Background: Biallelic variants in POLR1C are associated with POLR3-related leukodystrophy (POLR3-HLD), or 4H leukodystrophy (Hypomyelination, Hypodontia, Hypogonadotropic Hypogonadism), and Treacher Collins syndrome (TCS). The clinical spectrum of POLR3-HLD caused by variants in this gene has not been described. Methods: A cross-sectional observational study involving 25 centers worldwide was conducted between 2016 and 2018. The clinical, radiologic and molecular features of 23 unreported and previously reported cases of POLR3-HLD caused by POLR1C variants were reviewed. Results: Most participants presented between birth and age 6 years with motor difficulties. Neurological deterioration was seen during childhood, suggesting a more severe phenotype than previously described. The dental, ocular and endocrine features often seen in POLR3-HLD were not invariably present. Five patients (22%) had a combination of hypomyelinating leukodystrophy and abnormal craniofacial development, including one individual with clear TCS features. Several cases did not exhibit all the typical radiologic characteristics of POLR3-HLD. A total of 29 different pathogenic variants in POLR1C were identified, including 13 new disease-causing variants. Conclusions: Based on the largest cohort of patients to date, these results suggest novel characteristics of POLR1C-related disorder, with a spectrum of clinical involvement characterized by hypomyelinating leukodystrophy with or without abnormal craniofacial development reminiscent of TCS.

It is a truth universally acknowledged that every ambitious twenty-first century trade agreement is in want of a chapter on electronic commerce. One of the most politically sensitive and technically challenging issues is personal privacy, including cross-border transfer of information by electronic means, use and location of computing facilities, and personal information protection. States are learning to solve the problem of state responsibility for something that does not respect their borders while still allowing twenty-first century commerce to develop. A comparison of the Canada–European Union Comprehensive Economic and Trade Agreement (CETA) and the Trans-Pacific Partnership (TPP) allows us to see the evolution of the issues thought necessary for an e-commerce chapter, since both include Canada, and to see the differing priorities of the US and the EU, since they are each signatory to one of the agreements, but not of the other. I conclude by seeking generalizations about why we see a mix of aspirational and obligatory provisions in free trade agreements. I suggest that the reasons are that governments are learning how to work with each other in a new domain, and learning about the trade implications of these issues.

Human skeletal remains hold a wealth of information about past life-ways, but their documentation and recovery from archaeological contexts is challenging. Four challenges face bioarchaeological field work: poor skeletal preservation; complex mortuary traditions; sub-par excavation conditions; and excavation time limits. Poor preservation often prevents the collection of metric data from skeletons. This project tested whether a bioarchaeologist with basic photography skills and excavation equipment could address these challenges using photogrammetry. Photogrammetry was employed at the ancient Maya sites of Say Kah and Chan Chich, both located in northern Belize, to document human skeletal remains and their archaeological contexts. Steps are provided for implementing photogrammetry in the field, as well as addressing challenges for using the technique during burial documentation. This project produced only one adequate 3-D model and no metric data could be collected. Overall, photogrammetry seems to be a promising method for bioarchaeological research because it is low-cost, effective, and fairly straightforward to learn. The technique was faster than traditional line drawing, but sub-par excavation conditions and dark, small spaces hindered the creation of useful models. Photogrammetry provides an excellent alternative to traditional documentation addressing the challenges of poor skeletal preservation and holding potential for unraveling complex mortuary traditions.

Objectives: To adequately monitor the course of cognitive functioning in persons with moderate to severe dementia, relevant cognitive tests for the advanced dementia stages are needed. We examined the ability of a test developed for the advanced dementia stages, the Severe Impairment Battery Short version (SIB-S), to measure cognitive change over time. Second, we examined type of memory impairment measured with the SIB-S in different dementia stages. Methods: Participants were institutionalized persons with moderate to severe dementia (N = 217). The SIB-S was administered at 6-month intervals during a 2-year period. Dementia severity at baseline was classified according to Global Deterioration Scale criteria. We used mixed models to evaluate the course of SIB-S total and domain scores, and whether dementia stage at baseline affected these courses. Results: SIB-S total scores declined significantly over time, and the course of decline differed significantly between dementia stages at baseline. Persons with moderately severe dementia declined faster in mean SIB-S total scores than persons with moderate or severe dementia. Between persons with moderate and moderately severe dementia, there was only a difference in the rate of decline of semantic items, but not episodic and non-semantic items. Conclusions: Although modest floor and slight ceiling effects were noted in severe and milder cases, respectively, the SIB-S proved to be one of few available adequate measures of cognitive change in institutionalized persons with moderate to severe dementia. (JINS, 2019, 25, 204–214)

We used all 167 typhoon warnings issued by the Taiwanese Central Weather Bureau from 1958–2006 to assess the long-term effect of cyclone disturbance on vascular epiphytes. Tracks and eyes of past typhoons were plotted as circles with radii of Beaufort scale 7 and 10, and the frequency of each cohort in 1-km2 grid cells was calculated. The presence of vascular epiphytes in the same grid cells was predicted using species distribution models (SDMs). First, we used herbarium specimens and other sources to compile a comprehensive georeferenced vascular epiphyte database that contained 39084 records in 331 species. Next, we assigned each epiphyte record to a cell in the same 1-km2 grid as above. Finally, we used SDMs (MaXent), based on 30 environmental variables except typhoon frequency, to predict the potential presence of each species in the grid cells. For our analysis we only considered cells east of the central mountain ridge where typhoons hit with full force. After elimination of rare species and species that could not be validated in the SDMs, we were left with 156 epiphyte species in 10725 1-km2 cells. The number of projected species in the cells was 36.5 on average, varying between two and 82 species. Correlation analyses showed that, over time, typhoons led to a decrease in epiphyte richness at Beaufort scale 7 and 10 (Pearson's r = −0.07 and −0.08 respectively). Ferns, orchids, hemiepiphytes and dicotyledons generally showed the same pattern, except hemiepiphytes that showed a positive correlation at B7 (Pearson's r = 0.15). A partial canonical correspondence ordination analysis showed that, independent of temperature- and rainfall-related variables, Beaufort scale 7 and 10 typhoons also had significant influence on the species composition of the vascular epiphyte communities in the landscape. We recommend in situ monitoring of epiphytes over a long period to corroborate the suggestion from this indirect study that typhoons have a long-term effect on the distribution of epiphytes in Taiwan.

We present the first data release of the SkyMapper Southern Survey, a hemispheric survey carried out with the SkyMapper Telescope at Siding Spring Observatory in Australia. Here, we present the survey strategy, data processing, catalogue construction, and database schema. The first data release dataset includes over 66 000 images from the Shallow Survey component, covering an area of 17 200 deg2 in all six SkyMapper passbands uvgriz, while the full area covered by any passband exceeds 20 000 deg2. The catalogues contain over 285 million unique astrophysical objects, complete to roughly 18 mag in all bands. We compare our griz point-source photometry with Pan-STARRS1 first data release and note an RMS scatter of 2%. The internal reproducibility of SkyMapper photometry is on the order of 1%. Astrometric precision is better than 0.2 arcsec based on comparison with Gaia first data release. We describe the end-user database, through which data are presented to the world community, and provide some illustrative science queries.

From a 45ks Chandra observation of V42G Oph we have obtained high-resolution X-ray spectra at moderate signal-to-noise, and a, good quality, uninterrupted lightcurve. The spectra are reasonably fit with a cooling flow model, similar to EX Hya and U Gem. Our analysis of the Chandra and additional X-ray/optical lightcurves reveals a persistent modulation at 4.2 hr from 1988 to 2003, likely the white dwarf spin period indicating an intermediate polar nature for V426 Oph.

The discovery of the first electromagnetic counterpart to a gravitational wave signal has generated follow-up observations by over 50 facilities world-wide, ushering in the new era of multi-messenger astronomy. In this paper, we present follow-up observations of the gravitational wave event GW170817 and its electromagnetic counterpart SSS17a/DLT17ck (IAU label AT2017gfo) by 14 Australian telescopes and partner observatories as part of Australian-based and Australian-led research programs. We report early- to late-time multi-wavelength observations, including optical imaging and spectroscopy, mid-infrared imaging, radio imaging, and searches for fast radio bursts. Our optical spectra reveal that the transient source emission cooled from approximately 6 400 K to 2 100 K over a 7-d period and produced no significant optical emission lines. The spectral profiles, cooling rate, and photometric light curves are consistent with the expected outburst and subsequent processes of a binary neutron star merger. Star formation in the host galaxy probably ceased at least a Gyr ago, although there is evidence for a galaxy merger. Binary pulsars with short (100 Myr) decay times are therefore unlikely progenitors, but pulsars like PSR B1534+12 with its 2.7 Gyr coalescence time could produce such a merger. The displacement (~2.2 kpc) of the binary star system from the centre of the main galaxy is not unusual for stars in the host galaxy or stars originating in the merging galaxy, and therefore any constraints on the kick velocity imparted to the progenitor are poor.

A QTL (TM-QTL) identified on ovine chromosome 18 (Walling et al., 2004), which increases loin muscle depth by 4-8% in UK Texel sheep, is of interest for the sheep industry as a potential means to increase carcass value. Since the contribution of Texel genes to the UK slaughter generation is generally through use of Texel sires to produce crossbred slaughter lambs (e.g. Texel x Mule lambs), it is necessary to verify the effects of the TM-QTL on loin muscularity and other carcass traits in such crossbred progeny of Texel sires before explotiation of the TM-QTL in commercial sheep populations.

The Taipan galaxy survey (hereafter simply ‘Taipan’) is a multi-object spectroscopic survey starting in 2017 that will cover 2π steradians over the southern sky (δ ≲ 10°, |b| ≳ 10°), and obtain optical spectra for about two million galaxies out to z < 0.4. Taipan will use the newly refurbished 1.2-m UK Schmidt Telescope at Siding Spring Observatory with the new TAIPAN instrument, which includes an innovative ‘Starbugs’ positioning system capable of rapidly and simultaneously deploying up to 150 spectroscopic fibres (and up to 300 with a proposed upgrade) over the 6° diameter focal plane, and a purpose-built spectrograph operating in the range from 370 to 870 nm with resolving power R ≳ 2000. The main scientific goals of Taipan are (i) to measure the distance scale of the Universe (primarily governed by the local expansion rate, H 0) to 1% precision, and the growth rate of structure to 5%; (ii) to make the most extensive map yet constructed of the total mass distribution and motions in the local Universe, using peculiar velocities based on improved Fundamental Plane distances, which will enable sensitive tests of gravitational physics; and (iii) to deliver a legacy sample of low-redshift galaxies as a unique laboratory for studying galaxy evolution as a function of dark matter halo and stellar mass and environment. The final survey, which will be completed within 5 yrs, will consist of a complete magnitude-limited sample (i ⩽ 17) of about 1.2 × 106 galaxies supplemented by an extension to higher redshifts and fainter magnitudes (i ⩽ 18.1) of a luminous red galaxy sample of about 0.8 × 106 galaxies. Observations and data processing will be carried out remotely and in a fully automated way, using a purpose-built automated ‘virtual observer’ software and an automated data reduction pipeline. The Taipan survey is deliberately designed to maximise its legacy value by complementing and enhancing current and planned surveys of the southern sky at wavelengths from the optical to the radio; it will become the primary redshift and optical spectroscopic reference catalogue for the local extragalactic Universe in the southern sky for the coming decade.

State-owned enterprises (SOEs) are a major force in the Chinese economy and a growing presence in international trade and investment. The challenge to the WTO legal regime is commercial, given the size of SOEs and their share of Chinese output, and political, given worries that trade and investment by SOEs may be driven by public policy goals. And both challenges may be exacerbated by the murky world of Chinese SOEs. In this article, I first review whether Chinese SOEs are a problem for the WTO, and whether more sunshine on their operations might be a useful discipline. I then ask what we know about SOEs inside the WTO, including in the Trade Policy Review Mechanism. Since the answer is, not much, I consider whether mega-regional trade negotiations offer a better approach. My answer being negative, I finally consider whether an attempt to negotiate a WTO Reference Paper on SOEs might help. I conclude that transparency is likely to be a better discipline on the spillovers associated with SOEs than a search for binding rules, while also helping everyone better understand the efficiency effects.

We carried out a spectroscopic and photometric study of the 8 presently known B[e] -stars of the Magellanic Clouds (MC), i. e. R4 and R50 of the SMC, R66, R82, R126, Hen S12, Hen S22 and Hen S134 of the LMC. These stars are characterized by the following typical properties: a) strong Balmer emission lines, frequently with P Cygni profiles, b) permitted and forbidden emission lines predominantly of Fe II, [Fe II], [O I] etc., c) strong IR excess due to circumstellar dust. Photospheric absorption lines are usually weak or even not detectable.

We present an atlas of high dispersion spectra of 24 of the brightest peculiar emission-line stars of the Magellanic Clouds.

Our spectra cover the wavelength range from 3600 to 4900 Å. They have been obtained from 1970 to 1984 with the coudé spectrograph of the ESO 1.52 m telescope at La Silla, Chile. The spectral resolution is 0.4 Å for most of the spectra and 0.2 Å for the very brightest stars. Up to 11 spectra are available for one star. In addition, we have done UBVRIJHK(LM) photometry at several epochs of all stars of our sample.

We present coordinated spectroscopic and photometric observations obtained during the present bright phase of the luminous variable star S Dor in the LMC. High resolution spectrograms in the satellite UV and in the visual range were obtained with IUE and with CASPEC (attached to the ESO 3.6-m telescope), respectively. Moreover, photometric UBVRIJHKL observations were carried out.

The SkyMapper 1.3 m telescope at Siding Spring Observatory has now begun regular operations. Alongside the Southern Sky Survey, a comprehensive digital survey of the entire southern sky, SkyMapper will carry out a search for supernovae and other transients. The search strategy, covering a total footprint area of ~2 000 deg2 with a cadence of ⩽5 d, is optimised for discovery and follow-up of low-redshift type Ia supernovae to constrain cosmic expansion and peculiar velocities. We describe the search operations and infrastructure, including a parallelised software pipeline to discover variable objects in difference imaging; simulations of the performance of the survey over its lifetime; public access to discovered transients; and some first results from the Science Verification data.

Lichen element content is a reliable indicator for relative air pollution load in research and monitoring programmes requiring both efficiency and representation of many sites. We tested the value of costly rigorous field and handling protocols for sample element analysis using five lichen species. No relaxation of rigour was supported; four relaxed protocols generated data significantly different from rigorous protocols for many of the 20 validated elements. Minimally restrictive site selection criteria gave quality data from 86% of 81 permanent plots in northern Midwest USA; more restrictive criteria would likely reduce indicator reliability. Use of trained non-specialist field collectors was supported when target species choice considers the lichen community context. Evernia mesomorpha, Flavoparmelia caperata and Physcia aipolia/stellaris were successful target species. Non-specialists were less successful at distinguishing Parmelia sulcata and Punctelia rudecta from lookalikes, leading to few samples and some poor quality data.

The history and iconography of Swiss stained glass dating between the 16th and 18th centuries are well studied. However, the chemical and morphological characteristics of the glass and glass paints, particularly the nature of the raw materials, the provenance of the glass, and the technology used to produce it are less well understood. In this paper, we studied two sets of samples from stained-glass panels attributed to Switzerland, which date from the 16th to 17th centuries: the first set comes from Pena National Palace collection, the second from Vitrocentre Romont. The aims were to identify the materials used in the production of the glass, to find out more about their production origin and to characterize the glass paints. Both glass and the glass paints were analysed by particle-induced X-ray emission; the paints were additionally analysed by scanning electron microscopy–electron-dispersive X-ray spectroscopy (SEM-EDS). The results show that the glass from both sets was probably produced in the same region and that wood ash was used as a fluxing agent. Different recipes have been used to make the blue enamels. However, the cobalt ore used as a coloring agent in all of the blue enamels came from the mining district in Schneeberg, Germany.