Acetoacetate, 70 Acetylcholine, 75 Acetylcholinesterase, 253 Acetyl-L
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Index Acetoacetate, 70 Amphetamine, 88 – gliotoxins and, 148 Acetylcholine, 75 Amyotrophic lateral sclerosis, 381–383 – glutamate/glutamine cycle, Acetylcholinesterase, 253 Anaplerosis, 2, 8, 10–12 123, 126 Acetyl-L-carnitine, 256 Antiepileptic drugs, 177, 178, 183 – glutamate/pyruvate cycle, AD-Amino acids, discovery, 208 Antinociception, 177 123, 124 Adrenal medulla, 80, 88 Antioxidant, 350, 353, 355, 359–361, – GSH in, 148 Aerobic cells, 349 379, 381–383, 385 – interaction with neurons, 263, Aging, 378–381 Apparent diffusion coefficient, 323 267, 268 Agmatine, 100, 106 Appetite, 82, 86 – lactate, 123, 124 Agonists glutamate receptors, 179, 180 Arginase – localisation of SAAs in, 138 Alanine aminotransferase, 3, 9–11 – immunoreactivity, 105 – morphology of, 265–267, 272 Albumin, 64, 66, 69, 75–77, 85 – isoforms, 105 – oxidative stress in, 262, 265 Alcohol, 170, 182 Arginine – release of SAAs from, 139 Alzheimer’s disease, 310, 329, 333, 378, – concentration in brain and CSF, – SAA biosynthesis and metabolism 380, 381 100, 101 in, 135 – muscarinic sites, 254 – deimination, 102 – SAA transport into, 141 – type II astrocytosis, 251 – immunohistochemistry, 101 – sodium-calcium exchanger in, 139 Amfonelic acid, 87 – metabolism in astrocytes, Biosynthesis taurine, taurine – g-aminobutyrate receptors, 108, 109 transporter, 160 172, 173 – N-methylated analogues, 100 Blood-brain barrier (BBB), 48, 66, 352, – g-aminobutyric acid (GABA), – proteinogenic amino acid, 109 353, 385 357, 372, 374, 377 – substrate of NOS, 105 Bold signals, 322–324, 333, 335 Amidohydrolase I, 308 Arginine decarboxylase (ADC), Brain compartments, 309, 310 Amidohydrolase II, 309, 311, 314, 315, 106, 107 Brain peptides 317, 318, 326, 335, 336 Argininosuccinate lyase – ampholytes, 402 Amino acid, 2–4, 6–11, 349, 350, – localization, 103, 104 – cyclic peptides, 405–407 352–355, 362, 363, 369, 374, 376, Argininosuccinate synthetase – endogenous peptides, 405 378, 381, 383, 384 – immunoreactivity, 103, 104 – endorphins, 407 – aromatic, 61, 62, 85 – localization cellular/subcellular, – exogenous peptides, 408 – branched-chain, 62, 64, 73, 76, 85 103, 104 – exorphins, 408 – glutamate, 118–127 Argininosuccinate, 102–104, 109 – gamma-glutamyl peptides, 406 – large, neutral, 85 Aromatic amino acid decarboxylase – N-substistuted peptides, 404 – isoleucine, 118, 120 (AAAD), 74, 77, 78, 82 – pepetide ‘‘families’’, 405 – leucine, 118, 120–122, 124–127 Aromatic amino acids, 60–62, 85 Brain, 61 f – neutral, 64, 70, 85 ASC transport system Branched-chain amino acids (BCAAs), – valine, 118, 120 – localisation, 141 62, 66, 76, 85 – g-aminobutyric acid (GABA), – subtypes, 143 BCAT nitrogen shuttle in brain, 125 118, 119, 121, 122, 124–127 – transport of SAAs, 141–145 – catabolic pathway, 118–120, Aminoacidopathies, 279–289 Ascorbic acid, 360, 361 125, 126 Aminoacidurias, 281, 182 Aspartate aminotransferase, 3, 5, 7 – glial metabolism, 122 Aminotransferase, 3, 5, 7, 10, 11, 13, 14 Astrocytes – nitrogen transfer, 118, 120, 122, Ammonia effects, 171 – aminotransferase isoform, 126 124, 126 Ammonia, 251–253, 255, 256 – energy metabolism in, 267 – transport in brain, 121, 125 # Springer-Verlag Berlin Heidelberg 2007 414 Index Branched-chain aminotransferase Cold stress, 87 Disorders of glutathione metabolism (BCAT), 3, 4 Compartmentation, 3, 14 – g-glutamyl synthetase deficiency, – isoforms, 119 Control, ‘‘open loop’’, 64 279, 300 – localization in brain, 119 Corpus striatum, 64 – g-glutamyl transpeptidase – nitrogen shuttle in brain, 125 Creatine, synthesis, 106 deficiency, 279, 300 Branched-chain a-keto-acid Cysteine sulfinate decarboxylase – 5-oxoprolinase deficiency, – dehydrogenase enzyme – biosynthesis of taurine, 135 279, 300 complex, 120 – metabolism of L-cysteine, 135 – 5-oxoprolinuria (glutathione – maple syrup urine disease, Cysteine, 349 f synthetase deficiency), ‐ 120, 127 Cystine-glutamate (xc ) exchanger 279, 300 Breakfast, 86 – glutamate transport and, 144 Distribution taurine, 156–159 Calcium, 360, 368, 378 – GSH synthesis and, 145 Disulfide bonds, 370, 371 Canavan disease, 301, 311, 315–317, – interrelationship with glutamate DL-NAM, 75 335, 336 transporters, 144, 145 DOPA, 61, 72, 77–80, 82, 87 Cancer, 363, 364 – localisation, 144 Dopamine, 60 f Carbamyl phosphate synthetase, – neurotoxicity and, 146 D-Serine 250, 251 – SAA transport and, 141 – biosynthesis and serine racemase, ‘‘Carbohydrate-craving’’, 85, 86 D-Aspartate 211, 212 Casein, 62 – biosynthesis, 217–219 – metabolism and transport, Catecholamine, 60–62, 70, 75, 79–81, – localization in the nervous and 212–214 84, 87, 88 endocrine system, 217 – neuromodulator and, 216, 217 Catecholaminergic transmission – metabolism, 219 – new roles, 215 – dopamine, 270, 271 – neurobiology, 217–220 – NMDA receptor dysfunction and, – serotonin, 270 – precursor for endogenous 215, 216 Cationic amino acid transporter (CAT), NMDA, 219 – regulation of NMDA receptors, 107, 108 – role of endogenous, 218 209–211, 214 Cell cycle, 350, 363 – transport and release, 218 – regulation of production, 212 Cell swelling, 155, 166–170 DDAH (Dimethylarginine – release, 209, 212, 214–216 Cell volume regulation, 160, 165, 167 dimethylaminohydrolase), 105 Endogenous neuroprotectants Cell-damaging-conditions, 163–165, Degeneration retina, 161, 181 – taurine, 271 175, 179 Depression, 68, 78, 86, 87 End-product inhibition, 79 Central nervous system, 25, 29, 31, 34, Derivatives taurine, 169, 182–184 Energy drinks, 182 35, 39, 40 Development, 157–159, 161, 167, Energy metabolism, 123 Cerebellar cell migration, 158 174–176, 184 Enzyme defects, 251, 252 Cerebellum, 352, 354, 356, 368, 373 Developmental disability, 283 Enzyme phosphorylation, 73 Cerebral palsy, 250, 251 Dextrin, 85 Enzymopathy, 251–257 Chemometabolism, 340 Dextrose, 85 Eosinophilia-myalgia syndrome Cholesterol, 61 Diet, high-carbohydrate vs (EMS), 69 Choline, 75 high-protein, 86 Ephedrine, 88 Choline acetyltransferase, 253, 254 Dietary carbohydrates, 64, 78 Epilepsy, 177, 178, 183, 184 Choroid plexus, 60, 75 Dietary proteins, 62, 78, 85 Epinephrine, 60–62, 64, 79 Chronic fatigue syndrome, 378, 385 Dietary starches, 62 Essential amino acid, 184 Chronoarchitecture, 323, 340 Diffusion coefficient, 323, 331, 332 Estrogen, 61 Chronometabolism, 340 Dihydroxyphenylacetic acid Ethanol, 170, 171, 180, 182 a-chymotrypsin, 73 (DOPAC), 88 Excitotoxicity Cingulate cortex, 80 Diseases – glutamate receptors and, 146 Citrulline – Alzheimer (AD), 101, 104, 107 – SAA induced, 146 – concentration in brain and CSF,101 – multiple sclerosis, 102 Facilitated diffusion, 74, 75 – generation within proteins, 105 Disorders of GABA metabolism ‘‘False neurotransmitter’’, 78 – immunohistochemistry, 101 – GABA-transaminase deficiency, Fatty acids, 64, 75 – intermediate in citrulline-NO 279, 301 Fick’s law, 331, 332 cycle, 105 – pyridoxine dependency, Free radicals, 359, 360, 364, 370, 378, Citrullinemia, 109, 251, 256, 257 279, 300 381, 382, 384 Citrulline-NO cycle, 101, 103–106, – succinic semialdehyde Fumarate, 70 108, 109 dehydrogenase deficiency, Functional magnetic resonance Clozapine, 88 279, 301 imaging, 323–325, 332–334 Index 415 GABA, 252, 253 Glutamyl-L-cysteinyl glycine, 349 Hyperammonemic brain edema – branched chain aminotransferase, Glutathione peroxidase, 350, 352, 359, – cerebral blood flow in, 270 118, 119, 121, 126 361, 378, 379 – glutamine in, 269, 270 – GABA aminotransferase Glutathione reductase, 359, 379 – organic osmolytes in, 269, 270 (GABA-T), 126 Glutathione S-transferase, 350, 362 Hyperammonemic encephalopathies – GABA shunt, 5, 126 Glutathione synthetase, 350, 352 – animal models, 264 – metabolism, 126, 127 Glutathione, 350–370 – clinical characteristics in – SAA-induced release of, 141 Glycemic index, 62 humans, 263 GABA/glutamate-glutamine cycle, 4–9 Glycine receptors, 173–175, 177, Hypercapnia, 302, 329 GABAergic neurotransmission 179, 184 Hyperemia, 320, 324–326, 328, 329, – GABAA receptor complex, 268 Glycine, 25 f 333–336, 338, 339 – peripheral benzodiazepine GSH Hyperglycemia, 64 receptors, 268 – g-glutamyl cycle and, 135, 138 Hypernatremia, 239–241 Gabapentin, 124 – g-glutamyltranspeptidase and, Hypoacetylaspartia, 311, 314, 317, GABA-T, 5, 10, 13, 14 138, 147 336, 337 GAD,4,5,13 – L-cysteine/L-cystine as precursors, Hypoargininemia, 109 Gamma-butyrolactone, 87 134, 135, 141 Hyponatremia, 226–228, 232, 236, 239 Gastric transit, 88 – non-excitatory neurotoxicity Hypothalamus, 48, 50–54, 83, 84, 87 Gene therapy, 250, 257 and, 146 Identification of peptides Geometabolism, 340 Guanidino compounds, 100, 106, 109 – aggregation, 402 Gliotoxicity, 148, 149 Haber-Weiss reaction, 360 – Hplc, 402 Glucocorticoids, 68, 71 Haloperidol, 71, 87, 88 – immune tests, 404 Glutamate, 252 f Hemorrhagic shock, 80 – mass spectrometry, 402 Glutamate decarboxylase, 4, 5, 13 High-carbohydrate vs high-protein, 86 – mixed disulfides, 403 Glutamate dehydrogenase, 3, 5, 7, 8 Histamine H2 receptor, 53 – non-covalent binding, 402 Glutamate receptor agonists, Histamine, 48, 50–54 – sequence, 402 179, 180 Histidase gene, 50–53 Immobilization stress, 80 Glutamate receptors, 165, 168, 169, Histidine decarboxylase, 50, 53 In vivo microdialysis, 87 171, 179 Histidinemia, 48, 51–54 Inborn errors of metabolism, 297 – AMPA, 139 Homeotherms, 307, 331 Indoleamine 2,3-dioxygenase (IDO), – excitotoxicity and, 146 Homocystinuria 67–69, 74 – mGluRs, 140, 141 – cobalamin C disease, 279, 295 INF-alpha, 69 – NMDA, 139, 140, 146 – cystathionine synthetase INF-gamma, 69 – SAAs and, 139, 140, 146 deficiency, 291–294 Inner ear, 354,