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The tetrahydrobiopterin (BH 4 ) cofactor is essential for the biosynthesis of catecholamines and serotonin and for nitric-oxide synthase (NOS). Alterations in BH 4 metabolism are observed in various neurological and psychiatric diseases, and mutations in one of the human metabolic genes causes hyperphenylalaninemia and/or monoamine neurotransmitter deficiency. We report on a knockout mouse for the Pts gene, which codes for a BH 4 -biosynthetic enzyme. Homozygous Pts ؊/؊ mice developed with normal morphology but died after birth. Upon daily oral administration of BH 4 and neurotransmitter precursors the Pts ؊/؊ mice eventually survived. However, at sexual maturity (6 weeks) the mice had only one-third of the normal body weight and were sexually immature. Biochemical analysis revealed no hyperphenylalaninemia, normal brain NOS activity, and almost normal serotonin levels, but brain dopamine was 3% of normal. Low dopamine leads to impaired food consumption as reflected by the severe growth deficiency and a 7-fold reduced serum insulin-like growth factor-1 (IGF-1). This is the first link shown between 6-pyruvoyltetrahydropterin synthase-or BH 4 -biosynthetic activity and IGF-1.

This paper makes a contribution to the debate on university organisational actorhood by theorising the determinants of institutional strategic positioning. It argues that besides environmental forces and managerial rationality, the organisational dimension needs to be accounted for. Addressing the mixed empirical evidence in the relevant literature, we conceptualise the organisational dimension as a meso-level intervening variable mediating both external influences (outside-inside) and organisational action (inside-outside). We operationalise the organisational dimension along three components: organisational structure, identity and centrality, which are further elaborated in sub-components and indicators. A set of hypotheses to be tested in empirical research is provided. The paper offers new perspectives on the dynamics of change in higher education and on strategic agency of organisational actors.

Purpose The purpose of this paper is to investigate, through different interpretative theories, the implementation and operation of performance measurement systems (PMS) considering the factors crucial in influencing the development and the operational difficulties of the PMS in a context such as Italy, which is typically unresponsive to new public management-inspired ideas. Design/methodology/approach A theoretical framework is developed through the use of new institutional sociology and management control theory. The empirical study involves the whole ministerial sector, and explores some strategic documents belonging to the new PMS introduced in Italy in 2009. Findings The research illustrates a widespread dissemination of the reform in ministries. However, it has also shown the ceremonial and superficial implementation of the PMS. In addition, the findings confirm that the operation and the actual development of a PMS is strongly affected by the characteristics of the activity under examination. Research limitations/implications The peculiarity of the Italian context limits the generalizability of the findings to countries with similar public sector management and culture. Further studies may investigate the system through an individual perspective, i.e. exploring the role of individual managers in slowing down the operations of the evaluation systems. Originality/value This paper contributes to the debate on the implementation and operation of administrative reforms in legalistic countries also known as Rechtsstaat countries. The use of multiple theories allows investigating the subject matter by considering its complexity in a holistic way.

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