Madness and memory: the discovery of prions--a new biological principle of disease

343 Pages·2014·7.256 MB·English

by  Prusiner, Stanley B

Madness and Memory
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The Discovery of Prions—
A New Biological Principle
of Disease
STANLEY B. PRUSINER, M.D.
Copyright © 2014 by Stanley B. Prusiner, M.D.
All rights reserved.
Th is book may not be reproduced, in whole or in part, including illustrations, in any
form (beyond that copying permitted by Sections 107 and 108 of the U.S. Copyright
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Designed by Mary Valencia.
Set in Minion and Futura type by Westchester Book Group, Danbury, CT.
Printed in the United States of America.
Library of Congress Cataloging- in- Publication Data
Prusiner, Stanley B., 1942–, author.
Madness and memory : the discovery of prions— a new biological principle of
disease / Stanley B. Prusiner.
p. cm.
Includes bibliographical references and index.
ISBN 978- 0- 300-1 9114- 1 (cloth : alk. paper)
I. Title.
[DNLM: 1. Prusiner, Stanley B., 1942–. 2. Physicians— Autobiography.
3. History, 20th Century. 4. History, 21st Century. 5. Prions— history. WZ 100]
R690
610.92—dc23
[B]
2013041191
A cata logue record for this book is available from the British Library.
Th is paper meets the requirements of ANSI/NISO Z39.48- 1992 (Permanence of
Paper).
10 9 8 7 6 5 4 3 2 1
To scientifi c investigation that
creates new knowledge,
erases ignorance,
eradicates prejudice,
prevents disease,
alleviates suff ering,
and enhances well- being
To Sunnie, Helen, Leah, and William
In striving to do scientifi c work, the chance— even for very gift ed
persons— to achieve something of real value is very small.
—Albert Einstein
Of all the ser vices that can be rendered to science, the introduction
of new ideas is the greatest.
—J. J. Th omson
Th e true men of action in our time, those who transform the world,
are not the politicians and statesmen, but the scientists. Unfortu-
nately, poetry cannot celebrate them, because their deeds are
concerned with things, not persons, and are, therefore, speechless.
When I fi nd myself in the company of scientists, I feel like a shabby
curate who has strayed by mistake into a drawing room full of dukes.
—W. H. Auden
Th e only good histories are those that have been written by the
persons themselves who commanded in the aff airs whereof they
write.
—Michel de Montaigne
A man convinced against his will, is of the same opinion still.
—Samuel Butler
Contents
Preface ix
Introduction xiii
Author’s Note xix
1 Growing Up 1
2 Th e Beginning of an Odyssey 1 6
3 A Plethora of Th eories 26
4 Th e Scrapie Race 3 6
5 Dr. America and the Trembling Cannibals 5 0
6 Th e Battle for Tenure 6 5
7 What’s in a Name? 8 1
8 Lost in the Pacifi c Fog 9 7
9 Th e Amyloid Story 108
10 Finding the Gene 1 25
11 Jousting with the Press 1 43
12 Deciphering Human Prion Diseases 1 53
13 What’s in a Shape? 1 70
14 Turf Battles 1 84
15 Mad Cow and Other Prion Strains 1 93
16 Stockholm 2 13
17 Th e Th ird Judgment of Paris 232
Epilogue: Th e Quest for Th erapeutics 2 53
Notes 2 61
Glossary 2 83
Ac know ledg ments 299
Illustration Credits 303
Index 3 05
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Preface
“What if I’m right aft er all?” I said to myself as I exited an elevator on
the seventh fl oor of the Health Sciences East building at the University of
California, San Francisco, one morning in October 1986. Four years had
passed since my introduction of the word “prion” had elicited scorn, out-
rage, and disbelief from the scientifi c community. But one nugget of data
aft er another was supporting my hypothesis— I was beginning to con-
vince scholars that my novel perspective might have merit.
Th irty- fi ve years aft er Th omas Kuhn wrote his remarkable analysis
on the structure of scientifi c revolutions, Clayton Christensen wrote
about the technological equivalent, which he labeled “disruptive technol-
ogies or innovations.”1 Christensen divided new technologies into two
categories: sustaining and disruptive. Sustaining technologies rely on
incremental improvements or small advances to nourish an established
technology. In contrast, disruptive technologies are transformational
and discontinuous. Th ey create disorder by upsetting the status quo and
are oft en greeted with profound skepticism. Some have destroyed entire
industries— an example is digital photography, which Steven Sasson in-
vented in 1975, while working at Eastman Kodak. On November 17, 2009,
President Barack Obama awarded Sasson the National Medal of Technol-
ogy and Innovation at a ceremony in the East Room of the White House.
I had the privilege of meeting Sasson because I received the National
Medal of Science on the same occasion.
Th irty- seven years aft er Sasson’s disruptive innovation, Kodak fi led
for bankruptcy— its executives had numerous golden opportunities to
capitalize on Sasson’s invention; they possessed neither the necessary wis-
dom nor the uncommon vision. Digital photography did not fi t the Kodak
business model; how could the company survive if it were to abandon
photographic fi lm? Chemicals, fi lm, photographic paper, and inexpensive
cameras had become the heart and soul of a worldwide business with rev-
enues of billions of dollars annually.2 Th ere is a strange irony to the Kodak
ix