Physiology of Membrane Disorders

1123 Pages·1978·50.79 MB·English

by  J. David Robertson (auth.)

PHYSIOLOGY
OF
MEMBRANE
DISORDERS

PHYSIOLOGY
OF
MEMBRANE
DISORDERS
Edited by
Thomas E. Andreoli, M.D.
University of Alabama, Birmingham
Joseph F. Hoffman, Ph.D.
Yale University
and
Darrell D. Fanestil, M. D.
University of California, San Diego
PLENUM MEDICAL BOOK COMPANY
New York and London
Library of Congress Cataloging in Publication Data
Main entry under title:
Physiology of membrane disorders.
Includes index.
1. Membranes (Biology) 2. Biological transport. 3. Physiology, Pathological. I. Andreoli, Thomas E.,
1935- II. Hoffman, Joseph F. III. Fanestil, Darrell D. [DNLM: 1. Membranes-Physiology. 2.
Membranes-Physiopathology. 3. Biological transport. 4. Ions. QS532.5.M3 P578]
RB113.P49 616.07 784071
ISBN-13: 978-1-4613-3960-1 e-ISBN-13: 978-1-4613-3958-8
DOl: 10.lO07/978-1-4613-3958-8
© 1978 Plenum Publishing Corporation
227 West 17th Street, New York, N.Y. 10011
Softcover reprint ofthe hardcover 1st edition 1978
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, microfliming,
recording, or otherwise, without written permission from the Publisher
Contributors
EDWARD A. ADELBERG, M.D. ROLAND C. BLANTZ, M.D.
Professor of Human Genetics Chief, Nephrology
Yale University School of Medicine Veterans Administration Hospital
New Haven, Connecticut 06510 San Diego, California 92161; and
Associate Professor of Medicine
THOMAS E. ANDREOLI, M.D.
University of California, San Diego, School of Medicine
Professor of Medicine, and
La Jolla, California 92161
Director, Division of Nephrology and
Professor of Physiology and Biophysics MAURICE B. BURG, M.D.
University of Alabama School of Medicine Chief, Laboratory of Kidney and Electrolyte Metabolism
Birmingham, Alabama 35294 National Heart, Lung, and Blood Institute
National Institutes of Health
STANLEY H. APPEL, M.D.
Bethesda, Maryland 20014
Professor and Chairman of Neurology
Baylor University School of Medicine LINDA S. COSTANZO, PH.D.
Houston, Texas 77030 Assistant Professor of Physiology
Cornell University Medical College
CLAY M. ARMSTRONG, M.D.
Professor of Physiology New York, New York 10021
University of Pennsylvania School of Medicine
RALPH A. DeFRONZO, M.D.
Philadelphia. Pennsylvania 19174
Associate Professor of Medicine, Division of Nephrology
AMIR ASKARI, PH.D. Yale University School of Medicine
Professor and Chairman, New Haven, Connecticut 06510
Department of Pharmacology and Therapeutics
Medical College of Ohio DENNIS A. DIEDERICH, M.D.
Toledo, Ohio 43614 Associate Professor of Medicine
University of Kansas Medical Center
LOUIS V. AVIOLI, M.D. College of Health Sciences and Hospital, Nephrology
Professor of Medicine, Division of Bone and Mineral
Division
Metabolism Kansas City, Kansas 66103
Washington University School of Medicine
St. Louis, Missouri 63110 JOHN M. DIETSCHY, M.D.
Professor of Medicine
TED BEGENISICH, PH.D.
Assistant Professor of Physiology Southwestern Medical School
University of Rochester School of Medicine and Dentistry University of Texas Health
Rochester, New York 14642 Science Center at Dallas
Dallas, Texas 75235
ST ANLEY J. BIRGE, M.D.
Assistant Professor of Medicine, Division of Bone and PHILIP B. DUNHAM, PH.D.
Mineral Metabolism Professor of Biology
Washington University School of Medicine Syracuse University
St. Louis, Missouri 63110 Syracuse, New York 13210
v
VI CONTRIBUTORS
LAURENCE E. EARLEY, M.D. JOHN A. JACQUEZ, M.D.
Professor and Chairman Professor
Department of Medicine Department of Physiology, The Medical School, and
Hospital of the University of Pennsylvania Department of Biostatistics, The School of Public Health
Philadelphia, Pennsylvania 19104 The University of Michigan
Ann Arbor, Michigan 48109
DARRELL D. FANESTIL, M.D.,
Professor of Medicine, THOMAS N. JAMES, M.D.
Head, Division of Nephrology, Professor and Chairman
University of California, San Diego Department of Medicine
La Jolla, California 92037 University of Alabama Medical Center
MICHAEL FIELD, M.D. Birmingham, Alabama 35294
Professor of Medicine
JAMES D. JAMIESON, M.D., PH.D.
University of Chicago
Professor of Cell Biology
Chicago, lllinois 60637
Section of Cell Biology
ALAN FINKELSTEIN, PH.D. Yale University School of Medicine
Professor of Physiology and Biophysics New Haven, Connecticut 06510
and of Neurosciences
Albert Einstein College of Medicine LUKE I. KAO, M.D.
Assistant in Neurology
Bronx, New York 10461
Johns Hopkins School of Medicine
GERHARD H. GIEBISCH, M.D. Baltimore, Maryland 21205
Sterling Professor of Physiology
Yale University School of Medicine KEISHIRO KAWAMURA, M.D.
New Haven, Connecticut 06510 Assistant Professor of Medicine
University of Alabama Medical Center
ALBERT M. GORDON, PH.D. Birmingham, Alabama 35294
Professor of Physiology and Biophysics
University of Washington ROLF KINNE, M.D.
Seattle, Washington 98195 Max Planck Institute for Biophysics
Frankfurt, West Germany
JARED J. GRANTHAM, M.D.
Professor of Medicine SALLY KRASNE, PH.D.
Director, Division of Nephrology Assistant Professor of Physiology
University of Kansas Medical Center Department of Physiology, and
College of Health Sciences and Hospital The Ahmanson Neurobiology Laboratory of the Brain
Kansas City, Kansas 66103 Research Institute
University of California Medical School
GUIDO GUIDOTTI, PH.D. Los Angeles, California 90024
Professor of Biochemistry and Molecular Biology
The Biological Laboratories, Harvard University NORBERT H. LAME IRE, M.D.
Cambridge, Massachusetts 02138 Visiting Research Fellow
from the University of Ghent, Belgium
ROBERT BURNS GUNN, M.D.
Division of Renal Diseases
Associate Professor of Pharmacology, Department of
University of Texas Health Science Center
Pharmacological and Physiological Sciences,
San Antonio, Texas 78284
Pritzker School of Medicine
University of Chicago PETER K. LAUF, M.D.
Chicago, lllinois 60637 Professor of Physiology
Department of Physiology
JOSEPH F. HOFFMAN, PH.D.
Eugene Higgins Professor and Chairman Duke University Medical Center
Department of Physiology Durham, North Carolina 27710
Yale University School of Medicine
ALEXANDER LEAF, M.D.
New Haven, Connecticut 06510
Chief of Medical Services
PAUL HOROWICZ, PH.D. Massachusetts General Hospital, and
Professor and Chairman Jackson Professor of Clinical Medicine
Department of Physiology Harvard University Medical School
University of Rochester School of Medicine and Dentistry Boston, Massachusetts 02114
Rochester, New York 14642
WERNER R. LOEWENSTEIN, PH.D.
C. HUANG, PH.D. Professor and Chairman
Department of Biochemistry Department of Physiology and Biophysics
University of Virginia School of Medicine University of Miami School of Medicine
Charlottesville, Virginia 22901 Miami, Florida 33152
CONTRIBUTORS VB
MARIANNA M. LONG, PH.D. WARREN S. REHM, M.D., PH.D.
Research Coordinator Professor and Chairman
Laboratory of Molecular Biophysics, and Department of Physiology and Biophysics
The Cardiovascular Research and Training Center University of Alabama in Birmingham, The Medical Center
University of Alabama Medical Center Birmingham, Alabama 35294
Birmingham, Alabama 35294
J. DAVID ROBERTSON, M.D., PH.D.
HANS CHRISTOPH LUTTGAU, M.D. Professor and Chairman
Professor of Physiology Department of Anatomy
Ruhr University Duke University Medical Center
Bochum, West Germany Durham, North Carolina 27710
ROBERT I. MACEY, PH.D. ALLEN D. ROSES, M.D.
Professor of Physiology Assistant Professor of Neurology
Department of Physiology-Anatomy Duke University Medical Center
University of California Durham, North Carolina 27710
Berkeley, California 94720
G. SACHS, M.B., CH.B.
ANTHONY D. C. MACKNIGHT, M.D., PH.D. Professor of Medicine
Senior Lecturer Associate Professor of Physiology and Biophysics
Department of Physiology Director, Laboratory of Membrane Biology
The Medical School, University of Otago University of Alabama School of Medicine
Dunedin, New Zealand Birmingham, Alabama 35294
ROY H. MAFFLY, M.D. JAMES A. SCHAFER, PH.D.
Professor of Medicine Professor of Physiology and Biophysics, and
Co-Head, Nephrology Assistant Professor of Medicine, Division of Nephrology
Stanford University Medical Service University of Alabama School of Medicine
Veterans Administration Hospital Birmingham, Alabama 35294
Palo Alto, California 94304
JERRY A. SCHNEIDER, M.D.
JOHN A. MANGOS, M.D. Professor of Pediatrics
Professor of Pediatrics and Basic Dental Sciences University of California, San Diego
Chief, Division of Pulmonology and Cystic Fibrosis School of Medicine
University of Florida Colleges of Medicine and Dentistry La Jolla, California 92093
Gainesville, Florida 32610
MARTIN F. SCHNEIDER, PH.D.
THOMAS J. McMANUS, M.D. Assistant Professor of Physiology
Professor of Physiology and Pharmacology University of Rochester School of Medicine and Dentistry
Duke University Medical Center Rochester, New York 14642
Durham, North Carolina 27706
JOSEPH D. SCHULMAN, M.D.
GEORGE DIMITRIE MOISESCU, M.D. Chief, Section of Human Biochemical and Developmental
Department of Cell Physiology Genetics
Ruhr University National Institute of Child Health and Human
Bochum, West Germany Development
National Institutes of Health
L. GABRIEL NAVAR, PH.D. Bethesda, Maryland 20014
Professor of Physiology and Biophysics
Assistant Professor of Medicine ST ANLEY G. SCHULTZ, M.D.
Department of Physiology and Biophysics Professor of Physiology
University of Alabama School of Medicine University of Pittsburgh School of Medicine
Birmingham, Alabama 35294 Pittsburgh, Pennsylvania 15261
EUGENE P. ORRINGER, M.D. STEVEN SIEGELBAUM, PH.D.
Assistant Professor of Medicine Department of Physiology
University of North Carolina School of Medicine Yale University School of Medicine
Chapel Hill, North Carolina 27514 New Haven, Connecticut 06510
JOHN C. PARKER, M.D. CAROLYN W. SLAYMAN, PH.D.
Professor of Medicine Associate Professor of Human Genetics and Physiology
University of North Carolina School of Medicine Yale University School of Medicine
Chapel Hill, North Carolina 27514 New Haven, Connecticut 06510
JAMES W. PUTNEY, JR., PH.D. JAY H. STEIN, M.D.
Assistant Professor Chairman, Department of Medicine
Department of Pharmacology Director, Division of Renal Diseases
Wayne State University School of Medicine University of Texas Health Science Center
Detroit, Michigan 48201 San Antonio, Texas 78284
Vll1 CONTRIBUTORS
PHILIP R. STEINMETZ, M.D. FERDINAND URTHALER, M.D., F.M.H.
Professor of Medicine Associate Professor of Medicine
Director, Division of Nephrology University of Alabama Medicai Center
University of Iowa College of Medicine Birmingham, Alabama 35294
Iowa City, Iowa 52242
JOHN L. STEPHENSON, M.D., PH.D.
MICHAEL W. WEINER, M.D.
Chief, Section on Theoretical Biophysics
Assistant Professor of Medicine
National Heart, Lung, and Blood Institute
Stanford University Medical Service
National Institutes of Health
Veterans Administration Hospital
Bethesda, Maryland 20014
Palo Alto, California 94304
SAMUEL O. THIER, M.D.
Professor and Chairman
Department of Medicine, Division of Nephrology FREDERICK WHITTIER, M.D.
Yale University School of Medicine Associate Professor of Medicine
New Haven, Connecticut 06510 Veterans Administrati(>n Hospital, Nephrology Division
Kansas City, Missouri 64108 -
THOMAS E. THOMPSON, PH.D.
Professor
Department of Biochemistry
University of Virginia School of Medicine T. HASTINGS WILSON, M.D., PH.D.
Charlottesville, Virginia 22901 Professor
Department of Physiology
RICHARD W. TSIEN, PH.D. Harvard University Medical School
Associate Professor of Physiology Boston, Massachusetts 02115
Yale UDlversity School of Medicine
New Haven, Connecticut 06s10
DAN W. URRY, PH.D. ERICH E. WINDHAGER, M.D.
Professor of Biochemistry Professor and Chairman .
Director, Laboratory of Molecular Biophysics, and Department of Physiology
The Cardiovascular Research and Training Center Career Scientist, Irma HirschI Foundation
University of Alabama Medical Center Cornell University Medical College
Birmingham, Alabama 35294 New York, New York 10021
Preface
The purpose of this book is to provide the reader with a rational frame of reference
for assessing the pathophysiology of those disorders in which derangements of
membrane transport processes are a major factor responsible for the clinical
manifestations of disease. In the present context, we use the term "membrane
transport processes" in a catholic sense, to refer to those molecular processes
whose cardinal function, broadly speaking, is the vectorial transfer of molecules
either individually or as ensembles-across biological interfaces, the latter includ
ing those interfaces which separate different intracellular compartments, the cellu
lar and extracellular compartments, and secreted fluids-such as glomerular fil
trate-and extracellular fluids. Evidently, consideration of these processes, and of
the pathophysiology of membrane disorders, requires an understanding of the
composition and structure of biomembranes, of the physical rules governing mem
brane transport processes, of the way in which chemical regulators-either physio
logic or pharmacologic-regulate or modify membrane transport processes, and of
the ways in which these events are interpreted into specialized phenomena such as
cell volume regulation, signal transmission in excitable tissues, cell-to-cell commu
nication, and secretory processes in epithelia.
Accordingly, Physiology of Membrane Disorders is divided into five major
sections. Part 1, The Nature of Biological Membranes, provides an overview ofthe
physical structure and composition of plasma membranes, and of the dynamic
relations between structure and function. Part II, Methods for Studying Mem
branes, provides insights into the ways, including theoretical, chemical, and physi
cal techniques, in which one may assess the characteristics of membranes and
membrane systems. Part Ill, General Problems in Membrane Biology, furnishes a
general description of events-e.g., active and passive solute permeation, cell
volume regulation, cellular communications, and the effect of drugs, hormones, and
immune reactions on these phenomena-common to most biomembranes. Part IV,
Transport Events in Specialized Cells, Tissues, and Organs, is an account of
transport events unique to certain specialized systems, e.g., unique cell types,
excitable tissues, and various epithelia. Finally, Part V, Clinical Disorders of
Membrane Disorders, utilizes the concepts presented in Parts I-IV to analyze
lX
x
PREFACE
current information and views about the pathophysiology and chemical manifesta
tions of a number of disorders in which aberrancies of membrane structure and/or
function playa major role in disease pathogenesis. Finally, our particular thanks to
Donna Gunnin for her invaluable help in copyediting the book.
THOMAS E. ANDREOLI
JOSEPH F. HOFFMAN
DARRELL D. FANESTIL