Classification, diagnosis and potential mechanisms in Pontocerebellar Hypoplasia

Pontocerebellar Hypoplasia (PCH) is group of very rare, inherited progressive neurodegenerative disorders with prenatal onset. Up to now seven different subtypes have been reported (PCH1-7). The incidence of each subtype is unknown. All subtypes share common characteristics, including hypoplasia/atrophy of cerebellum and pons, progressive microcephaly, and variable cerebral involvement. Patients have severe cognitive and motor handicaps and seizures are often reported. Treatment is only symptomatic and prognosis is poor, as most patients die during infancy or childhood. The genetic basis of different subtypes has been elucidated, which makes prenatal testing possible in families with mutations. Mutations in three tRNA splicing endonuclease subunit genes were found to be responsible for PCH2, PCH4 and PCH5. Mutations in the nuclear encoded mitochondrial arginyl- tRNA synthetase gene underlie PCH6. The tRNA splicing endonuclease, the mitochondrial arginyl- tRNA synthetase and the vaccinia related kinase1 are mutated in the minority of PCH1 cases. These genes are involved in essential processes in protein synthesis in general and tRNA processing in particular. In this review we describe the neuroradiological, neuropathological, clinical and genetic features of the different PCH subtypes and we report on in vitro and in vivo studies on the tRNA splicing endonuclease and mitochondrial arginyl-tRNA synthetase and discuss their relation to pontocerebellar hypoplasia

Crystallographic structure of ultrathin Fe films on Cu(100)

We report bcc-like crystal structures in 2-4 ML Fe films grown on fcc Cu(100) using scanning tunneling microscopy. The local bcc structure provides a straightforward explanation for their frequently reported outstanding magnetic properties, i.e., ferromagnetic ordering in all layers with a Curie temperature above 300 K. The non-pseudomorphic structure, which becomes pseudomorphic above 4 ML film thickness is unexpected in terms of conventional rules of thin film growth and stresses the importance of finite thickness effects in ferromagnetic ultrathin films.Comment: 4 pages, 3 figures, RevTeX/LaTeX2.0

Excited States of Ladder-type Poly-p-phenylene Oligomers

Ground state properties and excited states of ladder-type paraphenylene oligomers are calculated applying semiempirical methods for up to eleven phenylene rings. The results are in qualitative agreement with experimental data. A new scheme to interpret the excited states is developed which reveals the excitonic nature of the excited states. The electron-hole pair of the S1-state has a mean distance of approximately 4 Angstroem.Comment: 24 pages, 21 figure

Лингвостилистическая организация дискурсивной стратегии продвижения университета в сопоставительном аспекте (на основе анализа сайтов российского и китайского вузов)

Объект исследования - университетский дискурс. Цель работы — выявление особенностей лингвостилистической организации дискурсивной стратегии продвижения на сайтах вузов РФ и КНР. В процессе исследования проводились дискурсивный, лингвистический, стилистический, сопоставительный анализ коммуникативной стратегии продвижения вузов. В результате исследования были выявлены лингвистические и стилистические особенности оформления жанров сайтов вузов, реализующих стратегию продвижения, проведен сопоставительный анализ, выработаны рекомендации по переводу. Область применения: переводческая практика переводов и разработки внешних версий сайтов вузов. Экономическая эффективность/значимость работы заключается в предоставлении рекомендаций по переводу сайтов в аспекте продвижения вуза.Object of research: university discourse. Aim of research is to determine linguistic and stylistic features of promotion as a discourse strategy conducted on websites of Russian and Chinese universities. The following activities were carried out inn the course of the research: discourse analysis, linguistic, stylistic and comparative analysis of the communication strategy of universities promotion. Research results: linguistic and stylistic features of universities websites genres that use communication strategy of promotion were determined, comparative analysis was carried out, translation recommendations were provided. Appliance scope: translational practice of translation and design of universities foreign language versions

Climate evolution across the Mid-Brunhes transition

The Mid-Brunhes Transition (MBT) began ∼ 430 ka with an increase in the amplitude of the 100 kyr climate cycles of the past 800 000 years. The MBT has been identified in ice-core records, which indicate interglaciations became warmer with higher atmospheric CO2 levels after the MBT, and benthic oxygen isotope (δ18O) records, which suggest that post-MBT interglaciations had higher sea levels and warmer temperatures than pre-MBT interglaciations. It remains unclear, however, whether the MBT was a globally synchronous phenomenon that included other components of the climate system. Here, we further characterize changes in the climate system across the MBT through statistical analyses of ice-core and δ18O records as well as sea-surface temperature, benthic carbon isotope, and dust accumulation records. Our results demonstrate that the MBT was a global event with a significant increase in climate variance in most components of the climate system assessed here. However, our results indicate that the onset of high-amplitude variability in temperature, atmospheric CO2, and sea level at ∼430 ka was preceded by changes in the carbon cycle, ice sheets, and monsoon strength during Marine Isotope Stage (MIS) 14 and MIS 13

The balloon-borne large-aperture submillimeter telescope for polarimetry: BLAST-Pol

The Balloon-borne Large Aperture Submillimeter Telescope for Polarimetry (BLAST-Pol) is a suborbital mapping experiment designed to study the role played by magnetic fields in the star formation process. BLAST-Pol is the reconstructed BLAST telescope, with the addition of linear polarization capability. Using a 1.8 m Cassegrain telescope, BLAST-Pol images the sky onto a focal plane that consists of 280 bolometric detectors in three arrays, observing simultaneously at 250, 350, and 500 um. The diffraction-limited optical system provides a resolution of 30'' at 250 um. The polarimeter consists of photolithographic polarizing grids mounted in front of each bolometer/detector array. A rotating 4 K achromatic half-wave plate provides additional polarization modulation. With its unprecedented mapping speed and resolution, BLAST-Pol will produce three-color polarization maps for a large number of molecular clouds. The instrument provides a much needed bridge in spatial coverage between larger-scale, coarse resolution surveys and narrow field of view, and high resolution observations of substructure within molecular cloud cores. The first science flight will be from McMurdo Station, Antarctica in December 2010.Comment: 14 pages, 9 figures Submitted to SPIE Astronomical Telescopes and Instrumentation Conference 201

Diffuse-interface model for rapid phase transformations in nonequilibrium systems

A thermodynamic approach to rapid phase transformations within a diffuse interface in a binary system is developed. Assuming an extended set of independent thermodynamic variables formed by the union of the classic set of slow variables and the space of fast variables, we introduce finiteness of the heat and solute diffusive propagation at the finite speed of the interface advancing. To describe the transformation within the diffuse interface, we use the phase-field model which allows us to follow the steep but smooth change of phases within the width of diffuse interface. The governing equations of the phase-field model are derived for the hyperbolic model, model with memory, and for a model of nonlinear evolution of transformation within the diffuse-interface. The consistency of the model is proved by the condition of positive entropy production and by the outcomes of the fluctuation-dissipation theorem. A comparison with the existing sharp-interface and diffuse-interface versions of the model is given.Comment: 15 pages, regular article submitted to Physical Review

Alkane and alkanethiol passivation of halogenated Ge nanowires

The ambient stability and surface coverage of halogen (Cl, Br, and I) passivated germanium nanowires were investigated by X-ray photoelectron and X-ray photoelectron emission spectroscopy. After exposure to air for 24 h, the stability of the halogen-terminated Ge nanowire surfaces toward reoxidation was found to improve with the increasing size of the halogen atoms, i.e., I > Br > Cl. Halogen termination was effective in removing the native Ge oxide (GeOx) and could also be utilized for further functionalization. Functionalization of the halogenated Ge nanowires was investigated using alkyl Grignard reagents and alkanethiols. The stability of the alkyl and alkanethiol passivation layers from the different halogen-terminated surfaces was investigated by X-ray photoelectron spectroscopy and attenuated total reflectance infrared spectroscopy. Alkanethiol functionalized nanowires showed greater resistance against reoxidation of the Ge surface compared to alkyl functionalization when exposed to ambient conditions for 1 week