Insights into muscle degeneration from heritable inclusion body myopathies

Muscle mass and function are gradually lost in age-related, degenerative neuromuscular disorders, which also reflect the clinical hallmarks of sarcopenia. The consensus definition of sarcopenia includes a condition of age-related loss of muscle mass, quality, and strength. The most common acquired muscle disease affecting adults aged over 50 years is sporadic inclusion body myositis (sIBM). Besides inflammatory effects and immune-mediated muscle injury, degenerative myofiber changes are characteristic features of the disease. Although the earliest triggering events in sIBM remain elusive, a plethora of downstream mechanisms are implicated in the pathophysiology of muscle wasting. Although it remains controversial whether hereditary forms of inclusion body myopathy (IBM) may be considered as degenerative sIBM disease models, partial pathophysiological aspects can mimic the much more frequent sporadic condition, in particular the occurrence of inclusion bodies in skeletal muscle. Various clinical aspects in genetically determined skeletal muscle disorders reflect age-related alterations observed in sarcopenia. Several intriguing clues from monogenic defects in heritable IBMs contributing to the molecular basis of muscle loss will be discussed with special emphasis on inclusion body myopathy with Pagets disease of bone and frontotemporal dementia (IBMPFD) and GNE myopathy. Finally, also the recently identified dominant multisystem proteinopathy will be considered, which may rarely present as IBM

The problem of constituting and substantiating media pedagogy

Dieser Beitrag verortet Medienpädagogik im Feld der Wissenschaften. Es wird die Frage diskutiert, inwieweit ein eigenständiger Zugang der Medienpädagogik zu ihren Forschungsanliegen als grundlegend für die Konstitution einer wissenschaftlichen Disziplin gilt bzw. geltend gemacht werden kann. Dazu werden, erstens, allgemeine Konstitutionsmöglichkeiten einer Disziplin schematisch aufgezeigt und ihre je unterschiedlichen Begründungszusammenhänge für wissenschaftliche Disziplinen diskutiert, um – zweitens bestehende Konstitutionsformen und Begründungszusammenhänge der Medienpädagogik einzuordnen, zu hinterfragen und dem Problem des Gemeinsamen und Spezifischen einer «Medienpädagogik» nachzugehen. Drittens, wird eine gemeinsame und spezifische Grundlegung im Verhältnis von Medien und Pädagogik geltend gemacht, die als Beitrag zu einer disziplinären Auffassung von Medienpädagogik gelten kann.This article situates Media Pedagogy in the field of science and humanities. We scrutinise, if or in how far Media Pedagogy can be constituted as its own discipline. Therefore, we discuss in general criteria for the constitution of academic disciplines that may include specific research objectives, styles of reasoning, and certain scientific practices. We conclude that disciplines can be constituted through shared approaches that unite academic practises and through specific criteria that distinguish academic practises from other disciplinary practices. Based on these two distinctive processes, we show how research object, methodology, and policy alone cannot sufficiently function as the common and the specific of Media Pedagogy. We layout, thirdly, that and how the relation between media and pedagogy is a common and specific starting point of Media Pedagogy as an academic discipline, and constitute media pedagogy through this relation

Auf der Flucht! Die Bedeutung persönlicher Netzwerke für den Wanderungsprozess syrischer Flüchtlinge nach Deutschland

Die syrische Flüchtlingskrise, die im Jahr 2015 bezogen auf die Ankunft von Migranten in Europa und insbesondere Deutschland ihren bisherigen Höhepunkt erreicht hat, stellt die Europäische Union vor große solidarische und finanzielle Herausforderungen. Diese Studie rekonstruiert die Wanderungsprozesse fünf syrischer Migranten, die im Zuge der Flüchtlingswelle der vergangenen Jahre aus Syrien nach Deutschland in die Stadt Hildesheim gekommen sind. Dabei wird insbesondere die Rolle persönlicher Netzwerke vor und während der Flucht im Hinblick auf die individuelle Bewertung des Wanderungserfolgs in den Fokus der Analyse gerückt. Die Ergebnisse der Studie zeigen, dass die Entscheidungen zur Flucht im engen Familienkreis getroffen wurden. Während der Wanderungsprozesse, die zum Teil nicht linear verliefen und sich durch verschiedene Etappen kennzeichneten, waren drei Arten von Netzwerken bedeutend: lose Bindungen (weak ties) der Flüchtlinge zu anderen Flüchtlingen, lose Bindungen zwischen Flüchtlingen und Schleppern sowie enge Bindungen (strong ties) zwischen den Mig-ranten und bereits im Zielland ansässigen Verwandten. Eine Voraussetzung für eine erfolgreiche Mig-ration waren strong ties jedoch nicht. Die Bewertung der individuellen Situation nach der Migration ist indes stark von der sozialen Einbettung in Deutschland abhängig. Vor dem Hintergrund des anhaltenden bewaffneten Konflikts in Syrien und schwindender Zukunftsperspektiven ist die Bereitschaft für eine Remigration allerdings bei allen fünf Flüchtlingen gering.The Syrian refugee crisis, which reached its maximum concerning the numbers of migrants in Europe and especially in Germany in 2015, has confronted the European Union with serious solidary and fi-nancial challenges. This paper reconstructs the migration movements of five Syrian refugees, who came as part of the wave of refugees to Germany into the city of Hildesheim. Especially the role of personal networks before and during the escape with regard to the individual evaluation of the migration’s success is the focal point of the study. The paper’s results show that the decisions to take refuge were made within the inner family circle. During the migration processes, which can partly not be recognized as linear and were characterized by different stages, three forms of networks were relevant for the refugees: weak ties to other refugees, weak ties to traffickers and strong ties to relatives who already lived in the target country. However, strong ties were not a pre-requirement for a successful migration. The individual evaluation after the migration depended strongly on the refugees’ social em-beddedness in Germany. Yet, the willingness of all five refugees to remigrate to Syria is low against the background of the ongoing armed conflict and decreasing future perspectives

Can Dedicated Lanes for Automated Vehicles on Urban Roads Improve Traffic Efficiency?

Connected and automated vehicles (CAVs) will behave fundamentally differently than human drivers. In mixed traffic, this could lead to inefficiencies and safety-critical situations since neither human drivers nor CAVs will be able to fully anticipate or predict surrounding traffic dynamics. Thus, some researchers proposed to separate CAVs from conventional vehicles by dedicating exclusive lanes to them. However, the separation of road infrastructure can negatively impact the system’s capacity. While the effects of CAV lanes were addressed for freeways, their deployment in urban settings is not yet fully understood. This paper systematically analyzes the effects of CAV-lanes in an urban setting accounting for the corresponding complexities. We employ microscopic traffic simulation to model traffic flow dynamics in a detailed manner and to be able to consider a wide array of supply-related characteristics. These concern intersection geometry, public transport operation, traffic signal control, and traffic management. Our study contributes to the existing literature by revealing the potential of CAV lanes in an urban setting while accounting for the behavioral and topological complexities. The results of this study can support decision-makers in the design of future urban transportation systems and to prepare cities for the upcoming era of automation in traffic

Prioritization strategies for pandemic influenza vaccine in 27 countries of the European Union and the Global Health Security Action Group: a review

Background: Although there is rapid progress in vaccine research regarding influenza pandemic vaccines it is expected that pandemic influenza vaccine production can only start once the pandemic virus has been recognized. Therefore, pandemic vaccine capacity will be limited at least during the first phase of an influenza pandemic, requiring vaccine prioritization strategies. WHO recommends developing preliminary priorities for pandemic vaccine use. The goal of this review is to provide a thorough overview of pandemic vaccine prioritization concepts in the 27 European Union (EU) member states and the four non-EU countries of the Global Health Security Action Group. Methods: Between September and December 2006 data was collected for each country through two data sources: (i) the national influenza pandemic plan; (ii) contacting key persons involved in pandemic planning by email and/or phone and/or fax Results: Twenty-six (84%) countries had established at least one vaccine priority group. Most common reported vaccine priority groups were health care workers (HCW) (100%), essential service providers (ESP) (92%) and high risk individuals (HRI) (92%). Ranking of at least one vaccine priority group was done by 17 (65%) of 26 countries. Fifteen (88%) of these 17 countries including a ranking strategy, decided that HCW with close contact to influenza patients should be vaccinated first; in most countries followed and/or ranked equally by ESP and subsequently HRI. Rationales for prioritization were provided by 22 (85%) of 26 countries that established vaccine priority groups. There was large variation in the phrasing and level of detailed specification of rationales. Seven (32%) of 22 countries providing rationales clearly associated each vaccine priority group with the specific rationale. Ten (32% of the 31 countries studied) countries have consulted and involved ethical experts to guide decisions related to vaccine prioritization. Conclusion: In the majority of the countries the establishment of vaccine priority groups, ranking and underlying rationales are in line with WHO recommendations. In most public plans the criteria by which prioritized groups are identified are not easily recognizable. Clarity however, may be necessary to assure public acceptability of the prioritization. Ethical experts, results of modelling exercises could play an increasing role in the future decision making process

Complete DNA sequences of the plastid genomes of two parasitic flowering plant species, Cuscuta reflexa and Cuscuta gronovii

<p>Abstract</p> <p>Background</p> <p>The holoparasitic plant genus <it>Cuscuta </it>comprises species with photosynthetic capacity and functional chloroplasts as well as achlorophyllous and intermediate forms with restricted photosynthetic activity and degenerated chloroplasts. Previous data indicated significant differences with respect to the plastid genome coding capacity in different <it>Cuscuta </it>species that could correlate with their photosynthetic activity. In order to shed light on the molecular changes accompanying the parasitic lifestyle, we sequenced the plastid chromosomes of the two species <it>Cuscuta reflexa </it>and <it>Cuscuta gronovii</it>. Both species are capable of performing photosynthesis, albeit with varying efficiencies. Together with the plastid genome of <it>Epifagus virginiana</it>, an achlorophyllous parasitic plant whose plastid genome has been sequenced, these species represent a series of progression towards total dependency on the host plant, ranging from reduced levels of photosynthesis in <it>C. reflexa </it>to a restricted photosynthetic activity and degenerated chloroplasts in <it>C. gronovii </it>to an achlorophyllous state in <it>E. virginiana</it>.</p> <p>Results</p> <p>The newly sequenced plastid genomes of <it>C. reflexa </it>and <it>C. gronovii </it>reveal that the chromosome structures are generally very similar to that of non-parasitic plants, although a number of species-specific insertions, deletions (indels) and sequence inversions were identified. However, we observed a gradual adaptation of the plastid genome to the different degrees of parasitism. The changes are particularly evident in <it>C. gronovii </it>and include (a) the parallel losses of genes for the subunits of the plastid-encoded RNA polymerase and the corresponding promoters from the plastid genome, (b) the first documented loss of the gene for a putative splicing factor, MatK, from the plastid genome and (c) a significant reduction of RNA editing.</p> <p>Conclusion</p> <p>Overall, the comparative genomic analysis of plastid DNA from parasitic plants indicates a bias towards a simplification of the plastid gene expression machinery as a consequence of an increasing dependency on the host plant. A tentative assignment of the successive events in the adaptation of the plastid genomes to parasitism can be inferred from the current data set. This includes (1) a loss of non-coding regions in photosynthetic <it>Cuscuta </it>species that has resulted in a condensation of the plastid genome, (2) the simplification of plastid gene expression in species with largely impaired photosynthetic capacity and (3) the deletion of a significant part of the genetic information, including the information for the photosynthetic apparatus, in non-photosynthetic parasitic plants.</p

The health-related quality of life, mental health and mental illnesses of patients with inclusion body myositis (IBM) : results of a mixed methods systematic review

BACKGROUND: Inclusion body myositis (IBM) is a rare neuromuscular disease (NMD) and effective therapies are not available. Thus, it is relevant to determine the health-related quality of life (HRQoL) in IBM patients including aspects of mental health and illnesses. OBJECTIVES: To identify and summarize the assessment of HRQoL, mental health and illnesses in IBM, the major factors that determine and influence them as well as the respective influence of IBM in general and compared to other NMD as a systematic review. METHODS: We performed a mixed methods systematic review according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The search was conducted within the databases PubMed, PsycINFO, LIVIVO and the Cochrane Database. Data were narratively summarized and categorized in the physical, psychological and social HRQoL dimensions. RESULTS: The systematic screening totalled 896 articles. Six studies were finally identified, comprising of 586 IBM patients. The applied patient reported outcome measures (PROMs) varied. Quantitatively, the main physical impairments (e.g. weakness, functioning, role perception) were assessed using the general population or other NMD as comparators. Results on social and psychological HRQoL were frequently inconsistent. Qualitatively, psychological and social limitations accompanied IBM related physical deteriorations. CONCLUSIONS: A research gap exists regarding rigour determinants of HRQoL and mental illness in IBM. In-depth qualitative studies could help to prepare the ground for the assessment of long-term HRQoL data combined with appropriately focussed psychological PROMs advancing the understanding of the HRQoL in IBM throughout the course of the disease from a patient perspective. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s13023-022-02382-x