Textbook of biochemistry: with clinical correlations
This book presents the biochemistry of mammalian cells, relates events at the cellular level to the subsequent physiological processes in the whole animal, and cites examples of human diseases derived from aberrant biochemical processes.
Saved in:
| Other Authors: | |
|---|---|
| Format: | Book |
| Language: | English |
| Published: |
New York
Wiley-Liss
2002
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| Edition: | 5. ed. |
| Subjects: | |
| Online Access: | Inhaltsverzeichnis |
| Summary: | This book presents the biochemistry of mammalian cells, relates events at the cellular level to the subsequent physiological processes in the whole animal, and cites examples of human diseases derived from aberrant biochemical processes. |
| Physical Description: | XXIV, 1216 S. Ill., graph. Darst. |
| ISBN: | 0471411361 |
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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 5. ed. | ||
| 264 | 1 | |a New York |b Wiley-Liss |c 2002 | |
| 300 | |a XXIV, 1216 S. |b Ill., graph. Darst. | ||
| 336 | |b txt |2 rdacontent | ||
| 337 | |b n |2 rdamedia | ||
| 338 | |b nc |2 rdacarrier | ||
| 520 | 3 | |a This book presents the biochemistry of mammalian cells, relates events at the cellular level to the subsequent physiological processes in the whole animal, and cites examples of human diseases derived from aberrant biochemical processes. | |
| 650 | 7 | |a Biochemistry |2 cabt | |
| 650 | 4 | |a Biochimie | |
| 650 | 4 | |a Biochimie clinique | |
| 650 | 7 | |a Biochimie clinique |2 ram | |
| 650 | 2 | |a Biochimie | |
| 650 | 7 | |a Biochimie |2 ram | |
| 650 | 4 | |a Physiopathologie | |
| 650 | 4 | |a Biochemistry | |
| 650 | 4 | |a Clinical biochemistry | |
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Record in the Search Index
| _version_ | 1804129016261640192 |
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| adam_text | ^^H CONTEN
PART I I STRUCTURE OF
MACROMOLECULES 1 1 EUKARYOTIC CELL STRUCTURE 3
2 DNA AND RNA: COMPOSITION AND STRUCTURE 27
3 PROTEINS I: COMPOSITION AND STRUCTURE 93
PART II I TRANSMISSION OF
INFORMATION 159 4 DNA REPLICATION, RECOMBINATION,
AND REPAIR 161
5 RNA: TRANSCRIPTION AND RNA PROCESSING 207
6 PROTEIN SYNTHESIS: TRANSLATION AND
POSTTRANSLATIONAL MODIFICATIONS 233
7 RECOMBINANT DNA AND BIOTECHNOLOGY 279
8 REGULATION OF GENE EXPRESSION 329
PART III | FUNCTIONS OF PROTEINS 365
9 PROTEINS II: STRUCTURE-FUNCTION
RELATIONSHIPS IN PROTEIN FAMILIES 367
10 ENZYMES: CLASSIFICATION, KINETICS AND
CONTROL 413
11 THE CYTOCHROMES P450 AND NITRIC OXIDE
SYNTHASES 465
12 BIOLOGICAL MEMBRANES: STRUCTURE AND
MEMBRANE TRANSPORT 493
TS IN BRIEF
PART IV I METABOLIC PATHWAYS
AND THEIR CONTROL 535
13 BIOENERCETICS AND OXIDATIVE METABOLISM 537
14 CARBOHYDRATE METABOLISM I: MAJOR METABOLIC
PATHWAYS AND THEIR CONTROL 597
15 CARBOHYDRATE METABOLISM II: SPECIAL PATHWAYS
AND CLYCOCONJUCATES 665
16 LIPID METABOLISM I: UTILIZATION AND
STORAGE OF ENERGY IN LIPID FORM 693
17 LIPID METABOLISM II: PATHWAYS OF
METABOLISM OF SPECIAL LIPIDS 727
18 AMINO ACID METABOLISM 779
19 PURINE AND PYRIMIDINE NUCLEOTIDE
METABOLISM 825
20 METABOLIC INTERRELATIONSHIPS 861
PARTY | PHYSIOLOGICAL PROCESSES 903
21 BIOCHEMISTRY OF HORMONES I: POLYPEPTIDE
HORMONES 905
22 BIOCHEMISTRY OF HORMONES II: STEROID
HORMONES 959
23 MOLECULAR CELL BIOLOGY 989
24 IRON AND HEME METABOLISM 1053
25 DIGESTION AND ABSORPTION OF NUTRITIONAL
CONSTITUENTS 1081
26 PRINCIPLES OF NUTRITION I:
MACRONUTRIENTS 1117
27 PRINCIPLES OF NUTRITION II:
MICRONUTRIENTS 1137
APPENDIX
REVIEW OF ORGANIC CHEMISTRY 1169
I^H CONTEN
PART I I STRUCTURE OF
| MACROMOLECULES 1 1 I EUKARYOTIC CELL STRUCTURE 3 Thomas M. Devlin
1.1 OVERVIEW: CELLS AND CELLULAR COMPARTMENTS 4
1.2 CELLULAR ENVIRONMENT: WATER AND SOLUTES 6
1.3 ORGANIZATION AND COMPOSITION
OF EUKARYOTIC CELLS 14
1.4 FUNCTIONAL ROLE OF SUBCELLULAR ORCANELLES AND
MEMBRANES 16
1.5 INTEGRATION AND CONTROL OF CELLULAR
FUNCTIONS 23
BIBLIOGRAPHY 24
QUESTIONS AND ANSWERS 24
CLINICAL CORRELATIONS
1.1 Blood Bicarbonate Concentration in Metabolic Acidosis 13
1.2 Mitochondrial Diseases 19
1.3 Lysosomal Enzymes and Gout 20
1.4 Lysosomal Acid Lipase Deficiency 21
1.5 Peroxisome Biogenesis Disorders (PBD) 22
1.6 Apoptosis: Programmed Cell Death 23
2 | DNA AND RNA:
COMPOSITION AND STRUCTURE 27 Stephen A. Woski and Francis J. Schmidt
2.1 DNA OVERVIEW 28
2.2 STRUCTURAL COMPONENTS OF NUCLEIC ACIDS: 29
2.3 STRUCTURE OF DNA 33
2.4 HIGHER ORDER DNA STRUCTURE 58
2.5 DNA SEQUENCE AND FUNCTION 71
2.6 RNA OVERVIEW 77
2.7 STRUCTURE OF RNA 78
2.8 TYPES OF RNA 82
TS
BIBLIOGRAPHY 89
QUESTIONS AND ANSWERS 89
CLINICAL CORRELATIONS
2.1 DNA Vaccines 29
2.2 Diagnostic Use of DNA Arrays in Medicine and Genetics 45
2.3 Antitumor Antibiotics that Change the Shape of DNA 49
2.4 Hereditary Persistence of Fetal Hemoglobin 54
2.5 Telomerase As a Target for Anticancer Agents 56
2.6 Expansion of DNA Triple Repeats and Human Disease 58
2.7 Topoisomerases in Treatment of Disease 65
2.8 Staphylococcal Resistance to Erythromycin 84
3 I PROTEINS I: COMPOSITION AND STRUCTURE 93
Richard M. Schultz and Michael N. Liebman
3.1 FUNCTIONAL ROLES OF PROTEINS IN HUMANS 94
3.2 AMINO ACID COMPOSITION OF PROTEINS 95
3.3 CHARGE AND CHEMICAL PROPERTIES OF AMINO ACIDS
AND PROTEINS 101
3.4 PRIMARY STRUCTURE OF PROTEINS 109
3.5 HIGHER LEVELS OF PROTEIN ORGANIZATION 112
3.6 OTHER TYPES OF PROTEINS 121
3.7 FOLDING OF PROTEINS FROM RANDOMIZED TO UNIQUE
STRUCTURES: PROTEIN STABILITY 132
3.8 DYNAMIC ASPECTS OF PROTEIN STRUCTURE 139
3.9 METHODS FOR CHARACTERIZATION,
PURIFICATION, AND STUDY OF PROTEIN
STRUCTURE AND ORGANIZATION 140
BIBLIOGRAPHY 154
QUESTIONS AND ANSWERS 155
CLINICAL CORRELATIONS
3.1 Plasma Proteins in Diagnosis of Disease 107
3.2 Differences in Primary Structure of Insulins Used in
Treatment of Diabetes Mellitus 111
3.3 A Nonconservative Mutation Occurs in Sickle Cell
Anemia 112
3.4 Symptoms of Diseases of Abnormal Collagen Synthesis 121
3.5 Hyperlipidemias 127
3.6 Hypolipoproteinemias 128
xviii | contents
3.7 Glycosylated Hemoglobin, HbAlc 132
3.8 Proteins as Infectious Agents: Human Transmissible
Spongiform Encephalopathies (TSEs) 134
3.9 Use of Amino Acid Analysis in Diagnosis of Disease 146
PART II I TRANSMISSION OF
I INFORMATION 159 4 | DNA REPLICATION, RECOMBINATION,
AND REPAIR 161
Howard J. Edenberg
4.1 COMMON FEATURES OF REPLICATION,
RECOMBINATION AND REPAIR 162
4.2 DNA REPLICATION 162
4.3 RECOMBINATION 186
4.4 REPAIR 191
BIBLIOGRAPHY 204
QUESTIONS AND ANSWERS 204
CLINICAL CORRELATIONS
4.1 Topoisomerases as Drug Targets 175
4.2 Cancer and the Cell Cycle 180
4.3 Nucleoside Analogs and Drug Resistance in HIV Therapy 184
4.4 The Human Genome Project 185
4.5 Genomic Medicine 186
4.6 Gene Therapy 191
4.7 Xeroderma Pigmentosum 196
4.8 Mismatch Repair and Cancer 198
5 I RNA: TRANSCRIPTION AND RNA PROCESSING 207
Francis J. Schmidt
5.1 OVERVIEW 208
5.2 MECHANISMS OF TRANSCRIPTION 208
5.3 TRANSCRIPTION IN EUKARYOTES 214
5.4 RNA PROCESSING 220
5.5 RNA EXPORT FROM THE NUCLEUS 228
5.6 TRANSCRIPTION-COUPLED DNA REPAIR 228
5.7 NUCLEASES AND RNA TURNOVER 229
BIBLIOGRAPHY 230
QUESTIONS AND ANSWERS 230
CLINICAL CORRELATIONS
5.1 Antibiotics and Toxins that Target RNA Polymerase 211
5.2 Fragile X Syndrome: A Chromatin Disease? 215
5.3 Involvement of Transcriptional Factors in Carcinogenesis 217
5.4 Thalassemia Due to Defects in Messenger RNA Synthesis 224
5.5 Autoimmunity in Connective Tissue Disease 225
5.6 Cockayne Syndrome: Transcription-Coupled DNA Repair 229
6 | PROTEIN SYNTHESIS; TRANSLATION AND
POSTTRANSLATIONAL MODIFICATIONS 233
Dohn Glitz
6.1 OVERVIEW 234
6.2 COMPONENTS OF THE TRANSLATIONS APPARATUS 234
6.3 PROTEIN BIOSYNTHESIS 245
6.4 PROTEIN MATURATION: FOLDING, MODIFICATION,
SECRETION, AND TARGETING 255
6.5 MEMBRANE AND ORGANELLE TARGETING 262
6.6 FURTHER POSTTRANSLATIONAL PROTEIN
MODIFICATIONS 265
6.7 REGULATION OF TRANSLATION 271
6.8 PROTEIN DEGRADATION AND TURNOVER 272
BIBLIOGRAPHY 274
QUESTIONS AND ANSWERS 275
CLINICAL CORRELATIONS
6.1 Missense Mutations: Hemoglobin 238
6.2 Disorders of Terminator Codons 238
6.3 a-Thalassemia 239
6.4 Programmed Frameshifting in the Biosynthesis
of HIV Proteins 241
6.5 Mutation in Mitochondrial Ribosomal RNA Results
in Antibiotic-induced Deafness 254
6.6 Deletion of a Codon, Incorrect Post-translational
Modification and Premature Protein Degradation:
Cystic Fibrosis 256
6.7 Protein Misfolding and Aggregation: Creutzfeld-Jacob
Disease, Mad Cow Disease, Alzheimer s Disease, and
Huntington Disease 257
6.8 I-Cell Disease 263
6.9 Familial Hyperproinsulinemia 266
6.10 Absence of Post-Translational Modification:
Multiple Sulfatase Deficiency 267
6.11 Defects in Collagen Synthesis 270
7 | RECOMBINANT DNA AND BIOTECHNOLOGY 279 _
Gerald Soslau
7.1 OVERVIEW 280
7.2 THE POLYMERASE CHAIN REACTION 281
7.3 RESTRICTION ENDONUCLEASE AND
RESTRICTION MAPS 282
7.4 DNA SEQUENCING 284
7.5 RECOMBINANT DNA AND CLONING 287
7.6 SELECTION OF SPECIFIC CLONED DNA IN LIBRARIES 293
7.7 TECHNIQUES FOR DETECTION AND IDENTIFICATION OF
NUCLEIC ACIDS AND DNA BINDING PROTEINS 296
7.8 COMPLEMENTARY DNA AND COMPLEMENTARY DNA
LIBRARIES 302
7.9 BACTERIOPHACE, COSMID, AND YEAST CLONING
VECTORS 305
7.10 TECHNIQUES TO ANALYZE LONG STRETCHES
OF DNA 307
7.11 EXPRESSION VECTORS AND FUSION PROTEINS 309
7.12 EXPRESSION VECTORS IN EUKARYOTIC CELLS 310
7.13 SITE-DIRECTED MUTAGENESIS 312
7.14 APPLICATIONS OF RECOMBINANT DNA
TECHNOLOGIES 318
7.15 MOLECULAR TECHNIQUES APPLIED TO THE WHOLE
ANIMAL 321
7.16 CONCLUDING REMARKS 323
BIBLIOGRAPHY 324
QUESTIONS AND ANSWERS 325
CLINICAL CORRELATIONS
7.1 Polymerase Chain Reaction 282
7.2 Restriction Mapping and Evolution 284
7.3 Direct Sequencing of DNA For Diagnosis of Genetic
Disorders 287
7.4 Multiplex PCR Analysis of HGPRTase Gene Defects
in Lesch-Nyhan Syndrome 293
7.5 Restriction Fragment Length Polymorphisms Determine
the Clonal Origin of Tumors 299
7.6 Single-Strand Conformational Polymorphism For Detection
of Spontaneous Mutations That May Lead to SIDS 300
7.7 Site-Directed Mutagenesis of HSV IgD 315
7.8 Normal Genes Can Be Introduced Into Cells With Defective
Genes in Gene Therapy 320
7.9 Transgenic Animal Models 322
7.10 Knockout Mice to Define a Role for the P2Y,
Purinoceptor 323
8 I REGULATION OF GENE EXPRESSION 329 Daniel L. Weeks and John E. Donelson
8.1 OVERVIEW 330
8.2 UNIT OF TRANSCRIPTION IN BACTERIA:
THE OPERON 330
8.3 LACTOSE OPERON OF E. COLI 332
8.4 TRYPTOPHAN OPERON OF E. COLI 338
8.5 OTHER BACTERIAL OPERONS 343
8.6 BACTERIAL TRANSPOSONS 345
8.7 GENE EXPRESSION IN EUKARYOTES 347
8.8 PREINITIATION COMPLEX IN EUKARYOTES:
GENERAL TRANSCRIPTION FACTORS, RNA
POLYMERASE II AND DNA 350
8.9 REGULATION OF EUKARYOTIC GENE EXPRESSION 357
BIBLIOGRAPHY 361
QUESTIONS AND ANSWERS 361
CONTENTS | Xix
CLINICAL CORRELATIONS
8.1 Transmissible Multiple Drug Resistance 346
8.2 Rubinstein-Taybi Syndrome 349
8.3 Tamoxifen and Targeting of Estrogen Receptor 358
8.4 Transcription Factors and Cardiovascular Diseases 359
PART IN | FUNCTIONS OF PROTEINS 365
9 | PROTEINS II: STRUCTURE-FUNCTION RELATIONSHIPS
IN PROTEIN FAMILIES 367
Richard M. Schultz and Michael N. Liebman
9.1 OVERVIEW 368
9.2 ANTIBODY MOLECULES: THE IMMUNOGLOBULIN
SUPERFAMILY OF PROTEINS 368
9.3 PROTEINS WITH A COMMON CATALYTIC MECHANISM:
SERINE PROTEASES 379
9.4 DNA-BINDING PROTEINS 387
9.5 HEMOGLOBIN AND MYOGLOBIN 393
BIBLIOGRAPHY 409
QUESTIONS AND ANSWERS 410
CLINICAL CORRELATIONS
9.1 The Complement Proteins )71
9.2 Functions of Different Antibody (lasses 372
9.3 Immunization 372
9.4 Fibrin Formation in a Myocardial Infarct and the Action of
Recombinant Tissue Plasminogen Activator (rt-PA) 378
9.5 Involvement of Serine Proteases in Tumor Cell Metastasis 379
9.6 Hemoglobinopathies 394
10 I ENZYMES: CLASSIFICATION, KINETICS,
AND CONTROL 413
J. Lyndal York
10.1 GENERAL CONCEPTS 414
10.2 CLASSIFICATION OF ENZYMES 415
10.3 KINETICS 418
10.4 COENZYMES: STRUCTURE AND FUNCTION 426
10.5 INHIBITION OF ENZYMES 431
10.6 ALLOSTERIC CONTROL OF ENZYME ACTIVITY 436
10.7 ENZYME SPECIFICITY: THE ACTIVE SITE 440
10.8 MECHANISM OF CATALYSIS 443
10.9 CLINICAL APPLICATIONS OF ENZYMES 452
10.10 REGULATION OF ENZYME ACTIVITY 459
BIBLIOGRAPHY 460
QUESTIONS AND ANSWERS 460
XX I CONTENTS
CLINICAL CORRELATIONS
10.1 A Case of Gout Demonstrates Two Phases in the
Mechanism of Enzyme Action 423
10.2 Physiological Effect of Changes in Enzyme Km Values 423
10.3 Mutation of a Coenzyme-Binding Site Results in Clinical
Disease 426
10.4 The Design of a Selective Inhibitor 432
10.5 A Case of Poisoning 435
10.6 A Case of Gout Demonstrates the Difference Between an
Allosteric and Substrate-Binding Site 437
10.7 Thermal Lability of Glucose-6-Phosphate Dehydrogenase
Results in Hemolytic Anemia 451
10.8 Alcohol Dehydrogenase Isoenzymes with Different pH
Optima 452
10.9 Identification and Treatment of an Enzyme Deficiency 454
10.10 Ambiguity in the Assay of Mutated Enzymes 454
11 I THE CYTOCHROMES P450 AND NITRIC
OXIDE SYNTHASES 465
Richard T. Okita and Bettie Sue Siler Masters
11.1 OVERVIEW 466
11.2 CYTOCHROME P450: GENERAL CLASSIFICATION
AND OVERALL REACTION DESCRIPTION 466
11.3 CYTOCHROME P450 ELECTRON TRANSPORT SYSTEMS 468
11.4 CYTOCHROME P450: NOMENCLATURE AND
TERMINOLOGY 470
11.5 SUBSTRATE SPECIFICITY OF CYTOCHROMES P450:
PHYSIOLOGICAL FUNCTIONS 471
11.6 INHIBITORS OF CYTOCHROME P450 480
11.7 REGULATION OF CYTOCHROME P450 EXPRESSION 482
11.8 OTHER HEMOPROTEIN AND FLAVOPROTEIN-MEDIATED
OXYGENATIONS: SOLUBLE CYTOCHROME P450S AND
THE NITRIC OXIDE SYNTHASES 486
BIBLIOGRAPHY 489
QUESTIONS AND ANSWERS 489
CLINICAL CORRELATIONS
11.1 Congenital Adrenal Hyperplasia:
Deficiency of CYP21A2 474
11.2 Steroid Hormone Production During Pregnancy 475
11.3 Role of Cytochrome P450 2E1 in Acetaminophen-lnduced
Liver Toxicity 480
11.4 Consequences of P450 Inhibition: Drug-Drug Interactions
and Adverse Effects 481
11.5 Consequences of Induction of P450 Enzymes 484
1 1 .(i Genetic Polymorphisms of P450 Enzymes 485
11.7 Clinical Aspects of Nitric Oxide Production 487
12 j BIOLOGICAL MEMBRANES: STRUCTURE
AND MEMBRANE TRANSPORT 493 Thomas M. Devlin
12.1 OVERVIEW 494
12.2 CHEMICAL COMPOSITION OF MEMBRANES 494
12.3 MICELLES AND LIPOSOMES 502
12.4 STRUCTURE OF BIOLOGICAL MEMBRANES 505
12.5 MOVEMENT OF MOLECULES THROUGH MEMBRANES 511
12.6 CHANNELS AND PORES 516
12.7 PASSIVE MEDIATED TRANSPORT SYSTEMS 519
12.8 ACTIVE MEDIATED TRANSPORT SYSTEMS 522
12.9 lONOPHORES 529
BIBLIOGRAPHY 531
QUESTIONS AND ANSWERS 531
CLINICAL CORRELATIONS
12.1 Liposomes as Carriers of Drugs and Enzymes 504
12.2 Abnormalities of Cell Membrane Fluidity in Disease
States 510
12.3 Cystic Fibrosis and the Cl-Channel 517
12.4 Diseases Due to Loss of Membrane Transport Systems 529
PART IV METABOLIC PATHWAYS
AND THEIR CONTROL 535
13 | BIOENERGETICS AND OXIDATIVE METABOLISM 537
Diana S. Beattie
13.1 ENERGY-PRODUCING AND ENERGY-UTILIZING
SYSTEMS 538
13.2 THERMODYNAMIC RELATIONSHIPS AND ENERGY-RICH
COMPONENTS 540
13.3 SOURCES AND FATES OF ACETYL COENZYME A 547
13.4 THE TRICARBOXYLIC ACID CYCLE 552
13.5 STRUCTURE AND COMPARTMENTATION BY
MITOCHONDRIAL MEMBRANES 561
13.6 ELECTRON TRANSPORT CHAIN 564
13.7 OXIDATIVE PHOSPHORYLATION 577
13.8 MITOCHONDRIAL INNER MEMBRANES CONTAIN
SUBSTRATE TRANSPORT SYSTEMS 583
13.9 MITOCHONDRIAL GENES AND MITOCHONDRIAL
DISEASES 587
13.10 REACTIVE OXYGEN SPECIES (ROS) 590
BIBLIOGRAPHY 592
QUESTIONS AND ANSWERS 593
CLINICAL CORRELATIONS
13.1 Pyruvate Dehydrogenase Deficiency 552
13.2 Fumarase Deficiency 557
13.3 Cyanide Poisoning 577
13.4 Leber s Hereditary Optic Neuropathy 588
13.5 Mitochondrial Myopathies Due to Mutations in
Mitochondrial tRNA Genes 589
13.6 Exercise Intolerance in Patients With Mutations In
Cytochrome b 589
13.7 Ischemia/Reperfusion Injury 591
14 I CARBOHYDRATE METABOLISM I: MAJOR METABOLIC
PATHWAYS AND THEIR CONTROL 597
Robert A. Harris
14.1 OVERVIEW 598
14.2 CLYCO LYSIS 599
14.3 THE CLYCOLYTIC PATHWAY 603
14.4 REGULATION OF THE GLYCOLYTIC PATHWAY 614
14.5 GLUCONEOGENESIS 629
14.6 GLYCOGENOLYSIS AND GLYCOGENESIS 643
BIBLIOGRAPHY 661
QUESTIONS AND ANSWERS 662
CLINICAL CORRELATIONS
14.1 Alcohol and Barbiturates 612
14.2 Arsenic Poisoning 614
14.3 Fructose Intolerance 616
14.4 Diabetes Mellitus 618
14.5 Lactic Acidosis 621
14.6 Pickled Pigs and Malignant Hyperthermia 621
14.7 Angina Pectoris and Myocardial Infarction 622
14.8 Pyruvate Kinase Deficiency and Hemolytic Anemia 628
14.9 Hypoglycemia and Premature Infants 629
14.10 Hypoglycemia and Alcohol Intoxication 643
14.11 Glycogen Storage Diseases 647
15 | CARBOHYDRATE METABOLISM II: SPECIAL PATHWAYS
AND GLYCOCONJUGATES 665 Nancy B Schwartz
15.1 OVERVIEW 666
15.2 PENTOSE PHOSPHATE PATHWAY 666
15.3 SUGAR INTERCONVERSIONS AND NUCLEOTIDE-LINKED
SUGAR FORMATION 671
15.4 BIOSYNTHESIS OF COMPLEX CARBOHYDRATES 678
15.5 GLYCOPROTEINS 679
15.6 PROTEOGLYCANS 683
BIBLIOGRAPHY 689
QUESTIONS AND ANSWERS 689
CLINICAL CORRELATIONS
15.1 Glucose-6-Phosphate Dehydrogenase: Genetic Deficiency
or Presence of Genetic Variants in Erythrocytes 668
15.2 Wernicke-Korsakoff Syndrome: Deficiency or Presence of
Genetic Variants of Transketolase 670
15.3 Carbohydrate-Deficient Glycoprotein Syndromes (CDGS) 672
15.4 Essential Fructosuria and Fructose Intolerance: Deficiency of
Fructokinase and Fructose 1-Phosphate Aldolase 673
15.5 Galactosemia: Inability to Transform Galactose Into
Glucose 674
15.6 Pentosuria: Deficiency of XyIitol Dehydrogenase 675
15.7 Glucuronic Acid: Physiological Significance of
Glucuronide Formation 675
15.8 Blood Group Substances 679
CONTENTS | Xxi
15.9 Common Carbohydrate Marker of Lysosomal Targeting and
I-Cell Disease 682
15.10 Aspartylglycosylaminuria: Absence of 4-L-
Aspartylglycosamine Amidohydrolase 683
15.11 Enzymic Defects in Degradation of Glycolipids 68.5
15.12 Heparin Is an Anticoagulant 685
15.13 Chondrodystrophies Due to Sulfation Defects 687
15.14 Mucopolysaccharidoses 688
16 | LIPID METABOLISM I: UTILIZATION AND STORAGE
OF ENERGY IN LIPID FORM 693
J. Denis McGarry
16.1 OVERVIEW 694
16.2 CHEMICAL NATURE OF FATTY ACIDS AND
ACYLGLYCEROLS 695
16.3 SOURCES OF FATTY ACIDS 697
16.4 STORAGE OF FATTY ACIDS AS TRIACYLGLYCEROLS 706
16.5 METHODS OF INTERORGAN TRANSPORT OF FATTY
ACIDS AND THEIR PRIMARY PRODUCTS 710
16.6 UTILIZATION OF FATTY ACIDS FOR ENERGY
PRODUCTION 713
BIBLIOGRAPHY 723
QUESTIONS AND ANSWERS 723
CLINICAL CORRELATIONS
16.1 Obesity 709
16.1 Leptin and Obesily 710
16.3 Genetic Abnormalities in Lipid-Energy Transport 711
16.4 Genetic Deficiencies in Carnitine or Carnitine Palmitoyl
Transferase 715
16.5 Genetic Deficiencies in the Acyl-CoA
Dehydrogenases 716
16.6 Refsum s Disease 719
16.7 Diabetic Ketoacidosis 721
17 I LIPID METABOLISM II: PATHWAYS OF METABOLISM
OF SPECIAL LIPIDS 727
Robert H. Glew
17.1 OVERVIEW 728
17.2 PHOSPHOLIPIDS 728
17.3 CHOLESTEROL 741
17.4 SPHINGOLIPIDS 754
17.5 PROSTAGLANDINS AND THROMBOXANES 766
17.6 LIPOXYGENASE AND OXY-EICOSATETRAENOIC ACIDS 770
BIBLIOGRAPHY 774
QUESTIONS AND ANSWERS 775
CLINICAL CORRELATIONS
17.1 Respiratory Distress Syndrome 721
17.2 Treatment of Hypercholesterolemia 751
17.3 Atherosclerosis 752
17.4 Diagnosis of Gaucher s Disease in an Adult 765
XXH | CONTENTS
18 | AMINO ACID METABOLISM 779 Marguerite W. Coomes
18.1 OVERVIEW 780
18.2 INCORPORATION OF NITROGEN INTO AMINO ACIDS 781
18.3 TRANSPORT OF NITROGEN TO LIVER AND KIDNEY 786
18.4 UREA CYCLE 787
18.5 SYNTHESIS AND DEGRADATION OF INDIVIDUAL AMINO
ACIDS 790
BIBLIOGRAPHY 821
QUESTIONS AND ANSWERS 821
CLINICAL CORRELATIONS
18.1 Carbamoylphosphate Synthetase and N-Acetylglutamate
Synthetase Deficiencies 790
18.2 Deficiencies of Urea Cycle Enzymes 791
18.3 Nonketotic Hyperglycinemia 795
18.4 Folic Acid Deficiency 797
18.5 Phenylketonuria 799
18.6 Disorders of Tyrosine Metabolism 801
18.7 Parkinson s Disease 801
18.8 Hyperhomocysteinemia and Atherogenesis 805
18.9 Other Diseases of Sulfur Amino Acids 805
18.10 Diseases of Metabolism of Branched-Chain Amino
Acids 813
18.11 Diseases of Propionate and Methylmalonate
Metabolism 814
18.12 Diseases Involving Lysine and Ornithine 816
18.13 Histidinemia 816
18.14 Diseases of Folate Metabolism 817
19 | PURINE AND PYRIMIDINE NUCLEOTIDE
METABOLISM 825
Joseph G. Cory
19.1 OVERVIEW 826
19.2 METABOLIC FUNCTIONS OF NUCLEOTIDES 826
19.3 CHEMISTRY OF NUCLEOTIDES 828
19.4 METABOLISM OF PURINE NUCLEOTIDES 830
19.5 METABOLISM OF PYRIMIDINE NUCLEOTIDES 839
19.6 DEOXYRIBONUCLEOTIDE FORMATION 843
19.7 NUCLEOSIDE AND NUCLEOTIDE KINASES 848
19.8 NUCLEOTIDE METABOLIZING ENZYMES AS A FUNCTION
OF THE CELL CYCLE AND RATE OF CELL DIVISION 848
19.9 NUCLEOTIDE COENZYME SYNTHESIS 850
19.10 SYNTHESIS AND UTILIZATION OF 5-PHOSPHORIBOSYL-
1-PYROPHOSPHATE 853
19.11 COMPOUNDS THAT INTERFERE WITH CELLULAR PURINE
AND PYRIMIDINE NUCLEOTIDE 854
BIBLIOGRAPHY 858
QUESTIONS AND ANSWERS 858
CLINICAL CORRELATIONS
19.1 Gout 834
19.2 Lesch-Nyhan Syndrome 836
19.3 Increased Cytosolic S -Nucleotidase Activity 839
19.4 Immunodeficiency Diseases Associated with Defects in
Purine Nucleoside Degradation 839
19.5 Cancer Patients Undergoing Radiation or Chemotherapy
Treatment 840
19.6 Subclass of Patients with Autism 840
19.7 Hereditary Orotic Aciduria 842
20 | METABOLIC INTERRELATIONSHIPS 861 Robert A. Harris and David W. Crabb
20.1 OVERVIEW 862
20.2 STARVE-FEED CYCLE 864
20.3 MECHANISMS INVOLVED IN SWITCHING LIVER
METABOLISM BETWEEN THE WELL-FED AND STARVED
STATES 876
20.4 METABOLIC INTERRELATIONSHIPS OF TISSUES IN
VARIOUS NUTRITIONAL AND HORMONAL STATES 885
BIBLIOGRAPHY 899
QUESTIONS AND ANSWERS 900
CLINICAL CORRELATIONS
20.1 Obesity 862
20.2 Protein Malnutrition 863
20.3 Starvation 863
20.4 Reye s Syndrome 869
20.5 Hyperglycemic, Hyperosmolar Coma 874
20.6 Hyperglycemia and Protein Glycosylation 875
20.7 Type 2 (Noninsulin-Dependent) Diabetes Mellitus 887
20.8 Type 1 (Insulin-Dependent) Diabetes Mellitus 888
20.9 Complications of Diabetes and the Polyol Pathway 889
20.10 Cancer Cachexia 893
PART V | PHYSIOLOGICAL PROCESSES 903
21 | BIOCHEMISTRY OF HORMONES I:
POLYPEPTIDE HORMONES 905 _
Gerald Litwack and Thomas J. Schmidt
21.1 OVERVIEW 906
21.2 HORMONES AND THE HORMONAL CASCADE SYSTEM 907
21.3 MAJOR POLYPEPTIDE HORMONES AND THEIR ACTIONS 912
21.4 GENES AND FORMATION OF POLYPEPTIDE
HORMONES 915
21.5 SYNTHESIS OF AMINO ACID-DERIVED HORMONES 919
21.6 INACTIVATION AND DEGRADATION OF HORMONES 923
21.7 CELL REGULATION AND HORMONE SECRETION 925
21.8 CYCLIC HORMONAL CASCADE SYSTEMS 931
21.9 HORMONE-RECEPTOR INTERACTIONS 936
21.10 STRUCTURE OF RECEPTORS: /3-ADRENERCIC
RECEPTOR 940
21.11 INTERNALIZATION OF RECEPTORS 941
21.12 INTRACELLULAR ACTION: PROTEIN KINASES 943
21.13 ONCOCENES AND RECEPTOR FUNCTIONS 952
BIBLIOGRAPHY 955
QUESTIONS AND ANSWERS 955
CLINICAL CORRELATIONS
21.1 Testing Activity of the Anterior Pituitary 910
21.2 Hypopituitarism 912
21.3 Lithium Treatment of Manic Depressive Illness: The
Phosphatidylinositol Cycle 931
21.4 Decreased Insulin Receptor Kinase Activity in Cestational
Diabetes Mellitus 946
21.5 Advances in Breast Cancer Chemotherapy 955
22 | BIOCHEMISTRY OF HORMONES II:
STEROID HORMONES 959
Gerald Litwack and Thomas J. Schmidt
22.1 OVERVIEW 960
22.2 STRUCTURES OF STEROID HORMONES 960
22.3 BIOSYNTHESIS OF STEROID HORMONES 963
22.4 METABOLIC INACTIVATION OF STEROID HORMONES 967
22.5 CELL-CELL COMMUNICATION AND CONTROL OF
SYNTHESIS AND RELEASE OF STEROID HORMONES 967
22.6 TRANSPORT OF STEROID HORMONES IN BLOOD 973
22.7 STEROID HORMONE RECEPTORS 974
22.8 RECEPTOR ACTIVATION UPREGULATION AND
DOWNREGULATION 982
22.9 SPECIFIC EXAMPLES OF STEROID HORMONE ACTION AT
THE CELL LEVEL: PROGRAMMED DEATH 985
BIBLIOGRAPHY 986
QUESTIONS AND ANSWERS 986
CLINICAL CORRELATIONS
22.1 Oral Contraception 972
22.2 Apparent Mineralocorticoid Excess Syndrome 977
22.3 Mineralocorticoid Receptor Mutation Results In
Hypertension and Toxemia of Pregnancy 984
22.4 Programmed Cell Death In the Ovarian Cycle 985
23 | MOLECULAR CELL BIOLOGY 989 Thomas E. Smith
23.1 OVERVIEW 990
23.2 NERVOUS TISSUE: METABOLISM AND FUNCTION 990
contents | xxiii
23.3 THE EYE: METABOLISM AND VISION 1002
23.4 MUSCLE CONTRACTION 1016
23.5 MECHANISM OF BLOOD COAGULATION 1031
BIBLIOGRAPHY 1049
QUESTIONS AND ANSWERS 1050
CLINICAL CORRELATIONS
23.1 Lambert-Eaton Myasthenic Syndrome 997
23.2 Myasthenia Gravis: A Neuromuscular Disorder 999
23.3 Macula Degeneration and Other Causes of Loss of
Vision 1006
23.4 Niemann-Pick Disease and Retinitis Pigmentosa 1007
23.5 Retinitis Pigmentosa from Mutation of the Gene for
Peripherin 1009
23.6 Chromosomal Location of Genes for Vision 1015
23.7 Familial Hypertropic Cardiomyopathies and Mutations
in Muscle Proteins 1019
23.8 Mutations in Actin and Dilated Cardiomyopathy 1022
23.9 Troponin Subunits as Markers for Myocardial
Infarction 1024
23.10 Voltage-Gated Ion Channelopathies 1026
23.11 Ion Channels and Cardiac Muscle Disease 1027
23.12 Intrinsic Pathway Defects - Prekallikrein
Deficiency 1034
23.13 Classic Hemophilia 1039
23.14 Thrombosis and Defects of the Protein C
Pathway 1044
24 I IRON AND HEME METABOLISM 1053 William M. Awad, Jr.
24.1 IRON METABOLISM: OVERVIEW 1054
24.2 IRON-CONTAINING PROTEINS 1054
24.3 INTESTINAL ABSORPTION OF IRON 1057
24.4 MOLECULAR REGULATION OF IRON
UTILIZATION 1059
24.5 IRON DISTRIBUTION AND KINETICS 1061
24.6 HEME BIOSYNTHESIS 1063
24.7 HEME CATABOLISM 1071
BIBLIOGRAPHY 1076
QUESTIONS AND ANSWERS 1077
CLINICAL CORRELATIONS
24.1 Iron Overload and Infection 1055
24.2 Iron-Sulfur Cluster Synthesis and Human
Disease 1057
24.3 Friedreich Ataxia 1057
24.4 Duodenal Iron Absorption 1058
24.5 Mutant Iron-Responsive Element 1060
24.6 Ceruloplasmin Deficiency 1061
24.7 Iron-deficiency Anemia 1062
24.8 Hemochromatosis Type I: Molecular Genetics
and the Issue of Iron-Fortified Diets 1062
XXIV | CONTENTS
24.9 Hemochromatosis Type II 1063
24.10 Acute Intermittent Porphyria 1066
24.11 Neonatal Isoimmune Hemolysis 1074
24.12 Bilirubin UDP-Glucuronosyltransferase Deficiency 1075
24.13 Elevation of Serum Conjugated Bilirubin 1075
25 | DIGESTION AND ABSORPTION OF BASIC
NUTRITIONAL CONSTITUENTS 1081
Ulrich Hopfer
25.1 OVERVIEW 1082
25.2 DIGESTION: GENERAL CONSIDERATIONS 1084
25.3 EPITHELIAL TRANSPORT 1088
25.4 DIGESTION AND ABSORPTION OF PROTEINS 1097
25.5 DIGESTION AND ABSORPTION OF CARBOHYDRATES 1100
25.6 DIGESTION AND ABSORPTION OF LIPIDS 1104
25.7 BILE ACID METABOLISM 1110
BIBLIOGRAPHY 1112
QUESTIONS AND ANSWERS 1113
CLINICAL CORRELATIONS
25.1 Familial Chloridorrhea Causes Metabolic Alkalosis 1092
25.2 Cystic Fibrosis 1093
25.3 Bacterial Toxigenic Diarrheas and Electrolyte Replacement
Therapy 1094
25.4 Neutral Amino Aciduria: Hartnup Disease 1100
25.5 Disaccharidase Deficiency 1103
25.6 Pharmacological Interventions to Prevent Fat Absorption
and Obesity 1106
25.7 Cholesterol Stones 1109
25.8 A-/?-Lipoproteinemia 1110
26 | PRINCIPLES OF NUTRITION I:
MACRONUTRIENTS 1117 Stephen G. Chaney
26.1 OVERVIEW 1118
26.2 ENERGY METABOLISM 1118
26.3 PROTEIN METABOLISM 1119
26.4 PROTEIN-ENERGY MALNUTRITION 1123
26.5 EXCESS PROTEIN-ENERGY INTAKE 1123
26.6 CARBOHYDRATES 1125
26.7 FATS 1126
26.8 FIBER 1127
26.9 COMPOSITION OF MACRONUTRIENTS IN THE DIET 1128
BIBLIOGRAPHY 1133
QUESTIONS AND ANSWERS 1133
CLINICAL CORRELATIONS
26.1 Vegetarian Diets and Protein-Energy Requirements 1121
26.2 Low-protein Diets and Renal Disease 1121
26.3 Providing Adequate Protein and Calories for Hospitalized
Patients 1123
26.4 Carbohydrate Loading and Athletic Endurance 1125
26.5 High-Carbohydrate Versus High-Fat Diet for Diabetics 1126
26.6 Polyunsaturated Fatty Acids and Risk Factors for Heart
Disease 1129
26.7 Metabolic Adaptation: Relationship Between Carbohydrate
Intake and Serum Triacylglycerols 1130
27 | PRINCIPLES OF NUTRITION II:
MICRONUTRIENTS 1137
Stephen C. Chaney
27.1 OVERVIEW 1138
27.2 ASSESSMENT OF MALNUTRITION 1138
27.3 DIETARY REFERENCE INTAKES 1138
27.4 FAT-SOLUBLE VITAMINS 1139
27.5 WATER-SOLUBLE VITAMINS 1148
27.6 ENERGY-RELEASING WATER-SOLUBLE VITAMINS 1148
27.7 HEMATOPOIETIC WATER-SOLUBLE VITAMINS 1154
27.8 OTHER WATER-SOLUBLE VITAMINS 1157
27.9 MACROMINERALS 1159
27.10 TRACE MINERALS 1161
27.11 THE AMERICAN DIET: FACT AND FALLACY 1164
27.12 ASSESSMENT OF NUTRITIONAL STATUS IN CLINICAL
PRACTICE 1164
BIBLIOGRAPHY 1166
QUESTIONS AND ANSWERS 1167
CLINICAL CORRELATIONS
27.1 Nutritional Considerations In Cystic Fibrosis 1142
27.2 Renal Osteodystrophy 1143
27.3 Nutritional Considerations in the Newborn 1147
27.4 Anticonvulsant Drugs and Vitamin Requirements 1148
27.5 Nutritional Considerations in Alcoholics 1150
27.6 Vitamin B6 Requirements In Users of Oral
Contraceptives 1153
27.7 Gene Polymorphisms and Folic Acid Requirements 1155
27.8 Diet and Osteoporosis 1160
27.9 Nutritional Needs of Elderly Persons 1165
| APPENDIX _____
REVIEW OF ORGANIC CHEMISTRY 1169
Caron N. Angstadt
INDEX 1181
|
| any_adam_object | 1 |
| author2 | Devlin, Thomas M. |
| author2_role | edt |
| author2_variant | t m d tm tmd |
| author_facet | Devlin, Thomas M. |
| building | Verbundindex |
| bvnumber | BV014144151 |
| callnumber-first | Q - Science |
| callnumber-label | QP514 |
| callnumber-raw | QP514.2 |
| callnumber-search | QP514.2 |
| callnumber-sort | QP 3514.2 |
| callnumber-subject | QP - Physiology |
| classification_rvk | WD 4000 WD 4010 |
| classification_tum | CHE 800f |
| ctrlnum | (OCoLC)300211056 (DE-599)BVBBV014144151 |
| 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 |
| edition | 5. ed. |
| format | Book |
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| genre | Matériel didactique |
| genre_facet | Matériel didactique |
| id | DE-604.BV014144151 |
| illustrated | Illustrated |
| indexdate | 2024-07-09T18:58:26Z |
| institution | BVB |
| isbn | 0471411361 |
| language | English |
| oai_aleph_id | oai:aleph.bib-bvb.de:BVB01-009692386 |
| oclc_num | 300211056 |
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| owner_facet | DE-703 DE-91G DE-BY-TUM DE-19 DE-BY-UBM DE-29 DE-355 DE-BY-UBR DE-526 DE-11 DE-188 |
| physical | XXIV, 1216 S. Ill., graph. Darst. |
| publishDate | 2002 |
| publishDateSearch | 2002 |
| publishDateSort | 2002 |
| publisher | Wiley-Liss |
| record_format | marc |
| spelling | Textbook of biochemistry with clinical correlations ed. by Thomas M. Devlin Biochemistry 5. ed. New York Wiley-Liss 2002 XXIV, 1216 S. Ill., graph. Darst. txt rdacontent n rdamedia nc rdacarrier This book presents the biochemistry of mammalian cells, relates events at the cellular level to the subsequent physiological processes in the whole animal, and cites examples of human diseases derived from aberrant biochemical processes. Biochemistry cabt Biochimie Biochimie clinique Biochimie clinique ram Biochimie ram Physiopathologie Clinical biochemistry Physiologische Chemie (DE-588)4076124-1 gnd rswk-swf Biochemie (DE-588)4006777-4 gnd rswk-swf Matériel didactique Biochemie (DE-588)4006777-4 s DE-604 Physiologische Chemie (DE-588)4076124-1 s 1\p DE-604 Devlin, Thomas M. edt HBZ Datenaustausch application/pdf http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=009692386&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 Biochemistry cabt Biochimie Biochimie clinique Biochimie clinique ram Biochimie ram Physiopathologie Biochemistry Clinical biochemistry Physiologische Chemie (DE-588)4076124-1 gnd Biochemie (DE-588)4006777-4 gnd |
| subject_GND | (DE-588)4076124-1 (DE-588)4006777-4 |
| 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_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 | Biochemistry cabt Biochimie Biochimie clinique Biochimie clinique ram Biochimie ram Physiopathologie Biochemistry Clinical biochemistry Physiologische Chemie (DE-588)4076124-1 gnd Biochemie (DE-588)4006777-4 gnd |
| topic_facet | Biochemistry Biochimie Biochimie clinique Physiopathologie Clinical biochemistry Physiologische Chemie Biochemie Matériel didactique |
| url | http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=009692386&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA |
| work_keys_str_mv | AT devlinthomasm textbookofbiochemistrywithclinicalcorrelations AT devlinthomasm biochemistry |