduction of planetary boundary layer height

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Zou, Jun; Sun, Jianning; Ding, Aijun; Wang, Minghuai; Guo, Weidong; Fu, Congbin

2017-09-01

Radiative aerosols are known to influence the surface energy budget and hence the evolution of the planetary boundary layer. In this study, we develop a method to estimate the aerosol-induced reduction in the planetary boundary layer height (PBLH) based on two years of ground-based measurements at a site, the Station for Observing Regional Processes of the Earth System (SORPES), at Nanjing University, China, and radiosonde data from the meteorological station of Nanjing. The observations show that increased aerosol loads lead to a mean decrease of 67.1 W m-2 for downward shortwave radiation (DSR) and a mean increase of 19.2 W m-2 for downward longwave radiation (DLR), as well as a mean decrease of 9.6 Wm-2 for the surface sensible heat flux (SHF) in the daytime. The relative variations of DSR, DLR and SHF are shown as a function of the increment of column mass concentration of particulate matter (PM2.5). High aerosol loading can significantly increase the atmospheric stability in the planetary boundary layer during both daytime and nighttime. Based on the statistical relationship between SHF and PM2.5 column mass concentrations, the SHF under clean atmospheric conditions (same as the background days) is derived. In this case, the derived SHF, together with observed SHF, are then used to estimate changes in the PBLH related to aerosols. Our results suggest that the PBLH decreases more rapidly with increasing aerosol loading at high aerosol loading. When the daytime mean column mass concentration of PM2.5 reaches 200 mg m-2, the decrease in the PBLH at 1600 LST (local standard time) is about 450 m.

Sporadic nocturnal frontal lobe epilepsy: A consecutive series of 8 cases

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Shih-Bin Yeh

2014-09-01

Discussion: These cases confirm that sporadic NFLE closely resembles familial NFLE, and comprises a set of distinct clinical manifestations, with variable intensity, and variable scalp EEG epileptiform abnormalities across sleep and wakefulness, which have previously been identified in Caucasian patients from Europe and North America.

CSF Neurofilament Proteins Levels are Elevated in Sporadic Creutzfeldt-Jakob Disease

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van Eijk, Jeroen J. J.; van Everbroeck, Bart; Abdo, W. Farid; Kremer, Berry P. H.; Verbeek, Marcel M.

2010-01-01

In this study we investigated the cerebrospinal fluid (CSF) levels of neurofilament light (NFL) and heavy chain (NFHp35), total tau (t-tau), and glial fibrillary acidic protein (GFAP) to detect disease specific profiles in sporadic Creutzfeldt Jakob disease (sCJD) patients and Alzheimer's disease

Precipitating clouds observed by 1.3-GHz boundary layer radars in equatorial Indonesia

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F. Renggono

2001-08-01

Full Text Available Temporal variations of precipitating clouds in equatorial Indonesia have been studied based on observations with 1357.5 MHz boundary layer radars at Serpong (6.4° S, 106.7° E near Jakarta and Bukittinggi (0.2° S, 100.3° E in West Sumatera. We have classified precipitating clouds into four types: stratiform, mixed stratiform-convective, deep convective, and shallow convective clouds, using the Williams et al. (1995 method. Diurnal variations of the occurrence of precipitating clouds at Serpong and Bukittinggi have showed the same characteristics, namely, that the precipitating clouds primarily occur in the afternoon and the peak of the stratiform cloud comes after the peak of the deep convective cloud. The time delay between the peaks of stratiform and deep convective clouds corresponds to the life cycle of the mesoscale convective system. The precipitating clouds which occur in the early morning at Serpong are dominated by stratiform cloud. Concerning seasonal variations of the precipitating clouds, we have found that the occurrence of the stratiform cloud is most frequent in the rainy season, while the occurrence of the deep convective cloud is predominant in the dry season.Key words. Meteorology and atmospheric dynamics (convective processes; precipitation; tropical meteorology

Counter electrojet features in the Brazilian sector: simultaneous observation by radar, digital sounder and magnetometers

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C. M. Denardini

2009-04-01

Full Text Available In the present work we show new results regarding equatorial counter electrojet (CEJ events in the Brazilian sector, based on the RESCO radar, two set of fluxgate magnetometer systems and a digital sounder. RESCO radar is a 50 MHz backscatter coherent radar installed in 1998 at São Luís (SLZ, 2.33° S, 44.60° W, an equatorial site. The Digital sounder routinely monitors the electron density profile at the radar site. The magnetometer systems are fluxgate-type installed at SLZ and Eusébio (EUS, 03.89° S, 38.44° W. From the difference between the horizontal component of magnetic field at SLZ station and the same component at EUS (EEJ ground strength several cases of westward morning electrojet and its normal inversion to the eastward equatorial electrojet (EEJ have been observed. Also, the EEJ ground strength has shown some cases of CEJ events, which been detected with the RESCO radar too. Detection of these events were investigated with respect to their time and height of occurrence, correlation with sporadic E (Es layers at the same time, and their spectral characteristics as well as the radar echo power intensity.

Sporadic and genetic forms of paediatric somatotropinoma: a retrospective analysis of seven cases and a review of the literature

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Nozières Cécile

2011-10-01

Full Text Available Abstract Background Somatotropinoma, a pituitary adenoma characterised by excessive production of growth hormone (GH, is extremely rare in childhood. A genetic defect is evident in some cases; known genetic changes include: multiple endocrine neoplasia type 1 (MEN1; Carney complex; McCune-Albright syndrome; and, more recently identified, aryl hydrocarbon receptor-interacting protein (AIP. We describe seven children with somatotropinoma with a special focus on the differences between genetic and sporadic forms. Methods Seven children who presented in our regional network between 1992 and 2008 were included in this retrospective analysis. First-type therapy was somatostatin (SMS analogues or transsphenoidal surgery. Control was defined as when insulin-like growth factor-1 (IGF-1 levels were within the normal range for the patient's age at 6 months after therapy, associated with decreasing tumour volume. Results Patients were aged 5-17 years and the majority (n = 6 were male. Four patients had an identified genetic mutation (McCune-Albright syndrome: n = 1; MEN1: n = 1; AIP: n = 2; the remaining three cases were sporadic. Accelerated growth rate was reported as the first clinical sign in four patients. Five patients presented with macroadenoma; invasion was noted in four of them (sporadic: n = 1; genetic: n = 3. Six patients were treated with SMS analogues; normalisation of IGF-1 occurred in one patient who had a sporadic intrasellar macroadenoma. Multiple types of therapy were necessary in all patients with an identified genetic mutation (4 types: n = 1; 3 types: n = 2; 2 types: n = 1, whereas two of the three patients with sporadic somatotropinoma required only one type of therapy. Conclusions This is the first series that analyzes the therapeutic response of somatotropinoma in paediatric patients with identified genetic defects. We found that, in children, genetic somatotropinomas are more invasive than sporadic somatotropinomas. Furthermore

Predictors of Preoperative Tinnitus in Unilateral Sporadic Vestibular Schwannoma

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Georgios Naros

2017-08-01

Full Text Available ObjectiveNearly two-thirds of patients with vestibular schwannoma (VS are reporting a significantly impaired quality of life due to tinnitus. VS-associated tinnitus is attributed to an anatomical and physiological damage of the hearing nerve by displacing growth of the tumor. In contrast, the current pathophysiological concept of non-VS tinnitus hypothesizes a maladaptive neuroplasticity of the central nervous system to a (hidden hearing impairment resulting in a subjective misperception. However, it is unclear whether this concept fits to VS-associated tinnitus. This study aims to determine the clinical predictors of VS-associated tinnitus to ascertain the compatibility of both pathophysiological concepts.MethodsThis retrospective study includes a group of 478 neurosurgical patients with unilateral sporadic VS evaluated preoperatively regarding the occurrence of ipsilateral tinnitus depending on different clinical factors, i.e., age, gender, tumor side, tumor size (T1–T4 according to the Hannover classification, and hearing impairment (Gardner–Robertson classification, GR1–5, using a binary logistic regression.Results61.8% of patients complain about a preoperative tinnitus. The binary logistic regression analysis identified male gender [OR 1.90 (1.25–2.75; p = 0.002] and hearing impairment GR3 [OR 1.90 (1.08–3.35; p = 0.026] and GR4 [OR 8.21 (2.29–29.50; p = 0.001] as positive predictors. In contrast, patients with large T4 tumors [OR 0.33 (0.13–0.86; p = 0.024] and complete hearing loss GR5 [OR 0.36 (0.15–0.84; p = 0.017] were less likely to develop a tinnitus. Yet, 60% of the patients with good clinical hearing (GR1 and 25% of patients with complete hearing loss (GR5 suffered from tinnitus.ConclusionThese data are good accordance with literature about non-VS tinnitus indicating hearing impairment as main risk factor. In contrast, complete hearing loss appears a negative predictor for tinnitus. For the first

Refinements in the use of equivalent latitude for assimilating sporadic inhomogeneous stratospheric tracer observations, 1: Detecting transport of Pinatubo aerosol across a strong vortex edge

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P. Good

2004-01-01

Full Text Available The use of PV equivalent latitude for assimilating stratospheric tracer observations is discussed - with particular regard to the errors in the equivalent latitude coordinate, and to the assimilation of sparse data. Some example measurements are assimilated: they sample the stratosphere sporadically and inhomogeneously. The aim was to obtain precise information about the isentropic tracer distribution and evolution as a function of equivalent latitude. Precision is important, if transport across barriers like the vortex edge are to be detected directly. The main challenges addressed are the errors in modelled equivalent latitude, and the non-ideal observational sampling. The methods presented allow first some assessment of equivalent latitude errors and a picture of how good or poor the observational coverage is. This information determines choices in the approach for estimating as precisely as possible the true equivalent latitude distribution of the tracer, in periods of good and poor observational coverage. This is in practice an optimisation process, since better understanding of the equivalent latitude distribution of the tracer feeds back into a clearer picture of the errors in the modelled equivalent latitude coordinate. Error estimates constrain the reliability of using equivalent latitude to make statements like 'this observation samples air poleward of the vortex edge' or that of more general model-measurement comparisons. The approach is demonstrated for ground-based lidar soundings of the Mount Pinatubo aerosol cloud, focusing on the 1991-92 arctic vortex edge between 475-520K. Equivalent latitude is estimated at the observation times and locations from Eulerian model tracers initialised with PV and forced by UK Meteorological Office analyses. With the model formulation chosen, it is shown that tracer transport of a few days resulted in an error distribution that was much closer to Gaussian form, although the mean error was not

The Asian Tropopause Aerosol Layer: Balloon-Borne Measurements, Satellite Observations and Modeling Approaches

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Fairlie, T. D.; Vernier, J.-P.; Natarajan, M.; Deshler, Terry; Liu, H.; Wegner, T.; Baker, N.; Gadhavi, H.; Jayaraman, A.; Pandit, A.;

An intelligent service-based layered architecture for e learning and assessment

International Nuclear Information System (INIS)

Javaid, Q.; Arif, F.

2017-01-01

The rapid advancement in ICT (Information and Communication Technology) is causing a paradigm shift in eLearning domain. Traditional eLearning systems suffer from certain shortcomings like tight coupling of system components, lack of personalization, flexibility, and scalability and performance issues. This study aims at addressing these challenges through an MAS (Multi Agent System) based multi-layer architecture supported by web services. The foremost objective of this study is to enhance learning process efficiency by provision of flexibility features for learning and assessment processes. Proposed architecture consists of two sub-system namely eLearning and eAssesssment. This architecture comprises of five distinct layers for each sub-system, with active agents responsible for miscellaneous tasks including content handling, updating, resource optimization, load handling and provision of customized environments for learners and instructors. Our proposed architecture aims at establishment of a facilitation level to learners as well as instructors for convenient acquisition and dissemination of knowledge. Personalization features like customized environments, personalized content retrieval and recommendations, adaptive assessment and reduced response time, are believed to significantly enhance learning and tutoring experience. In essence characteristics like intelligence, personalization, interactivity, usability, laidback accessibility and security, signify aptness of proposed architecture for improving conventional learning and assessment processes. Finally we have evaluated our proposed architecture by means of analytical comparison and survey considering certain quality attributes. (author)

Rainfall is a risk factor for sporadic cases of Legionella pneumophila pneumonia.

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Carolina Garcia-Vidal

Full Text Available It is not known whether rainfall increases the risk of sporadic cases of Legionella pneumonia. We sought to test this hypothesis in a prospective observational cohort study of non-immunosuppressed adults hospitalized for community-acquired pneumonia (1995-2011. Cases with Legionella pneumonia were compared with those with non-Legionella pneumonia. Using daily rainfall data obtained from the regional meteorological service we examined patterns of rainfall over the days prior to admission in each study group. Of 4168 patients, 231 (5.5% had Legionella pneumonia. The diagnosis was based on one or more of the following: sputum (41 cases, antigenuria (206 and serology (98. Daily rainfall average was 0.556 liters/m(2 in the Legionella pneumonia group vs. 0.328 liters/m(2 for non-Legionella pneumonia cases (p = 0.04. A ROC curve was plotted to compare the incidence of Legionella pneumonia and the weighted median rainfall. The cut-off point was 0.42 (AUC 0.54. Patients who were admitted to hospital with a prior weighted median rainfall higher than 0.42 were more likely to have Legionella pneumonia (OR 1.35; 95% CI 1.02-1.78; p = .03. Spearman Rho correlations revealed a relationship between Legionella pneumonia and rainfall average during each two-week reporting period (0.14; p = 0.003. No relationship was found between rainfall average and non-Legionella pneumonia cases (-0.06; p = 0.24. As a conclusion, rainfall is a significant risk factor for sporadic Legionella pneumonia. Physicians should carefully consider Legionella pneumonia when selecting diagnostic tests and antimicrobial therapy for patients presenting with CAP after periods of rainfall.

Observation of anomalous Stokes versus anti-Stokes ratio in MoTe2 atomic layers

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Goldstein, Thomas; Chen, Shao-Yu; Xiao, Di; Ramasubramaniam, Ashwin; Yan, Jun

We grow hexagonal molybdenum ditelluride (MoTe2), a prototypical transition metal dichalcogenide (TMDC) semiconductor, with chemical vapor transport methods and investigate its atomic layers with Stokes and anti-Stokes Raman scattering. We report observation of all six types of zone center optical phonons. Quite remarkably, the anti-Stokes Raman intensity of the low energy layer-breathing mode becomes more intense than the Stokes peak under certain experimental conditions, creating an illusion of 'negative temperature'. This effect is tunable, and can be switched from anti-Stokes enhancement to suppression by varying the excitation wavelength. We interpret this observation to be a result of resonance effects arising from the C excitons in the vicinity of the Brillouin zone center, which are robust even for multiple layers of MoTe2. The intense anti-Stokes Raman scattering provides a cooling channel for the crystal and opens up opportunities for laser cooling of atomically thin TMDC semiconductor devices. Supported by the University of Massachusetts Amherst, the National Science Foundation Center for Hierarchical Manufacturing (CMMI-1025020) and Office of Emerging Frontiers in Research and Innovation (EFRI-1433496).

Diffusion MR imaging in sporadic Creutzfeldt-Jakob disease

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Burcak Cakir Pekoz

2014-08-01

Full Text Available Creutzfeldt-Jakob disease (CJD is a rare dementing disease and is thought to caused by a prion. It is characterized by rapidly progressive dementia, ataxia, myoclonus, akinetic mutism and eventual death. Brain biopsy or autopsy is required for a definitive diagnosis of CJD. Diffusion-weighted imaging became an important tool for early diagnosis of CJD because of the high sensitivity. We present 59-year-old female patient diagnosed as sporadic CJD with typical MR imagings. [Cukurova Med J 2014; 39(4.000: 880-883

Observation of negative potential depression on double layer during a phase of current disruption

International Nuclear Information System (INIS)

Fujita, H.; Matsuo, K.; Yagura, S.

1984-01-01

The negative potential depression with a depth of approximately electron temperature is observed on the low potential tail of the double layer just at the moment when the electron current passing through the layer is disrupted. The depression is confirmed to serve as an electron thermal barrier and form an ion hole from phase-space measurements of electrons and ions, respectively. The depth of the depression becomes maximum when the density around the depression becomes most inhomogeneous. (author)

Cognitive disorders after sporadic ecstasy use? A case report.

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Ruis, Carla; Postma, Albert; Bouvy, Willem; van der Ham, Ineke

2015-01-01

Memory problems and changes in hippocampal structures after chronic ecstasy use are well described in the literature. Cognitive problems after incidental ecstasy use are rare, and the few patients described in case reports returned to their normal cognitive level after a relative short period. FV is a 39-year-old man who used an ecstasy tablet in 2005. This resulted in severe confusion for a few days. The confusion was followed by persistent memory complaints and difficulties orientating in new surroundings. An extensive neuropsychological examination 7 years after the ecstasy use revealed a severe memory disorder. Furthermore, his performance on a virtual reality test of navigation showed serious problems navigating in new surroundings. In comparison with matched control subjects (Bayesian approach for single case studies) his scores were significantly impaired on several subtasks of the navigation test. On a magnetic resonance imaging (MRI) scan of the brain bilateral hippocampal atrophy and sclerosis were visible, comparable to previous MRI studies describing hippocampal damage following ecstasy ingestion. This case report describes persistent memory and navigation disorders after sporadic ecstasy use, supported by structural brain abnormalities seen on the MRI scan. These findings revive the debate on whether sporadic ecstasy use can cause persistent cognitive deficits.

Effects of alterations of the E. coli lipopolysaccharide layer on membrane permeabilization events induced by Cecropin A.

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Agrawal, Anurag; Weisshaar, James C

2018-04-22

The outermost layer of Gram negative bacteria is composed of a lipopolysaccharide (LPS) network that forms a dense protective hydrophilic barrier against entry of hydrophobic drugs. At low μM concentrations, a large family of cationic polypeptides known as antimicrobial peptides (AMPs) are able to penetrate the LPS layer and permeabilize the outer membrane (OM) and the cytoplasmic membrane (CM), causing cell death. Cecropin A is a well-studied cationic AMP from moth. Here a battery of time-resolved, single-cell microscopy experiments explores how deletion of sugar layers and/or phosphoryl negative charges from the core oligosaccharide layer (core OS) of K12 E. coli alters the timing of OM and CM permeabilization induced by Cecropin A. Deletion of sugar layers, or phosphoryl charges, or both from the core OS shortens the time to the onset of OM permeabilization to periplasmic GFP and also the lag time between OM permeabilization and CM permeabilization. Meanwhile, the 12-h minimum inhibitory concentration (MIC) changes only twofold with core OS alterations. The results suggest a two-step model in which the core oligosaccharide layers act as a kinetic barrier to penetration of Cecropin A to the lipid A outer leaflet of the OM. Once a threshold concentration has built up at the lipid A leaflet, nucleation occurs and the OM is locally permeabilized to GFP and, by inference, to Cecropin A. Whenever Cecropin A permeabilizes the OM, CM permeabilization always follows, and cell growth subsequently halts and never recovers on the 45 min observation timescale. Copyright © 2018 Elsevier B.V. All rights reserved.

Rocket in situ observation of equatorial plasma irregularities in the region between E and F layers over Brazil

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S. Savio Odriozola

2017-03-01

Full Text Available A two-stage VS-30 Orion rocket was launched from the equatorial rocket launching station in Alcântara, Brazil, on 8 December 2012 soon after sunset (19:00 LT, carrying a Langmuir probe operating alternately in swept and constant bias modes. At the time of launch, ground equipment operated at equatorial stations showed rapid rise in the base of the F layer, indicating the pre-reversal enhancement of the F region vertical drift and creating ionospheric conditions favorable for the generation of plasma bubbles. Vertical profiles of electron density estimated from Langmuir probe data showed wave patterns and small- and medium-scale plasma irregularities in the valley region (100–300 km during the rocket upleg and downleg. These irregularities resemble those detected by the very high frequency (VHF radar installed at Jicamarca and so-called equatorial quasi-periodic echoes. We present evidence suggesting that these observations could be the first detection of this type of irregularity made by instruments onboard a rocket.

Nonlinear interaction of an intense radio wave with ionospheric D/E layer plasma

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Sodha, Mahendra Singh; Agarwal, Sujeet Kumar

2018-05-01

This paper considers the nonlinear interaction of an intense electromagnetic wave with the D/E layer plasma in the ionosphere. A simultaneous solution of the electromagnetic wave equation and the equations describing the kinetics of D/E layer plasma is obtained; the phenomenon of ohmic heating of electrons by the electric field of the wave causes enhanced collision frequency and ionization of neutral species. Electron temperature dependent recombination of electrons with ions, electron attachment to O 2 molecules, and detachment of electrons from O2 - ions has also been taken into account. The dependence of the plasma parameters on the square of the electric vector of the wave E0 2 has been evaluated for three ionospheric heights (viz., 90, 100, and 110 km) corresponding to the mid-latitude mid-day ionosphere and discussed; these results are used to investigate the horizontal propagation of an intense radio wave at these heights.

The Zodiacal Cloud Model applied to the Martian atmosphere. Diurnal variations in meteoric ion layers

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Carrillo-Sánchez, J. D.; Plane, J. M. C.; Withers, P.; Fallows, K.; Nesvorny, D.; Pokorný, P.

2016-12-01

Sporadic metal layers have been detected in the Martian atmosphere by radio occultation measurements using the Mars Express Orbiter and Mars Global Surveyor spacecraft. More recently, metallic ion layers produced by the meteor storm event following the close encounter between Comet Siding Spring (C/2013 A1) and Mars were identified by the Imaging UltraViolet Spectrograph (IUVS) and the Neutral Gas and Ion Mass Spectrometer (NGIMS) on the Mars Atmosphere and Volatile EvolutioN (MAVEN) spacecraft. Work is now in progress to detect the background metal layers produced by the influx of sporadic meteors. In this study we predict the likely appearance of these layers. The Zodiacal Dust Cloud (ZDC) model for particle populations released by asteroids (AST), and dust grains from Jupiter Family Comets (JFCs) and Halley-Type Comets (HTCs) has been combined with a Monte Carlo sampling method and the Chemical ABlation MODel (CABMOD) to predict the ablation rates of Na, K, Fe, Si, Mg, Ca and Al above 40 km altitude in the Martian atmosphere. CABMOD considers the standard treatment of meteor physics, including the balance of frictional heating by radiative losses and the absorption of heat energy through temperature increases, melting phase transitions and vaporization, as well as sputtering by inelastic collisions with the air molecules. The vertical injection profiles are input into the Leeds 1-D Mars atmospheric model which includes photo-ionization, and gas-phase ion-molecule and neutral chemistry, in order to explore the evolution of the resulting metallic ions and atoms. We conclude that the dominant contributor in the Martian's atmosphere is the JFCs over other sources. Finally, we explore the changes of the neutral and ionized Na, Mg and Fe layers over a diurnal cycle.

Effects of a conducting E layer on classical F region cross-field plasma diffusion

International Nuclear Information System (INIS)

Vickrey, J.F.; Kelley, M.C.

1982-01-01

The rate of cross-field plasma diffusion in the F region ionosphere is significantly increased when the magnetic field lines thread a highly conducting E region below. This reduces the lifetime of small-scale F region electron density irregularities in the polar ionosphere where the presence of a highly conducting E region is comonplace. A simple mmodel is developed to describe the effects of a conducting E layer on classical F region plasma diffusion. In the absence of an E region, the difference in ion and electron diffusion rates leads to a charge separation and, hence, to an electrostatic field that retards ion diffusion. When the highly conducting magnetic field lines are tied to a conducting E region, however, electrons can flow along B to reduce the ambipolar diffusion electric field, and ions can proceed perpendicular to B at a rate approaching their own (higher) diffusion velocity. It is shown that the enhanced total diffusion rate that results depends strongly on the height of the F layer and on the ratio of the E to F region Pedersen conductivities

Height of the E layer as a function of solar activity

International Nuclear Information System (INIS)

Antonova, L.A.; Ivanov-Kholodnyj, G.S.; Zhivolup, T.G.

1992-01-01

Variations of h m E height of E layer maximum with solar activity are investigated using data of from rocket measurements. These data are contradictory ones and requires more exact definition. h m E decrease with growth of solar activity is predicted theoretically: small monotone decrease during solar cycle on the one hand, and/or jump-like decrease of h m E at a certain intermediate value of F 10.7 - on the other hand. New rather reliable results are obtained due to data from incoherent dissipation station. On the basis of these measurements it is obtained that at low and even at rather high solar activity there is a small monotone decrease of h m E, while at intermediate activity, when F 10.7 =140-180 there occurs more abrupt variation of h m E

Comparison between hearing screening-detected cases and sporadic cases of delayed-onset hearing loss in preschool-age children.

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Lü, Jingrong; Huang, Zhiwu; Ma, Yan; Li, Yun; Mei, Ling; Yao, Guoyin; Wang, Yu; Shen, Xiaoming; Wu, Hao

2014-04-01

This study aimed to compare the diagnosis and ages of intervention for cases of delayed-onset hearing loss identified sporadically or via a preschool hearing screening program. Retrospective study with the comparative analysis of two groups of children. Cases identified from screening were selected from 34 321 preschool children who underwent screening for delayed-onset hearing loss between October 2009 and May 2011. Sporadic cases of delayed-onset hearing loss were selected from pediatric clinical records. Cases from the first group were excluded from the latter to avoid duplication. Two groups were given the same questionnaire to record risk indicators, diagnosis, and age at intervention. The average age of 26 children at the time of diagnosis in the screening group (52.81 ± 13.23 months) was significantly earlier than in the 33 cases identified in the sporadic group (62.03 ± 12.86 months; p children with bilateral moderate to severe hearing loss in the screening group (50.40 ± 10.76 months) was also earlier than in the sporadic group (62.73 ± 13.77 months; p hearing screening for preschool children with no significant symptoms of delayed-onset hearing loss.

Comparison of frailty of primary neurons, embryonic, and aging mouse cortical layers.

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Fugistier, Patrick; Vallet, Philippe G; Leuba, Geneviève; Piotton, Françoise; Marin, Pascale; Bouras, Constantin; Savioz, Armand

2014-02-01

Superficial layers I to III of the human cerebral cortex are more vulnerable toward Aβ peptides than deep layers V to VI in aging. Three models of layers were used to investigate this pattern of frailty. First, primary neurons from E14 and E17 embryonic murine cortices, corresponding respectively to future deep and superficial layers, were treated either with Aβ(1-42), okadaic acid, or kainic acid. Second, whole E14 and E17 embryonic cortices, and third, in vitro separated deep and superficial layers of young and old C57BL/6J mice, were treated identically. We observed that E14 and E17 neurons in culture were prone to death after the Aβ and particularly the kainic acid treatment. This was also the case for the superficial layers of the aged cortex, but not for the embryonic, the young cortex, and the deep layers of the aged cortex. Thus, the aged superficial layers appeared to be preferentially vulnerable against Aβ and kainic acid. This pattern of vulnerability corresponds to enhanced accumulation of senile plaques in the superficial cortical layers with aging and Alzheimer's disease. Copyright © 2014 Elsevier Inc. All rights reserved.

Association study between XRCC1 gene polymorphisms and sporadic amyotrophic lateral sclerosis.

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Coppedè, Fabio; Migheli, Francesca; Lo Gerfo, Annalisa; Fabbrizi, Maria Rita; Carlesi, Cecilia; Mancuso, Michelangelo; Corti, Stefania; Mezzina, Nicoletta; del Bo, Roberto; Comi, Giacomo P; Siciliano, Gabriele; Migliore, Lucia

2010-01-01

The aim of the present study was to investigate the possible contribution of three common functional polymorphisms in the DNA repair protein X-ray repair cross-complementing group 1 (XRCC1), namely Arg194Trp (rs1799782), Arg280His (rs25489) and Arg399Gln (rs25487), to sporadic amyotrophic lateral sclerosis (SALS). We genotyped 206 Italian SALS patients and 203 matched controls for XRCC1 Arg194Trp, Arg280His and Arg399Gln polymorphisms by means of PCR/RFLP technique, searching for association between any of the studied polymorphisms and disease risk, age and site of onset. We observed a statistically significant difference in XRCC1 Gln399 allele frequencies between SALS cases and controls (0.39/0.28; p=0.001). The present study suggests that the XRCC1 Arg399Gln polymorphism might contribute to SALS risk.

Síndrome hemolítico-urêmica esporádica pós-parto Sporadic postpartum hemolytic uremic syndrome

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Elza M. Moreira

2008-08-01

Full Text Available Anemia hemolítica microangiopática associado à trombocitopenia participa de um grupo de doenças que freqüentemente apresentam suas características clínicas muito semelhantes, sendo difícil distingui-las. A síndrome hemolítico-urêmica é dividida em duas apresentações: a forma não esporádica, que acomete comumente crianças após infecção bacteriana causando diarréia sanguinolenta, possui bom prognóstico; e a forma esporádica, que acomete adultos, sendo bem descritos casos em mulheres pósparto, é a forma sistêmica de trombocitopenia microangiopática de pior prognóstico com alta morbidade e mortalidade, cuja falência renal é o distúrbio predominante. Relatamos um caso de síndrome hemolítico-urêmica pós-parto em paciente previamente sadia, que apresentou quadro de insuficiência renal, anemia hemolítica e trombocitopenia. Instituída a terapêutica de suporte adequada e precocemente, a paciente evoluiu satisfatoriamente com normalização dos níveis pressóricos e recuperação da função renal.Microangiopathic hemolytic associated with thrombocytopenia is part of a disease group that frequently show likeness and that's why become difficult to separate them. There are two types of hemolytic uremic syndrome (HUS; the non sporadic type and the epidemic or "typical" type that is common on childreen that is associated with diarrhea and infection caused by verotoxinaproducing E. coli with a good prognostic; and the sporadic postpartum period. It is the systemic type of mocroangiophatic thrombocytopenia of poor prognostic with high morbidity and mortality which renal failure is the main disturb. We reported a case of HUS occuring in postpartum previously healthy, that showed abrupt renal failure, hemolytic anemia and thrombocytopenia. After proper therapy the patient developed a normal blood pressure and recovery renal function.

Formation of scandium nitride (ScN) layer on gallium arsenide (GaAs) substrate using a combined technique of e-beam evaporator and ammonia annealing treatment

Energy Technology Data Exchange (ETDEWEB)

Yong Shee Meng, Alvin [Institute of Nano Optoelectronics Research and Technology (INOR), sains@usm, Persiaran Bukit Jambul, 11900 Bayan Lepas, Penang (Malaysia); Zainal, Norzaini, E-mail: norzaini@usm.my [Nano Optoelectronics Research and Laboratory, Universiti Sains Malaysia, sains@usm, Persiaran Bukit Jambul, 11900, Bayan Lepas, Penang (Malaysia); Hassan, Zainuriah; Ibrahim, Kamarulazizi [Institute of Nano Optoelectronics Research and Technology (INOR), sains@usm, Persiaran Bukit Jambul, 11900 Bayan Lepas, Penang (Malaysia)

2015-12-30

Graphical abstract: - Highlights: • Forming ScN layer using electron e-beam evaporator with successive NH{sub 3} annealing thermal has been successfully demonstrated. • NH{sub 3} annealing played the role in changing the grain structure of the ScN layer. • The existence of Sc−N bonds was confirmed by XPS measurement. • The 900 °C annealed ScN layer showed the best structural and optical characteristics. • ScN layer annealed at 980 °C exhibited poor structural and optical characteristics. - Abstract: A demonstration on a new technique of growing ScN using electron beam (e-beam) evaporator, coupled with successive ammonia (NH{sub 3}) annealing treatment is presented in this paper. The annealing temperature was varied at 750, 800, 850, 900 and 980 °C in order to obtain the best ScN layer. It was found that as the annealing temperature increased, the surface morphology of the ScN layer changed and ScN grains formed abundantly on the surface. The best surface of ScN layer was found in the 900 °C annealed sample. However, the roughness of the ScN increased with temperature. The photoluminescence (PL) peak of the near-to-band-edge (NBE) of ScN was observable in all samples and its intensity was the highest in the 900 °C annealed sample. Note that when the annealing treatment was conducted at 980 °C, the GaN PL peak is observable. Raman peaks of TO(X) of ScN were much evident at the annealing temperature above 900 °C. The formation of Sc−N bonds was confirmed by X-ray spectroscopy (XPS) measurement. In the end of this work, we propose that the formation of ScN using the above techniques was successful, with thermal annealing at the temperature of 900 °C.

Formation of scandium nitride (ScN) layer on gallium arsenide (GaAs) substrate using a combined technique of e-beam evaporator and ammonia annealing treatment

International Nuclear Information System (INIS)

Yong Shee Meng, Alvin; Zainal, Norzaini; Hassan, Zainuriah; Ibrahim, Kamarulazizi

2015-01-01

Graphical abstract: - Highlights: • Forming ScN layer using electron e-beam evaporator with successive NH_3 annealing thermal has been successfully demonstrated. • NH_3 annealing played the role in changing the grain structure of the ScN layer. • The existence of Sc−N bonds was confirmed by XPS measurement. • The 900 °C annealed ScN layer showed the best structural and optical characteristics. • ScN layer annealed at 980 °C exhibited poor structural and optical characteristics. - Abstract: A demonstration on a new technique of growing ScN using electron beam (e-beam) evaporator, coupled with successive ammonia (NH_3) annealing treatment is presented in this paper. The annealing temperature was varied at 750, 800, 850, 900 and 980 °C in order to obtain the best ScN layer. It was found that as the annealing temperature increased, the surface morphology of the ScN layer changed and ScN grains formed abundantly on the surface. The best surface of ScN layer was found in the 900 °C annealed sample. However, the roughness of the ScN increased with temperature. The photoluminescence (PL) peak of the near-to-band-edge (NBE) of ScN was observable in all samples and its intensity was the highest in the 900 °C annealed sample. Note that when the annealing treatment was conducted at 980 °C, the GaN PL peak is observable. Raman peaks of TO(X) of ScN were much evident at the annealing temperature above 900 °C. The formation of Sc−N bonds was confirmed by X-ray spectroscopy (XPS) measurement. In the end of this work, we propose that the formation of ScN using the above techniques was successful, with thermal annealing at the temperature of 900 °C.

S182 and STM2 gene missense mutations in sporadic alzheimer disease

Energy Technology Data Exchange (ETDEWEB)

Higuchi, Susumu; Matsushita, Sachio; Hasegawa, Yoshio; Muramatsu, Taro [Kurihama National Hospital, Yokosuka (Japan)] [and others

1996-07-26

The linkage of genes S182 and STM2 to early-onset or late-onset sporadic Alzheimer disease (AD) was not found in a group of 97 clinically-diagnosed AD patients and 46 autopsy-confirmed AD cases, using PCR-RFLP methods. 7 refs.

Anorectal function and morphology in patients with sporadic proctalgia fugax.

Science.gov (United States)

Eckardt, V F; Dodt, O; Kanzler, G; Bernhard, G

1996-07-01

The pathophysiology of sporadic proctalgia fugax remains unknown. This study investigates whether patients with this syndrome exhibit alterations in anal function and morphology. Eighteen patients with sporadic proctalgia fugax and 18 sex-matched and age-matched healthy controls were studied. Manometric studies investigated anal resting and squeeze pressures, the rectoanal inhibitory reflex, rectal compliance, and smooth muscle response to edrophonium chloride administration. External and internal sphincter thickness was measured endosonographically. Patients had slightly higher (P = 0.0291) anal resting pressures (65.5 +/- 11.4 mmHg) than controls (56 +/- 9.9 mmHg). However, anal squeeze pressure, sphincter relaxation during rectal distention, and rectal compliance were similar in both groups, and no alterations were detected in external and internal anal sphincter thickness. Edrophonium chloride administration was followed by sharp postrelaxation contractions in two patients, whereas anal function remained unaltered in controls. Acute episodes of proctalgia, which occurred in two patients while under study, were associated with a rise in anal resting tone and an increase in slow wave amplitude. In the resting state, patients with proctalgia fugax have normal anorectal function and morphology. However, they may exhibit a motor abnormality of the anal smooth muscle during an acute attack.

Interactions between C and Cu atoms in single-layer graphene: direct observation and modelling.

Science.gov (United States)

Kano, Emi; Hashimoto, Ayako; Kaneko, Tomoaki; Tajima, Nobuo; Ohno, Takahisa; Takeguchi, Masaki

2016-01-07

Metal doping into the graphene lattice has been studied recently to develop novel nanoelectronic devices and to gain an understanding of the catalytic activities of metals in nanocarbon structures. Here we report the direct observation of interactions between Cu atoms and single-layer graphene by transmission electron microscopy. We document stable configurations of Cu atoms in the graphene sheet and unique transformations of graphene promoted by Cu atoms. First-principles calculations based on density functional theory reveal a reduction of energy barrier that caused rotation of C-C bonds near Cu atoms. We discuss two driving forces, electron irradiation and in situ heating, and conclude that the observed transformations were mainly promoted by electron irradiation. Our results suggest that individual Cu atoms can promote reconstruction of single-layer graphene.

MRI in sporadic Creutzfeldt-Jakob disease: Correlation with clinical and neuropathological data

International Nuclear Information System (INIS)

Urbach, H.; Solymosi, L.; Klisch, J.; Brechtelsbauer, D.; Wolf, H.K.; Gass, S.

1998-01-01

To ascertain whether increased grey matter signal intensity on T2-weighted images in patients with sporadic Creutzfeldt-Jakob disease (CJD) corresponds to the stage and severity of this disease, we correlated MRI findings in four of our own and previously reported patients with sporadic CJD with the clinical variants, neuropathological changes at autopsy, duration of the disease and survival time after MRI examination. Of 15 patients with the extrapyramidal type of CJD, 10 showed increased signal in the basal ganglia on T2-weighted images. One of seven patients with the Heidenhain variant had increased signal in the occipital cortex. Patients without increased grey matter signal intensity had a longer overall duration of CJD (P = 0.035). Although the interval between onset of neurological symptoms and MRI was not different, patients without increased grey matter signal also survived longer after MRI examination (P = 0.022). (orig.)

Exome-wide association study reveals novel susceptibility genes to sporadic dilated cardiomyopathy.

Directory of Open Access Journals (Sweden)

Ulrike Esslinger

Full Text Available Dilated cardiomyopathy (DCM is an important cause of heart failure with a strong familial component. We performed an exome-wide array-based association study (EWAS to assess the contribution of missense variants to sporadic DCM.116,855 single nucleotide variants (SNVs were analyzed in 2796 DCM patients and 6877 control subjects from 6 populations of European ancestry. We confirmed two previously identified associations with SNVs in BAG3 and ZBTB17 and discovered six novel DCM-associated loci (Q-value<0.01. The lead-SNVs at novel loci are common and located in TTN, SLC39A8, MLIP, FLNC, ALPK3 and FHOD3. In silico fine mapping identified HSPB7 as the most likely candidate at the ZBTB17 locus. Rare variant analysis (MAF<0.01 demonstrated significant association for TTN variants only (P = 0.0085. All candidate genes but one (SLC39A8 exhibit preferential expression in striated muscle tissues and mutations in TTN, BAG3, FLNC and FHOD3 are known to cause familial cardiomyopathy. We also investigated a panel of 48 known cardiomyopathy genes. Collectively, rare (n = 228, P = 0.0033 or common (n = 36, P = 0.019 variants with elevated in silico severity scores were associated with DCM, indicating that the spectrum of genes contributing to sporadic DCM extends beyond those identified here.We identified eight loci independently associated with sporadic DCM. The functions of the best candidate genes at these loci suggest that proteostasis regulation might play a role in DCM pathophysiology.

Gender- and age-dependent gamma-secretase activity in mouse brain and its implication in sporadic Alzheimer disease.

Directory of Open Access Journals (Sweden)

Lisa Placanica

Full Text Available Alzheimer disease (AD is an age-related disorder. Aging and female gender are two important risk factors associated with sporadic AD. However, the mechanism by which aging and gender contribute to the pathogenesis of sporadic AD is unclear. It is well known that genetic mutations in gamma-secretase result in rare forms of early onset AD due to the aberrant production of Abeta42 peptides, which are the major constituents of senile plaques. However, the effect of age and gender on gamma-secretase has not been fully investigated. Here, using normal wild-type mice, we show mouse brain gamma-secretase exhibits gender- and age-dependent activity. Both male and female mice exhibit increased Abeta42ratioAbeta40 ratios in aged brain, which mimics the effect of familial mutations of Presenilin-1, Presenlin-2, and the amyloid precursor protein on Abeta production. Additionally, female mice exhibit much higher gamma-secretase activity in aged brain compared to male mice. Furthermore, both male and female mice exhibit a steady decline in Notch1 gamma-secretase activity with aging. Using a small molecule affinity probe we demonstrate that male mice have less active gamma-secretase complexes than female mice, which may account for the gender-associated differences in activity in aged brain. These findings demonstrate that aging can affect gamma-secretase activity and specificity, suggesting a role for gamma-secretase in sporadic AD. Furthermore, the increased APP gamma-secretase activity seen in aged females may contribute to the increased incidence of sporadic AD in women and the aggressive Abeta plaque pathology seen in female mouse models of AD. In addition, deceased Notch gamma-secretase activity may also contribute to neurodegeneration. Therefore, this study implicates altered gamma-secretase activity and specificity as a possible mechanism of sporadic AD during aging.

Health professions and risk of sporadic Creutzfeldt- Jakob disease, 1965 to 2010

NARCIS (Netherlands)

E. Alcalde-Cabero; J. Almazán-Isla; J-P. Brandel (Jean-Philippe); M. Breithaupt; J. Catarino; S.J. Collins (Steven); J. Haybäck; R. Höftberger (Romana); E. Kahana; G.G. Kovacs (Gabor); A. Ladogana (Anna); E. Mitrová (Eva); A. Molesworth; Y. Nakamura; M. Pocchiari (Maurizio); M. Popovic; M. Ruiz-Tovar; A. Taratuto; C. van Duin; M. Yamada; R.G. Will (Robert); I. Zerr (Inga); J. de Pedro-Cuesta (Jesús)

2012-01-01

textabstractIn 2009, a pathologist with sporadic Creutzfeldt- Jakob Disease (sCJD) was reported to the Spanish registry. This case prompted a request for information on health-related occupation in sCJD cases from countries participating in the European Creutzfeldt Jakob Disease Surveillance network

TDP-43 in Familial and Sporadic Frontotemporal Lobar Degeneration with Ubiquitin Inclusions

NARCIS (Netherlands)

Cairns, Nigel J.; Neumann, Manuela; Bigio, Eileen H.; Holm, Ida E.; Troost, Dirk; Hatanpaa, Kimmo J.; Foong, Chan; White, Charles L.; Schneider, Julie A.; Kretzschmar, Hans A.; Carter, Deborah; Taylor-Reinwald, Lisa; Paulsmeyer, Katherine; Strider, Jeffrey; Gitcho, Michael; Goate, Alison M.; Morris, John C.; Mishrall, Manjari; Kwong, Linda K.; Stieber, Anna; Xu, Yan; Forman, Mark S.; Trojanowski, John Q.; Lee, Virginia M.-Y.; Mackenzie, Ian R. A.

2007-01-01

TAR DNA-binding protein 43 (TDP-43) is a major pathological protein of sporadic and familial frontotemporal lobar degeneration with ubiquitin-positive, tau-negative inclusions (FTLD-U) with or without motor neuron disease (MND). Thus, TDP-43 defines a novel class of neurodegenerative diseases called

High-resolution backscatter power observations of 440-MHz E region coherent echoes at Millstone Hill

International Nuclear Information System (INIS)

Foster, J.C.; Tetenbaum, D.

1991-01-01

A 40-μs pulse length has been used to provide 10-s temporal and 6-km range resolution observations of E region coherent backscatter from the premidnight eastward electrojet region to the north of Millstone Hill. The observations can be divided into two categories: strong events in which the backscattered amplitude nears saturation and weak events in which spatial structure and large-amplitude variations are common. Calibrated observations find a typical volume scattering coefficient of ∼10 -11 m -1 at 440 MHz during strong events with a maximum level of 9 x 10 -10 m -1 observed for brief intervals. During less intense events the radar backscatter is modulated by ∼30dB in amplitude at Pc 5 frequencies (150-500 s) by waves with spatial wavelength 50-100 km. The observations support the premise that the weak irregularities grow linearly with electric field strength and reach a saturation amplitude beyond which the oscillating electric field of the Pc pulsation has little effect. The observed variation of backscattered power with range is interpreted using a geometrical model which accounts for the detailed antenna beam pattern, a magnetic aspect angle sensitivity of -10 dB per degree, and a thin layer of irregularities centered at 110 km altitude. For strongly driven conditions a comparison of the range variation of backscattered power with the thin layer model suggests that the signal power becomes increasingly dominated by strong scatters confined to a narrower altitude range. The apparent altitude extent of the strongest irregularities decreases by a factor of 2 as the amplitude of the backscattered signal increases by a factor of 10

Glycoform-Selective Prion Formation in Sporadic and Familial Forms of Prion Disease

Science.gov (United States)

Xiao, Xiangzhu; Yuan, Jue; Haïk, Stéphane; Cali, Ignazio; Zhan, Yian; Moudjou, Mohammed; Li, Baiya; Laplanche, Jean-Louis; Laude, Hubert; Langeveld, Jan; Gambetti, Pierluigi; Kitamoto, Tetsuyuki; Kong, Qingzhong; Brandel, Jean-Philippe; Cobb, Brian A.; Petersen, Robert B.; Zou, Wen-Quan

2013-01-01

The four glycoforms of the cellular prion protein (PrPC) variably glycosylated at the two N-linked glycosylation sites are converted into their pathological forms (PrPSc) in most cases of sporadic prion diseases. However, a prominent molecular characteristic of PrPSc in the recently identified variably protease-sensitive prionopathy (VPSPr) is the absence of a diglycosylated form, also notable in familial Creutzfeldt-Jakob disease (fCJD), which is linked to mutations in PrP either from Val to Ile at residue 180 (fCJDV180I) or from Thr to Ala at residue 183 (fCJDT183A). Here we report that fCJDV180I, but not fCJDT183A, exhibits a proteinase K (PK)-resistant PrP (PrPres) that is markedly similar to that observed in VPSPr, which exhibits a five-step ladder-like electrophoretic profile, a molecular hallmark of VPSPr. Remarkably, the absence of the diglycosylated PrPres species in both fCJDV180I and VPSPr is likewise attributable to the absence of PrPres glycosylated at the first N-linked glycosylation site at residue 181, as in fCJDT183A. In contrast to fCJDT183A, both VPSPr and fCJDV180I exhibit glycosylation at residue 181 on di- and monoglycosylated (mono181) PrP prior to PK-treatment. Furthermore, PrPV180I with a typical glycoform profile from cultured cells generates detectable PrPres that also contains the diglycosylated PrP in addition to mono- and unglycosylated forms upon PK-treatment. Taken together, our current in vivo and in vitro studies indicate that sporadic VPSPr and familial CJDV180I share a unique glycoform-selective prion formation pathway in which the conversion of diglycosylated and mono181 PrPC to PrPSc is inhibited, probably by a dominant-negative effect, or by other co-factors. PMID:23527023

Risk factors and analysis of long-term headache in sporadic vestibular schwannoma: a multicenter cross-sectional study.

Science.gov (United States)

Carlson, Matthew L; Tveiten, Øystein Vesterli; Driscoll, Colin L; Boes, Christopher J; Sullan, Molly J; Goplen, Frederik K; Lund-Johansen, Morten; Link, Michael J

2015-11-01

The primary goals of this study were: 1) to examine the influence of disease and treatment on headache in patients with sporadic vestibular schwannoma (VS); and 2) to identify clinical predictors of long-term headache disability. This was a cross-sectional observational study with international multicenter enrollment. Patients included those with primary sporadic Headache Disability Inventory (HDI), the Hospital Anxiety and Depression Scale, and a VS symptom questionnaire. The main outcome measures were univariate and multivariable associations with HDI total score. The overall survey response rate was 79%. Data from 538 patients with VS were analyzed. The mean age at time of survey was 64 years, 56% of patients were female, and the average duration between treatment and survey was 7.7 years. Twenty-seven percent of patients received microsurgery, 46% stereotactic radiosurgery, and 28% observation. Patients with VS who were managed with observation were more than twice as likely to have severe headache disability compared with 103 control subjects without VS. When accounting for baseline differences, there was no statistically significant difference in HDI outcome between treatment modalities at time of survey. Similarly, among the microsurgery cohort, the long-term risk of severe headache disability was not different between surgical approaches. Multivariable regression demonstrated that younger age, greater anxiety and depression, and a preexisting diagnosis of headache were the primary predictors of severe long-term headache disability, while tumor size and treatment modality had little influence. At a mean of almost 8 years following treatment, approximately half of patients with VS experience headaches of varying frequency and severity. Patient-driven factors including age, sex, mental health, and preexisting headache syndrome are the strongest predictors of long-term severe headache disability. Tumor size and treatment modality have less impact. These data

Sporadic Creutzfeldt-Jakob Disease MM1+2C and MM1 are Identical in Transmission Properties.

Science.gov (United States)

Kobayashi, Atsushi; Matsuura, Yuichi; Iwaki, Toru; Iwasaki, Yasushi; Yoshida, Mari; Takahashi, Hitoshi; Murayama, Shigeo; Takao, Masaki; Kato, Shinsuke; Yamada, Masahito; Mohri, Shirou; Kitamoto, Tetsuyuki

2016-01-01

The genotype (methionine, M or valine, V) at polymorphic codon 129 of the PRNP gene and the type (1 or 2) of abnormal prion protein in the brain are the major determinants of the clinicopathological features of sporadic Creutzfeldt-Jakob disease (CJD), thus providing molecular basis for classification of sporadic CJD, that is, MM1, MM2, MV1, MV2, VV1 or VV2. In addition to these "pure" cases, "mixed" cases presenting mixed neuropathological and biochemical features have also been recognized. The most frequently observed mixed form is the co-occurrence of MM1 and MM2, namely MM1+2. However, it has remained elusive whether MM1+2 could be a causative origin of dura mater graft-associated CJD (dCJD), one of the largest subgroups of iatrogenic CJD. To test this possibility, we performed transmission experiments of MM1+2 prions and a systematic neuropathological examination of dCJD patients in the present study. The transmission properties of the MM1+2 prions were identical to those of MM1 prions because MM2 prions lacked transmissibility. In addition, the neuropathological characteristics of MM2 were totally absent in dCJD patients examined. These results suggest that MM1+2 can be a causative origin of dCJD and causes neuropathological phenotype similar to that of MM1. © 2015 International Society of Neuropathology.

Characterizing permafrost active layer dynamics and sensitivity to landscape spatial heterogeneity in Alaska

Science.gov (United States)

Yi, Yonghong; Kimball, John S.; Chen, Richard H.; Moghaddam, Mahta; Reichle, Rolf H.; Mishra, Umakant; Zona, Donatella; Oechel, Walter C.

2018-01-01

An important feature of the Arctic is large spatial heterogeneity in active layer conditions, which is generally poorly represented by global models and can lead to large uncertainties in predicting regional ecosystem responses and climate feedbacks. In this study, we developed a spatially integrated modeling and analysis framework combining field observations, local-scale ( ˜ 50 m resolution) active layer thickness (ALT) and soil moisture maps derived from low-frequency (L + P-band) airborne radar measurements, and global satellite environmental observations to investigate the ALT sensitivity to recent climate trends and landscape heterogeneity in Alaska. Modeled ALT results show good correspondence with in situ measurements in higher-permafrost-probability (PP ≥ 70 %) areas (n = 33; R = 0.60; mean bias = 1.58 cm; RMSE = 20.32 cm), but with larger uncertainty in sporadic and discontinuous permafrost areas. The model results also reveal widespread ALT deepening since 2001, with smaller ALT increases in northern Alaska (mean trend = 0.32±1.18 cm yr-1) and much larger increases (> 3 cm yr-1) across interior and southern Alaska. The positive ALT trend coincides with regional warming and a longer snow-free season (R = 0.60 ± 0.32). A spatially integrated analysis of the radar retrievals and model sensitivity simulations demonstrated that uncertainty in the spatial and vertical distribution of soil organic carbon (SOC) was the largest factor affecting modeled ALT accuracy, while soil moisture played a secondary role. Potential improvements in characterizing SOC heterogeneity, including better spatial sampling of soil conditions and advances in remote sensing of SOC and soil moisture, will enable more accurate predictions of active layer conditions and refinement of the modeling framework across a larger domain.

MRI in sporadic Creutzfeldt-Jakob disease: Correlation with clinical and neuropathological data

Energy Technology Data Exchange (ETDEWEB)

Urbach, H.; Solymosi, L. [Department of Neuroradiology, University of Wuerzburg (Germany); Klisch, J.; Brechtelsbauer, D. [Department of Neuroradiology, University of Bonn, Bonn (Germany); Wolf, H.K. [Department of Neuropathology, University of Bonn, Bonn (Germany); Gass, S. [Department of Neurology, University of Bonn, Bonn (Germany)

1998-02-01

To ascertain whether increased grey matter signal intensity on T2-weighted images in patients with sporadic Creutzfeldt-Jakob disease (CJD) corresponds to the stage and severity of this disease, we correlated MRI findings in four of our own and previously reported patients with sporadic CJD with the clinical variants, neuropathological changes at autopsy, duration of the disease and survival time after MRI examination. Of 15 patients with the extrapyramidal type of CJD, 10 showed increased signal in the basal ganglia on T2-weighted images. One of seven patients with the Heidenhain variant had increased signal in the occipital cortex. Patients without increased grey matter signal intensity had a longer overall duration of CJD (P = 0.035). Although the interval between onset of neurological symptoms and MRI was not different, patients without increased grey matter signal also survived longer after MRI examination (P = 0.022). (orig.) With 5 figs., 2 tabs., 23 refs.

Correlation of RET somatic mutations with clinicopathological features in sporadic medullary thyroid carcinomas

Science.gov (United States)

Moura, M M; Cavaco, B M; Pinto, A E; Domingues, R; Santos, J R; Cid, M O; Bugalho, M J; Leite, V

2009-01-01

Screening of REarranged during Transfection (RET) gene mutations has been carried out in different series of sporadic medullary thyroid carcinomas (MTC). RET-positive tumours seem to be associated to a worse clinical outcome. However, the correlation between the type of RET mutation and the patients' clinicopathological data has not been evaluated yet. We analysed RET exons 5, 8, 10–16 in fifty-one sporadic MTC, and found somatic mutations in thirty-three (64.7%) tumours. Among the RET-positive cases, exon 16 was the most frequently affected (60.6%). Two novel somatic mutations (Cys630Gly, c.1881del18) were identified. MTC patients were divided into three groups: group 1, with mutations in RET exons 15 and 16; group 2, with other RET mutations; group 3, having no RET mutations. Group 1 had higher prevalence (P=0.0051) and number of lymph node metastases (P=0.0017), and presented more often multifocal tumours (P=0.037) and persistent disease at last control (P=0.0242) than group 2. Detectable serum calcitonin levels at last screening (P=0.0119) and stage IV disease (P=0.0145) were more frequent in group 1, than in the other groups. Our results suggest that, among the sporadic MTC, cases with RET mutations in exons 15 and 16 are associated with the worst prognosis. Cases with other RET mutations have the most indolent course, and those with no RET mutations have an intermediate risk. PMID:19401695

Genetic architecture of sporadic frontotemporal dementia and overlap with Alzheimer's and Parkinson's diseases

DEFF Research Database (Denmark)

Ferrari, Raffaele; Wang, Yunpeng; Vandrovcova, Jana

2017-01-01

BACKGROUND: Clinical, pathological and genetic overlap between sporadic frontotemporal dementia (FTD), Alzheimer's disease (AD) and Parkinson's disease (PD) has been suggested; however, the relationship between these disorders is still not well understood. Here we evaluated genetic overlap between...

Prion-Seeding Activity Is widely Distributed in Tissues of Sporadic Creutzfeldt-Jakob Disease Patients

Directory of Open Access Journals (Sweden)

Hanae Takatsuki, PhD

2016-10-01

Full Text Available Human prion diseases are neurodegenerative disorders caused by abnormally folded prion proteins in the central nervous system. These proteins can be detected using the quaking-induced conversion assay. Compared with other bioassays, this assay is extremely sensitive and was used in the present study to determine prion distribution in sporadic Creutzfeldt-Jakob disease patients at autopsy. Although infectivity of the sporadic form is thought to be restricted within the central nervous system, results showed that prion-seeding activities reach 106/g from a 50% seeding dose in non-neuronal tissues, suggesting that prion-seeding activity exists in non-neural organs, and we suggested that non-neural tissues of 106/g SD50 did not exist the infectivity.

Triple-Layer Vascular Grafts Fabricated by Combined E-Jet 3D Printing and Electrospinning.

Science.gov (United States)

Huang, Ruiying; Gao, Xiangkai; Wang, Jian; Chen, Haoxiang; Tong, Chunyi; Tan, Yongjun; Tan, Zhikai

2018-05-29

Small-diameter tissue-engineered vascular grafts are urgently needed for clinic arterial substitute. To simulate the structures and functions of natural blood vessels, we designed a novel triple-layer poly(ε-caprolactone) (PCL) fibrous vascular graft by combining E-jet 3D printing and electrospinning techniques. The resultant vascular graft consisted of an interior layer comprising 3D-printed highly aligned strong fibers, a middle layer made by electrospun densely fibers, and an exterior structure composed of mixed fibers fabricated by co-electrospraying. The biocompatible triple-layer graft was used for in vivo implantation, and results demonstrated that the longitudinally-aligned fibers within the lumen of the graft could enhance the proliferation and migration of endothelial cells, while maintained good mechanical properties. The exterior layer provided a pathway that encouraged cells to migrate into the scaffold after implantation. This experimental graft overcame the limitations of conventionally electrospun vascular grafts of inadequate porosity and lowly cell penetration. The unique structure of the triple-layer vascular graft promoted cell growth and infiltration in vivo, thus provided an encouraging substitute for in situ tissue engineering.

Glycoform-selective prion formation in sporadic and familial forms of prion disease

NARCIS (Netherlands)

Xiao, X.; Yuan, J.; Haïk, S.; Cali, I.; Zhan, Y.; Moudjou, M.; Li, B.; Laplanche, J.L.; Laude, H.; Langeveld, J.P.M.; Gambetti, P.

2013-01-01

The four glycoforms of the cellular prion protein (PrP(C)) variably glycosylated at the two N-linked glycosylation sites are converted into their pathological forms (PrP(Sc)) in most cases of sporadic prion diseases. However, a prominent molecular characteristic of PrP(Sc) in the recently identified

Gene expression profiling for human iPS-derived motor neurons from sporadic ALS patients reveals a strong association between mitochondrial functions and neurodegeneration

Science.gov (United States)

Alves, Chrystian J.; Dariolli, Rafael; Jorge, Frederico M.; Monteiro, Matheus R.; Maximino, Jessica R.; Martins, Roberto S.; Strauss, Bryan E.; Krieger, José E.; Callegaro, Dagoberto; Chadi, Gerson

2015-01-01

Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disease that leads to widespread motor neuron death, general palsy and respiratory failure. The most prevalent sporadic ALS form is not genetically inherited. Attempts to translate therapeutic strategies have failed because the described mechanisms of disease are based on animal models carrying specific gene mutations and thus do not address sporadic ALS. In order to achieve a better approach to study the human disease, human induced pluripotent stem cell (hiPSC)-differentiated motor neurons were obtained from motor nerve fibroblasts of sporadic ALS and non-ALS subjects using the STEMCCA Cre-Excisable Constitutive Polycistronic Lentivirus system and submitted to microarray analyses using a whole human genome platform. DAVID analyses of differentially expressed genes identified molecular function and biological process-related genes through Gene Ontology. REVIGO highlighted the related functions mRNA and DNA binding, GTP binding, transcription (co)-repressor activity, lipoprotein receptor binding, synapse organization, intracellular transport, mitotic cell cycle and cell death. KEGG showed pathways associated with Parkinson's disease and oxidative phosphorylation, highlighting iron homeostasis, neurotrophic functions, endosomal trafficking and ERK signaling. The analysis of most dysregulated genes and those representative of the majority of categorized genes indicates a strong association between mitochondrial function and cellular processes possibly related to motor neuron degeneration. In conclusion, iPSC-derived motor neurons from motor nerve fibroblasts of sporadic ALS patients may recapitulate key mechanisms of neurodegeneration and may offer an opportunity for translational investigation of sporadic ALS. Large gene profiling of differentiated motor neurons from sporadic ALS patients highlights mitochondrial participation in the establishment of autonomous mechanisms associated with sporadic ALS

Typhoon 9707 observations with the MU radar and L-band boundary layer radar

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M. Teshiba

2001-08-01

Full Text Available Typhoon 9707 (Opal was observed with the VHF-band Middle and Upper atmosphere (MU radar, an L-band boundary layer radar (BLR, and a vertical-pointing C-band meteorological radar at the Shigaraki MU Observatory in Shiga prefecture, Japan on 20 June 1997. The typhoon center passed about 80 km southeast from the radar site. Mesoscale precipitating clouds developed due to warm-moist airmass transport from the typhoon, and passed over the MU radar site with easterly or southeasterly winds. We primarily present the wind behaviour including the vertical component which a conventional meteorological Doppler radar cannot directly observe, and discuss the relationship between the wind behaviour of the typhoon and the precipitating system. To investigate the dynamic structure of the typhoon, the observed wind was divided into radial and tangential wind components under the assumption that the typhoon had an axi-symmetric structure. Altitude range of outflow ascended from 1–3 km to 2–10 km with increasing distance (within 80–260 km range from the typhoon center, and in-flow was observed above and below the outflow. Outflow and inflow were associated with updraft and downdraft, respectively. In the tangential wind, the maximum speed of counterclockwise winds was confirmed at 1–2 km altitudes. Based on the vertical velocity and the reflectivity obtained with the MU radar and the C-band meteorological radar, respectively, precipitating clouds, accompanied by the wind behaviour of the typhoon, were classified into stratiform and convective precipitating clouds. In the stratiform precipitating clouds, a vertical shear of radial wind and the maximum speed of counterclockwise wind were observed. There was a strong reflectivity layer called a ‘bright band’ around the 4.2 km altitude. We confirmed strong updrafts and down-drafts below and above it, respectively, and the existence of a relatively dry layer around the bright band level from radiosonde

Typhoon 9707 observations with the MU radar and L-band boundary layer radar

Directory of Open Access Journals (Sweden)

M. Teshiba

Full Text Available Typhoon 9707 (Opal was observed with the VHF-band Middle and Upper atmosphere (MU radar, an L-band boundary layer radar (BLR, and a vertical-pointing C-band meteorological radar at the Shigaraki MU Observatory in Shiga prefecture, Japan on 20 June 1997. The typhoon center passed about 80 km southeast from the radar site. Mesoscale precipitating clouds developed due to warm-moist airmass transport from the typhoon, and passed over the MU radar site with easterly or southeasterly winds. We primarily present the wind behaviour including the vertical component which a conventional meteorological Doppler radar cannot directly observe, and discuss the relationship between the wind behaviour of the typhoon and the precipitating system. To investigate the dynamic structure of the typhoon, the observed wind was divided into radial and tangential wind components under the assumption that the typhoon had an axi-symmetric structure. Altitude range of outflow ascended from 1–3 km to 2–10 km with increasing distance (within 80–260 km range from the typhoon center, and in-flow was observed above and below the outflow. Outflow and inflow were associated with updraft and downdraft, respectively. In the tangential wind, the maximum speed of counterclockwise winds was confirmed at 1–2 km altitudes. Based on the vertical velocity and the reflectivity obtained with the MU radar and the C-band meteorological radar, respectively, precipitating clouds, accompanied by the wind behaviour of the typhoon, were classified into stratiform and convective precipitating clouds. In the stratiform precipitating clouds, a vertical shear of radial wind and the maximum speed of counterclockwise wind were observed. There was a strong reflectivity layer called a ‘bright band’ around the 4.2 km altitude. We confirmed strong updrafts and down-drafts below and above it, respectively, and the existence of a relatively dry layer around the bright band level from radiosonde

Numerical study on effect of boundary layer trips on aerodynamic performance of E216 airfoil

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B.K. Sreejith

2018-02-01

Full Text Available Simulation is carried out to find the performance of airfoil E216 using Transition γ-Reθ model at Reynolds number of 100,000. Flow behaviour and effect of angle of attack (AOA on laminar separation bubble (LSB formation are examined. The results are validated with wind tunnel experimental results. LSB formation is clearly spotted in the velocity vector plot and coefficient of pressure distribution over airfoil. LSB moved upstream towards the leading edge with increase in AOA. Effect of boundary layer trip on LSB formation over the airfoil and performance of airfoil are studied. Two different trip locations, 17% of chord and 10% of chord from leading edge, and different trip heights (0.3 mm, 0.5 mm, 0.7 mm, 1 mm are investigated in this study. Results showed that boundary layer trip could eliminate LSB partially or completely and improve aerodynamic performance of the airfoil. Maximum improvement in drag by 15.48% and lift to drag ratio by 21.62% are obtained at angle of attack of 60. In all the cases, improvement in performance is observed only up to trip height of 0.5 mm.

A large-scale international meta-analysis of paraoxonase gene polymorphisms in sporadic ALS

NARCIS (Netherlands)

Wills, A-M.; Cronin, S.; Slowik, A.; Kasperaviciute, D.; Van Es, M. A.; Morahan, J. M.; Valdmanis, P. N.; Meininger, V.; Melki, J.; Shaw, C. E.; Rouleau, G. A.; Fisher, E. M. C.; Shaw, P. J.; Morrison, K. E.; Pamphlett, R.; Van den Berg, L. H.; Figlewicz, D. A.; Andersen, P. M.; Al-Chalabi, A.; Hardiman, O.; Purcell, S.; Landers, J. E.; Brown, R. H.

2009-01-01

Background: Six candidate gene studies report a genetic association of DNA variants within the paraoxonase locus with sporadic amyotrophic lateral sclerosis (ALS). However, several other large studies, including five genome-wide association studies, have not duplicated this finding. Methods: We

Observations of transitional tidal boundary layers and their impact on sediment transport in the Great Bay, NH

Science.gov (United States)

Koetje, K. M.; Foster, D. L.; Lippmann, T. C.

2017-12-01

Observations of the vertical structure of tidal flows obtained in 2016 and 2017 in the Great Bay Estuary, NH show evidence of transitional tidal boundary layers at deployment locations on shallow mudflats. High-resolution bottom boundary layer currents, hydrography, turbidity, and bed characteristics were observed with an acoustic Doppler current profiler (ADCP), an acoustic Doppler velocimeter (ADV), conductivity-depth-temperature (CTD) sensors, optical backscatter sensors, multibeam bathymetric surveys, and sediment grab samples and cores. Over the 2.5 m tidal range and at water depths ranging from 0.3 m to 1.5 m at mean lower low water, peak flows ranged from 10 cm/s to 30 cm/s and were primarily driven by the tides. A downward-looking ADCP captured the velocity profile over the lowest 1 m of the water column. Results consistently show a dual-log layer system, with evidence of a lower layer within 15 cm of the bed, another layer above approximately 30 cm from the bed, and a transitional region where the flow field rotates between that the two layers that can be as much as 180 degrees out of phase. CTD casts collected over a complete tidal cycle suggest that the weak thermohaline stratification is not responsible for development of the two layers. On the other hand, acoustic and optical backscatter measurements show spatial and temporal variability in suspended sediments that are dependant on tidal phase. Current work includes an examination of the relationship between sediment concentrations in the water column and velocity profile characteristics, along with an effort to quantify the impact of rotation and dual-log layers on bed stress.

Analysis of FGGY as a risk factor for sporadic amyotrophic lateral sclerosis.

NARCIS (Netherlands)

Es, M.A. van; Vught, P.W. van; Veldink, J.H.; Andersen, P.M.; Birve, A.; Lemmens, R.; Cronin, S.; Kooi, A.J. van der; Visser, M. de; Schelhaas, H.J.; Hardiman, O.; Ragoussis, I.; Lambrechts, D.; Robberecht, W.; Wokke, J.H.J.; Ophoff, R.A.; Berg, L.H. van den

2009-01-01

A genome-wide association study (GWAS) using pooled DNA samples from 386 sporadic ALS patients and 542 controls from the USA, identified genetic variation in FGGY (FLJ10986) as a risk factor, as well as 66 additional candidate SNPs. Considering the large number of hypotheses that are tested in GWAS,

The clinical phenotype of hereditary versus sporadic prostate cancer: HPC definition revisited

NARCIS (Netherlands)

Cremers, R.G.H.M.; Aben, K.K.H.; Oort, I.M. van; Sedelaar, J.P.M.; Vasen, H.F.A.; Vermeulen, S.H.; Kiemeney, L.A.L.M.

2016-01-01

BACKGROUND: The definition of hereditary prostate cancer (HPC) is based on family history and age at onset. Intuitively, HPC is a serious subtype of prostate cancer but there are only limited data on the clinical phenotype of HPC. Here, we aimed to compare the prognosis of HPC to the sporadic form

Evaluating Models Of The Neutral, Barotropic Planetary Boundary Layer Using Integral Measures: Part Ii. Modelling Observed Conditions

Science.gov (United States)

Hess, G. D.; Garratt, J. R.

The steady-state, horizontally homogeneous, neutral, barotropiccase forms the foundation of our theoretical understanding of the planetary boundary layer (PBL).While simple analytical models and first-order closure models simulate atmospheric observationsof this case well, more sophisticated models, in general, do not. In this paperwe examine how well three higher-order closure models, E - - l, E - l, and LRR - l,which have been especially modified for PBL applications, perform in predicting the behaviour of thecross-isobaric angle 0, the geostrophic drag coefficient Cg, and the integral of the dissipationrate over the boundary layer, as a function of the surface Rossby number Ro. For comparison we alsoexamine the performance of three first-order closure mixing-length models, two proposed byA. K. Blackadar and one by H. H. Lettau, and the performance of the standard model forsecond-order closure and a modification of it designed to reduce the overprediction of turbulence inthe upper part of the boundary layer.

Susceptibility loci for sporadic brain arteriovenous malformation; a replication study and meta-analysis

NARCIS (Netherlands)

Kremer, P.H.; Koeleman, B.P.C.; Rinkel, G.J.; Diekstra, F.P.; Berg, L.H. van den; Veldink, J.H.; Klijn, C.J.M.

2016-01-01

BACKGROUND: Case-control studies have reported multiple genetic loci to be associated with sporadic brain arteriovenous malformations (AVMs) but most of these have not been replicated in independent populations. The aim of this study was to find additional evidence for these reported associations

Gold work function reduction by 2.2 eV with an air-stable molecular donor layer

Energy Technology Data Exchange (ETDEWEB)

Broeker, Benjamin; Blum, Ralf-Peter; Frisch, Johannes; Rabe, Juergen P.; Koch, Norbert [Institut fuer Physik, Humboldt-Universitaet zu Berlin (Germany); Vollmer, Antje [Berliner Elektronenspeicherring-Gesellschaft fuer Synchrotronstrahlung mbH, Berlin (Germany); Hofmann, Oliver T.; Zojer, Egbert [Institut of Solid State Physics, Graz University of Technology (Austria); Rieger, Ralph; Muellen, Klaus [Max Planck Institut fuer Polymerforschung, Mainz (Germany)

2009-07-01

Ultraviolet photoelectron spectroscopy was used to investigate neutral methyl viologen (1,1'-dimethyl-1H,1'H-[4,4']bipyridinylidene, MV0) deposited on Au(111) surfaces. As a result of molecule-to-metal electron transfer, the work function of Au(111) was decreased from 5.5 eV to 3.3 eV. The energy levels of electron transport layers deposited on top of modified Au surfaces were shifted to higher binding energy compared to layers on pristine Au, and the electron injection barrier was reduced by 0.8 eV for tris(8-hydroxyquinoline)aluminum (Alq3) and by 0.7 eV for C60. The air-stable donor MV0 can thus be used to facilitate electron injection into organic semiconductors even from high work function metals.

Prion-Seeding Activity Is widely Distributed in Tissues of Sporadic Creutzfeldt-Jakob Disease Patients.

Science.gov (United States)

Takatsuki, Hanae; Fuse, Takayuki; Nakagaki, Takehiro; Mori, Tsuyoshi; Mihara, Ban; Takao, Masaki; Iwasaki, Yasushi; Yoshida, Mari; Murayama, Shigeo; Atarashi, Ryuichiro; Nishida, Noriyuki; Satoh, Katsuya

2016-10-01

Human prion diseases are neurodegenerative disorders caused by abnormally folded prion proteins in the central nervous system. These proteins can be detected using the quaking-induced conversion assay. Compared with other bioassays, this assay is extremely sensitive and was used in the present study to determine prion distribution in sporadic Creutzfeldt-Jakob disease patients at autopsy. Although infectivity of the sporadic form is thought to be restricted within the central nervous system, results showed that prion-seeding activities reach 10 6 /g from a 50% seeding dose in non-neuronal tissues, suggesting that prion-seeding activity exists in non-neural organs, and we suggested that non-neural tissues of 10 6 /g SD50 did not exist the infectivity. Copyright © 2016 The Authors. Published by Elsevier B.V. All rights reserved.

Differential Expression of Several miRNAs and the Host Genes AATK and DNM2 in Leukocytes of Sporadic ALS Patients.

Science.gov (United States)

Vrabec, Katarina; Boštjančič, Emanuela; Koritnik, Blaž; Leonardis, Lea; Dolenc Grošelj, Leja; Zidar, Janez; Rogelj, Boris; Glavač, Damjan; Ravnik-Glavač, Metka

2018-01-01

Genetic studies have managed to explain many cases of familial amyotrophic lateral sclerosis (ALS) through mutations in several genes. However, the cause of a majority of sporadic cases remains unknown. Recently, epigenetics, especially miRNA studies, show some promising aspects. We aimed to evaluate the differential expression of 10 miRNAs, including miR-9, miR-338, miR-638, miR-663a, miR-124a, miR-143, miR-451a, miR-132, miR-206, and let-7b, for which some connection to ALS was shown previously in ALS culture cells, animal models or patients, and in three miRNA host genes, including C1orf61 (miR-9), AATK (miR-338), and DNM2 (miR-638), in leukocyte samples of 84 patients with sporadic ALS. We observed significant aberrant dysregulation across our patient cohort for miR-124a, miR-206, miR-9, let-7b, and miR-638. Since we did not use neurological controls we cannot rule out that the revealed differences in expression of investigated miRNAs are specific for ALS. Nevertheless, the group of these five miRNAs is worth of additional research in leukocytes of larger cohorts from different populations in order to verify their potential association to ALS disease. We also detected a significant up-regulation of the AAKT gene and down-regulation of the DNM2 gene, and thus, for the first time, we connected these with sporadic ALS cases. These findings open up new research toward miRNAs as diagnostic biomarkers and epigenetic processes involved in ALS. The detected significant deregulation of AAKT and DNM2 in sporadic ALS also represents an interesting finding. The DNM2 gene was previously found to be mutated in Charcot-Marie-Tooth neuropathy-type CMT2M and centronuclear myopathy (CNM). In addition, as recent studies connected AATK and frontotemporal dementia (FTD) and DNM2 and hereditary spastic paraplegia (HSP), these two genes together with our results genetically connect, at least in part, five diseases, including FTD, HSP, Charcot-Marie-Tooth (type CMT2M), CNM, and ALS

A expressão de genes reparadores do DNA nos tumores sincrônicos de câncer colorretal esporádico DNA repair gene expression in synchronic tumors of sporadic colorectal cancer

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Igor Proscurshim

2007-03-01

Full Text Available RACIONAL: Um dos mecanismos genéticos presentes em aproximadamente 80% dos pacientes com síndrome hereditária não-polipóide do câncer colorretal (HNPCC são os defeitos nos genes reparadores de DNA, como o MSH2, MSH6 e MLH1, onde os tumores sincrônicos são relativamente freqüentes. Já no câncer colorretal esporádico as lesões sincrônicas são raras. OBJETIVO: Verificar se o mesmo mecanismo genético presente no HNPCC está presente no câncer colorretal esporádico que apresentam com lesões sincrônicas. MÉTODOS: Foram incluídos no estudo todos os pacientes com câncer colorretal sincrônico não HNPCC. Imunoistoquímica com anticorpos para MSH2,MSH6, e MLH1 foi realizada para cada tumor. RESULTADOS: Todos os pacientes apresentaram expressão normal de MSH2 e MLH1. O único gene com imunoexpressão alterada foi o MSH6. CONCLUSÃO: Possivelmente outro mecanismo genético seja responsável pelo surgimento de dois tumores sincrônicos no câncer colorretal esporádico.BACKGROUND: Mismatch repair genes (such as MSH2, MLH1 and MSH6 mutations are present in over 80% of hereditary non-polyposis colorectal cancer (HNPCC tumors, which frequently exhibit synchronous lesions. Sporadic colorectal cancer is rarely associated with synchronous lesions. AIM: To investigate the role of mismatch repair gene mutation in synchronous sporadic colorectal cancer. METHODS: Patients with sporadic synchronous colorectal adenocarcinomas were included in the study. Immunohistochemistry was performed using MSH2, MLH1 and MSH6 antibodies. RESULTS: All patients had two synchoronous lesions. None of them had altered MSH2 or MLH1 expression. One patient had altered MSH6 expression in both tumors. CONCLUSION: Possibly, other molecular mechanisms are involved in carcinogenesis of sporadic synchronous colorectal cancer.

Patterns of Weakness, Classification of Motor Neuron Disease, and Clinical Diagnosis of Sporadic Amyotrophic Lateral Sclerosis.

Science.gov (United States)

Statland, Jeffrey M; Barohn, Richard J; McVey, April L; Katz, Jonathan S; Dimachkie, Mazen M

2015-11-01

When approaching a patient with suspected motor neuron disease (MND), the pattern of weakness on examination helps distinguish MND from other diseases of peripheral nerves, the neuromuscular junction, or muscle. MND is a clinical diagnosis supported by findings on electrodiagnostic testing. MNDs exist on a spectrum, from a pure lower motor neuron to mixed upper and lower motor neuron to a pure upper motor neuron variant. Amyotrophic lateral sclerosis (ALS) is a progressive mixed upper and lower motor neuron disorder, most commonly sporadic, which is invariably fatal. This article describes a pattern approach to identifying MND and clinical features of sporadic ALS. Copyright © 2015 Elsevier Inc. All rights reserved.

Advances in hepatitis E - II: Epidemiology, clinical manifestations, treatment and prevention.

Science.gov (United States)

Goel, Amit; Aggarwal, Rakesh

2016-09-01

Infection with hepatitis E virus (HEV) is the commonest cause of acute hepatitis worldwide. This infection, with fecal-oral transmission, was previously thought to be limited to humans residing in developing countries with poor sanitation, spreading via contaminated drinking water. In recent years, our understanding of epidemiology and clinical spectrum of this infection have changed markedly. This article reviews the epidemiology, including routes of transmission, and clinical manifestations of HEV infection around the world. In addition, recent findings on transmission-associated HEV infection, extrahepatic manifestations of hepatitis E and chronic infection with HEV, and treatment and prevention of this infection are discussed. Expert commentary: HEV infection has two distinct epidemiologic forms and clinical patterns of disease: (i) acute epidemic or sporadic hepatitis caused by fecal-oral (usually water-borne) transmission of genotype 1 and 2 HEV from a human reservoir in areas with poor hygiene and frequent water contamination, and (ii) infrequent sporadic hepatitis E caused by zoonotic infection, possibly from an animal source through ingestion of undercooked animal meal, of genotype 3 or 4 virus. In disease-endemic areas, pregnant women are at a particular risk of serious disease and high mortality. In less-endemic areas, chronic infection with HEV among immunosuppressed persons is observed. HEV can also be transmitted through Transfusion of blood and blood products. Ribivirin treatment is effective in chronic hepatitis E. Two efficacious vaccines have been tried in humans; one of these has received marketing approval in its country of origin.

Decreased regional cerebral blood flow in the bilateral thalami and medulla oblongata determined by an easy Z-score (eZIS) analysis of (99m)Tc-ECD-SPECT images in a case of MM2-thalamic-type sporadic Creutzfeldt-Jakob disease.

Science.gov (United States)

Hayashi, Yuichi; Iwasaki, Yasushi; Yoshikura, Nobuaki; Asano, Takahiko; Hatano, Taku; Tatsumi, Shinsui; Satoh, Katsuya; Kimura, Akio; Kitamoto, Tetsuyuki; Yoshida, Mari; Inuzuka, Takashi

2015-11-15

We report a case of autopsy-verified MM2-thalamic-type sporadic Creutzfeldt-Jakob disease (sCJD) in a 46-year-old patient with a 16-month history of abnormal behavior, progressive dementia, insomnia, and speech disturbances without family history. Neurological examination revealed progressive dementia, frontal signs, insomnia, speech disturbance, gait disturbance and bilaterally exaggerated tendon reflexes. Both brain MRI and cerebrospinal fluid examinations, including 14-3-3 protein, yielded normal results. An easy Z-score (eZIS) analysis for (99m)Tc-ethyl cysteinate dimer-single photon emission computed tomography ((99m)Tc-ECD-SPECT) revealed decreased regional cerebral blood flow in the bilateral thalami and medulla oblongata. PRNP gene analysis revealed methionine homozygosity at codon 129 without mutation. Neuropathological examinations revealed severe neuronal loss, gliosis, and hypertrophic astrocytosis in the medial thalamus and inferior olivary nucleus. A slight depletion of Purkinje cells was observed. PrP immunostaining showed no obvious PrP deposits in the basal ganglia, thalamus, cerebellum, or brainstem; however, mild synaptic-type PrP deposits with some smaller plaque-like structures were only partially observed in the localized region of the frontal lobe with the spongiform change. Western blot analyses of protease-resistant PrP showed a type 2 pattern. In conclusion, eZIS analysis of (99m)Tc-ECD-SPECT images is useful for detecting both thalamic and medullary lesions. This is the first case of medullary lesions detected in a live patient with MM2-thalamic-type sCJD using SPECT. Copyright © 2015 Elsevier B.V. All rights reserved.

Kras gene mutation and RASSF1A, FHIT and MGMT gene promoter hypermethylation: indicators of tumor staging and metastasis in adenocarcinomatous sporadic colorectal cancer in Indian population.

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Rupal Sinha

Full Text Available Colorectal cancer (CRC development involves underlying modifications at genetic/epigenetic level. This study evaluated the role of Kras gene mutation and RASSF1A, FHIT and MGMT gene promoter hypermethylation together/independently in sporadic CRC in Indian population and correlation with clinicopathological variables of the disease.One hundred and twenty four consecutive surgically resected tissues (62 tumor and equal number of normal adjacent controls of primary sporadic CRC were included and patient details including demographic characteristics, lifestyle/food or drinking habits, clinical and histopathological profiles were recorded. Polymerase chain reaction - Restriction fragment length polymorphism and direct sequencing for Kras gene mutation and Methylation Specific-PCR for RASSF1A, FHIT and MGMT genes was performed.Kras gene mutation at codon 12 & 13 and methylated RASSF1A, FHIT and MGMT gene was observed in 47%, 19%, 47%, 37% and 47% cases, respectively. Alcohol intake and smoking were significantly associated with presence of Kras mutation (codon 12 and MGMT methylation (p-value <0.049. Tumor stage and metastasis correlated with presence of mutant Kras codon 12 (p-values 0.018, 0.044 and methylated RASSF1A (p-values 0.034, 0.044, FHIT (p-values 0.001, 0.047 and MGMT (p-values 0.018, 0.044 genes. Combinatorial effect of gene mutation/methylation was also observed (p-value <0.025. Overall, tumor stage 3, moderately differentiated tumors, presence of lymphatic invasion and absence of metastasis was more frequently observed in tumors with mutated Kras and/or methylated RASSF1A, FHIT and MGMT genes.Synergistic interrelationship between these genes in sporadic CRC may be used as diagnostic/prognostic markers in assessing the overall pathological status of CRC.

Study of the active layer at the Spanish Antarctic station “Gabriel de Castilla”, Deception Island, Antarctica

International Nuclear Information System (INIS)

Pablo, M.A. de; Molina, A.; Recio, C.; Ramos, M.; Goyanes, G.; Ropero, M.A.

2017-01-01

The degradation of permanent frozen ground (permafrost) and the increase in the thickness of the active layer may be caused both by natural processes (such as global climate change) and by anthropic activity, which changes the natural environmental conditions that allow its existence, as has been widely reported to occur in the northern polar and subpolar regions. In the case of Antarctica, some scientific research stations are located in areas with permafrost, such as the Spanish Antarctic station “Gabriel de Castilla” on Deception Island. In the place where the station is located, an important increase in erosion has been observed in recent years, including the excavation of new gullies and the erosion of the coastal cliffs. In order to develop an initial analysis of the possible effects of the station on the permafrost degradation, ground temperature has been monitored since 2012 and the thickness and of the active layer and the temperature, both inside and beneath the station, have also been sporadically measured. Here we show the results and discuss how the station reduces the freezing of the ground during the winter when the station is closed and facilitates the warming of the ground during the living periods of the station in the Antarctic summer. Those initial results and conclusions make necessary to continue the study of the permafrost and the active layer in the station site by systematic monitoring of the ground temperature and the thickness of the active layer. [es

Dispersive O+ conics observed in the plasma-sheet boundary layer with CRRES/LOMICS during a magnetic storm

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M. Wüest

1996-06-01

Full Text Available We present initial results from the Low-energy magnetospheric ion composition sensor (LOMICS on the Combined release and radiation effects satellite (CRRES together with electron, magnetic field, and electric field wave data. LOMICS measures all important magnetospheric ion species (H+, He++, He+, O++, O+ simultaneously in the energy range 60 eV to 45 keV, as well as their pitch-angle distributions, within the time resolution afforded by the spacecraft spin period of 30 s. During the geomagnetic storm of 9 July 1991, over a period of 42 min (0734 UT to 0816 UT the LOMICS ion mass spectrometer observed an apparent O+ conic flowing away from the southern hemisphere with a bulk velocity that decreased exponentially with time from 300 km/s to 50 km/s, while its temperature also decreased exponentially from 700 to 5 eV. At the onset of the O+ conic, intense low-frequency electromagnetic wave activity and strong pitch-angle scattering were also observed. At the time of the observations the CRRES spacecraft was inbound at L~7.5 near dusk, magnetic local time (MLT, and at a magnetic latitude of –23°. Our analysis using several CRRES instruments suggests that the spacecraft was skimming along the plasma sheet boundary layer (PSBL when the upward-flowing ion conic arrived. The conic appears to have evolved in time, both slowing and cooling, due to wave-particle interactions. We are unable to conclude whether the conic was causally associated with spatial structures of the PSBL or the central plasma sheet.

Layering and Ordering in Electrochemical Double Layers

Energy Technology Data Exchange (ETDEWEB)

Liu, Yihua [Materials Science Division, Argonne National Laboratory, Argonne, Illinois 60439, United States; Kawaguchi, Tomoya [Materials Science Division, Argonne National Laboratory, Argonne, Illinois 60439, United States; Pierce, Michael S. [Rochester Institute of Technology, School of Physics and Astronomy, Rochester, New York 14623, United States; Komanicky, Vladimir [Faculty of Science, Safarik University, 041 54 Kosice, Slovakia; You, Hoydoo [Materials Science Division, Argonne National Laboratory, Argonne, Illinois 60439, United States

2018-02-26

Electrochemical double layers (EDL) form at electrified interfaces. While Gouy-Chapman model describes moderately charged EDL, formation of Stern layers was predicted for highly charged EDL. Our results provide structural evidence for a Stern layer of cations, at potentials close to hydrogen evolution in alkali fluoride and chloride electrolytes. Layering was observed by x-ray crystal truncation rods and atomic-scale recoil responses of Pt(111) surface layers. Ordering in the layer is confirmed by glancing-incidence in-plane diffraction measurements.

Pathological Effects of Aflatoxin and Their Amelioration by Vitamin E in White Leghorn Layers

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Wajid A Khan, M Zargham Khan*, Ahrar Khan and Iftikhar Hussain1

2010-07-01

Full Text Available White Leghorn layer breeder hens, 30 weeks of age, were divided into 12 groups (A-L. Group A was kept on basal feed and served as control, while group B was offered feed supplemented with vitamin E (100 mg/Kg. Groups C-G were offered feed containing 100, 500, 2,500, 5,000 and 10,000 µg/Kg aflatoxin B1 (AFB1, respectively, whereas groups H-L were offered same dietary levels of AFB1 along with vitamin E (100 mg/Kg. The experimental feeds were offered for three weeks and afterward all the groups were switched over to basal feed for next two weeks. Body weight, absolute and relative weights of liver and kidneys of AF fed birds were significantly higher than control group. Pathological lesions in aflatoxin (AF fed birds included enlarged, pale and friable liver, swollen kidneys and hemorrhages on different organs. Histopathological lesions in liver included fatty change, congestion and hemorrhages, while in kidneys tubular necrosis, cellular infiltration, congestion and hemorrhages were found in groups fed AFB1 at 500 μg/Kg and higher doses. In AF fed hens, no significant ameliorative effects of vitamin E could be observed upon AF induced decrease in feed intake, gross pathology and histopathological alterations and organ weight except body weights. It was concluded that the vitamin E ameliorated the AFB1 induced toxic effects in some of parameters studied.

A Tangled Web - Tau and Sporadic Parkinson's Disease

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Selina Wray

2010-12-01

Full Text Available Parkinson's disease (PD represents a major challenge for health care systems around the world: it is the most common degenerative movement disorder of old age, affecting over 100,000 people in the UK alone. A great deal of progress has been made in understanding the molecular basis of PD by taking advantage of advances in genetics, initially by the identification of genes responsible for rare mendellian forms of PD (outlined in table one, and more recently by applying genome wide association studies (GWAS to the sporadic form of the disease. Several such GWAS have now been carried out, with a meta-analysis currently under way. Using over 6000 cases and 10000 controls, two of these studies have identified variation at a number of loci as being associated with an increased risk of disease. Three genes stand out as candidates from these studies – the SNCA gene, coding for α -synuclein, the LRRK2 gene, coding for leucine rich repeat kinase 2, and MAPT, coding for the microtubule associated protein tau. Point mutations in α -synuclein, along with gene multiplication events, result in autosomal dominant PD, often with a significant dementia component. In addition to this, α -synuclein is the principle component of the main pathological hallmark of PD, the Lewy body. Mutations in LRRK2 are the most common genetic cause of PD, and so again were a likely candidate for a susceptibility locus for the sporadic form of disease. More surprising, perhaps, was the identification of tau as a susceptibility factor for Parkinson's. In this review we will outline the role of tau in neurodegeneration and in different forms of parkinsonism, and speculate as to what the functional basis of this association might be.

Influence of Ionospheric Weather on GNSS Radio Occultation Signals

Science.gov (United States)

Yue, X.; Schreiner, W. S.; Pedatella, N. M.; Kuo, Y. H.

2016-12-01

Transient loss of lock (LOL) is one of the key space weather effects on the Global Navigation Satellite System (GNSS). Based on the Constellation Observing System for Meteorology, Ionosphere, and Climate (COSMIC) Global Positioning System (GPS) radio occultation (RO) observations during 2007-2011, we have analyzed the signal cycle slip (CS) occurrence comprehensively and its correlation to the ionospheric weather phenomena such as sporadic E (Es), equatorial F region irregularity (EFI), and the ionospheric equatorial ionization anomaly (EIA). The high vertical resolution of RO observations enables us to distinguish the CS resulting from different ionospheric layers clearly on a global scale. In the E layer, the CS is dominated by the Es occurrence, while in the F layer, the CS is mainly related to the EIA and EFI at low and equatorial latitudes. In the polar region, the CS is primarily related to polar cap electron density gradients. The overall average CS (> 6 cycles) occurrence is 23% per occultation, with the E (50-150 km) and F (150-600 km) layers contributing 8.3% and 14.7%, respectively. Awareness of the effect of the ionospheric weather on the CS of the low-Earth-orbit (LEO)-based GNSS signal could be beneficial to a variety of applications, including the LEO-based GNSS data processing and the corresponding hardware/firmware design.

Physical Layer Multi-Core Prototyping A Dataflow-Based Approach for LTE eNodeB

CERN Document Server

Pelcat, Maxime; Piat, Jonathan; Nezan, Jean-François

2013-01-01

Base stations developed according to the 3GPP Long Term Evolution (LTE) standard require unprecedented processing power. 3GPP LTE enables data rates beyond hundreds of Mbits/s by using advanced technologies, necessitating a highly complex LTE physical layer. The operating power of base stations is a significant cost for operators, and is currently optimized using state-of-the-art hardware solutions, such as heterogeneous distributed systems. The traditional system design method of porting algorithms to heterogeneous distributed systems based on test-and-refine methods is a manual, thus time-expensive, task.   Physical Layer Multi-Core Prototyping: A Dataflow-Based Approach for LTE eNodeB provides a clear introduction to the 3GPP LTE physical layer and to dataflow-based prototyping and programming. The difficulties in the process of 3GPP LTE physical layer porting are outlined, with particular focus on automatic partitioning and scheduling, load balancing and computation latency reduction, specifically in sys...

Observation of the rare {eta}{yields}e{sup +}e{sup -}e{sup +}e{sup -} decay with the KLOE experiment

Energy Technology Data Exchange (ETDEWEB)

Ambrosino, F. [Dipartimento di Scienze Fisiche dell' Universita ' Federico II' , Napoli (Italy); INFN Sezione di Napoli, Napoli (Italy); Antonelli, A.; Antonelli, M. [Laboratori Nazionali di Frascati dell' INFN, Frascati (Italy); Archilli, F. [Dipartimento di Fisica dell' Universita ' Tor Vergata, Roma (Italy); INFN Sezione di Roma Tor Vergata, Roma (Italy); Balwierz, I. [Institute of Physics, Jagiellonian University, Krakow (Poland); Bencivenni, G. [Laboratori Nazionali di Frascati dell' INFN, Frascati (Italy); Bini, C. [Dipartimento di Fisica dell' Universita ' Sapienza' , Roma (Italy); INFN Sezione di Roma, Roma (Italy); Bloise, C. [Laboratori Nazionali di Frascati dell' INFN, Frascati (Italy); Bocchetta, S. [Dipartimento di Fisica dell' Universita ' Roma Tre' , Roma (Italy); INFN Sezione di Roma Tre, Roma (Italy); Bossi, F. [Laboratori Nazionali di Frascati dell' INFN, Frascati (Italy); Branchini, P. [INFN Sezione di Roma Tre, Roma (Italy); Capon, G.; Capussela, T. [Laboratori Nazionali di Frascati dell' INFN, Frascati (Italy); Ceradini, F. [Dipartimento di Fisica dell' Universita ' Roma Tre' , Roma (Italy); INFN Sezione di Roma Tre, Roma (Italy); Ciambrone, P.; Czerwinski, E.; De Lucia, E. [Laboratori Nazionali di Frascati dell' INFN, Frascati (Italy); De Santis, A. [Dipartimento di Fisica dell' Universita ' Sapienza' , Roma (Italy); INFN Sezione di Roma, Roma (Italy); De Simone, P. [Laboratori Nazionali di Frascati dell' INFN, Frascati (Italy); De Zorzi, G. [Dipartimento di Fisica dell' Universita ' Sapienza' , Roma (Italy); INFN Sezione di Roma, Roma (Italy)

2011-08-26

We report the first observation of the rare {eta}{yields}e{sup +}e{sup -}e{sup +}e{sup -}({gamma}) decay based on 1.7 fb{sup -1} collected by the KLOE experiment at the DA{Phi}NE {phi}-factory. The selection of the e{sup +}e{sup -}e{sup +}e{sup -} final state is fully inclusive of radiation. We have identified 362{+-}29 events resulting in a branching ratio of (2.4{+-}0.2{sub stat}+bckg{+-}0.1{sub syst})x10{sup -5}.

Agraphia of Kanji (Chinese characters): an early symptom of sporadic Creutzfeldt-Jakob disease in a Japanese patient: a case report.

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Nakamura, Keiko; Sakai, Kenji; Samuraki, Miharu; Nozaki, Ichiro; Notoya, Masako; Yamada, Masahito

2014-08-06

Slowly progressive cognitive decline is the most frequent initial manifestation in MM2-cortical-type sporadic Creutzfeldt-Jakob disease. Agraphia has never been noted in patients with this type of sporadic Creutzfeldt-Jakob disease, however, we report the case of a Japanese patient with sporadic Creutzfeldt-Jakob disease in whom agraphia of Kanji was an initial cardinal symptom. A 59-year-old right-handed Japanese woman complained of agraphia of Kanji (Chinese characters) as an initial symptom. A neurological examination revealed mild word-finding difficulty, constructive disturbance, hyperreflexia in her jaw and lower limbs, and bilateral extensor plantar reflexes. An examination of her cerebrospinal fluid revealed increased levels of 14-3-3 and total tau proteins, and abnormal conformation of the proteinase K-resistant prion protein. Diffusion-weighted magnetic resonance imaging showed diffuse hyperintensity in bilateral cerebral cortices. Single-photon emission computed tomography scans revealed hypoperfusion in the left temporal lobe, bilateral parietal and occipital lobes. An analysis of the prion protein gene demonstrated no mutation with homozygous for methionine at the codon 129. We diagnosed our patient with sporadic Creutzfeldt-Jakob disease. Although a histological examination was not performed, it was assumed that our patient could be the MM2-cortical type according to the clinical findings and the elevated levels of 14-3-3 protein in her cerebrospinal fluid. The left posterior inferior temporal area, which was affected in our patient as a hypoperfusion area, is associated with selecting and recalling Kanji characters. Focal signs as an early symptom and hypoperfusion areas in sporadic Creutzfeldt-Jakob disease are critical to recognize initial brain lesions damaged by the proteinase K-resistant prion protein accumulation.

RACORO Extended-Term Aircraft Observations of Boundary-Layer Clouds

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Vogelmann, Andrew M.; McFarquhar, Greg M.; Ogren, John A.; Turner, David D.; Comstock, Jennifer M.; Feingold, Graham; Long, Charles N.; Jonsson, Haflidi H.; Bucholtz, Anthony; Collins, Don R.;

Evaluation of surface layer flux parameterizations using in-situ observations

Science.gov (United States)

Katz, Jeremy; Zhu, Ping

2017-09-01

Appropriate calculation of surface turbulent fluxes between the atmosphere and the underlying ocean/land surface is one of the major challenges in geosciences. In practice, the surface turbulent fluxes are estimated from the mean surface meteorological variables based on the bulk transfer model combined with the Monnin-Obukhov Similarity (MOS) theory. Few studies have been done to examine the extent to which such a flux parameterization can be applied to different weather and surface conditions. A novel validation method is developed in this study to evaluate the surface flux parameterization using in-situ observations collected at a station off the coast of Gulf of Mexico. The main findings are: (a) the theoretical prediction that uses MOS theory does not match well with those directly computed from the observations. (b) The largest spread in exchange coefficients is shown in strong stable conditions with calm winds. (c) Large turbulent eddies, which depend strongly on the mean flow pattern and surface conditions, tend to break the constant flux assumption in the surface layer.

The sensitivity of auxiliary examinations in different stages of sporadic Creutzfeldt-Jakob disease

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Jiao-jiao JIANG

2017-06-01

Full Text Available Objective To analyze the sensitivity of auxiliary examinations in different periods of sporadic Creutzfeldt-Jakob disease (sCJD. Methods The clinical data of 53 sCJD patients were retrospectively analyzed including the different stages of skull diffusion-weighted magnetic resonance imaging (DWI, 24-hour ambulatory electroencephalogram (EEG, 18F-FDG PET/CT (PET-CT and cerebrospinal fluid 14-3-3 protein. When calculating the sensitivity of an auxiliary examination, the diagnostic criteria were defined by combining the specific clinical manifestations with two or more positive results of other auxiliary examinations. Results There were 24, 53 and 22 sCJD patients, respectively, met the criterion of early (E, middle (M and later (L stage of disease (some patients fit 2 or 3 stages. The sensitivity of DWI (E: 58.3%, M: 85.4%, L: 94.7%, EEG (E: 45.8%, M: 62.7%, L: 77.8%, 14-3-3 protein in cerebrospinal fluid (E: 11.1%, M: 52.9% and PET-CT (E: 80%, M: 100% increased gradually with disease progression. The sensitivity of PET-CT was higher than the other auxiliary examinations for E and M stages; no PET-CT was conducted in L stage. High signal regions mainly distributed in the cortex in E and M stages, but in L stage, no significant difference was found on the distribution of high signal regions between cortex and basal ganglia. Conclusions The sensitivities of the auxiliary examinations were different for sCJD patients in different stages. Reexaminations in different periods may improve the sensitivity for sCJD diagnosis. The sensitivity of PET-CT was high, and the combination of PET-CT and other auxiliary examinations may play a key role in the diagnosis of sCJD. DOI: 10.11855/j.issn.0577-7402.2017.05.15

Sensitivity Analysis in Observational Research: Introducing the E-Value.

Science.gov (United States)

VanderWeele, Tyler J; Ding, Peng

2017-08-15

Sensitivity analysis is useful in assessing how robust an association is to potential unmeasured or uncontrolled confounding. This article introduces a new measure called the "E-value," which is related to the evidence for causality in observational studies that are potentially subject to confounding. The E-value is defined as the minimum strength of association, on the risk ratio scale, that an unmeasured confounder would need to have with both the treatment and the outcome to fully explain away a specific treatment-outcome association, conditional on the measured covariates. A large E-value implies that considerable unmeasured confounding would be needed to explain away an effect estimate. A small E-value implies little unmeasured confounding would be needed to explain away an effect estimate. The authors propose that in all observational studies intended to produce evidence for causality, the E-value be reported or some other sensitivity analysis be used. They suggest calculating the E-value for both the observed association estimate (after adjustments for measured confounders) and the limit of the confidence interval closest to the null. If this were to become standard practice, the ability of the scientific community to assess evidence from observational studies would improve considerably, and ultimately, science would be strengthened.

Benthic boundary layer. IOS observational and modelling programme

International Nuclear Information System (INIS)

Saunders, P.M.; Richards, K.J.

1985-01-01

Near bottom currents, measured at three sites in the N.E. Atlantic, reveal the eddying characteristics of the flow. Eddies develop, migrate and decay in ways best revealed by numerical modelling simulations. Eddies control the thickness of the bottom mixed layer by accumulating and thickening or spreading and thinning the bottom waters. At the boundaries of eddies benthic fronts form providing a path for upward displacement of the bottom water. An experiment designed to estimate vertical diffusivity is performed. The flux of heat into the bottom of the Iberian basin through Discovery Gap is deduced from year long current measurements. The flux is supposed balanced by geothermal heating through the sea floor and diapycnal diffusion in the water. A diffusivity of 1.5 to 4 cm 2 s -1 is derived for the bottom few hundred meters of the deep ocean. Experiments to estimate horizontal diffusivity are described. If a tracer is discharged from the sea bed the volume of sea water in which it is found increases with time and after 20 years will fill an ocean basin of side 1000 km to a depth of only 1 to 2 km. (author)

The Helicopter Observation Platform for Marine and Continental Boundary Layer Studies

Science.gov (United States)

Avissar, R.; Broad, K.; Walko, R. L.; Drennan, W. M.; Williams, N. J.

2016-02-01

The University of Miami has acquired a commercial helicopter (Airbus H125) that was transformed into a one-of-a-kind Helicopter Observation Platform (HOP) that fills critical gaps in physical, chemical and biological observations of the environment. This new research facility is designed to carry sensors and instrument inlets in the undisturbed air in front of the helicopter nose at low airspeed and at various altitudes, from a few feet above the Earth's surface (where much of the climate and weather "action" takes place, and where we live) and up through the atmospheric boundary layer and the mid troposphere. The HOP, with its hovering capability, is also ideal for conducting various types of remote-sensing observations. It provides a unique and essential component of airborne measurement whose purpose, among others, is to quantify the exchanges of gases and energy at the Earth surface, as well as aerosol properties that affect the environment, the climate system, and human health. For its first scientific mission, an eddy-correlation system is being mounted in front of its nose to conduct high-frequency measurements of turbulence variables relevant to atmospheric boundary layer studies.Fully fueled and with both pilot and co-pilot on board, the HOP can carry a scientific payload of up to about 1,000 lbs internally (about 3,000 lbs externally) and fly for nearly 4 hours without refueling at an airspeed of 65 knots ( 30 m/s) that is ideal for in-situ observations. Its fast cruising speed is about 140 knots andits range, at that speed, is about 350 nautical miles. This specific helicopter was chosen because of its flat floor design, which is particularly convenient for installing scientific payload and also because of its high-altitude capability (it is the only commercial helicopter that ever landed at the top of Mt Everest).The HOP is available to the entire scientific community for any project that is feasible from a flight safety point of view and that fulfills

Protein-losing Enteropathy Caused by Intestinal or Colonic Lymphangiectasia Complicated by Sporadic Lymphangioleiomyomatosis: A Report of Two Cases.

Science.gov (United States)

Nishino, Koichi; Yoshimi, Kaku; Shibuya, Tomoyoshi; Hayashi, Takuo; Mitani, Keiko; Kobayashi, Etsuko; Ichikawa, Masako; Asao, Tetsuhiko; Suzuki, Yohei; Sato, Tadashi; Shiota, Satomi; Kodama, Yuzo; Takahashi, Kazuhisa; Seyama, Kuniaki

2017-01-01

This report describes two patients with sporadic lymphangioleiomyomatosis complicated by protein-losing enteropathy (PLE). Imaging studies indicated retroperitoneal lymphangioleiomyomas and abnormalities of the adjacent digestive tract. Endoscopic mucosal biopsy revealed colonic lymphangiectasia in one patient; whereas the site in the other patient was intestinal. Treatment with sirolimus led to the complete resolution of PLE within several months; additionally, marked shrinkage was observed in the lymphangioleiomyomas of both cases. These findings suggest that colonic or intestinal lymphatic congestion due to neighboring lymphangioleiomyomas was the mechanism for the development of PLE. At the time of writing this report, the beneficial effect of sirolimus has lasted for more than 3 years.

An exploratory case control study of risk factors for hepatitis E in rural Bangladesh.

Directory of Open Access Journals (Sweden)

Alain B Labrique

Full Text Available Hepatitis E virus (HEV is the major cause of epidemic and sporadic hepatitis globally. Outbreaks are associated with fecal contamination of drinking water, yet the environmental reservoir of HEV between epidemics remains unclear. In contrast to neighboring countries, where epidemics and sporadic disease co-occur, HEV-endemic communities in rural Bangladesh seldom report outbreaks; sporadic hepatitis E is reported from urban and rural areas of the country. Besides typical enteric risk factors, other routes for HEV infection and disease are unclear. We conducted monthly household surveillance of a southern Bangladeshi community of 23,500 people to find incident cases of acute hepatitis E over a 22 month period. An algorithm was used to capture 279 candidate cases, of which 46 were confirmed acute HEV infections. An exploratory case-control study was conducted to identify putative risk factors for disease. Nearly 70% of cases were over 15 years old. Female gender seemed protective (OR:0.34 against hepatitis E in this conservative setting, as was the use of sanitary latrines (OR:0.28. Socioeconomic status or animal exposures were not significant predictors of disease, although outdoor employment and recent urban travel were. Unexpectedly, recent contact with a "jaundiced" patient and a history of injection exposure in the 3 months prior to disease (OR:15.50 were significant. Susceptible individuals from "endemic" communities share similar enteric exposure risks to those commonly associated with tourists from non-endemic countries. This study also raises the novel possibility of parenteral and person-to-person transmission of HEV in non-epidemic, sporadic disease settings.

Aberrant gene promoter methylation associated with sporadic multiple colorectal cancer.

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Victoria Gonzalo

Full Text Available BACKGROUND: Colorectal cancer (CRC multiplicity has been mainly related to polyposis and non-polyposis hereditary syndromes. In sporadic CRC, aberrant gene promoter methylation has been shown to play a key role in carcinogenesis, although little is known about its involvement in multiplicity. To assess the effect of methylation in tumor multiplicity in sporadic CRC, hypermethylation of key tumor suppressor genes was evaluated in patients with both multiple and solitary tumors, as a proof-of-concept of an underlying epigenetic defect. METHODOLOGY/PRINCIPAL FINDINGS: We examined a total of 47 synchronous/metachronous primary CRC from 41 patients, and 41 gender, age (5-year intervals and tumor location-paired patients with solitary tumors. Exclusion criteria were polyposis syndromes, Lynch syndrome and inflammatory bowel disease. DNA methylation at the promoter region of the MGMT, CDKN2A, SFRP1, TMEFF2, HS3ST2 (3OST2, RASSF1A and GATA4 genes was evaluated by quantitative methylation specific PCR in both tumor and corresponding normal appearing colorectal mucosa samples. Overall, patients with multiple lesions exhibited a higher degree of methylation in tumor samples than those with solitary tumors regarding all evaluated genes. After adjusting for age and gender, binomial logistic regression analysis identified methylation of MGMT2 (OR, 1.48; 95% CI, 1.10 to 1.97; p = 0.008 and RASSF1A (OR, 2.04; 95% CI, 1.01 to 4.13; p = 0.047 as variables independently associated with tumor multiplicity, being the risk related to methylation of any of these two genes 4.57 (95% CI, 1.53 to 13.61; p = 0.006. Moreover, in six patients in whom both tumors were available, we found a correlation in the methylation levels of MGMT2 (r = 0.64, p = 0.17, SFRP1 (r = 0.83, 0.06, HPP1 (r = 0.64, p = 0.17, 3OST2 (r = 0.83, p = 0.06 and GATA4 (r = 0.6, p = 0.24. Methylation in normal appearing colorectal mucosa from patients with multiple and solitary CRC showed no relevant

Effects of Strong Local Sporadic E on ELF Propagation.

Science.gov (United States)

1978-08-15

Huygens diffraction model (e.g., Marcuse , 1972). The model is similar to that used by Crombie. Unlike Crombie’s work however , the Fresnel approximation...40. Marcuse , D., “Light transmission optics ,” Van Nostrand Reinhold Co., New York , 1972. Papper t , R. A. & Moler , W. F., “A theoretica’ study of...ATTN Donald Dubbert O1 CY ATTN Herbert Rend University of IllinoisDepartment of Electrical Engineering Develco Urbana , IL 61803 530 Logue Avenue O2CY

Differential Expression of Several miRNAs and the Host Genes AATK and DNM2 in Leukocytes of Sporadic ALS Patients

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Katarina Vrabec

2018-04-01

Full Text Available Genetic studies have managed to explain many cases of familial amyotrophic lateral sclerosis (ALS through mutations in several genes. However, the cause of a majority of sporadic cases remains unknown. Recently, epigenetics, especially miRNA studies, show some promising aspects. We aimed to evaluate the differential expression of 10 miRNAs, including miR-9, miR-338, miR-638, miR-663a, miR-124a, miR-143, miR-451a, miR-132, miR-206, and let-7b, for which some connection to ALS was shown previously in ALS culture cells, animal models or patients, and in three miRNA host genes, including C1orf61 (miR-9, AATK (miR-338, and DNM2 (miR-638, in leukocyte samples of 84 patients with sporadic ALS. We observed significant aberrant dysregulation across our patient cohort for miR-124a, miR-206, miR-9, let-7b, and miR-638. Since we did not use neurological controls we cannot rule out that the revealed differences in expression of investigated miRNAs are specific for ALS. Nevertheless, the group of these five miRNAs is worth of additional research in leukocytes of larger cohorts from different populations in order to verify their potential association to ALS disease. We also detected a significant up-regulation of the AAKT gene and down-regulation of the DNM2 gene, and thus, for the first time, we connected these with sporadic ALS cases. These findings open up new research toward miRNAs as diagnostic biomarkers and epigenetic processes involved in ALS. The detected significant deregulation of AAKT and DNM2 in sporadic ALS also represents an interesting finding. The DNM2 gene was previously found to be mutated in Charcot-Marie-Tooth neuropathy-type CMT2M and centronuclear myopathy (CNM. In addition, as recent studies connected AATK and frontotemporal dementia (FTD and DNM2 and hereditary spastic paraplegia (HSP, these two genes together with our results genetically connect, at least in part, five diseases, including FTD, HSP, Charcot-Marie-Tooth (type CMT2M, CNM

CAMS newly detected meteor showers and the sporadic background

Science.gov (United States)

Jenniskens, P.; Nénon, Q.; Gural, P. S.; Albers, J.; Haberman, B.; Johnson, B.; Morales, R.; Grigsby, B. J.; Samuels, D.; Johannink, C.

2016-03-01

The Cameras for Allsky Meteor Surveillance (CAMS) video-based meteoroid orbit survey adds 60 newly identified showers to the IAU Working List of Meteor Showers (numbers 427, 445-446, 506-507, and part of 643-750). 28 of these are also detected in the independent SonotaCo survey. In total, 230 meteor showers and shower components are identified in CAMS data, 177 of which are detected in at least two independent surveys. From the power-law size frequency distribution of detected showers, we extrapolate that 36% of all CAMS-observed meteors originated from ∼700 showers above the N = 1 per 110,000 shower limit. 71% of mass falling to Earth from streams arrives on Jupiter-family type orbits. The transient Geminids account for another 15%. All meteoroids not assigned to streams form a sporadic background with highest detected numbers from the apex source, but with 98% of mass falling in from the antihelion source. Even at large ∼7-mm sizes, a Poynting-Robertson drag evolved population is detected, which implies that the Grün et al. collisional lifetimes at these sizes are underestimated by about a factor of 10. While these large grains survive collisions, many fade on a 104-y timescale, possibly because they disintegrate into smaller particles by processes other than collisions, leaving a more resilient population to evolve.

Lidar observations of stratospheric aerosol layer after the Mt. Pinatubo volcanic eruption

International Nuclear Information System (INIS)

Nagai, Tomohiro; Uchino, Osamu; Fujimoto, Toshifumi.

1992-01-01

The volcano Mt. Pinatubo located on the Luzon Island, Philippines, had explosively erupted on June 15, 1991. The volcanic eruptions such as volcanic ash, SO2 and H2O reached into the stratosphere over 30 km altitude by the NOAA-11 satellite observation and this is considered one of the biggest volcanic eruptions in this century. A grandiose volcanic eruption influences the atmosphere seriously and causes many climatic effects globally. There had been many impacts on radiation, atmospheric temperature and stratospheric ozone after some past volcanic eruptions. The main cause of volcanic influence depends on stratospheric aerosol, that stay long enough to change climate and other meteorological conditions. Therefore it is very important to watch stratospheric aerosol layers carefully and continuously. Standing on this respect, we do not only continue stratospheric aerosol observation at Tsukuba but also have urgently developed another lidar observational point at Naha in Okinawa Island. This observational station could be thought valuable since there is no lidar observational station in this latitudinal zone and it is much nearer to Mt. Pinatubo. Especially, there is advantage to link up these two stations on studying the transportation mechanism in the stratosphere. In this paper, we present the results of lidar observations at Tsukuba and Naha by lidar systems with Nd:YAG laser

Lidar Observations of Stratospheric Aerosol Layer After the Mt. Pinatubo Volcanic Eruption

Science.gov (United States)

Nagai, Tomohiro; Uchino, Osamu; Fujimoto, Toshifumi

1992-01-01

The volcano Mt. Pinatubo located on the Luzon Island, Philippines, had explosively erupted on June 15, 1991. The volcanic eruptions such as volcanic ash, SO2 and H2O reached into the stratosphere over 30 km altitude by the NOAA-11 satellite observation and this is considered one of the biggest volcanic eruptions in this century. A grandiose volcanic eruption influences the atmosphere seriously and causes many climatic effects globally. There had been many impacts on radiation, atmospheric temperature and stratospheric ozone after some past volcanic eruptions. The main cause of volcanic influence depends on stratospheric aerosol, that stay long enough to change climate and other meteorological conditions. Therefore it is very important to watch stratospheric aerosol layers carefully and continuously. Standing on this respect, we do not only continue stratospheric aerosol observation at Tsukuba but also have urgently developed another lidar observational point at Naha in Okinawa Island. This observational station could be thought valuable since there is no lidar observational station in this latitudinal zone and it is much nearer to Mt. Pinatubo. Especially, there is advantage to link up these two stations on studying the transportation mechanism in the stratosphere. In this paper, we present the results of lidar observations at Tsukuba and Naha by lidar systems with Nd:YAG laser.

Exome sequencing in 53 sporadic cases of schizophrenia identifies 18 putative candidate genes.

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Michel Guipponi

Full Text Available Schizophrenia (SCZ is a severe, debilitating mental illness which has a significant genetic component. The identification of genetic factors related to SCZ has been challenging and these factors remain largely unknown. To evaluate the contribution of de novo variants (DNVs to SCZ, we sequenced the exomes of 53 individuals with sporadic SCZ and of their non-affected parents. We identified 49 DNVs, 18 of which were predicted to alter gene function, including 13 damaging missense mutations, 2 conserved splice site mutations, 2 nonsense mutations, and 1 frameshift deletion. The average number of exonic DNV per proband was 0.88, which corresponds to an exonic point mutation rate of 1.7×10(-8 per nucleotide per generation. The non-synonymous-to-synonymous mutation ratio of 2.06 did not differ from neutral expectations. Overall, this study provides a list of 18 putative candidate genes for sporadic SCZ, and when combined with the results of similar reports, identifies a second proband carrying a non-synonymous DNV in the RGS12 gene.

Magnesium protects cognitive functions and synaptic plasticity in streptozotocin-induced sporadic Alzheimer's model.

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Zhi-Peng Xu

Full Text Available Alzheimer's disease (AD is characterized by profound synapse loss and impairments of learning and memory. Magnesium affects many biochemical mechanisms that are vital for neuronal properties and synaptic plasticity. Recent studies have demonstrated that the serum and brain magnesium levels are decreased in AD patients; however, the exact role of magnesium in AD pathogenesis remains unclear. Here, we found that the intraperitoneal administration of magnesium sulfate increased the brain magnesium levels and protected learning and memory capacities in streptozotocin-induced sporadic AD model rats. We also found that magnesium sulfate reversed impairments in long-term potentiation (LTP, dendritic abnormalities, and the impaired recruitment of synaptic proteins. Magnesium sulfate treatment also decreased tau hyperphosphorylation by increasing the inhibitory phosphorylation of GSK-3β at serine 9, thereby increasing the activity of Akt at Ser473 and PI3K at Tyr458/199, and improving insulin sensitivity. We conclude that magnesium treatment protects cognitive function and synaptic plasticity by inhibiting GSK-3β in sporadic AD model rats, which suggests a potential role for magnesium in AD therapy.

Early detection of sporadic pancreatic cancer: strategic map for innovation--a white paper.

Science.gov (United States)

Kenner, Barbara J; Chari, Suresh T; Cleeter, Deborah F; Go, Vay Liang W

2015-07-01

Innovation leading to significant advances in research and subsequent translation to clinical practice is urgently necessary in early detection of sporadic pancreatic cancer. Addressing this need, the Early Detection of Sporadic Pancreatic Cancer Summit Conference was conducted by Kenner Family Research Fund in conjunction with the 2014 American Pancreatic Association and Japan Pancreas Society Meeting. International interdisciplinary scientific representatives engaged in strategic facilitated conversations based on distinct areas of inquiry: Case for Early Detection: Definitions, Detection, Survival, and Challenges; Biomarkers for Early Detection; Imaging; and Collaborative Studies. Ideas generated from the summit have led to the development of a Strategic Map for Innovation built upon 3 components: formation of an international collaborative effort, design of an actionable strategic plan, and implementation of operational standards, research priorities, and first-phase initiatives. Through invested and committed efforts of leading researchers and institutions, philanthropic partners, government agencies, and supportive business entities, this endeavor will change the future of the field and consequently the survival rate of those diagnosed with pancreatic cancer.

Inhomogeneous structure of ionospheric emission layers according to photographic observations at the Salyut-7 orbital station

International Nuclear Information System (INIS)

Platov, Yu.V.; Vanyarkha, N.Ya.; Vanyarkha, E.S.

1989-01-01

Shape of brightness vertical profile for the ionospheric emission layers, observed near the night horizon from space vehicles, depends essentially on structure of luminescence regions. Brightness profiles, obtained from photometry of the first emission layer photons at ∼ 100 km heights, are compared with calculated ones for model distribution of the excited atom concentration to determine typical dimensions of heterogeneities. Luminescence region in the used model was represented by symmetric spot with concentration exponentially decreasing in horizontal direction and with vertical distribution of concentration characterized by rather abrupt maximum at ∼ 10 km height

TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis.

Science.gov (United States)

Kabashi, Edor; Valdmanis, Paul N; Dion, Patrick; Spiegelman, Dan; McConkey, Brendan J; Vande Velde, Christine; Bouchard, Jean-Pierre; Lacomblez, Lucette; Pochigaeva, Ksenia; Salachas, Francois; Pradat, Pierre-Francois; Camu, William; Meininger, Vincent; Dupre, Nicolas; Rouleau, Guy A

2008-05-01

Recently, TDP-43 was identified as a key component of ubiquitinated aggregates in amyotrophic lateral sclerosis (ALS), an adult-onset neurological disorder that leads to the degeneration of motor neurons. Here we report eight missense mutations in nine individuals--six from individuals with sporadic ALS (SALS) and three from those with familial ALS (FALS)--and a concurring increase of a smaller TDP-43 product. These findings further corroborate that TDP-43 is involved in ALS pathogenesis.

A Vortical Dawn Flank Boundary Layer for Near-Radial IMF: Wind Observations on 24 October 2001

Science.gov (United States)

Farrugia, C. J.; Gratton, F. T.; Gnavi, G.; Torbert, R. B.; Wilson, Lynn B., III

2014-01-01

We present an example of a boundary layer tailward of the dawn terminator which is entirely populated by rolled-up flow vortices. Observations were made by Wind on 24 October 2001 as the spacecraft moved across the region at the X plane approximately equal to -13 Earth radii. Interplanetary conditions were steady with a near-radial interplanetary magnetic field (IMF). Approximately 15 vortices were observed over the 1.5 hours duration of Wind's crossing, each lasting approximately 5 min. The rolling up is inferred from the presence of a hot tenuous plasma being accelerated to speeds higher than in the adjoining magnetosheath, a circumstance which has been shown to be a reliable signature of this in single-spacecraft observations. A blob of cold dense plasma was entrained in each vortex, at whose leading edge abrupt polarity changes of field and velocity components at current sheets were regularly observed. In the frame of the average boundary layer velocity, the dense blobs were moving predominantly sunward and their scale size along the X plane was approximately 7.4 Earth radii. Inquiring into the generation mechanism of the vortices, we analyze the stability of the boundary layer to sheared flows using compressible magnetohydrodynamic Kelvin-Helmholtz theory with continuous profiles for the physical quantities. We input parameters from (i) the exact theory of magnetosheath flow under aligned solar wind field and flow vectors near the terminator and (ii) the Wind data. It is shown that the configuration is indeed Kelvin-Helmholtz (KH) unstable. This is the first reported example of KH-unstable waves at the magnetopause under a radial IMF.

Observation of nanometer-sized electro-active defects in insulating layers by fluorescence microscopy and electrochemistry.

Science.gov (United States)

Renault, Christophe; Marchuk, Kyle; Ahn, Hyun S; Titus, Eric J; Kim, Jiyeon; Willets, Katherine A; Bard, Allen J

2015-06-02

We report a method to study electro-active defects in passivated electrodes. This method couples fluorescence microscopy and electrochemistry to localize and size electro-active defects. The method was validated by comparison with a scanning probe technique, scanning electrochemical microscopy. We used our method for studying electro-active defects in thin TiO2 layers electrodeposited on 25 μm diameter Pt ultramicroelectrodes (UMEs). The permeability of the TiO2 layer was estimated by measuring the oxidation of ferrocenemethanol at the UME. Blocking of current ranging from 91.4 to 99.8% was achieved. Electro-active defects with an average radius ranging between 9 and 90 nm were observed in these TiO2 blocking layers. The distribution of electro-active defects over the TiO2 layer is highly inhomogeneous and the number of electro-active defect increases for lower degree of current blocking. The interest of the proposed technique is the possibility to quickly (less than 15 min) image samples as large as several hundreds of μm(2) while being able to detect electro-active defects of only a few tens of nm in radius.

Imaging and clinical characteristics of sporadic Creutzfeldt-Jakob disease

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HAN Shun-chang

2013-04-01

Full Text Available Five patients with sporadic Creutzfeldt-Jakob disease (sCJD presented rapidly progressive dementia which were subacute onset from 1 to 4 months. Among these cases, periodic synchronous discharge (PSD of electroencephalography (EEG was seen in 2 patients. Besides, 4 patients obtained positive results in cerebrospinal fluid (CSF analysis for 14-3-3 protein. The cranial MRI examination showed symmetrical or asymmetrical colored-ribbon-shaped high signals in cerebral cortex or basal ganglia by diffusion weighted imaging (DWI, suggesting that DWI had high sensitivity and specificity for the diagnosis of sCJD as a preferred method in the clinical examination of sCJD.

Generation of layering in the upper arctic troposphere away from the jet stream

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A. Karpetchko

Full Text Available Ozone sounding databases for two stations, So-dankylä (67° N, 27° E and Ny-Ålesund (79° N, 12° E were used in order to investigate the generation of layering in the upper and middle troposphere of the Arctic. We concentrated on dry, ozone-rich and stable layers observed below the thermal tropopause under light wind conditions. This condition ensures that the observed layer is not a tropopause fold, a well-known phenomenon that develops within frontal zones near the jet stream. Selection criteria for ozone, humidity and stability anomalies of the tropopause fold detection algorithm were used here to pick out for detailed studies the most pronounced examples of laminae. For all these cases the meteorological situations were investigated in order to establish the origin of the observed layers. We found that layers could be classified into two groups. Laminae of the first group were observed equatorward of the jet stream and those of a second group were observed poleward of the jet. The meteorological situation for the first group resembles that for equatorward stratospheric streamer propagation. It was found that this group accounts for only a small fraction of the layers observed at Sodankylä and for none of those observed at Ny-Ålesund during the period investigated. A large case-to-case variability in the synoptic situation was observed for the second group of laminae, which were detected northward of the jet stream. Nevertheless, in about half of the cases, streamers of tropospheric air were found in the vicinity of the stations on the isentropic surfaces just above the detected stratospheric layers. Back trajectory analyses showed that these layers originated in the vicinity of the polar jet stream. We suppose that laminae-like structures in the troposphere were caused, in both groups, by equatorward (poleward advection of the stratospheric (tropospheric air, together with differential vertical shear. Forward-trajectory calculations

Generation of layering in the upper arctic troposphere away from the jet stream

Directory of Open Access Journals (Sweden)

A. Karpetchko

2003-07-01

Full Text Available Ozone sounding databases for two stations, So-dankylä (67° N, 27° E and Ny-Ålesund (79° N, 12° E were used in order to investigate the generation of layering in the upper and middle troposphere of the Arctic. We concentrated on dry, ozone-rich and stable layers observed below the thermal tropopause under light wind conditions. This condition ensures that the observed layer is not a tropopause fold, a well-known phenomenon that develops within frontal zones near the jet stream. Selection criteria for ozone, humidity and stability anomalies of the tropopause fold detection algorithm were used here to pick out for detailed studies the most pronounced examples of laminae. For all these cases the meteorological situations were investigated in order to establish the origin of the observed layers. We found that layers could be classified into two groups. Laminae of the first group were observed equatorward of the jet stream and those of a second group were observed poleward of the jet. The meteorological situation for the first group resembles that for equatorward stratospheric streamer propagation. It was found that this group accounts for only a small fraction of the layers observed at Sodankylä and for none of those observed at Ny-Ålesund during the period investigated. A large case-to-case variability in the synoptic situation was observed for the second group of laminae, which were detected northward of the jet stream. Nevertheless, in about half of the cases, streamers of tropospheric air were found in the vicinity of the stations on the isentropic surfaces just above the detected stratospheric layers. Back trajectory analyses showed that these layers originated in the vicinity of the polar jet stream. We suppose that laminae-like structures in the troposphere were caused, in both groups, by equatorward (poleward advection of the stratospheric (tropospheric air, together with differential vertical shear. Forward-trajectory calculations

Association between the PRNP 1368 polymorphism and the occurrence of sporadic Creutzfeldt-Jakob disease

Science.gov (United States)

Bratosiewicz-Wąsik, Jolanta; Smoleń-Dzirba, Joanna; Rozemuller, Annemieke J.; Jansen, Casper; Spliet, Wim; Jansen, Gerard H.; Wąsik, Tomasz J.; Liberski, Paweł P.

2012-01-01

Creutzfeldt-Jakob disease (CJD) is a rare transmissible neurodegenerative disorder. The etiology of sporadic form of CJD remains unsolved. In addition to the codon 129 polymorphism, polymorphisms in the non-coding region of PRNP are considered as important factors in sCJD development. To assess a possible association between PRNP 1368 SNP and sCJD, we compared the genotype, allele and haplotype frequencies of the 1368 SNP among 46 sCJD patients of Dutch origin with the respective frequencies in healthy controls. We detected a significant association between sCJD and 1368T/T genotype. A significant difference was also observed in 1368 alleles’ distribution. In the haplotype analysis, haplotype 1368C-129G was associated with decreased risk of sCJD in Dutch population. Our findings support the hypothesis that genetic variations in the regulatory region of the PRNP gene may influence the pathogenesis of sCJD. PMID:22895088

Double layers in space

International Nuclear Information System (INIS)

Carlqvist, P.

1982-07-01

For more than a decade it has been realised that electrostatic double layers are likely to occur in space. We briefly discuss the theoretical background of such double layers. Most of the paper is devoted to an account of the observational evidence for double layers in the ionosphere and magnetosphere of the Earth. Several different experiments are reviewed including rocket and satellite measurements and ground based observations. It is concluded that the observational evidence for double layers in space is very strong. The experimental results indicate that double layers with widely different properties may exist in space. (Author)

Double layers in space

International Nuclear Information System (INIS)

Carlqvist, P.

1982-01-01

For more than a decade it has been realised that electrostatic double layers are likely to occur in space. The author briefly discusses the theoretical background of such double layers. Most of the paper is devoted to an account of the observational evidence for double layers in the ionosphere and magnetosphere of the Earth. Several different experiments are reviewed including rocket and satellite measurements and ground based observations. It is concluded that the observational evidence for double layers in space is very strong. The experimental results indicate that double layers with widely different properties may exist in space. (Auth.)

Synthesis and properties of layered synthetic microstructure (LSM) dispersion elements for 62 eV (200A) to 1.24 keV (10A) radiation. Final report

International Nuclear Information System (INIS)

Barbee, T.W. Jr.

1981-08-01

The opportunities offered by engineered synthetic multilayer dispersion elements for x-rays have been recognized since the earliest days of x-ray diffraction analysis. In this paper, application of sputter deposition technology to the synthesis of Layered Synthetic Microstructure (LSMs) of sufficient quality for use as x-ray dispersion elements is discussed. It will be shown that high efficiency, controllable bandwidth dispersion elements, with d spacings varying from 15 A to 180 A, may be synthesized onto both mechanically stiff and flexible substrates. Multilayer component materials include tungsten, niobium, molybdenum, titanium, vanadium, and silicon layers separated by carbon layers. Experimental observations of peak reflectivity in first order, integrated reflectivity in first order, and diffraction performance at selected photon energies in the range, 100 to 15,000 eV, are reported and compared to theory

Update on Sporadic Colorectal Cancer Genetics.

Science.gov (United States)

Hardiman, Karin M

2018-05-01

Our understanding of the genetics of colorectal cancer has changed dramatically over recent years. Colorectal cancer can be classified in multiple different ways. Along with the advent of whole-exome sequencing, we have gained an understanding of the scale of the genetic changes found in sporadic colorectal cancer. We now know that there are multiple pathways that are commonly involved in the evolution of colorectal cancer including Wnt/β-catenin, RAS, EGFR, and PIK3 kinase. Another recent leap in our understanding of colorectal cancer genetics is the recognition that many, if not all tumors, are actually genetically heterogeneous within individual tumors and also between tumors. Recent research has revealed the prognostic and possibly therapeutic implications of various specific mutations, including specific mutations in BRAF and KRAS . There is increasing interest in the use of mutation testing for screening and surveillance through stool and circulating DNA testing. Recent advances in translational research in colorectal cancer genetics are dramatically changing our understanding of colorectal cancer and will likely change therapy and surveillance in the near future.

Minimally Invasive Treatment of Sporadic Burkitt’s Lymphoma Causing Ileocaecal Invagination

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Paolo Panaccio

2018-01-01

Full Text Available Introduction. Primary NHL (non-Hodgkin lymphoma of the colon represents only 0.2% to 1.2% of all colonic malignancies. Burkitt’s lymphoma (BL is usually a disease reported in children and young people, most of them associated with EBV or HIV infection. We describe a rare case of intestinal obstruction due to sporadic Burkitt’s lymphoma causing ileocaecal invagination explaining our experience Methods. A 31-year-old man presented with diffuse colic pain and weight loss. Clinical examination revealed an abdominal distension with pain in the right iliac fossa. Colonoscopy documented a caecal large lesion with ulcerated mucosa. Computed tomography (CT have shown a 60 × 50 mm right colic parietal lesion with signs of ileocolic intussusception. Results. Laparoscopic right hemicolectomy was performed. Postoperative period was uneventful. CD20+ high-grade B-cell Burkitt’s lymphoma was confirmed by immunohistochemistry (CD20+, CD79+, and CD10+ and FISH test (t (8;14 (q24; q32. The patient was subsequently treated with adjuvant combination chemotherapy (Hyper-CVAD and is alive and disease-free at 8 months follow-up. Discussion. Adult sporadic Burkitt’s lymphoma (BL causing intestinal obstruction due to ileocaecal intussusception is an extremely rare occurrence and a diagnostic dilemma. Despite the surgical approach is selected based on patient’s conditions and surgeon’s expertise, minimally invasive method could be preferred.

Independent Induction of Caspase-8 and cFLIP Expression during Colorectal Carcinogenesis in Sporadic and HNPCC Adenomas and Carcinomas

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D. M. Heijink

2007-01-01

Full Text Available Background: TNF-Related Apoptosis Inducing Ligand (TRAIL is a promising agent for the induction of apoptosis in neoplastic tissues. Important determinants of TRAIL sensitivity are two intracellular proteins of the TRAIL pathway, caspase-8 and its anti-apoptotic competitor cellular Flice-Like Inhibitory Protein (cFLIP. Methods: The aim of this study was to investigate basic expression of caspase-8 and cFLIP in normal colorectal epithelium (n = 20, colorectal adenomas (n = 66 and colorectal carcinomas (n = 44 using immunohistochemistry performed on both sporadic and Hereditary Non-Polyposis Colorectal Cancer (HNPCC or Lynch syndrome-associated adenomas and carcinomas. Results: Expression of both caspase-8 and cFLIP was similar in cases with sporadic and hereditary origin. Expression of caspase-8 in colorectal adenomas and carcinomas was increased when compared to normal colon tissue (P = 0.02. Nuclear, paranuclear as well as cytoplasmic localizations of caspase-8 were detected. Immunohistochemistry revealed an upregulation of cFLIP in colorectal carcinomas in comparison to normal epithelium and colorectal adenomas (P < 0.001. A large variation in the caspase-8/cFLIP ratio was observed between the individual adenomas and carcinomas. Conclusion: Caspase-8 and cFLIP are upregulated during colorectal carcinogenesis. Upregulation of caspase-8 and/or downregulation of cFLIP may be interesting approaches to maximize TRAIL sensitivity in colorectal neoplasms.

An autopsied case of MV2K + C-type sporadic Creutzfeldt-Jakob disease presenting with widespread cerebral cortical involvement and Kuru plaques.

Science.gov (United States)

Iwasaki, Yasushi; Saito, Yufuko; Aiba, Ikuko; Kobayashi, Atsushi; Mimuro, Maya; Kitamoto, Tetsuyuki; Yoshida, Mari

2017-06-01

MV2-type sporadic Creutzfeldt-Jakob disease (sCJD), which was previously called "Kuru-plaque variant", was gradually revealed to have a wide spectrum and has been classified into three pathological subtypes: MV2K, MV2C and MV2K + C. We herein describe the detailed clinical findings and neuropathologic observations from an autopsied MV2K + C-type Japanese sCJD case with widespread cerebral cortical pathology and Kuru plaques. In the early stages of the disease, the patient exhibited gait disturbance with ataxia and dysarthria as well as gradual appearance of cognitive dysfunction. Diffusion-weighted images (DWI) on MRI revealed extensive cerebral cortical hyperintensity. Pathologic investigation revealed extensive spongiform change in the cerebral cortex, particularly in the deeper layers. Vacuole size varied, and some were confluent. Prion protein (PrP) immunostaining revealed extensive PrP deposition in the cerebral cortex, basal ganglia, thalamus, cerebellum, brainstem and spinal cord. In the cerebral cortex, synaptic-type, Kuru plaque-like, and coarse plaque-type PrP depositions were mainly observed, along with some perivacuolar-type PrP depositions. Kuru plaques and coarse plaque-type PrP depositions also were observed in the cerebellar cortex. PrP gene analysis revealed no mutations, and polymorphic codon 129 exhibited Met/Val heterozygosity. Western blot analysis revealed a mixture of intermediate-type PrP Sc and type 2 PrP Sc . Based on previous reports regarding MV2-type sCJD and the clinicopathologic findings of the present case, we speculated that it may be possible to clinically distinguish each MV2 subtype. Clinical presentation of the MV2K + C subtype includes predominant cerebral cortical involvement signs with ataxia and DWI hyperintensity of the cerebral cortex on MRI. © 2016 Japanese Society of Neuropathology.

Direct in situ transmission electron microscopy observation of Al push up during early stages of the Al-induced layer exchange

International Nuclear Information System (INIS)

Birajdar, B.I.; Antesberger, T.; Butz, B.; Stutzmann, M.; Spiecker, E.

2012-01-01

The mechanism of Al transport during Al-induced layer exchange and crystallization of amorphous Si (a-Si) has been investigated by in situ and analytical transmission electron microscopy. Significant grain boundary realignment and coarsening of Al grains close to the Si crystallization growth front as well as push up of excess Al into the a-Si layer at distances even a few micrometers away from the crystallization front were observed. Stress-mediated diffusion of Al is postulated to explain the experimental observations.

Diagnostics of the Tropical Tropopause Layer from in-situ observations and CCM data

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C. M. Volk

2009-12-01

Full Text Available A suite of diagnostics is applied to in-situ aircraft measurements and one Chemistry-Climate Model (CCM data to characterize the vertical structure of the Tropical Tropopause Layer (TTL. The diagnostics are based on vertical tracer profiles and relative vertical tracer gradients, using tropopause-referenced coordinates, and tracer-tracer relationships in the tropical Upper Troposphere/Lower Stratosphere (UT/LS.

Observations were obtained during four tropical campaigns performed from 1999 to 2006 with the research aircraft Geophysica and have been compared to the output of the ECHAM5/MESSy CCM. The model vertical resolution in the TTL (~500 m allows for appropriate comparison with high-resolution aircraft observations and the diagnostics used highlight common TTL features between the model and the observational data.

The analysis of the vertical profiles of water vapour, ozone, and nitrous oxide, in both the observations and the model, shows that concentration mixing ratios exhibit a strong gradient change across the tropical tropopause, due to the role of this latter as a transport barrier and that transition between the tropospheric and stratospheric regimes occurs within a finite layer. The use of relative vertical ozone and carbon monoxide gradients, in addition to the vertical profiles, helps to highlight the region where this transition occurs and allows to give an estimate of its thickness. The analysis of the CO-O₃ and H₂O-O₃ scatter plots and of the Probability Distribution Function (PDF of the H₂O-O₃ pair completes this picture as it allows to better distinguish tropospheric and stratospheric regimes that can be identified by their different chemical composition.

The joint analysis and comparison of observed and modelled data allows to state that the model can represent the background TTL structure and its seasonal variability rather accurately. The model

Electromagnetic ion cyclotron waves observed in the plasma depletion layer

Science.gov (United States)

Anderson, B. J.; Fuselier, S. A.; Murr, D.

1991-01-01

Observations from AMPTE/CCE in the earth's magnetosheath on October 5, 1984 are presented to illustrate 0.1 - 4.0 Hz magnetic field pulsations in the subsolar plasma depletion layer (PDL) for northward sheath field during a magnetospheric compression. The PDL is unambiguously identified by comparing CCE data with data from IRM in the upstream solar wind. Pulsations in the PDL are dominated by transverse waves with F/F(H+) 1.0 or less and a slot in spectral power at F/F(H+) = 0.5. The upper branch is left hand polarized while the lower branch is linearly polarized. In the sheath the proton temperature anisotropy is about 0.6 but it is about 1.7 in the PDL during wave occurrence. The properties and correlation of waves with increased anisotropy indicate that they are electromagnetic ion cyclotron waves.

Detection of infectivity in blood of persons with variant and sporadic Creutzfeldt-Jakob disease.

Science.gov (United States)

Douet, Jean Yves; Zafar, Saima; Perret-Liaudet, Armand; Lacroux, Caroline; Lugan, Séverine; Aron, Naima; Cassard, Herve; Ponto, Claudia; Corbière, Fabien; Torres, Juan Maria; Zerr, Inga; Andreoletti, Olivier

2014-01-01

We report the presence of infectivity in erythrocytes, leukocytes, and plasma of 1 person with variant Creutzfeldt-Jakob disease and in the plasma of 2 in 4 persons whose tests were positive for sporadic Creutzfeldt-Jakob disease. The measured infectivity levels were comparable to those reported in various animals with transmissible spongiform encephalopathies.

The monster sporadic group and a theory underlying superstring models

International Nuclear Information System (INIS)

Chapline, G.

1996-09-01

The pattern of duality symmetries acting on the states of compactified superstring models reinforces an earlier suggestion that the Monster sporadic group is a hidden symmetry for superstring models. This in turn points to a supersymmetric theory of self-dual and anti-self-dual K3 manifolds joined by Dirac strings and evolving in a 13 dimensional spacetime as the fundamental theory. In addition to the usual graviton and dilaton this theory contains matter-like degrees of freedom resembling the massless states of the heterotic string, thus providing a completely geometric interpretation for ordinary matter. 25 refs

High signal of the striatum in sporadic Creutzfeldt-Jakob disease: sequential change on T2-weighted MRI

International Nuclear Information System (INIS)

Uemura, A.; O'uchi, T.; Sakamoto, T.; Yashiro, N.

2002-01-01

The object of this study is to describe the sequential change of high signal of the striatum on T2-weighted MRI in sporadic Creutzfeldt-Jakob disease (CJD). Three cases of autopsy-proven sporadic CJD and a total of 18 serial MR images are included in this study. The degree of high signal of the striatum on T2-weighted MRI was evaluated by two neuroradiologists and divided into four grades by mutual agreement. Initial MRI of all three cases showed a slightly high signal of the bilateral striatum, and the conspicuity of the high signal became more prominent as the disease progressed. In each case the pathological change of striatum and globus pallidus was compared with the high signal on the last MR image. (orig.)

High signal of the striatum in sporadic Creutzfeldt-Jakob disease: sequential change on T2-weighted MRI

Energy Technology Data Exchange (ETDEWEB)

Uemura, A.; O' uchi, T.; Sakamoto, T.; Yashiro, N. [Department of Radiology, Kameda Medical Center, Kamogawa, Chiba (Japan)

2002-04-01

The object of this study is to describe the sequential change of high signal of the striatum on T2-weighted MRI in sporadic Creutzfeldt-Jakob disease (CJD). Three cases of autopsy-proven sporadic CJD and a total of 18 serial MR images are included in this study. The degree of high signal of the striatum on T2-weighted MRI was evaluated by two neuroradiologists and divided into four grades by mutual agreement. Initial MRI of all three cases showed a slightly high signal of the bilateral striatum, and the conspicuity of the high signal became more prominent as the disease progressed. In each case the pathological change of striatum and globus pallidus was compared with the high signal on the last MR image. (orig.)

Layer-dependent band alignment and work function of few-layer phosphorene.

Science.gov (United States)

Cai, Yongqing; Zhang, Gang; Zhang, Yong-Wei

2014-10-20

Using first-principles calculations, we study the electronic properties of few-layer phosphorene focusing on layer-dependent behavior of band gap, work function band alignment and carrier effective mass. It is found that few-layer phosphorene shows a robust direct band gap character, and its band gap decreases with the number of layers following a power law. The work function decreases rapidly from monolayer (5.16 eV) to trilayer (4.56 eV), and then slowly upon further increasing the layer number. Compared to monolayer phosphorene, there is a drastic decrease of hole effective mass along the ridge (zigzag) direction for bilayer phosphorene, indicating a strong interlayer coupling and screening effect. Our study suggests that 1). Few-layer phosphorene with a layer-dependent band gap and a robust direct band gap character is promising for efficient solar energy harvest. 2). Few-layer phosphorene outperforms monolayer counterpart in terms of a lighter carrier effective mass, a higher carrier density and a weaker scattering due to enhanced screening. 3). The layer-dependent band edges and work functions of few-layer phosphorene allow for modification of Schottky barrier with enhanced carrier injection efficiency. It is expected that few-layer phosphorene will present abundant opportunities for a plethora of new electronic applications.

Benthic boundary layer modelling studies

International Nuclear Information System (INIS)

Richards, K.J.

1984-01-01

A numerical model has been developed to study the factors which control the height of the benthic boundary layer in the deep ocean and the dispersion of a tracer within and directly above the layer. This report covers tracer clouds of horizontal scales of 10 to 100 km. The dispersion of a tracer has been studied in two ways. Firstly, a number of particles have been introduced into the flow. The trajectories of these particles provide information on dispersion rates. For flow conditions similar to those observed in the abyssal N.E. Atlantic the diffusivity of a tracer was found to be 5 x 10 6 cm 2 s -1 for a tracer within the boundary layer and 8 x 10 6 cm 2 s -1 for a tracer above the boundary layer. The results are in accord with estimates made from current meter measurements. The second method of studying dispersion was to calculate the evolution of individual tracer clouds. Clouds within and above the benthic boundary layer often show quite different behaviour from each other although the general structure of the clouds in the two regions were found to have no significant differences. (author)

Origin of non-spherical particles in the boundary layer over Beijing, China: based on balloon-borne observations.

Science.gov (United States)

Chen, Bin; Yamada, Maromu; Iwasaka, Yasunobu; Zhang, Daizhou; Wang, Hong; Wang, Zhenzhu; Lei, Hengchi; Shi, Guangyu

2015-10-01

Vertical structures of aerosols from the ground to about 1,000 m altitude in Beijing were measured with a balloon-borne optical particle counter. The results showed that, in hazy days, there were inversions at approximately 500-600 m, below which the particulate matters were well mixed vertically, while the concentration of particles decreased sharply above the mixing layer. Electron microscopic observation of the particles collected with the balloon-borne impactor indicates that the composition of particles is different according to weather conditions in the boundary mixing layer of Beijing city and suggests that dust particles are always dominant in coarse-mode particles. Interestingly, sea-salt particles are frequently identified, suggesting the importance of marine air inflow to the Beijing area even in summer. The Ca-rich spherical particles are also frequently identified, suggesting chemical modification of dust particle by NOx or emission of CaO and others from local emission. Additionally, those types of particles showed higher concentration above the mixing layer under the relatively calm weather condition of summer, suggesting the importance of local-scale convection found in summer which rapidly transported anthropogenic particles above the mixing layer. Lidar extinction profiles qualitatively have good consistency with the balloon-borne measurements. Attenuation effects of laser pulse intensity are frequently observed due to high concentration of particulate matter in the Beijing atmosphere, and therefore quantitative agreement of lidar return and aerosol concentration can be hardly observed during dusty condition. Comparing the depolarization ratio obtained from the lidar measurements with the balloon-borne measurements, the contribution of the dry sea-salt particles, in addition to the dust particles, is suggested as an important factor causing depolarization ratio in the Beijing atmosphere.

Photoluminescence of phosphorus atomic layer doped Ge grown on Si

Science.gov (United States)

Yamamoto, Yuji; Nien, Li-Wei; Capellini, Giovanni; Virgilio, Michele; Costina, Ioan; Schubert, Markus Andreas; Seifert, Winfried; Srinivasan, Ashwyn; Loo, Roger; Scappucci, Giordano; Sabbagh, Diego; Hesse, Anne; Murota, Junichi; Schroeder, Thomas; Tillack, Bernd

2017-10-01

Improvement of the photoluminescence (PL) of Phosphorus (P) doped Ge by P atomic layer doping (ALD) is investigated. Fifty P delta layers of 8 × 1013 cm-2 separated by 4 nm Ge spacer are selectively deposited at 300 °C on a 700 nm thick P-doped Ge buffer layer of 1.4 × 1019 cm-3 on SiO2 structured Si (100) substrate. A high P concentration region of 1.6 × 1020 cm-3 with abrupt P delta profiles is formed by the P-ALD process. Compared to the P-doped Ge buffer layer, a reduced PL intensity is observed, which might be caused by a higher density of point defects in the P delta doped Ge layer. The peak position is shifted by ˜0.1 eV towards lower energy, indicating an increased active carrier concentration in the P-delta doped Ge layer. By introducing annealing at 400 °C to 500 °C after each Ge spacer deposition, P desorption and diffusion is observed resulting in relatively uniform P profiles of ˜2 × 1019 cm-3. Increased PL intensity and red shift of the PL peak are observed due to improved crystallinity and higher active P concentration.

Ion acoustic waves and double-layers in electronegative expanding plasmas

International Nuclear Information System (INIS)

Plihon, Nicolas; Chabert, Pascal

2011-01-01

Ion acoustic waves and double-layers are observed in expanding plasmas in electronegative gases, i.e., plasmas containing an appreciable fraction of negative ions. The reported experiments are performed in argon gas with a variable amount of SF 6 . When varying the amount of SF 6 , the negative ion fraction increases and three main regimes were identified previously: (i) the plasma smoothly expands at low negative ion fraction, (ii) a static double-layer (associated with an abrupt potential drop and ion acceleration) forms at intermediate negative ion fraction, (iii) double-layers periodically form and propagate (in the plasma expansion direction) at high negative ion fraction. In this paper, we show that transition phases exist in between these regimes, where fluctuations are observed. These fluctuations are unstable slow ion acoustic waves, propagating in the direction opposite to the plasma expansion. These fluctuations are excited by the most unstable eigenmodes and display turbulent features. It is suggested that the static double layer forms when the ion acoustic fluctuations become non-linearly unstable: the double layer regime being a bifurcated state of the smoothly expanding regime. For the highest negative ion fraction, a coexistence of (upstream propagating) slow ion acoustic fluctuations and (downstream) propagating double layers was observed.

Initializing a Mesoscale Boundary-Layer Model with Radiosonde Observations

Science.gov (United States)

Berri, Guillermo J.; Bertossa, Germán

2018-01-01

A mesoscale boundary-layer model is used to simulate low-level regional wind fields over the La Plata River of South America, a region characterized by a strong daily cycle of land-river surface-temperature contrast and low-level circulations of sea-land breeze type. The initial and boundary conditions are defined from a limited number of local observations and the upper boundary condition is taken from the only radiosonde observations available in the region. The study considers 14 different upper boundary conditions defined from the radiosonde data at standard levels, significant levels, level of the inversion base and interpolated levels at fixed heights, all of them within the first 1500 m. The period of analysis is 1994-2008 during which eight daily observations from 13 weather stations of the region are used to validate the 24-h surface-wind forecast. The model errors are defined as the root-mean-square of relative error in wind-direction frequency distribution and mean wind speed per wind sector. Wind-direction errors are greater than wind-speed errors and show significant dispersion among the different upper boundary conditions, not present in wind speed, revealing a sensitivity to the initialization method. The wind-direction errors show a well-defined daily cycle, not evident in wind speed, with the minimum at noon and the maximum at dusk, but no systematic deterioration with time. The errors grow with the height of the upper boundary condition level, in particular wind direction, and double the errors obtained when the upper boundary condition is defined from the lower levels. The conclusion is that defining the model upper boundary condition from radiosonde data closer to the ground minimizes the low-level wind-field errors throughout the region.

Magnetic domain observation of FeCo thin films fabricated by alternate monoatomic layer deposition

Energy Technology Data Exchange (ETDEWEB)

Ohtsuki, T., E-mail: ohtsuki@spring8.or.jp; Kotsugi, M.; Ohkochi, T. [Japan Synchrotron Radiation Research Institute (JASRI), 1-1-1 Koto, Sayo-cho, Sayo-gun, Hyogo 679-5198 (Japan); Kojima, T.; Mizuguchi, M.; Takanashi, K. [Institute for Materials Research, Tohoku University, 2-1-1 Katahira, Aoba-ku, Sendai 980-8577 (Japan)

2014-01-28

FeCo thin films are fabricated by alternate monoatomic layer deposition method on a Cu{sub 3}Au buffer layer, which in-plane lattice constant is very close to the predicted value to obtain a large magnetic anisotropy constant. The variation of the in-plane lattice constant during the deposition process is investigated by reflection high-energy electron diffraction. The magnetic domain images are also observed by a photoelectron emission microscope in order to microscopically understand the magnetic structure. As a result, element-specific magnetic domain images show that Fe and Co magnetic moments align parallel. A series of images obtained with various azimuth reveal that the FeCo thin films show fourfold in-plane magnetic anisotropy along 〈110〉 direction, and that the magnetic domain structure is composed only of 90∘ wall.

Investigation of CD28 gene polymorphisms in patients with sporadic breast cancer in a Chinese Han population in Northeast China.

Directory of Open Access Journals (Sweden)

Shuang Chen

Full Text Available BACKGROUND: CD28 is one of a number of costimulatory molecules that play crucial roles in immune regulation and homeostasis. Accumulating evidence indicates that immune factors influence breast carcinogenesis. To clarify the relationships between polymorphisms in the CD28 gene and breast carcinogenesis, a case-control study was conducted in women from Heilongjiang Province in northeast of China. METHODOLOGY/PRINCIPAL FINDINGS: Our research subjects consisted of 565 female patients with sporadic breast cancer and 605 age- and sex-matched healthy controls. In total, 12 single nucleotide polymorphisms (SNPs in the CD28 gene were successfully determined using the polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP method. The relationship between the CD28 variants and clinical features, including histological grade, tumor size, lymph node metastasis, human epidermal growth factor receptor 2 (C-erbB2, estrogen receptor (ER, progesterone receptor (PR, and tumor protein 53 (P53 status were analyzed. A statistically significant association was observed between rs3116496 and breast cancer risk under different genetic models (additive P = 0.0164, dominant P = 0.0042. Different distributions of the rs3116496 'T' allele were found in patients and controls, which remained significant after correcting the P value for multiple testing using Haploview with 10,000 permutations (corrected P = 0.0384. In addition, significant associations were observed between rs3116487/rs3116494 (D' = 1, r(2 = 0.99 and clinicopathological features such as C-erbB2 and ER status, in breast cancer patients. CONCLUSIONS/SIGNIFICANCE: Our findings indicate that CD28 gene polymorphisms contribute to sporadic breast cancer risk and have a significant association with clinicopathological features in a northeast Chinese Han population.

A genome wide association study links glutamate receptor pathway to sporadic Creutzfeldt-Jakob disease risk

NARCIS (Netherlands)

P. Sanchez-Juan (Pascual); M.T. Bishop (Matthew); G.G. Kovacs (Gabor); M. Calero (Miguel); Y.S. Aulchenko (Yurii); A. Ladogana (Anna); A. Boyd (Alison); V. Lewis (Victoria); C. Ponto (Claudia); Calero, O. (Olga); A. Poleggi (Anna); A. Carracedo (Angel); S.J. van der Lee (Sven); T. Ströbel (Thomas); F. Rivadeneira Ramirez (Fernando); A. Hofman (Albert); S. Haik; O. Combarros (Onofre); J. Berciano (José); A.G. Uitterlinden (André); S.J. Collins (Steven); H. Budka (Herbert); J-P. Brandel (Jean-Philippe); J.-L. Laplanche (Jean-Louis); M. Pocchiari (Maurizio); I. Zerr (Inga); R. Knight (Richard); R.G. Will (Robert); C.M. van Duijn (Cornelia)

2015-01-01

textabstractWe performed a genome-wide association (GWA) study in 434 sporadic Creutzfeldt-Jakob disease (sCJD) patients and 1939 controls from the United Kingdom, Germany and The Netherlands. The findings were replicated in an independent sample of 1109 sCJD and 2264 controls provided by a

Local layer structure of smectic liquid crystals by X-ray micro-diffraction

CERN Document Server

Takanishi, Y

2003-01-01

The local layer structure of smectic liquid crystal has been measured using time-resolved synchrotron X-ray micro-diffraction. Typical layer disorders observed in surface stabilized (anti-) ferroelectric liquid crystals, i.e. a stripe texture, a needed-like defect and a zigzag defect, are directly analyzed. The detailed analysis slows that the surface anchoring force due to the interaction between the liquid crystal molecule and the alignment thin film plays an important role to realize both the static and dynamic local layer structures. The layer structure of the circular domain observed in the liquid crystal of bent-shaped molecules found to depend on the applied electric field though the optical micrograph shows little difference. The frustrated, double and single layer structures of the bent-shaped molecule liquid crystal are determined depending on the terminal alkyl chain length. (author)

Italian field survey reveals a high diffusion of avian metapneumovirus subtype B in layers and weaknesses in the vaccination strategy applied.

Science.gov (United States)

Cecchinato, Mattia; Lupini, Caterina; Ricchizzi, Enrico; Falchieri, Marco; Meini, Amelio; Jones, Richard C; Catelli, Elena

2012-12-01

The current information on the prevalence of avian metapneumovirus (aMPV) infection in layers is fragmentary and its true impact on egg production often remains unknown or unclear. In order to draw an epidemiologic picture of aMPV presence in layer flocks in Italy, a survey was performed on 19 flocks of pullets and layers based on longitudinal studies or sporadic samplings. aMPV was detected by reverse transcription (RT)-PCR, and blood samples were collected for serology by aMPV ELISA. Occurrences of respiratory signs and a drop in egg production were recorded. Possible involvement of infectious bronchitis (IB) and egg drop syndrome (EDS) viruses that could have caused loss of egg production we ruled out for IB virus by RT-PCR, and EDS virus was ruled out by hemagglutination-inhibition (HI). Only subtype B of aMPV was found in both pullet and layer farms. Surveys of pullets showed that most groups became infected prior to the onset of lay without showing clear respiratory signs. At the point of lay, these groups were serologically positive to aMPV. In two layer flocks, egg drops were observed and could be strongly linked to the presence of aMPV infection. Results were correlated with aMPV vaccination programs applied to the birds in three flocks on the same farm. Only a vaccination program which included two live and one killed vaccines gave complete protection from aMPV infection to the birds, while a single live vaccine application was not efficacious. The current study gives an inside view of field aMPV diffusion in Italy and its control in layers.

The ‘Pokemon’ (ZBTB7) Gene: No Evidence of Association with Sporadic Breast Cancer

Science.gov (United States)

Salas, Antonio; Vega, Ana; Milne, Roger L.; García-Magariños, Manuel; Ruibal, Álvaro; Benítez, Javier; Carracedo, Ángel

2008-01-01

It has been proposed that the excess of familiar risk associated with breast cancer could be explained by the cumulative effect of multiple weakly predisposing alleles. The transcriptional repressor FBI1, also known as Pokemon, has recently been identified as a critical factor in oncogenesis. This protein is encoded by the ZBTB7 gene. Here we aimed to determine whether polymorphisms in ZBTB7 are associated with breast cancer risk in a sample of cases and controls collected in hospitals from North and Central Spanish patients. We genotyped 15 SNPs in ZBTB7, including the flanking regions, with an average coverage of 1 SNP/2.4 Kb, in 360 sporadic breast cancer cases and 402 controls. Comparison of allele, genotype and haplotype frequencies between cases and controls did not reveal associations using Pearson’s chi-square test and a permutation procedure to correct for multiple test. In this, the first study of the ZBTB7 gene in relation to, sporadic breast cancer, we found no evidence of an association. PMID:21892298

Characterizing the Asian Tropopause Aerosol Layer (ATAL) Using Satellite Observations, Balloon Measurements and a Chemical Transport Model

Science.gov (United States)

Fairlie, T. D.; Vernier, J.-P.; Liu, H.; Deshler, T.; Natarajan, M.; Bedka, K.; Wegner, T.; Baker, N.; Gadhavi, H.; Ratnam, M. V.;

Layer-specific morphological and molecular differences in neocortical astrocytes and their dependence on neuronal layers.

Science.gov (United States)

Lanjakornsiripan, Darin; Pior, Baek-Jun; Kawaguchi, Daichi; Furutachi, Shohei; Tahara, Tomoaki; Katsuyama, Yu; Suzuki, Yutaka; Fukazawa, Yugo; Gotoh, Yukiko

2018-04-24

Non-pial neocortical astrocytes have historically been thought to comprise largely a nondiverse population of protoplasmic astrocytes. Here we show that astrocytes of the mouse somatosensory cortex manifest layer-specific morphological and molecular differences. Two- and three-dimensional observations revealed that astrocytes in the different layers possess distinct morphologies as reflected by differences in cell orientation, territorial volume, and arborization. The extent of ensheathment of synaptic clefts by astrocytes in layer II/III was greater than that by those in layer VI. Moreover, differences in gene expression were observed between upper-layer and deep-layer astrocytes. Importantly, layer-specific differences in astrocyte properties were abrogated in reeler and Dab1 conditional knockout mice, in which neuronal layers are disturbed, suggesting that neuronal layers are a prerequisite for the observed morphological and molecular differences of neocortical astrocytes. This study thus demonstrates the existence of layer-specific interactions between neurons and astrocytes, which may underlie their layer-specific functions.

The influence of sporadic anovulation on hormone levels in ovulatory cycles

Science.gov (United States)

Hambridge, H.L.; Mumford, S.L.; Mattison, D.R.; Ye, A.; Pollack, A.Z.; Bloom, M.S.; Mendola, P.; Lynch, K.L.; Wactawski-Wende, J.; Schisterman, E.F.

2013-01-01

STUDY QUESTION Do ovulatory hormone profiles among healthy premenopausal women differ between women with and without sporadic anovulation? SUMMARY ANSWER Women with one anovulatory cycle tended to have lower estradiol, progesterone and LH peak levels during their ovulatory cycle. WHAT IS KNOWN ALREADY Anovulation occurs sporadically in healthy premenopausal women, but the influence of hormones in a preceding cycle and the impact on a subsequent cycle's hormone levels is unknown. STUDY DESIGN, SIZE, DURATION The BioCycle Study was a prospective cohort including 250 healthy regularly menstruating women, 18–44 years of age, from Western New York with no history of menstrual or ovulation disorders. The women were followed with up to eight study visits per cycle for two cycles, most of which were consecutive. PARTICIPANTS/MATERIALS, SETTING AND METHODS All study visits were timed to menstrual cycle phase using fertility monitors and located at the University at Buffalo women's health research center from 2005 to 2007. The main outcomes measured were estradiol, progesterone, LH and follicle-stimulating hormone levels in serum at up to 16 visits over two cycles. Anovulation was defined as peak serum progesterone concentrations ≤5 ng/ml and no serum LH peak detected during the mid- or late-luteal phase visit. MAIN RESULTS AND THE ROLE OF CHANCE Reproductive hormone concentrations were lower during anovulatory cycles, but significant reductions were also observed in estradiol (−25%, P = 0.003) and progesterone (−22%, P = 0.001) during the ovulatory cycles of women with one anovulatory cycle compared with women with two ovulatory cycles. LH peak concentrations were decreased in the ovulatory cycle of women with an anovulatory cycle (significant amplitude effect, P = 0.004; geometric mean levels 38% lower, P cycles, and no ultrasound assessment of ovulation was available. Data were missing for a total of 168 of a possible 4072 cycle visits (4.1%), though all women had

Impact of deep convection in the tropical tropopause layer in West Africa: in-situ observations and mesoscale modelling

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F. Fierli

2011-01-01

Full Text Available We present the analysis of the impact of convection on the composition of the tropical tropopause layer region (TTL in West-Africa during the AMMA-SCOUT campaign. Geophysica M55 aircraft observations of water vapor, ozone, aerosol and CO₂ during August 2006 show perturbed values at altitudes ranging from 14 km to 17 km (above the main convective outflow and satellite data indicates that air detrainment is likely to have originated from convective cloud east of the flights. Simulations of the BOLAM mesoscale model, nudged with infrared radiance temperatures, are used to estimate the convective impact in the upper troposphere and to assess the fraction of air processed by convection. The analysis shows that BOLAM correctly reproduces the location and the vertical structure of convective outflow. Model-aided analysis indicates that convection can influence the composition of the upper troposphere above the level of main outflow for an event of deep convection close to the observation site. Model analysis also shows that deep convection occurring in the entire Sahelian transect (up to 2000 km E of the measurement area has a non negligible role in determining TTL composition.

Source attribution of human salmonellosis using a meta-analysis of case-control studies of sporadic infections

DEFF Research Database (Denmark)

Coutinho Calado Domingues, Ana Rita; Pires, Sara Monteiro; Hisham Beshara Halasa, Tariq

2012-01-01

Salmonella is an important cause of human illness. Disease is frequently associated with foodborne transmission, but other routes of exposure are recognized. Identifying sources of disease is essential for prioritizing public health interventions. Numerous case-control studies of sporadic salmone...

Study of toughening mechanisms through the observations of crack propagation in nanostructured and layered metallic sheet

International Nuclear Information System (INIS)

Chen, A.Y.; Li, D.F.; Zhang, J.B.; Liu, F.; Liu, X.R.; Lu, J.

2011-01-01

Highlights: → A nanostructured and layered steel exhibits high strength and large ductility. → The excellent combination originates from a multiple interlaminar cracking. → The initiation and propagation of cracks are controlled by three aspects. → The cracks are deflected by interface and arrested by compressive residual stress. → Finally, the cracks are blunted by the graded grain size distribution. - Abstract: A layered and nanostructured (LN) 304 SS sheet was produced by combination of surface mechanical attrition treatment (SMAT) with warm co-rolling. The microstructure of LN sheet is characterized by a periodic distribution of nanocrystalline layers and micron-grained layers with a graded transition of grain size. Tensile test results show that exceptional properties of high yield strength and large elongation to fracture are achieved. A multiple interlaminar cracking was observed by scanning electron microscopy, which is induced by repeated crack initiation and propagation. The toughening mechanisms of the LN sheet are proposed to be controlling the crack propagation path by several strategies. The main cracks initiating at interface defects are arrested by large compressive residual stress, deflected by weak interface bonding and blunted by the graded grain size distribution.

Investigation of the spatio-temporal variability of atmospheric boundary layer depths over mountainous terrain observed with a suite of ground-based and airborne instruments during the MATERHORN field experiment

Science.gov (United States)

Pal, S.; De Wekker, S.; Emmitt, G. D.

2013-12-01

We present first results of the spatio-temporal variability of atmospheric boundary layer depths obtained with a suite of ground-based and airborne instruments deployed during the first field phase of The Mountain Terrain Atmospheric Modeling and Observations (MATERHORN) Program (http://www3.nd.edu/~dynamics/materhorn/index.php) at Dugway Proving Ground (DPG, Utah, USA) in Fall 2012. We mainly use high-resolution data collected on selected intensive observation periods obtained by Doppler lidars, ceilometer, and in-situ measurements from an unmanned aerial vehicle for the measurements of atmospheric boundary layer (ABL) depths. In particular, a Navy Twin Otter aircraft flew 6 missions of about 5 hours each during the daytime, collecting remotely sensed (Doppler lidar, TODWL) wind data in addition to in-situ turbulence measurements which allowed a detailed investigation of the spatial heterogeneity of the convective boundary layer turbulence features over a steep isolated mountain of a horizontal and vertical scale of about 10 km and 1 km, respectively. Additionally, we use data collected by (1) radiosonde systems at two sites of Granite Mountain area in DPG (Playa and Sagebrush), (2) sonic anemometers (CSAT-3D) for high resolution turbulence flux measurements near ground, (3) Pyranometer for incoming solar radiation, and (4) standard meteorological measurements (PTU) obtained near the surface. In this contribution, we discuss and address (1) composites obtained with lidar, ceilometer, micro-meteorological measurements, and radiosonde observations to determine the quasi-continuous regime of ABL depths, growth rates, maximum convective boundary layer (CBL) depths, etc., (2) the temporal variability in the ABL depths during entire diurnal cycle and the spatial heterogeneity in the daytime ABL depths triggered by the underlying orography in the experimental area to investigate the most possible mechanisms (e.g. combined effect of diurnal cycle and orographic trigger

Emerging therapeutic options for sporadic inclusion body myositis

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Alfano LN

2015-09-01

Full Text Available Lindsay N Alfano, Linda P Lowes Nationwide Children’s Hospital, Center for Gene Therapy, Columbus, OH, USA Abstract: Sporadic inclusion body myositis is the most common inflammatory muscle disorder preferentially affecting males over the age of 40 years. Progressive muscle weakness of the finger flexors and quadriceps muscles results in loss of independence with activities of daily living and eventual wheelchair dependence. Initial signs of disease are often overlooked and can lead to mis- or delayed diagnosis. The underlying cause of disease is unknown, and disease progression appears refractory to available treatment options. This review discusses the clinical presentation of inclusion body myositis and the current efforts in diagnosis, and focuses on the current state of research for both nonpharmacological and pharmacological treatment options for this patient group. Keywords: myositis, inclusion body myositis, inflammatory myopathy, treatment, function, outcomes

Energetic magnetospheric protons in the plasma depletion layer

International Nuclear Information System (INIS)

Fuselier, S.A.

1992-01-01

Interplanetary magnetic field draping against the Earth's dayside subsolar magnetopause creates a region of reduced plasma density and increased magnetic field called the plasma depletion layer. In this region, leakage of energetic ions from the Earth's magnetosphere onto magnetic field lines in the plasma depletion layer can be studied without interference from ions accelerated at the Earth's quasi-parallel bow shock. Active Magnetospheric Particle Tracer Experiment/Charge Composition Explorer (AMPTE/CCE) observations for 13 plasma depletion layer events are used to determine the characteristics of energetic protons between a few keV/e and ∼100keV/e leaked from the magnetosphere. Results indicate that the leaked proton distributions resemble those in the magnetosphere except that they have lower densities and temperatures and much higher velocities parallel (or antiparallel) and perpendicular to the magnetic field. Compared to the low-energy magnetosheath proton distributions present in the depletion layer, the leaked energetic proton distributions typically have substantially higher flow velocities along the magnetic field indicate that the leaked energetic proton distributions to contribute to the energetic proton population seen upstream and downstream from the quasi-parallel bow shock. However, their contribution is small compared to the contribution from acceleration of protons at the bow shock because the leaked proton densities are on the order of 10 times smaller than the energetic proton densities typically observed in the vicinity of the quasi-parallel bow shock

Barrier layer engineering: Performance evaluation of E-mode InGaN/AlGaN/GaN HEMT

Science.gov (United States)

Majumdar, Shubhankar; Das, S.; Biswas, D.

2015-08-01

Impact on DC characteristics of InGaN/AlGaN/GaN HEMT due to variation in the hetero-structure parameters i.e. molar fraction of Al and thickness of AlGaN barrier layer is presented in this paper. Gate controllability over the channel is dependent on barrier layer thickness, and molar fraction has an impact on band offset and 2DEG, which further affects the current. HEMT device that is simulated in SILVACO has InGaN cap layer of 2 nm thickness with 15% In molar fraction, variation of Al percentage and thickness of the AlGaN barrier layer are taken as 15-45% and 5-20nm, respectively. A tremendous change in threshold voltage (Vth), maximum transconductance (Gmmax) and subthreshold swing is found due to variation in hetero-structure parameter of barrier layer and the values are typically 1.3-0.1 V, 0.6-0.44 S/mm and 75-135 mV/dec respectively.

Surface layer and bloom dynamics observed with the Prince William Sound Autonomous Profiler

Science.gov (United States)

Campbell, R. W.

2016-02-01

As part of a recent long term monitoring effort, deployments of a WETLabs Autonomous Moored Profiler (AMP) began Prince William Sound (PWS) in 2013. The PWS AMP consists of a positively buoyant instrument frame, with a winch and associated electronics that profiles the frame from a park depth (usually 55 m) to the surface by releasing and retrieving a thin UHMWPE tether; it generally conducts a daily cast and measures temperature, salinity, chlorophyll-a fluorescence, turbidity, and oxygen and nitrate concentrations. Upward and downward looking ADCPs are mounted on a float below the profiler, and an in situ plankton imager is in development and will be installed in 2016. Autonomous profilers are a relatively new technology, and early deployments experienced a number of failures from which valuable lessons may be learned. Nevertheless, an unprecedented time series of the seasonal biogeochemical procession in the surface waters coastal Gulf of Alaska was collected in 2014 and 2015. The northern Gulf of Alaska has experienced a widespread warm anomaly since early 2014, and surface layer temperature anomalies in PWS were strongly positive during winter 2014. The spring bloom observed by the profiler began 2-3 weeks earlier than average, with surface nitrate depleted by late April. Although surface temperatures were still above average in 2015, bloom timing was much later, with a short vigorous bloom in late April and a subsurface bloom in late May that coincided with significant nitrate drawdown. As well as the vernal blooms, wind-driven upwelling events lead to several small productivity pulses that were evident in changes in nitrate and oxygen concentrations, and chlorophyll-a fluorescence. As well as providing a mechanistic understanding of surface layer biogeochemistry, high frequency observations such as these put historical observations in context, and provide new insights into the scales of variability in the annual cycles of the surface ocean in the North

Case series of probable sporadic Creutzfeldt-Jakob disease from Eastern India

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Atanu Biswas

2013-01-01

Full Text Available Background: Creutzfeldt-Jakob disease is a rapidly progressive, fatal, transmissible neurodegenerative disorder caused by prion protein. It is still considered rare in countries like India. This is probably due to nonavailability of autopsy studies in majority of the center. The recent European diagnostic criterion for sporadic CJD (sCJD is useful for making an early diagnosis. Objective: To report a series of patients of probable sCJD from a neurology institute of eastern India. Materials and Methods: Patients of rapidly developing dementia fulfilling the diagnostic criteria for sCJD were included. All were investigated in detail to find out any possible treatable cause including electroencephalography (EEG, magnetic resonance imaging (MRI of brain, and cerebrospinal fluid analysis. Results: A total 10 patients of probable sCJD diagnosed using the European diagnostic criterion between December 2011 and January 2013. The clinical features are consistent with other reported series. While 60% of patients had the classical EEG findings, 100% had typical MRI features. Eight patients died within a mean duration of 4.56 months from the disease onset. Conclusions: The clinical features are similar to other reported series. Our observation raises question about the prevalence of this disease in India which needs more elaborate studies.

Short telomeres: from dyskeratosis congenita to sporadic aplastic anemia and malignancy.

Science.gov (United States)

Gramatges, Maria M; Bertuch, Alison A

2013-12-01

Telomeres are DNA-protein structures that form a protective cap on chromosome ends. As such, they prevent the natural ends of linear chromosomes from being subjected to DNA repair activities that would result in telomere fusion, degradation, or recombination. Both the DNA and protein components of the telomere are required for this essential function, because insufficient telomeric DNA length, loss of the terminal telomeric DNA structure, or deficiency of key telomere-associated factors may elicit a DNA damage response and result in cellular senescence or apoptosis. In the setting of failed checkpoint mechanisms, such DNA-protein defects can also lead to genomic instability through telomere fusions or recombination. Thus, as shown in both model systems and in humans, defects in telomere biology are implicated in cellular and organismal aging as well as in tumorigenesis. Bone marrow failure and malignancy are 2 life-threatening disease manifestations in the inherited telomere biology disorder dyskeratosis congenita. We provide an overview of basic telomere structure and maintenance. We outline the telomere biology defects observed in dyskeratosis congenita, focusing on recent discoveries in this field. Last, we review the evidence of how telomere biology may impact sporadic aplastic anemia and the risk for various cancers. Copyright © 2013 Mosby, Inc. All rights reserved.

Nosocomial transmission of sporadic Creutzfeldt-Jakob disease: results from a risk-based assessment of surgical interventions

DEFF Research Database (Denmark)

de Pedro-Cuesta, Jesús; Mahillo-Fernández, Ignacio; Rábano, Alberto

2011-01-01

Evidence of surgical transmission of sporadic Creutzfeldt-Jakob disease (sCJD) remains debatable in part due to misclassification of exposure levels. In a registry-based case-control study, the authors applied a risk-based classification of surgical interventions to determine the association...

Direct observation of strain in InAs quantum dots and cap layer during molecular beam epitaxial growth using in situ X-ray diffraction

Energy Technology Data Exchange (ETDEWEB)

Shimomura, Kenichi; Ohshita, Yoshio; Kamiya, Itaru, E-mail: kamiya@toyota-ti.ac.jp [Toyota Technological Institute, 2-12-1 Hisakata, Tempaku, Nagoya 468-8511 (Japan); Suzuki, Hidetoshi [Faculty of Engineering, University of Miyazaki, 1-1 Gakuen Kibanadai-nishi, Miyazaki 889-2192 (Japan); Sasaki, Takuo; Takahasi, Masamitu [Quantum Beam Science Center, Japan Atomic Energy Agency, Koto 1-1-1, Sayo-cho, Hyogo 679-5148 (Japan)

2015-11-14

Direct measurements on the growth of InAs quantum dots (QDs) and various cap layers during molecular beam epitaxy are performed by in situ X-ray diffraction (XRD). The evolution of strain induced both in the QDs and cap layers during capping is discussed based on the XRD intensity transients obtained at various lattice constants. Transients with different features are observed from those obtained during InGaAs and GaAs capping. The difference observed is attributed to In-Ga intermixing between the QDs and the cap layer under limited supply of In. Photoluminescence (PL) wavelength can be tuned by controlling the intermixing, which affects both the strain induced in the QDs and the barrier heights. The PL wavelength also varies with the cap layer thickness. A large redshift occurs by reducing the cap thickness. The in situ XRD observation reveals that this is a result of reduced strain. We demonstrate how such information about strain can be applied for designing and preparing novel device structures.

Direct observation of strain in InAs quantum dots and cap layer during molecular beam epitaxial growth using in situ X-ray diffraction

International Nuclear Information System (INIS)

Shimomura, Kenichi; Ohshita, Yoshio; Kamiya, Itaru; Suzuki, Hidetoshi; Sasaki, Takuo; Takahasi, Masamitu

2015-01-01

Direct measurements on the growth of InAs quantum dots (QDs) and various cap layers during molecular beam epitaxy are performed by in situ X-ray diffraction (XRD). The evolution of strain induced both in the QDs and cap layers during capping is discussed based on the XRD intensity transients obtained at various lattice constants. Transients with different features are observed from those obtained during InGaAs and GaAs capping. The difference observed is attributed to In-Ga intermixing between the QDs and the cap layer under limited supply of In. Photoluminescence (PL) wavelength can be tuned by controlling the intermixing, which affects both the strain induced in the QDs and the barrier heights. The PL wavelength also varies with the cap layer thickness. A large redshift occurs by reducing the cap thickness. The in situ XRD observation reveals that this is a result of reduced strain. We demonstrate how such information about strain can be applied for designing and preparing novel device structures

Development of the mesospheric Na layer at 69° N during the Geminids meteor shower 2010

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T. Dunker

2013-01-01

Full Text Available The ECOMA sounding rocket campaign in 2010 was performed to investigate the charge state and number density of meteoric smoke particles during the Geminids meteor shower in December 2010. The ALOMAR Na lidar contributed to the campaign with measurements of sodium number density, temperature and line-of-sight wind between 80 and 110 km altitude over Andøya in northern Norway. This paper investigates a possible connection between the Geminids meteor shower and the mesospheric sodium layer. We compare with data from a meteor radar and from a rocket-borne in situ particle instrument on three days. Our main result is that the sodium column density is smaller during the Geminids meteor shower than the winter average at the same latitude. Moreover, during two of the three years considered, the sodium column density decreased steadily during these three weeks of the year. Both the observed decrease of Na column density by 30% and of meteoric smoke particle column density correlate well with a corresponding decrease of sporadic meteor echoes. We found no correlation between Geminids meteor flux rates and sodium column density, nor between sporadic meteors and Na column density (R = 0.25. In general, we found the Na column density to be at very low values for winter, between 1.8 and 2.6 × 1013 m−2. We detected two meteor trails containing sodium, on 13 December 2010 at 87.1 km and on 19 December 2010 at 84 km. From these meteor trails, we estimate a global meteoric Na flux of 121 kg d−1 and a global total meteoric influx of 20.2 t d−1.

A Risk Prediction Model for Sporadic CRC Based on Routine Lab Results.

Science.gov (United States)

Boursi, Ben; Mamtani, Ronac; Hwang, Wei-Ting; Haynes, Kevin; Yang, Yu-Xiao

2016-07-01

Current risk scores for colorectal cancer (CRC) are based on demographic and behavioral factors and have limited predictive values. To develop a novel risk prediction model for sporadic CRC using clinical and laboratory data in electronic medical records. We conducted a nested case-control study in a UK primary care database. Cases included those with a diagnostic code of CRC, aged 50-85. Each case was matched with four controls using incidence density sampling. CRC predictors were examined using univariate conditional logistic regression. Variables with p value CRC prediction models which included age, sex, height, obesity, ever smoking, alcohol dependence, and previous screening colonoscopy had an AUC of 0.58 (0.57-0.59) with poor goodness of fit. A laboratory-based model including hematocrit, MCV, lymphocytes, and neutrophil-lymphocyte ratio (NLR) had an AUC of 0.76 (0.76-0.77) and a McFadden's R2 of 0.21 with a NRI of 47.6 %. A combined model including sex, hemoglobin, MCV, white blood cells, platelets, NLR, and oral hypoglycemic use had an AUC of 0.80 (0.79-0.81) with a McFadden's R2 of 0.27 and a NRI of 60.7 %. Similar results were shown in an internal validation set. A laboratory-based risk model had good predictive power for sporadic CRC risk.

On the inclusive annihilation of polarized e+e--pair with two observed hadrons

International Nuclear Information System (INIS)

Khachatryan, G.N.; Shakhnazaryan, Yu.G.

1976-01-01

The general consideration of the inclusive annihilation of polarized e + e - -pair with two observed hadrons in final state (e + e - →h 1 h 2 X) is carried out. The annihilation cross section is expressed in terms of five structure functions describing the transition γ*→h 1 h 2 X. The partial widths of the corresponding decay of a virtual photon for different polarizations of the photon are also introduced and the annihilation cross section is written through these widths. The density matrix of the virtual photon and its polarizational multipole moments are given as well

Photoreflectance study of InAs ultrathin layer embedded in Si-delta-doped GaAs/AlGaAs quantum wells

Energy Technology Data Exchange (ETDEWEB)

Dhifallah, I., E-mail: ines.dhifallah@gmail.co [Laboratoire de Photovoltaique, des Semiconducteurs et des Nanostructures, Centre de Recherche et des Technologies de l' energie, BP 95 Hammam-Lif 2050 (Tunisia); Daoudi, M.; Bardaoui, A. [Laboratoire de Photovoltaique, des Semiconducteurs et des Nanostructures, Centre de Recherche et des Technologies de l' energie, BP 95 Hammam-Lif 2050 (Tunisia); Eljani, B. [Unite de recherche sur les Hetero-Epitaxie et Applications, Faculte des Sciences de Monastir (Tunisia); Ouerghi, A. [Laboratoire de Photonique et de Nanostructures, CNRS Route de Nozay 91 46a0, Marcoussis (France); Chtourou, R. [Laboratoire de Photovoltaique, des Semiconducteurs et des Nanostructures, Centre de Recherche et des Technologies de l' energie, BP 95 Hammam-Lif 2050 (Tunisia)

2011-05-15

Photoreflectance and photoluminescence studies were performed to characterize InAs ultrathin layer embedded in Si-delta-doped GaAs/AlGaAs high electron mobility transistors. These structures were grown by Molecular Beam Epitaxy on (1 0 0) oriented GaAs substrates with different silicon-delta-doped layer densities. Interband energy transitions in the InAs ultrathin layer quantum well were observed below the GaAs band gap in the photoreflectance spectra, and assigned to electron-heavy-hole (E{sub e-hh}) and electron-light-hole (E{sub e-lh}) fundamental transitions. These transitions were shifted to lower energy with increasing silicon-{delta}-doping density. This effect is in good agreement with our theoretical results based on a self-consistent solution of the coupled Schroedinger and Poisson equations and was explained by increased escape of photogenerated carriers and enhanced Quantum Confined Stark Effect in the Si-delta-doped InAs/GaAs QW. In the photoreflectance spectra, not only the channel well interband energy transitions were observed, but also features associated with the GaAs and AlGaAs bulk layers located at about 1.427 and 1.8 eV, respectively. By analyzing the Franz-Keldysh Oscillations observed in the spectral characteristics of Si-{delta}-doped samples, we have determined the internal electric field introduced by ionized Si-{delta}-doped centers. We have observed an increase in the electric field in the InAs ultrathin layer with increasing silicon content. The results are explained in terms of doping dependent ionized impurities densities and surface charges. - Research highlights: {yields} Studying HEMTs structures with different silicon doping content. {yields} An increase of the electric field in the InAs layer with increasing Si content. {yields} The interband energy transitions in the HEMTs structures have been obtained from PR. {yields} Experimental and theoretical values of transitions energies were in good agreement.

Photoreflectance study of InAs ultrathin layer embedded in Si-delta-doped GaAs/AlGaAs quantum wells

International Nuclear Information System (INIS)

Dhifallah, I.; Daoudi, M.; Bardaoui, A.; Eljani, B.; Ouerghi, A.; Chtourou, R.

2011-01-01

Photoreflectance and photoluminescence studies were performed to characterize InAs ultrathin layer embedded in Si-delta-doped GaAs/AlGaAs high electron mobility transistors. These structures were grown by Molecular Beam Epitaxy on (1 0 0) oriented GaAs substrates with different silicon-delta-doped layer densities. Interband energy transitions in the InAs ultrathin layer quantum well were observed below the GaAs band gap in the photoreflectance spectra, and assigned to electron-heavy-hole (E e-hh ) and electron-light-hole (E e-lh ) fundamental transitions. These transitions were shifted to lower energy with increasing silicon-δ-doping density. This effect is in good agreement with our theoretical results based on a self-consistent solution of the coupled Schroedinger and Poisson equations and was explained by increased escape of photogenerated carriers and enhanced Quantum Confined Stark Effect in the Si-delta-doped InAs/GaAs QW. In the photoreflectance spectra, not only the channel well interband energy transitions were observed, but also features associated with the GaAs and AlGaAs bulk layers located at about 1.427 and 1.8 eV, respectively. By analyzing the Franz-Keldysh Oscillations observed in the spectral characteristics of Si-δ-doped samples, we have determined the internal electric field introduced by ionized Si-δ-doped centers. We have observed an increase in the electric field in the InAs ultrathin layer with increasing silicon content. The results are explained in terms of doping dependent ionized impurities densities and surface charges. - Research highlights: → Studying HEMTs structures with different silicon doping content. → An increase of the electric field in the InAs layer with increasing Si content. → The interband energy transitions in the HEMTs structures have been obtained from PR. → Experimental and theoretical values of transitions energies were in good agreement.