According to phenotype and functional studies, p.H62L is a mild mutation, responsible for a more severe phenotype when associated with another mild mutation. These data are important for patient management and genetic counseling.

[eng] Towards a typology of farms based on the plans and the situation of farmers - After presenting the objectives and the discriminating criteria of some farm typologies, the writers show that if one wants to construct typologies that better translate the development of farms, one must not only take into account their present state in fixed factors but also consider the farmers' plans and situations. These concepts enable their economic behaviour to be better understood. [fre] Les auteurs après avoir présenté les objectifs et les critères discriminants de quelques typologies d'exploitation, montrent que si on veut construire des typologies qui traduisent mieux l'évolution des exploitations agricoles, il faut non seulement tenir compte de la dotation en facteurs fixes mais aussi prendre en compte les projets et les situations des agriculteurs. En effet, ces concepts permettent de mieux comprendre leur comportement économique.

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-Agrarian system and water quality. Concept validity and negotiated elaboration of research company asto the limits and subdivisions of the a rea needing protection are the two controversial points laken as examples. The authors of this article present and analyse the overall approach used in an interdisciplinary research programme for which they were responsible. The programme was initiated by the following question, put forward by an important industrial company producing mineral water, Société Vittel: 'What changes are required concerning the farming activity used on the site, and under what conditions, in arder to reduce the rate of nitrates fou nd beneath the roots of cultivated plants and grassland, and to en sure that this rate remains beneath the limit of 10 mg per litre?' This led them to question the relevance and the limits of the agrarian system concept, as the concept on which an interdisciplinary approach was based. ln the first part, the authors present the merging th at occurred within this research programme of the agrarian system concept with that of sustainable development and with the systems approach. Drawing on past experience with this concept and several previous research projects, they locus on two perspectives. First, an agrarian system is a complex reality which links on a local level the people involved, the activities and a land area undergoing development. The second perspective underlines the graduai elaboration of the agrarian system; how it is formed by th ose involved. Keeping these two points of view in mi nd, an agrarian system madel is presented here, which served as the basis for organising and conducting the research programme. Once the programme objectives had been discussed and the scientific structure established, the decision was made to conduct the research in a reactive way according to its own development and the evolution of the agrarian system being formed. This option was facilitated by the existence of a 'mediator' between the research team and the other players within the agrarian system. The authors propose the notion of ·negotiated research' to describe this approach. This sa me agrarian system madel is used by the authors to make a critical analysis of the research programme itself; it serves as an analyser for controversial points. The water catch ment area as an emerging spatial entity, or the difference in the points of view held by the researchers and the The evolution of local relationships between the people involved played a crucial raie in solving the problem of water quality, and th us in building the agrarian system, through the development of learning processes by the different players: the industrial company, the farmers and the researchers. ln short, in arder to fulfil the two conditions required for sustainable development, i.e. first the creation of negotiation and consultation opportunities, and second the creation of knowledge, reference to the agrarian system concept appears relevant. The authors consider, as shawn by the Vittel res...

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