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Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) represent a disease continuum with common genetic causes and molecular pathology. We recently identified mutations in the T-cell restricted intracellular antigen-1 (TIA1) protein as a cause of ALS +/− FTD. TIA1 is an RNA-binding protein containing a low complexity domain (LCD) that promotes the assembly of membrane-less organelles, such as stress granules (SG). Whole exome sequencing of two family members with fALS/FTD revealed a novel missense mutation in the TIA1 LCD (P362L). Subsequent screening identified five more TIA1 mutations in six additional ALS patients, but none in controls. All mutation carriers presented with weakness, behavioral abnormalities or language impairments and had a final diagnosis of ALS +/− FTD. Autopsy on five TIA1 mutation carriers showed widespread neurodegeneration with TDP-43 pathology. Round eosinophilic inclusions in lower motor neurons were a consistent feature. Cellular assays revealed abnormal SG dynamics in the presence of TIA1 mutations. In summary, missense mutations in the LCD of TIA1 are a newly recognized cause of ALS/FTD with TDP-43 pathology and strengthen the role of RNA metabolism in the pathogenesis in this disease.
Although Saturn's broad A, B, and C rings epitomize the concept of “planetary rings” in the minds of most people, much of our detailed knowledge of ring dynamics has come from the investigation of smaller-scale features such as density and bending waves, the numerous narrow gaps and their embedded ringlets, and the sharp edges which often demarcate various ring regions. In the case of Uranus, almost all of the ring mass is in the form of narrow rings. Narrow ringlets and gaps, and their associated sharp edges (including those of broad rings) form the subject of this chapter, along with the dynamical theories their study has spawned and the puzzles that continue to surround them.
Examples of several narrow gaps and ringlets in Saturn's rings, as well as the very prominent sharp outer edge of the B ring, are shown in Figure 11.1, from French et al. (2016b). Here one can see a total of eight narrow gaps in the region known as the Cassini Division, ranging in width from 5 km to 360 km, as well as four narrow ringlets. At least six more narrow gaps are found in the outer A ring and in the C ring, while three additional narrow ringlets occur in the C ring. The present chapter will cover all of these features, as well as the ten narrow Uranian rings. Recently a pair of narrow, dense rings has been discovered around the centaur object, Chariklo (Braga-Ribas et al., 2014). These are discussed separately in Chapter 7. We also do not discuss the more tenuous and dusty Jovian and Neptunian ring systems, nor the dusty ringlets found at Saturn and Uranus, all of which are covered in Chapter 12. The complex and unique F ring is described in Chapter 13.
We begin with a short overview of the relevant observations and their limitations in Section 11.2, before reviewing the kinematics, systematic width variations and internal structure of narrow ringlets in Section 11.3. In Section 11.4 we discuss the gaps in Saturn's rings, including searches for any embedded satellites. Section 11.5 deals with individual ringlet and gap edges, especially those that are controlled by resonances with external satellites and those that show evidence for local perturbations by unseen, embedded objects.
A marginal ice-contact lake at Miage glacier, Mont Blanc, Italian Alps, has been studied to reconstruct changes in lake area. Historical sources, comprising sketches, maps, photographs and scientific surveys, have been supplemented by recent field surveys. These include surveys of glacier surface velocity (which varied along the glacier tongue from 70 ma–1 in the upper part to about 6 ma–1 close to the snout, consistent with data in the literature, showing that velocity rates have remained constant during the last 40 years), volumetric ice-cliff loss (–92 000±180m3 in 2002–03), lake temperature and bathymetry, and qualitative observation of calving events, crevassing, and meltwater production. Results indicate that the lake has been stable for the last half-century following a period of enlargement due to ice-marginal retreat. The lake hydrology is complex, with possible reversals of englacial water flow causing infrequent emptying episodes. The debris cover on the glacier and ice-cliff surfaces seems to have played an important role in the ice-cliff evolution and the calving phenomena; calving is driven by undercutting at the water-line aided by the opening of water- and debris-filled crevasses in the glacier surface.
An almost inertial celestial reference frame based upon extragalactic sources is in the process of being established. This reference frame is to be global with a minimum density of one source/100 square degrees. The source positions will be based upon radio observations and will allow optical reference frames to be related to this frame at the 0.03 arc second level.
The quasar 3C273 lies at a declination of +2°. Maps made with northern hemisphere VLBI Networks clearly show superluminal motion, but the north-south resolution is poor and details cannot be seen. We have now made two observations at 10.7 GHz with the Itapetinga Radio Observatory in Brazil, and one at 5.0 GHz using the Hartebeesthoek Observatory in South Africa along with the European and U.S. VLBI Networks (Biretta et al. 1985).
On hill farms where breeding cattle are kept there are advantages in retaining until the following spring at least a proportion of the surplus calves. The ability to winter them presents wider scope in choice of markets, while the sale of calves in spring, in addition to the dung produced, can provide a greater profit margin if wintering costs are minimised. Grass used efficiently in autumn offers an opportunity to reduce the requirement of the relatively expensive hay, silage, other crops and purchased feed. The value of extended grazing throughout autumn and winter months has been demonstrated by Smith (1953-4) for Blackface ewe hoggs, and for store stock and in-calf heifers by Hughes (1954) and Corbett (1954). In the case of calves, however, the period when late grass can be utilised depends on the continuance of moderate temperatures and availability of shelter from wind and rain, and therefore in most seasons will be relatively short. With these considerations in mind it was decided to compare the performance of two groups of weaned calves, one of which was housed and fed in courts immediately after weaning, while the other remained on grass for several weeks prior to court feeding.
Periodicity and new properties of the frequency curve. Bruno Hanisch uses the method of autocorrelation introduced by W. Pollack in geophysics for the discovery of periods in the frequency series of sunspots from 1794 to 1925. Dividing the whole interval into three sections he finds an eleven- and an eight-year period common to the three sections, whereas other periods found in the three sections differ widely from each other. The new method gives for the length of the main period II-8 years for the interval 1880 to 1925. This result agrees strikingly with the revolution period of Jupiter (Gerlands Beiträge zur Geophysik, 46, 1935).
The celestial positions of extragalactic radio sources may be determined to a precision of less than a milliarcsecond. Further, since these sources are believed to be at great distances from the galaxy, little or no proper motion is expected on scales of order a milliarcsecond. Therefore a reference frame based on the positions of carefully selected sources so that display compact radiation on scales less than a milliarcsecond will noticeably improve the precision of present celestial reference frames. If the radio objects making up the reference frame also emit radiation at optical wavelengths, and assuming the optical/radio radiation is coincident, the radio frame can update the optical frame to the accuracy of the individual optical positions.
Observations of speckles in the scattering disk of the Vela pulsar are presented and speckle techniques for studying and circumventing scattering of radio waves by the turbulent interstellar plasma are discussed. The speckle pattern contains, in a hologrammatic fashion, complete information on the structure of the radio source as well as the distribution of the scattering material. Speckle observations of interstellar scattering of radio waves are difficult because of their characteristically short timescales (≈seconds) and narrow bandwidths (≈kHz). Here, we present first observations, taken at 13 cm wavelength with elements of the SHEVE VLBI network, of speckles in interstellar scattering.
The Deep Space Network (DSN) [operated by JPL under contract to the National Aeronautics and Space Administration] is implementing a Very Long Baseline Interferometry (VLBI) capability at DSS 63 (Spain), DSS 14 (California, USA), and DSS 43 (Australia) to support the navigation requirements of planetary space missions. The early development work for this system has already demonstrated the capability of measuring UT1 with a formal accuracy as low as 0.6 msec with only 6 hours of data. Further, a radio astrometric catalog of approximately 45 sources whose positions are known to better than has been constructed. In addition to these measurements, this paper describes the characteristics and anticipated performance of the complete VLBI system being implemented within the DSN for operational use in mid-1979. In particular, one of the capabilities of this system will be the measurement of UT1 and polar motion at weekly intervals. Although the navigation accuracy requirement is only 50 cm for the Voyager mission, this system should be capable of delivering UT1 and polar motion determinations with decimeter accuracy if it is operated at maximum performance. An additional requirement of this operational system is that it have the capability of providing these results within 24 hours of the actual observations.
To identify factors that affected well-being among British embassy staff based in Japan after the 2011 earthquake, tsunami, and nuclear meltdown.
In-depth qualitative interviews were conducted with 36 members of staff 8 to 9 months after the earthquake.
Participants described their crisis work as stressful, exciting, and something of which they were proud. Aside from disaster-specific stressors, factors identified as stressful included unclear roles, handing over work to new personnel, being assigned to office-based work, feeling that work was not immediately beneficial to the public, not taking good-quality breaks, and difficulties with relatives. The radiation risk provoked mixed feelings, with most participants being reassured by contact with senior scientists.
Interventions to safeguard the well-being of personnel during crisis work must consider the impact of a broad range of stressors.(Disaster Med Public Health Preparedness. 2014;0:1-7)
Data were extracted from the case records of UK patients admitted with laboratory-confirmed influenza A(H1N1)pdm09. White and non-White patients were characterized by age, sex, socioeconomic status, pandemic wave and indicators of pre-morbid health status. Logistic regression examined differences by ethnicity in patient characteristics, care pathway and clinical outcomes; multivariable models controlled for potential confounders. Whites (n = 630) and non-Whites (n = 510) differed by age, socioeconomic status, pandemic wave of admission, pregnancy, recorded obesity, previous and current smoking, and presence of chronic obstructive pulmonary disease. After adjustment for a priori confounders non-Whites were less likely to have received pre-admission antibiotics [adjusted odds ratio (aOR) 0·43, 95% confidence interval (CI) 0·28–0·68, P < 0·001) but more likely to receive antiviral drugs as in-patients (aOR 1·53, 95% CI 1·08–2·18, P = 0·018). However, there were no significant differences by ethnicity in delayed admission, severity at presentation for admission, or likelihood of severe outcome.
Understanding the nutritional demands on serving military personnel is critical to inform training schedules and dietary provision. Troops deployed to Afghanistan face austere living and working environments. Observations from the military and those reported in the British and US media indicated possible physical degradation of personnel deployed to Afghanistan. Therefore, the present study aimed to investigate the changes in body composition and nutritional status of military personnel deployed to Afghanistan and how these were related to physical fitness. In a cohort of British Royal Marines (n 249) deployed to Afghanistan for 6 months, body size and body composition were estimated from body mass, height, girth and skinfold measurements. Energy intake (EI) was estimated from food diaries and energy expenditure measured using the doubly labelled water method in a representative subgroup. Strength and aerobic fitness were assessed. The mean body mass of volunteers decreased over the first half of the deployment ( − 4·6 (sd 3·7) %), predominately reflecting fat loss. Body mass partially recovered (mean +2·2 (sd 2·9) %) between the mid- and post-deployment periods (P< 0·05). Daily EI (mean 10 590 (sd 3339) kJ) was significantly lower than the estimated daily energy expenditure (mean 15 167 (sd 1883) kJ) measured in a subgroup of volunteers. However, despite the body mass loss, aerobic fitness and strength were well maintained. Nutritional provision for British military personnel in Afghanistan appeared sufficient to maintain physical capability and micronutrient status, but providing appropriate nutrition in harsh operational environments must remain a priority.
Bacteriophytochromes (Bphs) are red-light photoreceptors found in photosynthetic and non-photosynthetic bacteria that have been engineered into infrared fluorescent protein markers. Bphs are composed of a photosensory module that is covalently linked to an effector/regulatory module, usually a histidine kinase (HK) domain. Light-induced, global structural changes are proposed to originate within the covalently attached biliverdin chromophore, a linear tetrapyrrole, and propagate through the protein. Bphs undergo reversible photoconversion between two distinct red and far-red light absorbing states, denoted Pr and Pfr respectively. For most Bphs, Pr is the dark-adapted state. The energy dissipated during Pr/Pfr photoconversion is proposed to directly impact the infrared fluorescence quantum yield. At this time, only structures of three different Bphs have been published, all of truncated proteins in their respective dark-adapted states. We have utilized scanning probe microscopy (SPM) to investigate the structure of intact Bphs in the light-adapted state in order to gain new insight into the mechanism of photoconversion and fluorescence. Scanning tunneling microscopy (STM) analysis of a pair of Bphs from photosynthetic bacterium R. palustris, RpBphP2 (P2) and RpBphP3 (P3) in their light-adapted states is presented in these proceedings. The concentration of the depositing protein has a key role in the molecular arrangements observed on the highly-ordered pyrolytic graphite (HOPG) surface. For example, at a high protein concentration, a hexagonal lattice of Bphs is observed by STM on a HOPG surface. Upon dilution, the photoreceptors self-organize into fiber-like structures on the surface. In these fibers, the dimer interface and the individual domains of the Bphs can be assigned and directly compared to a structural model of the intact, full-length proteins. In summary, SPM has potential to be an effective method for gaining new insight into Bph structure and dynamics.
Geopolymers are made by adding aluminosilicates to concentrated alkali solutions for dissolution and subsequent polymerization to form a solid. They are amorphous to semicrystalline three dimensional aluminosilicate networks. Although they have been used in several applications their widespread use is restricted due to lack of long term durability studies and detailed scientific understanding. Three important tools for the study of geopolymers are transmission electron microscopy (TEM), solid state magic angle spinning nuclear magnetic resonance (MAS NMR) and infra red (IR) spectroscopy.
Cs and Sr are two of the most difficult radionuclides to immobilize and are therefore suitable elements to study in assessing geopolymers as matrices for immobilization of radioactive wastes. In this study Cs or Sr was added to geopolymer samples prepared using fly ash precursors. A commercial metakaolinite geopolymer was studied for comparison.
The geopolymers were mainly amorphous as shown by TEM, whether they were made from fly ash or metakaolinite. In the fly ash geopolymer, Cs preferentially inhabited the amorphous phase over the minor crystalline phases, whereas Sr was shared in both. The MAS NMR showed that Cs is held mostly in the geopolymer structure for both fly ash and metakaolinite geopolymers. The IR spectra showed a slight shift in antisymmetric Si-O-Al stretch band to a lower wavenumber for the fly ash geopolymer, which implies that more Al is incorporated in this geopolymer structure than in the metakaolinite geopolymer.
The FCC superstructure phase diagram for Cu-Pd is calculated by means of isotropic effective interactions derived from an ab initio KKR-CPA electronic structure calculation. We prove the existence of a region in which one-dimensional long period superstructures are stable. This region is composed of several single phase fields in which the modulation period is constant. The value of the modulation period is in close agreement with results from High Resolution Electron Microscopy. Adding oxygen to Cu-Pd is found to sharpen the antiphase boundaries. This experiment enables us to clarify the role of the Fermi surface in these alloys.
We have performed calculations of the electronic structure of the random substitutional bcc Fe1-xCrx alloys, using the spin-polarized, self-consistent Korringa, Kohn and Rostoker coherent potential approximation (KKR-CPA) method. This is a first principles method based on a local spin density approximation for electron exchange and correlation energy. For the iron-rich alloys, we find that the average moment decreases linearly with Cr concentration, although the individual moments show a different concentration dependence and the Cr moment is anti-parallel to the Fe moment. This system is similar to Fe1-xVx system, although some details are different.
H3O+- and NH4 + -A12O3 polycrystals have been conductivity-optimised by eliminating metastable grain-boundary phases (NaA1O2 and MgAl2O4) and texturing the microstructure. The grain-boundary conductivity is considerably improved (up to 5x10-5(Ω-cm)-i) from 10–6 (Ω-cm)-l). The dc-field-assisted, H3O+ ion exchange process for K+/Na+-β"-A12O3 in HAC has been accelerated by cyclic switching of the dc field (cycle = 3secs) or injecting ultrasonic energy. The formeroubles the steady-state ion-exchange current to 12 μA by dispersing the HAC/electrolyte, interfacial, H3O+ polarisation layer. The ion-exchange currentefficiency is not improved from 0.15. The ultrasound (23 MHz) markedly shortens the time to complete ion-exchange (400 hrs.vs. > 700 hrs), more than doubles the final ion-exchange current (360 μA.vs. 150 μA) and improves the ion-exchange current efficiency to 0.24. These results are interpreted in terms of ionic “activation ”in the electrolyte. Evidence is presented of a mixed-cation-effect in H3O+-K-β"-AI2O3.