Physiology of Membrane Disorders

1062 Pages·1986·34.19 MB·English

by  J. David Robertson (auth.)

Second Edition
~~~== ~
PHYSIOLOGY
OF
MEMBRANE
DISORDERS

Second Edition
PHYSIOLOGY
OF
MEMBRANE
DISORDERS
Edited by
Thomas E. Andreoli, M.D.
University oj Texas Health Science Center at Houston
Houston, Texas
Joseph F. Hoffman, Ph.D.
Yale University School oj Medicine
New Haven, Connecticut
Darrell D. Fanestil, M.D.
University oj California, San Diego
La Jolla, California
and
Stanley G. Schultz, M.D.
University oj Texas Health Science Center at Houston
Houston, Texas
PLENUM MEDICAL BOOK COMPANY
New York and London
Library of Congress Cataloging in Publication Data
Main entry under title:
Physiology of membrane disorders.
Includes bibliographies and index.
1. Membranes (Biology) 2. Biological transport. 3. Physiology,
Pathological. I. Andreoli, Thomas E., 1935- [DNLM: 1. Biological
Transport. 2. Ions. 3. Membranes-physiology. 4. Membranes-physio
pathology. 5. Metabolic Diseases. QS 532.5.M3 P578]
RBl13.P49 1985 616.07 85-19367
ISBN-13:978-1-4612-9242-5 e-ISBN-13:978-1-4613-2097-5
DOl: 10.1007/978-1-4613-2097-5
© 1986 Plenum Publishing Corporation
Softcover reprint of the hardcover 1st edition 1986
233 Spring Street, New York, N.Y. 10013
Plenum Medical Book Company is an imprint of Plenum Publishing Corporation
All rights reserved
No part of this book may be reproduced, stored in a retrieval system, or transmitted
in any form or by any means, electronic, mechanical, photocopying, microfilming,
recording, or otherwise, without written permission from the Publisher
Contributors
EDWARD A. ADELBERG, M.D. P. DARWIN BELL, PH.D.
Professor of Human Genetics Assistant Professor of Physiology
Department of Human Genetics and Biophysics
Yale University School of Medicine Nephrology Research and Training Center and
New Haven, Connecticut 06510 Department of Physiology and Biophysics
University of Alabama
RICHARD W. ALDRICH, PH.D.
Birmingham, Alabama 35294
Postdoctoral Fellow
Section of Molecular Neurobiology
Yale University School of Medicine
LEE R. BERKOWITZ, M.D.
New Haven, Connecticut 06510
Department of Medicine
THOMAS E. ANDREOLI, M.D. University of North Carolina
Professor and Chairman School of Medicine
Department of Internal Medicine Chapel Hill, North Carolina 27514
Department of Physiology
University of Texas Health Science Center
Houston, Texas 77025 FRANCISCO BEZANILLA, PH.D.
Professor
STANLEY H. APPEL, M.D. Department of Physiology
Professor and Chairman University of California Medical School
Department of Neurology Los Angeles, California 90024
Baylor College of Medicine
Houston, Texas 77030
STANLEY J. BIRGE, M.D.
PETER S. ARONSON, M.D.
Associate Professor of Medicine
Associate Professor of Medicine
Department of Medicine
and Physiology
Washington University School of Medicine
Departments of Physiology and Internal Medicine
Yale University School of Medicine The Jewish Hospital of St. Louis
St. Louis, Missouri 63110
New Haven, Connecticut 06510
AMIR ASKARI, PH.D.
ROLAND C. BLANTZ, M.D.
Professor and Chairman
Professor of Medicine
Department of Pharmacology and Therapeutics
Department of Medicine
Medical College of Ohio
School of Medicine
Toledo, Ohio 43699
University of California at San Diego
LOUIS V. AV IOLI, M.D. Chief of Nephrology
Professor of Medicine Veterans Administration Medical Center
Department of Medicine San Diego, California 92161
Washington University School of Medicine
The Jewish Hospital of St. Louis
St. Louis, Missouri 63110 JOSEPH V. BONVENTRE, M.D., PH.D.
Assistant Professor of Medicine
TED BEGENISICH, PH.D. Department of Medicine
Associate Professor of Physiology Harvard Medical School
Department of Physiology Department of Preventive Medicine
University of Rochester School of and Clinical Epidemiology
Medicine and Dentistry Massachusetts General Hospital
Rochester, New York 14642 Boston, Massachusetts 02114
v
VI CONTRIBUTORS
WALTER F. BORON, M.D., PH.D. WILLIAM P. DUBINSKY, PH.D.
Assistant Professor Assistant Professor
Department of Physiology Department of Physiology and Cell Biology
Yale University School of Medicine University of Texas Health Science Center
New Haven, Connecticut 06510 Houston, Texas 77025
JAMES L. BOYER, M.D. ANDREW P. EVAN, PH.D.
Professor of Medicine Professor of Anatomy
Director, Liver Study Unit Department of Anatomy
Department of Medicine Indiana University Medical Center
Yale University School of Medicine Indianapolis, Indiana 46223
New Haven, Connecticut 06510
DARRELL D. F ANESTIL, M.D.
MICHAEL D. CAHALAN, PH.D. Professor of Medicine
Assistant Professor Head, Division of Nephrology
Department of Physiology and Biophysics University of California at San Diego
University of California La Jolla, California 92093
Irvine, California 92717
JOHN G. FORTE, PH.D.
MICHAELJ.CAPLAN
Professor of Physiology
Graduate Assistant
Department of Physiology-Anatomy
Department of Cell Biology
University of California
Yale University School of Medicine
Berkeley, California 94720
New Haven, Connecticut 06510
GERHARD GIEBISCH, M.D.
JOSEPH Y. CHEUNG, M.D. Sterling Professor of Physiology
Research Fellow in Medicine Department of Physiology
Department of Medicine Yale University School of Medicine
Harvard Medical School New Haven, Connecticut 06510
Department of Preventive Medicine
and Clinical Epidemiology ROBERT F. GILMOUR, JR., PH.D.
Massachusetts General Hospital Assistant Professor of Pharmacology
Boston, Massachusetts 02114 and Medicine
Krannert Institute of Cardiology
TED H. CHIU, PH.D. Department of Medicine and Pharmacology
Associate Professor of Pharmacology Indiana University School of Medicine
Department of Pharmacology and Therapeutics Indianapolis, Indiana 46223
Medical College of Ohio
Toledo, Ohio 43699
ALBERT M. GORDON, PH.D.
LINDA S. COSTANZO, PH.D. Professor
Department of Physiology and Biophysics
Assistant Professor
University of Washington School of Medicine
Department of Physiology and Biophysics
Medical College of Virginia Seattle, Washington 98195
Richmond, Virginia 23289
GUIDO GUIDOTTI, PH.D.
R. MICHAEL CULPEPPER, M.D.
Professor
Assistant Professor of Medicine Department of Biochemistry and Molecular Biology
Division of Nephrology
Harvard University
University of Texas Health Science Center
Cambridge, Massachusetts 02138
Houston, Texas 77025
RALPH A. DEFRONZO, M.D. EDWARD HA WROT, PH.D.
Associate Professor of Medicine Assistant Professor of Pharmacology
Department of Medicine Department of Pharmacology
Yale University School of Medicine Yale University School of Medicine
New Haven, Connecticut 06510 New Haven, Connecticut 06510
JOHN M. DIETSCHY, M.D.
STEVEN C. HEBERT, M.D.
Professor of Medicine
Assistant Professor of Medicine
Department of Internal Medicine
Harvard Medical School Renal Division
Southwestern Medical School
Brigham and Women's Hospital
University of Texas Health Science Center
Boston, Massachusetts 02115
Dallas, Texas 75235
VINCENT E. DIONNE PETER HESS, M.D.
Division of Pharmacology Research Associate
Department of Medicine Department of Physiology
University of California at San Diego Yale University School of Medicine
La Jolla, California 92093 New Haven, Connecticut 06510
CONTRIBUTORS vii
JOSEPH F. HOFFMAN, PH.D. ALEXANDER LEAF, M.D.
Eugene Higgins Professor of Physiology Professor of Medicine
Department of Physiology Ridley Watts Professor of Preventive Medicine
Yale University School of Medicine Department of Medicine
New Haven, Connecticut 06510 Harvard Medical School
Department of Preventive Medicine
PAUL HOROWICZ, PH.D. and Clinical Epidemiology
Professor and Chainnan Massachusetts General Hospital
Department of Physiology Boston, Massachusetts 02114
University of Rochester School of
Medicine and Dentistry
Rochester, New York 14642 WERNER R. LOEWENSTEIN, PH.D.
Professor
C. HUANG, PH.D. Department of Physiology and Biophysics
Professor University of Miami School of Medicine
Department of Biochemistry Miami, Florida 33101
University of Virginia School of Medicine
Charlottesville, Virginia 22908 MARIANNA M. LONG, PH.D.
Associate Professor
MICHAEL J. JACKSON, PH.D. Laboratory of Molecular Biophysics
Professor of Physiology University of Alabama School of Medicine
Department of Physiology Binningham, Alabama 35294
George Washington University Medical Center
Washington, D.C. 20037 HANS CHRISTOPH LUTTGAU, M.D.
Professor of Physiology
JOHN A. JACQUEZ, M.D. Department of Cell Physiology
Professor Ruhr University
Department of Physiology Bochum, West Gennany
The University of Michigan Medical School
Department of Biostatistics ROBERT I. MACEY, PH.D.
The School of Public Health Professor of Physiology
The University of Michigan Department of Physiology-Anatomy
Ann Arbor, Michigan 48109 University of California
Berkeley, California 94720
JAMES D. JAMIESON, M.D., PH.D.
Professor of Cell Biology TERRY E. MACHEN, PH.D.
Department of Cell Biology Associate Professor of Physiology
Yale University School of Medicine Department of Physiology-Anatomy
New Haven, Connecticut 06510 University of California
Berkeley, California 94720
LEONARD R. JOHNSON, PH.D.
Professor of Physiology and Cell Biology ANTHONY D. C. MACKNIGHT, M.D., PH.D.
Department of Physiology and Cell Biology Department of Physiology
University of Texas Health Science Center University of Otago Medical School
Houston, Texas 77025 Dunedin, New Zealand
H. RONALD KABACK, M.D.
LAZARO J. MANDEL, PH.D.
Head, Laboratory of Membrane Biochemistry
Associate Professor
Roche Institute of Molecular Biology
Department of Physiology
Roche Research Center
Duke University Medical Center
Nutley, New Jersey 07110
Durham, North Carolina 27710
ROLF KINNE, M.D. JOHN A. MANGOS, M.D.
Max-Planck-Institut for Systemphysiology
Professor of Physiology and Pediatrics
Dortmund, West Gennany Chainnan, Department of Pediatrics
Albert Einstein College of Medicine
University of Texas Health Science Center
Bronx, New York 10461 San Antonio, Texas 78284
PHILIP A. KNAUF, PH.D. L. GABRIEL NAVAR, PH.D.
Associate Professor Professor of Physiology and Biophysics
Department of Radiation Biology and Nephrology Research and Training Center
Biophysics and Department of Physiology and Biophysics
University of Rochester School of University of Alabama
Medicine and Dentistry Birmingham, Alabama 35294
Rochester, New York 14642
JOSEPH PALMISANO, M.D.
MARK A. KNEPPER, M.D., PH.D.
Assistant Professor of Medicine
Medical Staff Fellow Division of Nephrology
National Heart, Lung and Blood Institute Department of Medicine
National Institutes of Health University of Connecticut School of Medicine
Bethesda, Maryland 20205 Farmington, Connecticut 06032
viii CONTRIBUTORS
PAMELA A. PAPPONE, PH.D. ROBERT L. RUFF, M.D., PH.D.
Postgraduate Researcher Assistant Professor
Department of Animal Physiology Department of Neurology
University of California Case Western Reserve University
Davis, California 95616 Cleveland Veterans Administration Hospital
Cleveland, Ohio 44106
JOHN C. PARKER, M.D.
Professor of Medicine GEORGE SACHS, M.B., CH. B
Department of Medicine Professor of Medicine and Physiology
University of North Carolina Department of Membrane Biology
School of Medicine University of California
Chapel Hill, North Carolina 27514 Los Angeles, California 90073
JUAN C. PELAYO, M.D. JAMES A. SCHAFER, PH.D.
Assistant Professor of Medicine Professor of Physiology and Biophysics
Department of Medicine Nephrology Research and Training Center
School of Medicine Departments of Physiology and
University of California at San Diego Biophysics, and Medicine
La Jolla, California 92093 University of Alabama
Birmingham, Alabama 35294
GORDON A. PLISHKER, PH.D.
Assistant Professor of Neurology JERRY A. SCHNEIDER, M.D.
Program in Neuroscience Professor of Pediatrics
Department of Neurology Metabolic Diseases Division
Baylor College of Medicine Department of Pediatrics
Houston, Texas 77030 University of California at San Diego
La Jolla, California 92093
DON W. POWELL, M.D.
Professor and Chief MARTIN F. SCHNEIDER, PH.D.
Division of Digestive Disease and Associate Professor of Physiology
Nutrition Department of Physiology
Department of Medicine University of Rochester School of
'University of North Carolina School Medicine and Dentistry
of Medicine Rochester, New York 14642
Chapel Hill, North Carolina 27514
JOSEPH D. SCHULMAN, M.D.
JAMES W. PUTNEY, JR., PH.D. Director
Associate Professor Genetics and IVF Institute
Department of Pharmacology Department of Obstetrics and Gynecology
Medical College of Virginia Fairfax Hospital
Richmond, Virginia 23289 Fairfax, Virginia 22031
BENGT RIPPE, M.D. STANLEY G. SCHULTZ, M.D.
Department of Physiology Professor and Chairman
University of South Alabama Department of Physiology and Cell Biology
College of Medicine University of Texas Health Science Center
Mobile, Alabama 36688 Houston, Texas 77025
J. DAVID ROBERTSON, M.D., PH.D. CAROLYN W. SLAYMAN, PH.D.
James B. Duke Professor and Chairman Professor of Human Genetics and Physiology
Department of Anatomy Departments of Human Genetics and Physiology
Duke University Medical Center Yale University School of Medicine
Durham, North Carolina 27710 New Haven, Connecticut 06510
HOWARD C. ROSENBERG, M.D., PH.D. PHILIP R. STEINMETZ, M.D.
Associate Professor of Pharmacology Professor of Medicine
Department of Pharmacology and Therapeutics Chairman, Division of Nephrology
Medical College of Ohio Department of Medicine
Toledo, Ohio ~3699 University of Connecticut School of Medicine
Farmington, Connecticut 06032
STEVEN A. ROSENZWEIG
Assistant Professor of Cell Biology
GEORGE D1MITRIE STEPHENSON
Department of Cell Biology
Yale University School of Medicine Department of Zoology
New Haven, Connecticut 06510 La Trobe University
Melbourne, Australia
GARY RUDNICK, PH.D.
Associate Professor of Pharmacology JOHN L. STEPHENSON, M.D.
Department of Pharmacology Department of Physiology
Yale University School of Medicine Cornell University Medical College
New Haven, Connecticut 06510 New York. New York 10021
ix
CONTRIBUTORS
CHARLES F. STEVENS, M.D., PH.D. MICHAEL J. WELSH, M.D.
Professor of Physiology Assistant Professor
Section of Molecular Neurobiology Pulmonary Division
Yale University School of Medicine Department of Internal Medicine
New Haven, Connecticut 06510 University of Iowa College of Medicine
Iowa City, Iowa 52242
AUBREY E. TAYLOR, PH.D.
Professor and Chairman
Department of Physiology HENRIK WESTERGAARD, M.D.
University of South Alabama Assistant Professor of Medicine
College of Medicine Department of Internal Medicine
Mobile, Alabama 36688 Southwestern Medical School
University of Texas Health Science Center
SAMUEL O. THIER, M.D. Dallas, Texas 75235
Professor and Chairman
Department of Medicine
Yale :Jniversity School of Medicine MICHAEL M. WHITE, PH.D.
New Haven, Connecticut 06510 Postdoctoral Researcher
Department of Physiology
T. E. THOMPSON, PH.D. University of California Medical School
Professor of Biochemistry Los Angeles, California 90024
Department of Biochemistry
University of Virginia School
of Medicine ERICH E. WINDHAGER, M.D.
Charlottesville, Virginia 22908 Professor and Chairman
Department of Physiology
RICHARD W. TSIEN, D. PHIL. Cornell University Medical College
Professor of Physiology New York, New York 10021
Department of Physiology
Yale University School of Medicine
New Haven, Connecticut 06510 DOUGLAS P. ZIPES, M.D.
Professor of Medicine
DAN W. URRY, PH.D. Director of Cardiovascular Research
Director, Laboratory of Molecular Biophysics Krannert Institute of Cardiology
Professor of Physiology and Biophysics Department of Medicine
and Biochemistry Indiana University School of Medicine
University of Alabama Roudebush Veterans Administration
School of Medicine Medical Center
Birmingham, Alabama 35294 Indianapolis, Indiana 46223