New Animal Models of Human Neurological Diseases

Transcription

1 New Animal Models of Human Neurological Diseases

2 BioValley Monographs Vol. 2 Series Editor P. Poindron, Strasbourg Official Publication of the

3 New Animal Models of Human Neurological Diseases Volume Editors Philippe Poindron, Strasbourg Pascale Piguet, Basel 28 figures, 5 in color, and 9 tables, 2008 Basel Freiburg Paris London New York Bangalore Bangkok Singapore Tokyo Sydney

4 Prof. Philippe Poindron Dr. Pascale Piguet 11, rue Galliéni Pharmazentrum FR Boulogne (France) Klingelbergstrasse 50 CH-4056 Basel (Switzerland) Library of Congress Cataloging-in-Publication Data New animal models of human neurological diseases / volume editors, Philippe Poindron, Pascale Piguet. p. ; cm. - (Biovalley monographs, ISSN ; v. 2) Includes bibliographical references and index. ISBN (hard cover : alk. paper) 1. Nervous system-disease-animal models. I. Poindron, Phillippe. II. Piguet, Pascale. III. Series. [DNLM: 1. Nervous System Diseases. 2. Disease Models, Animal. WL 140 N ] RC347.N dc Bibliographic Indices. This publication is listed in bibliographic services, including Current Contents and Index Medicus. Disclaimer. The statements, options and data contained in this publication are solely those of the individual authors and contributors and not of the publisher and the editor(s). The appearance of advertisements in the book is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements. Drug Dosage. The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any change in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Copyright 2008 by S. Karger AG, P.O. Box, CH 4009 Basel (Switzerland) Printed in Switzerland on acid-free and non-aging paper (ISO 9706) by Reinhardt Druck, Basel ISSN ISBN

5 Contents VII Acknowledgment Poindron, P. (Strasbourg) 1 Theoretical Considerations on Animal Models Poindron, P. (Strasbourg); Callizot, N. (Godmanchester); Piguet, P. (Basel) 11 Mouse Models with Motor Neuron Defects as a Tool for Deciphering Amyotrophic Lateral Sclerosis. Applications, Limits and Future Challenges Echaniz-Laguna, A.; Fricker, B.; Fergani, A.; Loeffler, J.-P.; René, F. (Strasbourg) 39 A Chronic Relapsing Animal Model for Multiple Sclerosis. Myelin Oligodendrocyte Glycoprotein Induced Experimental Autoimmune Encephalomyelitis in Dark Agouti Rats Kinter, J.; Zeis, T.; Schaeren-Wiemers, N. (Basel) 52 Occlusion of the Middle Cerebral Artery in Rats. Refinement of an Animal Model for Human Stroke Cam, E.; Kilic, E. (Zürich); Yulug, B (Bursa); Ritz, M.-F. (Basel) 66 Pyridoxine-Induced Peripheral Neuropathy. Animal Models and Uses Callizot, N. (Godmanchester); Poindron, P. (Strasbourg) V

6 81 A Dopamine-Sensitive Inherited Motor Syndrome Linked to the frissonnant Mutation Callizot, N. (Godmanchester); Guenet, J.-L. (Paris); Poindron, P. (Strasbourg) 97 Author Index 98 Subject Index Contents VI

7 Acknowledgment This second volume of the Biovalley Monographs would never have been published without the generous financial support of Dr. Georg Endress. Dr. Endress is the father of the Biovalley concept. More than 10 years ago, he launched the great adventure which consisted in creating a network of universities, scientists, companies, financial and political institutions to promote the development of biotechnologies in the Upper Rhine Area. As you certainly know, the Biovalley is located in the quadrilateral delimited by Basel, Freiburg im Breisgau, Strasbourg and Mulhouse, and concentrates in a rather narrow space major pharmaceutical firms, prestigious universities and numerous small and middle companies involved in biotechnologies (in the pharmaceutical, environmental as well as in medical or alimentary fields). The idea of Dr. Endress was to give life to a network of cooperating actors decided to share their knowledge and know-how and to create the first international biotechnology cluster in Europe and possibly in the world. Ten years after the launching of this project, Dr. Endress can be proud. The Biovalley is no longer a dream but a living reality. In France, the Région Alsace has got the label of Pôle de Compétitivité à Vocation Internationale in Innovations Thérapeutiques ; Basel continues to welcome in its bio-incubators numerous start-ups and spin-offs; Mulhouse has gained an international visibility in ecopharmacology (environmental remediation), fine chemistry and biometry; Freiburg can pride itself on developing innovative tissue engineering techniques or green biotechnologies. Moreover, cooperative scientific programs could be developed between teams from the Louis Pasteur University and Albert Ludwig University, or between Basel, Louis Pasteur and Albert Ludwig Universities. VII

8 Regularly, international conferences are held in our golden quadrilateral; the next will take place in Colmar (8th and 9th of November) and will be devoted to The new RNA frontiers, in the frame of the now classical Colmar Symposia. Certainly we should intensify the trinational cooperation and give up our natural tendency to national chauvinism. But the publication of the Monographs, the papers of which were written by scientists working in the Biovalley, obviously proves that we have progressed toward the settlement of a friendly and efficient cluster. We are indebted for this to Dr. Endress and his somewhat prophetic vision. Thank you again, Dr. Endress. Philippe Poindron, Strasbourg Acknowledgment VIII