(BQ) Part 1 book Lippincott illustrated reviews flash cards biochemistry presents the following contents: Protein structure and function, bioenergetics and carbohydrate metabolism, lipid metabolism.
http://tm.firebrandtech.com/WK/WK_FileRepository/WK_IMAGES/9781451191110.jpg[5/30/2014 12:55:29 PM] Lippincott Illustrated Reviews Flash Cards BIOCHEMISTRY Denise R Ferrier, PhD Bradford A Jameson, PhD Professor of Biochemistry Department of Biochemistry and Molecular Biology Drexel University College of Medicine Philadelphia, Pennsylvania Professor of Biochemistry Department of Biochemistry and Molecular Biology Drexel University College of Medicine Philadelphia, Pennsylvania Ferrier_FM.indd i 5/3/14 4:48 AM Acquisitions Editor: Tari Broderick Product Development Editor: Stephanie Roulias Production Project Manager: David Orzechowski Design Coordinator: Holly McLaughlin Illustration Coordinator: Doug Smock Manufacturing Coordinator: Margie Orzech Prepress Vendor: Absolute Service, Inc Copyright © 2015 Wolters Kluwer Health All rights reserved This book is protected by copyright No part of this book may be reproduced or transmitted in any form or by any means, including as photocopies or scanned-in or other electronic copies, or utilized by any information storage and retrieval system without written permission from the copyright owner, except for brief quotations embodied in critical articles and reviews Materials appearing in this book prepared by individuals as part of their official duties as U.S government employees are not covered by the above-mentioned copyright To request permission, please contact Wolters Kluwer Health at Two Commerce Square, 2001 Market Street, Philadelphia, PA 19103, via email at permissions@lww.com, or via our website at lww.com (products and services) 987654321 Printed in China 978-1-4511-9111-0 1-4511-9111-1 Library of Congress Cataloging-in-Publication Data is available upon request Care has been taken to confirm the accuracy of the information presented and to describe generally accepted practices However, the author(s), 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 author(s), 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 his or her clinical practice Ferrier_FM.indd ii 5/23/14 1:09 AM Features: Three-Step Review SPOT FLASH Test your grasp of key concepts or equations on a lecture-by-lecture basis! COURSE REVIEW Ensure a thorough understanding of course material through in-depth questions High-yield facts for course- and Board-exam review! CLINICAL CORRELATIONS Explain how the basic science helps predict outcomes in a clinical setting! Featuring the same visionary artwork found in Lippincott Illustrated Reviews: Biochemistry With Lippincott Illustrated Reviews, Seeing is Understanding Lippincott Illustrated Reviews Flash Cards: Biochemistry Ferrier_FM.indd iii Copyright © 2015 Wolters Kluwer 5/3/14 4:48 AM Ferrier_FM.indd iv 5/3/14 4:48 AM Preface Lippincott Illustrated Reviews Flash Cards: Biochemistry is a portable study tool designed for self-assessment and review of medical biochemistry The flash cards were developed primarily for use by medical students studying biochemistry and preparing for United States licensing exams, but information is presented with a clarity and level of detail that makes them ideal supplements for any of the allied health sciences The deck contains three card types: Question (Q) cards, Case cards, and Summary cards Q CARDS The majority of cards are Q cards that prompt the reader with questions (on the front) to assess level of understanding, depth of knowledge, and ability to apply biochemical concepts The answers (on the back) are more inclusive than those found on typical flash cards Most Q cards contain three questions or sets of questions on a common topic: The first tests for retention of basic facts, whereas the next two test understanding and/or application of related concepts and clinical correlations Each question type is denoted by icons SPOT FLASH: Illustration-based questions test your grasp of key facts and are intended for use on a lecture-by-lecture assessment and review basis COURSE REVIEW: In-depth questions promote a thorough understanding of related concepts The answers focus on high-yield facts to help consolidate and apply material during course- and licensing-exam review CLINICAL CORRELATIONS: Clinical questions highlight the basic science foundations of medicine They help students apply biochemical concepts to clinical problems and are particularly useful when studying for licensing exams Continued, over Lippincott Illustrated Reviews Flash Cards: Biochemistry Ferrier_FM.indd v Copyright © 2015 Wolters Kluwer 5/3/14 4:48 AM Preface Q cards include several features to facilitate learning and retaining the material: • Illustrations: Richly detailed illustrations from the popular companion text, Lippincott Illustrated Reviews: Biochemistry, appear on both sides of the cards Many of the illustrations include narrative boxes that guide readers through complex concepts • Notes: Answers may be supplemented with information that goes beyond the need-to-know basics to provide context or to enrich and help anchor a concept • Emphasis: Key terms, disease names, and pathologic findings are bolded for rapid review and assimilation CASE CARDS AND SUMMARY CARDS Case cards use common clinical presentations to highlight biochemical concepts Summary cards (for the vitamins and the fed/fasted states) highlight key features of these information-rich areas of medical biochemistry The card deck is designed to be comprehensive, covering all significant biochemical concepts Lippincott Illustrated Reviews Flash Cards: Biochemistry Ferrier_FM.indd vi Copyright © 2015 Wolters Kluwer 5/3/14 4:48 AM Acknowledgments The authors wish to thank John Swaney, PhD, our colleague at Drexel University College of Medicine, for his careful reading of the manuscript and constructive comments Any errors are ours alone We thank the publishing team assembled by Wolters Kluwer Stephanie Roulias, product development editor, and Kelly Horvath, freelance development editor, along with Doug Smock, Teresa Exley, and David Orzechowski, gave invaluable assistance in the development and production of the finished product We also thank Robin R Preston, PhD, for his design of the flash card format Dedication The authors dedicate this work to the medical, biomedical graduate, and professional studies students of Drexel University You have challenged and inspired us, and have made us better teachers Lippincott Illustrated Reviews Flash Cards: Biochemistry Ferrier_FM.indd vii Copyright © 2015 Wolters Kluwer 5/3/14 4:48 AM Figure Credits Card 3.6 Question and Answer: Modified photo courtesy of Photodyne Incorporated, Hartland, WI Card 4.2 Answer: Kronauer and Buhler, Images in Clinical Medicine, The New England Journal of Medicine, June 15, 1995, Vol 332, No 24, p 1611 Card 4.5 Question and Answer: Modified photo from Web site Derma.de Modified Lippincott Illustrated Reviews Flash Cards: Biochemistry Ferrier_FM.indd viii from Jorde LB, Carey JC, Bamshad MJ, et al Medical Genetics 2nd ed St Louis, MO: Mosby; 2000 http://medgen.genetics utah.edu/index.htm Card 13.6 Answer: From the Crookston Collection, University of Toronto Card 21.2 Answer: Modified from Rich MW Porphyria cutanea tarda Postgrad Med 1999;105:208–214 Card 21.4 Question and Answer: From Custom Medical School Stock Photo, Inc Card 22 Case Card Question: Modified from WebMD Inc http://www.samed.com/sam/ forms/index.htm Card 23.6 Question and Answer: Modified from Cryer PE, Fisher JN, Shamoon H Hypoglycemia Diabetes Care 1994;17: 734–753 Copyright © 2015 Wolters Kluwer 5/3/14 4:48 AM Contents UNIT Protein Structure and Function 1.1 UNIT Bioenergetics and Carbohydrate Metabolism 6.1 UNIT Lipid Metabolism 15.1 UNIT Nitrogen Metabolism 19.1 UNIT Metabolism Integration 23.1 UNIT Genetic Information Storage and Expression 29.1 CHAPTER 34 Blood Clotting 34.1 APPENDIX Abbreviations A-1 Lippincott Illustrated Reviews Flash Cards: Biochemistry Ferrier_FM.indd ix Copyright © 2015 Wolters Kluwer 5/3/14 4:48 AM Eicosanoids What 20-carbon, polyunsaturated, -6 FA serves as the precursor for the synthesis of the predominate series of PGs, TXs, and LTs (collectively known as the eicosanoids), as shown? Eicosanoids differ from endocrine hormones in that eicosanoids are produced in amounts in tissues, are not , act , and have their biologic actions mediated by cell membrane and changes in the concentration of 17.5 Question cleaved by PLA2 Membrane phospholipids cortisol ? is precursor to Leukotrienes (LTs) Prostaglandins (PGs) Thromboxanes (TXs) Why would a deficiency of linoleic acid, an essential -6 FA, decrease the synthesis of the eicosanoids? Lippincott Illustrated Reviews Flash Cards: Biochemistry Ferrier_Unit03.indd 201 Copyright © 2015 Wolters Kluwer 5/2/14 7:30 PM Eicosanoids 17.5 Answer COO The eicosatetraenoic FA, arachidonic acid [20:4(5,8,11,14)], is the precursor of the predominant series of eicosanoids Eicosanoids differ from endocrine hormones in that eicosanoids are produced in very small amounts in virtually all tissues, are not stored, act locally, and have their biologic actions mediated by cell membrane GPCRs and changes in the concentration of cAMP – Dietary linoleic acid 18:2 (9,12) (an ω-6 fatty acid) Desaturation Elongation Because humans cannot insert double bonds after C-10 in a FA, we are unable to synthesize arachidonic acid de novo However, we are able to elongate and desaturate dietary linoleic acid,18:2(9,12), to arachidonic acid Therefore, linoleic acid deficiency would decrease availability of the precursor for eicosanoid synthesis [Note: Arachidonic acid is incorporated into membrane PLs (primarily PI) at C-2 until it is released by PLA2 Cortisol inhibits PLA2 activity.] COO– Arachidonic acid 20:4 (5,8,11,14) (an ω-6 fatty acid) Cyclooxygenase O2 O COO O Membrane phospholipids cleaved by PLA2 cortisol – OOH PGG2 Arachidonic acid Peroxidase is precursor to G-SH G-S-S-G O COO Leukotrienes (LTs) Prostaglandins (PGs) Thromboxanes (TXs) O – OH PGH2 Lippincott Illustrated Reviews Flash Cards: Biochemistry Ferrier_Unit03.indd 202 Copyright © 2015 Wolters Kluwer 5/2/14 7:30 PM Eicosanoids cleaved by PLA2 Membrane phospholipids cortisol 17.6 Question Arachidonic acid is precursor to Leukotrienes (LTs) Prostaglandins (PGs) Thromboxanes (TXs) 5-Lipoxygenase (LOX ) Cyclooxygenase (COX-1) Cyclooxygenase (COX-2) 5-Hydroperoxyeicosatetraenoic acid (5-HPETE) LTC4 LTD4 LTE4 LTA4 LTB4 Glutathione PGG2 PGE2 Peroxidase TXA2 PGH2 PGF2 PGI2 Which isozyme, COX-1 or COX-2 shown, is inducible (nonconstitutive)? How the NSAIDs (including aspirin) affect the activity of COX ? Of LOX ? Why might inhibitors specific for COX-2 be associated with increased risk of an MI? Lippincott Illustrated Reviews Flash Cards: Biochemistry Ferrier_Unit03.indd 203 Copyright © 2015 Wolters Kluwer 5/2/14 7:30 PM Eicosanoids 17.6 Answer cleaved by PLA2 Membrane phospholipids cortisol Arachidonic acid is precursor to Leukotrienes (LTs) Prostaglandins (PGs) Thromboxanes (TXs) 5-Lipoxygenase (LOX ) Cyclooxygenase (COX-1, constitutive) LTA4 LTB4 Glutathione PGE2 TXA2 PGH2 Cytokines, endotoxin, growth factors, tumor promoters Selective COX-2 inhibitors (for example, celecoxib) PGG2 Peroxidase + Aspirin Indomethacin Phenylbutazone Other NSAIDs 5-Hydroperoxyeicosatetraenoic acid (5-HPETE) LTC4 LTD4 LTE4 Cyclooxygenase (COX-2, nonconstitutive) PGF2 PGI2 COX-2 is inducible in a limited number of tissues Induction results in the synthesis of PGs that mediate the pain, heat, redness and swelling of inflammation, and the fever of infection COX-1, constitutively expressed in most tissues, maintains healthy gastric tissue and modulates renal function and platelet aggregation NSAIDs (including aspirin) inhibit both COX isozymes Aspirin, but not other NSAIDs, causes irreversible inhibition by covalent acetylation Aspirin does not inhibit LOX and may even favor use of arachidonic acid by LOX for synthesis of the LTs, mediators of bronchoconstriction [Note: Cortisol indirectly inhibits both COX and LOX by directly inhibiting PLA2 and the release of arachidonic acid, their substrate.] COX-2 synthesizes PGI2 (prostacyclin) that decreases platelet aggregation and vasoconstriction Its specific inhibition would increase the risk of clotting (e.g., in a cardiac vessel in an MI) [Note: COX-1 synthesizes TXA2 that increases platelet aggregation and vasoconstriction COX-1 inhibition causes decreased clotting, the most common side effect of aspirin use.] Lippincott Illustrated Reviews Flash Cards: Biochemistry Ferrier_Unit03.indd 204 Copyright © 2015 Wolters Kluwer 5/2/14 7:30 PM Cholesterol In addition to de novo synthesis, what are the other two major sources of liver cholesterol, as shown? Cholesterol is a structural component of in the adrenal cortex, and , the precursor of in the skin 18.1 Question Major sources of liver cholesterol in the liver, ? In cholesterol synthesis, which enzymatic steps are identical to those required for KB synthesis? What is different? ? De novo synthesis in the liver Why might patients with hypercholesterolemia be prescribed ezetimibe? Why should they include plant sterols (phytosterols) in their diet? Liver Cholesterol Pool Free cholesterol secreted in the bile Secretion of VLDL Conversion to bile acids/salts Major routes by which cholesterol leaves the liver Lippincott Illustrated Reviews Flash Cards: Biochemistry Ferrier_Unit03.indd 205 Copyright © 2015 Wolters Kluwer 5/2/14 7:30 PM 18.1 Answer Cholesterol Liver receives cholesterol from the diet via CM remnants and from peripheral tissues via HDLs Cholesterol is a structural component of membranes, the precursor of bile acids in the liver, steroid hormones in the adrenal cortex, and vitamin D in the skin Two acetyl CoA → acetoacetyl CoA → HMG CoA are steps common to cholesterol and KB synthesis The difference is that the liver has two isozymes of HMG CoA synthase that convert acetoacetyl CoA to HMG CoA The mitochondrial isoenzyme is used for KB synthesis, and the cytosolic one is used for cholesterol synthesis Major sources of liver cholesterol Dietary cholesterol Cholesterol from extrahepatic tissues Chylomicron remnants HDL De novo synthesis in the liver Ezetimibe and phytosterols decrease intestinal absorption of dietary cholesterol (via different mechanisms) Consequently, they have a hypocholesterolemic effect and are used to treat elevated blood cholesterol Liver Cholesterol Pool Free cholesterol secreted in the bile Secretion of VLDL Conversion to bile acids/salts Major routes by which cholesterol leaves the liver Lippincott Illustrated Reviews Flash Cards: Biochemistry Ferrier_Unit03.indd 206 Copyright © 2015 Wolters Kluwer 5/2/14 7:30 PM Cholesterol What enzyme (denoted by the red question mark) is the rate-limiting, regulated enzyme in cholesterol biosynthesis? How is the enzyme regulated at the transcriptional level when intracellular cholesterol is low? How else is it regulated? 18.2 Question O – C O CH3 OH O C C CH2 CH2 CoA HMG CoA How statins affect cholesterol biosynthesis? ? CoA O – O C NADPH + H+ NADP+ CH3 OH C CH2OH CH2 CH2 Mevalonate Lippincott Illustrated Reviews Flash Cards: Biochemistry Ferrier_Unit03.indd 207 Copyright © 2015 Wolters Kluwer 5/2/14 7:30 PM Cholesterol 18.2 Answer Expression is inhibited by cholesterol SREBP DNA SRE SREBPSCAP Transcription CYTOSOL Proteolytic cleavage NUCLEUS O Golgi mRNA Phosphoprotein H2O phosphatase HMG CoA reductase (inactive) mRNA – C O SREBPSCAP CH3 OH O C C CH2 CH2 CoA HMG CoA Translation P HMG CoA reductase HMG CoA reductase (active) Endoplasmic reticulum CoA NADPH + H+ NADP+ AMPK P + ADP Mevalonate ATP O AMP HMG CoA Cholesterol – O C CH3 OH C CH2OH CH2 CH2 Mevalonate The rate-limiting, regulated enzyme of cholesterol biosynthesis is HaMG CoA reductase When intracellular cholesterol is low, the SREBP–SCAP complex of the ER membrane moves to the Golgi, where SREBP is sequentially cleaved to a soluble fragment (SREBP-2) that enters the nucleus, binds SRE regions on DNA, and functions as a TF that increases expression of HMG CoA reductase A rise in cholesterol inhibits this process [Note: SREBP-1 upregulates FA and TAG synthesis.] Additional sterol-dependent regulation is the accelerated degradation of the reductase Sterol-independent regulation includes phosphorylation of the reductase by AMPK [Note: When AMPK is active, acetyl CoA is oxidized in the TCA cycle and not used in FA and cholesterol synthesis.] Statins are competitive inhibitors of HMG CoA reductase Consequently, they increase the apparent Km of the enzyme (decrease the affinity of enzyme for substrate) but not affect the Vmax Lippincott Illustrated Reviews Flash Cards: Biochemistry Ferrier_Unit03.indd 208 Copyright © 2015 Wolters Kluwer 5/2/14 7:30 PM Bile Acids and Bile Salts 18.3 Question What enzyme (denoted by the red question mark) is the rate-limiting and regulated enzyme of BA synthesis? How is the enzyme regulated? How does the amphipathic nature of BSs and TAG aid in dietary lipid emulsification? Why are agents such as cholestyramine that interfere with the enterohepatic circulation of BSs useful in hypercholesterolemia treatment? HO O2 H2O Cholesterol NADPH + H+ NADP+ HO ? OH 7-α-Hydroxycholesterol Cholic acid (a triol) Lippincott Illustrated Reviews Flash Cards: Biochemistry Ferrier_Unit03.indd 209 Chenodeoxycholic acid (a diol) Copyright © 2015 Wolters Kluwer 5/2/14 7:30 PM 18.3 Answer Bile Acids and Bile Salts The rate-limiting and regulated enzyme of BA synthesis is cholesterol 7-␣-hydroxylase, a CYP enzyme of the SER in hepatocytes [Note: The products, the primary BAs, undergo hepatic conjugation with taurine or Gly and form conjugated BAs that are stored in the gallbladder and released by CCK At the pH of bile, the conjugated derivatives are negatively charged, contributing to their amphipathic nature, and are termed BSs Intestinal bacteria can dehydroxylate them, generating secondary BSs.] Regulation is transcriptional: BAs (via a nuclear receptor) cause decreased transcription of the gene for the hydroxylase HO The amphipathic nature of BSs is largely the result of hydroxylation, which creates a water-soluble face (that interacts with the aqueous environment) and a water-insoluble face (that interacts with dietary lipids) that allow them to stabilize dietary lipid droplets (prevent their coalescence) as they become smaller (emulsification) O2 H2O Cholesterol Cholesterol 7-α-hydroxylase NADPH + H+ NADP+ Bile acids Over 95% of BSs are reabsorbed in the proximal ileum, returned to the liver, and reused (enterohepatic circulation) Excretion of a small amount in stool is the primary mechanism by which cholesterol is removed from the body [Note: BS deficiency can result in cholesterol stone formation (cholelithiasis).] Cholestyramine binds BSs, preventing their reabsorption and increasing the amount excreted Consequently, more cholesterol is used for BA synthesis, thereby decreasing blood cholesterol levels HO OH 7-α-Hydroxycholesterol Cholic acid (a triol) Lippincott Illustrated Reviews Flash Cards: Biochemistry Ferrier_Unit03.indd 210 Chenodeoxycholic acid (a diol) Copyright © 2015 Wolters Kluwer 5/2/14 7:30 PM Plasma Lipoproteins 18.4 Question What molecules are contained in the core of a LP, as shown? What are the sources of these molecules? Which LP is the largest? Which is densest? Inner core of Compare and contrast CMs and VLDLs What is the total cholesterol (C) if LDL-C ϭ 136 mg/dl, HDL-C ϭ 45 mg/dl, and TAGs ϭ 150 mg/dl? ? and ? Apolipoprotein Phospholipids Unesterifed cholesterol Unesterifed cholesterol Lippincott Illustrated Reviews Flash Cards: Biochemistry Ferrier_Unit03.indd 211 Copyright © 2015 Wolters Kluwer 5/2/14 7:30 PM 18.4 Answer Plasma Lipoproteins TAGs and CEs, nonpolar molecules, are in the LP core They come from exogenous (dietary) sources or from endogenous synthesis CMs are the largest (and least dense) of the LPs HDLs are the densest (and smallest) VLDLs are made in the liver and secreted into the blood, contain the structural protein apo B-100, obtain apo C-II and apo E from circulating HDLs, and carry endogenous TAGs (and cholesterol) to most peripheral tissues where they are degraded in capillaries by endothelial LPL that is activated by apo C-II on the LP’s surface The TAG-poor, CE-rich LDL product is endocytosed by LDL receptors found on virtually all cells and that recognize apo B-100 Inner core of triacylglycerols and cholesteryl esters Apolipoprotein Phospholipids Unesterifed cholesterol CMs are made in the small intestine and secreted first into the lymph then the blood, contain the structural protein apo B-48, and carry exogenous (dietary) TAGs to muscle and adipose tissue primarily The TAG-poor, CE-rich CM remnant produced by intravascular LPL- and apo CII-mediated degradation is endocytosed by remnant receptors found on hepatocytes and that recognize apo E [Note: IDLs (formed during VLDL catabolism) are endocytosed via receptors that recognize apo E.] Total C ؍LDL-C ؉ HDL-C ؉ TAG/5 Therefore, 136 ϩ 45 ϩ 150/5 ϭ 211 mg/dl TAG/5 is a measure of VLDL-C [Note: The goal value for total cholesterol is Ͻ200 mg/dl.] Unesterifed cholesterol Lippincott Illustrated Reviews Flash Cards: Biochemistry Ferrier_Unit03.indd 212 Copyright © 2015 Wolters Kluwer 5/2/14 7:30 PM Plasma Lipoproteins What mediates the decrease in LDL receptor synthesis when intracellular cholesterol is high, as shown? 18.5 Question SYNTHESIS OF LDL RECEPTORS SYNTHESIS OF CHOLESTEROL What is reverse cholesterol transport (RCT)? HMG CoA reductase DNA Match the hyperlipidemia to its cause: Type I a apo E-2 homozygosity Type IIa b LPL deficiency Type III c LDL receptor deficiency mRNA OVERSUPPLY OF CHOLESTEROL Ribosome ACAT + ER Receptor protein Lippincott Illustrated Reviews Flash Cards: Biochemistry Ferrier_Unit03.indd 213 Copyright © 2015 Wolters Kluwer 5/2/14 7:30 PM 18.5 Answer Ap LIVER SMALL INTESTINE C, CE C PC lyso-PC Hepatic lipase LDL o A-I Discoidal nascent HDL SR-B1 IDL LCAT CE e Fre terol les cho AB + TP CE Decreased LDL receptor synthesis when intracellular cholesterol is high is mediated by retention of the SREBP–SCAP complex in the ER membrane This prevents formation (in the Golgi) of SREBP-2, a TF that binds SREs on DNA and increases expression of the gene for the LDL receptor The gene for HMG CoA reductase, the rate-limiting enzyme of cholesterol synthesis, is regulated in the same way (see Card 18.2) [Note: Cytosolic ACAT esterifies the excess cholesterol to CE for storage in cytosolic droplets.] Plasma Lipoproteins CA Apo E, C-II CE HDL3 Apo A-1 PERIPHERAL TISSUES VLDL LCAT HDL2 C + VLDL Free cholesterol Apo A-1 RCT is an atheroprotective process by which cholesterol is sent out of peripheral cells (via ABCA1) for uptake onto nascent HDLs, esterified by LCAT, and sequestered in the core HDL (as HDL2) carries CEs to the liver, where they are selectively taken up by SR-B1 and used for BA synthesis or excreted into bile [Note: CETP exchanges HDL CEs for VLDL TAGs.] Type I hyperlipidemiaϭ b, LPL deficiency; Type IIa ϭ c, LDL receptor deficiency; Type III ϭ a, Apo E2 homozygosity LPL deficiency results in a rise in CMs and hypertriglyceridemia LDL receptor deficiency results in a rise in LDLs and hypercholesterolemia Apo E-2 homozygosity results in CM remnant and IDL accumulation (due to decreased uptake) and hypercholesterolemia Lippincott Illustrated Reviews Flash Cards: Biochemistry Ferrier_Unit03.indd 214 Copyright © 2015 Wolters Kluwer 5/2/14 7:30 PM Steroid Hormones 18.6 Question What molecule (denoted by the red question mark) is the parent compound for all steroid hormones? What role does desmolase play in its synthesis? Cholesterol (27C) NADPH O2 What hormone stimulates cortisol synthesis and release? What are the effects of cortisol? How are these effects mediated? Desmolase (CYP11A , P scc ) ? Which congenital adrenal hyperplasia (CAH) is characterized by decreased aldosterone and cortisol production and increased androstenedione production? 3-β-Hydroxysteroid dehydrogenase Progesterone (21C) 17-α-Hydroxylase (CYP17) 21-α-Hydroxylase 11-Deoxycorticosterone (21C) 17-α-Hydroxyprogesterone (21C) 17, 20-Lyase (CYP17 ) 11-Deoxycortisol (21C) 11-β-Hydroxylase (CYP11B1) Corticosterone 17-β-Hydroxysteroid dehydrogenase (11-β-hydroxylase) Testosterone (C19) (CYP11B1) 18-α-hydroxylase (aldosterone synthase) (CYP11B2) Aldosterone (21C) Lippincott Illustrated Reviews Flash Cards: Biochemistry Ferrier_Unit03.indd 215 Androstenedione (19C) Aromatase (CYP19 ) Cortisol (21C) Estradiol (18C) Copyright © 2015 Wolters Kluwer 5/2/14 7:30 PM ... 20 01 Market Street, Philadelphia, PA 19 103, via email at permissions@lww.com, or via our website at lww.com (products and services) 9876543 21 Printed in China 978 -1- 4 511 - 911 1-0 1- 4 511 - 911 1 -1 Library... visionary artwork found in Lippincott Illustrated Reviews: Biochemistry With Lippincott Illustrated Reviews, Seeing is Understanding Lippincott Illustrated Reviews Flash Cards: Biochemistry Ferrier_FM.indd... Metabolism 19 .1 UNIT Metabolism Integration 23 .1 UNIT Genetic Information Storage and Expression 29 .1 CHAPTER 34 Blood Clotting 34 .1 APPENDIX Abbreviations A -1 Lippincott Illustrated Reviews Flash Cards: