SPG10 is a rare cause of spastic paraplegia in European families

Background: SPG10 is an autosomal dominant form of hereditary spastic paraplegia (HSP), which is caused by mutations in the neural kinesin heavy chain KIF5A gene, the neuronal motor of fast anterograde axonal transport. Only four mutations have been identified to date.Objective: To determine the frequency of SPG10 in European families with HSP and to specify the SPG10 phenotype.Patients and methods: 80 index patients from families with autosomal dominant HSP were investigated for SPG10 mutations by direct sequencing of the KIF5A motor domain. Additionally, the whole gene was sequenced in 20 of these families.Results: Three novel KIF5A mutations were detected in German families, including one missense mutation (c.759G>T, p.K253N), one in frame deletion (c.768_770delCAA, p.N256del) and one splice site mutation (c.217G>A). Onset of gait disturbance varied from infancy to 30 years of age. All patients presented clinically with pure HSP, but a subclinical sensory--motor neuropathy was detected by neurophysiology studies.Conclusions: SPG10 accounts for approximately 3% of European autosomal dominant HSP families. All mutations affect the motor domain of kinesin and thus most likely impair axonal transport. Clinically, SPG10 is characterised by spastic paraplegia with mostly subclinical peripheral neuropathy

Compression and strength behaviour of viscose/polypropylene nonwoven fabrics

Compression and strength properties of viscose/polypropylene nonwoven fabrics has been studied. Compressionbehavior of the nonwoven samples (sample compressibility, sample thickness loss & sample compressive resilience) havebeen analyzed considering the magnitude of applied pressure, fabric weight, fabric thickness, and the porosity of thesamples. Based on the calculated porosity of the samples, pore compression behavior (pore compressibility, porosity loss &pore compressive resilience) are determined. Equations for the determination of pore compressibility, porosity loss, and porecompressive resilience, are established. Tensile strength and elongation as well as bursting strength and ball traverseelongation are also determined. The results show that the sample compression behavior as well as pore compressionbehavior depend on the magnitude of applied pressure. At the high level of applied pressure, a sample with highercompressibility has the lower sample compressive resilience. Differences in pore compressibility and porosity loss betweeninvestigated samples have also been registered, except in pore compressive resilience. Sample with the higher fabric weight,higher thickness, and lower porosity shows the lower sample compressibility, pore compressibility, sample thickness loss,porosity loss, and tensile elongation, but the higher tensile strength, bursting strength, and ball traverse elongation

Quality of clothing fabrics in terms of their comfort properties

Quality of various clothing woven fabrics with respect to their comfort properties, such as electro-physical properties, air permeability, and compression properties has been studied. Fabrics are produced from cotton and cotton/polyester fibre blends in plain, twill, satin and basket weave. Results show that cotton fabrics have lower values of the volume resistivity, air permeability and compressive resilience but higher values of effective relative dielectric permeability and compressibility as compared to fabrics that have been produced from cotton/PES fibre blends. Regression analysis shows a strong linear correlative relationship between the air permeability and the porosity of the woven fabrics with very high coefficient of linear correlation (0.9807). It is also observed that comfort properties are determined by the structure of woven fabrics (raw material composition, type of weave) as well as by the fabrics surface condition. Findings of the studies have been used for estimating the quality of woven fabrics in terms of their comfort properties by the application of ranking method. It is concluded that the group of cotton fabrics exhibits better quality of comfort as compared to the group of cotton/PES blend fabrics.

The origin of paramagnetic magnetization in field-cooled YBa2Cu3O7 films

Temperature dependences of the magnetic moment have been measured in YBa_2Cu_3O_{7-\delta} thin films over a wide magnetic field range (5 <= H <= 10^4 Oe). In these films a paramagnetic signal known as the paramagnetic Meissner effect has been observed. The experimental data in the films, which have strong pinning and high critical current densities (J_c ~ 2 \times 10^6 A/cm^2 at 77 K), are quantitatively shown to be highly consistent with the theoretical model proposed by Koshelev and Larkin [Phys. Rev. B 52, 13559 (1995)]. This finding indicates that the origin of the paramagnetic effect is ultimately associated with nucleation and inhomogeneous spatial redistribution of magnetic vortices in a sample which is cooled down in a magnetic field. It is also shown that the distribution of vortices is extremely sensitive to the interplay of film properties and the real experimental conditions of the measurements.Comment: RevTex, 8 figure

Aging Effect in Ceramic Superconductors

A three-dimensional lattice of the Josephson junctions with a finite self-conductance is employed to model ceramic superconductors. Using Monte Carlo simulations it is shown that the aging disappears in the strong screening limit. In the weeak screening regime aging is present even at low temperatures. For intermediate values of the self-inductance aging occurs at intermediate temperatures interval but is suppressed entirely at high and low temperatures. Our results are in good agreement with experiments.Comment: 5 pages, 5 eps figures, to appear in Physical Review Letter

Polyphenol Content and Antioxidant Activity of Sour Cherries From Serbia

The aim of this study was to evaluate the content of phenolics: the total phenols (TP), flavonoids (TF), anthocyanins (TA), as well as the total antioxidant\ud capacity (TAC) in three sour cherry cultivars (Prunus cerasus L.) introduced to the southeast Serbia climate conditions. Among the investigated sour cherries,\ud „Oblačinska“ cultivar contained the highest amounts of all groups of phenolics, followed by „Cigančica“ > „Marela“. A significant difference were observed in the phenolic content among different cultivars and growing seasons (p  0.05), and the phenolic compounds were significantly higher in the growing season 2009. The examined cultivars possess a high antioxidant capacity, and all phenolics of highy correlation with TAC. The following compounds were identified and quantified using HPLC-DAD: 4 anthocyanins, the most abundant of which was cyanidin-3-glucoside in “Marela” and “Oblačinska”, and cyanidin-3-glucosylrutinoside in „Cigančica“, and 4 hydroxycinnamic acids, the most abundant of which was neochlorogenic acid in all sour cherry cultivars. The growing and ripening process on the tree of sour cherry cv. „Oblačinska“ was evaluated also. The results showed significant increases in total phenols during the ripening, the total anthocyanins and total antioxidant capacity and 4 quantified anthocyanins, however the neochlorogenic acid decreased during the ripening. The study indicated that the growing and climate conditions in southeast Serbia are convenient for introducing sour cherry cultivars.\u

Techno-functional, textural and sensorial properties of frankfurters as affected by the addition of bee pollen powder

The objective of this study was to determine whether the addition of different pollen powder concentrations (0.5; 1.0 and 1.5 g/100 g) had an influence on techno-functional, textural and sensorial traits of frankfurters. Examining the techno-functional characteristics of pollen, a conclusion was reached that the higher the concentration, the higher the emulsification and better techno-functional properties. Also, FTIR-ATR analysis has shown that specific pollen molecules provided good emulsifying properties of sausages. On the other hand, sensory analysis showed that sausages with the addition of 1.0% and 1.5% of pollen powder have a more pronounced floral odor. Warner-Bratzler shear force test has shown that the incorporation of pollen caused a more stable product throughout sixty days of storage than the control sample. It could be explained by the formation of more protein-protein interactions due to the addition of non-meat proteins in the formulation of frankfurters and obtaining a more stable product than the control one. All things considered, it can be concluded that pollen exhibits good techno-functional properties and could be utilized in the formulation of frankfurters with improved and steady techno-functional properties during two months of refrigerated storage

Paramagnetic Meissner Effect in Multiply-Connected Superconductors

We have measured a paramagnetic Meissner effect in Nb-Al2O3-Nb Josephson junction arrays using a scanning SQUID microscope. The arrays exhibit diamagnetism for some cooling fields and paramagnetism for other cooling fields. The measured mean magnetization is always less than 0.3 flux quantum (in terms of flux per unit cell of the array) for the range of cooling fields investigated. We demonstrate that a new model of magnetic screening, valid for multiply-connected superconductors, reproduces all of the essential features of paramagnetism that we observe and that no exotic mechanism, such as d-wave superconductivity, is needed for paramagnetism.Comment: 4 pages, 3 figures, LaTe

DEMO ion cyclotron heating: Status of ITER-type antenna design

The ITER ICRF system will gain in complexity relative to the existing systems on modern devices, and the same will hold true for DEMO. The accumulated experience can help greatly in designing an ICRF system for DEMO. In this paper the current status of the pre-conceptual design of the DEMO ICRF antenna and some related components is presented. While many aspects strongly resemble the ITER system, in some design solutions we had to take an alternative route to be able to adapt to DEMO specific. One of the key points is the toroidal antenna extent needed for the requested ICRF heating performance, achieved by splitting the antenna in halves, with appropriate installation. Modelling of the so far largest ICRF antenna in RAPLICASOL and associated challenges are presented. Calculation are benchmarked with TOPICA. Results of the analysis of the latest model and an outlook for future steps are given.Comment: Published in Fusion Engineering and Design 165 (2021) 11226

Plan integrisanog monitoringa kvaliteta zemljišta kao indikatora kvaliteta životne sredine u Novom Sadu

U radu je prikazan plan integisanog upravljanja životnom sredinom na primeru kontrole kvaliteta zemljišta u gradu Novom Sadu u Republici Srbiji. S obzirom da ima veoma povoljan geografski položaj, Novi Sad je grad gde se prepliću važni magistralni putevi, ima sve veći priliv stanovništva, kao i brzi razvoj industrije i poljoprivrede. Usled navedenih činjenica postoji i povećano zagađivanje vazduha, vode i zemljišta, kao i generisanje velike količine otpada. Pored parkova i devastiranih područja u okolini industrijskih zona, rasprostranjena poljoprivredna delatnost u prigradskim delovima, naročito je osetljiva na kvalitet zemljišta i obrnuto. U ovoj studiji analizirani su geografska pozicija i geomorfološke karakteristike područja grada, kao i vodeći ekološki problemi. Na osnovu datih činjenica predložen je integrisani monitoring jednog od indikatora kvaliteta životne sredine, kvalitet zemljišta. Predložena je mreža od ukupno 15 senzora za merenje pH vrednosti, vlage i temperature zemljišta na teritoriji grada. Podaci o kvalitetu zemljišta koji se na ovakav način dobijaju u realnom vremenu veoma značajni su za eliminisanje ili ublažavanje potencijalnih nepoželjnih posledica. Zahvaljujući brzom razvoju tehnologije primena senzorskih mreža sve više se koristi u modernim sistemima monitoringa i dostupna je za efikasno i ekonomski isplativo rešavanje problema zaštite životne sredine