Further evidence of linkage at 7p22 with familial hyperaldosteronism type II and exclusion of genetic defects in the RBAK coding regions

Once thought rare, primary aldosteronism (PAL) is now reported to be responsible for 5–10% of hypertension. Unlike familial hyperaldosteronism type I (FH-I), FH-II is not glucocorticoidremediable and not associated with the hybrid CYP11B1/CYP11B2 gene mutation. At least five times more common than FH-I, FH-II is clinically indistinguishable from apparently sporadic PAL, suggesting an even higher incidence. Studies performed in collaboration with C Stratakis (NIH, Bethesda) on our largest Australian family (eight affected members) demonstrated linkage at chromosome 7p22. Linkage at this region was also found in a South American family (DNA provided by MI New, Mount Sinai School of Medicine, New York) and in a second Australian family. The combined multipoint LOD score for these 3 families is 4.61 (q = 0) with markers D7S462 and D7S517, providing strong support for this locus harbouring mutations responsible for FH-II. A newly identified recombination event in our largest Australian family has narrowed the region of linkage by 1.8 Mb, permitting exclusion of approximately half the genes residing in the originally reported 5 Mb linked locus. Candidate genes that are involved in cell cycle control are of interest as adrenal hyperplasia and adrenal adenomas are common in FH-II patients. A novel candidate gene in this linked region produces the retinoblastoma-associated Kruppel-associated box protein (RBaK) which interacts with the retinoblastoma gene product to repress the expression of genes activated by members of the E2F family of transcription factors.

Acquirer and owner perceptions of technology integration:the South African case

Developing countries are subject to the same global pressures as their developed counterparts but have additional domestic challenges that may place them at a significant, and perhaps insurmountable, disadvantage. However, technology still offers them the opportunity to participate in the international economy. Difficult conditions in their countries do not absolve managers from formulating and implementing technology policies that can make their firms globally competitive. At a macro-economic level, a number of broad developmental issues impact on the use of technology in developing countries. The subject of this paper is to examine the challenge for South African firms in their efforts to master technology, despite internal and external difficulties. Owners of technology need to consider the local context when supplying their technology to developing markets. The paper aims to investigate the views of technology recipients by examining the perceptions of South African managers regarding technology integration in a manufacturing environment. A number of technology suppliers were also interviewed in order to obtain their opinions on the issues raised by the technology acquirers. The importance of different factors in integrating technology is studied in relation to managers ’ abilities to control these variables. An importance-control grid framework is used to identify critical parameters and to assess how they can be managed in a complex environment.

Transfer of technology to China:A Scandinavian and European perspective

This paper examines the question of technology transfer from the perspective of techno-economic security and how companies respond to the possibility of losing competitive advantage through misappropriation or leakage. It explores transfers from Europe to China and addresses in particular the operations of Scandinavian companies within the context of the general picture for other European firms. Its point of departure is the authors' earlier research that looked at the motivations for transfer and the awareness of companies of techno-economic security issues. This has been supplemented by new data gathered by the authors from a number of Scandinavian companies in China. Specific actions have been identified and the ownership issue is introduced together with consideration of the role of the companies against the 'Ferdows' model. The analysis shows that the nature of the security question has changed together with the evolving context in which the companies are operating. In turn, the response of companies is contingent on a number of factors including the time horizon of the strategy for a unit in China and the nature of the strategy. It is also influenced by the form of ownership and management style in a particular organisation. © 2002 Elsevier Science Ltd. All rights reserved.

International technology transfer:perceptions and reality of quality and reliability

Impressions about product quality and reliability can depend as much on perceptions about brands and country of origin as on data regarding performance and failure. This has implications for companies in developing countries that need to compete with importers. For manufacturers in industrialised countries it has implications for the value of transferred technologies. This article considers the issue of quality and reliability when technology is transferred between countries with different levels of development. It is based on UK and Chinese company case studies and questionnaire surveys undertaken among three company groups: UK manufacturers; Chinese manufacturers; Chinese users. Results show that all three groups recognise quality and reliability as important and support the premise that foreign technology based machines made in China carry a price premium over Chinese machines based on local technology. Closer examination reveals a number of important differences concerning the perceptions and reality of quality and reliability between the groups.

An Investigation into the adoption and implementation of computer aided production management systems:using the Rogers model of adoption and implementation and a social constuctionist account of technology

The research investigates the processes of adoption and implementation, by organisations, of computer aided production management systems (CAPM). It is organised around two different theoretical perspectives. The first part is informed by the Rogers model of the diffusion, adoption and implementation of innovations, and the second part by a social constructionist approach to technology. Rogers' work is critically evaluated and a model of adoption and implementation is distilled from it and applied to a set of empirical case studies. In the light of the case study data, strengths and weaknesses of the model are identified. It is argued that the model is too rational and linear to provide an adequate explanation of adoption processes. It is useful for understanding processes of implementation but requires further development. The model is not able to adequately encompass complex computer based technologies. However, the idea of 'reinvention' is identified as Roger's key concept but it needs to be conceptually extended. Both Roger's model and definition of CAPM found in the literature from production engineering tend to treat CAPM in objectivist terms. The problems with this view are addressed through a review of the literature on the sociology of technology, and it is argued that a social constructionist approach offers a more useful framework for understanding CAPM, its nature, adoption, implementation, and use. CAPM it is argued, must be understood on terms of the ways in which it is constituted in discourse, as part of a 'struggle for meaning' on the part of academics, professional engineers, suppliers, and users.

An assessment of factors affecting performance and growth of new technology based firms in the UK

New Technology Based Firms (NTBF) are considered to be important for the economic development of a country in regards to both employment growth and innovative activity. The latter is believed to contribute significantly to the increase in productivity and therefore the competitiveness of UK’s economy. This study contributes to the above literature by investigating two of the factors believed to limit the growth of such firms in the UK. The first concerns the existence of a ‘knowledge gap’ while the second the existence of a ‘financial gap’. These themes are developed along three main research lines. Firstly, based upon the human capital theory initially proposed by Backer (1964) new evidence is provided on the human capital characteristics (experience and education) of the current UK NTBF entrepreneurs. Secondly, the causal relationship between general and specific human capital (as well as their interactions) upon the company performance and growth is investigated via its traditional direct effect as well as via its indirect effect upon the access to external finance. Finally, more light is shed on the financial structure and the type of financial constraints that high-tech firms face at start-up. In particular, whether a financial gap exists is explored by distinguishing between the demand and the supply of external finance as well as by type of external source of financing. The empirical testing of the various research hypotheses has been obtained by carrying out an original survey of new technology based firms defined as independent companies, established in the past 25 years in R&D intensive sectors. The resulting dataset contains information for 412 companies on a number of general company characteristics and the characteristics of their entrepreneurs in 2004. Policy and practical implications for future and current entrepreneurs and also providers of external finance are provided.