Human Biochemistry

Front-matter_2018_Human-Biochemistry
	Human Biochemistry
Copyright_2018_Human-Biochemistry
	Copyright
Dedication_2018_Human-Biochemistry
	Dedication
Preface_2018_Human-Biochemistry
	Preface
Introduction_2018_Human-Biochemistry
	Introduction
Chapter-1---Organ-Systems-and-Tissues_2018_Human-Biochemistry
	1 Organ Systems and Tissues
		Treatment of the Injured Knee: Use of Stem Cells to Replace Damaged Cartilage
		Development of Organs
		Stem Cells
		Gross Structures and Functions of Organ Systems
		Skin
		The Skeleton
		Cellular Composition of Tissues
		Summary
		suggested reading
			Literature
			Books
Chapter-2---The-Cell_2018_Human-Biochemistry
	2 The Cell
		Cellular Trafficking in Alzheimer’s Disease
		Cell Membrane
		Nucleus and Cell Division
		Cytoplasm and Cytoskeleton
		Endoplasmic Reticulum
		Golgi Apparatus
		Mitochondria
		Peroxisome
		Lysosome
		Summary
		Suggested Reading
			Literature
			Books
Chapter-3---Introductory-Discussion-on-Water--pH--Buffers-and_2018_Human-Bio
	3 Introductory Discussion on Water, pH, Buffers and General Features of Receptors, Channels and Pumps
		Diabetes Insipidus
		Thirst and Arginine Vasopressin (AVP)
		Action of Arginine Vasopressin on the Distal Kidney Tubule
		Water and Biological Roles
		Water Channels: Aquaporins (AQPs)
		The Role of Water in Protein Folding
		Protein–Water Interactions in Enzymatic Reactions
		Metabolic Water
		Proton Transfer in Liquid Water
		The Concentration of Hydrogen Ions (Protons) in Solution (pH)
		Buffers
		Receptors
		Ion Channels
		Enzymatic Pumping Mechanism
		Summary
		Suggested Reading
			Literature
			Books
Chapter-4---Proteins_2018_Human-Biochemistry
	4 Proteins
		Prion Disease, A Disease of Protein Conformational Change
		Amino Acids
			Amino-Acid-Related Diseases
			Biochemistry of the Amino Acids
			Amino Acids Have Two or More Potential Charges
			Numbering of Carbons in Amino Acids
			Charge State
			Synthesis of Nonessential Amino Acids and Amino Acid Degradation
		Proteins
			Amino Acid Sequence
			Secondary, Tertiary, and Quarternary Structures
			Protein Folding
			Protein Degradation
		Protein Classification
			Proteomics
			Protein Microarray
		Summary
		Suggested Reading
			Literature
			Books
Chapter-5---Enzymes_2018_Human-Biochemistry
	5 Enzymes
		Diagnostic Enzymology
			Enzymes with Multiple Subunits: Tissue-Specific Isozymes
		General Aspects of Catalysis
			Lineweaver–Burk Equation
			Michaelis–Menten Equation
			Inhibition of Enzymatic Activity
				Competitive Inhibition
				Noncompetitive Inhibition
				Uncompetitive Inhibition
				Two-substrate Systems
			Allosterism–Non-Michaelis–Menten Kinetics
		Classification
		Coenzymes
		Prosthetic Groups
		Drugs That Operate as Enzyme Inhibitors
			Drug Resistance
				Chemotherapeutics Targeting Tyrosine Kinases
			Rational Drug Design
		Enzyme Replacement Therapy—Gaucher Disease
		Summary
		Suggested Reading
			Literature
			Books
Chapter-6---Insulin-and-Sugars_2018_Human-Biochemistry
	6 Insulin and Sugars
		Diabetes
		Insulin
		The Pancreatic β-Cell and Insulin Secretion
		Detrimental Effects of Diabetes
		Synthetic Sweeteners
		Chemistry of Simple Sugars
		Glucose Transport
		Pentose Phosphate Pathway
		Conversion of Ribose to Deoxyribose
		Carbohydrate Constituents of Proteins—Glycoproteins
		Transfer of Nucleotide Sugars into the Golgi cisternae
		Sugars in Blood Group Proteins
		Lactose Intolerance
		Glycobiology
		Summary
		Suggested reading
			Literature
			Books
Chapter-7---Glycogen-and-Glycogenolysis_2018_Human-Biochemistry
	7 Glycogen and Glycogenolysis
		Glycogen Storage Disease (GSD) Type I, von Gierke disease (and others: At Least 11 Types of GSD)
		Glycogen—The Storage Carbohydrate
		Glucose Metabolism in Muscle
		Glycogenin and Formation of Glycogen
		Glycogenolysis (Releasing Glucose from Glycogen)
		Hormonal Control of Glycogen Metabolism and Blood Glucose Level
		Glucagon
		Epinephrine
		Insulin
		Glycogen Phosphorylase
		Different Glucose Transporters (GLUTs) in Different Tissues
		Summary
		Suggested Reading
			Literature
			Books
Chapter-8---Glycolysis-and-Gluconeogenesis_2018_Human-Biochemistry
	8 Glycolysis and Gluconeogenesis
		Hemolytic Anemia: Glyceraldehyde-3-Phosphate Dehydrogenase (G3PDH) Deficiency (A Rare Disease)
			Hemolytic Anemia
			Methemoglobin Reductase Pathway
			Rapoport-Luebering Shunt
		The Pentose Phosphate Pathway (PPP)
		Glycolysis, the Emden–Meyerhof Pathway
		Phosphofructokinase Enzymes Involved in the Conversion of Fructose-6-Phosphate to Fructose-1,6-Bisphosphate
		Cell Proliferation and Tumor Growth—the Warburg Effect
		Gluconeogenesis
			Alanine Cycle
			Glucose Can Be Formed From Glycerol
			Small Molecule Regulation of Gluconeogenesis
		Glucose Transporters
		Summary
		Suggested Reading
			Literature
			Books
Chapter-9---Lipids_2018_Human-Biochemistry
	9 Lipids
		Hypercholesterolemia
			Autosomal Recessive Hypercholesterolemia (ARH) Protein
			ApoB-100 Protein Mutations
			Normal LDL Function at Tissue Cells
		Biosynthesis of Cholesterol
		Inhibition of Liver HMGCoA Reductase by Drugs (Statins)
		The ARH Protein
		Bile Acids
		Fatty Acids and Fat
			Glycerol and Its Substituents; Triglycerides
			Fat (Triglyceride) Digestion and Uptake
			Chylomicrons
			β-Oxidation
			Activation and Transport of Fatty Acids Into Mitochondria
			Unsaturated Fatty Acids
			Ketone Bodies
			Lipid Metabolism, Lipid Droplets, and Hormonal Control
			Fatty Acid Synthesis
			Phospholipids and Membranes
			Lipins
			Synthesis and Degradation of Ceramide
			Glycosphingolipids
		Properties of Lipoproteins
		Lipid Anchoring of Proteins to Membranes
		Summary
		Suggested Reading
			Literature
			Books
Chapter-10---Nucleic-Acids-and-Molecular-Genetics_2018_Human-Biochemistry
	10 Nucleic Acids and Molecular Genetics
		Huntington’s Disease, a Single Gene Mutation
		Purines and Pyrimidines
		Base Pairing
		The Structure of DNA
		Biosynthesis of Purines and Pyrimidines
		Purine Interconversions
		Catabolism of Purine and Pyrimidine Nucleotides
		Salvage Pathway
			Pyrimidine Catabolism
			Deoxyribose-Containing Nucleotides
			Formation of Deoxyribose From Ribose Only in Its Nucleotide Form
			Disorders of Purine and Pyrimidine Metabolism
			Biosynthesis of Deoxyribonucleic Acid in the Nucleus
		Mitochondrial DNA Synthesis
		DNA Mutations and Damage
		Restriction Enzymes
		Probing Libraries for Specific Genes
		Hybridization Techniques
		Amplification of DNA Sequences: Polymerase Chain Reaction
		Identification of a Specific Gene on a Chromosome
		Determining DNA Sequence
		Inhibitors of DNA Synthesis
		RNA Interference
		Coding DNA
		Noncoding DNA
			Introns
		Transposons
		Alu Elements
		5′ Capping of RNA Containing Exons and Introns
		Polyadenylation of Pre-mRNA
		Overall Transcription–Translation Process
		Intron Exclusion From Pre-mRNA
		Coding Ribonucleic Acid (RNA)
			Messenger RNA
		Noncoding RNAs
			Transfer RNA (tRNA)
		Ribosomal RNA (rRNA)
		Small Nucleolar RNA (snoRNA)
			MicroRNA (miRNA)
			Piwi-interacting RNA (piRNA)
			Small Interfering RNA or Short Interfering RNA or Silencing RNA (siRNA)
			RNA Components of the Spliceosome
			Self-Splicing RNAs
			RNA Secondary Structures
		Genomics
			Next-Generation Sequencing
			Proteomics
			Gene Therapy
		Summary
		Suggested Reading
			Literature
			Books
Chapter-11---Protein-Biosynthesis_2018_Human-Biochemistry
	11 Protein Biosynthesis
		Defects in Mitochondrial Oxidative Phosphorylation and Disease; Deficiency in Mitochondrial Translation
			Protein Synthesis in the Mitochondrion
			Mitochondrial Encephalomyopathy With Lactic Acidosis and Stroke-Like Episodes (MELAS)
		Protein Synthesis Directed by the Nucleus
		The Ribosome
			WARNING!!! DUMMY ENTRY
				Structure of Transfer RNA (tRNA)
				Initiation: Amino Acid tRNA Synthase
				Elongation and Peptidyltransferase Ribozyme
				Termination
				Inhibitors of Protein Synthesis
			Proteins Synthesized in the Cytoplasm but Destined for the Mitochondria
			Proteins Destined for the Nucleus
			Proteins Destined for Other Sites Including the Plasma Membrane and Secretion from the Cell
		Summary
		Suggested Reading
			Literature
			Books
Chapter-12---Transcription_2018_Human-Biochemistry
	12 Transcription
		Congenital Heart Disease; Mutations of Transcription Factors
			Normal Heart Development
			Transcription Factors Control Heart Development
			GATA4
			Nkx2.5
			Tbx5
			Transcription Factors Involved in Cardiac Hypertrophy
		Transcription Factors and the Transcription Complex
		Enhancers
		Coactivators and Corepressors
		The Glucocorticoid Receptor as a Model Transcription Factor
			The Inactive Glucocorticoid Receptor (GR) in the Cytoplasm and its Activation
		Classes of Nuclear Receptors
		Cell Membrane Receptors
		Receptor Isoforms
		Chromatin
		Summary
		Suggested Reading
			Literature
			Books
Chapter-13---Metabolism-of-Amino-Acids_2018_Human-Biochemistry
	13 Metabolism of Amino Acids
		Urea-Cycle-Related Disease: Hyperammonemia
		The Urea Cycle
		Amino Acid Metabolism: Amino and Amide Group Transfers
		Transamination
		Transamidation
		Deamination
		Oxidation of Amino Acids
		Amino Acid Racemization
		l-Amino Acid Decarboxylation
		Metabolism of Amino Acids to Active Substances
			Methionine
			Phenylalanine and Tyrosine
			Formation of Catecholamines DOPA (Dopamine, Norepinephrine, and Epinephrine)
			Melanin
			Blockages in the Metabolism of Phenylalanine and Tyrosine and Disease
			Tryptophan
			Arginine
			Histidine
			Glutamate
			Serine
		Catabolism of Amino Acids
		Summary
		Suggested Reading
			Literature
			Books
Chapter-14---Metabolism-of-Fat--Carbohydrate--and-Nucle_2018_Human-Biochemis
	14 Metabolism of Fat, Carbohydrate, and Nucleic Acids
		Gaucher Disease: Most Common Lipid Storage Disease
		Lipid Metabolism
			Fatty Acid Degradation
			Fat as Storage Energy
			Joint Regulation of Lipid and Carbohydrate Metabolism
			Glucagon and Glucagon-Like Peptides
			Metabolism of Cholesterol
			Sunlight and Vitamin D
			Metabolism of Steroid Hormones Formed From Cholesterol
			Bile Acid Metabolism
		Carbohydrate Metabolism
			Glycolysis
			Gluconeogenesis
			Vigorous Exercise and Gluconeogenesis
		The Tricarboxylic Acid Cycle (TCA Cycle), Citric Acid Cycle, or Kreb’s Cycle
			The Electron Transport Chain
		Nucleic Acid Metabolism
			RNA Degradation
			DNA Degradation
			DNA Damage and Repair
		Overview of Metabolism
		Metabolism in Stem Cells
		Summary
		Suggested Reading
			Literature
			Books
Chapter-15---Polypeptide-Hormones_2018_Human-Biochemistry
	15 Polypeptide Hormones
		Panhypopituitarism: Malfunction of the Hypothalamus–Pituitary-End Organ Axis
		Hormonal Signaling Pathways
		Signaling From Hypothalamus to Posterior Pituitary
			Hormonal Signaling From the Hypothalamus to the Anterior Pituitary
		Models of Hormone Action of Anterior Pituitary Hormones
			ACTH
			α-MSH (Melanocyte-Stimulating Hormone); Melanotropin, Intermedin
			Growth Hormone
			Prolactin
			Thyroid Stimulating Hormone (TSH), Thyrotropin
			The Gonadotropins: Luteinizing Hormone (LH) and Follicle Stimulating Hormone (FSH)
			Overview of the Release and Functioning of the Vasopressin (AVP) and Oxytocin (OT) Hormones from the Posterior Pituitary
		Orexins (Hypocretins): Hypothalamic Hormones Controlling Sleep and Feeding
		Adiponectin From Adipose (Fat) Tissue
		Hormones of the Gastrointestinal (GI) Tract
			Gastrin
			Food Intake and Hormones
			Hormones Affecting Food Intake
		Summary
		Suggested Reading
			Literature
			Books
Chapter-16---Steroid-Hormones_2018_Human-Biochemistry
	16 Steroid Hormones
		Stress
			Nociceptin
			Responses to Stress
			Production of High Levels of Cortisol (Cushing’s Disease) and Subnormal Levels of Cortisol (Addison’s Disease)
			Adrenal Cortex
		Structures of Steroid Hormone Receptors
			Coactivators and Corepressors
		Physiological Functions of Steroid Hormones from Specific Receptor Knockouts
		Steroid Transporting Proteins in Plasma
		Enzymatic Inactivation of Cortisol
		Cortisol and Aldosterone
		DEHYDROEPIANDROSTERONE (DHEA)
		Structural Considerations of Steroid Hormones
		Receptor Activation
		Vitamin D Hormone
		Thyroid Hormone
		Environmental Xenobiotics That Agonize or Antagonize the Estrogen Receptor
		Crosstalk Between Steroid Receptors and Peptide Hormones
		Sex Hormones
		Peroxisome Proliferators and Their Receptors
		Glucocorticoid Induction of Programmed Cell Death (Apoptosis)
		Summary
		Suggested Reading
			Literature
			Books
Chapter-17---Growth-Factors-and-Cytokines_2018_Human-Biochemistry
	17 Growth Factors and Cytokines
		Prospects for Cytokine TRAIL (TNF-Related Apoptosis Inducing Ligand) and Ovarian Cancer
			TRAIL and Its Mechanism
		The Tumor Necrosis Factor (TNF) Superfamily
		Growth Factors
			Platelet-Derived Growth Factor
			Epidermal Growth Factor
			Transforming Growth Factor
			Fibroblast Growth Factor
			Neurotrophins and Nerve Growth Factor (NGF)
			Colony Stimulating Factor
			Erythropoietin
			Interferon
			Insulin-Like Growth Factors (IGF-I, IGF-II)
			Interleukins
			Overview
		Summary
		Suggested Reading
			Literature
			Books
Chapter-18---Membrane-Transport_2018_Human-Biochemistry
	18 Membrane Transport
		Cystic Fibrosis (Mucoviscidosis) and Aberrant Ion Transport
		Types of Membrane Transport
			Absorption of Large Molecules Binding to Receptors on the Cell Surface
			Exocytosis
			Passive Diffusion (Osmosis)
			Facilitated Diffusion
			Active Transport Requiring Energy
			Simple and Coupled Transporters
			Ions and Gradients
			Entry of Magnesium and Divalent Ions into Cells
			Proton (H+) Transport
			Monocarboxylate Transporter (MCT), a Cotransporter (Symporter)
			Citrate Transporters in Mitochondria and Plasma Membrane
			Intestinal Transport of Di- and Tripeptides
			Amino Acid Transporters
			The Glutamate Synapse and Excitatory Amino Acid Transporters
		Fatty Acid Transport Proteins
		Voltage-Gated Sodium Channels
		Epithelial Sodium Conductance Channel
		Mutidrug Resistance Channel (MDR), a Member of the ABC Transporter Superfamily
		Blood–Brain-Barrier
		Summary
		Suggested Reading
			Literature
			Books
Chapter-19---Micronutrients--Metals-and-Iodine-_2018_Human-Biochemistry
	19 Micronutrients (Metals and Iodine)
		Iron Deficiency Anemia
		Uptake of Iron During Digestion
			Hepcidin, A Peptide Hormone, is the Principal Regulator of Iron Homeostasis
			Hemojuvelin (HJV), An Anchored Membrane Protein Stimulates Hepcidin Transcription Through Bone Morphogenic (Morphogenetic) ...
		Heme Synthesis
		Hemoglobin Formation
		Trace Elements
			Trace Metals
			Copper
				Ceruloplasmin
				Wilson’s Disease
				Human Copper Transporter 1
				Cellular Prion Protein
				Copper–Zinc Superoxide Dismutase (SOD)
			Magnesium (Mg2+)
			Zinc (Zn2+)
			Molybdenum (Mo2+)
			Selenium (Se2−)
			Selenodeiodinases
			Selenophosphate Synthase (SePS)
			Calcium (Ca2+)
			Iodine (I)
		Summary
		Suggested reading
			Literature
			Books
Chapter-20---Vitamins-and-Nutrition_2018_Human-Biochemistry
	20 Vitamins and Nutrition
		Vitamin D Deficiency
		Vitamins
			Water-Soluble Vitamins
			Thiamine (Vitamin B1, Aneurine)
			Riboflavin (Vitamin B2)
			Niacin (Vitamin B3)
			Pantothenic Acid (Vitamin B5, Pantothenate)
			Pyridoxine, Pyridoxal, and Pyridoxamine (Vitamin B6)
			Biotin (Vitamin H)
			Cobalamin (Vitamin B12)
			Folic Acid (Vitamin B9, Pteroylglutamic Acid)
			Ascorbic Acid (Vitamin C)
		Fat-Soluble Vitamins
			Vitamin A (β-Carotene)
			Vitamin D (1,25-Dihydroxyvitamin D3, Active Form)
			Vitamin E (α-Tocopherol)
			Vitamin K
		Balanced Nutrition
			Nutrient Intake
		Summary
		Suggested Reading
			Literature
			Books
Chapter-21---Blood-and-Lymphatic-System_2018_Human-Biochemistry
	21 Blood and Lymphatic System
		Deep Vein Thrombosis
			Factor V Leiden
		Blood-Clotting Mechanism
		Blood
			Transport of Oxygen
			Carbon Dioxide
			Degradation of the Red Blood Cell
			Bilirubin
			Blood Cells
			Blood Proteins
			Blood Type and Rh Factor
			Origin of Nonself Blood Type Antibodies
			Rh Antigen
		Lymphatic System
			Thymosin
		Summary
		Suggested Reading
			Literature
			Books
List-of-Abbreviations_2018_Human-Biochemistry
	List of Abbreviations
Appendix-1---Abbreviations-of-the-Common-Amino-Acids_2018_Human-Biochemistry
	Appendix 1 Abbreviations of the Common Amino Acids
Appendix-2---The-Genetic-Code_2018_Human-Biochemistry
	Appendix 2 The Genetic Code
Appendix-3---Weights-and-Measures_2018_Human-Biochemistry
	Appendix 3 Weights and Measures
Index_2018_Human-Biochemistry
	Index