Novel TBK1 truncating mutation in a familial amyotrophic lateral sclerosis patient of Chinese origin

Missense and frameshift mutations in TRAF family member-associated NF-kappa-B activator (TANK)-binding kinase 1 (TBK1) have been reported in European sporadic and familial amyotrophic lateral sclerosis (ALS) cohorts. To assess the role of TBK1 in ALS patient cohorts of wider ancestry, we have analyzed whole-exome sequence data from an Australian cohort of familial ALS (FALS) patients and controls. We identified a novel TBK1 deletion (c.1197delC) in a FALS patient of Chinese origin. This frameshift mutation (p.L399fs) likely results in a truncated protein that lacks functional domains required for adapter protein binding, as well as protein activation and structural integrity. No novel or reported TBK1 mutations were identified in FALS patients of European ancestry. This is the first report of a TBK1 mutation in an ALS patient of Asian origin and indicates that sequence variations in TBK1 are a rare cause of FALS in Australia. © 2015 Elsevier Inc.

Social and environmental reporting practices of organisations operating in, or sourcing products from, a developing country : evidence from Bangladesh

This research consists of a broad study in three parts of the social and environmental reporting practices of organisations operating in or sourcing products from a developing country, in this case Bangladesh. The first part of this study explores the social and environmental disclosure practices of the Bangladesh Garment Manufacturers and Exporters Association (BGMEA), the body responsible for organising the activities of 4,200 entities involved in the export of garments from Bangladesh. By way of interview, this part documents the opinions of numerous senior executives from the BGMEA with regard to any changes in the degree of social and environmental pressures since 1985. Utilising a complementary theoretical perspective that includes legitimacy theory, stakeholder theory and institutional theory this part then performs an analysis of the BGMEA's annual reports (1987-2005), t o explore the link between the perceived pressures and changes entailed therein and the social and environmental disclosure practices of the BGMEA across the period of analysis. The results show that the disclosure practices of BGMEA appear to be directly driven by the changing expectations of multinational buying companies- the group deemed to be the most powerful stakeholder group. This section is the first known study to interview managers from a large organisation in a developing country about shifting stakeholder expectations and then to link these changing expectations to annual report disclosures across an extended period of analysis. The findings then directly lead to the second major part of this thesis which investigates the social and environmental disclosure practices of two major multinational buying companies: Nike and H&M. Adopting a joint consideration of legitimacy theory and media agenda setting theory, this second part investigates the linkage between negative media attention and positive corporate social and environmental disclosures over a 19 year period. The results support the view that for those industry-related social and environmental issues that attract the greatest amount of negative media attention, these companies react by providing positive social and environmental disclosures. The results were particularly significant in relation to labour practices in developing countries-the issue that attracts the greatest amount of negative media attention for the companies in question. While the second part demonstrates that the media influences particular disclosure practices, the third part of the thesis shows what drives the media. Based on the speculation provided in the second part, the third part tests the proposition that the media is an important ally of NGOs in their quest to influence change in corporate accountabilities. Through the use of interviews, the results of this part of the study provide evidence to support previously untested perspectives about NGOs' utilisation of the m edia. The results reveal that NGOs use the media because the media is responsible for creating real changes in the operations and disclosure policies of organisations sourcing products from Bangladesh. The various pressures impacting the activities of organisations operating in or sourcing products from developing countries constitutes a fascinating area of investigation, and it is hoped that this study will motivate further research in this area.

Captain Cook's country dance

James Cook was born into a working class family and rose to become a national hero, one of the greatest explorers of all time. He was celebrated in the popular culture through dance, music, song, and theatre. Today little is remembered of these highly esteemed works, although they remained well-known in the nineteenth-century.

Quality certification and company performance: The newly developed country experience

This study investigates the impacts of ISO 9001:2008 certification on companies in Malaysia. Data were collected from CEOs and Managers through a questionnaire survey. A multivariate analysis and SPSS macro were used as statistical techniques to assess the effects of ISO 9001 certification. Results of the study indicate that ISO 9001 certified companies were having significantly greater benefits and financial performance compared to non-certified companies. However, no significant direct relationship between ISO 9001 certification and company's financial performance was found. A further investigation revealed that financial performance is actually directly related to quality and local and international business performance, which are significantly influenced by ISO 9001 certification. Therefore quality and business performances are involved in the mediational process between the financial performance of companies and ISO 9001 certification. The novelty of this research lies in the establishment of, for the first time, high level statistical relationship between ISO 9001 certification, its mediating factors and financial performance of companies.

My country

The film is an ode to the history of Indigenous peoples and the meaning and spirit of belonging. Inspired by verbal and documented accounts of Aboriginal experiences from the history of colonialism, MY COUNTRY is the touching story of the lasting impact of one such experience on an elderly Aboriginal man. Triggered by a work of art and its interpretation, his story is a journey through time and cultural dislocation.

Geomolecular dating and the origin of placental mammals

In modern evolutionary divergence analysis the role of geological information extends beyond providing a timescale, to informing molecular rate variation across the tree. Here I consider the implications of this development. I use fossil calibrations to test the accuracy of models of molecular rate evolution for placental mammals, and reveal substantial misspecification associated with life history rate correlates. Adding further calibrations to reduce dating errors at specific nodes unfortunately tends to transfer underlying rate errors to adjacent branches. Thus, tight calibration across the tree is vital to buffer against rate model errors. I argue that this must include allowing maximum bounds to be tight when good fossil records permit, otherwise divergences deep in the tree will tend to be inflated by the interaction of rate errors and asymmetric confidence in minimum and maximum bounds. In the case of placental mammals I sought to reduce the potential for transferring calibration and rate model errors across the tree by focusing on well-supported calibrations with appropriately conservative maximum bounds. The resulting divergence estimates are younger than others published recently, and provide the long-anticipated molecular signature for the placental mammal radiation observed in the fossil record near the 66 Ma Cretaceous–Paleogene extinction event.

The Palu Metamorphic Complex, NW Sulawesi, Indonesia: Origin and evolution of a young metamorphic terrane with links to Gondwana and Sundaland

The Palu Metamorphic Complex (PMC) is exposed in a late Cenozoic orogenic belt in NW Sulawesi, Indonesia. It is a composite terrane comprising a gneiss unit of Gondwana origin, a schist unit composed of meta-sediments deposited along the SE Sundaland margin in the Late Cretaceous and Early Tertiary, and one or more slivers of amphibolite with oceanic crust characteristics. The gneiss unit forms part of the West Sulawesi block underlying the northern and central sections of the Western Sulawesi Province. The presence of Late Triassic granitoids and recycled Proterozoic zircons in this unit combined with its isotopic signature suggests that the West Sulawesi block has its origin in the New Guinea margin from which it rifted in the late Mesozoic. It docked with Sundaland sometime during the Late Cretaceous. U–Th–Pb dating results for monazite suggest that another continental fragment may have collided with the Sundaland margin in the earliest Miocene. High-pressure (HP) and ultrahigh-pressure (UHP) rocks (granulite, peridotite, eclogite) are found as tectonic slices within the PMC, mostly along the Palu–Koro Fault Zone, a major strike-slip fault that cuts the complex. Mineralogical and textural features suggest that some of these rocks resided at depths of 60–120 km during a part of their histories. Thermochronological data (U–Th–Pb zircon and 40Ar/39Ar) from the metamorphic rocks indicate a latest Miocene to mid-Pliocene metamorphic event, which was accompanied by widespread granitoid magmatism and took place in an extensional tectonic setting. It caused recrystallization of, and new overgrowths on, pre-existing zircon crystals, and produced andalusite–cordierite–sillimanite–staurolite assemblages in pelitic protoliths, indicating HT–LP (Buchan-type) metamorphism. The PMC was exhumed as a core complex at moderate rates (c. 0.7–1.0 mm/yr) accompanied by rapid cooling in the Plio-Pleistocene. Some of the UHP rocks were transported to the surface at significantly higher rates (⩾16 mm/yr). The results of our study do not support recent plate tectonic reconstructions that propose a NW Australia margin origin for the West Sulawesi block (e.g. Hall et al., 2009).