The molecular basis of copper-transport diseases

Copper (Cu) is a potentially toxic yet essential element. Menkes disease, a copper deficiency disorder, and Wilson disease, a copper toxicosis condition, are two human genetic disorders, caused by mutations of two closely related Cu-transporting ATPases. Both molecules efflux copper from cells. Quite diverse clinical phenotypes are produced by different mutations of these two Cu-transporting proteins. The understanding of copper homeostasis has become increasingly important in clinical medicine as the metal could be involved in the pathogenesis of some important neurological disorders such as Alzheimer's disease, motor neurone diseases and prion diseases.

Molecular and cellular aspects of copper transport in developing mammals

Copper is an essential trace element that requires tightly regulated homeostatic mechanisms to ensure adequate supplies without any toxic effects because of the ability of the metal ion to catalyze the formation of free radicals. The Cu-ATPases, ATP7A and ATP7B, play an important role in the physiological regulation of copper. Adequate supplies of copper are particularly important in developing animals, and in humans this is illustrated by mutations of ATP7A that cause the copper deficiency condition Menkes disease, which is fatal in early childhood. In contrast, mutations in ATP7B result in the genetic toxicosis, Wilson disease. We propose that the physiological regulation of copper is accomplished mainly by the intracellular copper-regulated trafficking of the Cu-ATPases. This process allows the overall copper status in the body to be maintained when levels of copper in the diet alter. A study of the defects in mouse models of Menkes and Wilson diseases has demonstrated that both ATPases play an important role in supplying copper to the developing fetus and neonate

Four by two theory of non-profit museum governance

As plainly illustrated by the collapse of Enron in the US and HIH in Australia, the world of organisational governance is complicated. In response to ongoing trustee tensions, legislative reforms have been adapted to encourage organisational performance. However, limited theoretical foundation exists regarding how trustee dynamics are forced to adjust within non-profit museums. The trustee’s world requires insights from different mindsets to be synthesised into a whole. The purpose of this article is to examine the research conducted on governance and apply it to the non-profit museum. The article contends that most research on governance has been conducted in the for-profit arena, with little robust empirical research having been conducted on non-profit governance. It identifies concerns with the thrust of articles published as they restrict new theory development. The article provides a four by two theory of non-profit museum governance, that is characterised by close interaction with the research published and application to the non-profit museum. It concludes by demonstrating the increased performance opportunity of a model to the non-profit museum seeking to be accountable in an increasingly complex and demanding environment.

The minimum moral content of law : a critique of Hart's descriptive theory of positive and natural law

Review of H.L.A. Hart's account of the minimum moral content of law - assesses its consistency with the methodology provided in his description of the focal meaning or central case of law - particular focus is Hart's consideration of the ultimate end of man - how difficulties faced by Hart's account of the minimum moral content of law can be overcome.

The separation of ownership and control under a happiness-based theory of the corporation

Explores the happiness-based theory of the corporation, suggesting that there is no conflict between the pursuance of economic and social objectives on the basis that their interplay is required to facilitate shareholder happiness. Considers: (1) the Berle-Means hypothesis and the separation of ownership and control, the dominant governance structure for large companies; (2) a happiness-based perspective on the separation; and (3) law reform applicable to a happiness-based theory. Argues that the separation of ownership and control is not in shareholders' best interests because the structure is not conducive to the happiness of individual shareholders and should be reformed.

Toward a receiver-based theory of knowledge sharing

Managers and researchers alike have sought new ways to address the challenges of sharing dispersed knowledge in modern business environments. Careful consideration by sharers of receivers' knowledge needs and behaviours may improve the effectiveness of knowledge sharing. This research examines how sharers react to their perceptions of receivers' knowledge needs and behaviours when making choices relating to sharing knowledge. The focus of this article is to propose and empirically explore a theoretical framework for a study of the role of the receiver in knowledge sharing--receiver-based theory. Data collected from two case studies highlight a key role played by perceived receiver knowledge needs and behaviours in shaping sharer choices when explicit knowledge is shared. A set of receiver influences on knowledge sharing is provided that highlights key receiver and sharer issues. The paper concludes that companies should develop better ways to connect potential sharers with receivers' real knowledge needs. Further, the findings suggest that sharing on a need-to-know basis hinders change in organisational power structures, and prevents the integration of isolated pockets of knowledge that may yield new value.

Towards a Formalisation of Electronic Contracting Environments

The IST-CONTRACT project is in the process of creating an electronic contracting language. One of the goals of this language is that it has formal underpinnings, and formalizations at a number of levels have been created. One of the lowest levels, upon which the other levels are built is the normative level. At this level, we identify how contract clauses (modeled as norms) may evolve over time. In this paper, we describe this formalization, and show how we may associate various states with a norm throughout its lifecycle. We also show how more complex evaluations may be carried out over a norm, and conclude with an example showing the application of the framework over a contract and its associated norms.

Implemetation of electronic commerce and human resource strategies in an Australian retail banking context

As organisations deploy eCommerce and internet technologies for competitive advantage and to satisfy increasingly demanding customers, they will need to develop human resource (HR) strategies that prepare their employees to work with these technologies effectively. Little systematic investigation has been undertaken to discover how companies manage their HR functions to achieve these outcomes. In the retail banking sector these issues have become very important with increased competition, industry changes and heightened competition. This paper examines HR management strategy in one Australian bank as it moves towards online service provision and adopts other eCommerce applications. The paper draws on theoretical insights from Porter’s (2001) views of competitive advantage from the internet and writers discussing the informational society (Castells, 2001) and post-fordist organisations (Clegg, 1990). An analysis of interview data from this case study shows the issues that one bank is dealing with as it seeks competitive advantage from its customer service offerings while it revises its HR strategies.

Concepts, models and analyses of electronic demolition of buildings

The demolition of building structures produces enormous amounts of waste materials. In most current demolition projects, a great number of demolished materials are directly sent to landfill after their primary usage due to the difficulties in finding their next usage immediately. At the same time, because of limited supply of second-hand materials, new and high quality materials are used in construction projects whose design standards can be fitted using the secondary or used materials. However, this is an inefficient method to reduce waste because off the flow nature of the current waste-exchange systems and the demolition procedure. The recent concept using deconstruction rather than destruction for demolishing a constructed facility fails to achieve widespread understanding or acceptance due to various practical limitations. In this paper, for the purpose of envisaging the deconstruction implementations in practice and promoting cascading usages of construction materials, the concept of electronic demolition (e-Demotion, eDemolition) is put forward for the first time. E-demolition is a virtual demolition approach by which the demolition information, progress and outputs are operated before the physical demolition. Furthermore, the authors set up the essential models to implement electronic demolition of buildings from the viewpoints of demolition progress, business, and information. Each model is demonstrated in accord with the conventional demolition practice and subject to the ideal deconstruction implementation. Following the electronic demolition of a real project, the physical demolition can be anticipated with a minimum of construction waste emission.