Recasting the history and politics of European integration ‘beyond Brussels’

History and International Relation

MutSβ exceeds MutSα in dinucleotide loop repair

The target substrates of DNA mismatch recognising factors MutSalpha (MSH2+MSH6) and MutSbeta (MSH2+MSH3) have already been widely researched. However, the extent of their functional redundancy and clinical substance remains unclear. Mismatch repair (MMR)-deficient tumours are strongly associated with microsatellite instability (MSI) and the degree and type of MSI seem to be dependent on the MMR gene affected, and is linked to its substrate specificities. Deficiency in MSH2 and MSH6 is associated with both mononucleotide and dinucleotide repeat instability. Although no pathogenic MSH3 mutations have been reported, its deficiency is also suggested to cause low dinucleotide repeat instability

Understanding family dynasty: Nurturing the corporate identity across generations

This study aims to analyse the Ahlstrom annual reports. The content analysis contributes to family business corporate identity. According to the results family business corporate identity is based both on history and on the future. Human resource management, customer relationships, high quality, and also family ownership reflect corporate identity in large family corporations. Modern family business corporate identity is based on continuously developing the business concept and its core competency. Meeting the needs of customers and technical quality standards combined with upgrading and developing the business idea characterises family business corporate identity

Family Structure and Ownership Transition as “Polar Opposites”: An Emotional Embeddedness Perspective

Family firms provide a fertile ground to study emotions as a source of contradiction. The interplay of a firm and an owner family exerts influence on strategic decisions in a way that differs from decision-making in non-family firms, such as ownership transition choices. I apply an emotional embeddedness perspective to explain ownership transition choices, which contradict the prevailing instrumental logic in management research. Repeated interactions between actors with different roles in a family firm shape the quality of the family's structure and its effect on important strategic outcomes. The interplay of family structure and emotional embeddedness can lead to ownership transition choices that contradict an instrumental logic of action. Although family structure might be conducive to internal ownership transition, this choice is not always the preferred option because of intervening conditions and the application of alternative principles of action. Researchers in the field of contradiction studies should probe into situations in the management context, in which circumstances may favour the application of an instrumental logic but actually lead to unexpected choices and outcomes. This would enhance our understanding of contingencies that foster alternative action principles in economic action and interaction

Agenesis of the corpus callosum and gray matter heterotopia in three patients with constitutional mismatch repair deficiency syndrome

Constitutional mismatch repair deficiency (CMMR-D) syndrome is a rare inherited childhood cancer predisposition caused by biallelic germline mutations in one of the four mismatch repair (MMR)-genes, MLH1, MSH2, MSH6 or PMS2. Owing to a wide tumor spectrum, the lack of specific clinical features and the overlap with other cancer predisposing syndromes, diagnosis of CMMR-D is often delayed in pediatric cancer patients. Here, we report of three new CMMR-D patients all of whom developed more than one malignancy. The common finding in these three patients is agenesis of the corpus callosum (ACC). Gray matter heterotopia is present in two patients. One of the 57 previously reported CMMR-D patients with brain tumors (therefore all likely had cerebral imaging) also had ACC. With the present report the prevalence of cerebral malformations is at least 4/60 (6.6%). This number is well above the population birth prevalence of 0.09-0.36 live births with these cerebral malformations, suggesting that ACC and heterotopia are features of CMMR-D. Therefore, the presence of cerebral malformations in pediatric cancer patients should alert to the possible diagnosis of CMMR-D. ACC and gray matter heterotopia are the first congenital malformations described to occur at higher frequency in CMMR-D patients than in the general population. Further systematic evaluations of CMMR-D patients are needed to identify possible other malformations associated with this syndrome