Lippincott’s Illustrated Reviews: Biochemistr Sixth Edition Lippincott’s Illustrated Reviews: Biochemistr Sixth Edition Denise R. Ferrier, PhD Professor Department of Biochemistry and Molecular Biology Drexel University College of Medicine Philadelphia, Pennsylvania Health Philadelphia • Baltimore • New York • London Buenos Aires • Hong Kong • Sydney • Tokyo Acquisitions Editor: Susan Rhyner Product Manager: Angela Collins Development Editor: Kelly Horvath Marketing Manager: Joy Fisher-Williams Production Manager: David Saltzberg Art and Page Design: Debbie McQuade Cover Design: Holly McLaughlin Sixth Edition Copyright © 2014 (2011, 2008, 2005, 1994, 1987) Lippincott Williams & Wilkins, a Wolters Kluwer business 351 West Camden Street Two Commerce Square; 2001 Market Street Baltimore, MD 21201 Philadelphia, PA 19103 Printed in China All rights reserved. This book is protected by copyright. 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Includes bibliographical references and index. ISBN 978-1-4511-7562-2 (alk. paper) I. Title. II. Series: Lippincott’s illustrated reviews. [DNLM: 1. Biochemistry--Examination Questions. QU 18.2] 612.3′9--dc23 2012025941 DISCLAIMER Care has been taken to confirm the accuracy of the information presented and to describe generally accepted practices. However, the authors, editors, and publisher are not responsible for errors or omissions or for any consequences from application of the information in this book and make no warranty, expressed or implied, with respect to the currency, completeness, or accuracy of the contents of the publication. Application of this information in a particular situation remains the professional responsibility of the practitioner; the clinical treatments described and recommended may not be considered absolute and universal recommendations. The authors, editors, and publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accordance with the current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any change in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new or infrequently employed drug. Some drugs and medical devices presented in this publication have Food and Drug Administration (FDA) clearance for limited use in restricted research settings. It is the responsibility of the health care provider to ascertain the FDA status of each drug or device planned for use in their clinical practice. The publishers have made every effort to trace the copyright holders for borrowed material. If they have inadvertently overlooked any, they will be pleased to make the necessary arrangements at the first opportunity. To purchase additional copies of this book, call our customer service department at (800) 638-3030 or fax orders to (301) 223-2320. International customers should call (301) 223-2300. Visit Lippincott Williams & Wilkins on the Internet: http://www.lww.com. Lippincott Williams & Wilkins customer service representatives are available from 8:30 am to 6:00 pm, EST. Acknowledgments I am grateful to my colleagues at Drexel University College of Medicine who generously shared their expertise to help make this book as accurate and as useful to medical students as possible. I am particularly appreciative of the many helpful comments of Dr. Åke Rökaeus of the Karolinska Institute as they have enhanced the accuracy of this work. In addition, the author thanks Dr. Susan K. Fried and Dr. Richard B. Horenstein for their valuable contributions to the Obesity chapter in previous editions of this text. A special thank you to Dr. Alan Katz for his helpful comments on the clinical aspects of the cases in the Appendix. Ms. Barbara Engle was an invaluable sounding board throughout the process. The editors and production staff of Lippincott Williams & Wilkins were an important source of encouragement. I particularly want to acknowledge the contributions of Susan Ryner, the Acquisitions Editor, and Angela Collins, the Managing Editor. Many thanks are due to Kelly Horvath, Development Editor, for her assistance in the final editing of this book. I also want to thank Deborah McQuade for her work in the assembly of the 6th edition. This book is dedicated to my husband John, whose loving support made the task possible; to my students, who have taught me so much over the last 20 years; and to Richard Harvey and the late Pamela Champe, who helped me develop as an author. Contents UNIT I: Protein Structure and Function Chapter 1: Amino Acids Chapter 2: Structure of Proteins Chapter 3: Globular Proteins Chapter 4: Fibrous Proteins Chapter 5: Enzymes UNIT II: Bioenergetics and Carbohydrate Metabolism Chapter 6: Bioenergetics and Oxidative Phosphorylation Chapter 7: Introduction to Carbohydrates Chapter 8: Introduction to Metabolism and Glycolysis Chapter 9: Tricarboxylic Acid Cycle and Pyruvate Dehydrogenase Complex Chapter 10: Gluconeogenesis Chapter 11: Glycogen Metabolism Chapter 12: Metabolism of Monosaccharides and Disaccharides Chapter 13: Pentose Phosphate Pathway and Nicotinamide Adenine Dinucleotide Phosphate Chapter 14: Glycosaminoglycans, Proteoglycans, and Glycoproteins UNIT III: Lipid Metabolism Chapter 15: Dietary Lipids Metabolism Chapter 16: Fatty Acid, Ketone Body, and Triacylglycerol Metabolism Chapter 17: Phospholipid, Glycosphingolipid, and Eicosanoid Metabolism Chapter 18: Cholesterol, Lipoprotein, and Steroid Metabolism UNIT IV: Nitrogen Metabolism Chapter 19: Amino Acids: Disposal of Nitrogen Chapter 20: Amino Acid Degradation and Synthesis Chapter 21: Conversion of Amino Acids to Specialized Products Chapter 22: Nucleotide Metabolism UNIT V: Integration of Metabolism Chapter 23: Metabolic Effects of Insulin and Glucagon Chapter 24: The Feed–Fast Cycle Chapter 25: Diabetes Mellitus Chapter 26: Obesity Chapter 27: Nutrition Chapter 28: Vitamins UNIT VI: Storage and Expression of Genetic Information Chapter 29: DNA Structure, Replication, and Repair Chapter 30: RNA Structure, Synthesis, and Processing Chapter 31: Protein Synthesis Chapter 32: Regulation of Gene Expression Chapter 33: Biotechnology and Human Disease Appendix: Clinical Cases Index Bonus chapter online! Chapter 34: Blood Clotting (Use your scratch-off code provided in the front of this book for access to this and other free online resources on the point.) UNIT I: Protein Structure and Function Amino Acids 1