Textbook of biochemistry: with clinical correlations
Gespeichert in:
| Format: | Buch |
|---|---|
| Sprache: | English |
| Veröffentlicht: |
Hoboken, NJ
Wiley-Liss
2006
|
| Ausgabe: | 6. ed. |
| Schlagworte: | |
| Online-Zugang: | Inhaltsverzeichnis |
| Beschreibung: | XXVII, 1208 S. Ill, graph. Darst. |
| ISBN: | 0471678082 9780471678083 |
Internformat
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| 245 | 1 | 0 | |a Textbook of biochemistry |b with clinical correlations |c ed. by Thomas M. Devlin |
| 246 | 1 | 3 | |a Biochemistry |
| 250 | |a 6. ed. | ||
| 264 | 1 | |a Hoboken, NJ |b Wiley-Liss |c 2006 | |
| 300 | |a XXVII, 1208 S. |b Ill, graph. Darst. | ||
| 336 | |b txt |2 rdacontent | ||
| 337 | |b n |2 rdamedia | ||
| 338 | |b nc |2 rdacarrier | ||
| 650 | 4 | |a Biochimie | |
| 650 | 4 | |a Biochimie clinique | |
| 650 | 4 | |a Biochemistry | |
| 650 | 4 | |a Clinical biochemistry | |
| 650 | 0 | 7 | |a Biochemie |0 (DE-588)4006777-4 |2 gnd |9 rswk-swf |
| 650 | 0 | 7 | |a Physiologische Chemie |0 (DE-588)4076124-1 |2 gnd |9 rswk-swf |
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| 700 | 1 | |a Devlin, Thomas M. |e Sonstige |4 oth | |
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Datensatz im Suchindex
| _version_ | 1804135102476713986 |
|---|---|
| adam_text | CONTENTS
PART I I STRUCTURE OF MACROMOLECULES
1 Eukaryotic Cell Structure, 1
2 DNA and RNA: Composition and Structure, 23
3 Proteins I: Composition and Structure, 75
PART II | TRANSMISSION OF INFORMATION
4 DNA Replication, Recombination, and Repair,
133
5 RNA: Transcription and RNA Processing, 175
6 Protein Synthesis: Translation and
Posttranslational Modifications, 201
7 Recombinant DNA and Biotechnology, 245
8 Regulation of Gene Expression, 291
PART |jl | FUNCTIONS OF PROTEINS
9 Proteins II: Structure Function Relationships in
Protein Families, 319
10 Enzymes: Classification, Kinetics, and Control,
365
11 The Cytochromes P450 and Nitric Oxide
Synthases, 413
12 Biological Membranes: Structure and Membrane
Transport, 443
13 Fundamentals of Signal Transduction, 489
PART W I METABOLIC PATHWAYS AND THEIR
CONTROL
14 Bioenergetics and Oxidative Metabolism, 529
ITS IN BRIEF
15 Carbohydrate Metabolism I: Major Metabolic
Pathways and their Control, 581
16 Carbohydrate Metabolism II: Special Pathways
and Glycoconjugates, 637
17 Lipid Metabolism I: Synthesis, Storage, and
Utilization of Fatty Acids and Triacylglycerols,
661
18 Lipid Metabolism II: Pathways of Metabolism of
Special Lipids, 695
19 Amino Acid Metabolism, 743
20 Purine and Pyrimidine Nucleotide Metabolism,
789
21 Iron and Heme Metabolism, 823
22 Metabolic Interrelationships, 849
PART V I PHYSIOLOGICAL PROCESSES
23 Biochemistry of Hormones, 891
24 Molecular Cell Biology, 949
25 Cell Cycle, Programmed Cell Death, and Cancer,
1013
26 Digestion and Absorption of Basic Nutritional
Constituents, 1037
27 Principles of Nutrition I: Macronutrients, 1071
28 Principles of Nutrition II: Micronutrients, 1091
Appendix Review of Organic Chemistry, 1121
Glossary, 1133
Index. 1157
PREFACE, xxi
ACKNOWLEDGMENTS, xxiii
CONTRIBUTORS, xxv
PART g 1 STRUCTURE OF MACROMOLECULES
1 | EUKARYOTIC CELL STRUCTURE, 1
Thomas M. Devlin
1.1 OVERVIEW: CELLS AND CELLULAR
COMPARTMENTS, 2
1.2 WATER, pH, AND SOLUTES: THE AQUEOUS
ENVIRONMENT OF CELLS, 3
1.3 COMPOSITION OF EUKARYOTIC CELLS:
FUNCTIONAL ROLES OF SUBCELLULAR
ORGANELLES AND MEMBRANE SYSTEMS, 11
1.4 INTEGRATION AND CONTROL OF CELLULAR
FUNCTIONS, 19
BIBLIOGRAPHY, 20
QUESTIONS AND ANSWERS, 20
CLINICAL CORRELATIONS
1.1 Blood Bicarbonate Concentration in
Metabolic Acidosis, 10
1.2 Mitochondrial Diseases, 15
1.3 Lysosomal Enzymes and Gout, 16
1.4 Lysosomal Acid Lipase Deficiency, 18
1.5 Peroxisome Biogenesis Disorders (PBD), 19
2 ! DNA AND RNA: COMPOSITION AND STRUCTURE,
23
Stephen A. Woski and Francis J. Schmidt
2.1 OVERVIEW, 24
2.2 STRUCTURAL COMPONENTS OF NUCLEIC
ACIDS: NUCLEOBASES, NUCLEOSIDES, AND
NUCLEOTIDES, 25
2.3 STRUCTURE OF DNA, 29
2.4 HIGHER ORDER DNA STRUCTURE, 46
2.5 DNA SEQUENCE AND FUNCTION, 58
2.6 RNA STRUCTURE, 62
2.7 TYPES OF RNA, 64
TS
BIBLIOGRAPHY, 71
QUESTIONS AND ANSWERS, 71
CLINICAL CORRELATIONS
2.1 DNA Vaccines, 25
2.2 Diagnostic Use of DNA Arrays in Medicine
and Genetics, 39
2.3 Antitumor Antibiotics that Change the Shape
of DNA, 42
2.4 Hereditary Persistence of Fetal
Hemoglobin, 45
2.5 Telomerase as a Target for Anticancer
Agents, 46
2.6 Expansion of DNA Triple Repeats and Human
Disease, 49
2.7 Topoisomerases in the Treatment of
Disease, 53
2.8 Staphylococcal Resistance to
Erythromycin, 67
3 | PROTEINS I: COMPOSITION AND STRUCTURE, 75
Richard M. Schultz and Michael N. Liebman
3.1 FUNCTIONAL ROLES OF PROTEINS IN
HUMANS, 76
3.2 AMINO ACID COMPOSITION OF PROTEINS, 77
3.3 CHARGE AND CHEMICAL PROPERTIES OF
AMINO ACIDS AND PROTEINS, 81
3.4 PRIMARY STRUCTURE OF PROTEINS, 90
3.5 HIGHER LEVELS OF PROTEIN
ORGANIZATION, 92
3.6 OTHER TYPES OF PROTEINS, 99
3.7 FOLDING OF PROTEINS FROM RANDOMIZED
TO UNIQUE STRUCTURES: PROTEIN
STABILITY, 109
3.8 DYNAMIC ASPECTS OF PROTEIN
STRUCTURE, 116
3.9 CHARACTERIZATION, PURIFICATION, AND
DETERMINATION OF PROTEIN STRUCTURE
AND ORGANIZATION, 117
BIBLIOGRAPHY, 129
QUESTIONS AND ANSWERS, 131
X CONTENTS
CLINICAL CORRELATIONS
3.1 Plasma Proteins in Diagnosis of Disease, 87
3.2 Differences in Insulins Used in Treatment of
Diabetes Mellitus, 91
3.3 A Nonconservative Mutation Occurs in Sickle
Cell Anemia, 92
3.4 Diseases of Collagen Synthesis, 100
3.5 Hyperlipidemias, 105
3.6 Hypolipoproteinemias, 107
3.7 Glycosylated Hemoglobin, HbAic, 110
3.8 Proteins as Infectious Agents: Prions and
Human Transmissible Spongiform
Encephalopathies (TSEs), 111
3.9 Use of Amino Acid Analysis in Diagnosis of
Disease, 123
PART It I TRANSMISSION OF INFORMATION
4 ! DNA REPLICATION, RECOMBINATION, AND
REPAIR, 133
Howard J. Edenberg
4.1 COMMON FEATURES OF REPLICATION,
RECOMBINATION, AND REPAIR, 134
4.2 DNA REPLICATION, 135
4.3 RECOMBINATION, 153
4.4 REPAIR, 158
BIBLIOGRAPHY, 171
QUESTIONS AND ANSWERS, 171
CLINICAL CORRELATIONS
4.1 Chemotherapy Can Target Precursors of DNA
Synthesis, 136
4.2 Topoisomerases as Drug Targets, 146
4.3 Cancer and the Cell Cycle, 150
4.4 Nucleoside Analogs and Drug Resistance in
HIV Therapy, 151
4.5 Gene Therapy, 158
4.6 Chemotherapy, DNA Damage, and
Repair, 160
4.7 Nucleoside Analogs as Drugs:
Thiopurines, 161
4.8 Individualized Medicine, 161
4.9 Xeroderma Pigmentosum, 164
4.10 Mismatch Repair and Cancer, 166
5 j RNA: TRANSCRIPTION AND RNA PROCESSING,
175
Francis J. Schmidt and David R. Setzer
5.1 OVERVIEW, 176
5.2 MECHANISMS OF TRANSCRIPTION, 176
5.3 TRANSCRIPTION IN EUKARYOTES, 182
5.4 RNA PROCESSING, 188
5.5 RNA EXPORT AND QUALITY CONTROL, 195
5.6 SMALL INHIBITORY RNAs, 196
5.7 TRANSCRIPTION COUPLED DNA REPAIR, 196
5.8 NUCLEASES AND RNA TURNOVER, 197
BIBLIOGRAPHY, 198
QUESTIONS AND ANSWERS, 199
CLINICAL CORRELATIONS
5.1 Antibiotics and Toxins that Target RNA
Polymerase, 179
5.2 Fragile X Syndrome: An RNA Chromatin
Disease? 183
5.3 Involvement of Transcriptional Factors in
Carcinogenesis, 185
5.4 Thalassemia Due to Defects in Messenger
RNA Synthesis, 192
5.5 Autoimmunity in Connective Tissue
Disease, 193
5.6 Cockayne Syndrome, 197
6 | PROTEIN SYNTHESIS: TRANSLATION AND
POSTTRANSLATIONAL MODIFICATIONS, 201
Dohn Glitz
6.1 OVERVIEW, 202
6.2 COMPONENTS OF THE TRANSLATIONAL
APPARATUS, 202
6.3 PROTEIN BIOSYNTHESIS, 213
6.4 PROTEIN MATURATION: FOLDING,
MODIFICATION, SECRETION, AND
TARGETING, 222
6.5 MEMBRANE AND ORGANELLE
TARGETING, 227
6.6 FURTHER POSTTRANSLATIONAL
MODIFICATIONS, 231
6.7 REGULATION OF TRANSLATION, 236
6.8 PROTEIN DEGRADATION AND
TURNOVER, 239
BIBLIOGRAPHY, 240
QUESTIONS AND ANSWERS, 242
CLINICAL CORRELATIONS
6.1 Missense Mutations: Hemoglobin, 206
6.2 Mutation to Form a Terminator Codon, 206
6.3 a Thalassemia, 207
6.4 Programmed Frameshifting in Biosynthesis of
HIV Proteins, 208
6.5 Mutation in Mitochondrial Ribosomal RNA
Results in Antibiotic Induced Deafness, 221
6.6 Deletion of a Codon, Incorrect
Posttranslational Modification, and
Premature Protein Degradation: Cystic
Fibrosis, 223
6.7 Protein Misfolding and Aggregation:
Creutzfeldt Jacob Disease, Mad Cow
Disease, Alzheimer s Disease, and
Huntington s Disease, 224
6.8 Diseases of Lysosome Function, 230
6.9 Familial Hyperproinsulinemia, 233
6.10 Absence of Posttranslational Modification:
Multiple Sulfatase Deficiency, 234
6.11 Defects in Collagen Synthesis, 237
7 ! RECOMBINANT DNA AND BIOTECHNOLOGY,
245
Gerald Soslau
7.1 OVERVIEW, 246
7.2 THE POLYMERASE CHAIN REACTION, 247
7.3 RESTRICTION ENDONUCLEASE AND
RESTRICTION MAPS, 248
7.4 DNA SEQUENCING, 249
7.5 RECOMBINANT DNA AND CLONING, 251
7.6 SELECTION OF SPECIFIC CLONED DNA IN
LIBRARIES, 254
7.7 DETECTION AND IDENTIFICATION OF
NUCLEIC ACIDS AND DNA BINDING
PROTEINS, 258
7.8 COMPLEMENTARY DNA AND
COMPLEMENTARY DNA LIBRARIES, 263
7.9 BACTERIOPHAGE, COSMID, AND YEAST
CLONING VECTORS, 266
7.10 ANALYSIS OF LONG STRETCHES OF
DNA, 268
7.11 EXPRESSION VECTORS AND FUSION
PROTEINS, 269
7.12 EXPRESSION VECTORS IN EUKARYOTIC
CELLS, 271
7.13 SITE DIRECTED MUTAGENESIS, 272
7.14 APPLICATIONS OF RECOMBINANT DNA
TECHNOLOGIES, 277
7.15 GENOMICS, PROTEOMICS, AND
MICROARRAY ANALYSIS, 282
BIBLIOGRAPHY, 287
QUESTIONS AND ANSWERS, 287
CLINICAL CORRELATIONS
7.1 Poiymerase Chain Reaction, 248
7.2 Restriction Mapping and Evolution, 249
CONTENTS XJ
7.3 Direct Sequencing of DNA for the Diagnosis
of Genetic Disorders, 251
7.4 Multiplex PCR Analysis of the HGPTase
Gene Defects in the Lesch Nyhan
Syndrome, 254
7.5 Restriction Fragment Length Polymorphisms
Determine the Clonal Origin of
Tumors, 260
7.6 Single Strand Conformational
Polymorphism for Detection of
Spontaneous Mutations that May Lead to
SIDS, 261
7.7 Site Directed Mutagenesis of HSVIgD, 275
7.8 RNA Mediated Inhibition of HIV, 278
7.9 Gene Therapy: Normal Genes Can Be
Introduced into Cells with Defective
Genes, 279
7.10 Transgenic Animal Models, 280
7.11 Knockout Mice to Define a Role for the
P2Y, Purinoceptor, 281
7.12 Microarray Analysis of Breast Cancer, 283
8 REGULATION OF GENE EXPRESSION, 291
Daniel L. Weeks and John E. Donelson
8.1 OVERVIEW, 292
8.2 UNIT OF TRANSCRIPTION IN BACTERIA: THE
OPERON, 292
8.3 LACTOSE OPERON OF E. COLI, 293
8.4 TRYPTOPHAN OPERON OF £. COLI, 297
8.5 OTHER BACTERIAL OPERONS, 302
8.6 BACTERIAL TRANSPOSONS, 304
8.7 GENE EXPRESSION IN EUKARYOTES, 305
8.8 PREINITIATION COMPLEX IN EUKARYOTES:
TRANSCRIPTION FACTORS, RNA
POLYMERASE II, AND DNA, 307
8.9 REGULATION OF EUKARYOTIC GENE
EXPRESSION, 312
BIBLIOGRAPHY, 316
QUESTIONS AND ANSWERS, 317
CLINICAL CORRELATIONS
8.1 Transmissible Multiple Drug
Resistance, 305
8.2 Rubinstein Taybi Syndrome, 307
8.3 Tamoxifen and Targeting of Estrogen
Receptor, 314
8.4 Transcription Factors and Cardiovascular
Disease, 314
XH CONTENTS
PART fil | FUNCTIONS OF PROTEINS
9 I PROTEINS II: STRUCTURE FUNCTION
RELATIONSHIPS IN PROTEIN FAMILIES, 319
Richard M. Schultz
9.1 OVERVIEW, 320
9.2 ANTIBODY MOLECULES:
IMMUNOGLOBULIN SUPERFAMILY OF
PROTEINS, 320
9.3 PROTEINS WITH A COMMON CATALYTIC
MECHANISM: SERINE PROTEASES, 328
9.4 HEMOGLOBIN AND MYOGLOBIN, 338
9.5 THE BASAL LAMINA PROTEIN
COMPLEX, 352
BIBLIOGRAPHY, 360
QUESTIONS AND ANSWERS, 361
CLINICAL CORRELATIONS
9.1 The Complement Proteins, 323
9.2 Functions of Different Antibody
Classes, 323
9.3 Immunization, 324
9.4 Fibrin Formation in a Myocardial Infarct and
Use of Recombinant Tissue Plasminogen
Activator (rt PA), 330
9.5 Involvement of Serine Proteases in Tumor
Cell Metastasis, 330
9.6 Hemoglobinopathies, 339
10 I ENZYMES: CLASSIFICATION, KINETICS, AND
CONTROL, 365
Henry Weiner
10.1 OVERVIEW, 366
10.2 CLASSIFICATIONS OF ENZYMES, 367
10.3 GENERAL CONCEPTS OF ENZYME
MECHANISMS, 371
10.4 ACTIVE SITE OF AN ENZYME, 375
10.5 COENZYMES, CO SUBSTRATES, AND
COFACTORS, 378
10.6 KINETICS OF CHEMICAL REACTIONS, 384
10.7 ENZYME KINETICS OF ONE SUBSTRATE
REACTIONS, 386
10.8 KINETICS OF TWO SUBSTRATE
REACTIONS, 394
10.9 INHIBITORS, 395
10.10 REGULATION OF ENZYME ACTIVITY, 401
10.11 REGULATION OF METABOLIC
PATHWAYS, 404
10.12 CLINICAL APPLICATIONS OF
ENZYMES, 405
BIBLIOGRAPHY, 410
QUESTIONS AND ANSWERS, 410
CLINICAL CORRELATIONS
10.1 Mutation of a Coenzyme Binding Site
Results in Clinical Disease, 378
10.2 A Case of Gout Demonstrates Two
Phases in the Mechanism of Enzyme
Action, 389
10.3 Physiological Effect of Changes in
Enzyme Km Values, 390
10.4 Thermal Lability of Glucose 6 Phosphate
Dehydrogenase Results in Hemolytic
Anemia, 393
10.5 Alcohol Dehydrogenase Isoenzymes
with Different pH Optima, 394
10.6 Inhibitors of Xanthine Oxidase Isolated
from Plants, 396
10.7 Design of a Selective Inhibitor, 397
10.8 A Case of Poisoning, 400
10.9 Mushrooms and Alcohol
Metabolism, 400
10.10 A Case of Gout Demonstrates the
Difference Between an Allosteric and
Substrate Binding Site, 401
10.11 Identification and Treatment of an
Enzyme Deficiency, 407
10.12 Ambiguity in the Assay of Mutated
Enzymes, 407
11 | THE CYTOCHROMES P450 AND NITRIC OXIDE
SYNTH ASES, 413
Linda J. Roman and Bettie Sue Siler Masters
11.1 OVERVIEW, 414
11.2 CYTOCHROMES P450: PROPERTIES AND
FUNCTION, 414
11.3 CYTOCHROME P450 REACTION
CYCLE, 415
11.4 CYTOCHROME P450 ELECTRON
TRANSPORT SYSTEMS, 416
11.5 CYTOCHROME P450: NOMENCLATURE
AND ISOFORMS, 418
11.6 CYTOCHROME P450: SUBSTRATES AND
PHYSIOLOGICAL FUNCTIONS, 419
11.7 CYTOCHROMES P450 PARTICIPATE IN THE
SYNTHESIS OF STEROID HORMONES AND
OXYGENATION OF ENDOGENOUS
COMPOUNDS, 420
11.8 CYTOCHROME P450 INDUCTION AND
INHIBITION, 428
11.9 THE NITRIC OXIDE SYNTHASES:
PROPERTIES AND FUNCTION, 432
11.10 NITRIC OXIDE SYNTHASE ISOFORMS AND
PHYSIOLOGICAL FUNCTIONS, 433
BIBLIOGRAPHY, 439
QUESTIONS AND ANSWERS, 440
CLINICAL CORRELATIONS
11.1 Congenital Adrenal Hyperplasia:
Deficiency of CYP21A2, 423
11.2 Steroid Hormone Production During
Pregnancy, 423
11.3 Cytochrome P450 Inhibition:
Drug Drug Interactions and Adverse
Effects, 426
11.4 RoleofCYP2E1 in
Acetaminophen lnduced Liver
Toxicity, 428
11.5 Cytochrome P450 Induction:
Drug Drug Interactions and Adverse
Effects, 429
11.6 Genetic Polymorphisms of P450
Enzymes, 431
11.7 Mechanism of Action of Sildenafil, 436
11.8 Clinical Aspects of Nitric Oxide
Production, 437
11.9 History of Nitroglycerin, 438
12 I BIOLOGICAL MEMBRANES: STRUCTURE AND
MEMBRANE TRANSPORT, 443
Thomas M. Devlin
12.1 OVERVIEW, 444
12.2 CHEMICAL COMPOSITION OF
MEMBRANES, 444
12.3 MICELLES, LIPID BILAYERS, AND
LIPOSOMES, 450
12.4 STRUCTURE OF BIOLOGICAL
MEMBRANES, 453
12.5 MOVEMENT OF MOLECULES THROUGH
MEMBRANES, 461
12.6 MEMBRANE CHANNELS, 464
12.7 MEMBRANE TRANSPORTERS, 472
12.8 PASSIVE TRANSPORT, 474
12.9 ACTIVE TRANSPORT, 475
12.10 IONOPHORES, 483
BIBLIOGRAPHY, 485
QUESTIONS AND ANSWERS, 485
CLINICAL CORRELATIONS
12.1 Liposomes as Carriers of Drugs and
Enzymes, 452
CONTENTS Xili
12.2 Abnormalities of Cell Membrane Fluidity
in Disease, 459
12.3 Cystic Fibrosis and the Cl~
Channel, 466
12.4 The Mammalian Kidney and
Aquaporins, 468
12.5 Diseases Involving the Superfamily of
ABC Transporters, 481
12.6 Diseases Due to Loss of Membrane
Transport Systems, 483
13 FUNDAMENTALS OF SIGNAL TRANSDUCTION,
489
George R. Dubyak
13.1 OVERVIEW, 490
13.2 INTERCELLULAR SIGNAL
TRANSDUCTION, 491
13.3 RECEPTORS FOR SECRETED
MOLECULES, 493
13.4 INTRACELLULAR SIGNAL TRANSDUCTION
BY CELL SURFACE RECEPTORS, 494
13.5 LIGAND GATED ION CHANNEL
RECEPTORS, 500
13.6 ENZYME LINKED RECEPTORS, 503
13.7 CYTOKINE RECEPTORS, 507
13.8 G PROTEIN COUPLED RECEPTORS, 509
13.9 CYCLIC AMP BASED SIGNAL
TRANSDUCTION, 515
13.10 CYCLIC GMP BASED SIGNAL
TRANSDUCTION, 518
13.11 CALCIUM BASED SIGNAL
TRANSDUCTION, 519
13.12 PHOSPHOLIPID BASED SIGNAL
TRANSDUCTION, 522
13.13 INTEGRATION OF SIGNAL TRANSDUCTION
PATHWAYS INTO SIGNAL TRANSDUCTION
NETWORKS, 524
BIBLIOGRAPHY, 525
QUESTIONS AND ANSWERS, 526
CLINICAL CORRELATIONS
13.1 ErbB/HER Farnily Receptor Tyrosine
Kinases as Targets for Cancer
Chemotherapy, 505
13.2 G Protein Coupled Chemokine
Receptors as Targets for the Human
Immunodeficiency Virus (HIV), 510
13.3 Gsa G Protein Mutations in Pituitary
Gland Tumors and Endocrine
Diseases, 512
XiV CONTENTS
13.4 Alterations in /3 Adrenergic Receptor
Signaling Proteins in Congestive Heart
Failure, 515
13.5 Nitric Oxide/cGMP Signaling Axis as
Therapeutic Targets in Cardiac and
Vascular Disorders, 519
PART IV | METABOLIC PATHWAYS AND
THEIR CONTROL
14 | BIOENERGETICS AND OXIDATIVE METABOLISM,
529
Diana S. Beattie
14.1 ENERGY PRODUCING AND
ENERGY UTILIZING SYSTEMS, 530
14.2 THERMODYNAMIC RELATIONSHIPS AND
ENERGY RICH COMPONENTS, 532
14.3 SOURCES AND FATES OF ACETYL
COENZYMEA, 538
14.4 THE TRICARBOXYLIC ACID CYCLE, 543
14.5 STRUCTURE AND COMPARTMENTATION
BY MITOCHONDRIAL MEMBRANES, 550
14.6 ELECTRON TRANSPORT CHAIN, 552
14.7 OXIDATIVE PHOSPHORYLATION, 562
14.8 MITOCHONDRIAL INNER MEMBRANE
CONTAINS SUBSTRATE TRANSPORT
SYSTEMS, 568
14.9 MITOCHONDRIAL GENES AND
DISEASES, 573
14.10 REACTIVE OXYGEN SPECIES (ROS), 574
BIBLIOGRAPHY, 578
QUESTIONS AND ANSWERS, 579
CLINICAL CORRELATIONS
14.1 Pyruvate Dehydrogenase
Deficiency, 543
14.2 Fumarase Deficiency, 546
14.3 Cyanide Poisoning, 562
14.4 Leber s Hereditary Optic
Neuropathy, 574
14.5 Mitochondrial Myopathies from
Mutations in tRNA Genes, 575
14.6 Exercise Intolerance in Patients with
Mutations in Cytochrome b, 575
14.7 Ischemia/Reperfusion Injury, 577
15 { CARBOHYDRATE METABOLISM I: MAJOR
METABOLIC PATHWAYS AND THEIR CONTROL,
581
Robert A. Harris
15.1 OVERVIEW, 582
15.2 GLYCOLYSIS, 583
15.3 GLYCOLYSIS PATHWAY, 587
15.4 REGULATION OF GLYCOLYSIS, 595
15.5 GLUCONEOGENESIS, 608
15.6 GLYCOGENOLYSIS AND
GLYCOGENESIS, 618
BIBLIOGRAPHY, 633
QUESTIONS AND ANSWERS, 634
CLINICAL CORRELATIONS
15.1 Alcohol and Barbiturates, 594
15.2 Arsenic Poisoning, 595
15.3 Fructose Intolerance, 597
15.4 Diabetes Mellitus, 599
15.5 Lactic Acidosis, 601
15.6 Pickled Pigs and Malignant
Hyperthermia, 602
15.7 Angina Pectoris and Myocardial
Infarction, 603
15.8 Pyruvate Kinase Deficiency and
Hemolytic Anemia, 608
15.9 Hypoglycemia and Premature
Infants, 609
15.10 Hypoglycemia and Alcohol
Intoxication, 618
15.11 Glycogen Storage Diseases, 621
16 | CARBOHYDRATE METABOLISM II: SPECIAL
PATHWAYS AND GLYCOCONJUGATES, 637
Nancy B. Schwartz
16.1 OVERVIEW, 638
16.2 PENTOSE PHOSPHATE PATHWAY, 638
16.3 SUGAR INTERCONVERSIONS AND
NUCLEOTIDE LINKED SUGAR
FORMATION, 643
16.4 BIOSYNTHESIS OF COMPLEX
POLYSACCHARIDES, 648
16.5 GLYCOPROTEINS, 649
16.6 PROTEOGLYCANS, 652
BIBLIOGRAPHY, 658
QUESTIONS AND ANSWERS, 658
CLINICAL CORRELATIONS
16.1 Glucose 6 Phosphate Dehydrogenase:
Genetic Deficiency or Variants in
Erythrocytes, 640
16.2 Wernicke Korsakoff Syndrome:
Deficiency or Genetic Variants of
Transketolase, 641
16.3 Carbohydrate Deficient Glycoprotein
Syndromes (CDGS), 644
16.4 Essential Fructosuria and Fructose
Intolerance: Deficiency of Fructokinase
and Fructose 1 phosphate
Aldolase, 644
16.5 Gaiactosemia: Inability to Transform
Galactose into Glucose, 645
16.6 Pentosuria: Deficiency of Xylitol
Dehydrogenase, 646
16.7 Glucuronic Acid: Physiological
Significance of Glucuronide
Formation, 647
16.8 Blood Group Substances, 649
16.9 Common Carbohydrate Marker of
Lysosomal Targeting and I Cell
Disease, 652
16.10 Aspartylglycosylaminuria: Absence of
4 L Aspartylglycosamine
Amidohydrolase, 653
16.11 Glycolipid Disorders, 654
16.12 Heparin Is an Anticoagulant, 655
16.13 Chondrodystrophies Due to Sulfation
Defects, 655
16.14 Mucopolysaccharidoses, 657
17 ! LIPID METABOLISM I: SYNTHESIS, STORAGE,
AND UTILIZATION OF FATTY ACIDS AND
TRIACYLGLYCEROLS, 661
Martin D. Snider, J. Denis McGarry, and Richard
W. Hanson
17.1 OVERVIEW, 662
17.2 CHEMICAL NATURE OF FATTY ACIDS AND
ACYLGLYCEROLS, 663
17.3 INTERORGAN TRANSPORT OF FATTY
ACIDS AND THEIR PRIMARY
PRODUCTS, 665
17.4 SYNTHESIS OF FATTY ACIDS:
LIPOGENESIS, 668
17.5 STORAGE OF FATTY ACIDS AS
TRIACYLGLYCEROL, 676
17.6 UTILIZATION OF FATTY ACIDS FOR
ENERGY PRODUCTION, 680
17.7 REGULATION OF LIPID METABOLISM, 690
BIBLIOGRAPHY, 692
QUESTIONS AND ANSWERS, 693
CLINICAL CORRELATIONS
17.1 Obesity, 665
17.2 Role of Fatty Acid Metabolism in Type 2
Diabetes, 666
CONTENTS XV
17.3 Triacylglycerol/Fatty Acid Cycle, 679
17.4 Genetic Deficiencies in Carnitine
Transport or Carnitine
Palmitoyltransferase, 682
17.5 Genetic Deficiencies in the Acyl CoA
Dehydrogenases, 684
17.6 Refsum s Disease, 686
17.7 Ketone Bodies as a Fuel: The Atkins
Diet, 689
18 i LIPID METABOLISM II: PATHWAYS OF
METABOLISM OF SPECIAL LIPIDS, 695
Robert H. Glew
18.1 OVERVIEW, 696
18.2 PHOSPHOLIPIDS, 696
18.3 CHOLESTEROL, 707
18.4 SPHINGOLIPIDS, 720
18.5 PROSTAGLANDINSAND
THROMBOXANES, 730
18.6 UPOXYGENASEAND
OXYEICOSATETRAENOIC ACIDS, 734
BIBLIOGRAPHY, 738
QUESTIONS AND ANSWERS, 739
CLINICAL CORRELATIONS
18.1 Respiratory Distress Syndrome, 699
18.2 Treatment of
Hypercholesterolemia, 717
18.3 Atherosclerosis, 717
18.4 Diagnosis of Gaucher s Disease in an
Adult, 729
19 AMINO ACID METABOLISM, 743
Marguerite W. Coomes
19.1 OVERVIEW, 744
19.2 INCORPORATION OF NITROGEN INTO
AMINO ACIDS, 745
19.3 TRANSPORT OF NITROGEN TO LIVER AND
KIDNEY, 750
19.4 UREA CYCLE, 751
19.5 SYNTHESIS AND DEGRADATION OF
INDIVIDUAL AMINO ACIDS, 754
BIBLIOGRAPHY, 784
QUESTIONS AND ANSWERS, 785
CLINICAL CORRELATIONS
19.1 Carbamoyl Phosphate Synthetase and
iV Acetylglutarnate Synthetase
Deficiencies, 754
19.2 Deficiency of Urea Cycle Enzymes, 755
XVi CONTENTS
19.3 Diseases of Proline Metabolism, 756
19.4 Selenoproteins, 759
19.5 Nonketotic Hyperglycinemia, 760
19.6 Folic Acid Deficiency, 762
19.7 Phenylketonuria, 764
19.8 Disorders of Tyrosine Metabolism, 766
19.9 Parkinson s Disease, 766
19.10 Hyperhomocysteinemia and
Atherogenesis, 770
19.11 Diseases of Sulfur Amino Acids, 770
19.12 Glutaric Aciduria, 773
19.13 Schizophrenia and Other
Tryptophan Derived
Neurotransmitter Associated
Diseases, 775
19.14 Diseases of Metabolism of
Branched Chain Amino Acids, 777
19.15 Diseases of Propionate and
Methylmalonate Metabolism, 780
19.16 Diseases Involving Lysine and
Ornithine, 780
19.17 Histidinemia, 781
19.18 Diseases of Folate Metabolism, 782
«
20 ! PURINE AND PYRIMIDINE NUCLEOTIDE
METABOLISM, 789
Joseph G. Cory
20.1 OVERVIEW, 790
20.2 METABOLIC FUNCTIONS OF
NUCLEOTIDE, 790
20.3 METABOLISM OF PURINE
NUCLEOTIDES, 791
20.4 METABOLISM OF PYRIMIDINE
NUCLEOTIDES, 802
20.5 DEOXYRIBONUCLEOTIDE
FORMATION, 806
20.6 NUCLEOSIDE AND NUCLEOTIDE
KINASES, 808
20.7 NUCLEOTIDE METABOLIZING ENZYMES
AS A FUNCTION OF THE CELL CYCLE AND
RATE OF CELL DIVISION, 810
20.8 NUCLEOTIDE COENZYME SYNTHESIS, 811
20.9 SYNTHESIS AND UTILIZATION OF
5 PHOSPHORIBOSYL 1
PYROPHOSPHATE, 813
20.10 CHEMOTHERAPEUTIC AGENTS THAT
INTERFERE WITH PURINE AND
PYRIMIDINE NUCLEOTIDE
METABOLISM, 814
BIBLIOGRAPHY, 819
QUESTIONS AND ANSWERS, 820
CLINICAL CORRELATIONS
20.1 Gout, 796
20.2 Lesch Nyhan Syndrome, 798
20.3 Increased Cytosolic 5 Nucleotidase
Activity, 800
20.4 Immunodeficiency Diseases Associated
with Defects in Purine Nucleoside
Degradation, 801
20.5 Patients Undergoing Cancer
Treatment, 802
20.6 Subclass of Patients with Autism, 803
20.7 Hereditary Orotic Aciduria, 804
21 [ IRON AND HEME METABOLISM, 823
William M. Awad, Jr.
21.1 IRON METABOLISM: OVERVIEW, 824
21.2 IRON CONTAINING PROTEINS, 824
21.3 INTESTINAL ABSORPTION OF IRON, 826
21.4 MOLECULAR REGULATION OF IRON
UTILIZATION, 828
21.5 IRON DISTRIBUTION AND KINETICS, 830
21.6 HEME BIOSYNTHESIS, 833
21.7 HEME CATABOLISM, 840
BIBLIOGRAPHY, 845
QUESTIONS AND ANSWERS, 845
CLINICAL CORRELATIONS
21.1 Iron Overload and Infection, 825
21.2 Microbial Pathogenicity and Iron, 825
21.3 Iron Sulfur Cluster Synthesis and
Human Disease, 827
21.4 Friedreich s Ataxia, 827
21.5 Duodenal Iron Absorption, 828
21.6 Mutant Iron Responsive Elements, 830
21.7 Ceruloplasmin Deficiency, 831
21.8 Iron Deficiency Anemia, 832
21.9 Hemochromatosis Type I: Molecular
Genetics and the Issue of Iron Fortified
Diets, 832
21.10 Hemochromatosis Type III, 833
21.11 Acute Intermittent Porphyria, 836
21.12 Cytoprotective Role of Heme
Oxygenase, 841
21.13 Neonatal Isoimmune Hemolysis, 843
21.14 Bilirubin UDP Glucuronosyltransferase
Deficiency, 843
21.15 Elevation of Serum Conjugated
Bilirubin, 844
22 | METABOLIC INTERRELATIONSHIPS, 849
Robert A. Harris and David W. Crabb
22.1 OVERVIEW, 850
22.2 STARVE FEED CYCLE, 850
22.3 MECHANISMS INVOLVED IN SWITCHING
LIVER METABOLISM BETWEEN THE
WELL FED AND STARVED STATES, 863
22.4 INTERRELATIONSHIP OF TISSUES IN
NUTRITIONAL AND HORMONAL
STATES, 873
BIBLIOGRAPHY, 887
QUESTIONS AND ANSWERS, 889
CLINICAL CORRELATIONS
22.1 Obesity, 851
22.2 Protein Malnutrition, 852
22.3 Starvation, 852
22.4 Reye s Syndrome, 857
22.5 Hyperglycemic, Hyperosmolar
Coma, 861
22.6 Hyperglycemia and Protein
Glycation, 861
22.7 Diabetes Mellitus, Type 2, 876
22.8 Diabetes Mellitus, Type 1, 877
22.9 Polyol Pathway and Complications of
Diabetes, 878
22.10 Cancer Cachexia, 879
PART V | PHYSIOLOGICAL PROCESSES
23 { BIOCHEMISTRY OF HORMONES, 891
Thomas J. Schmidt and Gerald Litwack
23.1 OVERVIEW, 892
23.2 HORMONES AND THE HORMONAL
CASCADE SYSTEM, 893
23.3 SYNTHESIS OF POLYPEPTIDE AND AMINO
ACID DERIVED HORMONES, 900
23.4 PROTEIN HORMONE SIGNALING, 906
23.5 MEMBRANE HORMONE RECEPTORS, 914
CONTENTS XVM
23.6 INTRACELLULAR HORMONE CASCADE:
PROTEIN KINASES, 917
23.7 STEROID HORMONES, 925
23.8 STEROID HORMONE RECEPTORS. 938
BIBLIOGRAPHY, 945
QUESTIONS AND ANSWERS, 946
CLINICAL CORRELATIONS
23.1 Testing Activity of the Anterior
Pituitary, 896
23.2 Hypopituitarism, 901
23.3 Decreased Insulin Receptor Kinase
Activity in Gestational Diabetes
Mellitus, 920
23.4 Oral Contraception, 937
23.5 Apparent Mineralocorticoid Excess
Syndrome, 940
23.6 Mineralocorticoid Receptor Mutation
Results in Hypertension and Toxemia of
Pregnancy, 943
24 j MOLECULAR CELL BIOLOGY, 949
Thomas E. Smith
24.1 OVERVIEW, 950
24.2 NERVOUS TISSUE: METABOLISM AND
FUNCTION, 950
24.3 THE EYE: METABOLISM AND VISION, 961
24.4 MOLECULAR MOTORS AND ASSOCIATED
PROTEINS, 976
24.5 MECHANISM OF BLOOD
COAGULATION, 991
BIBLIOGRAPHY, 1008
QUESTIONS AND ANSWERS, 1010
CLINICAL CORRELATIONS
24.1 Lambert Eaton Myasthenic
Syndrome, 957
24.2 Myasthenia Gravis: A Neuromuscular
Disorder, 958
24.3 Macula Degeneration and Loss of
Vision, 965
24.4 Niemann Pick Disease and Retinitis
Pigmentosa, 966
24.5 Retinitis Pigmentosa from a Mutation in
the Gene for Peripherin, 967
24.6 Leber Congenital Amaurosis: Retinal
Dystrophy Leading to Blindness, 973
24.7 Glycation and Myosin Structure and
Function, 980
24.8 Familial Hypertropic Cardiomyopathies
and Mutations in Muscle Proteins, 981
24.9 Dilated Cardiomyopathy and Mutations
inActin, 982
XViii CONTENTS
24.10 Troponin Subunits as Markers for
Myocardial Infarction, 985
24.11 Voltage Gated Ion
Channelopathies, 986
24.12 Ion Channels and Cardiac Muscle
Disease, 986
24.13 Mutations Affecting Pigmentation: Is
There a Molecular Motor
Connection? 989
24.14 Intrinsic Pathway Defects: Prekallikrein
Deficiency, 994
24.15 Classic Hemophilia, 999
24.16 Use of Recombinant Factor Vila to
Control Bleeding, 999
24.17 Thrombosis: Defects of the Protein C
Pathway and Increased Levels of
Coagulation Factors, 1004
25 ! CELL CYCLE, PROGRAMMED CELL DEATH, AND
CANCER, 1013
Richard M. Schultz
25.1 OVERVIEW, 1014
25.2 CELL CYCLE, 1014
25.3 APOPTOSIS: PROGRAMMED CELL
DEATH, 1020
25.4 CANCER, 1024
BIBLIOGRAPHY, 1032
QUESTIONS AND ANSWERS, 1033
CLINICAL CORRELATIONS
25.1 Oncogenic DNA Viruses, 1025
25.2 Molecularly Targeted Anticancer
Drug, 1029
25.3 Environmental Cause of Human
Cancers, 1030
26 DIGESTION AND ABSORPTION OF BASIC
NUTRITIONAL CONSTITUENTS, 1037
Ulrich Hopfer
26.1 OVERVIEW, 1038
26.2 GENERAL CONSIDERATIONS, 1039
26.3 EPITHELIAL TRANSPORT, 1044
26.4 DIGESTION AND ABSORPTION OF
PROTEINS, 1052
26.5 DIGESTION AND ABSORPTION OF
CARBOHYDRATES, 1056
26.6 DIGESTION AND ABSORPTION OF
LIPIDS, 1059
26.7 BILE ACID METABOLISM, 1065
BIBLIOGRAPHY, 1067
QUESTIONS AND ANSWERS, 1068
CLINICAL CORRELATIONS
26.1 Familial Chloridorrhea Causes Metabolic
Alkalosis, 1046
26.2 Cystic Fibrosis, 1049
26.3 Bacterial Toxigenic Diarrheas and
Electrolyte Replacement Therapy, 1049
26.4 Neutral Amino Aciduria: Hartnup
Disease, 1054
26.5 Disaccharidase Deficiency, 1058
26.6 Pharmacological Interventions to
Prevent Fat Absorption and
Obesity, 1061
26.7 Cholesterol Stones, 1064
26.8 A /3 Lipoproteinemia, 1065
27 ! PRINCIPLES OF NUTRITION I: MACRONUTRIENTS,
1071
Stephen G. Chaney
27.1 OVERVIEW, 1072
27.2 ENERGY METABOLISM, 1072
27.3 PROTEIN METABOLISM, 1073
27.4 PROTEIN ENERGY MALNUTRITION, 1076
27.5 EXCESS PROTEIN ENERGY INTAKE, 1077
27.6 CARBOHYDRATES, 1079
27.7 FATS, 1079
27.8 FIBER, 1080
27.9 COMPOSITION OF MACRONUTRIENTS IN
THE DIET, 1082
BIBLIOGRAPHY, 1086
QUESTIONS AND ANSWERS, 1088
CLINICAL CORRELATIONS
27.1 Vegetarian Diets and Protein Energy
Requirements for Children, 1075
27.2 Dietary Protein Intake and Renal
Disease, 1076
27.3 Providing Adequate Protein and
Calories for Hospitalized Patients, 1077
27.4 Carbohydrate Loading and Athletic
Endurance, 1080
27.5 High Carbohydrate Versus High Fat Diet
for Diabetics, 1081
27.6 Polyunsaturated Fatty Acids and Risk
Factors for Heart Disease, 1083
27.7 Metabolic Adaptation: Relationship
Between Carbohydrate Intake and
Serum Triacylglycerols, 1086
28 | PRINCIPLES OF NUTRITION II: MICRONUTRIENTS,
1091
Stephen G. Chaney
28.1 OVERVIEW, 1092
28.2 ASSESSMENT OF MALNUTRITION, 1092
28.3 DIETARY REFERENCE INTAKES, 1093
28.4 FAT SOLUBLE VITAMINS, 1093
28.5 WATER SOLUBLE VITAMINS, 1102
28.6 ENERGY RELEASING WATER SOLUBLE
VITAMINS, 1102
28.7 HEMATOPOIETIC WATER SOLUBLE
VITAMINS, 1107
28.8 OTHER WATER SOLUBLE VITAMINS, 1110
28.9 MACROMINERALS, 1112
28.10 TRACE MINERALS, 1113
28.11 THE AMERICAN DIET: FACT AND
FALLACY, 1115
28.12 ASSESSMENT OF NUTRITIONAL STATUS IN
CLINICAL PRACTICE, 1116
BIBLIOGRAPHY, 1117
QUESTIONS AND ANSWERS, 1119
CLINICAL CORRELATIONS
28.1 Nutritional Considerations in Cystic
Fibrosis, 1096
CONTENTS XIX
28.2 Renal Osteodystrophy, 1097
28.3 Nutritional Considerations in Newborn
Infants, 1101
28.4 Anticonvulsant Drugs and Vitamin
Requirements, 1101
28.5 Nutritional Considerations in
Alcoholics, 1103
28.6 Vitamin B6 Requirements and Oral
Contraceptives, 1106
28.7 Gene Polymorphisms and Folic Acid
Requirement, 1108
28.8 Diet and Osteoporosis, 1113
28.9 Nutritional Needs of Elderly
Persons, 1117
APPENDIX REVIEW OF ORGANIC
CHEMISTRY, 1121
Carol N. Angstadt
GLOSSARY, 1133
Francis Vella
INDEX, 1157
|
| adam_txt |
CONTENTS
PART I I STRUCTURE OF MACROMOLECULES
1 Eukaryotic Cell Structure, 1
2 DNA and RNA: Composition and Structure, 23
3 Proteins I: Composition and Structure, 75
PART II | TRANSMISSION OF INFORMATION
4 DNA Replication, Recombination, and Repair,
133
5 RNA: Transcription and RNA Processing, 175
6 Protein Synthesis: Translation and
Posttranslational Modifications, 201
7 Recombinant DNA and Biotechnology, 245
8 Regulation of Gene Expression, 291
PART |jl | FUNCTIONS OF PROTEINS
9 Proteins II: Structure Function Relationships in
Protein Families, 319
10 Enzymes: Classification, Kinetics, and Control,
365
11 The Cytochromes P450 and Nitric Oxide
Synthases, 413
12 Biological Membranes: Structure and Membrane
Transport, 443
13 Fundamentals of Signal Transduction, 489
PART W I METABOLIC PATHWAYS AND THEIR
CONTROL
14 Bioenergetics and Oxidative Metabolism, 529
ITS IN BRIEF
15 Carbohydrate Metabolism I: Major Metabolic
Pathways and their Control, 581
16 Carbohydrate Metabolism II: Special Pathways
and Glycoconjugates, 637
17 Lipid Metabolism I: Synthesis, Storage, and
Utilization of Fatty Acids and Triacylglycerols,
661
18 Lipid Metabolism II: Pathways of Metabolism of
Special Lipids, 695
19 Amino Acid Metabolism, 743
20 Purine and Pyrimidine Nucleotide Metabolism,
789
21 Iron and Heme Metabolism, 823
22 Metabolic Interrelationships, 849
PART V I PHYSIOLOGICAL PROCESSES
23 Biochemistry of Hormones, 891
24 Molecular Cell Biology, 949
25 Cell Cycle, Programmed Cell Death, and Cancer,
1013
26 Digestion and Absorption of Basic Nutritional
Constituents, 1037
27 Principles of Nutrition I: Macronutrients, 1071
28 Principles of Nutrition II: Micronutrients, 1091
Appendix Review of Organic Chemistry, 1121
Glossary, 1133
Index. 1157
PREFACE, xxi
ACKNOWLEDGMENTS, xxiii
CONTRIBUTORS, xxv
PART g 1 STRUCTURE OF MACROMOLECULES
1 | EUKARYOTIC CELL STRUCTURE, 1
Thomas M. Devlin
1.1 OVERVIEW: CELLS AND CELLULAR
COMPARTMENTS, 2
1.2 WATER, pH, AND SOLUTES: THE AQUEOUS
ENVIRONMENT OF CELLS, 3
1.3 COMPOSITION OF EUKARYOTIC CELLS:
FUNCTIONAL ROLES OF SUBCELLULAR
ORGANELLES AND MEMBRANE SYSTEMS, 11
1.4 INTEGRATION AND CONTROL OF CELLULAR
FUNCTIONS, 19
BIBLIOGRAPHY, 20
QUESTIONS AND ANSWERS, 20
CLINICAL CORRELATIONS
1.1 Blood Bicarbonate Concentration in
Metabolic Acidosis, 10
1.2 Mitochondrial Diseases, 15
1.3 Lysosomal Enzymes and Gout, 16
1.4 Lysosomal Acid Lipase Deficiency, 18
1.5 Peroxisome Biogenesis Disorders (PBD), 19
2 ! DNA AND RNA: COMPOSITION AND STRUCTURE,
23
Stephen A. Woski and Francis J. Schmidt
2.1 OVERVIEW, 24
2.2 STRUCTURAL COMPONENTS OF NUCLEIC
ACIDS: NUCLEOBASES, NUCLEOSIDES, AND
NUCLEOTIDES, 25
2.3 STRUCTURE OF DNA, 29
2.4 HIGHER ORDER DNA STRUCTURE, 46
2.5 DNA SEQUENCE AND FUNCTION, 58
2.6 RNA STRUCTURE, 62
2.7 TYPES OF RNA, 64
TS
BIBLIOGRAPHY, 71
QUESTIONS AND ANSWERS, 71
CLINICAL CORRELATIONS
2.1 DNA Vaccines, 25
2.2 Diagnostic Use of DNA Arrays in Medicine
and Genetics, 39
2.3 Antitumor Antibiotics that Change the Shape
of DNA, 42
2.4 Hereditary Persistence of Fetal
Hemoglobin, 45
2.5 Telomerase as a Target for Anticancer
Agents, 46
2.6 Expansion of DNA Triple Repeats and Human
Disease, 49
2.7 Topoisomerases in the Treatment of
Disease, 53
2.8 Staphylococcal Resistance to
Erythromycin, 67
3 | PROTEINS I: COMPOSITION AND STRUCTURE, 75
Richard M. Schultz and Michael N. Liebman
3.1 FUNCTIONAL ROLES OF PROTEINS IN
HUMANS, 76
3.2 AMINO ACID COMPOSITION OF PROTEINS, 77
3.3 CHARGE AND CHEMICAL PROPERTIES OF
AMINO ACIDS AND PROTEINS, 81
3.4 PRIMARY STRUCTURE OF PROTEINS, 90
3.5 HIGHER LEVELS OF PROTEIN
ORGANIZATION, 92
3.6 OTHER TYPES OF PROTEINS, 99
3.7 FOLDING OF PROTEINS FROM RANDOMIZED
TO UNIQUE STRUCTURES: PROTEIN
STABILITY, 109
3.8 DYNAMIC ASPECTS OF PROTEIN
STRUCTURE, 116
3.9 CHARACTERIZATION, PURIFICATION, AND
DETERMINATION OF PROTEIN STRUCTURE
AND ORGANIZATION, 117
BIBLIOGRAPHY, 129
QUESTIONS AND ANSWERS, 131
X CONTENTS
CLINICAL CORRELATIONS
3.1 Plasma Proteins in Diagnosis of Disease, 87
3.2 Differences in Insulins Used in Treatment of
Diabetes Mellitus, 91
3.3 A Nonconservative Mutation Occurs in Sickle
Cell Anemia, 92
3.4 Diseases of Collagen Synthesis, 100
3.5 Hyperlipidemias, 105
3.6 Hypolipoproteinemias, 107
3.7 Glycosylated Hemoglobin, HbAic, 110
3.8 Proteins as Infectious Agents: Prions and
Human Transmissible Spongiform
Encephalopathies (TSEs), 111
3.9 Use of Amino Acid Analysis in Diagnosis of
Disease, 123
PART It I TRANSMISSION OF INFORMATION
4 ! DNA REPLICATION, RECOMBINATION, AND
REPAIR, 133
Howard J. Edenberg
4.1 COMMON FEATURES OF REPLICATION,
RECOMBINATION, AND REPAIR, 134
4.2 DNA REPLICATION, 135
4.3 RECOMBINATION, 153
4.4 REPAIR, 158
BIBLIOGRAPHY, 171
QUESTIONS AND ANSWERS, 171
CLINICAL CORRELATIONS
4.1 Chemotherapy Can Target Precursors of DNA
Synthesis, 136
4.2 Topoisomerases as Drug Targets, 146
4.3 Cancer and the Cell Cycle, 150
4.4 Nucleoside Analogs and Drug Resistance in
HIV Therapy, 151
4.5 Gene Therapy, 158
4.6 Chemotherapy, DNA Damage, and
Repair, 160
4.7 Nucleoside Analogs as Drugs:
Thiopurines, 161
4.8 Individualized Medicine, 161
4.9 Xeroderma Pigmentosum, 164
4.10 Mismatch Repair and Cancer, 166
5 j RNA: TRANSCRIPTION AND RNA PROCESSING,
175
Francis J. Schmidt and David R. Setzer
5.1 OVERVIEW, 176
5.2 MECHANISMS OF TRANSCRIPTION, 176
5.3 TRANSCRIPTION IN EUKARYOTES, 182
5.4 RNA PROCESSING, 188
5.5 RNA EXPORT AND QUALITY CONTROL, 195
5.6 SMALL INHIBITORY RNAs, 196
5.7 TRANSCRIPTION COUPLED DNA REPAIR, 196
5.8 NUCLEASES AND RNA TURNOVER, 197
BIBLIOGRAPHY, 198
QUESTIONS AND ANSWERS, 199
CLINICAL CORRELATIONS
5.1 Antibiotics and Toxins that Target RNA
Polymerase, 179
5.2 Fragile X Syndrome: An RNA Chromatin
Disease? 183
5.3 Involvement of Transcriptional Factors in
Carcinogenesis, 185
5.4 Thalassemia Due to Defects in Messenger
RNA Synthesis, 192
5.5 Autoimmunity in Connective Tissue
Disease, 193
5.6 Cockayne Syndrome, 197
6 | PROTEIN SYNTHESIS: TRANSLATION AND
POSTTRANSLATIONAL MODIFICATIONS, 201
Dohn Glitz
6.1 OVERVIEW, 202
6.2 COMPONENTS OF THE TRANSLATIONAL
APPARATUS, 202
6.3 PROTEIN BIOSYNTHESIS, 213
6.4 PROTEIN MATURATION: FOLDING,
MODIFICATION, SECRETION, AND
TARGETING, 222
6.5 MEMBRANE AND ORGANELLE
TARGETING, 227
6.6 FURTHER POSTTRANSLATIONAL
MODIFICATIONS, 231
6.7 REGULATION OF TRANSLATION, 236
6.8 PROTEIN DEGRADATION AND
TURNOVER, 239
BIBLIOGRAPHY, 240
QUESTIONS AND ANSWERS, 242
CLINICAL CORRELATIONS
6.1 Missense Mutations: Hemoglobin, 206
6.2 Mutation to Form a Terminator Codon, 206
6.3 a Thalassemia, 207
6.4 Programmed Frameshifting in Biosynthesis of
HIV Proteins, 208
6.5 Mutation in Mitochondrial Ribosomal RNA
Results in Antibiotic Induced Deafness, 221
6.6 Deletion of a Codon, Incorrect
Posttranslational Modification, and
Premature Protein Degradation: Cystic
Fibrosis, 223
6.7 Protein Misfolding and Aggregation:
Creutzfeldt Jacob Disease, Mad Cow
Disease, Alzheimer's Disease, and
Huntington's Disease, 224
6.8 Diseases of Lysosome Function, 230
6.9 Familial Hyperproinsulinemia, 233
6.10 Absence of Posttranslational Modification:
Multiple Sulfatase Deficiency, 234
6.11 Defects in Collagen Synthesis, 237
7 ! RECOMBINANT DNA AND BIOTECHNOLOGY,
245
Gerald Soslau
7.1 OVERVIEW, 246
7.2 THE POLYMERASE CHAIN REACTION, 247
7.3 RESTRICTION ENDONUCLEASE AND
RESTRICTION MAPS, 248
7.4 DNA SEQUENCING, 249
7.5 RECOMBINANT DNA AND CLONING, 251
7.6 SELECTION OF SPECIFIC CLONED DNA IN
LIBRARIES, 254
7.7 DETECTION AND IDENTIFICATION OF
NUCLEIC ACIDS AND DNA BINDING
PROTEINS, 258
7.8 COMPLEMENTARY DNA AND
COMPLEMENTARY DNA LIBRARIES, 263
7.9 BACTERIOPHAGE, COSMID, AND YEAST
CLONING VECTORS, 266
7.10 ANALYSIS OF LONG STRETCHES OF
DNA, 268
7.11 EXPRESSION VECTORS AND FUSION
PROTEINS, 269
7.12 EXPRESSION VECTORS IN EUKARYOTIC
CELLS, 271
7.13 SITE DIRECTED MUTAGENESIS, 272
7.14 APPLICATIONS OF RECOMBINANT DNA
TECHNOLOGIES, 277
7.15 GENOMICS, PROTEOMICS, AND
MICROARRAY ANALYSIS, 282
BIBLIOGRAPHY, 287
QUESTIONS AND ANSWERS, 287
CLINICAL CORRELATIONS
7.1 Poiymerase Chain Reaction, 248
7.2 Restriction Mapping and Evolution, 249
CONTENTS XJ
7.3 Direct Sequencing of DNA for the Diagnosis
of Genetic Disorders, 251
7.4 Multiplex PCR Analysis of the HGPTase
Gene Defects in the Lesch Nyhan
Syndrome, 254
7.5 Restriction Fragment Length Polymorphisms
Determine the Clonal Origin of
Tumors, 260
7.6 Single Strand Conformational
Polymorphism for Detection of
Spontaneous Mutations that May Lead to
SIDS, 261
7.7 Site Directed Mutagenesis of HSVIgD, 275
7.8 RNA Mediated Inhibition of HIV, 278
7.9 Gene Therapy: Normal Genes Can Be
Introduced into Cells with Defective
Genes, 279
7.10 Transgenic Animal Models, 280
7.11 Knockout Mice to Define a Role for the
P2Y, Purinoceptor, 281
7.12 Microarray Analysis of Breast Cancer, 283
8 REGULATION OF GENE EXPRESSION, 291
Daniel L. Weeks and John E. Donelson
8.1 OVERVIEW, 292
8.2 UNIT OF TRANSCRIPTION IN BACTERIA: THE
OPERON, 292
8.3 LACTOSE OPERON OF E. COLI, 293
8.4 TRYPTOPHAN OPERON OF £. COLI, 297
8.5 OTHER BACTERIAL OPERONS, 302
8.6 BACTERIAL TRANSPOSONS, 304
8.7 GENE EXPRESSION IN EUKARYOTES, 305
8.8 PREINITIATION COMPLEX IN EUKARYOTES:
TRANSCRIPTION FACTORS, RNA
POLYMERASE II, AND DNA, 307
8.9 REGULATION OF EUKARYOTIC GENE
EXPRESSION, 312
BIBLIOGRAPHY, 316
QUESTIONS AND ANSWERS, 317
CLINICAL CORRELATIONS
8.1 Transmissible Multiple Drug
Resistance, 305
8.2 Rubinstein Taybi Syndrome, 307
8.3 Tamoxifen and Targeting of Estrogen
Receptor, 314
8.4 Transcription Factors and Cardiovascular
Disease, 314
XH CONTENTS
PART fil | FUNCTIONS OF PROTEINS
9 I PROTEINS II: STRUCTURE FUNCTION
RELATIONSHIPS IN PROTEIN FAMILIES, 319
Richard M. Schultz
9.1 OVERVIEW, 320
9.2 ANTIBODY MOLECULES:
IMMUNOGLOBULIN SUPERFAMILY OF
PROTEINS, 320
9.3 PROTEINS WITH A COMMON CATALYTIC
MECHANISM: SERINE PROTEASES, 328
9.4 HEMOGLOBIN AND MYOGLOBIN, 338
9.5 THE BASAL LAMINA PROTEIN
COMPLEX, 352
BIBLIOGRAPHY, 360
QUESTIONS AND ANSWERS, 361
CLINICAL CORRELATIONS
9.1 The Complement Proteins, 323
9.2 Functions of Different Antibody
Classes, 323
9.3 Immunization, 324
9.4 Fibrin Formation in a Myocardial Infarct and
Use of Recombinant Tissue Plasminogen
Activator (rt PA), 330
9.5 Involvement of Serine Proteases in Tumor
Cell Metastasis, 330
9.6 Hemoglobinopathies, 339
10 I ENZYMES: CLASSIFICATION, KINETICS, AND
CONTROL, 365
Henry Weiner
10.1 OVERVIEW, 366
10.2 CLASSIFICATIONS OF ENZYMES, 367
10.3 GENERAL CONCEPTS OF ENZYME
MECHANISMS, 371
10.4 ACTIVE SITE OF AN ENZYME, 375
10.5 COENZYMES, CO SUBSTRATES, AND
COFACTORS, 378
10.6 KINETICS OF CHEMICAL REACTIONS, 384
10.7 ENZYME KINETICS OF ONE SUBSTRATE
REACTIONS, 386
10.8 KINETICS OF TWO SUBSTRATE
REACTIONS, 394
10.9 INHIBITORS, 395
10.10 REGULATION OF ENZYME ACTIVITY, 401
10.11 REGULATION OF METABOLIC
PATHWAYS, 404
10.12 CLINICAL APPLICATIONS OF
ENZYMES, 405
BIBLIOGRAPHY, 410
QUESTIONS AND ANSWERS, 410
CLINICAL CORRELATIONS
10.1 Mutation of a Coenzyme Binding Site
Results in Clinical Disease, 378
10.2 A Case of Gout Demonstrates Two
Phases in the Mechanism of Enzyme
Action, 389
10.3 Physiological Effect of Changes in
Enzyme Km Values, 390
10.4 Thermal Lability of Glucose 6 Phosphate
Dehydrogenase Results in Hemolytic
Anemia, 393
10.5 Alcohol Dehydrogenase Isoenzymes
with Different pH Optima, 394
10.6 Inhibitors of Xanthine Oxidase Isolated
from Plants, 396
10.7 Design of a Selective Inhibitor, 397
10.8 A Case of Poisoning, 400
10.9 Mushrooms and Alcohol
Metabolism, 400
10.10 A Case of Gout Demonstrates the
Difference Between an Allosteric and
Substrate Binding Site, 401
10.11 Identification and Treatment of an
Enzyme Deficiency, 407
10.12 Ambiguity in the Assay of Mutated
Enzymes, 407
11 | THE CYTOCHROMES P450 AND NITRIC OXIDE
SYNTH ASES, 413
Linda J. Roman and Bettie Sue Siler Masters
11.1 OVERVIEW, 414
11.2 CYTOCHROMES P450: PROPERTIES AND
FUNCTION, 414
11.3 CYTOCHROME P450 REACTION
CYCLE, 415
11.4 CYTOCHROME P450 ELECTRON
TRANSPORT SYSTEMS, 416
11.5 CYTOCHROME P450: NOMENCLATURE
AND ISOFORMS, 418
11.6 CYTOCHROME P450: SUBSTRATES AND
PHYSIOLOGICAL FUNCTIONS, 419
11.7 CYTOCHROMES P450 PARTICIPATE IN THE
SYNTHESIS OF STEROID HORMONES AND
OXYGENATION OF ENDOGENOUS
COMPOUNDS, 420
11.8 CYTOCHROME P450 INDUCTION AND
INHIBITION, 428
11.9 THE NITRIC OXIDE SYNTHASES:
PROPERTIES AND FUNCTION, 432
11.10 NITRIC OXIDE SYNTHASE ISOFORMS AND
PHYSIOLOGICAL FUNCTIONS, 433
BIBLIOGRAPHY, 439
QUESTIONS AND ANSWERS, 440
CLINICAL CORRELATIONS
11.1 Congenital Adrenal Hyperplasia:
Deficiency of CYP21A2, 423
11.2 Steroid Hormone Production During
Pregnancy, 423
11.3 Cytochrome P450 Inhibition:
Drug Drug Interactions and Adverse
Effects, 426
11.4 RoleofCYP2E1 in
Acetaminophen lnduced Liver
Toxicity, 428
11.5 Cytochrome P450 Induction:
Drug Drug Interactions and Adverse
Effects, 429
11.6 Genetic Polymorphisms of P450
Enzymes, 431
11.7 Mechanism of Action of Sildenafil, 436
11.8 Clinical Aspects of Nitric Oxide
Production, 437
11.9 History of Nitroglycerin, 438
12 I BIOLOGICAL MEMBRANES: STRUCTURE AND
MEMBRANE TRANSPORT, 443
Thomas M. Devlin
12.1 OVERVIEW, 444
12.2 CHEMICAL COMPOSITION OF
MEMBRANES, 444
12.3 MICELLES, LIPID BILAYERS, AND
LIPOSOMES, 450
12.4 STRUCTURE OF BIOLOGICAL
MEMBRANES, 453
12.5 MOVEMENT OF MOLECULES THROUGH
MEMBRANES, 461
12.6 MEMBRANE CHANNELS, 464
12.7 MEMBRANE TRANSPORTERS, 472
12.8 PASSIVE TRANSPORT, 474
12.9 ACTIVE TRANSPORT, 475
12.10 IONOPHORES, 483
BIBLIOGRAPHY, 485
QUESTIONS AND ANSWERS, 485
CLINICAL CORRELATIONS
12.1 Liposomes as Carriers of Drugs and
Enzymes, 452
CONTENTS Xili
12.2 Abnormalities of Cell Membrane Fluidity
in Disease, 459
12.3 Cystic Fibrosis and the Cl~
Channel, 466
12.4 The Mammalian Kidney and
Aquaporins, 468
12.5 Diseases Involving the Superfamily of
ABC Transporters, 481
12.6 Diseases Due to Loss of Membrane
Transport Systems, 483
13 FUNDAMENTALS OF SIGNAL TRANSDUCTION,
489
George R. Dubyak
13.1 OVERVIEW, 490
13.2 INTERCELLULAR SIGNAL
TRANSDUCTION, 491
13.3 RECEPTORS FOR SECRETED
MOLECULES, 493
13.4 INTRACELLULAR SIGNAL TRANSDUCTION
BY CELL SURFACE RECEPTORS, 494
13.5 LIGAND GATED ION CHANNEL
RECEPTORS, 500
13.6 ENZYME LINKED RECEPTORS, 503
13.7 CYTOKINE RECEPTORS, 507
13.8 G PROTEIN COUPLED RECEPTORS, 509
13.9 CYCLIC AMP BASED SIGNAL
TRANSDUCTION, 515
13.10 CYCLIC GMP BASED SIGNAL
TRANSDUCTION, 518
13.11 CALCIUM BASED SIGNAL
TRANSDUCTION, 519
13.12 PHOSPHOLIPID BASED SIGNAL
TRANSDUCTION, 522
13.13 INTEGRATION OF SIGNAL TRANSDUCTION
PATHWAYS INTO SIGNAL TRANSDUCTION
NETWORKS, 524
BIBLIOGRAPHY, 525
QUESTIONS AND ANSWERS, 526
CLINICAL CORRELATIONS
13.1 ErbB/HER Farnily Receptor Tyrosine
Kinases as Targets for Cancer
Chemotherapy, 505
13.2 G Protein Coupled Chemokine
Receptors as Targets for the Human
Immunodeficiency Virus (HIV), 510
13.3 Gsa G Protein Mutations in Pituitary
Gland Tumors and Endocrine
Diseases, 512
XiV CONTENTS
13.4 Alterations in /3 Adrenergic Receptor
Signaling Proteins in Congestive Heart
Failure, 515
13.5 Nitric Oxide/cGMP Signaling Axis as
Therapeutic Targets in Cardiac and
Vascular Disorders, 519
PART IV | METABOLIC PATHWAYS AND
THEIR CONTROL
14 | BIOENERGETICS AND OXIDATIVE METABOLISM,
529
Diana S. Beattie
14.1 ENERGY PRODUCING AND
ENERGY UTILIZING SYSTEMS, 530
14.2 THERMODYNAMIC RELATIONSHIPS AND
ENERGY RICH COMPONENTS, 532
14.3 SOURCES AND FATES OF ACETYL
COENZYMEA, 538
14.4 THE TRICARBOXYLIC ACID CYCLE, 543
14.5 STRUCTURE AND COMPARTMENTATION
BY MITOCHONDRIAL MEMBRANES, 550
14.6 ELECTRON TRANSPORT CHAIN, 552
14.7 OXIDATIVE PHOSPHORYLATION, 562
14.8 MITOCHONDRIAL INNER MEMBRANE
CONTAINS SUBSTRATE TRANSPORT
SYSTEMS, 568
14.9 MITOCHONDRIAL GENES AND
DISEASES, 573
14.10 REACTIVE OXYGEN SPECIES (ROS), 574
BIBLIOGRAPHY, 578
QUESTIONS AND ANSWERS, 579
CLINICAL CORRELATIONS
14.1 Pyruvate Dehydrogenase
Deficiency, 543
14.2 Fumarase Deficiency, 546
14.3 Cyanide Poisoning, 562
14.4 Leber's Hereditary Optic
Neuropathy, 574
14.5 Mitochondrial Myopathies from
Mutations in tRNA Genes, 575
14.6 Exercise Intolerance in Patients with
Mutations in Cytochrome b, 575
14.7 Ischemia/Reperfusion Injury, 577
15 { CARBOHYDRATE METABOLISM I: MAJOR
METABOLIC PATHWAYS AND THEIR CONTROL,
581
Robert A. Harris
15.1 OVERVIEW, 582
15.2 GLYCOLYSIS, 583
15.3 GLYCOLYSIS PATHWAY, 587
15.4 REGULATION OF GLYCOLYSIS, 595
15.5 GLUCONEOGENESIS, 608
15.6 GLYCOGENOLYSIS AND
GLYCOGENESIS, 618
BIBLIOGRAPHY, 633
QUESTIONS AND ANSWERS, 634
CLINICAL CORRELATIONS
15.1 Alcohol and Barbiturates, 594
15.2 Arsenic Poisoning, 595
15.3 Fructose Intolerance, 597
15.4 Diabetes Mellitus, 599
15.5 Lactic Acidosis, 601
15.6 Pickled Pigs and Malignant
Hyperthermia, 602
15.7 Angina Pectoris and Myocardial
Infarction, 603
15.8 Pyruvate Kinase Deficiency and
Hemolytic Anemia, 608
15.9 Hypoglycemia and Premature
Infants, 609
15.10 Hypoglycemia and Alcohol
Intoxication, 618
15.11 Glycogen Storage Diseases, 621
16 | CARBOHYDRATE METABOLISM II: SPECIAL
PATHWAYS AND GLYCOCONJUGATES, 637
Nancy B. Schwartz
16.1 OVERVIEW, 638
16.2 PENTOSE PHOSPHATE PATHWAY, 638
16.3 SUGAR INTERCONVERSIONS AND
NUCLEOTIDE LINKED SUGAR
FORMATION, 643
16.4 BIOSYNTHESIS OF COMPLEX
POLYSACCHARIDES, 648
16.5 GLYCOPROTEINS, 649
16.6 PROTEOGLYCANS, 652
BIBLIOGRAPHY, 658
QUESTIONS AND ANSWERS, 658
CLINICAL CORRELATIONS
16.1 Glucose 6 Phosphate Dehydrogenase:
Genetic Deficiency or Variants in
Erythrocytes, 640
16.2 Wernicke Korsakoff Syndrome:
Deficiency or Genetic Variants of
Transketolase, 641
16.3 Carbohydrate Deficient Glycoprotein
Syndromes (CDGS), 644
16.4 Essential Fructosuria and Fructose
Intolerance: Deficiency of Fructokinase
and Fructose 1 phosphate
Aldolase, 644
16.5 Gaiactosemia: Inability to Transform
Galactose into Glucose, 645
16.6 Pentosuria: Deficiency of Xylitol
Dehydrogenase, 646
16.7 Glucuronic Acid: Physiological
Significance of Glucuronide
Formation, 647
16.8 Blood Group Substances, 649
16.9 Common Carbohydrate Marker of
Lysosomal Targeting and I Cell
Disease, 652
16.10 Aspartylglycosylaminuria: Absence of
4 L Aspartylglycosamine
Amidohydrolase, 653
16.11 Glycolipid Disorders, 654
16.12 Heparin Is an Anticoagulant, 655
16.13 Chondrodystrophies Due to Sulfation
Defects, 655
16.14 Mucopolysaccharidoses, 657
17 ! LIPID METABOLISM I: SYNTHESIS, STORAGE,
AND UTILIZATION OF FATTY ACIDS AND
TRIACYLGLYCEROLS, 661
Martin D. Snider, J. Denis McGarry, and Richard
W. Hanson
17.1 OVERVIEW, 662
17.2 CHEMICAL NATURE OF FATTY ACIDS AND
ACYLGLYCEROLS, 663
17.3 INTERORGAN TRANSPORT OF FATTY
ACIDS AND THEIR PRIMARY
PRODUCTS, 665
17.4 SYNTHESIS OF FATTY ACIDS:
LIPOGENESIS, 668
17.5 STORAGE OF FATTY ACIDS AS
TRIACYLGLYCEROL, 676
17.6 UTILIZATION OF FATTY ACIDS FOR
ENERGY PRODUCTION, 680
17.7 REGULATION OF LIPID METABOLISM, 690
BIBLIOGRAPHY, 692
QUESTIONS AND ANSWERS, 693
CLINICAL CORRELATIONS
17.1 Obesity, 665
17.2 Role of Fatty Acid Metabolism in Type 2
Diabetes, 666
CONTENTS XV
17.3 Triacylglycerol/Fatty Acid Cycle, 679
17.4 Genetic Deficiencies in Carnitine
Transport or Carnitine
Palmitoyltransferase, 682
17.5 Genetic Deficiencies in the Acyl CoA
Dehydrogenases, 684
17.6 Refsum's Disease, 686
17.7 Ketone Bodies as a Fuel: The Atkins
Diet, 689
18 i LIPID METABOLISM II: PATHWAYS OF
METABOLISM OF SPECIAL LIPIDS, 695
Robert H. Glew
18.1 OVERVIEW, 696
18.2 PHOSPHOLIPIDS, 696
18.3 CHOLESTEROL, 707
18.4 SPHINGOLIPIDS, 720
18.5 PROSTAGLANDINSAND
THROMBOXANES, 730
18.6 UPOXYGENASEAND
OXYEICOSATETRAENOIC ACIDS, 734
BIBLIOGRAPHY, 738
QUESTIONS AND ANSWERS, 739
CLINICAL CORRELATIONS
18.1 Respiratory Distress Syndrome, 699
18.2 Treatment of
Hypercholesterolemia, 717
18.3 Atherosclerosis, 717
18.4 Diagnosis of Gaucher's Disease in an
Adult, 729
19 AMINO ACID METABOLISM, 743
Marguerite W. Coomes
19.1 OVERVIEW, 744
19.2 INCORPORATION OF NITROGEN INTO
AMINO ACIDS, 745
19.3 TRANSPORT OF NITROGEN TO LIVER AND
KIDNEY, 750
19.4 UREA CYCLE, 751
19.5 SYNTHESIS AND DEGRADATION OF
INDIVIDUAL AMINO ACIDS, 754
BIBLIOGRAPHY, 784
QUESTIONS AND ANSWERS, 785
CLINICAL CORRELATIONS
19.1 Carbamoyl Phosphate Synthetase and
iV Acetylglutarnate Synthetase
Deficiencies, 754
19.2 Deficiency of Urea Cycle Enzymes, 755
XVi CONTENTS
19.3 Diseases of Proline Metabolism, 756
19.4 Selenoproteins, 759
19.5 Nonketotic Hyperglycinemia, 760
19.6 Folic Acid Deficiency, 762
19.7 Phenylketonuria, 764
19.8 Disorders of Tyrosine Metabolism, 766
19.9 Parkinson's Disease, 766
19.10 Hyperhomocysteinemia and
Atherogenesis, 770
19.11 Diseases of Sulfur Amino Acids, 770
19.12 Glutaric Aciduria, 773
19.13 Schizophrenia and Other
Tryptophan Derived
Neurotransmitter Associated
Diseases, 775
19.14 Diseases of Metabolism of
Branched Chain Amino Acids, 777
19.15 Diseases of Propionate and
Methylmalonate Metabolism, 780
19.16 Diseases Involving Lysine and
Ornithine, 780
19.17 Histidinemia, 781
19.18 Diseases of Folate Metabolism, 782
«
20 ! PURINE AND PYRIMIDINE NUCLEOTIDE
METABOLISM, 789
Joseph G. Cory
20.1 OVERVIEW, 790
20.2 METABOLIC FUNCTIONS OF
NUCLEOTIDE, 790
20.3 METABOLISM OF PURINE
NUCLEOTIDES, 791
20.4 METABOLISM OF PYRIMIDINE
NUCLEOTIDES, 802
20.5 DEOXYRIBONUCLEOTIDE
FORMATION, 806
20.6 NUCLEOSIDE AND NUCLEOTIDE
KINASES, 808
20.7 NUCLEOTIDE METABOLIZING ENZYMES
AS A FUNCTION OF THE CELL CYCLE AND
RATE OF CELL DIVISION, 810
20.8 NUCLEOTIDE COENZYME SYNTHESIS, 811
20.9 SYNTHESIS AND UTILIZATION OF
5 PHOSPHORIBOSYL 1
PYROPHOSPHATE, 813
20.10 CHEMOTHERAPEUTIC AGENTS THAT
INTERFERE WITH PURINE AND
PYRIMIDINE NUCLEOTIDE
METABOLISM, 814
BIBLIOGRAPHY, 819
QUESTIONS AND ANSWERS, 820
CLINICAL CORRELATIONS
20.1 Gout, 796
20.2 Lesch Nyhan Syndrome, 798
20.3 Increased Cytosolic 5' Nucleotidase
Activity, 800
20.4 Immunodeficiency Diseases Associated
with Defects in Purine Nucleoside
Degradation, 801
20.5 Patients Undergoing Cancer
Treatment, 802
20.6 Subclass of Patients with Autism, 803
20.7 Hereditary Orotic Aciduria, 804
21 [ IRON AND HEME METABOLISM, 823
William M. Awad, Jr.
21.1 IRON METABOLISM: OVERVIEW, 824
21.2 IRON CONTAINING PROTEINS, 824
21.3 INTESTINAL ABSORPTION OF IRON, 826
21.4 MOLECULAR REGULATION OF IRON
UTILIZATION, 828
21.5 IRON DISTRIBUTION AND KINETICS, 830
21.6 HEME BIOSYNTHESIS, 833
21.7 HEME CATABOLISM, 840
BIBLIOGRAPHY, 845
QUESTIONS AND ANSWERS, 845
CLINICAL CORRELATIONS
21.1 Iron Overload and Infection, 825
21.2 Microbial Pathogenicity and Iron, 825
21.3 Iron Sulfur Cluster Synthesis and
Human Disease, 827
21.4 Friedreich's Ataxia, 827
21.5 Duodenal Iron Absorption, 828
21.6 Mutant Iron Responsive Elements, 830
21.7 Ceruloplasmin Deficiency, 831
21.8 Iron Deficiency Anemia, 832
21.9 Hemochromatosis Type I: Molecular
Genetics and the Issue of Iron Fortified
Diets, 832
21.10 Hemochromatosis Type III, 833
21.11 Acute Intermittent Porphyria, 836
21.12 Cytoprotective Role of Heme
Oxygenase, 841
21.13 Neonatal Isoimmune Hemolysis, 843
21.14 Bilirubin UDP Glucuronosyltransferase
Deficiency, 843
21.15 Elevation of Serum Conjugated
Bilirubin, 844
22 | METABOLIC INTERRELATIONSHIPS, 849
Robert A. Harris and David W. Crabb
22.1 OVERVIEW, 850
22.2 STARVE FEED CYCLE, 850
22.3 MECHANISMS INVOLVED IN SWITCHING
LIVER METABOLISM BETWEEN THE
WELL FED AND STARVED STATES, 863
22.4 INTERRELATIONSHIP OF TISSUES IN
NUTRITIONAL AND HORMONAL
STATES, 873
BIBLIOGRAPHY, 887
QUESTIONS AND ANSWERS, 889
CLINICAL CORRELATIONS
22.1 Obesity, 851
22.2 Protein Malnutrition, 852
22.3 Starvation, 852
22.4 Reye's Syndrome, 857
22.5 Hyperglycemic, Hyperosmolar
Coma, 861
22.6 Hyperglycemia and Protein
Glycation, 861
22.7 Diabetes Mellitus, Type 2, 876
22.8 Diabetes Mellitus, Type 1, 877
22.9 Polyol Pathway and Complications of
Diabetes, 878
22.10 Cancer Cachexia, 879
PART V | PHYSIOLOGICAL PROCESSES
23 { BIOCHEMISTRY OF HORMONES, 891
Thomas J. Schmidt and Gerald Litwack
23.1 OVERVIEW, 892
23.2 HORMONES AND THE HORMONAL
CASCADE SYSTEM, 893
23.3 SYNTHESIS OF POLYPEPTIDE AND AMINO
ACID DERIVED HORMONES, 900
23.4 PROTEIN HORMONE SIGNALING, 906
23.5 MEMBRANE HORMONE RECEPTORS, 914
CONTENTS XVM
23.6 INTRACELLULAR HORMONE CASCADE:
PROTEIN KINASES, 917
23.7 STEROID HORMONES, 925
23.8 STEROID HORMONE RECEPTORS. 938
BIBLIOGRAPHY, 945
QUESTIONS AND ANSWERS, 946
CLINICAL CORRELATIONS
23.1 Testing Activity of the Anterior
Pituitary, 896
23.2 Hypopituitarism, 901
23.3 Decreased Insulin Receptor Kinase
Activity in Gestational Diabetes
Mellitus, 920
23.4 Oral Contraception, 937
23.5 Apparent Mineralocorticoid Excess
Syndrome, 940
23.6 Mineralocorticoid Receptor Mutation
Results in Hypertension and Toxemia of
Pregnancy, 943
24 j MOLECULAR CELL BIOLOGY, 949
Thomas E. Smith
24.1 OVERVIEW, 950
24.2 NERVOUS TISSUE: METABOLISM AND
FUNCTION, 950
24.3 THE EYE: METABOLISM AND VISION, 961
24.4 MOLECULAR MOTORS AND ASSOCIATED
PROTEINS, 976
24.5 MECHANISM OF BLOOD
COAGULATION, 991
BIBLIOGRAPHY, 1008
QUESTIONS AND ANSWERS, 1010
CLINICAL CORRELATIONS
24.1 Lambert Eaton Myasthenic
Syndrome, 957
24.2 Myasthenia Gravis: A Neuromuscular
Disorder, 958
24.3 Macula Degeneration and Loss of
Vision, 965
24.4 Niemann Pick Disease and Retinitis
Pigmentosa, 966
24.5 Retinitis Pigmentosa from a Mutation in
the Gene for Peripherin, 967
24.6 Leber Congenital Amaurosis: Retinal
Dystrophy Leading to Blindness, 973
24.7 Glycation and Myosin Structure and
Function, 980
24.8 Familial Hypertropic Cardiomyopathies
and Mutations in Muscle Proteins, 981
24.9 Dilated Cardiomyopathy and Mutations
inActin, 982
XViii CONTENTS
24.10 Troponin Subunits as Markers for
Myocardial Infarction, 985
24.11 Voltage Gated Ion
Channelopathies, 986
24.12 Ion Channels and Cardiac Muscle
Disease, 986
24.13 Mutations Affecting Pigmentation: Is
There a Molecular Motor
Connection? 989
24.14 Intrinsic Pathway Defects: Prekallikrein
Deficiency, 994
24.15 Classic Hemophilia, 999
24.16 Use of Recombinant Factor Vila to
Control Bleeding, 999
24.17 Thrombosis: Defects of the Protein C
Pathway and Increased Levels of
Coagulation Factors, 1004
25 ! CELL CYCLE, PROGRAMMED CELL DEATH, AND
CANCER, 1013
Richard M. Schultz
25.1 OVERVIEW, 1014
25.2 CELL CYCLE, 1014
25.3 APOPTOSIS: PROGRAMMED CELL
DEATH, 1020
25.4 CANCER, 1024
BIBLIOGRAPHY, 1032
QUESTIONS AND ANSWERS, 1033
CLINICAL CORRELATIONS
25.1 Oncogenic DNA Viruses, 1025
25.2 Molecularly Targeted Anticancer
Drug, 1029
25.3 Environmental Cause of Human
Cancers, 1030
26 DIGESTION AND ABSORPTION OF BASIC
NUTRITIONAL CONSTITUENTS, 1037
Ulrich Hopfer
26.1 OVERVIEW, 1038
26.2 GENERAL CONSIDERATIONS, 1039
26.3 EPITHELIAL TRANSPORT, 1044
26.4 DIGESTION AND ABSORPTION OF
PROTEINS, 1052
26.5 DIGESTION AND ABSORPTION OF
CARBOHYDRATES, 1056
26.6 DIGESTION AND ABSORPTION OF
LIPIDS, 1059
26.7 BILE ACID METABOLISM, 1065
BIBLIOGRAPHY, 1067
QUESTIONS AND ANSWERS, 1068
CLINICAL CORRELATIONS
26.1 Familial Chloridorrhea Causes Metabolic
Alkalosis, 1046
26.2 Cystic Fibrosis, 1049
26.3 Bacterial Toxigenic Diarrheas and
Electrolyte Replacement Therapy, 1049
26.4 Neutral Amino Aciduria: Hartnup
Disease, 1054
26.5 Disaccharidase Deficiency, 1058
26.6 Pharmacological Interventions to
Prevent Fat Absorption and
Obesity, 1061
26.7 Cholesterol Stones, 1064
26.8 A /3 Lipoproteinemia, 1065
27 ! PRINCIPLES OF NUTRITION I: MACRONUTRIENTS,
1071
Stephen G. Chaney
27.1 OVERVIEW, 1072
27.2 ENERGY METABOLISM, 1072
27.3 PROTEIN METABOLISM, 1073
27.4 PROTEIN ENERGY MALNUTRITION, 1076
27.5 EXCESS PROTEIN ENERGY INTAKE, 1077
27.6 CARBOHYDRATES, 1079
27.7 FATS, 1079
27.8 FIBER, 1080
27.9 COMPOSITION OF MACRONUTRIENTS IN
THE DIET, 1082
BIBLIOGRAPHY, 1086
QUESTIONS AND ANSWERS, 1088
CLINICAL CORRELATIONS
27.1 Vegetarian Diets and Protein Energy
Requirements for Children, 1075
27.2 Dietary Protein Intake and Renal
Disease, 1076
27.3 Providing Adequate Protein and
Calories for Hospitalized Patients, 1077
27.4 Carbohydrate Loading and Athletic
Endurance, 1080
27.5 High Carbohydrate Versus High Fat Diet
for Diabetics, 1081
27.6 Polyunsaturated Fatty Acids and Risk
Factors for Heart Disease, 1083
27.7 Metabolic Adaptation: Relationship
Between Carbohydrate Intake and
Serum Triacylglycerols, 1086
28 | PRINCIPLES OF NUTRITION II: MICRONUTRIENTS,
1091
Stephen G. Chaney
28.1 OVERVIEW, 1092
28.2 ASSESSMENT OF MALNUTRITION, 1092
28.3 DIETARY REFERENCE INTAKES, 1093
28.4 FAT SOLUBLE VITAMINS, 1093
28.5 WATER SOLUBLE VITAMINS, 1102
28.6 ENERGY RELEASING WATER SOLUBLE
VITAMINS, 1102
28.7 HEMATOPOIETIC WATER SOLUBLE
VITAMINS, 1107
28.8 OTHER WATER SOLUBLE VITAMINS, 1110
28.9 MACROMINERALS, 1112
28.10 TRACE MINERALS, 1113
28.11 THE AMERICAN DIET: FACT AND
FALLACY, 1115
28.12 ASSESSMENT OF NUTRITIONAL STATUS IN
CLINICAL PRACTICE, 1116
BIBLIOGRAPHY, 1117
QUESTIONS AND ANSWERS, 1119
CLINICAL CORRELATIONS
28.1 Nutritional Considerations in Cystic
Fibrosis, 1096
CONTENTS XIX
28.2 Renal Osteodystrophy, 1097
28.3 Nutritional Considerations in Newborn
Infants, 1101
28.4 Anticonvulsant Drugs and Vitamin
Requirements, 1101
28.5 Nutritional Considerations in
Alcoholics, 1103
28.6 Vitamin B6 Requirements and Oral
Contraceptives, 1106
28.7 Gene Polymorphisms and Folic Acid
Requirement, 1108
28.8 Diet and Osteoporosis, 1113
28.9 Nutritional Needs of Elderly
Persons, 1117
APPENDIX REVIEW OF ORGANIC
CHEMISTRY, 1121
Carol N. Angstadt
GLOSSARY, 1133
Francis Vella
INDEX, 1157 |
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| ctrlnum | (OCoLC)58729134 (DE-599)BVBBV021301092 |
| dewey-full | 612/.015 |
| dewey-hundreds | 600 - Technology (Applied sciences) |
| dewey-ones | 612 - Human physiology |
| dewey-raw | 612/.015 |
| dewey-search | 612/.015 |
| dewey-sort | 3612 215 |
| dewey-tens | 610 - Medicine and health |
| discipline | Biologie Chemie Medizin |
| discipline_str_mv | Biologie Chemie Medizin |
| edition | 6. ed. |
| format | Book |
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| id | DE-604.BV021301092 |
| illustrated | Illustrated |
| index_date | 2024-07-02T13:52:36Z |
| indexdate | 2024-07-09T20:35:07Z |
| institution | BVB |
| isbn | 0471678082 9780471678083 |
| language | English |
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| physical | XXVII, 1208 S. Ill, graph. Darst. |
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| spelling | Textbook of biochemistry with clinical correlations ed. by Thomas M. Devlin Biochemistry 6. ed. Hoboken, NJ Wiley-Liss 2006 XXVII, 1208 S. Ill, graph. Darst. txt rdacontent n rdamedia nc rdacarrier Biochimie Biochimie clinique Clinical biochemistry Biochemie (DE-588)4006777-4 gnd rswk-swf Physiologische Chemie (DE-588)4076124-1 gnd rswk-swf Biochemie (DE-588)4006777-4 s DE-604 Physiologische Chemie (DE-588)4076124-1 s 1\p DE-604 Devlin, Thomas M. Sonstige oth HBZ Datenaustausch application/pdf http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=014621790&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA Inhaltsverzeichnis 1\p cgwrk 20201028 DE-101 https://d-nb.info/provenance/plan#cgwrk |
| spellingShingle | Textbook of biochemistry with clinical correlations Biochimie Biochimie clinique Biochemistry Clinical biochemistry Biochemie (DE-588)4006777-4 gnd Physiologische Chemie (DE-588)4076124-1 gnd |
| subject_GND | (DE-588)4006777-4 (DE-588)4076124-1 |
| title | Textbook of biochemistry with clinical correlations |
| title_alt | Biochemistry |
| title_auth | Textbook of biochemistry with clinical correlations |
| title_exact_search | Textbook of biochemistry with clinical correlations |
| title_exact_search_txtP | Textbook of biochemistry with clinical correlations |
| title_full | Textbook of biochemistry with clinical correlations ed. by Thomas M. Devlin |
| title_fullStr | Textbook of biochemistry with clinical correlations ed. by Thomas M. Devlin |
| title_full_unstemmed | Textbook of biochemistry with clinical correlations ed. by Thomas M. Devlin |
| title_short | Textbook of biochemistry |
| title_sort | textbook of biochemistry with clinical correlations |
| title_sub | with clinical correlations |
| topic | Biochimie Biochimie clinique Biochemistry Clinical biochemistry Biochemie (DE-588)4006777-4 gnd Physiologische Chemie (DE-588)4076124-1 gnd |
| topic_facet | Biochimie Biochimie clinique Biochemistry Clinical biochemistry Biochemie Physiologische Chemie |
| url | http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=014621790&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA |
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