Correction of a mouse model of Menkes disease by the human Menkes gene

The brindled mouse is an accurate model of the fatal human X-linked copper deficiency disorder, Menkes disease. Males carrying the mutant allele of the Menkes gene orthologue Atp7a die in the second week of life. To determine whether the genetic defect in the brindled mice could be corrected by expression of the human Menkes gene, male transgenic mice expressing ATP7A from the chicken β-actin composite promoter (CAG) were mated with female carriers of the brindled mutation (Atp7aMo-br). Mutant males carrying the transgene survived and were fertile but the copper defect was not completely corrected. Unexpectedly males corrected with one transgenic line (T25#5) were mottled and resembled carrier females, this effect appeared to be caused by mosaic expression of the transgene. In contrast, males corrected with another line (T22#2) had agouti coats. Copper concentrations in tissues of the rescued mutants also resembled those of the heterozygous females, with high levels in kidney (84.6 ± 4.9 μg/g in corrected males vs. 137.0 ± 44.3 μg/g in heterozygotes) and small intestine (15.6 ± 2.5 μg/g in corrected males vs. 15.7 ± 2.8 μg/g in heterozygotes). The results show that the Menkes defect in mice is corrected by the human Menkes gene and that adequate correction is obtained even when the transgene expression does not match that of the endogenous gene.

A review of critical information infrastructure protection within IT security guidelines

This research takes the form of a review and looks at the current advisories offered to informationl security professionals in Ihe area of critical information infrastructure protection A critical information infrastructure protection mode! is also presented along with a critical review of some of lhe recent formal guidance that has been offered. The Critical lnformation Infrastructure Protection - Risk Analysis-Methodology (CIlP-RAM) is then offered as a solution to the lack of information and advice.

Raising the funds - spending the funds: a case study of the effectiveness of both roles of NGO's

Non-governmental organisations (NGOs) must be effective in both raising funds and using these funds to implement programs in order to improve the circumstances of the poor. This chapter presents a case study of World Vision Australia's (WVA) fundraising and programming responses to the Asian Tsunami. The initial stages of the fundraising campaign were largely unplanned and reactive. Yet WVA received over $110 million for its tsunami appeal in just a little over three months. This is incomparable with other recent large-scale disaster fundraising campaigns. The experiences of how the initial phases of the response were implemented arc gauged through a qualitative analysis of interviews conducted with twenty-one staff members who completed short-term secondments during the initial tsunami response. These interviews extract a number of reflections of WVA's programming and provide a number of lessons for WVA for future complex humanitarian emergencies. This chapter thus reviews the effectiveness of both roles of NGOs - raising funds and expending these funds to implement programs.

Distance-driven fusion of gait and face for human identification in video

Gait and face are two important biometrics for human identification. Complementary properties of these two biometrics suggest fusion of them. The relationship between gait and face in the fusion is affected by the subject-to-camera distance. On the one hand, gait is a suitable biometric trait for human recognition at a distance. On the other hand, face recognition is more reliable when the subject is close to the camera. This paper proposes an adaptive fusion method called distance-driven fusion to combine gait and face for human identification in video. Rather than predefined fixed fusion rules, distance-driven fusion dynamically adjusts its rule according to the subject-to-camera distance in real time. Experimental results show that distance-driven fusion performs better than not only single biometric, but also the conventional
static fusion rules including MEAN, PRODUCT, MIN, and MAX.

The serendipity of leadership effectiveness in management and business practices

Purpose – The objective of this paper is to conceptualize the serendipity of leadership effectiveness in management and business practices. The term “serendipity” is defined as the mix of leadership effectiveness by accident and sagacity in management and business practices.
Design/methodology/approach – The paper provides a conceptual discussion of the serendipity of leadership effectiveness in management and business practices.
Findings – This paper contributes a number of models and a matrix that are introduced to address the underlying criteria of the cause-effect relationship between leadership effectiveness and organizational achievements.
Research limitations/implications – This paper challenges the idealistic picture that flourishes in the management literature and in management practice of the direct, positive impact of leadership on prosperous management and business practices. In fact, it reinforces and underpins the critical or sceptical views of leadership effectiveness raised in the literature.
Practical implications – Normally, views of organizational achievements are based on the assumption that contextual, timely and skilful precisions in leadership effectiveness are high. Shareholders and stakeholders may benefit from a thorough examination of these issues in organizational achievements. It would not be surprising to find that leadership effectiveness in management and business practices to a minor or major extent is derived from pure luck and coincidence in contextual and timely precisions: right place, right time. This means that such leadership effectiveness may be based on serendipity rather than skilfulness in terms of organizational achievements.
Originality/value – The authors contend that the term “serendipity” contributes to enhance the ongoing discussion in the literature of the link between leadership effectiveness and organizational achievements. It also provides a fundament of understanding, explanation and prediction of leadership effectiveness in management and business practices.

Sustainable components of leadership effectiveness in organizational performance

Purpose – The objective is to describe a selection of sustainability components of leadership effectiveness in organizational performance.
Design/methodology/approach – The paper is based on a conceptual discussion. Findings – The paper contributes to descriptive models that address sustainability components of leadership effectiveness in organizational performance. The study highlights some common views that exist in the management literature and in prosperous management practice related to the direct impact of the relationship in organizational performance between leadership and effectiveness. In fact, it also highlights the critical or sceptical views of leadership effectiveness in organizational performance raised in the literature. Research limitations/implications – The paper contends that the actual leadership effectiveness in organizational performance varies over time and across contexts. At times, the achievement in organizational performance is the outcome of prosperous and conscious leadership, while it at other times may be the outcome of poor and deficient leadership. The topic at hand is positioned and limited to the interface that may describe and explain the connection between these two views. Furthermore, it is limited to corporate decision making and business behaviour in relation to leadership effectiveness and organizational performance. Practical implications – The leadership of an organization need not only to be successful today, but they also need to be successful tomorrow to stay in control and to flourish. Quality control and quality assurance are no longer enough for most organizations. They need to build an awareness of the sustainability components into processes of their management and business practices (i.e. internal and external ones) in order to be judged as successful in corporate decision-making and business behaviour in organizational performance in the long term. Originality/value – The principal contributions of the study are a model of timely leadership effectiveness, a model of contextual leadership effectiveness, and a typology of leadership effectiveness in corporate decision-making and business behaviour. These contributions provide theoretical and managerial ideas and insights into the sustainability components of leadership effectiveness in organizational performance.

Macronutrient innovations: the role of fats and sterols in human health

Dietary intake of fats and sterols has long been known to play a critical role in human health. High proportions of saturated fat, which increase blood cholesterol levels, are mainly found in animal fat and some plant oil (e.g. cocoa butter, palm oil etc.). The predominant polyunsaturated fatty acid (PUFA) in the Western diet is linoleic acid (LA; 18:2n-6), an essential fatty acid, which is commonly found in vegetable seed oils. This is the parent fatty acid of n-6 series PUFA, which can be converted in vivo to C20 and C22 n-6 long chain (LC) PUFA. α‐linolenic acid (ALA; 18:3n-3) is less abundant than LA and is another essential fatty acid; ALA is also present in some vegetable oils such as perilla, flaxseed, canola, soybean and walnut oils, and is the precursor of C20 and C22 n-3 LC PUFA. Sterols are widely distributed in animal tissue and plants, with cholesterol being the major sterol in animal tissue and β-sitosterol, campesterol and stigmasterol being the main sterols in plants. It has long been recognized that an increased dietary intake of saturated fat and (to a lesser extent) cholesterol, raises plasma/serum total and low-density lipoprotein (LDL)-cholesterol, and PUFA decreases these levels. Results from recent studies have shown that plasma/serum levels of lipids and lipoprotein lipids can also be decreased by plant sterols (phytosterols) and diacylglycerol (DAG). Conjugated linoleic acid (CLA, cis-9,trans-11−18:2) has been reported to have anticancer and antidiabetic activities. Fat as the DAG form has also been reported to have anti-obesity effects. Omega-3 PUFA have a beneficial effect on increased heart rate variability, decreased risk of stroke, reduction of both systolic and diastolic blood pressure and may be effective in managing depression in adults. Gamma-linolenic acid (GLA) and phytosterols have an anti-inflammatory activity. The GLA, when combined with docosahexaenoic acid (DHA), have been reported to have a beneficial effect in hyperactive children. These data show that various lipids are powerful bioactive compounds.

Brokering knowledge : managing knowledge in a network of government and non-government human service delivery agencies

This paper examines aspects of knowledge management that are particularly important in the network of human service delivery agencies in Victoria. This network is characterised by four features: it is a cluster of networked organisations; professionals and others may act as knowledge brokers within and between organisations in the network; rapid change in both knowledge and organisation accentuates the importance of innovative knowledge and emergent organisation over and above routine instrumental knowledge within stable organisation; and consequently there is an underlying concern with dialogical rather than instrumental knowledge and its management, and particularly how it constitutes and is constituted by organisation. The paper describes the analytical tools that we consider particularly important in examining this situation – in particular, the distinction between instrumental and dialogical knowledge, and the role of knowledge brokers (and professionals as knowledge brokers). It concludes by relating this analysis to broader issues in organisation studies, and suggests paths for further examination of these issues.

A reservoir of brown adipocyte progenitors in human skeletal muscle

Brown adipose tissue uncoupling protein-1 (UCP1) plays a major role in the control of energy balance in rodents. It has long been thought, however, that there is no physiologically relevant UCP1 expression in adult humans. In this study we show, using an original approach consisting of sorting cells from various tissues and differentiating them in an adipogenic medium, that a stationary population of skeletal muscle cells expressing the CD34 surface protein can differentiate in vitro into genuine brown adipocytes with a high level of UCP1 expression and uncoupled respiration. These cells can be expanded in culture, and their UCP1 mRNA expression is strongly increased by cell-permeating cAMP derivatives and a peroxisome-proliferator-activated receptor-{gamma} (PPAR{gamma}) agonist. Furthermore, UCP1 mRNA was detected in the skeletal muscle of adult humans, and its expression was increased in vivo by PPAR{gamma} agonist treatment. All the studies concerning UCP1 expression in adult humans have until now been focused on the white adipose tissue. Here we show for the first time the existence in human skeletal muscle and the prospective isolation of progenitor cells with a high potential for UCP1 expression. The discovery of this reservoir generates a new hope of treating obesity by acting on energy dissipation.

Managing behavioural symptoms of dementia : Effectiveness of staff education and peer support

This study was designed to investigate the impact of staff education on the behaviour and quality of life of residents with dementia and on staff members' attitudes about working with people with dementia and level of burnout. Staff from three aged care facilities participated in the study (n = 52). These facilities were randomly assigned to one of two intervention groups or a control group. Staff assigned to the intervention groups received an eight-week behaviourally-based programme. Staff from one aged care facility also participated in a peer support group designed to reinforce educational material and facilitate positive changes among staff members. Behavioural symptoms displayed by residents (n = 76) in each of the facilities were also assessed. Assessments were conducted at pre-intervention, post-intervention, three- and six-month follow-up. The results of this study indicated that education or peer support was not associated with an improvement in resident behaviour or quality of life. Education or peer support also did not impact on staff members' level of burnout. There was, however, a change in staff members' attitudes about working with people with dementia. Possible explanations for these findings and implication for further research are considered.