图解生物化学（英文版）Harper's Illustrated Biochemistry(26th.pdf

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1、Harper s Illustrated Biochemistry a LANGE medical book twenty sixth edition Robert K Murray MD PhD Professor Emeritus of Biochemistry University of Toronto Toronto Ontario Daryl K Granner MD Joe C Davis Professor of Biomedical Science Director Vanderbilt Diabetes Center Professor of Molecular Physio

2、logy and Biophysics and of Medicine Vanderbilt University Nashville Tennessee Peter A Mayes PhD DSc Emeritus Professor of Veterinary Biochemistry Royal Veterinary College University of London London Victor W Rodwell PhD Professor of Biochemistry Purdue University West Lafayette Indiana Lange Medical

3、 Books McGraw Hill Medical Publishing Division New York Chicago San Francisco Lisbon London Madrid Mexico City Milan New Delhi San Juan Seoul Singapore Sydney Toronto Harper s Illustrated Biochemistry Twenty Sixth Edition Copyright 2003 by The McGraw Hill Companies Inc All rights reserved Printed in

4、 the United States of America Except as permitted under the United States Copyright Act of 1976 no part of this publication may be reproduced or distributed in any form or by any means or stored in a data base or retrieval system without the prior written permission of the publisher Previous edition

5、s copyright 2000 1996 1993 1990 by Appleton copyright 1988 by Lange Medical Publications 2 3 4 5 6 7 8 9 0 DOC DOC 0 9 8 7 6 5 4 3 ISBN 0 07 138901 6 ISSN 1043 9811 Notice Medicine is an ever changing science As new research and clinical experience broaden our knowledge changes in treat ment and dru

6、g therapy are required The authors and the publisher of this work have checked with sources believed to be reliable in their efforts to provide information that is complete and generally in accord with the standards accepted at the time of publication However in view of the possibility of human erro

7、r or changes in medical sciences neither the authors nor the publisher nor any other party who has been involved in the preparation or publication of this work warrants that the information contained herein is in every respect accurate or complete and they disclaim all responsibility for any er rors

8、 or omissions or for the results obtained from use of the information contained in this work Readers are encouraged to confirm the information contained herein with other sources For example and in particular readers are advised to check the product information sheet included in the package of each

9、drug they plan to administer to be certain that the information contained in this work is accurate and that changes have not been made in the recommended dose or in the contraindications for administration This recommendation is of particular importance in connection with new or infre quently used d

10、rugs This book was set in Garamond by Pine Tree Composition The editors were Janet Foltin Jim Ransom and Janene Matragrano Oransky The production supervisor was Phil Galea The illustration manager was Charissa Baker The text designer was Eve Siegel The cover designer was Mary McKeon The index was pr

11、epared by Kathy Pitcoff RR Donnelley was printer and binder This book is printed on acid free paper ISBN 0 07 121766 5 International Edition Copyright 2003 Exclusive rights by the McGraw Hill Companies Inc for manufacture and export This book cannot be re exported from the country to which it is con

12、signed by McGraw Hill The International Edition is not available in North America Authors David A Bender PhD Sub Dean Royal Free and University College Medical School Assistant Faculty Tutor and Tutor to Med ical Students Senior Lecturer in Biochemistry De partment of Biochemistry and Molecular Biol

13、ogy University College London Kathleen M Botham PhD DSc Reader in Biochemistry Royal Veterinary College University of London Daryl K Granner MD Joe C Davis Professor of Biomedical Science Director Vanderbilt Diabetes Center Professor of Molecular Physiology and Biophysics and of Medicine Vander bilt

14、 University Nashville Tennessee Frederick W Keeley PhD Associate Director and Senior Scientist Research Insti tute Hospital for Sick Children Toronto and Pro fessor Department of Biochemistry University of Toronto Peter J Kennelly PhD Professor of Biochemistry Virginia Polytechnic Insti tute and Sta

15、te University Blacksburg Virginia Peter A Mayes PhD DSc Emeritus Professor of Veterinary Biochemistry Royal Veterinary College University of London Robert K Murray MD PhD Professor Emeritus of Biochemistry University of Toronto Margaret L Rand PhD Scientist Research Institute Hospital for Sick Chil

16、dren Toronto and Associate Professor Depart ments of Laboratory Medicine and Pathobiology and Department of Biochemistry University of Toronto Victor W Rodwell PhD Professor of Biochemistry Purdue University West Lafayette Indiana P Anthony Weil PhD Professor of Molecular Physiology and Biophysics V

17、anderbilt University School of Medicine Nash ville Tennessee vii Preface ix The authors and publisher are pleased to present the twenty sixth edition of Harper s Illustrated Biochemistry Review of Physiological Chemistry was first published in 1939 and revised in 1944 and it quickly gained a wide re

18、adership In 1951 the third edition appeared with Harold A Harper University of California School of Medicine at San Fran cisco as author Dr Harper remained the sole author until the ninth edition and co authored eight subsequent edi tions Peter Mayes and Victor Rodwell have been authors since the te

19、nth edition Daryl Granner since the twentieth edition and Rob Murray since the twenty first edition Because of the increasing complexity of biochemical knowl edge they have added co authors in recent editions Fred Keeley and Margaret Rand have each co authored one chapter with Rob Murray for this an

20、d previous edi tions Peter Kennelly joined as a co author in the twenty fifth edition and in the present edition has co authored with Victor Rodwell all of the chapters dealing with the structure and function of proteins and enzymes The follow ing additional co authors are very warmly welcomed in th

21、is edition Kathleen Botham has co authored with Peter Mayes the chapters on bioenergetics biologic oxidation oxidative phosphorylation and lipid metabolism David Bender has co authored also with Peter Mayes the chapters dealing with carbohydrate metabolism nutrition diges tion and vitamins and miner

22、als P Anthony Weil has co authored chapters dealing with various aspects of DNA of RNA and of gene expression with Daryl Granner We are all very grateful to our co authors for bringing their ex pertise and fresh perspectives to the text CHANGES IN THE TWENTY SIXTH EDITION A major goal of the authors

23、 continues to be to provide both medical and other students of the health sciences with a book that both describes the basics of biochemistry and is user friendly and interesting A second major ongoing goal is to reflect the most significant advances in biochemistry that are important to medicine Ho

24、wever a third major goal of this edition was to achieve a substantial reduction in size as feedback indicated that many readers pre fer shorter texts To achieve this goal all of the chapters were rigorously edited involving their amalgamation division or dele tion and many were reduced to approximat

25、ely one half to two thirds of their previous size This has been effected without loss of crucial information but with gain in conciseness and clarity Despite the reduction in size there are many new features in the twenty sixth edition These include A new chapter on amino acids and peptides which em

26、phasizes the manner in which the properties of biologic peptides derive from the individual amino acids of which they are comprised A new chapter on the primary structure of proteins which provides coverage of both classic and newly emerging proteomic and genomic methods for identifying proteins A n

27、ew section on the application of mass spectrometry to the analysis of protein structure has been added including comments on the identification of covalent modifica tions The chapter on the mechanisms of action of enzymes has been revised to provide a comprehensive description of the various physica

28、l mechanisms by which enzymes carry out their catalytic functions The chapters on integration of metabolism nutrition digestion and absorption and vitamins and minerals have been completely re written Among important additions to the various chapters on metabolism are the following update of the inf

29、ormation on oxidative phosphorylation including a description of the rotary ATP synthase new insights into the role of GTP in gluconeogenesis additional information on the regulation of acetyl CoA carboxylase new information on receptors involved in lipoprotein metabolism and reverse cholesterol tra

30、nsport discussion of the role of leptin in fat storage and new information on bile acid regulation including the role of the farnesoid X receptor FXR The chapter on membrane biochemistry in the previous edition has been split into two yielding two new chapters on the structure and function of membra

31、nes and intracellular traffic and sorting of proteins Considerable new material has been added on RNA synthesis protein synthesis gene regulation and various as pects of molecular genetics Much of the material on individual endocrine glands present in the twenty fifth edition has been replaced with

32、new chapters dealing with the diversity of the endocrine system with molecular mechanisms of hormone action and with signal transduction The chapter on plasma proteins immunoglobulins and blood coagulation in the previous edition has been split into two new chapters on plasma proteins and immunoglob

33、ulins and on hemostasis and thrombosis New information has been added in appropriate chapters on lipid rafts and caveolae aquaporins connexins dis orders due to mutations in genes encoding proteins involved in intracellular membrane transport absorption of iron and conformational diseases and pharma

34、cogenomics A new and final chapter on The Human Genome Project HGP has been added which builds on the material covered in Chapters 35 through 40 Because of the impact of the results of the HGP on the future of biology and medicine it appeared appropriate to conclude the text with a summary of its ma

35、jor findings and their implica tions for future work As initiated in the previous edition references to useful Web sites have been included in a brief Appendix at the end of the text ORGANIZATION OF THE BOOK The text is divided into two introductory chapters Biochemistry vitamins and minerals intrac

36、ellular traffic and sorting of proteins glycoproteins the extracellular matrix muscle and the cy toskeleton plasma proteins and immunoglobulins hemostasis and thrombosis red and white blood cells the me tabolism of xenobiotics and the Human Genome Project ACKNOWLEDGMENTS The authors thank Janet Folt

37、in for her thoroughly professional approach Her constant interest and input have had a significant impact on the final structure of this text We are again immensely grateful to Jim Ransom for his excel lent editorial work it has been a pleasure to work with an individual who constantly offered wise

38、and informed alter natives to the sometimes primitive text transmitted by the authors The superb editorial skills of Janene Matragrano Oransky and Harriet Lebowitz are warmly acknowledged as is the excellent artwork of Charissa Baker and her col leagues The authors are very grateful to Kathy Pitcoff

39、 for her thoughtful and meticulous work in preparing the Index Suggestions from students and colleagues around the world have been most helpful in the formulation of this edition We look forward to receiving similar input in the future Robert K Murray MD PhD Daryl K Granner MD Peter A Mayes PhD DSc

40、Victor W Rodwell PhD Toronto Ontario Nashville Tennessee London West Lafayette Indiana March 2003 x PREFACE Contents iii Authors vii Preface ix 1 Biochemistry these are described in later chapters and established that these conditions were genetically determined Garrod desig nated these conditions a

41、s inborn errors of metabo lism His insights provided a major foundation for the development of the field of human biochemical genet ics More recent efforts to understand the basis of the genetic disease known as familial hypercholesterol emia which results in severe atherosclerosis at an early age h

42、ave led to dramatic progress in understanding of cell receptors and of mechanisms of uptake of choles terol into cells Studies of oncogenes in cancer cells have directed attention to the molecular mechanisms involved in the control of normal cell growth These and many other examples emphasize how th

43、e study of INTRODUCTION Biochemistry can be defined as the science concerned with the chemical basis of life Gk bios life The cell is the structural unit of living systems Thus biochem istry can also be described as the science concerned with the chemical constituents of living cells and with the re

44、ac tions and processes they undergo By this definition bio chemistry encompasses large areas of cell biology of molecular biology and of molecular genetics The Aim of Biochemistry Is to Describe some of them are summarized in Table 1 1 A Knowledge of Biochemistry Is Essential to All Life Sciences Th

45、e biochemistry of the nucleic acids lies at the heart of genetics in turn the use of genetic approaches has been critical for elucidating many areas of biochemistry Physiology the study of body function overlaps with biochemistry almost completely Immunology employs numerous biochemical techniques a

46、nd many immuno logic approaches have found wide use by biochemists Pharmacology and pharmacy rest on a sound knowl edge of biochemistry and physiology in particular most drugs are metabolized by enzyme catalyzed reac tions Poisons act on biochemical reactions or processes this is the subject matter

47、of toxicology Biochemical ap proaches are being used increasingly to study basic as pects of pathology the study of disease such as in flammation cell injury and cancer Many workers in microbiology zoology and botany employ biochemical approaches almost exclusively These relationships are not surpri

48、sing because life as we know it depends on biochemical reactions and processes In fact the old barriers among the life sciences are breaking down and 2 CHAPTER 1 disease can open up areas of cell function for basic bio chemical research The relationship between medicine and biochem istry has importa

49、nt implications for the former As long as medical treatment is firmly grounded in a knowledge of biochemistry and other basic sciences the practice of medicine will have a rational basis that can be adapted to accommodate new knowledge This contrasts with unorthodox health cults and at least some al

50、ternative medicine practices which are often founded on little more than myth and wishful thinking and generally lack any intellectual basis NORMAL BIOCHEMICAL PROCESSES ARE THE BASIS OF HEALTH The World Health Organization WHO defines health as a state of complete physical mental and so cial well b