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Medical Biochemistry

SIXTH EDITION

John W. Baynes, PhD

Carolina Distinguished Professor Emeritus, Department of Pharmacology, Physiology and Neuroscience, University of South Carolina School of Medicine, Columbia, SC, USA

Marek H. Dominiczak, MD, Dr Hab

Med, FRCPath, FRCP (Glas)

Hon Professor of Clinical Biochemistry and Medical Humanities, College of Medical, Veterinary and Life Sciences, University of Glasgow, Glasgow, UK

Consultant Biochemist Emeritus, Clinical Biochemistry Service, National Health Service (NHS) Greater Glasgow and Clyde, Glasgow, UK

Table of Contents

Title page

Any screen, Any time, Anywhere

Copyright

List of Contributors

Acknowledgments

Dedication

Preface

Abbreviations

Section 1: Introduction

Chapter 1. Biochemistry and Clinical Medicine: Introduction and Overview

Abstract

Introduction

What is the Sixth Edition of Medical Biochemistry?

What is Biochemistry: The Short Overview

Further Reading

Relevant websites

Abbreviations

Section 2: Molecules and Cells

Chapter 2. Amino Acids and Proteins

Abstract

Introduction

Amino Acids

Buffers

Peptides and Proteins

Purification and Characterization of Proteins

Analysis of Protein Structure

Summary

Abbreviations

Further Reading

Relevant Websites

Chapter 3. Carbohydrates and Lipids

Abstract

Introduction

Carbohydrates

Lipids

Structure of Biomembranes

Summary

Further Reading

Relevant Websites

Abbreviations

Chapter 4. Membranes and Transport

Abstract

Introduction

Types of Transport Processes

Summary Further Reading

Relevant Websites

Abbreviations

Section 3: Metabolism

Chapter 5. Oxygen Transport

Abstract

Introduction

Characteristics of Mammalian Globin Proteins

Allosteric Modulation of the Oxygen Affinity of Hemoglobin

Selected Topics

Summary

Further Reading

Relevant Websites

Abbreviations

Chapter 6. Catalytic Proteins - Enzymes

Abstract

Introduction

Enzymatic Reactions

Enzyme Kinetics

Mechanism of Enzyme Action

Enzyme Inhibition

Regulation of Enzyme Activity

Enzymatic Measurement of Blood Glucose

Summary

Further Reading

Relevant Websites

Abbreviations

Chapter 7. Vitamins and Minerals

Abstract

Introduction

Fat-Soluble Vitamins

Water-Soluble Vitamins

Dietary Supplementation of Vitamins

Minerals

Summary

Further Reading

Relevant Websites

More Clinical Cases

Abbreviations

Chapter 8. Bioenergetics and Oxidative Metabolism

Abstract

Introduction

Oxidation As A Source Of Energy

FREE ENERGY

Conservation Of Energy By Coupling Of Reactions To Hydrolysis Of ATP

Mitochondrial Synthesis Of Adenosine Triphosphate From Reduced Coenzymes

The Mitochondrial Electron Transport System

Transfer Of Electrons From NADH Into Mitochondria

Synthesis Of Adenosine Triphosphate: The Chemiosmotic Hypothesis

Inhibitors Of Oxidative Metabolism

Regulation Of Oxidative Phosphorylation

Summary

Further Reading

Relevant Websites

Abbreviations

Chapter 9. Glycolysis and the Pentose Phosphate Pathway

Abstract

Introduction

The Erythrocyte

Glycolysis

Synthesis of 2,3-Bisphosphoglycerate (2,3-BPG)

The Pentose Phosphate Pathway

Summary

Further Reading

Relevant Websites

Abbreviations

Chapter 10. The Tricarboxylic Acid Cycle

Abstract

Introduction

Functions of the Tca Cycle

Pyruvate Carboxylase

The Pyruvate Dehydrogenase Complex

Enzymes and Reactions of the Tricarboxylic Acid Cycle

Energy Yield from the Tricarboxylic Acid Cycle

Cataplerosis and Anaplerosis

Regulation of the Tricarboxylic Acid Cycle

Summary

Further Reading

Relevant Websites

Abbreviations

Chapter 11. Fay Acid and Triglyceride Catabolism

Abstract

Introduction

Activation of Fatty Acids for Transport Into the Mitochondrion

Oxidation of Fatty Acids

Ketogenesis, a Metabolic Pathway Unique to Liver

Summary

Further Reading

Relevant Websites

Abbreviations

Chapter 12. Biosynthesis and Storage of Carbohydrate

Abstract

Introduction

Structure of Glycogen

Pathway of Glycogenesis from Blood Glucose in the Liver

Pathway of Glycogenolysis in the Liver

Hormonal Regulation of Hepatic Glycogenolysis

Mechanism of Action of Glucagon

Mobilization of Hepatic Glycogen by Epinephrine

Glycogenolysis in Muscle

Regulation of Glycogenesis

Gluconeogenesis

Summary

Further Reading

Relevant Websites

Abbreviations

Chapter 13. Biosynthesis and Storage of Fay Acids

Abstract

Introduction

Fatty Acid Synthesis

Storage and Transport of Fatty Acids: Synthesis of Triacylglycerols (Triglycerides)

Regulation of Total Body Fat Stores

Summary

Further Reading

Abbreviations

Chapter 14. Biosynthesis of Cholesterol and Steroids

Abstract

Introduction

The Cholesterol Molecule

Cholesterol is Esterified Within Cells and in Plasma

Biosynthesis of Cholesterol

Cholesterol Clearance: The Bile Acids

Steroid Hormones

Vitamin D

Summary

Further Reading

Relevant Websites

More Clinical Cases

Abbreviations

Chapter 15. Biosynthesis and Degradation of Amino Acids

Abstract

Introduction

Metabolism of Dietary and Endogenous Proteins

Amino acid Degradation

Metabolism of the Carbon Skeletons of Amino Acids

Biosynthesis of Amino Acids

Inherited Diseases of Amino Acid Metabolism

Summary

Further Reading

Relevant Websites

Abbreviations

Chapter 16. Biosynthesis and Degradation of Nucleotides

Abstract Introduction

Purine Metabolism

Pyrimidine Metabolism

Formation of Deoxynucleotides

Summary

Further Reading

Relevant Websites

Abbreviations

Chapter 17. Complex Carbohydrates: Glycoproteins

Abstract

Introduction

Structures and Linkages

Interconversions of Dietary Sugars

Other Pathways of Sugar Nucleotide Metabolism

Biosynthesis of Oligosaccharides

Functions of the Oligosaccharide Chains of Glycoproteins

Summary

Further Reading

Relevant Websites

Abbreviations

Chapter 18. Complex Lipids

Abstract

Introduction

Synthesis and Turnover of Glycerophospholipids

Sphingolipids

Lysosomal Storage Diseases Resulting from Defects in Glycolipid Degradation

ABO Blood Group Antigens

Summary

Further Reading

Relevant Websites

Abbreviations

Chapter 19. The Extracellular Matrix

Abstract

Introduction

Noncollagenous Proteins in The Extracellular Matrix

Proteoglycans

Communication of Cells With The Extracellular Matrix

Summary

Further Reading

Relevant Websites

Abbreviations

Section 4: Molecular Basis of Inheritance

Chapter 20. Deoxyribonucleic Acid

Abstract

Introduction

Structure of Deoxyribonucleic Acid

The Cell Cycle in Eukaryotes

Dna Replication

DNA Repair

Recombinant Dna Technology

Principles of Molecular Hybridization

Cloning of DNA

Summary

Further Reading

Relevant Websites

Abbreviations

Chapter 21. Ribonucleic Acid

Abstract

Introduction

Molecular Anatomy Of Ribonucleic Acid Molecules

Ribonucleic Acid Polymerases

Messenger Ribonucleic Acid: Transcription

Posttranscriptional Processing of Ribonucleic Acids

Selective Degradation or Inactivation of Mrna

Further Reading

Relevant Websites and Videos

Abbreviations

Chapter 22. Protein Synthesis and Turnover

Abstract

Introduction

The Genetic Code

The Machinery of Protein Synthesis

The Process of Protein Synthesis

Protein Folding and Endoplasmic Reticulum (ER) Stress

Protein Targeting and Posttranslational Modifications

Summary

Further Reading

Relevant Websites

Abbreviations

Chapter 23. Regulation of Gene Expression: Basic Mechanisms

Abstract

Introduction

Basic Mechanisms of Gene Expression

Steroid Receptors

Alternative Approaches to Gene Regulation in Humans

Summary

Further Reading

Relevant Websites

CRISPR

Abbreviations

Chapter 24. Genomics, Proteomics, and Metabolomics

Abstract

Introduction

Genomics

Proteomics

Metabolomics

Summary

Further Reading

Relevant Websites

Abbreviations

Section 5: Signalling and Growth

Chapter 25. Membrane Receptors and Signal Transduction

Abstract

Introduction

Types of Hormone and Monoamine Receptors

Receptor Coupling to Intracellular Signal Transduction

Second Messengers

Summary

Further Reading

Relevant Websites

Abbreviations

Chapter 26. Neurotransmiers

Abstract

Introduction

Neurotransmission

Classes of Neurotransmitters

Summary

Further Reading

Relevant Websites

Abbreviations

Chapter 27. Biochemical Endocrinology

Abstract

Introduction

Hormones

Laboratory Assessment of Hormone Action

Causes of Endocrine Disease

The Hypothalamus and the Pituitary Gland

Thyroid Function: The HYPOTHALAMO-Pituitary–Thyroid Axis

The HYPOTHALAMO–Pituitary–Adrenal Axis

The Hypothalamo–Pituitary–Gonadal Axis

The Growth Hormone Axis

The Prolactin Axis

Endocrine Systems not Considered in this Chapter

Summary

Further Reading

Relevant Websites

More Clinical Cases

Abbreviations

Chapter 28. Cellular Homeostasis: Cell Growth and Cancer

Abstract

Introduction

Cell Cycle

Regulation of Cell Proliferation and Growth: Growth Factors

Regulation of the Cell Cycle

Cell Death Cancer

Summary

Further Reading

Relevant Websites

Abbreviations

Chapter 29. Aging

Abstract

Introduction

Theories of Aging

Genetic Models of Increased Life span

Antiaging Interventions: What Works and What Doesn’t

Summary

Further Reading

Relevant Websites

Abbreviations

Section 6: Fuels Nutrients and Minerals

Chapter 30. Digestion and Absorption of Nutrients: The Gastrointestinal Tract

Abstract

Introduction

Water and Electrolyte Handling in the Gastrointestinal Tract

Components of Digestion

Digestion and Absorption of Carbohydrates

Digestion and Absorption of Lipids

Digestion and Absorption of Proteins

Summary

Further Reading

Relevant Websites

Abbreviations

Chapter 31. Glucose Homeostasis and Fuel Metabolism: Diabetes Mellitus

Abstract

Introduction

Glucose Homeostasis

Glucagon and other Antiinsulin Hormones

Incretin Hormones

The Feed–Fast Cycle

Diabetes Mellitus

Hypoglycemia

Laboratory Assessment of Fuel Metabolism

Treating Diabetes

Summary

Further Reading

Relevant Websites

Abbreviations

Chapter 32. Nutrients and Diets

Abstract

Introduction

Main Classes of Nutrients

Essential Nutrients

Role of the Adipose Tissue in Controlling Energy Balance

Regulation of Food Intake

Nutrigenomics

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