Ciba Foundation Symposium 126 - Selective Neuronal Death

280 Pages·9.421 MB·English

SELECTIVE
NEURONAL
DEATH
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Ciba Foundation Symposium 126
SELECTIVE
NEURONAL
DEATH
A Wiley - lnterscience Publication
1987
JOHN WlLEY 8, SONS
____ ~~-
Chichester .New York . Brisbane . Toronto . Singapore
0C iba Foundation 1987
Published in 1987 by John Wiley & Sons Ltd, Baffins Lane.
Chichester, Sussex PO19 lUD, UK.
Suggested series entry for library catalogues:
Ciba Foundation Symposia
Ciba Foundation Symposium 126
x + 271 pages, 30 figures, 16 tables
Library of Congress Cataloging.in-Publication Data
Selective neuronal death.
(Ciba Foundation symposium ; 126)
Papers from a symposium on selective neuronal death,
held at the Ciba Foundation, London, 15-17 April 1986.
Editors: Gregory Bock and Maeve O’Connor.
‘A Wiley-Interscience publication.’
Includes indexes.
1. Neurons-Congresses. 2. Cell death-Congresses.
3. Nervous system-Degeneration-Congresses.
I. Bock, Gregory. 11. O’Connor, Maeve. 111. Ciba
Foundation. IV. Series. [DNLM: 1. Cell Survival-
congresses. 2. Nerve Degeneration-congresses.
3. Neurons-physiology-congresses.
W3 C161F v. 126 I WL]
QP363. S46 1987 611 ‘ ,01815 86 - 28 0 8 2
ISBN 0 471 91092 9
British Library Cataloguing in Publication Data:
Selective neuronal death. - (Ciba
Foundation symposium ; 126)
1. Nervous system - Degeneration
1. Series
591.87‘65 QP363
ISBN 0 471 91092 9
Printed and bound in Great Britain.
Contents
Symposium on Selective neuronal death, held at the Ciba Foundation, London,
15-1 7 April 1986
This symposium is based on a proposalmade by Professor Alan Davison
Edirors: Gregory Bock (Organizer) and Maeve O’Connor
H.M. Wisniewski Introduction 1
Y. Agid and J. Blin Nerve cell death in degenerative diseases of the central
nervous system: clinical aspects 3
Discussion 19
D.L. Price, L.C. Cork, R.G. Struble, C.A. Kitt, L.C. Walker, R.E. Powers,
P.J. Whitehouse and J.W. Griftin Dysfunction and death of neurons in
human degenerative neurological diseases and in animal models 30
Discussion 43
C.L. Masters and K. Beyreuther Neuronal origin of cerebral amyloidogenic
proteins: their role in Alzheimer’s disease and unconventional virus diseases
of the nervous system 49
Discussion 58
S.E. Fahrbach and J.W. Truman Mechanisms for programmed cell death in
the nervous system of a moth 65
Discussion 76
H. Thoenen, Y.-A. Barde, A.M. Davies and J.E. Johnson Neurotrophic
factors and neuronal death 82
Discussion 91
R.W. Oppenheim Muscle activity and motor neuron death in the spinal cord
of the chick embryo 96
Discussion 108
VI CONTENTS
D.D.M. O’Leary Remodelling of early axonal projections through the
selective elimination of neurons and long axon collaterals 113
Discussion 130
F.H. Gage and A. Bjorklund Trophic and growth-regulating mechanisms in
the central nervous system monitored by intracerebral neural transplants
143
Discussion 155
L.C. Doering and A.J. Aguayo Cytoskeletal abnormalities in long-term
embryonic CNS transplants isolated within peripheral nerve 160
Discussion 169
M. Konishi and E. Akutagawa Hormonal control of cell death in a sexually
dimorphic song nucleus in the zebra finch 173
Discussion 180
J.T. Coyle Kainic acid: insights into excitatory mechanisms causing selective
neuronal degeneration 186
Discussion 198
T.W. Stone, J.H. Connick, P. Winn, M.H. Hastings and M. English
Endogenous excitotoxic agents 204
Discussion 214
P.S. Spencer, J. Hugon, A. Ludolph, P.B. Nunn, S.M. Ross, D.N. Roy and
H.H. Schaumburg Discovery and partial characterization of primate
motor-system toxins 221
Discussion 23 1
C.D. Marsden and P.G. Jenner The significance of l-methyl-4-phenyl-
1,2,3,6-tetrahydropyridine 239
Discussion 250
H.M. Wisniewski Summary 257
Index of contributors 260
Subject index 262
Participants
Y. Agid Laboratoire de Medecine Experimentale et Clinique de Neurologie
et Neuropsychologie, CHU Pitie-Salpetriere, 91 Boulevard de l’H6pita1,
75634 Paris Cedex 13, France
M. J. Ball Department of Pathology, Health Sciences Centre, University of
Western Ontario, London N6A XI,C anada
A. Bjorklund Department of Histology, University of Lund, Biskopsgatan 5,
S-223 62 Lund, Sweden
W.G. Bradley Department of Neurology, University of Vermont College of
Medicine, 1S outh Prospect Street, Burlington, Vermont 05405, USA
J.T. Coyle Department of Psychiatry, Division of Child Psychiatry, The
Johns Hopkins University School of Medicine, 600 North Wolfe Street,
Baltimore, Maryland 21205, USA
A.R. Crossman Department of Anatomy, University of Manchester Medical
School, Oxford Road, Manchester M13 9PT, UK
A.N. Davison Department of Neurochemistry, Institute of Neurology, The
National Hospital, Queen Square, London WC13BG. UK
L.C. Doering Neurosciences Unit, McGill University, Montreal General
Hospital Research Institute, 1650 Cedar Avenue, Montreal, Quebec
H3G 1A4, Canada
D. Ehrlich (Ciba Foundation Bursar) Department of Anatomy, Monash
University, Clayton, Victoria, Australia 3168
S. Fahrbach Department of Zoology, NJ-15, University of Washington,
Seattle, Washington 98195, USA
vii
viii PARTICIPANTS
S.D. Iversen Neuroscience Research Centre, Merck Sharp & Dohme
Research Laboratories, Tsrlings Park, Eastwick Road, Harlow, Essex
CM20 2QR, UK
J.F.R. Kerr Department of Pathology, University of Queensland, Medical
School, Herston, Queensland 4006, Australia
M. Konishi Division of Biology 216-76, California Institute of Technology,
Pasadena, California 91125, USA
C.D. Marsden Department of Neurology, Institute of Psychiatry & King’s
College Hospital Medical School, De Crespigny Park, Denmark Hill,
London SE5 8AF, UK
C.L. Masters Department of Neuropathology, Royal Perth Hospital, Box X
2213, GPO, Perth, Western Australia 6001
D.D.M. O’Leary Department of Neurosurgery, Box 8057, Washington
University School of Medicine, 660 South Euclid Avenue, St Louis,
Missouri 63110, USA
J.W. Olney Department of Psychiatry, Washington University School of
Medicine, Medical Center, 4940 Audubon Avenue, St Louis, Missouri
63110, USA
R.W. Oppenheim Department of Anatomy, Wake Forest University,
Bowman Gray School of Medicine, 300 South Hawthorne Road, Winston-
Salem, North Carolina, North Carolina 27103, USA
D.L. Price Neuropathology Laboratory, 509 Pathology Building, The Johns
Hopkins University School of Medicine, 600 North Wolfe Street,
Baltimore, Maryland 21205-2182, USA
G. Raisman Laboratory of Neurobiology and Development, MRC National
Institute for Medical Research, The Ridgeway, Mill Hill, London
NW7 lAA, UK
M. Rossor The National Hospital, Queen Square, London WClN 3BG, UK
R. Schwarcz Maryland Psychiatric Research Center, PO Box 21247,
Baltimore, MD 21228, USA
P.S. Spencer Departments of Neuroscience, Neurology and Pathology,
PARTICIPANTS ix
Institute of Neurotoxicology, Albert Einstein College of Medicine of
Yeshiva University, 1300 Morris Park Avenue, Bronx, New York 10461,
USA
T.W. Stone Department of Physiology, St George’s Hospital School of
Medicine, Cranmer Terrace, London SW17 ORE, UK
H. Thoenen Department of Neurochemistry, Max Planck Institute for
Psychiatry, Am Klopferspitz MA, 8033 Planegg-Martinsried, Federal
Republic of Germany
H.M. Wisniewski (Chairman)I nstitute for Basic Research in
Developmental Disabilities, 1050 Forest Hill Road, Staten Island, New
York 10314, LJSA
Novartis Foundation Symposium
Edited by GregoIy Bock, Maeve O’Connor
Copyright 0 1987 by Ciba Foundation
Introduction
HENRYK M. WlSNlEWSKl
lnstitufe for Basic Research in Developmental Disabilities, 1050 forest Hill Road, Stafen
Island, NY 10314. USA
1987 Selective neiironal death. Wiley, Chichester (Ciba Foundation Symposium 126)
p 1-2
Among the questions we will address at this symposium on selective neuronal
death are:
(1) Why do certain neurons die?
(2) What are the causes of neuronal death during development, sexual
differentiation, and in chronic neurodegenerative diseases?
(3) What do we know about the rate of neuronal death during development
and in chronic brain diseases?
(4) Are all susceptible nerve cells ‘sick’ from the day the disease affects a
given area of the brain, or do they become ‘sick’ because of continuous
exposure to causative agents such as endotoxins or exotoxins?
(5) Why do some neurons die immediately and others over a period of days,
weeks or months?
(6) How many neurons have to die before clinical signs and symptoms occur?
(7) What are the factors which govern brain growth and regeneration?
(8) Can we expect that brain transplants will replace lost neurons?
Any consideration of how the CNS operates must take into account the
high degree of structural and functional specialization that is built into the
brain. In almost every other organ in the body, there is a very direct relation-
ship between structure and function (e.g. muscle mass and strength). There is
a basic cell or group of cells that serves as the fundamental functional unit.
The total functional capacity of the organ then becomes a matter of how many
of these units there are. While it is true that the neuron is the basic functional
unit of the nervous system, the mosaic of behaviours and capacities embodied
in the nervous system cannot be accounted for on a simple additive basis.
Because of the unique architecture of the nervous system it is best regarded as
a ‘multiorgan organ’. Unlike most other organs, the brain is responsible for
carrying out the integrated expression of many different functions. This is
accomplished in several ways. Many of the functions are separated anatomic-
ally in different regions of the brain (e.g. auditory and visual regions). How-
ever, even this regional specialization is not sufficient to account for all the
varieties of brain activity. It is evident that in the brain a given function is