Distinct clinical and neuropathological features of G51D SNCA mutation cases compared with SNCA duplication and H50Q mutation

Background: We and others have described the neurodegenerative disorder caused by G51D SNCA mutation which shares characteristics of Parkinson’s disease (PD) and multiple system atrophy (MSA). The objective of this investigation was to extend the description of the clinical and neuropathological hallmarks of G51D mutant SNCA-associated disease by the study of two additional cases from a further G51D SNCA kindred and to compare the features of this group with a SNCA duplication case and a H50Q SNCA mutation case. Results: All three G51D patients were clinically characterised by parkinsonism, dementia, visual hallucinations, autonomic dysfunction and pyramidal signs with variable age at disease onset and levodopa response. The H50Q SNCA mutation case had a clinical picture that mimicked late-onset idiopathic PD with a good and sustained levodopa response. The SNCA duplication case presented with a clinical phenotype of frontotemporal dementia with marked behavioural changes, pyramidal signs, postural hypotension and transiently levodopa responsive parkinsonism. Detailed post-mortem neuropathological analysis was performed in all cases. All three G51D cases had abundant α-synuclein pathology with characteristics of both PD and MSA. These included widespread cortical and subcortical neuronal α-synuclein inclusions together with small numbers of inclusions resembling glial cytoplasmic inclusions (GCIs) in oligodendrocytes. In contrast the H50Q and SNCA duplication cases, had α-synuclein pathology resembling idiopathic PD without GCIs. Phosphorylated α-synuclein was present in all inclusions types in G51D cases but was more restricted in SNCA duplication and H50Q mutation. Inclusions were also immunoreactive for the 5G4 antibody indicating their highly aggregated and likely fibrillar state. Conclusions: Our characterisation of the clinical and neuropathological features of the present small series of G51D SNCA mutation cases should aid the recognition of this clinico-pathological entity. The neuropathological features of these cases consistently share characteristics of PD and MSA and are distinct from PD patients carrying the H50Q or SNCA duplication.

The impact of variable sea ice roughness on changes in Arctic Ocean surface stress: a model study

The Arctic sea ice cover is thinning and retreating, causing changes in surface roughness that in turn modify the momentum flux from the atmosphere through the ice into the ocean. New model simulations comprising variable sea ice drag coefficients for both the air and water interface demonstrate that the heterogeneity in sea ice surface roughness significantly impacts the spatial distribution and trends of ocean surface stress during the last decades. Simulations with constant sea ice drag coefficients as used in most climate models show an increase in annual mean ocean surface stress (0.003 N/m2 per decade, 4.6%) due to the reduction of ice thickness leading to a weakening of the ice and accelerated ice drift. In contrast, with variable drag coefficients our simulations show annual mean ocean surface stress is declining at a rate of -0.002 N/m2 per decade (3.1%) over the period 1980-2013 because of a significant reduction in surface roughness associated with an increasingly thinner and younger sea ice cover. The effectiveness of sea ice in transferring momentum does not only depend on its resistive strength against the wind forcing but is also set by its top and bottom surface roughness varying with ice types and ice conditions. This reveals the need to account for sea ice surface roughness variations in climate simulations in order to correctly represent the implications of sea ice loss under global warming.

Performance of four tomato cultivars, with and without ground cover

The aim of the study was to evaluate the production of four tomato cultivars of determinate growth, in the field, with and without ground cover. The cultivars examined were: 'AP-533', 'AP-529', 'Hypeel 5131 and 'PS-41816', which were cultivated with a spacing of 1 m between rows and 0.4 m between plants. The experimental design utilized was that of subdivided parcels, where parcels were provided with and without a ground cover of black polyethylene, and subparcels consisted of the cultivars; eight repetitions were used. The eight central plants in the parcel were evaluated according to the following characteristics: fresh weight of fruit per plant and number of fruit per plant, separated by size. For the classification of fruit, the same plants were divided into classes based on the diameter of the fruit. The diameter size was as follows: class 1, 3 cm; class II, 4 cm; class III, 4.5 cm; and class IV, 5.5 cm. The results showed that there were no significant differences in production among the cultivars studied. Cultivars 'AP-533' and 'Hypeel 513' demonstrated greater production per plant, when ground cover was used, yielding 1474 and 1404 g/plant, respectively. When black polyethylene ground cover was not used, these cultivars produced 1258 and 1271 g of fruit per plant, respectively. The cultivars 'AP-529' and 'PS41818' showed a greater yield of fruit when cultivated without ground cover, with 1548 and 1663 g/plant, respectively. The cultivar 'Hypeel 513' had the highest percentage of fruit with the largest dimensions. An interaction was appeared between 'AP-529' and ground cover, where the production of this cultivar was lower with black polyethylene ground cover compared to bare ground. It is concluded that there are no significant differences in the cultivation of the tomato hybrids 'AP-533', 'Hypeel 513' and 'PS-41816' with and without ground cover, but that the cultivar 'AP-529' should be planted preferentially in soil without black polyethylene cover.

Black string corrections in variable tension braneworld scenarios

Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)

Variable parameter and double sampling (X)over-bar charts in the presence of correlation: The Markov chain approach

The general assumption under which the (X) over bar chart is designed is that the process mean has a constant in-control value. However, there are situations in which the process mean wanders. When it wanders according to a first-order autoregressive (AR (1)) model, a complex approach involving Markov chains and integral equation methods is used to evaluate the properties of the (X) over bar chart. In this paper, we propose the use of a pure Markov chain approach to study the performance of the (X) over bar chart. The performance of the chat (X) over bar with variable parameters and the (X) over bar with double sampling are compared. (C) 2011 Elsevier B.V. All rights reserved.

Bivariate control charts with double sampling

In this article, we consider the T(2) chart with double sampling to control bivariate processes (BDS chart). During the first stage of the sampling, n(1) items of the sample are inspected and two quality characteristics (x; y) are measured. If the Hotelling statistic T(1)(2) for the mean vector of (x; y) is less than w, the sampling is interrupted. If the Hotelling statistic T(1)(2) is greater than CL(1), where CL(1) > w, the control chart signals an out-of-control condition. If w < T(1)(2) <= CL(1), the sampling goes on to the second stage, where the remaining n(2) items of the sample are inspected and T(2)(2) for the mean vector of the whole sample is computed. During the second stage of the sampling, the control chart signals an out-of-control condition when the statistic T(2)(2) is larger than CL(2). A comparative study shows that the BDS chart detects process disturbances faster than the standard bivariate T(2) chart and the adaptive bivariate T(2) charts with variable sample size and/or variable sampling interval.

Controle estatístico aplicado ao processo de colheita mecanizada de cana-de-açúcar

O avanço da mecanização na colheita da cana-de-açúcar (Saccharum spp.) proporcionou o uso de novas tecnologias e ganho em produtividade para a cultura. O controle da qualidade do processo de colheita da cana-de-açúcar é fundamental para reduzir as perdas. Este trabalho teve o objetivo de avaliar as perdas na colheita mecanizada de cana-de-açúcar, utilizando-as como indicadores de qualidade do processo de colheita. Os dados foram coletados em duas propriedades próximas a Jaboticabal - SP, com a variedade SP80-3280, em 3º e 4º cortes. Caracterizou-se o porte do canavial e, após a colheita, demarcou-se área de 1,5 ha, sendo demarcados 25 pontos, espaçados de 12 x 50 m, quantificando-se as perdas visíveis. Posteriormente, foi aplicado o controle estatístico do processo pela média, que consta de três vezes o desvio-padrão para mais ou para menos, sendo esses os limites superior e inferior de controle, respectivamente. A média das perdas de pedaço solto foi estatisticamente maior do que as médias de perdas em pedaço fixo, cana inteira, cana-ponta e toco. A ocorrência de perdas em rebolo estilhaçado foi menor para o 4º corte em relação ao 3º corte, enquanto as perdas em pedaço fixo e toco foram menores no 3º corte, comparadas às perdas no 4º corte. em cada corte, as médias para as perdas totais estiveram próximas dos valores encontrados na bibliografia. Pedaço solto foi a variável de perdas visíveis com maior percentagem de ocorrência. As perdas demonstraram que a colheita mecanizada não se encontra sob controle estatístico de processo.

Joint (X)over-bar and R charts with two-stage samplings

When joint (X) over bar and R charts are in use, samples of fixed size are regularly taken from the process, and their means and ranges are plotted on the (X) over bar and R charts, respectively. In this article, joint (X) over bar and R charts have been used for monitoring continuous production processes. The sampling is performed, in two stages. During the first stage, one item of the sample is inspected and, depending on the result, the sampling is interrupted if the process is found to be in control; otherwise, it goes on to the second stage, where the remaining sample items are inspected. The two-stage sampling procedure speeds up the detection of process disturbances. The proposed joint (X) over bar and R charts are easier to administer and are more efficient than the joint (X) over bar and R charts with variable sample size where the quality characteristic of interest can be evaluated either by attribute or variable. Copyright (C) 2004 John Wiley Sons, Ltd.

Sn-polymetallic greisen-type deposits associated with late-stage rapakivi granites, Brazil: fluid inclusion and stable isotope characteristics

Tin-polymetallic greisen-type deposits in the Itu Rapakivi Province and Rondonia Tin Province, Brazil are associated with late-stage rapakivi fluorine-rich peraluminous alkali-feldspar granites. These granites contain topaz and/or muscovite or zinnwaldite and have geochemical characteristics comparable to the low-P sub-type topaz-bearing granites. Stockworks and veins are common in Oriente Novo (Rondonia Tin Province) and Correas (Itu Rapakivi Province) deposits, but in the Santa Barbara deposit (Rondonia Tin Province) a preserved cupola with associated bed-like greisen is predominant. The contrasting mineralization styles reflect different depths of formation, spatial relationship to tin granites, and different wall rock/fluid proportions. The deposits contain a similar rare-metal suite that includes Sri (+/-W, +/-Ta, +/-Nb), and base-metal suite (Zn-Cu-Pb) is present only in Correas deposit. The early fluid inclusions of the Correas and Oriente Novo deposits are (1) low to moderate-salinity (0-19 wt.% NaCl eq.) CO2-bearing aqueous fluids homogenizing at 245-450 degreesC, and (2) aqueous solutions with low CO2, low to moderate salinity (0-14 wt.% NaCl eq.), which homogenize between 100 and 340 T. In the Santa Barbara deposit, the early inclusions are represented by (1) low-salinity (5-12 wt.% NaCl eq.) aqueous fluids with variable CO2 contents, homogenizing at 340 to 390 T, and (2) low-salinity (0-3 wt.% NaCl eq.) aqueous fluid inclusions, which homogenize at 320380 degreesC. Cassiterite, wolframite, columbite-tantalite, scheelite, and sulfide assemblages accompany these fluids. The late fluid in the Oriente Novo and Correas deposit was a low-salinity (0-6 wt.% NaCl eq.) CO2-free aqueous solution, which homogenizes at (100-260 degreesC) and characterizes the sulfide fluorite-sericite association in the Correas deposit. The late fluid in the Santa Barbara deposit has lower salinity (0-3 wt.% NaCl eq.) and characterizes the late-barren-quartz, muscovite and kaolinite veins. Oxygen isotope thermometry coupled with fluid inclusion data suggest hydrothermal activity at 240-450 degreesC, and 1,0-2.6 kbar fluid pressure at Correas and Oriente Novo. The hydrogen isotope composition of breccia-greisen, stockwork, and vein fluids (delta(18)O quartz from 9.9parts per thousand to 10.9parts per thousand, deltaDH(2)O from 4.13parts per thousand to 6.95parts per thousand) is consistent with a fluid that was in equilibrium with granite at temperatures from 450 to 240 degreesC. In the Santa Barbara deposit, the inferred temperatures for quartz-pods and bed-like greisens are much higher (570 and 500 degreesC, respectively), and that for the cassiterite-quartz-veins is 415 degreesC. The oxygen and hydrogen isotope composition of greisen and quartz-pods fluids (delta(18)O(qtz-H2O)=5.5-6.1parts per thousand) indicate that the fluid equilibrated with the albite granite, consistent with a magmatic origin. The values for mica (delta(18)O(mica-H2O)=33-9.8parts per thousand) suggest mixing with meteoric water. Late muscovite veins (delta(18)O(qtz-H2O)=-6.4parts per thousand) and late quartz (delta(18)O(mica-H2O)=-3.8parts per thousand) indicate involvement of a meteoric fluid. Overall, the stable isotope and fluid inclusion data imply three fluid types: (1) an early orthomagmatic fluid, which equilibrated with granite; (2) a mixed orthomagmatic-meteoric fluid; and (3) a late hydrothermal meteoric fluid. The first two were responsible for cassiterite, wolframite, and minor coluChange in the redox conditions related to mixing-of magmatic and meteoric fluids favored important sulfide mineralization in the Correas deposit. (C) 2004 Elsevier B.V. All rights reserved.

Self-Structuring of Lamellar Bridged Silsesquioxanes with Long Side Spacers

Diurea cross-linked bridged silsesquioxanes (BSs) C(10)C(11)C(10) derived from organosilane precursors, including decylene chains as side spacers and alkylene chains with variable length as central spacers (EtO)(3)Si- (CH(2))(10)-Y(CH(2))(n)-Y-(CH(2))(10)-Si(OEt)(3) (n = 7, 9-12; Y = urea group and Et = ethyl), have been synthesized through the combination of self-directed assembly and an acid-catalyzed sol gel route involving the addition of dimethylsulfoxide (DMSO) and a large excess of water. This new family of hybrids has enabled us to conclude that the length of the side spacers plays a unique role in the structuring of alkylene-based BSs, although their morphology remains unaffected. All the samples adopt a lamellar structure. While the alkylene chains are totally disordered in the case of the C(10)C(7)C(10) sample, a variable proportion of all-trans and gauche conformers exists in the materials with longer central spacers. The highest degree of structuring occurs for n = 9. The inclusion of decylene instead of propylene chains as side spacers leads to the formation of a stronger hydrogen-bonded urea-urea array as evidenced by two dimensional correlation Fourier transform infrared spectroscopic analysis. The emission spectra and emission quantum yields of the C(10)C(n)C(10) Cm materials are similar to those reported for diurea cross-linked alkylene-based BSs incorporating propylene chains as side spacers and prepared under different experimental conditions. The emission of the C(10)C(n)C(10) hybrids is ascribed to the overlap of two distinct components that occur within the urea cross-linkages and within the siliceous nanodomains. Time-resolved photoluminescence spectroscopy has provided evidence that the average distance between the siliceous domains and the urea cross-links is similar in the C(10)C(n)C(10) BSs and in oxyethylene-based hybrid analogues incorporating propylene chains as side spacers (diureasils), an indication that the longer side chains in the former materials adopt gauche conformations. It has also allowed us to demonstrate for the first time that the emission features of the urea-related component of the emission of alkylene-based BSs depend critically on the length of the side spacers.

The ultrastructure of the intestinal epithelium in Oreochromis niloticus on a diet with different concentrations of zinc

The effects of diets with variable zinc levels on the midgut epithelial cells were studied in Oreochromis niloticus L. One hundred and twenty fry of tilapia were apportioned into 4 experimental groups (I, II, III and IV groups), with 30 fish in each treatment, 5 replicate aquaria per treatment containing 6 fish each. The animals of the 4 groups were fed with isonitrogenous (30% crude protein) and isoenergetic (3000 Kcal/Kg of digestible energy) diets with increasing quantities of zinc (44.59; 149.17; 309.93; 599.67 mg Zn/kg of diet), twice a day, for 93 days. Three fish from each group were sacrificed at 36, 66 and 93 days and samples of midgut were removed for ultrastructural analysis. After 93 days of treatment, 3 animals of each experimental group were used for the analysis of zinc concentration by atomic absorption spectrophotometry. The comparative relative index (CRI) revealed that the animals in groups II, III and IV contained, respectively, 1.99%, 34.67% and 22.78% more zinc than the mean concentration in animals from group I. The ultrastructural analysis showed enterocytes with swelling of smooth surfaced endoplasmic reticulum and dilated mitochondria with variable matrix rarefaction and cristae number reduction in the fish exposed to 599.67 mg Zn/Kg of diet at 66 and 93 days of treatment.

Progression of liver fibrosis in blood donors infected with hepatitis C virus

Background/Aims. Chronic hepatitis by HCV is progressive towards cirrhosis, with variable rate. We evaluated the rate of fibrosis progression (RFP), risk factors associated with advanced fibrosis (F3 and F4), and estimated the evolution time to cirrhosis. Methods. We transversely selected 142 blood donors infected only with HCV, with a known route of infection, submitted to liver biopsy at admission. RFP= ratio between stage of fibrosis (METAVIR)/estimated duration of infection in years. Non-parametric tests and logistic regression analysis, with significance level of 5% were used. Results. Median RFP was 0.086 U/year (0.05 - 0.142). Ten patients had F4 and 25 had F3. Median RFP values were significantly different (p=0.001) from one age group at contamination to the others and ALT and AST levels. There were no differences in the expected evolution to cirrhosis between intermediate fibrosers (F2) and the rapid fibrosers (F3 and F4). The independent variables associated with advanced fibrosis were ALT (OR 7.2) and GGT (OR 6.4) and age at inclusion (OR 1.12). Conclusion. This study suggests that RFP is extremely variable, it is exponential with age, and mainly influenced by host characteristics, especially age at contamination and possibly ethnical group. These asymptomatic patients had high percentage of fibrosis F2, F3 and F4.

Synthetic control charts with two-stage sampling for monitoring bivariate processes

In this article, we consider the synthetic control chart with two-stage sampling (SyTS chart) to control bivariate processes. During the first stage, one item of the sample is inspected and two correlated quality characteristics (x;y) are measured. If the Hotelling statistic T1 2 for these individual observations of (x;y) is lower than a specified value UCL 1 the sampling is interrupted. Otherwise, the sampling goes on to the second stage, where the remaining items are inspected and the Hotelling statistic T2 2 for the sample means of (x;y) is computed. When the statistic T2 2 is larger than a specified value UCL2, the sample is classified as nonconforming. According to the synthetic control chart procedure, the signal is based on the number of conforming samples between two neighbor nonconforming samples. The proposed chart detects process disturbances faster than the bivariate charts with variable sample size and it is from the practical viewpoint more convenient to administer.

Evolutionary dynamics of 5S rDNA location in acridid grasshoppers and its relationship with H3 histone gene and 45S rDNA location

We analyze the chromosomal location of 5S rDNA clusters in 29 species of grasshoppers belonging to the family Acrididae. There was extensive variation among species for the number and location of 5S rDNA sites. Out of 148 sites detected, 75% were proximally located, 21.6% were interstitial, and only 3.4% were distal. The number of 5S rDNA sites per species varied from a single chromosome pair (in six species) to all chromosome pairs (in five species), with a range of intermediate situations. Thirteen chromosomes from eight species carried two 5S rDNA clusters. At intraspecific level, differences among populations were detected in Eyprepocnemis plorans, and some heteromorphisms have also been observed in some species. Double FISH for 5S rDNA and H3 histone gene DNA, performed on 17 of these 29 species, revealed that both markers are sometimes placed in a same chromosome but at different location, whereas they appeared to co-localize in five species (Calliptamus barbarus, Heteracris adpersa, Aiolopus strepens, Oedipoda charpentieri and O. coerulescens). Double fiber-FISH in A. strepens and O. coerulescens showed that the two DNAs are closely interspersed with variable relative amounts of both classes of DNA. Finally, no correlation was observed between the number of 5S and 45S rDNA clusters in 23 species where this information was available. These results are discussed in the light of possible mechanisms of spread that led to the extensive variation in the number of clusters observed for both rDNA types in acridid grasshoppers. © 2011 Springer Science+Business Media B.V.

Model to design drip hose lateral line using two spacing between emitters

The use of non-pressure compensating drip hose in horticultural and annual cycle fruits is growing in Brazil. In this case, the challenge for designers is getting longer lateral lines with high values of uniformity. The objective of this study was to develop a model to design longer lateral lines using non-pressure compensating drip hose. Using the developed model, the hypotheses to be evaluated were: a) the use of two different spacing between emitters in the same lateral line allows longer length; b) it is possible to get longer lateral lines using high values of pressure variation in the lateral lines since the distribution uniformity stays below allowable limits. A computer program was developed in Delphi based on the model developed and it is able to design lateral lines in level using non-pressure compensating drip hose. The input data are: desired distribution uniformity (DU); initial and final pressure in the lateral line; coefficients of relationship between emitter discharge and pressure head; hose internal diameter; pipe cross-sectional area with the dripper; and roughness coefficient for the Hazen-Williams equation. The program allows calculate the lateral line length with three possibilities: selecting two spacing between emitters and defining the exchange point; using two pre-established spacing between emitters and calculating the length of each section with different spacing; using one emitter spacing. Results showed that the use of two sections with different spacing between drippers in the lateral line didn't allow longer length but got better uniformity when compared with lateral line with one spacing between emitters. The adoption of two spacing increased the flow rate per meter in the final section which represented approximately 80% of the lateral line total length and this justifies their use. The software allowed DU above 90% with pressure head variation of 40% and the use of two spacing between emitters. The developed model/software showed to be accurate, easy to handle and useful for lateral line design using non-pressure compensating drip hose.