Essential Medical Biochemistry and Metabolic Disease : A Pocket Guide for Medical Students and Residents

This practical, pocket-sized guide presents everything that a medical student needs to know to master the biochemistry and human metabolism content for the USMLE Step 1. It is a clear concise review of these topics in an easy to understand and digestible format. Each of the five sections provides high-yield information on the essentials of carbohydrate, lipid, protein, nucleotide and vitamin metabolism, respectively, including diagrammatic figures and tables for at-a-glance referral. Cover Half Title Essential Medical Biochemistry and Metabolic Disease: A Pocket Guide for Medical Students and Residents Copyright Dedication Foreword Preface Contents About the Author 1. Carbohydrate Metabolism Pyruvate Metabolism Pyruvate Dehydrogenase Succinate Thiokinase α-Ketoglutarate Dehydrogenase Complex Oxidative Phosphorylation Poisons of Oxidative Phosphorylation The Mitochondrial Shuttles of Carbohydrate Metabolism Glycogen Glycogen Synthesis Glycogenolysis The Glycogen Storage Disorders (GSDs) Gluconeogenesis Conversion of Pyruvate to Phosphoenolpyruvate Conversion of Fructose-1,6-Bisphosphate to Fructose-6-Phosphate Conversion of Glucose-6-Phosphate to Glucose The Cori Cycle Hexose Monophosphate Shunt (Pentose Phosphate Pathway) Insulin and Glucagon: Regulation of Glucose Metabolism Diseases Fructose Metabolism The Sorbitol Pathway Galactose Metabolism Biochemical Changes During Exercise Important Diseases of Carbohydrate Metabolism Major Metabolic Diseases Some Important Enzyme Deficiencies in Carbohydrate Metabolism 2. Lipid Metabolism Biologically Important Lipids Essential Fatty Acids Harmful Fats Simple Fatty Acids and Nutrition Oxidation of Fatty Acids Transport into the Mitochondrion: The Carnitine Shuttle β-Oxidation Energy Accounting Oxidation of Unsaturated Fatty Acids Peroxisomal Fatty Acid Metabolism Ketone Body Formation Lipogenesis (Fatty Acid Synthesis) Fatty Acid Elongation Synthesis of Monounsaturated and Polyunsaturated Fatty Acids Regulation of Fatty Acid Synthesis Insulin in Fatty Acid Metabolism Comparing and Contrasting Fatty Acid Synthesis and Oxidation Metabolism of Glycerolipids Metabolism of Sphingolipids The Sphingolipidoses and Sulfatidoses Eicosanoids Essential Differences Among the Eicosanoid Families Metabolic Changes During Fasting Cholesterol Cholesterol Biosynthesis Regulation of HMG-CoA Reductase Lipid Transport Apolipoproteins Chylomicrons Summary of Chylomicrons VLDL, IDL, and LDL VLDL Summary LDL Summary HDL and Reverse Cholesterol Transport HDL Summary Other Lipoproteins of Note Disorders of Lipid Transport Lipid-Lowering Drugs Clinical Aspects of Cholesterol Homeostasis A Model of Atherogenesis Cholesterol Metabolism Bile Acids Steroids Congenital Adrenal Hyperplasia Androgens Female Sex Hormones Vitamin D Important Diseases of Lipid Metabolism Major Metabolic Diseases Disorders of Lipid Transport (Table 2.4) Some Important Enzyme Deficiencies in Carbohydrate Metabolism 3. Amino Acid Metabolism Biologically Important Amino Acids Overview of Amino Acid Metabolism Biosynthesis of the Nutritionally Nonessential Amino Acids Important Amino Acid Derivatives The Essence of Protein Synthesis Protein Degradation Amino Acid Degradation The Urea Cycle Regulation of the Urea Cycle Hepatic Encephalopathy Ammonia Transport: The Glutamine Cycle The Alanine Cycle Catabolism of the Carbon Skeletons Insulin and Glucagon in Amino Acid Metabolism Porphyrin Synthesis and the Porphyrias Hemoglobin Porphyrin Degradation Important Diseases of Amino Acid Metabolism Major Metabolic Diseases Some Important Enzyme Deficiencies in Amino Acid Metabolism The Urea Cycle Defects The Porphyrias 4. Nucleotide Metabolism Biologically Important Nucleotides Purine Nucleotide Biosynthesis Regulation of De Novo Purine Biosynthesis Chemotherapeutic Agents that Block Purine Synthesis Enzymes Purine Salvage Synthesis of Deoxyribonucleotides Purine Degradation: The Production of Uric Acid Urate Pools Causes of Hyperuricemia Pathophysiology and Clinical Manifestations of Gout Treatment Pyrimidine Biosynthesis Regulation of Pyrimidine Biosynthesis Chemotherapeutic Agents that Block Pyrimidine Synthesis Enzymes Pyrimidine Salvage Pyrimidine Degradation Rational Drug Design Important Diseases of Nucleotide Metabolism Major Metabolic Diseases Some Important Enzyme Deficiencies in Carbohydrate Metabolism 5. Vitamins NAD+/NADH and FAD/FADH2: The Biological REDOX Reagents A Quick Review of REDOX Chemistry Coupling Redox Reactions: The Importance of Energetic Matching NAD+/NADH FAD/FADH2 Reductions Pyridoxine, Pyridoxal, and Pyridoxamine: The Chemistry of Vitamin B6 Pyridoxal Phosphate (PLP): Chemical Reactivity Folic Acid and Vitamin B12: One Carbon Chemistry Index