Index

Acetoacetate, 70 Acetylcholine, 75 Acetylcholinesterase, 253 Acetyl-L-carnitine, 256 AD-Amino acids, discovery, 208 Adrenal medulla, 80, 88 Aerobic cells, 349 Aging, 378381 Agmatine, 100, 106 Agonists glutamate receptors, 179, 180 Alanine aminotransferase, 3, 911 Albumin, 64, 66, 69, 7577, 85 Alcohol, 170, 182 Alzheimers disease, 310, 329, 333, 378, 380, 381 muscarinic sites, 254 type II astrocytosis, 251 Amfonelic acid, 87 g-aminobutyrate receptors, 172, 173 g-aminobutyric acid (GABA), 357, 372, 374, 377 Amidohydrolase I, 308 Amidohydrolase II, 309, 311, 314, 315, 317, 318, 326, 335, 336 Amino acid, 24, 611, 349, 350, 352355, 362, 363, 369, 374, 376, 378, 381, 383, 384 aromatic, 61, 62, 85 branched-chain, 62, 64, 73, 76, 85 glutamate, 118127 large, neutral, 85 isoleucine, 118, 120 leucine, 118, 120122, 124127 neutral, 64, 70, 85 valine, 118, 120 g-aminobutyric acid (GABA), 118, 119, 121, 122, 124127 Aminoacidopathies, 279289 Aminoacidurias, 281, 182 Aminotransferase, 3, 5, 7, 10, 11, 13, 14 Ammonia effects, 171 Ammonia, 251253, 255, 256
#

Amphetamine, 88 Amyotrophic lateral sclerosis, 381383 Anaplerosis, 2, 8, 1012 Antiepileptic drugs, 177, 178, 183 Antinociception, 177 Antioxidant, 350, 353, 355, 359361, 379, 381383, 385 Apparent diffusion coefcient, 323 Appetite, 82, 86 Arginase immunoreactivity, 105 isoforms, 105 Arginine concentration in brain and CSF, 100, 101 deimination, 102 immunohistochemistry, 101 metabolism in astrocytes, 108, 109 N-methylated analogues, 100 proteinogenic amino acid, 109 substrate of NOS, 105 Arginine decarboxylase (ADC), 106, 107 Argininosuccinate lyase localization, 103, 104 Argininosuccinate synthetase immunoreactivity, 103, 104 localization cellular/subcellular, 103, 104 Argininosuccinate, 102104, 109 Aromatic amino acid decarboxylase (AAAD), 74, 77, 78, 82 Aromatic amino acids, 6062, 85 ASC transport system localisation, 141 subtypes, 143 transport of SAAs, 141145 Ascorbic acid, 360, 361 Aspartate aminotransferase, 3, 5, 7 Astrocytes aminotransferase isoform, 126 energy metabolism in, 267

gliotoxins and, 148 glutamate/glutamine cycle, 123, 126 glutamate/pyruvate cycle, 123, 124 GSH in, 148 interaction with neurons, 263, 267, 268 lactate, 123, 124 localisation of SAAs in, 138 morphology of, 265267, 272 oxidative stress in, 262, 265 release of SAAs from, 139 SAA biosynthesis and metabolism in, 135 SAA transport into, 141 sodium-calcium exchanger in, 139 Biosynthesis taurine, taurine transporter, 160 Blood-brain barrier (BBB), 48, 66, 352, 353, 385 Bold signals, 322324, 333, 335 Brain compartments, 309, 310 Brain peptides ampholytes, 402 cyclic peptides, 405407 endogenous peptides, 405 endorphins, 407 exogenous peptides, 408 exorphins, 408 gamma-glutamyl peptides, 406 N-substistuted peptides, 404 pepetide families, 405 Brain, 61 f Branched-chain amino acids (BCAAs), 62, 66, 76, 85 BCAT nitrogen shuttle in brain, 125 catabolic pathway, 118120, 125, 126 glial metabolism, 122 nitrogen transfer, 118, 120, 122, 124, 126 transport in brain, 121, 125

Springer-Verlag Berlin Heidelberg 2007

414

Index
Branched-chain aminotransferase (BCAT), 3, 4 isoforms, 119 localization in brain, 119 nitrogen shuttle in brain, 125 Branched-chain a-keto-acid dehydrogenase enzyme complex, 120 maple syrup urine disease, 120, 127 Breakfast, 86 Calcium, 360, 368, 378 Canavan disease, 301, 311, 315317, 335, 336 Cancer, 363, 364 Carbamyl phosphate synthetase, 250, 251 Carbohydrate-craving, 85, 86 Casein, 62 Catecholamine, 6062, 70, 75, 7981, 84, 87, 88 Catecholaminergic transmission dopamine, 270, 271 serotonin, 270 Cationic amino acid transporter (CAT), 107, 108 Cell cycle, 350, 363 Cell swelling, 155, 166170 Cell volume regulation, 160, 165, 167 Cell-damaging-conditions, 163165, 175, 179 Central nervous system, 25, 29, 31, 34, 35, 39, 40 Cerebellar cell migration, 158 Cerebellum, 352, 354, 356, 368, 373 Cerebral palsy, 250, 251 Chemometabolism, 340 Cholesterol, 61 Choline, 75 Choline acetyltransferase, 253, 254 Choroid plexus, 60, 75 Chronic fatigue syndrome, 378, 385 Chronoarchitecture, 323, 340 Chronometabolism, 340 a-chymotrypsin, 73 Cingulate cortex, 80 Citrulline concentration in brain and CSF, 101 generation within proteins, 105 immunohistochemistry, 101 intermediate in citrulline-NO cycle, 105 Citrullinemia, 109, 251, 256, 257 Citrulline-NO cycle, 101, 103106, 108, 109 Clozapine, 88 Cold stress, 87 Compartmentation, 3, 14 Control, open loop, 64 Corpus striatum, 64 Creatine, synthesis, 106 Cysteine sulnate decarboxylase biosynthesis of taurine, 135 metabolism of L-cysteine, 135 Cysteine, 349 f Cystine-glutamate (xc) exchanger glutamate transport and, 144 GSH synthesis and, 145 interrelationship with glutamate transporters, 144, 145 localisation, 144 neurotoxicity and, 146 SAA transport and, 141 D-Aspartate biosynthesis, 217219 localization in the nervous and endocrine system, 217 metabolism, 219 neurobiology, 217220 precursor for endogenous NMDA, 219 role of endogenous, 218 transport and release, 218 DDAH (Dimethylarginine dimethylaminohydrolase), 105 Degeneration retina, 161, 181 Depression, 68, 78, 86, 87 Derivatives taurine, 169, 182184 Development, 157159, 161, 167, 174176, 184 Developmental disability, 283 Dextrin, 85 Dextrose, 85 Diet, high-carbohydrate vs high-protein, 86 Dietary carbohydrates, 64, 78 Dietary proteins, 62, 78, 85 Dietary starches, 62 Diffusion coefcient, 323, 331, 332 Dihydroxyphenylacetic acid (DOPAC), 88 Diseases Alzheimer (AD), 101, 104, 107 multiple sclerosis, 102 Disorders of GABA metabolism GABA-transaminase deficiency, 279, 301 pyridoxine dependency, 279, 300 succinic semialdehyde dehydrogenase deficiency, 279, 301 Disorders of glutathione metabolism g-glutamyl synthetase deficiency, 279, 300 g-glutamyl transpeptidase deficiency, 279, 300 5-oxoprolinase deficiency, 279, 300 5-oxoprolinuria (glutathione synthetase deficiency), 279, 300 Distribution taurine, 156159 Disulde bonds, 370, 371 DL-NAM, 75 DOPA, 61, 72, 7780, 82, 87 Dopamine, 60 f D-Serine biosynthesis and serine racemase, 211, 212 metabolism and transport, 212214 neuromodulator and, 216, 217 new roles, 215 NMDA receptor dysfunction and, 215, 216 regulation of NMDA receptors, 209211, 214 regulation of production, 212 release, 209, 212, 214216 Endogenous neuroprotectants taurine, 271 End-product inhibition, 79 Energy drinks, 182 Energy metabolism, 123 Enzyme defects, 251, 252 Enzyme phosphorylation, 73 Enzymopathy, 251257 Eosinophilia-myalgia syndrome (EMS), 69 Ephedrine, 88 Epilepsy, 177, 178, 183, 184 Epinephrine, 6062, 64, 79 Essential amino acid, 184 Estrogen, 61 Ethanol, 170, 171, 180, 182 Excitotoxicity glutamate receptors and, 146 SAA induced, 146 Facilitated diffusion, 74, 75 False neurotransmitter, 78 Fatty acids, 64, 75 Ficks law, 331, 332 Free radicals, 359, 360, 364, 370, 378, 381, 382, 384 Fumarate, 70 Functional magnetic resonance imaging, 323325, 332334

Index
GABA, 252, 253 branched chain aminotransferase, 118, 119, 121, 126 GABA aminotransferase (GABA-T), 126 GABA shunt, 5, 126 metabolism, 126, 127 SAA-induced release of, 141 GABA/glutamate-glutamine cycle, 49 GABAergic neurotransmission GABAA receptor complex, 268 peripheral benzodiazepine receptors, 268 Gabapentin, 124 GABA-T, 5, 10, 13, 14 GAD, 4, 5, 13 Gamma-butyrolactone, 87 Gastric transit, 88 Gene therapy, 250, 257 Geometabolism, 340 Gliotoxicity, 148, 149 Glucocorticoids, 68, 71 Glutamate, 252 f Glutamate decarboxylase, 4, 5, 13 Glutamate dehydrogenase, 3, 5, 7, 8 Glutamate receptor agonists, 179, 180 Glutamate receptors, 165, 168, 169, 171, 179 AMPA, 139 excitotoxicity and, 146 mGluRs, 140, 141 NMDA, 139, 140, 146 SAAs and, 139, 140, 146 Glutamate transporter family localisation, 142 SAAs as substrates, 142, 143 subtypes of, 142 Glutamate-glutamine cycle, 6, 8, 9 Glutaminase, 5, 6 Glutamine synthetase, 3, 5, 710, 12, 118, 119, 123 Glutamine, 26, 10, 1214, 251, 252, 256, 257 Glutaminergic neurotransmission nitrosative stress, 268 NMDA receptor activation, 267 NMDA receptor/nitric oxide/ cGMP pathway, 263 g-glutamyl cycle astrocytes and, 135, 138 GSH synthesis and 135, 138 L-cysteine and, 135 g-glutamylcysteine synthase, 138, 148 Glutamyl cysteine synthetase, 350, 354, 361, 364 Glutamyl transferase, 350, 363, 374 Glutamyl-L-cysteinyl glycine, 349 Glutathione peroxidase, 350, 352, 359, 361, 378, 379 Glutathione reductase, 359, 379 Glutathione S-transferase, 350, 362 Glutathione synthetase, 350, 352 Glutathione, 350370 Glycemic index, 62 Glycine receptors, 173175, 177, 179, 184 Glycine, 25 f GSH g-glutamyl cycle and, 135, 138 g-glutamyltranspeptidase and, 138, 147 L-cysteine/L-cystine as precursors, 134, 135, 141 non-excitatory neurotoxicity and, 146 Guanidino compounds, 100, 106, 109 Haber-Weiss reaction, 360 Haloperidol, 71, 87, 88 Hemorrhagic shock, 80 High-carbohydrate vs high-protein, 86 Histamine H2 receptor, 53 Histamine, 48, 5054 Histidase gene, 5053 Histidine decarboxylase, 50, 53 Histidinemia, 48, 5154 Homeotherms, 307, 331 Homocystinuria cobalamin C disease, 279, 295 cystathionine synthetase deficiency, 291294 hereditary folate malabsorption, 279, 295 methionine synthase deficiency (Cobalamin E disease), 279, 295 methylenetetrahydrofolate reductase deficiency, 279, 294 remethylation deficiency homocystinuria, 279, 294 Homovanillic acid (HVA), 78, 88 Huntingtons disease, 332 Hydrogen peroxide, 350, 352, 359361, 381 5-hydroxyindole acetic acid (5-HIAA), 78, 85, 86 5-hydroxyindoleacetic acid, 254 6-hydroxydopamine, 87 Hydroxyindole-O-methyltransferase (HIOMT), 74 5-hydroxytryptophan (5-HTP), 61, 77, 78, 87 Hyperacetylaspartia, 311, 314 Hyperammonemia, 109, 171 Hyperammonemic brain edema cerebral blood flow in, 270 glutamine in, 269, 270 organic osmolytes in, 269, 270 Hyperammonemic encephalopathies animal models, 264 clinical characteristics in humans, 263 Hypercapnia, 302, 329 Hyperemia, 320, 324326, 328, 329, 333336, 338, 339 Hyperglycemia, 64 Hypernatremia, 239241 Hypoacetylaspartia, 311, 314, 317, 336, 337 Hypoargininemia, 109 Hyponatremia, 226228, 232, 236, 239 Hypothalamus, 48, 5054, 83, 84, 87 Identication of peptides aggregation, 402 Hplc, 402 immune tests, 404 mass spectrometry, 402 mixed disulfides, 403 non-covalent binding, 402 sequence, 402 Immobilization stress, 80 In vivo microdialysis, 87 Inborn errors of metabolism, 297 Indoleamine 2,3-dioxygenase (IDO), 6769, 74 INF-alpha, 69 INF-gamma, 69 Inner ear, 354, 356, 357 Inositol phosphates, enhanced production by SAAs, 140 Insomnia, 69, 86 Insulin, 6264, 66, 71, 72, 7678, 85, 86 Ischemia, 163165, 167, 169, 178180, 184 Isoleucine, 62, 64, 73, 76 a-ketoglutarate dehydrogenase, effect of ammonia, 255 Kynurenic acid, 68, 69, 74 regulation of synthesis by L-CSA, 145 a-lactalbumin, 62 Large neutral amino acids (LNAA), 60, 64, 66, 76, 77, 8588 LAT1, 75 LAT14F2hc heterodimer, 75 Lateral tegmentum, 79 L-carnitine, 256 L-cysteic acid biosynthesis and metabolism, 135 interaction with excitatory receptors, 146

415

416

Index
localisation in the brain, 135, 136 release, 139 toxicity, 146 transport, 143, 144 L-cysteine biosynthesis and metabolism, 135 GSH synthesis and, 135, 138, 141, 149 interaction with excitatory receptors, 146 localisation in the brain, 139 release, 139 toxicity, 134, 146, 147 transport, 138, 142, 143, 145 L-cysteine sulphinic acid biosynthesis and metabolism, 135 interaction with excitatory receptors, 146 localisation in the brain, 135, 136 release, 139 toxicity, 146 transport, 140 L-cystine biosynthesis and metabolism, 135 GSH synthesis and, 135, 138, 141, 145, 146 localisation in the brain, 135, 136, 141, 142, 145 release, 135, 138 toxicity, 146 transport, 142, 143, 145 Learning and memory, 88, 365, 378, 381 Leucine, 62, 64, 65, 73, 86 L-homocysteic acid interaction with excitatory receptors, 146 localisation in the brain, 136 release, 139 toxicity, 146, 148 transport, 138, 140, 142144 L-homocysteine biosynthesis, 135, 137 neurotoxicity, 147148 potentiation of b-amyloid toxicity, 147 vitamin B12 and, 137, 148 L-homocysteine sulphinic acid biosynthesis and metabolism, 135 interaction with excitatory receptors, 141 localisation in the brain, 139 release, 139 L-Homocystine, 135 Lipid peroxidation, 350, 360, 379, 381 Liver transplantation, 256 L-methionine, precursor for L-Hcys, 135 Locus coeruleus, 79 Long-lasting potentiation, 174 Long-term potentiation, 175, 370, 372, 378 L-serine, 2535 L-serine-O-sulphate alanine aminotransferase and, 148 gliotoxicity, 148 L-System, 75 L-threonine, 3941 Lysolecithin, 73 L-a-aminoadipate cystine-glutamate exchanger and, 144 gliotoxicity, 148 Macromolecule synthesis, 363, 364 Magnetic resonance imaging (MRI), 326 Magnetic resonance spectroscopy (MRS), 309, 311, 323, 324, 337, 338 Maple syrup urine disease (MSUD), 120, 127 classical disease, 279, 289 intermittent form, 279 thiamine responsive form, 279 Medial prefrontal cortex, 60, 80, 8288 Melatonin, 60, 61, 63, 68, 74, 75 Melphalan, 75 Membrane potentials, 172 Memory, 365, 378, 381, 383 Mental retardation, 250, 251, 256, 281, 283, 286, 288, 290, 293295, 298, 300, 301 Mesocortical tracts, 79, 80, 82, 87, 88 Mesolimbic tracts, 79 Metabolism, 2, 3, 57, 9, 1113 Metabotropic glutamate receptor, 307 Methionine, 65, 73, 76 a-methyldopa, 78 Microdialysis, in vivo, 87 Mitochondria, 3, 5, 14, 354, 357, 359, 379, 381 Mitochondrial oxidative metabolism, 118, 119, 126 Molecular water pump, 314, 317, 340 Monoamine neurotransmitter, 74, 75, 77, 78 Monoamine oxidase (MAO), 74, 254 Multiple sclerosis, 310, 333 Muscarinic cholinergic sites, 254 NAA dynamic model, 311313, 338 NAA synthase, 308, 311, 314, 326, 336 NAAG peptidase inhibitors, 322 NAAG peptidase, 308, 309, 317, 322, 325327, 332337 NAAG synthase, 309, 326 N-acetyl cysteine, 377, 382, 383 N-acetylaspartylglutamate, 308, 309 Neurodegenerative diseases, 120, 357, 378, 385 Neuromodulator, 356, 357, 364, 365, 385 Neuron, branched chain aminotransferase isoform, 126 Neuron codes, 329 Neuron spikes, 328, 329, 331, 333, 335 Neuroprotection, 180 Neurosteroids, 253 Neurotoxicity by SAAs, 146 L-cysteine-induced, 147 L-homocysteine-induced, 147, 148 protection against, 149 S-methylcysteine-induced, 148 Neurotransmission, 358, 367, 371, 383 Neurotransmitter acetylcholine, 75 catecholamine, 6062, 70, 75, 79, 80, 81, 84, 87, 88 dopamine, 82, 85, 87, 88 norepinephrine, 6062, 70, 73, 79, 80, 8385, 87, 88 serotonin, 6064, 66, 68, 7378, 80, 8287 Neurotransmitters branched-chain amino acids and, 118 de novo glutamate synthesis, 123125 glutamate/glutamine cycle, 123 glutamate/pyruvate cycle, 125 Neurotransmitters, 158, 161, 162, 164, 169, 176 N-formylkynurenine, 6668 a7 nicotinic receptors, 68, 69 Nicotinamide adenine dinucleotide (NAD), 68, 69 Nicotine withdrawal, 85, 86 Nitric oxide synthase(NOS) inducible/induction, 101, 104, 105, 109 neuronal, 101, 105 Nitric oxide(NO), 100, 101, 103110, 255, 360, 367 Nitrosoglutathione, 362, 367, 370 NMDA receptors, 68, 252, 255 N-methyl-D-aspartate (NMDA) receptor, 358, 362, 365367, 369373

Index
Non-esteried fatty acids (NEFA), 64, 66, 75, 76 Non-ketotic hyperglycinemia, 289291 Non-NMDA receptors, 365, 367, 372, 373 Noradrenaline, stimulation of release by SAAs, 141 Norepinephrine, 60, 62, 7073, 79, 80, 8385, 87, 88 Norleucine, 73 Obesity, 66, 86 Olfactory bulb, 79 Open-loop control, 64 Ornithine, concentration in brain and CSF, 101 Ornithine decarboxylase, (ODC), 107, 109 Ornithine transcarbamylase (OTC), 251257 Osmolarity, 161 Osmoregulation, 314, 237, 340 Osmotic disequilibrium, 314, 315, 340 Ovalbumin, 62 Oxidation, 352, 360, 364, 371, 376, 379381 Oxidative stress, 352, 353, 355, 358362, 364, 368, 379382, 385 Cystine-glutamate exchanger and, 146 Pain, 69, 86 Parkinsons disease, 78, 84, 87, 88, 357, 365, 380 Peak E, 69 Peptide formation g- pseudopeptide bond, 403 b-aspartyl transfer, 403 g-glutamyl transfer, 403 peptidase effect on proteins, 403 phosphate activation and direct synthesis, 403 Peptide function co-transmitter, 402 growth control, 403, 406 hormesis, 403 neuro-modulator, 403, 404 transmitter, 403, 405 transport-regulator, 406 Peptide purication gel filtration, 404 hydrolysis and ninhydrin coloring, 404 ion exchange, 404 normal phase chromatography, 404 reverse phase chromatography, 404 UV detection, 404 Peptides and behavior growth control, 403, 406 releasing factor, 403, 404 Peptidylarginine deiminase (PAD), 101, 102, 105 Peripheral-type benzodiazepine receptors, 253 Peroxynitrite, 360, 368, 382 Pharmacology, 374376 Phenylacetate, 255, 256 Phenylalanine hydroxylase (PAH), 61, 62, 72, 73 Phenylalanine, 6063, 65, 69, 70, 72, 73, 79, 87, 88 Phenylethylamine, 72 Phenylketonuria defects of biopterin metabolism, 279, 280 phenylalanine hydroxylase deficiency, 285287 Phenylketonuria (PKU), 60 Phenylpropanolamine, 88 Phenylpyruvic acid, 72 Phophate activated glutaminase, 6, 12 Phosphorylation, 73, 77, 79, 80 p-hydroxyphenylpyruvate, 70 Pineal gland, 60 Plasma tryptophan ratio, 76, 77, 82, 86 Poikilotherms, 307, 330 Portal vascular system, 72 Potassium stimulation, 167, 168 1433 protein, 77, 78 Premenstrual syndrome, 85, 86 Putrescine, 107, 109 Pyruvate carboxylase anaplerosis, 124 glutamate/pyruvate cycle, 123, 124 Pyruvate carboxylase, 7, 8, 11, 12 Pyruvate recycling, 68 Quinolinic acid, 68, 255 Raphe nuclei, 82 Receptors g-aminobutyrate, 172, 173 Receptors glutamate, 165, 168, 169, 171, 179 Receptors glycine, 173175, 177, 179, 184 Receptors taurine, 172, 175, 176 Receptors, 358 f Reduction, 350, 352, 354, 357, 359364, 370, 379, 382, 384 Refractive period, 328, 330, 340 Regulation cell volume, 160, 165, 167 Regulatory volume decrease, 228231 Release of SAAs, 141 SAA-mediated stimulation of, 141 Release taurine, depolarization, 165, 169 Re-polarization, 328331, 333336, 338, 339 Reserpine, 87, 88 Retina degeneration, 161, 181 Retina, 79, 80, 88, 180, 181, 183, 184, 354, 356, 357, 373 Schizophrenia, 310, 322, 332, 333, 383385 Seasonal affective disorder syndrome (SADS), 86 Seizures, 177, 178, 183 Sensors, variable ratio, 82, 86 Serotonin N-acetyltransferase, 74 Serotonin, 60 f Signal transduction, 364, 365, 385 Sleep, 64, 86 S-methylcysteine, toxicity of, 148 Sodium benzoate, 255, 256 Sparse-fur mouse (spf mouse), 252, 255, 257 Spinal cord, 352, 354, 356, 357, 373, 382, 383 Splanchnic system, 63 S-sulpho-L-cysteine stimulation of excitatory receptors by, 135, 138 stimulation of noradrenaline release by, 141 Substantia nigra, 354, 356, 357 Sucrose, 62, 85 Superoxide dismutase (SOD), 69, 359, 360, 361, 379, 380, 382 Superoxide radical, 359 Swelling, 227238, 242, 243 Sympathetic nervous system, 7981, 83, 84 Synapses, 80, 84, 87 Synaptosomes, 72, 75 System L transport, 49 System N transport, 49 System y+ induction, 107 Taurine, 156 f biosynthesis, 159, 184 derivatives, 169, 182184 distribution, 156159 receptors, 172, 175, 176 TCA cycle, 211, 14 Testosterone, 61 Tetrahydrobiopterin (BH4), 72, 77, 79

417

418

Index
Thermogenesis, 88 Thermoregulation, 176, 177 Thimerosal, 149 Thiol, 349, 360, 363366, 368371, 373, 376, 379 Thyroid gland, 61 TNF-alpha, 69 Tocopherol, 360, 361, 379, 382 Transpeptidase, 352 Transport, 2, 3, 8, 9 ASC transporter, 141143 cystine-glutamate exchanger, 141, 142, 144, 145 glutamate transporter family, 142, 143 into astrocytes, 142, 144, 145 of SAAs, 141145 Transport mechanisms, 349 Transporter taurine, taurine uptake, 160162, 170, 175, 181, 184 Tricarboxylic acid cycle (TCA cycle) a-ketoglutarate, 120 Tripeptide, 349, 364, 366 Tryptophan dioxygenase (TDO), 62, 6670 Tryptophan hydroxylase 1 (TPH1), 61, 72, 77, 79 Tryptophan hydroxylase 2 (TPH2), 77, 78, 87 Tryptophan pyrrolase, 68 Tryptophan, 60 f Tuberohypophyseal neurons, 79 Tuberomamillary neurons, 51 Tyrosine aminotransferase (TAT), 62, 68, 7072 Tyrosine hydroxylase (TH), 72, 80 Tyrosine, 7983 Tyrosinemia, 71 Urea cycle, 251257 extramitochondrial enzymes, 103 Urea cycle defects arginase deficiency, 279, 298 argininosuccinic aciduria, 279, 298 carbamyl phosphate synthetase deficiency, 279, 297 citrullinemia, 279, 298 hyperammonemia, hyperornithinemia, homocitrullinuria syndrome, 279, 298 lysinuric protein intolerance, 279, 298, 299 N-acetylglutamate synthetase deciency, 279, 297 ornithine transcarbamylase deficiency, 279, 297, 298 Urokanic acid, 50 Valine, 60, 62, 64, 65, 70, 73, 87 Variable ratio sensors, 82, 86 Vitamin B12, L-Hcys and, 148 Wedensky inhibition, 329, 331, 332 Wedge preparation, 374, 376 XAG- transporters, See glutamate transporter family Xanthurenic acid, 68 Xcexchanger, See cystine-glutamate exchanger Xenobiotics, 350, 362, 363, 379