SummaryX-linked inhibitor of apoptosis (XIAP) deficiency, caused by mutations in BIRC4, is an immunodeficiency associated with immune dysregulation and a highly variable clinical presentation. Current diagnostic screening tests such as flow cytometry for XIAP expression or lymphocyte apoptosis assays have significant limitations. Based on recent evidence that XIAP is essential for nucleotide-binding and oligomerization domains (NOD)1/2 signalling, we evaluated the use of a simple flow cytometric assay assessing tumour necrosis factor (TNF) production of monocytes in response to NOD2 stimulation by muramyl dipeptides (L18-MDP) for the functional diagnosis of XIAP deficiency. We investigated 12 patients with XIAP deficiency, six female carriers and relevant disease controls. Irrespective of the diverse clinical phenotype, the extent of residual protein expression or the nature of the mutation, the TNF response was severely reduced in all patients. On average, L18-MDP induced TNF production in 25% of monocytes from healthy donors or female carriers, while fewer than 6% of monocytes responded in affected patients. Notably, the assay clearly discriminated affected patients from disease controls with other immunodeficiencies accompanied by lymphoproliferation, hypogammaglobulinaemia or inflammatory bowel disease. Functional testing of the NOD2 signalling pathway is an easy, fast and reliable assay in the diagnostic evaluation of patients with suspected XIAP deficiency.

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The present review essay is of a novel format: two authors working in the same field introduce each other's works, and then pose a number of questions to each other. The aim is to facilitate dialogue between scholars occupied with similar issues, theories, methods or problems, and to share their discussions with others. Here, Alam Saleh,

NOTE:This is author's pre-print version. The article has been published and reference should be made to the published version: Elling, Rasmus Christian. 2008. 'State of mind, state of order: Reactions to ethnic unrest in the Islamic Republic of Iran', Studies in Ethnicity and Nationalism, Vol. 8,. AbstractBy analysing the symbols and language employed in official statements on two cases of ethnic minority unrest in Iran in 2005-6, the article shows how the Islamic Republic's ideolo-gues and leaders are responding to threats against national security and to alternative definitions of identity. In this emerging discourse, religious and secular notions of patriotism and loyalty are interwoven and an Islamist/nationalist conceptualisation of Iranian nationhood is defended. This interesting process of paradoxical dynamics is an important part of the ongoing struggle to define the identity of Iran in a region boiling with political and cultural conflicts. ArticleBy examining the reactions of the leaders of the Islamic Republic to two cases of ethnic minority protest in the Iranian provinces of Khuzestan and Azerbaijan in 2005-6, this article aims to demonstrate how loyalty to the official definition of nationhood is formulated and defended through interconnected notions of territorial integrity and ideological commitment. The official response to the ethnic unrest was shaped by a careful approach, which sought not to reject the protestors by simply dismissing their grievances altogether. However, it was also used to warn Iranian citizens against perceived plots allegedly threatening the integrity of the nation-state. Thus, the official reaction is a case study of the two-pronged strategy of repressing dissent and accommodating the conformists, and of the way the leaders perceive the Iranian nationstate / the Islamic Republic as both a territorial-political and spatial entity as well as a cultural-historical and temporal entity being threatened by outsiders, traitors and counter-revolutionaries.An analysis of the language employed in statements by Iran's rulers in dealing with incidents of ethnic unrest presents an interesting study in the protean ideology of the establishment and in the processes of inclusion and exclusion that shape the political project that is the Islamic Republic. While Iran certainly falls outside of the theoretical 2 category of 'democracy', the current, official concept of national identity is nonetheless the product of the clashing dynamics of a complex, modern state and society, and by no means the result of a monolithic or medievalist theocracy, which might be the conclusion one could draw from the global media portrayal of Iran. Iranian leaders import notions of both an inclusive, civic democracy and of an exclusivist, Islamist system into their discourse and combine them in a conceptualisation of nationhood that utilizes both non-religious nationalist and religious sentiments. The official response to the cases of ethnic unrest discussed in this article shows how these sentiments are emplo...