Our data demonstrate that the clinical course of symptomatic LVNC can be severe. The identified pathogenic variants and distribution of disease genes-a titin-related pathomechanism is found in every fourth patient-should be considered in genetic counselling of patients. Pathogenic variants in the nuclear proteins Lamin A/C and RBM20 were associated with worse outcome.

This paper reports findings of a pilot study that examined the pedagogical potential of Second Life (SL), a popular three-dimensional multi-user virtual environment (3-D MUVE) developed by the Linden Lab. The study is part of a 1-year research and development project titled 'Modelling of Secondlife Environments' (http://www.le.ac.uk/moose) funded by the UK Joint Information Systems Committee. The research question addressed in this paper is: how can learning activities that facilitate social presence and foster socialisation among distance learners for collaborative learning be developed in SL, a 3-D MUVE?The study was carried out at the University of Leicester (UoL) within an undergraduate module on Archaeological Theory, where two tutors and four students took part in four learning activities designed to take place in SL within the UoL Media Zoo island. The learning activities and training in SL were based on Salmon's five-stage model of online learning. Students' engagement in SL was studied through interviews, observations and records of chat logs. The data analysis offers four key findings in relation to the nature and pattern of in-world 'socialisation' and its impact on real-world network building; the pattern of in-world 'socialisation' stage in Salmon's 5-stage model; perspectives on students' progress in-world through the first stage of the model-'access and motivation'-and perspectives on their entry into, and progress through, the second stage of the model-'socialisation'-and the role of identity presented through avatars in the process of socialisation. The paper offers implications for research and practice in the light of these findings. IntroductionThe academic community shows strong interest in the use of three-dimensional multiuser virtual environments (3-D MUVEs) for education (Eduserve Symposium, 2007;

Background: In the 1990s, Salmon developed a 5-stage model (Salmon 2000(Salmon , 2004 for enabling and scaffolding remote groups to work and learn together using asynchronous bulletin boards. The model has informed online learning and development practice across different levels and education for online and blended learning. Purpose: This paper reports our testing of the usefulness and relevance of the model for Second Life 1 (SL). Programme description and sample: Our case studies included students and tutors from three different disciplines: Archaeology, Digital Photography and Media and Communications. For the first case study we collaborated with a postgraduate distance learning course in Archaeology at the University of Leicester. The second case study involved a campus-based undergraduate course in Digital Photography at the London South Bank University. The third case study was of a postgraduate campus-based course in Media and Communication. Design and methods:In each study, we developed artefacts and activities (SL-tivities) for students and tutors, to enable them to interact in groups. The SL-tivities were designed based on the 5stage model to provide scaffolding of learning in a group. Using qualitative methods, we studied students' and tutors' engagement with SL-tivities and their learning experiences at each stage of the model. We captured data through semi-structured interviews and from chat logs in SL, and mapped student dialogue against each stage of the model. We analysed the data using cognitive mapping, created causal understanding of the individuals and the groups and their changing views, feelings and experiences. Results and conclusions:The case studies gave us examples of learning opportunities in SL at each stage of the model. Our initial study showed that using a structured model for scaffolding learning in groups has value in a 3-D multi-user virtual environment such as SL, as well as in textbased asynchronous environments. The model helps to inform design and delivery so that learners' and teachers' confidence in each other and in the environment builds up and that they work productively with each other. We continue to build further research using SL-tivities and the 5 Stage model to explore and develop further understanding of its applicability.

Open educational resources (OER) and open educational practices (OEP) are relatively new areas in educational research. How OER and OEP can help practitioners enhance curricula is one of a number of under-researched topics. This article aims to enable practitioners to identify and implement appropriate open practices to enhance higher education curricula. To that aim, we put forward a framework of four open educational practices based on patterns of OER reuse ('as-is' or adapted), mapped against the processes of curriculum design and delivery. The framework was developed from the in-depth analysis of 20 cases of higher education (HE) practitioners, which revealed patterns of OER reuse across disciplines, institutions and needs. For each open practice we offer evidence, examples and ideas for application by practitioners. We also put forward recommendations for institutional policies on OER and OEP.