Cystic Fibrosis Diagnosis and Protocols, Volume II: Methods and Reso

Despite the many milestones in cystic fibrosis (CF) research, progress toward curing the disease has been slow, and it is increasingly difficult to grasp and use the already wide and still growing range of diverse methods currently employed to study CF so

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MO L E C U L A R BI O L O G Y

Series Editor John M. Walker School of Life Sciences University of Hertfordshire Hatfield, Hertfordshire, AL10 9AB, UK

For other titles published in this series, go to www.springer.com/series/7651

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Cystic Fibrosis Diagnosis and Protocols, Volume II: Methods and Resources to Understand Cystic Fibrosis

Edited by

Margarida D. Amaral Centre for Biodiversity and Functional and Integrative Genomics, Faculty of Sciences, University of Lisboa, Lisboa, Portugal

Karl Kunzelmann Department of Physiology, University of Regensburg, Regensburg, Germany

Editors Margarida D. Amaral Centre for Biodiversity & Functional and Integrative Genomics University of Lisboa Lisboa 1749-016, Portugal [email protected]

Karl Kunzelmann Department of Physiology University of Regensburg Regensburg 93053, Germany [email protected]

ISSN 1064-3745 e-ISSN 1940-6029 ISBN 978-1-61779-119-2 e-ISBN 978-1-61779-120-8 DOI 10.1007/978-1-61779-120-8 Springer New York Dordrecht Heidelberg London Library of Congress Control Number: 2011925926 © Springer Science+Business Media, LLC 2011 All rights reserved. This work may not be translated or copied in whole or in part without the written permission of the publisher (Humana Press, c/o Springer Science+Business Media, LLC, 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. While the advice and information in this book are believed to be true and accurate at the date of going to press, neither the authors nor the editors nor the publisher can accept any legal responsibility for any errors or omissions that may be made. The publisher makes no warranty, express or implied, with respect to the material contained herein. Printed on acid-free paper Humana Press is part of Springer Science+Business Media (www.springer.com)

Foreword This book represents a milestone in the worldwide cystic fibrosis (CF) community’s efforts to continue to pave the way toward the development of new and innovative therapies that address the basic defect in CF. But no book on this subject would be possible without the invaluable contributions of the many patients, families, and disease-related organizations that played a key role in creating the science outlined in these chapters. As an orphan disease, CF does not receive sufficient funding from traditional supporting agencies but depends instead on a vast network of people who selflessly give their time and energy to raise the dollars to support the research that will lead to new treatments and a cure. Much of the science de