Defects of Secretion in Cystic Fibrosis ADVANCES IN EXPERIMENTAL MEDICINE AND BIOLOGY Editorial Board: NATHAN BACK, State University of New York at Buffalo IRUN R. COHEN, The Weizmann Institute of Science DAVID KRITCHEVSKY, Wistar Institute ABEL LAJTHA, A^. S. Kline Institute for Psychiatric Research RODOLFO PAOLETTI, University of Milan Recent Volumes in this Series Volume 550 BRAIN DEATH AND DISORDERS OF CONSCIOUSNESS Edited by Calixto Machado and D. Alan Shewmon Volume 551 POST GENOMIC PERSPECTIVES IN MODELING AND CONTROL OF BREATHING Edited by Jean Champagnat, Monique Denavit-Saubie, Gilles Fortin, Arthur S. Foutz, and Muriel Thoby-Brisson Volume 552 TYPE I DIABETES: Biomedical Science and Technology Edited by George Eisenbarth Volume 553 BIOMATERIALS: From Molecules to Engineered Tissues Edited by Nesrin Hasirci and Vasif Hasirci Volume 554 PROTECTING INFANTS THROUGH HUMAN MILK: Advancing the Scientific Evidence Edited by Larry K. Pickering, Ardythe L. Morrow, Guillermo M. Ruiz-Palacios, and Richard J. Schanler Volume 555 BREAST FEEEDING: Early Influences on Later Health Edited by Gail Goldberg, Andrew Prentice, Ann Prentice, Suzanne Filteau, and Elsie Widdowson Volume 556 IMMUNOINFORMATICS: Opportunities and Challenges of Bridging Immunology with Computer and Information Sciences Edited by Christian Schoenbach, V. Brusic, and Akihiko Konagaya Volume 557 BRAIN REPAIR Edited by M. Bahr Volume 558 DEFECTS OF SECRETION IN CYSTIC FIBROSIS Edited by Carsten Schultz A Continuation Order Plan is available for this series. A continuation order will bring delivery of each new volume immediately upon publication. Volumes are billed only upon actual shipment. For further information please contact the publisher. Defects of Secretion in Cystic Fibrosis Edited by Carsten Schultz European Molecular Biology Laboratory Heidelberg, Germany Sprrii nger Library of Congress Cataloging-in-Publication Data Defects of secretion in cystic fibrosis/edited by Carsten Schultz. p. ; cm. — (Advances in experimental medicine and biology; v. 558) Includes bibliographical references and index. ISBN 0-387-23076-9 (alk. paper) 1. Cystic fibrosis—Pathophysiology. 2. Cystic fibrosis—Molecular aspects. I. Schultz, Carsten. II. Series. [DNLM: 1. Cystic Fibrosis—physiopathology—Congresses. 2. Cystic Fibrosis—therapy—Congresses. 3. Cystic Fibrosis Transmembrane Conductance Regulator—Congresses. 4. Pancreas—secretion—Congresses. WI 820 D313 2005] RC858.C95D44 2005 616.372—dc22 2004063217 ISBN: 0-387-23076-9 elSBN: 0-387-23250-8 ©2005 Springer Science+Business Media, Inc. All rights reserved. This work may not be translated or copied in whole or in part without the written permission of the publisher (Springer Science-!-Business Media, Inc., 233 Spring Street, New York, NY 10013, USA), except for brief excerpts in connection with reviews or scholarly analysis. Use in connection with any form of information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed is forbidden. The use in this publication of trade names, trademarks, service marks and similar terms, even if they are not identified as such, is not to be taken as an expression of opinion as to whether or not they are subject to proprietary rights. Printed in the United States of America. (BS/DH) 9 8 7 6 5 4 3 21 springeronline.com Acknowledgments I would like to thank Sylke Helbing and Bettina Schafer for editorial help and Nicole Heath for critically reading through part of the manuscripts. I also would like to acknowledge all speakers and participants of the 'Defects of Secretion' symposium held in Heidelberg in November 2003. Your contributions and lively discussions made the compilation of this book worth while. Contributing Authors Gabriele Adam Universitat Regensburg, Germany Susan J. Anderson University of Alabama at Birmingham, AL, USA Tanja Bachhuber Universitat Regensburg, Germany Dale J. Benos University of Alabama at Birmingham, AL, USA Christoph Bohmer Eberhard-Karls-Universitat Tubingen, Germany Horst Fischer Children's Hospital Oakland Research Institute, Oakland, CA, USA Catherine M. Fuller University of Alabama at Birmingham, AL, USA Sherif Gabriel University of North Carolina, Chapel Hill, NC, USA Erich Gulbins Eberhard-Karls-Universitat Tubingen, Germany 1 CONTRIBUTING AUTHORS Gunnar C. Hansson Goteborg University, Gothenburg, Sweden Jean-Daniel Horisberger University Lausanne, Switzerland. Beate lUek Children's Hospital Oakland Research Institute, Oakland, CA, USA Malin E. V. Johansson Goteborg University, Gothenburg, Sweden Reinhard Kandolf Eberhard-Karls-Universitat Tubingen, Germany RolfK.-H.Kinne Max-Planck-Institut fur molekulare Physiologie, Dortmund, Germany Helmut Kipp Max-Planck-Institut fiir molekulare Physiologie, Dortmund, Germany Karin Klingel Eberhard-Karls-Universitat Tubingen, Germany Gergely Kovacs University of Alabama at Birmingham, AL, USA Karl Kunzelmann Universitat Regensburg, Germany Florian Lang Eberhard-Karls-Universitat Tubingen, Germany Albrecht Lepple-Wienhues Eberhard-Karls-Universitat Tubingen, Germany Martin E. Lidell Goteborg University, Gothenburg, Sweden Marcus Mall The University of North Carolina, Chapel Hill, NC, USA CONTRIBUTING AUTHORS Mark Moody Inologic Inc., Seattle, WA, USA Bettina Murle Universitat Regensburg, Germany Paul M. Quinton UCSD School of Medicine, La JoUa, CA, USA Monica Palmada Eberhard-Karls-Universitat Tubingen, Germany Caria M. Pedrosa Ribeiro The University of North Carolina, Chapel Hill, NC, USA Andres Ponce UCSD School of Medicine, La JoUa, CA, USA Marsh M, Reddy UCSD School of Medicine, La Jolla, CA, USA Philippe Roussel Universite de Lille 2, Lille, France Rainer Schreiber Universitat Regensburg, Germany Carsten Schultz European Molecular Biology Laboratory, Heidelberg, Germany Steve B. Shears N.LE.H.S./N.LH./D.H.S.S., Research Triangle Park, NC, USA Udicko Szabo Eberhard-Karls-Universitat Tubingen, Germany Alexis Traynor-Kaplan hiologic Lie, Seattle, WA, USA Frank Thevenot, Universitat Witten/Herdecke, Witten, Germany. CONTRIBUTING AUTHORS Thilo Voelcker Universitat Regensburg, Germany. Sabine Wallisch Eberhard-Karls-Universitat Tubingen, Germany Ling Yang N.LE.H.S./N.LH./D.H.S.S., Research Triangle Park, NC, USA.