Handbook of Neurochemistry

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.