Sodium Channels and Neuronal Hyperexcitability
.pdfSodium Channels and Neuronal Hyperexcitability.
Novartis 241
Copyright & 2002 JohnWiley & Sons Ltd
Print ISBN 0-471-48530-6 Online ISBN 0-470-84668-2
SODIUM CHANNELS AND NEURONAL HYPEREXCITABILITY
Sodium Channels and Neuronal Hyperexcitability.
Novartis 241
Copyright & 2002 JohnWiley & Sons Ltd
Print ISBN 0-471-48530-6 Online ISBN 0-470-84668-2
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Sodium Channels and Neuronal Hyperexcitability.
Novartis 241
Copyright & 2002 JohnWiley & Sons Ltd
Print ISBN 0-471-48530-6 Online ISBN 0-470-84668-2
Novartis Foundation Symposium 241
SODIUM CHANNELS AND NEURONAL HYPEREXCITABILITY
2002
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Sodium Channels and Neuronal Hyperexcitability.
Novartis 241
Copyright & 2002 JohnWiley & Sons Ltd
Print ISBN 0-471-48530-6 Online ISBN 0-470-84668-2
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Novartis Foundation Symposium 241 viii+244 pages, 42 ¢gures, 5 tables
Library of Congress Cataloging-in-Publication Data
Sodium channels and neuronal hyperexcitability / [editors], Gregory Bock, Jamie A. Goode. p. cm. ^ (Novartis Foundation symposium ; 241) (Ciba Foundation symposium)
Includes bibliographical references and index. ISBN 0-471-48530-6 (alk. paper)
1. Sodium channels ^Congresses. 2. Molecular neurobiology^Congresses. 3. Nervous
system^Diseases ^Molecular aspects ^Congresses. 4. Neurons ^Congresses. |
I. Bock, |
Gregory. II. Goode, Jamie. III. Series. IV. Series: Ciba Foundation symposium |
|
QP356.2.S65 2001 |
|
612.8’042^dc21 |
2001046625 |
British Library Cataloguing in Publication Data
A catalogue record for this book is available from the British Library
ISBN 0 471 48530 6
Typeset in 1012 on 1212 pt Garamond by DobbieTypesetting Limited,Tavistock, Devon. Printed and bound in Great Britain by Biddles Ltd, Guildford and King’s Lynn.
This book is printed on acid-free paper responsibly manufactured from sustainable forestry, in which at least two trees are planted for each one used for paper production.
Sodium Channels and Neuronal Hyperexcitability.
Novartis 241
Copyright & 2002 JohnWiley & Sons Ltd
Print ISBN 0-471-48530-6 Online ISBN 0-470-84668-2
Contents
Symposium on Sodium channels and neuronal hyperexcitability, held atthe Novartis Foundation, London,14^16 November 2000
Editors: Gregory Bock (Organizer) and Jamie A. Goode
Thissymposiumisbased on aproposalmadeby JohnWood and StephenWaxman in conjunction with theYale^University College London Collaboration
Stephen G.Waxman Chair’s introduction: Sodium channels and neuronal |
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dysfunction . emerging concepts, converging themes |
1 |
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Richard D. Keynes Studies of multimodal gating of the sodium channel |
5 |
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Discussion |
14 |
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Richard Horn Molecular basis for function in sodium channels |
21 |
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Discussion |
26 |
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Stephen G.Waxman,Theodore R. Cummins, Joel A. Black and |
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Sulayman Dib-Hajj Diverse functions and dynamic expression of |
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neuronal sodium channels 34 |
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Discussion |
51 |
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Wayne E. Crill, Peter C. Schwindt and John C. Oakley |
Enhanced transmission |
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of glutamate current £owing from the dendrite to the soma in rat neocortical |
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layer 5 neurons 61 |
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Discussion |
68 |
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Miriam H. Meisler, Jennifer A. Kearney, Leslie K. Sprunger, |
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BryanT. MacDonald, David A. Buchner and Andrew Escayg |
Mutations of |
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voltage-gated sodium channels in movement disorders and epilepsy 72 |
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Discussion |
82 |
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Louis Ptacek Channelopathies: episodic disorders of the nervous system |
87 |
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Discussion |
104 |
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Je¡rey L. Noebels Sodium channel gene expression and epilepsy |
109 |
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Discussion |
120 |
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v
vi |
CONTENTS |
Lori L. Isom b subunits: players in neuronal hyperexcitability? 124
Discussion 138
Stuart Bevan and Nina Storey Modulation of sodium channels in primary a¡erent neurons 144
Discussion 153
John N.Wood, Armen N. Akopian, Mark Baker,Yanning Ding,
Fleur Geoghegan, Mohammed Nassar, Misbah Malik-Hall, Kenji Okuse, Louisa Poon, Samantha Ravenall, Madhu Sukumaran and
Veronika Souslova Sodium channels in primary sensory neurons: relationship to pain states 159
Discussion 168
Michael M. Segal Sodium channels and epilepsy electrophysiology 173
Discussion 180
Gary R. Strichartz, Zhongren Zhou, Catherine Sinnott and Alla Khodorova Therapeutic concentrations of local anaesthetics unveil the potential role of sodium channels in neuropathic pain 189
Discussion 202
William A. Catterall Molecular mechanisms of gating and drug block of sodium channels 206
Discussion 218
Final general discussion 226
Index of contributors 233
Subject index 235
Sodium Channels and Neuronal Hyperexcitability.
Novartis 241
Copyright & 2002 JohnWiley & Sons Ltd
Print ISBN 0-471-48530-6 Online ISBN 0-470-84668-2
Participants
Mark D. Baker Department of Biology, University College London, Medawar Building, Gower Street, LondonWC1E 6BT, UK
Bruce Bean Department of Neurobiology, Harvard Medical School, 220 Longwood Avenue, Boston, MA 02115, USA
Stuart Bevan Novartis Institute for Medical Science, University College London, Gower Street, LondonWC1E 6BT, UK
Hugh Bostock Sobell Department of Neurophysiology, Institute of Neurology, Queen Square, LondonWC1N 3BG, UK
William A. Catterall Department of Pharmacology, F-427 Health Sciences Center, University of Washington, Seattle,WA 98195-7280, USA
Wayne Crill Department of Physiology and Biophysics, University of Washington, 1959 NE Paci¢c Street, HSB Room G424, Box 357290, Seattle, WA 98195-7290, USA
Ted R. Cummins Department of Neurology,Yale University, New Haven, CT 06520, USA
Michael S. Gold Oral and Craniofacial Biological Sciences, University of Maryland Dental School, 666 W. Baltimore Street, Baltimore, MD 21201, USA
Alan L. Goldin Department of Microbiology & Molecular Genetics, University of California at Irvine, Irvine, CA 92697-4025, USA
Richard Horn Department of Physiology, Institute of Hyperexcitability, Je¡erson Medical College, 1020 Locust Street, Philadelphia, PA 19107, USA
vii
viii |
PARTICIPANTS |
Lori L. Isom Department of Pharmacology,The University of Michigan Medical School, 1301MSRB III, 1150 W. Medical Center Drive, Ann Arbor, MI 48109-0632, USA
Richard Keynes Department of Physiology, University of Cambridge, Downing Street, Cambridge CB2 3EG, UK
Miriam Meisler Department of Human Genetics, University of Michigan Medical School, Ann Arbor, MI 48109-0618, USA
Je¡rey L. Noebels Department of Neurology, Baylor College of Medicine, One Baylor Plaza, Houston,TX 77030, USA
Louis Ptacek Howard Hughes Medical Institute, University of Utah School of Medicine, Room 4425, Salt Lake City, UT 84112-5331, USA
Indira M. Raman Department of Neurobiology and Physiology, Northwestern University, 2153 North Campus Drive, Evanston, Illinois 60208-3520, USA
Michael Segal Harvard Medical School and Brigham & Women’s Hospital, Longwood Medical Research Center, 221 Longwood Ave, Boston MA 02115, USA
Nelson Spruston Department of Neurobiology and Physiology, Northwestern University, 2153 N. Campus Drive, Evanston, IL 60208-3520, USA
Gary R. Strichartz Pain Research Center, Harvard Medical School, Anaesthesia Research Laboratories, Medical Research Building 611, Brigham and Women’s Hospital, 75 Francis Street, Boston, MA 02115, USA
StephenWaxman (Chair) Department of Neurology,Yale Medical School, 333 Cedar Street, LCI-707, New Haven, CT 06510, USA
JohnWood Department of Biology, University College London, Medawar Building, Gower Street, LondonWC1E 6BT, UK
Sodium Channels and Neuronal Hyperexcitability.
Novartis 241
Copyright & 2002 JohnWiley & Sons Ltd
Print ISBN 0-471-48530-6 Online ISBN 0-470-84668-2
Subject index
A |
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accessory subunits |
161^162 |
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see also b subunits |
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acetazolamide (Diamox) |
91, 93, 231 |
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acetylcholine (ACh) |
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95, 96 |
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see also nicotinic acetylcholine receptor |
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activation |
22 |
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b scorpion toxin action 209, 210 |
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coupling to inactivation |
211^213 |
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gate immobilization |
24^25, 27 |
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hyperpolarized dependence |
38 |
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molecular basis |
31, 208^209, 210 |
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Ad ¢bres |
161 |
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adenosine |
149, 178 |
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adenovirus vectors |
165 |
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allodynia, mechanical |
191, 192, 204 |
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a scorpion toxins |
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binding sites 207 |
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local anaesthetic actions and |
192^194, 204 |
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mechanism of action |
213, 214, 222^224 |
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a subunits |
2, 89, 206^207 |
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interaction site with b subunits 31^32, |
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126^128 |
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modulation by b subunits |
124^125 |
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nomenclature |
3 |
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phosphorylation |
145 |
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in sensory neurons |
160 |
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D-2-amino-5-phosphonopentoic acid see APV amygdala
in epileptogenesis 114 SCN5A localization 112, 113
analgesics, future prospects 161^162 Andersen’s syndrome 107
ankyrin 129^130, 134, 142 ankyrinG 133, 135 antagonists, Na+ channel
anticonvulsant activity 176^177 molecular mechanisms 213^216, 218^222 in neuropathic pain 189^205
novel 216, 219
anti-arrhythmic drugs 186, 215, 223
anticonvulsants
binding site 183^184, 215
mechanisms of action 176^178, 184^185, 186, 223
antisense Nav1.8 (SNS, PN3) 170^171, 190
APV 62, 63, 64, 175, 185^186 astrocytes 52, 123, 134, 135, 142 ataxia
episodic see episodic ataxia
mutant mouse models 45^48, 76^78, 94^95
progressive cerebellar (SCA6) 94, 99 axotomized neurons 42^45, 46, 51^52, 53,
55^59
B
BIII 890 CL (novel Na+ channel blocker)
216, 219 |
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BAC clones |
86 |
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ba¢lomycin A |
152 |
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bed nucleus of stria terminalis 112 |
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benzophenone-4-carboxamidocysteine |
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methanethiosulfonate (BPMTS) |
23, 24, |
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26^27, 28, 29, 30^31 |
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b scorpion toxins 207, 208^209, 210, 213, |
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222^224 |
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b subunits |
2, 89, 124^143, 161, 206 |
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a subunit interaction site |
31^32, 126^128 |
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in cardiac myocytes 130^131 |
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evolution |
229 |
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function as CAMs |
128^130, 208 |
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in heterologous expression systems |
125 |
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homophilic interactions |
129^130 |
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in human disease |
131^133 |
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modulation of channel gating |
124^125 |
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mutations |
107 |
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phosphorylation |
139^140, 141^142 |
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structural homology to CAMs |
126 |
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structure |
207^208 |
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see also speci¢c subtypes |
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235
236
b1 subunits 124^125, 126, 161 a scorpion toxin and 222^223 in cardiac physiology 131 function as CAMs 129^130
in heterologous expression systems 125 Ig loop region in a subunit interactions
126^128 |
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mutations |
110^111 |
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in neuropathic pain |
133 |
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NGF e¡ects |
162 |
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structure |
207^208 |
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b1A subunits |
125, 126, 161 |
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b2 subunits |
124^125, 126, 161 |
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in cardiac physiology |
131 |
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function as CAMs |
128, 129^130 |
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knockout mice |
142 |
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in neuropathic pain |
133 |
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structure |
207^208 |
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b3 subunit 125, 126, 161 |
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Bezanilla’s crevices |
9^11, 23, 32^33 |
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bicarbonate ions |
231 |
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biolistic gene gun |
164 |
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blockers, Na+ channel see antagonists, Na+ channel
brefeldin 157 8-bromo-cAMP 146^147, 149
Brugada syndrome 123, 132^133 a-bungarotoxin 96
bursting
frog skeletal muscle channels 11, 13, 16^17
neuronal 17, 178, 204
C
C-¢bres 53, 59, 161 CA1 neurons 71, 73^74 Ca2+
intracellular, DRG neurons 156, 157 spikes 69, 70^71, 88
zero 185^186
Ca2+ channel blockers 69 Ca2+ channels 229
b subunits 125 L-type 88, 213
mutations 92, 94^95, 99 neocortical layer 5 neurons 68^69 structure 22, 88^89
CACNA1A mutations 94, 100^101 cadmium (Cd2+) 36, 37, 38, 71
SUBJECT INDEX
Caenorhabditis elegans 229 CAG repeat expansions 94 calcineurin 145
calcium see Ca2+
CAMs see cell adhesion molecules carbamazepine 186
carbonic anhydrase inhibitors 91, 93, 230, 231
cardiac glycosides 122 cardiac myocytes
b subunits 130^131
Na+ channel targeting and clustering 135
cartwheel cells, dorsal cochlear nucleus 76
cell adhesion molecules (CAMs) |
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b subunit function |
128^130, 208 |
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L1 family |
129, 130, 142 |
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nodes of Ranvier |
133^135, 138 |
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structural homology of b subunits |
126 |
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cell migration, b subunits and |
128^129 |
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cellular aggregation, b subunits and |
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129^130 |
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channelopathies |
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acquired |
2 |
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inherited see inherited channelopathies |
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chloroquine |
152 |
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choreoathetosis, paroxysmal dystonic |
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101 |
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chronic constriction injury |
133, 163 |
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Cl7 channels |
87^88 |
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mutations |
92^93, 99 |
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structure |
89 |
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CLCN1 mutations |
92^93 |
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CNQX |
175, 185^186 |
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Cole^Moore e¡ect |
32 |
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m conotoxin |
223 |
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contactin |
126, 134, 135, 138 |
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convulsions see seizures |
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cooperativity, S4 segment domains |
15^16, |
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29 |
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cornea 160^161 |
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Cre/lox-P system |
164^165 |
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crevices, Bezanilla’s |
9^11, 23, 32^33 |
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cultures, microisland |
174^178 |
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cyclic AMP (cAMP) |
145^147, 149, 162 |
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cycloheximide |
158 |
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cysteine accessibility scanning |
8^9, 22^23, |
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31^32, 209, 211 |
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cytoplasmic loop domains |
229 |
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cytoskeletal interactions 130, 133^135, 139, 228