Systemic regulation of intestinal iron absorption

The intestinal absorption of the essential trace element iron and its mobilization from storage sites in the body are controlled by systemic signals that reflect tissue iron requirements. Recent advances have indicated that the liver-derived peptide hepcidin plays a central role in this process by repressing iron release from intestinal enterocytes, macrophages and other body cells. When iron requirements are increased, hepcidin levels decline and more iron enters the plasma. It has been proposed that the level of circulating diferric transferrin, which reflects tissue iron levels, acts as a signal to alter hepcidin expression. In the liver, the proteins HFE, transferrin receptor 2 and hemojuvelin may be involved in mediating this signal as disruption of each of these molecules decreases hepcidin expression. Patients carrying mutations in these molecules or in hepcidin itself develop systemic iron loading (or hemochromatosis) due to their inability to down regulate iron absorption. Hepcidin is also responsible for the decreased plasma iron or hypoferremia that accompanies inflammation and various chronic diseases as its expression is stimulated by pro-inflammatory cytokines such as interleukin 6. The mechanisms underlying the regulation of hepcidin expression and how it acts on cells to control iron release are key areas of ongoing research.

Purification and partial characterisation of recombinant human hepcidin

Hepcidin is a liver-expressed antimicrobial and iron regulatory peptide. A number of studies have indicated that hepcidin is important for the correct regulation of body iron homeostasis. The aims of this study were to analyse the expression, trafficking and regulation of human hepcidin in an in vitro cell culture system. Human hepcidin was transfected into human embryonic kidney cells. Immunofluorescence and confocal microscopy analysis revealed that recombinant hepcidin localised to the Golgi complex. Recombinant hepcidin is secreted from the cell within 1 h of its synthesis. Recombinant hepcidin was purified from the cell culture medium using ion-exchange and metal-affinity chromatography and was active in antimicrobial assays. Amino-terminal sequence analysis of the secreted peptide revealed that it was the mature 25 amino acid form of hepcidin. Our results show that recombinant myc-His tagged human hepcidin was expressed, processed and secreted correctly and biologically active in antimicrobial assays. (C) 2005 Elsevier SAS. All rights reserved.

Non-HFE hemochromatosis

Hereditary hemochromatosis (HH) is an autosomal recessive disorder classically related to HFE mutations. However, since 1996, it is known that HFE mutations explain about 80% of HH cases, with the remaining around 20% denominated non-HFE hemochromatosis. Nowadays, four main genes are implicated in the pathophysiology of clinical syndromes classified as non-HFE hemochromatosis: hemojuvelin (HJV, type 2Ajuvenile HH), hepcidin (HAMP, type 2B juvenile HH), transferrin receptor 2 (TFR2, type 3 HH) and ferroportin (SLC40A1, type 4 HH). The aim of this review is to explore molecular, clinical and management aspects of non-HFE hemochromatosis.

Safeguarding rural Australia : addressing masculinity and violence in rural settings. Data report no. 5. Consequences of violence - juvenile offenders, long-term health consequences, anxiety and repression, other chronic disabilities

This report considers extant data which have been sourced with respect to some of the consequences of violent acts and incidents and risky behaviour for males living in regional and remote Australia . This has been collated and presented under the headings: juvenile offenders; long-term health consequences; anxiety and repression; and other chronic disabilities. Additional commentary resulting from exploration, examination and analyses of secondary data is published online in complementary reports in this series.

Juvenile justice in Queensland and Canada : new legislation reflecting new directions

Due to their similar colonial histories and common law heritage, Australia and Canada provide an ideal comparative context for examining legislation reflecting new directions in the field of juvenile justice. Toward this end, this article compares the revised juvenile justice legislation which came into force in Queensland and Canada in 2003 (Canada, Youth Criminal Justice Act, enacted on 19 February 2002 and proclaimed in force 1 April 2003; Queensland, Juvenile Justice Act, amended 2003). There are a series of questions that could be addressed including: How similar and how sweeping have been the legislative changes introduced in each jurisdiction?; What are likely to be some of the effects of the implementation of these new legislative regimes?; and, how well does the legislation enacted in either jurisdiction address the fundamental difficulties experienced by children who have been caught up in juvenile justice systems? This article addresses mainly the first of these questions, offering a systematic comparison of recent Queensland and Canadian legislative changes. Although, due to the recentness of these changes, there is no data available to assess long-term effects, anecdotal evidence and preliminary research findings from our comparative study are offered to provide a start at answering the second question. We also offer critical yet sympathetic comments on the ability of legislation to address the fundamental difficulties experienced by children caught up in juvenile justice systems. Specifically, we conclude that while more than simple legislative responses are required to address the difficulties faced by youth offenders, and especially overrepresented Indigenous young offenders, the amended Queensland and new Canadian legislation appear to provide some needed reforms that can be used to help address some of these fundamental difficulties.

Juvenile justice and truancy legislation : The move towards parental responsibilisation in Queensland

Much current Queensland media rhetoric, government policy and legislation on truancy and youth justice appears to be based on ideas of responsibilisation – of sheeting responsibility for children’s behaviour back onto their parents. This article examines the evidence of parental responsibility provisions in juvenile justice and truancy legislation in Queensland and the drivers behind this approach. It considers recent legislative initiatives as part of an international trend toward making parents ‘responsible’ for the wrongs of their children. It identifies the parental responsibility rhetoric appearing in recent ministerial statements and associated media reports. It then asks the questions – are these legislative provisions being enforced? And if so, are they successful? Are they simply adding to the administrative burdens placed on teachers and schools, and the socioeconomic burdens placed on already disadvantaged parents? Parental responsibility provisions have been discussed at length in the context of juvenile offending and research suggests that punishing parents for the acts of their children does not decrease delinquency. The paper asks how, as a society, we intend to evaluate these punitive measures against parents?

The Punitive Turn in Juvenile Justice: Cultures of Control and Rights of Compliance in Western Europe and the USA

Separate systems of justice for children and young people have always been beset by issues of contradiction and compromise. There is compelling evidence that such ambiguity is currently being `resolved' by a greater governmental resort to neo-conservative punitive and correctional interventions and a neo-liberal responsibilizing mentality in which the protection historically afforded to children is rapidly dissolving. This resurgent authoritarianism appears all the more anachronistic when it is set against the widely held commitment to act within the guidelines established by various children's rights conventions. Of note is the United Nations Convention on the Rights of the Child, frequently described as the most ratified human rights convention in the world, but lamentably also the most violated. Based on international research on juvenile custody rates and children's rights compliance in the USA and Western Europe, this article examines why and to what extent `American exceptionalism' might be permeating European nation states.