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Pathogenic variants in FLNA cause a diversity of X‐linked developmental disorders associated with either preserved or diminished levels of filamin A protein and are conceptualized dichotomously as relating to underlying gain‐ or loss‐of‐function pathogenic mechanisms. Hemizygosity for germline deletions or truncating variants in FLNA is generally considered to result in embryonic lethality. Structurally, filamin A is composed of an N‐terminal actin‐binding region, followed by 24 immunoglobulin‐like repeat units. The repeat domains are separated into distinct segments by two regions of low‐complexity known as hinge‐1 and hinge‐2. Hinge‐1 is proposed to confer flexibility to the otherwise rigid protein and is a target for cleavage by calpain with the resultant filamin fragments mediating crucial cellular signaling processes. Here, three families with pathogenic variants in FLNA that impair the function of hinge‐1 in males are described, leading to distinct clinical phenotypes. One large in‐frame deletion that includes the hinge leads to frontometaphyseal dysplasia in affected males and females, while two germline truncating variants located within the exon encoding hinge 1 result in phenotypes in males that are explained by exon skipping and under‐expression of a transcript that deletes hinge‐1 from the resultant protein. These three variants affecting hinge‐1 indicate that this domain does not mediate cellular functions that, when deficientresult in embryonic lethality in males and that germline truncating variants in this region of FLNA can result in viable phenotypes in males. [ABSTRACT FROM AUTHOR]

This paper provides a critical assessment of the key recommendations for internationalising higher education in Hong Kong issued by the University Grants Committee in December 2010. Key topics include the rationale for internationalisation, the process by which internationalisation will be carried out, the proposal that Hong Kong aspire to become a regional "education hub," the roles of English, Cantonese and Putongua in instruction and the impact of internationalisation on curriculum reform and student learning. In general, the report represents a missed opportunity to provide clear and compelling guidance to the university sector in Hong Kong regarding internationalisation.

In higher education circles, it has become commonplace to observe that global awareness must be an important educational goal if students are to cope successfully as citizens and professionals with the challenges of globalization. Yet despite the growing acknowledgment that international education must be a central priority, most indicators suggest that American higher education as a whole is doing an inadequate job in helping students understand the world beyond the borders of the United States. The chief problem is that, in their international educational efforts, most schools offer adequate depth for the few, but inadequate breadth for the many. The resources devoted to international education tend to focus overwhelmingly on the needs of that small percentage of students who pursue a major in some international field of study. While there is no doubt such allocation is appropriate, the most glaring shortcomings in international education concern the vast majority of students who will not specialize in an international area of study, but nevertheless need some degree of global awareness and intercultural competence. The authors believe that the most promising approach to achieving broad yet meaningful levels of internationalization involves the infusion of international perspectives and opportunities throughout the institution. Infusion broadens the reach of international education beyond the small number of students who major in an international field, and does so in a manner that is more meaningful and relevant to students than the general education model. Infusion draws upon successful across-the-curriculum approaches applied in the areas of writing, speaking, critical thinking and citizenship, among others. Although an infusion approach to internationalization has been adopted at many schools, each such initiative must be tailored to the structure, mission and needs of particular institutions. Drake University's own internationalization program, still in its early stages, combines a number of elements that fit its particular needs as a medium-sized (approximately 3,000 undergraduates), private, comprehensive university located in the Midwest. This article takes a look at Drake's three major new internationalization initiatives: (1) the Center for Global Citizenship for the development of opportunities for interdisciplinary collaboration around international themes; (2) a new and innovative approach to language training; and (3) the initiation of strategic partnerships with overseas universities in selected countries. While dependent upon support from the highest levels of the university's administration, the success of these programs also depends heavily upon the engagement of intersecting networks of faculty, staff and students. (Contains 1 table and 19 notes.)

In this article, we reappraise the model of Discourse Analysis developed by Sinclair and Coulthard (1975) to analyse classroom talk. We analyse an extract of teacher-student dialogue using this model, then re-analyse the same extract drawing on conventions and concepts developed within the framework of Conversation Analysis. We argue that this more fine-grained perspective makes visible the co-constructed nature of teacher-student dialogue, and reveals improvisation strategies, such as the morality tale, used by the teacher to incorporate divergent points of view within the polyphony of classroom discussion. Our findings indicate the communicative importance of speech prosody in educational settings. We conclude by suggesting avenues for further research in the field and suggest possible applications of our perspective to the practice of classroom teaching and learning. (Contains 2 tables and 5 extracts.)

This paper explores the use of thinking aloud made by young people who have sustained a severe acquired brain injury (ABI). The phenomenon is compared with the concepts of egocentric speech and inner speech before the form of thinking aloud by pupils with ABI is examined. It is suggested that by using thinking aloud, this group of pupils is able to engage in internally persuasive dialogue and is therefore enabled to take part in classroom learning. (Contains 4 tables and 1 note.)

Prosody refers to features of speech such as intonation, volume and pace. In this paper, we examine teacher-student dialogue in an English lesson at a secondary school in England, using Conversation Analysis notation to mark features of prosody. We also make connections with Goffman's theoretical concept of footing. We show that, within an episode of teacher-led plenary discourse, prosody may be used to signal shifts in footing between different kinds of pedagogic activity. We identify: (i) teacher-led IRF (Initiation-Response-Feedback) discussion; (ii) the teacher's modelling of exploratory talk; (iii) a shift to instruction-giving. If teachers are able to model the enquiring tone of exploratory talk, they may in turn encourage more thoughtful contributions from students.

This paper extends and develops the metaphor of scaffolding to take account of the specific needs of pupils with an Acquired Brain Injury (ABI), drawing on observational evidence gathered for an empirical enquiry into the learning of pupils with ABI in mainstream classroom conditions. This is an area in which there are few published studies to date. After considering the needs of this particular group of pupils, the development of the scaffolding metaphor is outlined. The use of scaffolding for a group of pupils with ABI is illustrated and the need for modifications discussed, before suggesting the acronym PEDER (Point out, Explain, Demonstrate, Encourage, Repeat) to focus on the particular needs of this group. However, in practice, this acronym has been found to assist a wider group than just pupils with an ABI. (Contains 2 tables and 2 notes.)

This paper seeks to draw parallels between different approaches to classroom instruction and two contrasting musical styles and to examine how pupils with Acquired Brain Injuries (ABI) might fare in each. A polyphonic classroom is defined as one where an awareness of multiple layers of meaning are encouraged to enhance the learning opportunities, whereas in a classical classroom most of the layers expressly support a single dominant line. While this analogy cannot be stretched too far, it is suggested that, after an ABI, a pupil might progress their learning more easily in a classical-style classroom.

With the increasing number of disabled students entering the higher education sector, much research work has focused on the support services arena and the elimination of barriers that the disabled students have encountered. While producing useful advice on meeting the needs of disabled students, this line of research has done little to locate disability issues within the mainstream learning and teaching debate. By adopting a socio-educational model of "learning difficulty", the study upon which this article draws examined through a survey the issue of "learning support" for the whole student population of one university. The survey employed the Learning for All Questionnaire (LfAQ), a newly developed instrument that aimed to operationalise a holistic view of learning support. The analysis of the collected data provided directions for developing university policies and practices through a significant reformulation of the existing support provision. The article concludes by exploring the concept of "institutional habitus" as a tool for understanding institutional practices, and effecting change to enhance learning and promote inclusion. The implications of current funding arrangements (the Disabled Students' Allowance) for a mainstream model of learning support are also discussed. (Contains 4 tables and 1 note.)

Discusses the quality of teacher-pupil dialogue in the guided reading session of the Literacy Hour and its relationship to pupils' powers of comprehension. Suggests that talk in this context is teacher-dominated. Argues that there is a case for making time available during the guided reading session for a period of discussion in which pupils are invited to play a more leading role in shaping the topic of conversation. (SG)

Analyzes two examples of classroom discourse between a teacher and a small group of students in English primary schools. Both transcripts are extracts from discussions that took place during guided reading sessions. Drawing on concepts from the work of the Bakhtin Circle, argues that one of the sequences exemplifies "pedagogical dialogue," in which someone who knows and posses the truth instructs someone who is ignorant of it and in error. (Author/VWL)

Although the "consensus thesis" assumes that learning difficulties arise from school structural deficiencies, a study of two English secondary schools concludes that the thesis fails to account for complexities of school development. Instead, open-ended dialog between contrasting discourses would foster dynamic school cultures. (SK)

Discusses responses of five (British) local education authorities to managing special education within the new policy context created by the Code of Practice, based on case-study evidence. A new, distinctive governance model has emerged. Consultation between central and local governments has created a favorable climate for developing special-needs provision at the local level. Community and strategic leadership are key factors. (18 references) (MLH)