Handbook of Neurochemistry: Volume VII Pathological Chemistry of the Nervous System

ISBN-13: 9781461571742 / Angielski / Miękka / 2012 / 675 str.

Abel Lajtha

Opis

Anyone who has any contact with mental patients, old or young, or their families, or just visits a mental hospital or school for the retarded, is aware of the tremendous suffering caused by malfunctioning of the brain. The func- tion of no other organ is so crucial for our everyday life, our proper func- tioning, indeed our happiness, and no other illness causes as much anguish to patients or their families as mental illness. It is surprising and sad, therefore, how little effort has been devoted to research in this area; more so because such research is the only hope to ameliorate this suffering, or, to speak in the language of politics or economics, to decrease the enormous sums that we spend on trying to help our patients, with what is must generally be agreed are the most primitive and inadequate methods of treatment. Clearly, since functions of the brain are vital not only in illness, but in health, pathology is not the only area of concern to neurochemists, but it is an area that urgently needs neurochemical contributions. Progress in this field has been slower than in other areas of neurochemistry, and it seems that solutions in this field are very elusive. The reason for this is that the experimental approach is especially difficult in conditions specific for humans, or specific for complex behavior.

Spis treści

1 Disorders of Glycogen and Related Macromolecules in the Nervous System.- I. The Normal Metabolism of Glycogen.- II. The Normal Distribution of Glycogen in the Nervous System.- III. Glycogen Deposition in Reactive Astrocytosis.- IV. Glycogen Deposition in Astrocytes after Irradiation.- V. Glycogen Deposition in Astrocytes in Severe Liver Disease.- VI. Glycogen Accumulation in Neurons and Schwann Cells.- VII. Increase of CNS Glycogen by Barbiturates.- VIII. The Effects of Certain Other Drugs and Altered Physiologic States on Glycogen in Cerebral Cortex.- IX. The Effect of Diabetes on CNS Glycogen.- X. Glycogen Storage Disease Involving the CNS: (Type 2 Glycogenosis).- XI. Glycogen Storage in an Unusual Degenerative Disorder.- XII. Polyglucosan Deposits in the Brain in Lafora’s Disease.- XIII. Corpora Amylacea in the Nervous System.- XIV. Glycogen Deposits in Hereditary Ataxia of Rabbits.- XV. “Spongy” Change and Its Possible Relationship with Rapid Glycogen Breakdown.- XVI. References.- 2 Neurochemical Aspects of Mucopolysaccharidoses.- I. Introduction.- II. Classical Mucopolysaccharidoses.- A. Hurler-Hunter Syndrome (Mucopolysaccharidoses Types I and II).- B. Syndromes of Sanfilippo, Morquio, Scheié, and Maro-teaux-Lamy (Mucopolysaccharidosis III, IV, V, and VI).- III. Other Diseases Involving Mucopolysaccharide or Glycoprotein Metabolism.- A. GM1-Gangliosidosis.- B. Fucosidosis.- C. Mannosidosis.- D. Austin-Type Metachromatic Leukodystrophy (Total Arylsulfatase Deficiency).- E. Other Disorders.- IV. References.- 3 Lipidoses.- I. Introduction.- II. Neutral Glycolipid Lipidoses.- A. Gaucher’s Disease (Glucocerebroside Lipidosis).- B. Fabry’s Disease (Ceramide Trihexoside Lipidosis).- C. Related Conditions.- III. Acidic Glycolipid Lipidoses.- A. Metachromatic Leukodystrophy (Sulfatide Lipidosis).- B. Tay-Sachs Disease (GM2-Gangliosidosis).- C. Generalized Gangliosidosis (GM1-Gangliosidosis).- IV. Phospholipid Lipidoses.- A. Niemann-Pick Disease (Sphingomyelin Lipidosis).- V. Conclusions.- VI. References.- 4 Biochemical Aspects of Multiple Sclerosis.- I. Introduction.- II. General Aspects.- A. Possibilities and Limitations of Chemical Research.- B. Concepts of Research.- C. Consequences of Neural Injury on the Biological Functions of the Organism.- III. The Problem of Selective Vulnerability of the Nervous Tissue.- A. Reaction of Tissue to Injurious Agents.- B. Relation of Axons and Myelin Sheath and Their Pathology.- IV. Morpho-Physiological Relations of the Barrier-Transport Pathways in the White Matter and Their Pathology.- V. Edema and Its Consequences.- A. General Aspects.- B. Cellular Reaction in Brain Edema.- C. The Pathogenesis of Demyelination in Brain Edema.- VI. General Outline of Inflammation.- A. Correlations of Local and Systemic Reactions.- B. Participation of the Nervous System in the Inflammatory Reaction.- C. Reaction of Tissue to Primary Cell Damage.- D. The Role of Lysosomes in Tissue Inflammation.- E. Pharmacologically Active Phospholipid Derivatives and Their Possible Role in Inflammation.- F. Microvascular Aspects of Inflammation.- G. Factors Influencing Vascular Permeability.- H. Reaction of the Blood to Injury.- I. Chronic and Reparative Phases of Inflammation.- J. Summary.- VII. Metabolic Aspects of Multiple Sclerosis.- A. Changes in Liver Function.- B. Endocrine Activity in MS.- C. Electrolyte and Oligoelements.- D. Alteration in Carbohydrate Metabolism.- E. Alteration in Lipid Metabolism.- VIII. Concluding Remarks.- IX. References.- 5 Lipid Metabolism in Wallerian Degeneration.- I. Introduction.- II. Nerve Degeneration.- A. Peripheral Nerve.- B. Central Nervous System.- III. Nerve Regeneration.- A. Nerve Cells.- B. Proximal Part of Nerve.- C. Distal Part of Nerve.- IV. Discussion.- A. Connection Between Cellular Events and Lipogenesis in Injured Nerve.- B. Character of Lipogenesis in Degeneration and Regeneration.- C. Decomposition of Myelin Lipids and the Role of Lyso Substances.- V. References.- 6 Basic Protein of Myelin and Its Role in Experimental Allergic Encephalomyelitis and Multiple Sclerosis.- I. Introduction.- II. Chemical and Physical Characterization.- A. Isolation of the Encephalitogen.- B. Localization and Extractability of the Encephalitogenic Protein from Neural Tissue and Myelin.- C. Variation in Molecular Size.- D. Modification of the Encephalitogen.- E. Physicochemical Properties of the Encephalitogen.- F. Amino Acid Sequence and Synthesis of the Active Peptide.- G. Comparison Between Two Basic Proteins: Encephalitogen and Histone of Neural Tissue.- III. Immunological Characterization.- A. General Introduction.- B. Relationship Between Encephalitogenic Activity and Myelination.- C. Transfer of EAE: Prevention and Suppression.- IV. Pathology.- A. Breakdown of the Myelin: A Decrease of the Encephalitogenic Protein, an Increase of Acid Proteinase.- V. Relationship Between EAE and Multiple Sclerosis.- VI. References.- 7 Pathological Cytosomes.- I. Introduction.- II. Isolation Procedures.- A. Standard Isolation Procedure.- B. Modifications for Specific Diseases.- III. Chemical Composition of Isolated Cytosomes.- IV. Discussion.- A. Assessment of Purity of Isolated Fractions.- B. Problem of Morphological and Chemical Alterations during Isolation Procedure.- C. Origin of Pathological Cytosomes.- D. Remaining Problems.- V. References.- VI. Note Added in Proof.- 8 Brain Damage in the Aminoacidurias.- I. Introduction.- II. Phenylketonuria.- III. Hereditary Tyrosinemia.- IV. Maple Syrup Disease (Branched-Chain Ketoaciduria).- V. Histidinemia.- VI. Idiopathic Hyperglycinemia (Ketotic Hyperglycinemia).- VII. Hyperglycinemia with Hypooxaluria (Nonketotic Hyperglycinemia).- VIII. Hyperlysinemia.- IX. Disorders of Urea Formation.- A. Ornithine Transcarbamylase Deficiency (Hyperammonemia).- B. Argininosuccinic Acid Synthetase Deficiency (Citrulli-nemia).- C. Arginosuccinase Deficiency (Arginosuccinic Aciduria).- D. Congenital Lysine Intolerance with Periodic Ammonia Intoxication.- X. The Iminoacidurias.- A. Hyperprolinemia, Type 1.- B. Hyperprolinemia, Type II.- C. Hydroxyprolinemia.- D. Iminoglycinuria.- XI. Homocystinuria.- XII. Lowe’s Syndrome (Oculocerebrorenal Syndrome).- XIII. Hartnup’s Disease.- XIV. References.- 9 Abnormal Metabolism of Sulfur-Containing Amino Acids Associated with Brain Dysfunction.- I. Homocystinuria.- A. Clinical Aspects.- B. Enzymatic Etiology.- C. Biochemical Manifestations.- D. Loading Tests and Whole Body Metabolism.- E. Cysteine as an Essential Amino Acid.- F. Other Biochemical Abnormalities.- G. Pathogenesis.- H. Treatment.- II. Cystathioninuria.- A. Clinical Aspects.- B. Enzymatic Etiology.- C. Biochemical Manifestations.- D. Loading Tests and Whole Body Metabolism.- E. Treatment with Massive Doses of Pyridoxine.- F. Secondary Cystathioninuria.- III. Sulfite Oxidase Deficiency Disease.- A. Clinical Aspects.- B. Enzymatic Etiology.- C. Biochemical Manifestations.- D. Pathogenesis.- IV. Miscellaneous Abnormalities of Metabolism of Sulfur-Containing Amino Acids Associated with Mental Disorders.- A. Excretion of ?-Mercaptolactate-Cysteine Disulfide.- B. Schizophrenia.- C. Methionine Malabsorption Syndrome.- D. Methionine Sulfoximine-Induced Seizures.- E. Down’s Syndrome.- V. Hypermethioninemia.- VI. Note Added in Proof.- VII. References.- 10 Amino Acid and Protein Metabolism in Wallerian Degeneration.- I. Introduction.- II. Nerve Degeneration.- A. General.- B. Protein and Amino Acid Changes.- C. Changes of Other Related Compounds.- D. Protein Breakdown.- E. Protein Synthesis.- F. Discussion.- III. Nerve Regeneration.- A. General.- B. Protein and Amino Acid Changes.- C. Changes of Other Related Compounds.- D. Protein Breakdown.- E. Protein Synthesis.- F. Discussion. Some Main Differences between Nerve Degeneration and Nerve Regeneration.- IV. A Discussion of the Possible Differences between Wallerian Degeneration and Other Secondary Demyelinations.- V. Conclusions. Current Research Problems.- VI. References.- 11 Changes in Metabolic Processes During Pathogenesis of Experimental Allergic Encephalomyelitis.- I. Introduction.- II. Lipid Metabolism.- III. Glycolytic and Oxidative Metabolism.- IV. Enzymes Involved in Nitrogen Metabolism.- V. Changes in Proteolytic Enzymes.- VI. Oxidation-Reduction and Ester Hydrolyzing Enzymes.- VII. Carbohydrate Hydrolyzing Enzymes.- VIII. Other Enzymes.- IX. Transglutaminase Levels in EAE.- X. References.- 12 Proteins of the Central Nervous System in Pathology.- I. Introduction.- II. Results in Diseased States.- A. Protein Synthesis and Metabolism.- B. Proteins Soluble in Aqueous Media.- C. Proteins Complexed with Lipids.- D. Miscellaneous Fractions.- E. Structural Changes in Proteins.- III. Conclusions.- IV. References.- 13 Biochemical Effects of Adrenocortical Steroids on the Central Nervous System.- I. Introduction.- II. Neurophysiological and Neuropharmacological Effects of Adrenocorticosteroids.- A. Effects on Brain Excitability.- B. Effects on Electrical Activity of the Brain.- C. Effects on Psyche and Behavior.- III. Quantity and Localization of Corticoids in Nervous Tissue.- IV. Metabolism of Corticosteroids by the Nervous System.- V. Effects of Adrenocorticosteroids on the Metabolism of the Central Nervous System.- A. Effects on Water and Electrolyte Metabolism.- B. Effects on Cerebral Blood Flow and Cerebral Oxygen and Glucose Consumption In Vivo and In Vitro.- C. Effects on Carbohydrate Metabolism.- D. Effects on Amino Acid and Protein Metabolism.- E. Effects on Lipid Metabolism.- F. Effects on Nucleic Acid Metabolism.- G. Effects on Putative Synaptic Transmitters.- VI. Summary.- VII. References.- 14 Quantitative Neurochemical Histology.- I. Neurohistological Complexity: Biochemical Constituents as Quantitative Indices of Histology.- II. Methodology for Establishing Chemoanatomical Indices.- A. Light and Electron Microscopic Histochemistry: In Situ Demonstration of Biochemical Constituents.- B. Isolation and Analysis of Cells or Parts of Cells.- C. Frozen Section Sampling and Microanalysis of Selected Cell Populations.- III. Assessment of Biochemical Indices of Subcellular Components in Neural Cells.- A. Definition and Requirements for a Good Index.- B. Subcellular Anatomical Components: Present and Potential Indices.- IV. Illustrative Use of Indices.- A. Selected Tracts and Nuclei of Human CNS.- B. Human and Rat Cerebral Cortex.- V. General Conclusions and Usefulness of Biochemical Indices in Neuropathology.- VI. References.- 15 Biochemistry of Middle and Late Life Dementias.- I. Introduction.- II. Alzheimer’s Disease.- A. Structural Chemistry.- B. Metabolism and Enzymology.- C. Histochemistry.- D. Summary—Table I.- III. Senile Dementia.- A. Structural Chemistry.- B. Metabolism.- C. Histochemistry.- D. Summary—Table II.- IV. Experimental Models of Neurofibrillary Degeneration.- A. Summary.- V. Pick’s Lobar Atrophy.- VI. Huntington’s Chorea.- A. Structural Chemistry.- B. Metabolism and Enzymology.- C. Summary—Table III.- VII. Creutzfeldt-Jakob Disease.- A. Structural Chemistry.- B. Metabolism and Enzymology.- C. Summary—Table IV.- VIII. General Discussion and Conclusions.- IX. References.- X. Note Added in Proof.- 16 The Biochemistry of Affective Disorders.- I. Introduction.- II. Brain Amines and Behavior.- A. Brain Amines and Stress.- B. The Selective Accumulation and Depletion of Brain Amines and Their Effects on Behavior.- C. The Mechanism of Action of Arousal Amines.- D. The Diurnal Variation of Brain Amines and Their Function in the Regulation of Sleep.- E. Paradoxical Central Effects of Transmitter Amines.- III. Brain Amines and Affective Disorders.- A. The Catecholamine Hypothesis of Affective Disorders.- B. Indole Metabolism in Affective Disorders.- IV. Water and Mineral Metabolism in Affective Disorders.- V. Carbohydrate Metabolism in Affective Disorders.- VI. Pituitary-Adrenocortical Function in Affective Disorders.- VII. Conclusion.- VIII. Addendum: Developments 1968-1970.- A. Brain Amines and Behavior.- B. Brain Amines and Affective Disorders.- C. Mineral Metabolism in Affective Disorders.- D. Carbohydrate Metabolism in Affective Disorders.- E. Pituitary-Adrenocortical Function in Affective Disorders IX. References 400.- 17 Alteration of the Brain Barrier System in Pathological States.- I. Introduction.- II. Cerebral Edema.- III. Cerebral Anoxia.- IV. Brain Injuries.- V. Kernicterus.- VI. Brain Tumors.- VII. Other Pathological Conditions.- A. Epilepsy.- B. Radiation.- C. Allergic Encephalomyelitis.- D. Bacterial and Viral Infections.- VIII. References.- 18 Chemical Physiopathology of the Cerebrospinal Fluid.- I. Introduction.- II. Proteins.- A. Total Proteins.- B. Electrophoresis.- C. Immunoelectrophoresis.- D. Miscellaneous.- E. Enzymes.- F. Lipoproteins and Glycoproteins.- III. Lipids.- A. Fatty Acids.- IV. Carbohydrates.- V. Simple Organic Substances.- A. Organic Acids.- B. Vitamins.- C. Nonprotein Nitrogen.- VI. Inorganic Substances.- A. Metals.- B. Ions.- VII. Blood-Brain Barrier.- VIII. Conclusions.- IX. Addendum.- X. References.- 19 Neurochemistry of Parkinsonism.- I. General Considerations.- A. Parkinsonism in Man.- B. “Parkinsonism” in Animals.- II. Chemical Pathology of Human Parkinsonism.- A. Brain.- B. Cerebrospinal Fluid.- C. Periphery.- III. Chemical Pathology of Experimentally Induced Parkinsonism.- A. Chemical Agents.- B. Brain Lesions in Animals.- IV. Summary and Conclusions.- V. References.- 20 Biochemistry of Brain Tumors.- I. Introduction.- II. Changes in Chemical Constituents of Cerebral Tissue in Brain Tumors.- A. Polysaccharides.- B. Lipids.- C. Proteins.- D. Nucleic Acids.- E. Ions.- III. Changes of Enzyme Activities in Brain Tumors.- A. Respiratory Enzymes.- B. Krebs Cycle Enzymes.- C. Hexose Monophosphate Shunt Enzymes.- D. Glycolytic Enzymes.- E. Enzymes of Lipid Metabolism.- F. Enzymes of Protein Metabolism.- G. Metabolism of Neurotransmitters.- H. Metabolism of Nucleic Acids and Nucleotides.- I. Hydrolases.- IV. Conclusions and Summary.- V. References.- 21 Amino Acid Transport.- I. Introduction.- II. Transport Systems in Brain Slices.- A. Influx.- B. “Steady State”.- C. Efflux.- III. Kinetics of Uptake.- IV. Interactions Between Amino Acids.- A. Transport Classes.- B. Interactions in Influx.- C. Effects on Long-Term Uptake.- D. Interactions in Efflux.- V. Requirements of Transport.- A. Energy Requirements.- B. Effect of Inhibitors.- C. Requirements for Oxidative Metabolism.- D. Uptake at O.- E. Ion Requirements.- F. Effect of pH.- VI. Transport in Living Brain.- VII. Tissue Heterogeneity.- VIII. Changes During Development.- A. In Vitro.- B. In Vivo.- IX. References.