Reflections on quasi-loss of nationality in comparative, international and European perspective. CEPS Liberty and Security in Europe No. 66/August 2014

This paper focuses on situations in which a person is said never to have had the nationality of a country, even though (s)he assumed (and in many cases the authorities of the country concerned shared that assumption) that (s)he possessed that nationality. Contrary to situations of loss of nationality, where something is taken away that had existed, quasi-loss involves situations in which nationality was never acquired. This contribution seeks to examine whether a person should under certain circumstances be protected against quasi-loss of nationality. In order to do so, the paper first maps out situations of quasi-loss in EU member states, describing typical cases in which a person never acquired the nationality of the country, although (s)he was at some time considered as a national. Drawing on this taxonomy, the paper attempts to uncover whether national, European and international laws offer some protection, and if yes, to which extent, for situations of quasi-loss. It concludes with outlining best practices which Member States should comply with in handling such situations.

Best Practices in Involuntary Loss of Nationality in the EU. CEPS Liberty and Security in Europe No. 73/November 2014

This paper was prepared as a ILEC Policy Brief for discussion at the final conference of the project on Involuntary Loss of European Citizenship: Exchanging Knowledge and Identifying Guidelines for Europe, 11-12 December 2014. Co-funded by the European Commission’s DG for Justice, Citizenship and Fundamental Rights, the ILEC project has aimed to establish a framework for debate on international norms on involuntary loss of nationality. For more information visit: www.ilecproject.eu.

European Citizenship at the Crossroads: Enhancing European Cooperation on Acquisition and Loss of Nationality. CEPS Liberty and Security in Europe No. 73/December 2014

This paper was prepared as a Policy Brief for discussion at the final conference of the project on Involuntary Loss of European Citizenship: Exchanging Knowledge and Identifying Guidelines for Europe, 11-12 December 2014. Co-funded by the European Commission’s DG for Justice, Citizenship and Fundamental Rights, the ILEC project has aimed to establish a framework for debate on international norms on involuntary loss of nationality. For more information visit: www.ilecproject.eu.

How to deal with quasi-loss of nationality situations? Learning from promising practices. CEPS Liberty and Security in Europe No. 71/December 2014

This paper was prepared as a Policy Brief for discussion at the final conference of the project on Involuntary Loss of European Citizenship: Exchanging Knowledge and Identifying Guidelines for Europe, 11-12 December 2014. Co-funded by the European Commission’s DG for Justice, Citizenship and Fundamental Rights, the ILEC project has aimed to establish a framework for debate on international norms on involuntary loss of nationality. For more information visit: www.ilecproject.eu.

Statistics on Loss of Nationality in the EU. CEPS Liberty and Security in Europe No. 70/December 2014

This paper was prepared as a Policy Brief for discussion at the final conference of the project on Involuntary Loss of European Citizenship: Exchanging Knowledge and Identifying Guidelines for Europe, 11-12 December 2014. Co-funded by the European Commission’s DG for Justice, Citizenship and Fundamental Rights, the ILEC project has aimed to establish a framework for debate on international norms on involuntary loss of nationality. For more information visit: www.ilecproject.eu.

Mapping Statistics on Loss of Nationality in the EU: A new online database. CEPS Liberty and Security in Europe No. 76/December 2014

Statistics can be useful when assessing the practical relevance of varying rules and practices on the involuntary loss of nationality across EU member states. Yet while much progress has been made within the EU in recent years with regard to the collection of comparable and reliable information on the acquisition of nationality, statistics on the loss of nationality are hard to find and, where available, difficult to interpret. In this comparative report, the authors explore the landscape of existing statistical data on loss of nationality in the European Union. They identify challenges to the existing methods of data collection and data interpretation and introduce an online statistical database, bringing together all existing statistical data on loss of nationality in the EU. These data are summarised in tables and graphs and discussed with reference to the relevant national and European sources. The authors conclude with recommendations to policy-makers on how to improve data collection in this area.

A Comparative Analysis of Regulations on Involuntary Loss of Nationality in the European Union. CEPS Liberty and Security in Europe, 75/December 2014

This study examines the workings of the Common European Asylum System (CEAS), in order to assess the need and potential for new approaches to ensure access to protection for people seeking it in the EU, including joint processing and distribution of asylum seekers. Rather than advocating the addition of further complexity and coercion to the CEAS, the study proposes a focus on front-line reception and streamlined refugee status determination, in order to mitigate the asylum challenges facing Member States, and vindicate the rights of asylum seekers and refugees according to the EU acquis and international legal standards. Joint processing could contribute to front-line reception and processing capacity, but is no substitute for proper investment in national systems. The Dublin system as currently configured leads inexorably to increasing coercion and detention, and must thus be reconfigured to remove coercion as a principle and ensure consistency with human rights and other fundamental values of the EU.

How European Integration Impacts on National Legislatures: The Europeanization of The German Bundestag. CES Germany and Europe Working Papers, No. 06.2, 2006

In looking at the Europeanization of the German Bundestag, the paper brings together two different debates: the well-established debate on the democratic legitimacy of the European Union sees national Parliaments as guarantor of one branch of a "dual" legitimacy. The more recent debate on "Europeanization" addresses the impacts that European integration has had on its Member States. Analyzing the Europeanization of the German Bundestag, the paper identifies and analyzes three dimensions: legislative Europeanization – the extent to which legislative decision making by the German Bundestag has been influenced by European stipulations over the last twenty years; institutional Europeanization – how the Bundestag as an institution reacted to this loss of function by establishing institutional and procedural provisions for influencing the government's Euro-politics; and strategic Europeanization – the ways in which individual MPs started more recently to develop euro-political strategies that go beyond controlling the national government. The paper shows that the Bundestag only hesitantly reacted to the increasing loss of functions through legislative Europeanization by establishing effective institutional and procedural provisions for controlling the government's Euro-political activities. What is more, the establishment of institutions does not guarantee their effective use. All in all, Euro- politics continues to remain the activity of few MPs. These few, however, have more recently started to europeanize their strategies. The empirical findings support the claim that the traditional concept of chains of legitimacy is inadequate, both in conceptual and in empirical terms. With regard to the democ- ratic legitimacy of EU governance, this indicates that, apart from major reform projects, especially with regard to everyday legislation, not too great a burden should be placed on national Parliaments.

Ferroportin Disease (Haemochromatosis-type IV): a case report

Introdution: Haemochromatosis-type IV, the ferroportin disease, is characterized by an autosomal-dominant transmission and early iron accumulation in macrophages. It is caused by mutations in the transmembrane iron exporter protein ferroportin1 (SLC40A1 gene). In form A (classic), ferroportin loss of function mutants are unable to export iron from cells leading to cellular iron accumulation with decreased availability of iron for serum transferrin (TS). We present a Portuguese rare clinical case of HH-IV. Materials and Methods: A 41-year-old woman with hyperferritinemia and normal TS. Causes of hyperferritinemia (inflammation, chronic alcohol consumption, metabolic syndrome, cell necrosis, non-alcoholic fatty liver disease and aceruloplasminemia) were assessed. Liver iron, evaluated by magnetic resonance imaging (MRI) was carried out. Screening for mutation in HFE and SCL40A1 genes were performed by Sanger sequencing. Baseline: Ferritin:708ng/ml; TS: 27%; MRI:85µmol/g; Hb:13,6g/dl. Therapy: weekly 450ml Therapeutic Phlebotomies (TP) until ferritin≤50ng/ml. Results: Hyperferritinemia comorbidities and common genetic mutations for haemochromatosis were negative. However, sequencing of the patient SLC40A1 gene has revealed the presence in heterozygosity of the variant c.238G>A; p.Gly80Ser. Due to low tolerance to TP, we adopted smaller phlebotomies every three weeks. Conclusion: This patient has a rare autosomal-dominant Ferroportin disease due to a mutated ferroportin which is predicted to be defective in iron cellular export. In agreement, she presents hyperferritinemia, with normal TS and liver iron overload. The genotype/phenotype association allowed to diagnosis this rare FD case. Although a mild form A, we decided to start TP. Her father also has been treated for iron overload.

Genomic amplification of the caprine EDNRA locus might lead to a dose dependent loss of pigmentation

The South African Boer goat displays a characteristic white spotting phenotype, in which the pigment is limited to the head. Exploiting the existing phenotype variation within the breed, we mapped the locus causing this white spotting phenotype to chromosome 17 by genome wide association. Subsequent whole genome sequencing identified a 1 Mb copy number variant (CNV) harboring 5 genes including EDNRA. The analysis of 358 Boer goats revealed 3 alleles with one, two, and three copies of this CNV. The copy number is correlated with the degree of white spotting in goats. We propose a hypothesis that ectopic overexpression of a mutant EDNRA scavenges EDN3 required for EDNRB signaling and normal melanocyte development and thus likely lead to an absence of melanocytes in the non-pigmented body areas of Boer goats. Our findings demonstrate the value of domestic animals as reservoir of unique mutants and for identifying a precisely defined functional CNV.

The Plasmodium serine-type SERA proteases display distinct expression patterns and non-essential in vivo roles during life cycle progression of the malaria parasite

Parasite proteases play key roles in several fundamental steps of the Plasmodium life cycle, including haemoglobin degradation, host cell invasion and parasite egress. Plasmodium exit from infected host cells appears to be mediated by a class of papain-like cysteine proteases called 'serine repeat antigens' (SERAs). A SERA subfamily, represented by Plasmodium falciparum SERA5, contains an atypical active site serine residue instead of a catalytic cysteine. Members of this SERAser subfamily are abundantly expressed in asexual blood stages, rendering them attractive drug and vaccine targets. In this study, we show by antibody localization and in vivo fluorescent tagging with the red fluorescent protein mCherry that the two P. berghei serine-type family members, PbSERA1 and PbSERA2, display differential expression towards the final stages of merozoite formation. Via targeted gene replacement, we generated single and double gene knockouts of the P. berghei SERAser genes. These loss-of-function lines progressed normally through the parasite life cycle, suggesting a specialized, non-vital role for serine-type SERAs in vivo. Parasites lacking PbSERAser showed increased expression of the cysteine-type PbSERA3. Compensatory mechanisms between distinct SERA subfamilies may thus explain the absence of phenotypical defect in SERAser disruptants, and challenge the suitability to develop potent antimalarial drugs based on specific inhibitors of Plasmodium serine-type SERAs.