Geographical interdependence, international trade and economic dynamics: the Chinese and German solar energy industries

The trajectories of the German and Chinese photovoltaic industries differ significantly yet are strongly interdependent. Germany has seen a rapid growth in market demand and a strong increase in production, especially in the less developed eastern half of the country. Chinese growth has been export driven. These contrasting trajectories reflect the roles of market creation, investment and credit and the drivers of innovation and competitiveness. Consequent differences in competiveness have generated major trade disputes

Hydrogen-like nitrogen radio line from hot interstellar and warm-hot intergalactic gas

Hyperfine structure lines of highly-charged ions may open a new window in observations of hot rarefied astrophysical plasmas. In this paper we discuss spectral lines of isotopes and ions abundant at temperatures 10^5-10^7 K, characteristic for warm-hot intergalactic medium, hot interstellar medium, starburst galaxies, their superwinds and young supernova remnants. Observations of these lines will allow to study bulk and turbulent motions of the observed target and will broaden the information about the gas ionization state, chemical and isotopic composition. The most prospective is the line of the major nitrogen isotope having wavelength 5.65 mm (Sunyaev and Churazov 1084). Wavelength of this line is well-suited for observation of objects at z=0.15-0.6 when it is redshifted to 6.5-9 mm spectral band widely-used in ground-based radio observations, and, for example, for z>=1.3, when the line can be observed in 1.3 cm band and at lower frequencies. Modern and future radio telescopes and interferometers are able to observe the absorption by 14-N VII in the warm-hot intergalactic medium at redshifts above z=0.15 in spectra of brightest mm-band sources. Sub-millimeter emission lines of several most abundant isotopes having hyperfine splitting might also be detected in spectra of young supernova remnants.Comment: 12 pages, 5 figures, accepted by Astronomy Letters; v3: details added; error fixe

CHIASM, the human brain albinism and achiasma MRI dataset

We describe a collection of T1-, diffusion- and functional T2*-weighted magnetic resonance imaging data from human individuals with albinism and achiasma. This repository can be used as a test-bed to develop and validate tractography methods like diffusion-signal modeling and fiber tracking as well as to investigate the properties of the human visual system in individuals with congenital abnormalities. The MRI data is provided together with tools and files allowing for its preprocessing and analysis, along with the data derivatives such as manually curated masks and regions of interest for performing tractography

An Instrument For Investigation of the Cosmic Microwave Background Radiation at Intermediate Angular Scales

We describe an off-axis microwave telescope for observations of the anisotropy in the cosmic microwave background (CMB) radiation on angular scales between 0.5 deg and 3 deg. The receiver utilizes cryogenic high-electron-mobility transistor (HEMT) amplifiers and detects the total power in multiple 3 GHz wide channels. Both frequency and polarization information are recorded allowing discrimination between CMB radiation and potential foreground sources and allowing checks for systematic effects. The instrumental radiometric offset is small (~1 mK). Data are taken by rapidly sampling while sweeping the beam many beamwidths across the sky. After detection, a spatio-temporal filter is formed in software which optimizes the sensitivity in a multipole band in the presence of atmospheric fluctuations. Observations were made from Saskatoon, Saskatchewan (SK), Canada during the winter of 1993 with six channels between 27.6 and 34.0 GHz, in 1994 with twelve channels between 27.6 and 44.1 GHz, and in 1995 with six channels between 38.2 and 44.1 GHz. The performance of the instrument and assessment of the atmospheric noise at this site are discussed.Comment: latex file is called inst.tex. 30 pages with 14 Postscript figures. Uses aas2pp4.sty (included). Submitted to Ap

Unusual multisystemic involvement and a novel BAG3 mutation revealed by NGS screening in a large cohort of myofibrillar myopathies

Myofibrillar myopathies (MFM) are a group of phenotypically and genetically heterogeneous neuromuscular disorders, which are characterized by protein aggregations in muscle fibres and can be associated with multisystemic involvement.Methods We screened a large cohort of 38 index patients with MFM for mutations in the nine thus far known causative genes using Sanger and next generation sequencing (NGS). We studied the clinical and histopathological characteristics in 38 index patients and five additional relatives (n = 43) and particularly focused on the associated multisystemic symptoms.Results We identified 14 heterozygous mutations (diagnostic yield of 37%), among them the novel p.Pro209Gln mutation in the BAG3 gene, which was associated with onset in adulthood, a mild phenotype and an axonal sensorimotor polyneuropathy, in the absence of giant axons at the nerve biopsy. We revealed several novel clinical phenotypes and unusual multisystemic presentations with previously described mutations: hearing impairment with a FLNC mutation, dysphonia with a mutation in DES and the first patient with a FLNC mutation presenting respiratory insufficiency as the initial symptom. Moreover, we described for the first time respiratory insufficiency occurring in a patient with the p.Gly154Ser mutation in CRYAB. Interestingly, we detected a polyneuropathy in 28% of the MFM patients, including a BAG3 and a MYOT case, and hearing impairment in 13%, including one patient with a FLNC mutation and two with mutations in the DES gene. In four index patients with a mutation in one of the MFM genes, typical histological findings were only identified at the ultrastructural level (29%).Conclusions We conclude that extraskeletal symptoms frequently occur in MFM, particularly cardiac and respiratory involvement, polyneuropathy and/or deafness. BAG3 mutations should be considered even in cases with a mild phenotype or an adult onset. We identified a genetic defect in one of the known genes in less than half of the MFM patients, indicating that more causative genes are still to be found. Next generation sequencing techniques should be helpful in achieving this aim