Page last updated: 2024-09-02

valerates and glycine

valerates has been researched along with glycine in 100 studies

Compound Research Comparison

Studies
(valerates)
Trials
(valerates)
Recent Studies (post-2010)
(valerates)
Studies
(glycine)
Trials
(glycine)
Recent Studies (post-2010) (glycine)
2,25129445735,1637568,415

Protein Interaction Comparison

ProteinTaxonomyvalerates (IC50)glycine (IC50)
Glutamate receptor ionotropic, NMDA 1 Rattus norvegicus (Norway rat)1.4852
Sodium- and chloride-dependent glycine transporter 1Homo sapiens (human)106
Glutamate receptor ionotropic, NMDA 2A Rattus norvegicus (Norway rat)0.3253
Glutamate receptor ionotropic, NMDA 2BRattus norvegicus (Norway rat)0.3253
Glutamate receptor ionotropic, NMDA 2CRattus norvegicus (Norway rat)0.3253
Glutamate receptor ionotropic, NMDA 2DRattus norvegicus (Norway rat)0.3253
Glutamate receptor ionotropic, NMDA 3BRattus norvegicus (Norway rat)0.3253
Glutamate receptor ionotropic, NMDA 3ARattus norvegicus (Norway rat)0.3253
Sodium- and chloride-dependent glycine transporter 2Homo sapiens (human)128

Research

Studies (100)

TimeframeStudies, this research(%)All Research%
pre-199085 (85.00)18.7374
1990's3 (3.00)18.2507
2000's10 (10.00)29.6817
2010's2 (2.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Horton, RW1
Tanaka, K2
Carchon, H; Casaer, P; Corbeel, L; Eeckels, R; Eggermont, E; Jaeken, J1
Bartlett, K1
Wolf, B1
Debray-Ritzen, P; Kamoun, P; Parvy, P1
Kølvraa, S1
Constanti, A1
Horton, RW; Meldrum, BS1
Cohn, RM; Puschak, R; Rothman, R; Segal, S; Yudkoff, M1
Cohn, RM; Rothman, R; Segal, S; Yudkoff, M1
O'Brien, WE1
Beccaria, L; Chiumello, G; Gianazza, E; Luppis, B; Righetti, PG1
Ehinger, B1
Mandell, R; Shih, VE; Tanaka, K1
Kelly, S; Leikhim, E1
Krieger, I; Tanaka, K1
Dembure, P; Elsas, L; Madsen, K; Naglak, M; Salvo, R1
Holme, E; Jacobson, CE; Kristiansson, B1
Bachmann, C; Dionisi-Vici, C; Graziani, MC; Sabetta, G1
Elsas, LJ; Naglak, M1
Mock, DM; Mock, NI; Weintraub, S1
Hagenfeldt, L; Naglo, AS1
Goodman, SI; Hack, AM; Hine, DG; Tanaka, K1
Bartlett, K; Cannon, RA; Conde, C; Gomez Vazquez, J; Lipson, M; Middleton, B; Nyhan, WL; Romanos, A; Sweetman, L1
Inoue, F; Maltby, DA; Millington, DS; Roe, CR1
Baker, H; Baswell, DL; Holman, RT; Mock, DM; Sweetman, L1
Bradley, D; Chalmers, RA; de Sousa, C; Stacey, TE; Tracey, BM; Weaver, C1
Bradley, D; Chalmers, RA; de Sousa, C; Stacey, TE; Tracey, BM; Weaver, CM1
Hynes, MJ; Pateman, JA1
Draffan, GH; Gompertz, D; Hull, D; Watts, JL1
Erickson, AM; Kurtz, DJ; Levy, HL; Lott, IT1
Biscoe, TJ; Duggan, AW; Lodge, D1
Bohunicky, L; Cerny, V; Halko, J; Koza, I; Krizan, Z; Petrek, C; Poliakova, L1
Johns, RB; Looney, FD; Whelan, DJ1
De Groat, WC; Lalley, PM; Saum, WR1
Kimura, K1
George, JM; Kier, LB1
Ikeda, K; Roberts, E; Swagel, MW1
Koda, A; Mori, H; Nagai, H; Watanabe, S1
Eldjarn, L; Jellum, E; Stokke, O1
Drobník, J; Spacek, P; Wichterle, O1
Kokkonen, H; Mäntsälä, P; Nurmikko, V1
Shigesada, K1
Capek, A; Cerný, A; Semonský, M1
Ando, T; Bachmann, C; Nyhan, WL; Rasmussen, K; Scott, R; Smith, EK1
Daum, RS; Delvin, E; Goldman, H; Lamm, P; Mamer, OA; Scriver, CR1
Beart, PM; Johnston, GA1
Kean, EA; Rainford, IJ1
Evered, DF; Hargreaves, BM1
Bartlett, K; Blair, D; Gompertz, D; Stern, CM1
Bartlett, K; Gompertz, D1
Ajl, SJ; Rabin, R; Reeves, HC; Wegener, WS1
Koga, T; Ressler, C1
Barker, HA; Winnacker, EL1
Folk, JE; Gross, M1
Isselbacher, KJ; Shih, V; Tanaka, K1
Prescott, JM; Wagner, FW; Wilkes, SH1
Draffan, GH; Gompertz, D1
Eldjarn, L; Jellum, E; Pande, H; Stokke, O; Waaler, PE1
Barrio, JR; Domínguez, JM; Gallina, DL; Hoschoian, JC1
Mády, G; Moravcsik, E; Otvös, L1
Cerný, V1
Reisener, HJ1
Cook, ES; Nutini, LG; Tanaka, K; Tsuchiya, Y1
Austic, RE; Nesheim, MC1
Boyarsky, LL; Wheeler, DD1
Engberg, I; Ten Bruggencate, G1
Behki, RM; Hochster, RM1
Ghadially, FN; Neame, KD1
Prieto, EC; Velazquez, A1
Audenaert, SM; Squillaro, EJ; Wilson, WG1
Hine, DG; Tanaka, K2
Bohan, TP; Kahler, SG; Maltby, DA; Millington, DS; Roe, CR1
Bartlett, K; Leonard, JV; Ng, H1
Batenburg-Plenter, AM; Bruinvis, L; Duran, M; Ketting, D; van Pelt, BC; Wadman, SK1
Burnham, BF; Kushner, JP; Morton, KA; Straka, JG1
Gregersen, N; Hobolth, N; Kølvraa, S; Lauritzen, R; Rasmussen, K; Rosleff, F1
Burnham, BF; Horton, WJ; Kushner, JP; Morton, KA1
Inoue, Y; Kameyama, J; Momoi, T; Shigematsu, Y; Sudo, M; Suzuki, Y1
Bakkeren, JA; Houben, ML; Ruitenbeek, W; Sengers, RC; Trijbels, JM; Van der Zeè, SP1
Al-Bassam, SS; Sherratt, HS1
Dahlberg, L; Schuberth, J1
Berry, SA; Nyhan, WL; Thuy, LP; Tuchman, M1
Berry, SA; Gage, DA; Huang, ZH; Iwamoto, T; Johnston, K; Millington, DS; Packman, S; Röschinger, W; Sweetman, L; Yano, S1
Bakkeren, J; Baumgartner, R; de Jong, J; Mourmans, J; Suormala, T; van Diggelen, OP; Wendel, U; Wevers, R1
Almashanu, S; Baumgartner, ER; Baumgartner, MR; Dantas, MF; Fowler, B; Friebel, D; Gebhardt, B; Giunta, C; Hoffmann, GF; Suormala, T; Valle, D1
Andresen, BS; Boneh, A; Gutman, A; Korman, SH; Pitt, JJ; Zeharia, A1
Baumgartner, MR; Baykal, T; Can, G; Dantas, MF; Demir, F; Demirkol, M; Fowler, B; Gokcay, GH; Ince, Z1
Barea, L; Baumgartner, MR; Dantas, MF; Fowler, B; Giugliani, R; Graziadio, C; Paskulin, G; Perla, A; Pinto, L; Rosa, R; Vargas, C; Wajner, M; Zen, P1
Yoon, HR1
Andresen, BS; Engel, K; Ensenauer, R; Häberle, J; Lehnert, W; Mégarbané, A; Reich, H; Röschinger, W; Sass, JO; Schirrmacher, O; Steuerwald, U; Zschocke, J1
Baldellou, A; Briones, P; Campistol, J; Cortés, N; Delpiccolo, C; Fernández, C; Fons, C; Fuentes-Castelló, MA; García-Villoria, J; González, I; Hernández-Gonzalez, A; Messeguer, A; Navarro-Sastre, A; Pérez-Cerdá, C; Ribes, A1
Calado, AR; Faustino, CM; Garcia-Rio, L1
Baumgartner, MR; Biberoglu, G; Demir, E; Eminoglu, FT; Hasanoglu, A; Okur, I; Ozcelik, AA; Tumer, L1
Amaral, AU; Beskow, AP; da Silva, Lde B; Fernandes, CG; Leipnitz, G; Ribeiro, CA; Seminotti, B; Vargas, CR; Wajner, M; Zanatta, A1
Choi, JH; Jung, CW; Kim, GH; Kim, JH; Lee, BH; Lee, J; Yoo, HW1
Afroze, B; Ali, ZZ; Fatimah, M; Jafri, L; Jamil, A; Khan, AH; Majid, H; Yusufzai, N1

Reviews

1 review(s) available for valerates and glycine

ArticleYear
Alternate pathways of metabolism of short-chain fatty acids.
    Bacteriological reviews, 1968, Volume: 32, Issue:1

    Topics: Acetates; Acetoacetates; Carbon Dioxide; Escherichia coli; Fatty Acids; Glutarates; Glycine; Glyoxylates; Ketoglutaric Acids; Lyases; Malates; Mutation; Oxaloacetates; Propionates; Pyruvates; Valerates

1968

Other Studies

99 other study(ies) available for valerates and glycine

ArticleYear
The role of 2-keto-4-pentenoic acid in seizures induced by allylglycine.
    Biochemical pharmacology, 1978, May-15, Volume: 27, Issue:10

    Topics: Animals; Brain; Glutamate Decarboxylase; Glycine; Hydrogen-Ion Concentration; Keto Acids; Kinetics; Mice; Pentanoic Acids; Seizures; Time Factors; Valerates

1978
Ackee poisoning.
    Lancet (London, England), 1977, Feb-12, Volume: 1, Issue:8007

    Topics: Animals; Butyrates; Dicarboxylic Acids; Foodborne Diseases; Fruit; Glycine; Humans; Hypoglycins; Rats; Valerates

1977
Dipropylacetate (valproate) and glycine metabolism.
    Lancet (London, England), 1977, Sep-17, Volume: 2, Issue:8038

    Topics: Epilepsy; Glycine; Humans; Infant; Valerates; Valproic Acid

1977
Sodium valproate and hyperglycinaemia.
    Lancet (London, England), 1977, Oct-01, Volume: 2, Issue:8040

    Topics: Acetyltransferases; Glycine; Valerates; Valproic Acid

1977
Dipropylacetate and propionyl CoA carboxylase.
    Lancet (London, England), 1978, Aug-12, Volume: 2, Issue:8085

    Topics: Carboxy-Lyases; Glycine; Humans; Valerates; Valproic Acid

1978
[Hyperglycinuria induced by n-dipropylacetate. Possible model of propionic acidemia].
    La Nouvelle presse medicale, 1977, Jun-11, Volume: 6, Issue:24

    Topics: Child; Child, Preschool; Glycine; Humans; Infant; Metabolic Diseases; Models, Biological; Propionates; Valerates; Valproic Acid

1977
Inhibition of the glycine cleavage system by branched-chain amino acid metabolites.
    Pediatric research, 1979, Volume: 13, Issue:8

    Topics: Amino Acids, Branched-Chain; Animals; Butyrates; Coenzyme A; Glycine; Mitochondria, Liver; Rats; Valerates

1979
The GABA dose/conductance relationship on lobster muscle.
    Journal de physiologie, 1979, Volume: 75, Issue:6

    Topics: Amino Acids; Amino Acids, Neutral; Animals; Bicuculline; Dose-Response Relationship, Drug; Electric Conductivity; GABA Antagonists; gamma-Aminobutyric Acid; Glycine; Guanidines; In Vitro Techniques; Muscles; Nephropidae; Picrotoxin; Piperazines; Propionates; Valerates

1979
A comparison of the effects of allylglycine and 2-keto-4-pentenoic acid on cerebral glutamic acid decarboxylase activity and convulsions in mice [proceedings].
    British journal of pharmacology, 1977, Volume: 61, Issue:3

    Topics: Animals; Brain; Carboxy-Lyases; Glutamate Decarboxylase; Glycine; Keto Acids; Mice; Pentanoic Acids; Seizures; Valerates

1977
Glycine therapy in isovaleric acidemia.
    The Journal of pediatrics, 1978, Volume: 92, Issue:5

    Topics: Acidosis; Administration, Oral; Amino Acid Metabolism, Inborn Errors; Child, Preschool; Drug Administration Schedule; Female; Glycine; Humans; Keto Acids; Ketone Bodies; Lactates; Leucine; Pyruvates; Valerates

1978
Isovaleric acidemia: use of glycine therapy in neonates.
    The New England journal of medicine, 1978, Nov-02, Volume: 299, Issue:18

    Topics: Amino Acid Metabolism, Inborn Errors; Female; Glycine; Humans; Infant, Newborn; Male; Time Factors; Valerates

1978
Inhibition of glycine synthase by branched-chain alpha-keto acids. A possible mechanism for abnormal glycine metabolism in ketotic hyperglycinemia.
    Archives of biochemistry and biophysics, 1978, Volume: 189, Issue:2

    Topics: Acidosis; Acyl Coenzyme A; Animals; Caproates; Glycine; Keto Acids; Ketosis; Liver; Male; Rats; Transferases; Valerates

1978
Hemoglobin A1C separation by isoelectric focusing.
    American journal of hematology, 1978, Volume: 4, Issue:4

    Topics: Alanine; Aminocaproic Acid; Diabetes Mellitus, Type 1; Glycine; Hemoglobin A; Histidine; Humans; Isoelectric Focusing; Proline; Threonine; Valerates

1978
Swelling of rabbit retinal amacrines in response to omega-amino acids.
    Brain research, 1977, Apr-08, Volume: 125, Issue:1

    Topics: Alanine; Amino Acids; Animals; gamma-Aminobutyric Acid; Glutamates; Glycine; Rabbits; Retina; Taurine; Valerates; Valine

1977
Metabolism of [1-(14)C] and [2-(14)C] leucine in cultured skin fibroblasts from patients with isovaleric acidemia. Characterization of metabolic defects.
    The Journal of clinical investigation, 1976, Volume: 58, Issue:1

    Topics: 2-Methyl-4-chlorophenoxyacetic Acid; Amino Acid Metabolism, Inborn Errors; Carbon Dioxide; Cell Line; Cells, Cultured; Depression, Chemical; Fibroblasts; Flavin-Adenine Dinucleotide; Glycine; Humans; Leucine; Maple Syrup Urine Disease; Oxidation-Reduction; Skin; Succinates; Valerates; Valine

1976
Isovalerylglycine detection by thin-layer chromatography: additional observations.
    Clinica chimica acta; international journal of clinical chemistry, 1975, Mar-10, Volume: 59, Issue:2

    Topics: Child; Chromatography, Gas; Chromatography, Thin Layer; Evaluation Studies as Topic; Glycine; Humans; Hydroxybutyrates; Ketosis; Male; Methods; Valerates

1975
Therapeutic effects of glycine in isovaleric acidemia.
    Pediatric research, 1976, Volume: 10, Issue:1

    Topics: Acidosis; Alkalies; Amino Acid Metabolism, Inborn Errors; Child; Female; Glycine; Humans; Inactivation, Metabolic; Ketosis; Oxidoreductases; Valerates

1976
The treatment of isovaleric acidemia with glycine supplement.
    Pediatric research, 1988, Volume: 24, Issue:1

    Topics: Carbon Dioxide; Cells, Cultured; Child; Child, Preschool; Female; Fibroblasts; Glycine; Hemiterpenes; Humans; Isovaleryl-CoA Dehydrogenase; Leucine; Male; Oxidoreductases; Oxidoreductases Acting on CH-CH Group Donors; Pentanoic Acids; Valerates

1988
Biotin-responsive multiple carboxylase deficiency in an 8-year-old boy with normal serum biotinidase and fibroblast holocarboxylase-synthetase activities.
    Journal of inherited metabolic disease, 1988, Volume: 11, Issue:3

    Topics: Amidohydrolases; Biotin; Biotinidase; Carbon-Nitrogen Ligases; Child; Fibroblasts; Glycine; Humans; Kinetics; Ligases; Male; Multiple Carboxylase Deficiency; Valerates

1988
Laryngeal stridor as a leading symptom in a biotinidase-deficient patient.
    Journal of inherited metabolic disease, 1988, Volume: 11, Issue:3

    Topics: Amidohydrolases; Biotinidase; Female; Glycine; Humans; Infant; Lactates; Larynx; Propionates; Valerates

1988
Acute and chronic-intermittent isovaleric acidemia: diagnosis and glycine therapy.
    Acta paediatrica Japonica : Overseas edition, 1988, Volume: 30, Issue:4

    Topics: Acute Disease; Child; Child, Preschool; Chronic Disease; Female; Glycine; Hemiterpenes; Humans; Isovaleryl-CoA Dehydrogenase; Leucine; Male; Oxidoreductases; Oxidoreductases Acting on CH-CH Group Donors; Pentanoic Acids; Valerates

1988
Abnormal organic aciduria in biotin deficiency: the rat is similar to the human.
    The Journal of laboratory and clinical medicine, 1988, Volume: 112, Issue:2

    Topics: Animals; Biotin; Disease Models, Animal; Gas Chromatography-Mass Spectrometry; Glycine; Male; Random Allocation; Rats; Rats, Inbred Strains; Valerates

1988
New conjugated urinary metabolites in intermediate type maple syrup urine disease.
    Clinica chimica acta; international journal of clinical chemistry, 1987, Oct-30, Volume: 169, Issue:1

    Topics: Amino Acids; Amino Acids, Branched-Chain; Carboxylic Acids; Female; Gas Chromatography-Mass Spectrometry; Glycine; Humans; Infant; Lactates; Lactic Acid; Maple Syrup Urine Disease; Valerates

1987
Stable isotope dilution analysis of isovalerylglycine in amniotic fluid and urine and its application for the prenatal diagnosis of isovaleric acidemia.
    Pediatric research, 1986, Volume: 20, Issue:3

    Topics: Amino Acid Metabolism, Inborn Errors; Amniotic Fluid; Female; Gas Chromatography-Mass Spectrometry; Glycine; Hemiterpenes; Humans; Pentanoic Acids; Pregnancy; Prenatal Diagnosis; Risk; Valerates

1986
3-Ketothiolase deficiency.
    European journal of pediatrics, 1986, Volume: 144, Issue:6

    Topics: 3-Hydroxybutyric Acid; Acetyl Coenzyme A; Acetyl-CoA C-Acyltransferase; Acyl Coenzyme A; Acyltransferases; Child; Female; Fibroblasts; Glycine; Humans; Hydroxybutyrates; Infant; Isoleucine; Ketosis; Male; Valerates

1986
Endogenous catabolism is the major source of toxic metabolites in isovaleric acidemia.
    The Journal of pediatrics, 1987, Volume: 110, Issue:1

    Topics: Amino Acid Metabolism, Inborn Errors; Carnitine; Dietary Proteins; Glycine; Humans; Isovaleryl-CoA Dehydrogenase; Leucine; Male; Oxidoreductases; Oxidoreductases Acting on CH-CH Group Donors; Valerates

1987
Biotin deficiency complicating parenteral alimentation: diagnosis, metabolic repercussions, and treatment.
    The Journal of pediatrics, 1985, Volume: 106, Issue:5

    Topics: Alopecia; Biotin; Citrates; Diagnosis, Differential; Erythema; Fatty Acids, Essential; Female; Glycine; Humans; Infant; Isomerism; Lactates; Lactic Acid; Male; Nervous System Diseases; Parenteral Nutrition; Valerates; Zinc

1985
L-carnitine and glycine therapy in isovaleric acidaemia.
    Journal of inherited metabolic disease, 1985, Volume: 8 Suppl 2

    Topics: Amino Acid Metabolism, Inborn Errors; Carnitine; Child, Preschool; Female; Glycine; Hemiterpenes; Humans; Pentanoic Acids; Valerates

1985
The response to L-carnitine and glycine therapy in isovaleric acidaemia.
    European journal of pediatrics, 1986, Volume: 144, Issue:5

    Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Carboxylic Acids; Carnitine; Child, Preschool; Combined Modality Therapy; Drug Therapy, Combination; Female; Glycine; Hemiterpenes; Humans; Pentanoic Acids; Valerates

1986
The use of amides as nitrogen sources by Aspergillus nidulans.
    Journal of general microbiology, 1970, Volume: 63, Issue:3

    Topics: Acetamides; Acrylates; Amides; Amidohydrolases; Arylformamidase; Aspergillus; Barbiturates; Butyrates; Carbamates; Enzyme Induction; Formamides; Fumarates; Glycine; Glycolates; Lactates; Malonates; Niacinamide; Nitrogen; Phenylacetates; Propionates; Valerates

1970
Biotin-responsive beta-methylcrotonylglycinuria.
    Lancet (London, England), 1971, Jul-03, Volume: 2, Issue:7714

    Topics: Acidosis; Administration, Oral; Amino Acid Metabolism, Inborn Errors; Biotin; Butyrates; Carbon Dioxide; Carboxy-Lyases; Chromatography, Gas; Fatty Acids; Glycine; Humans; Infant; Leucine; Male; Partial Pressure; Valerates; Valine; Vomiting

1971
Isovaleric acidemia: results of family study and dietary treatment.
    Pediatrics, 1973, Volume: 52, Issue:1

    Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Child; Child, Preschool; Developmental Disabilities; Diet Therapy; Fatty Acids; Female; Glycine; Humans; Infant, Newborn; Intellectual Disability; Intelligence Tests; Leucine; Male; Odorants; Pedigree; Valerates

1973
Antagonism between bicuculline, strychnine, and picrotoxin and depressant amino-acids in the rat nervous system.
    Comparative and general pharmacology, 1972, Volume: 3, Issue:12

    Topics: Alanine; Alkaloids; Amino Acids; Aminobutyrates; Animals; Central Nervous System; Central Nervous System Stimulants; Cerebral Cortex; Dioxoles; Drug Antagonism; Electric Stimulation; Glycine; In Vitro Techniques; Isoquinolines; Picrotoxin; Rats; Receptors, Drug; Sciatic Nerve; Spinal Cord; Strychnine; Valerates

1972
Treatment of the blastic phase of chronic myeloid leukaemia. A question to solve.
    Haematologica, 1972, Volume: 57, Issue:11

    Topics: Adult; Antineoplastic Agents; Child; Cytarabine; Glycine; Humans; Hydroxyurea; Leukemia, Myeloid; Purines; Remission, Spontaneous; Sulfides; Valerates; Vincristine

1972
Photochemistry of biological molecules. I. Photolysis of amino acids in the solid state.
    Biochimica et biophysica acta, 1967, Oct-23, Volume: 147, Issue:2

    Topics: Alanine; Amino Acids; Aminobutyrates; Aminoisobutyric Acids; Crystallization; Deuterium; Electron Spin Resonance Spectroscopy; Free Radicals; Glycine; Isoleucine; Radiation; Radiation Effects; Ultraviolet Rays; Valerates; Valine

1967
Depolarization of dorsal root ganglia in the cat by GABA and related amino acids: antagonism by picrotoxin and bicuculline.
    Brain research, 1972, Sep-15, Volume: 44, Issue:1

    Topics: Action Potentials; Alanine; Alkaloids; Aminobutyrates; Animals; Cats; Ganglia, Spinal; Glutamates; Glycine; Isoquinolines; Neural Inhibition; Picrotoxin; Spinal Nerve Roots; Strychnine; Sulfonic Acids; Synaptic Transmission; Valerates

1972
[Electroencephalography studies on the actions of hypnotic agents. 2. effects of various hypnotic agents on electrical activity of the lower brain stem of rabbits].
    Nihon yakurigaku zasshi. Folia pharmacologica Japonica, 1973, Volume: 69, Issue:4

    Topics: Amides; Animals; Brain Stem; Bromine; Electric Stimulation; Electrodes, Implanted; Electroencephalography; Evoked Potentials; Eye Movements; Glycine; Hippocampus; Hypnotics and Sedatives; Male; Pentobarbital; Phthalic Acids; Rabbits; Reserpine; Sleep; Sleep Stages; Sleep, REM; Urea; Valerates

1973
Molecular orbital studies of the conformation around the 5 position in angiotensin II.
    Journal of theoretical biology, 1974, Volume: 46, Issue:1

    Topics: Alanine; Aminobutyrates; Angiotensin II; Chemical Phenomena; Chemistry, Physical; Cyclohexanes; Cyclopentanes; Glycine; Isoleucine; Molecular Conformation; Norleucine; Valerates; Valine

1974
Effects of GABA, imidazoleacetic acid, and related substances on conductance of crayfish abdominal stretch receptor.
    Nature: New biology, 1973, Nov-21, Volume: 246, Issue:151

    Topics: Acetates; Alanine; Aminobutyrates; Aminocaproates; Animals; Astacoidea; Electric Conductivity; Glycine; Imidazoles; Mechanoreceptors; Valerates

1973
Proceedings: Effect of drugs on antibody formation. VII. Tumor immunity.
    Japanese journal of pharmacology, 1974, Volume: 24, Issue:0

    Topics: Amino Acids, Sulfur; Animals; Antibody Formation; Glycine; Mice; Neoplasms, Experimental; Valerates

1974
Combined use of gas chromatography, mass spectrometry, and computer in diagnosis and studies of metabolic disorders.
    Clinical chemistry, 1972, Volume: 18, Issue:8

    Topics: Adult; Biopsy; Blood Chemical Analysis; Butyrates; Carbon Dioxide; Chemistry, Clinical; Chromatography, Gas; Computers; Diagnosis, Computer-Assisted; Female; Glycine; Humans; Infant; Infant, Newborn; Ligases; Male; Malonates; Mass Spectrometry; Metabolic Diseases; Metabolism, Inborn Errors; Methods; Pentanols; Pyrrolidonecarboxylic Acid; Valerates

1972
Diffusion of anti-tumor drugs through membranes from hydrophilic methacrylate gels.
    Journal of biomedical materials research, 1974, Volume: 8, Issue:1

    Topics: Acrylates; Acrylic Resins; Antineoplastic Agents; Diffusion; Fluorouracil; Glycine; Membranes, Artificial; Mercaptopurine; Methacrylates; Polyhydroxyethyl Methacrylate; Purines; Sulfides; Tegafur; Valerates

1974
On the regulation of the formation of the pantothenate transport system during the growth of Pseudomonas fluorescens P-2.
    Acta chemica Scandinavica, 1972, Volume: 26, Issue:1

    Topics: Biological Transport, Active; Carbon Isotopes; Citric Acid Cycle; Densitometry; Formates; Glycine; Glycolates; Glyoxylates; Malates; Pantothenic Acid; Pseudomonas; Pseudomonas fluorescens; Time Factors; Valerates

1972
Possible occurrence of a succinate-glycine cycle in Rhodospirillum rubrum.
    Journal of biochemistry, 1972, Volume: 71, Issue:6

    Topics: Amino Acids; Anaerobiosis; Carbon Isotopes; Cell-Free System; Chromatography; Esterases; Glutamates; Glutarates; Glycine; Levulinic Acids; Lyases; Oxidoreductases; Photosynthesis; Rhodospirillum; Rhodospirillum rubrum; Succinates; Valerates

1972
[Antineoplastic agents. 48. Microbial catabolites of the ethyl esters of N-( -(6-purinylithio)-valeryl)-glycine, -diglycine and triglycine and their corresponding acids].
    Die Pharmazie, 1972, Volume: 27, Issue:5

    Topics: Amino Acids; Amino Alcohols; Antineoplastic Agents; Aspergillus nidulans; Bacillus cereus; Biotransformation; Chromatography, Paper; Chromatography, Thin Layer; Escherichia coli; Esters; Ethanol; Glycine; Purines; Sulfides; Valerates

1972
On the mode of action of hypoglycin A. 3. Isolation and identification of cis-4-decene-1,10-dioic, cis, cis-4,7-decadiene-1,10-dioic, cis-4-octene-1,8-dioic, glutaric, and adipic acids, N-(methylenecyclopropyl)acetylglycine, and N-isovalerylglycine from u
    The Journal of biological chemistry, 1972, Dec-10, Volume: 247, Issue:23

    Topics: Acetates; Adipates; Alanine; Animals; Carbon Isotopes; Chromatography, Gas; Chromatography, Thin Layer; Coenzyme A; Cyclopropanes; Dicarboxylic Acids; Dipeptides; Fatty Acids, Unsaturated; Glutarates; Glycine; Magnetic Resonance Spectroscopy; Male; Oxidoreductases; Rats; Valerates

1972
Isovaleric acidemia: identification of isovalerate, isovalerylglycine, and 3-hydroxyisovalerate in urine of a patient previously reported as having butyric and hexanoic acidemia.
    The Journal of pediatrics, 1973, Volume: 82, Issue:2

    Topics: Butyrates; Caproates; Child; Chromatography, Gas; Chromatography, Thin Layer; Coenzyme A; Glycine; Humans; Mass Spectrometry; Metabolism, Inborn Errors; Valerates

1973
An inherited disorder of isoleucine catabolism causing accumulation of alpha-methylacetoacetate and alpha-methyl-beta -hydroxybutyrate, and intermittent metabolic acidosis.
    Pediatric research, 1973, Volume: 7, Issue:3

    Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Butyrates; Cells, Cultured; Child; Child, Preschool; Chromatography, Gas; Chromatography, Paper; Chromatography, Thin Layer; Female; Fibroblasts; Glycine; Humans; Hydroxybutyrates; Infant; Isoleucine; Keto Acids; Ketones; Male; Mass Spectrometry; Mutation; Pedigree; Phenotype; Skin; Valerates

1973
GABA uptake in rat brain slices: inhibition by GABA analogues and by various drugs.
    Journal of neurochemistry, 1973, Volume: 20, Issue:2

    Topics: Acetylation; Acylation; Aminobutyrates; Animals; Aspartic Acid; Betaine; Brain; Brain Chemistry; Chlorine; Choline; Esters; Fatty Acids; Fluorine; Glutamates; Glycine; Hydroxylation; Imidazoles; In Vitro Techniques; Methylation; Rats; Sulfur; Tritium; Valerates

1973
Evidence of multiple and independent mechanisms for the action of hypoglycin in vivo.
    Biochemical pharmacology, 1973, Jun-15, Volume: 22, Issue:12

    Topics: Alanine; Animals; Blood Glucose; Body Weight; Carbon Isotopes; Chromatography, Thin Layer; Coenzyme A; Cyclopropanes; Esters; Fasting; Glycine; Male; Oxidation-Reduction; Oxidoreductases; Rats; Valerates

1973
Metabolism of omega-amino acids by rat tissues in vitro.
    Xenobiotica; the fate of foreign compounds in biological systems, 1973, Volume: 3, Issue:4

    Topics: Alanine; Amino Acids; Aminobutyrates; Aminocaproates; Aminoisobutyric Acids; Animals; Brain; Caprylates; Cyclohexanecarboxylic Acids; Glycine; Intestinal Mucosa; Kidney; Kinetics; Liver; Male; Methionine; Pyridoxal Phosphate; Rats; Schiff Bases; Spectrophotometry, Ultraviolet; Valerates; Valine

1973
Child with a defect in leucine metabolism associated with beta-hydroxyisovaleric aciduria and beta-methylcrotonylglycinuria.
    Archives of disease in childhood, 1973, Volume: 48, Issue:12

    Topics: Amino Acid Metabolism, Inborn Errors; Biotin; Creatinine; Crotonates; Glycine; Humans; Infant; Leucine; Male; Pentanols; Pneumonia; Valerates

1973
The specificity of glycine-N-acylase and acylglycine excretion in the organicacidaemias.
    Biochemical medicine, 1974, Volume: 10, Issue:1

    Topics: Acetyl Coenzyme A; Acyltransferases; Amino Acid Metabolism, Inborn Errors; Animals; Benzoates; Butyrates; Cattle; Chromatography, Gel; Chromatography, Ion Exchange; Computers; Crotonates; Glycine; Kinetics; Malonates; Mitochondria, Liver; Propionates; Regression Analysis; Spectrophotometry; Spectrophotometry, Ultraviolet; Valerates

1974
-cyanoamino acids and related nitriles as inhibitors of glutamate decarboxylase.
    Biochimica et biophysica acta, 1971, Aug-20, Volume: 242, Issue:2

    Topics: Acetates; Amino Acids; Aminobutyrates; Animals; Carbon Isotopes; Carboxy-Lyases; Chickens; Cyanides; Depression; Escherichia coli; Glutamate Dehydrogenase; Glutarates; Glycine; Humans; Hypnotics and Sedatives; Infrared Rays; Kinetics; Nitriles; Propionates; Pyridoxal Phosphate; Seizures; Spectrophotometry; Structure-Activity Relationship; Succinates; Valerates

1971
Aerobic metabolism of -amino-n-butyric acid by Pseudomonas putida.
    Biochimica et biophysica acta, 1971, May-18, Volume: 237, Issue:2

    Topics: Acetoacetates; Acyltransferases; Aerobiosis; Alanine; Amines; Aminobutyrates; Aminocaproates; Aminoisobutyric Acids; Cell-Free System; Chromatography, Paper; Coenzyme A; Deamination; Electrophoresis; Glycine; Glyoxylates; Isocitrate Dehydrogenase; Lyases; Lysine; Malates; Models, Biological; Paper; Pseudomonas; Pyruvates; Spectrophotometry; Stereoisomerism; Succinates; Transaminases; Valerates

1971
Mechanism of action of guinea pig liver transglutaminase. 8. Active site studies with "reporter" group-labeled halomethyl ketones.
    The Journal of biological chemistry, 1971, Volume: 246, Issue:21

    Topics: Acetophenones; Acyltransferases; Animals; Binding Sites; Bromine; Calcium Chloride; Carbon Isotopes; Catalysis; Chemical Phenomena; Chemistry; Chromatography, Thin Layer; Edetic Acid; Glutamine; Glycine; Guinea Pigs; Kinetics; Liver; Mathematics; Nitrophenols; Protein Binding; Spectrophotometry; Stereoisomerism; Sulfhydryl Compounds; Ultraviolet Rays; Valerates

1971
Isovaleric and -methylbutyric acidemias induced by hypoglycin A: mechanism of Jamaican vomiting sickness.
    Science (New York, N.Y.), 1972, Jan-07, Volume: 175, Issue:4017

    Topics: Animals; Blood Glucose; Butyrates; Cyclopropanes; Fruit; Glutarates; Glycine; Leucine; Male; Plant Extracts; Rats; Valerates

1972
Specificity of Aeromonas aminopeptidase toward amino acid amides and dipeptides.
    The Journal of biological chemistry, 1972, Feb-25, Volume: 247, Issue:4

    Topics: Aeromonas; Alanine; Amides; Amino Acids; Aminocaproates; Aminopeptidases; Dipeptides; Glycine; Isoleucine; Kinetics; Leucine; Methionine; Phenylalanine; Structure-Activity Relationship; Tryptophan; Tyrosine; Valerates; Valine

1972
The identification of tiglylglycine in the urine of a child with -methylcrotonylglycinuria.
    Clinica chimica acta; international journal of clinical chemistry, 1972, Volume: 37

    Topics: Amino Acid Metabolism, Inborn Errors; Butyrates; Child; Chromatography, Gas; Chromatography, Thin Layer; Coenzyme A; Diet Therapy; Dietary Proteins; Glycine; Humans; Hydro-Lyases; Male; Mass Spectrometry; Valerates

1972
Beta-methylcrotonyl-CoA carboxylase deficiency: a new metabolic error in leucine degradation.
    Pediatrics, 1972, Volume: 49, Issue:5

    Topics: Amino Acid Metabolism, Inborn Errors; Biotin; Bronchopneumonia; Butyrates; Carbon Dioxide; Carbon-Carbon Ligases; Chromatography, Gas; Diet Therapy; Female; Glycine; Humans; Infant; Leucine; Ligases; Mass Spectrometry; Neurologic Manifestations; Odorants; Pentanols; Valerates

1972
Maintenance of nitrogen balance in a young woman by substitution of -ketoisovaleric acid for valine.
    The Journal of nutrition, 1971, Volume: 101, Issue:9

    Topics: Adolescent; Amino Acids; Body Weight; Citrates; Diet; Dietary Proteins; Eggs; Feces; Female; Glycine; Humans; Keto Acids; Metabolism; Nitrogen; Quaternary Ammonium Compounds; Stimulation, Chemical; Valerates; Valine

1971
Investigation on the mechanism of acylase-I-catalyzed acylamino acid hydrolysis.
    Biochemical and biophysical research communications, 1971, Volume: 44, Issue:5

    Topics: Acetates; Amidohydrolases; Amino Acids; Benzoates; Bromine; Catalysis; Chlorine; Ethers; Glycine; Kinetics; Mathematics; Nitrobenzenes; Phenylacetates; Propionates; Structure-Activity Relationship; Valerates

1971
Preliminary clinical trial with a purine analogue butocine.
    Neoplasma, 1971, Volume: 18, Issue:5

    Topics: Antineoplastic Agents; Bronchial Neoplasms; Carcinoma, Bronchogenic; Drug Tolerance; Glycine; Humans; Leukemia; Leukemia, Myeloid; Leukemia, Myeloid, Acute; Mercaptopurine; Purines; Sulfides; Valerates

1971
[The metabolism of alanin, glycine and arginine in uredospores of Puccinia graminis var. tritici during germination].
    Archiv fur Mikrobiologie, 1969, Volume: 69, Issue:2

    Topics: Alanine; Amino Acids; Arginine; Basidiomycota; Carbon Dioxide; Carbon Isotopes; Chromatography, Paper; Glucose; Glycine; Guanidines; Spores; Valerates

1969
Effects of omega-amino acids and related compounds on staphylococcal infections in mice: a combined prophylactic-therapeutic procedure.
    Applied microbiology, 1970, Volume: 19, Issue:5

    Topics: Alanine; Amino Acids; Aminobutyrates; Aminocaproates; Animals; Culture Media; Cyclohexanecarboxylic Acids; Cysteine; Dipeptides; Female; Glycine; Histidine; Hydroxybutyrates; Injections, Subcutaneous; Mice; Staphylococcal Infections; Staphylococcus; Sulfinic Acids; Taurine; Valerates

1970
Role of kidney arginase in variations of the arginine requirement of chicks.
    The Journal of nutrition, 1970, Volume: 100, Issue:7

    Topics: Aminoisobutyric Acids; Animals; Arginase; Arginine; Chickens; Feeding Behavior; Glycine; Kidney; Nutritional Requirements; Threonine; Time Factors; Urea; Valerates

1970
Influx of glutamic acid in peripheral nerve--characteristics in influx.
    Journal of neurochemistry, 1968, Volume: 15, Issue:9

    Topics: Aminobutyrates; Animals; Anura; Aspartic Acid; Carbon Dioxide; Carbon Isotopes; Chromatography; Extracellular Space; Glutamates; Glutamine; Glycine; In Vitro Techniques; Ketoglutaric Acids; Lysine; Metabolic Clearance Rate; Radiochemistry; Sciatic Nerve; Valerates

1968
Analysis of glycine actions on spinal interneurones by intracellular recording.
    Brain research, 1968, Volume: 11, Issue:2

    Topics: Aminocaproates; Animals; Cats; Electrophysiology; Glycine; Interneurons; Membrane Potentials; Spinal Cord; Synapses; Valerates

1968
Metabolism of amino acids in Agrobacterium tumefaciens. II. Uptake of L-valine by growing cells.
    Canadian journal of biochemistry, 1967, Volume: 45, Issue:2

    Topics: Amino Acids; Carbon Isotopes; Chloramphenicol; Glutamates; Glycine; Hydroxylamines; Puromycin; Rhizobium; Valerates; Valine

1967
Uptake of L-histidine alone and in the presence of other amino acids by carcinogen-induced sarcomas of the rat in vitro.
    Cancer research, 1967, Volume: 27, Issue:3

    Topics: Alanine; Amino Acids; Aminobutyrates; Aminocaproates; Animals; Benz(a)Anthracenes; Brain; Carcinogens; Glycine; Histidine; In Vitro Techniques; Intestinal Mucosa; Kidney; Male; Neoplasm Transplantation; Rats; Sarcoma, Experimental; Spleen; Testis; Valerates

1967
Glycine in acute management of isovalericacidaemia.
    Lancet (London, England), 1980, Feb-09, Volume: 1, Issue:8163

    Topics: Amino Acid Metabolism, Inborn Errors; Female; Glycine; Humans; Infant; Leucine; Oxidoreductases; Valerates

1980
Hyperammonaemia in a preterm infant with isovaleric acidaemia.
    Journal of inherited metabolic disease, 1984, Volume: 7, Issue:2

    Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Female; Glycine; Hemiterpenes; Humans; Infant, Newborn; Infant, Premature; Pentanoic Acids; Valerates

1984
Capillary gas chromatographic/mass spectrometric analysis of abnormal metabolites in hypoglycin-treated rat urine.
    Biomedical mass spectrometry, 1984, Volume: 11, Issue:7

    Topics: Adipates; Animals; Cyclopropanes; Dicarboxylic Acids; Gas Chromatography-Mass Spectrometry; Glucuronates; Glucuronic Acid; Glucuronidase; Glycine; Hydroxy Acids; Hypoglycins; Isoleucine; Male; Rats; Rats, Inbred Strains; Valerates; Valine

1984
The identification and the excretion pattern of isovaleryl glucuronide in the urine of patients with isovaleric acidemia.
    Pediatric research, 1984, Volume: 18, Issue:6

    Topics: Acyl Coenzyme A; Amino Acid Metabolism, Inborn Errors; Chromatography, Gas; Gas Chromatography-Mass Spectrometry; Glucuronates; Glycine; Humans; Isovaleryl-CoA Dehydrogenase; Leucine; Oxidoreductases; Oxidoreductases Acting on CH-CH Group Donors; Valerates

1984
L-carnitine therapy in isovaleric acidemia.
    The Journal of clinical investigation, 1984, Volume: 74, Issue:6

    Topics: Amino Acid Metabolism, Inborn Errors; Carnitine; Child, Preschool; Chromatography, Ion Exchange; Glycine; Hemiterpenes; Humans; Isovaleryl-CoA Dehydrogenase; Male; Mass Spectrometry; Oxidoreductases; Oxidoreductases Acting on CH-CH Group Donors; Pentanoic Acids; Valerates

1984
A combined defect of three mitochondrial carboxylases presenting as biotin-responsive 3-methylcrotonyl glycinuria and 3-hydroxyisovaleric aciduria.
    Clinica chimica acta; international journal of clinical chemistry, 1980, Jan-15, Volume: 100, Issue:2

    Topics: Acidosis; Acyl Coenzyme A; Amino Acid Metabolism, Inborn Errors; Biotin; Butyrates; Carbon Dioxide; Carboxy-Lyases; Child, Preschool; Crotonates; Female; Fibroblasts; Glycine; Humans; Hydroxy Acids; Ligases; Mitochondria; Propionates; Pyruvate Carboxylase Deficiency Disease; Valerates

1980
Isovaleric acidaemia presenting with dwarfism, cataract and congenital abnormalities.
    Journal of inherited metabolic disease, 1982, Volume: 5, Issue:2

    Topics: Abnormalities, Multiple; Amino Acid Metabolism, Inborn Errors; Cataract; Child; Dwarfism; Female; Glycine; Hemiterpenes; Humans; Intellectual Disability; Pentanoic Acids; Valerates

1982
Biosynthesis of 5-aminolevulinic acid and heme from 4,5-dioxovalerate in the rat.
    The Journal of clinical investigation, 1983, Volume: 71, Issue:6

    Topics: Aminolevulinic Acid; Anemia, Hemolytic; Animals; Glycine; Heme; Levulinic Acids; Liver; Male; Phenylhydrazines; Rats; Rats, Inbred Strains; Valerates

1983
Non-ketotic C6-C10-dicarboxylic aciduria: biochemical investigations of two cases.
    Clinica chimica acta; international journal of clinical chemistry, 1980, Mar-28, Volume: 102, Issue:2-3

    Topics: Adipates; Caproates; Caprylates; Child, Preschool; Chromatography, Gas; Decanoic Acids; Dicarboxylic Acids; Fatty Acid Desaturases; Fatty Acids; Glycine; Humans; Hydroxy Acids; Hydroxybutyrates; Hypoglycemia; Infant; Lipid Metabolism, Inborn Errors; Male; Mass Spectrometry; Valerates

1980
Biosynthesis of porphyrins and heme from gamma, delta-dioxovalerate by intact hepatocytes.
    Proceedings of the National Academy of Sciences of the United States of America, 1981, Volume: 78, Issue:9

    Topics: Alanine; Aminolevulinic Acid; Animals; Cells, Cultured; Glycine; Heme; Keto Acids; Levulinic Acids; Liver; Male; Porphyrins; Rats; Succinates; Succinic Acid; Valerates

1981
Changing plasma and urinary organic acid levels in a patient with isovaleric acidemia during an attack.
    Pediatric research, 1982, Volume: 16, Issue:9

    Topics: Amino Acid Metabolism, Inborn Errors; Child, Preschool; Female; Glycine; Hemiterpenes; Humans; Hydroxybutyrates; Leucine; Pentanoic Acids; Valerates

1982
[Isovaleric acidemia: identical biochemical picture in 3 patients with variable clinical manifestations].
    Tijdschrift voor kindergeneeskunde, 1982, Volume: 50, Issue:5

    Topics: Amino Acid Metabolism, Inborn Errors; Cells, Cultured; Child; Child, Preschool; Female; Fibroblasts; Glycine; Hemiterpenes; Humans; Infant; Infant, Newborn; Leucine; Male; Oxidation-Reduction; Pentanoic Acids; Valerates

1982
The antagonism of the toxicity of hypoglycin by glycine.
    Biochemical pharmacology, 1981, Volume: 30, Issue:20

    Topics: Animals; Blood Glucose; Body Temperature; Cyclopropanes; Fatty Acids, Volatile; Gluconeogenesis; Glycine; Hemiterpenes; Hypoglycins; Kinetics; Liver; Male; Pentanoic Acids; Rats; Rats, Inbred Strains; Valerates

1981
Antagonistic effects of isovalerate and glycine on plasma choline levels in rabbits.
    Life sciences, 1980, Jan-28, Volume: 26, Issue:4

    Topics: Animals; Choline; Glycine; Male; Rabbits; Serine; Valerates

1980
Partial methylcrotonyl-coenzyme A carboxylase deficiency in an infant with failure to thrive, gastrointestinal dysfunction, and hypertonia.
    Pediatrics, 1993, Volume: 91, Issue:3

    Topics: Biotin; Carbon-Carbon Ligases; Failure to Thrive; Gastrointestinal Diseases; Glycine; Humans; Infant; Ligases; Male; Metabolism, Inborn Errors; Muscle Hypertonia; Valerates

1993
3-Hydroxyisovalerylcarnitine in patients with deficiency of 3-methylcrotonyl CoA carboxylase.
    Clinica chimica acta; international journal of clinical chemistry, 1995, Aug-31, Volume: 240, Issue:1

    Topics: Carbon-Carbon Ligases; Carnitine; Child, Preschool; Female; Gas Chromatography-Mass Spectrometry; Glycine; Humans; Ligases; Male; Valerates

1995
Isolated (biotin-resistant) 3-methylcrotonyl-CoA carboxylase deficiency: four sibs devoid of pathology.
    Journal of inherited metabolic disease, 1995, Volume: 18, Issue:5

    Topics: Carbon-Carbon Ligases; Carnitine; Child; Child, Preschool; Dietary Proteins; Female; Gas Chromatography-Mass Spectrometry; Glycine; Humans; Infant; Ligases; Male; Valerates

1995
Isolated 3-methylcrotonyl-CoA carboxylase deficiency: evidence for an allele-specific dominant negative effect and responsiveness to biotin therapy.
    American journal of human genetics, 2004, Volume: 75, Issue:5

    Topics: Alleles; Base Sequence; Biotin; Carbon-Carbon Ligases; DNA Mutational Analysis; Dose-Response Relationship, Drug; Fibroblasts; Gene Expression; Genetic Vectors; Germany; Glycine; Greece; Humans; Infant, Newborn; Male; Mitochondrial Diseases; Molecular Sequence Data; Mutation; Reverse Transcriptase Polymerase Chain Reaction; Sequence Analysis, DNA; Transfection; Valerates

2004
2-ethylhydracrylic aciduria in short/branched-chain acyl-CoA dehydrogenase deficiency: application to diagnosis and implications for the R-pathway of isoleucine oxidation.
    Clinical chemistry, 2005, Volume: 51, Issue:3

    Topics: Biomarkers; Butyrates; Butyryl-CoA Dehydrogenase; Gas Chromatography-Mass Spectrometry; Glycine; Humans; Infant, Newborn; Isoleucine; Mutation; Oxidation-Reduction; Stereoisomerism; Valerates

2005
Consanguineous 3-methylcrotonyl-CoA carboxylase deficiency: early-onset necrotizing encephalopathy with lethal outcome.
    Journal of inherited metabolic disease, 2005, Volume: 28, Issue:2

    Topics: Carbon-Carbon Ligases; Consanguinity; Diagnosis, Differential; Fatal Outcome; Glycine; Humans; Infant; Infant, Newborn; Leukoencephalitis, Acute Hemorrhagic; Male; Metabolism, Inborn Errors; Mutation; RNA Splice Sites; Valerates

2005
Isolated 3-methylcrotonyl-coenzyme A carboxylase deficiency in a child with metabolic stroke.
    Journal of inherited metabolic disease, 2006, Volume: 29, Issue:1

    Topics: Amino Acid Metabolism, Inborn Errors; Carbon-Carbon Ligases; Child, Preschool; Glycine; Humans; Male; Phenotype; Stroke; Valerates

2006
Two step derivatization for the analyses of organic, amino acids and glycines on filter paper plasma by GC-MS/SIM.
    Archives of pharmacal research, 2007, Volume: 30, Issue:3

    Topics: Amino Acids; Blood Specimen Collection; Filtration; Gas Chromatography-Mass Spectrometry; Glycine; Humans; Infant; Infant, Newborn; Lactic Acid; Orotic Acid; Valerates

2007
2-Methylbutyryl-coenzyme A dehydrogenase deficiency: functional and molecular studies on a defect in isoleucine catabolism.
    Molecular genetics and metabolism, 2008, Volume: 93, Issue:1

    Topics: Amino Acid Metabolism, Inborn Errors; Butyryl-CoA Dehydrogenase; Cells, Cultured; Child; Child, Preschool; Female; Genotype; Glycine; Humans; Infant; Isoleucine; Male; Metabolism; Valerates

2008
Study of patients and carriers with 2-methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) deficiency: difficulties in the diagnosis.
    Clinical biochemistry, 2009, Volume: 42, Issue:1-2

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Alcohol Oxidoreductases; Amino Acid Metabolism, Inborn Errors; Female; Glycine; Heredodegenerative Disorders, Nervous System; Humans; Hydroxybutyrates; Infant; Infant, Newborn; Male; Valerates

2009
New urea-based surfactants derived from alpha,omega-amino acids.
    The journal of physical chemistry. B, 2009, Jan-29, Volume: 113, Issue:4

    Topics: Alanine; Amino Acids; Calorimetry; Electric Conductivity; Ethanolamines; Glycine; Organophosphates; Spectroscopy, Fourier Transform Infrared; Structure-Activity Relationship; Surface Properties; Surface-Active Agents; Urea; Valerates

2009
3-Methylcrotonyl-CoA carboxylase deficiency: phenotypic variability in a family.
    Journal of child neurology, 2009, Volume: 24, Issue:4

    Topics: Adult; Brain; Carbon-Carbon Ligases; Carnitine; Cells, Cultured; Child, Preschool; DNA Mutational Analysis; Epilepsy; Fibroblasts; Gene Deletion; Genetic Predisposition to Disease; Genetic Testing; Genotype; Glycine; Humans; Infant; Language Development Disorders; Male; Mutation; Phenotype; Valerates

2009
Striatum is more vulnerable to oxidative damage induced by the metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency as compared to liver.
    International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience, 2009, Volume: 27, Issue:4

    Topics: Animals; Corpus Striatum; Glutarates; Glutathione; Glycine; Humans; Liver; Male; Meglutol; Oxidative Stress; Oxo-Acid-Lyases; Rats; Rats, Wistar; Thiobarbituric Acid Reactive Substances; Valerates

2009
Uneventful clinical courses of Korean patients with methylcrotonylglycinuria and their common mutations.
    Journal of human genetics, 2012, Volume: 57, Issue:1

    Topics: Asian People; Carbon-Carbon Ligases; Child; Child, Preschool; Disease Progression; Female; Glycine; Humans; Infant; Infant, Newborn; Male; Mutation; Republic of Korea; Urea Cycle Disorders, Inborn; Valerates

2012
Diagnostic dilemma of patients with methylmalonic aciduria: Experience from a tertiary care centre in Pakistan.
    JPMA. The Journal of the Pakistan Medical Association, 2018, Volume: 68, Issue:4

    Topics: Alanine; Amino Acid Metabolism, Inborn Errors; Child, Preschool; Citrates; Cross-Sectional Studies; Female; Glycine; Humans; Infant; Lactic Acid; Male; Methionine; Mitochondrial Diseases; Pakistan; Tertiary Care Centers; Urinalysis; Valerates; Vitamin B 12 Deficiency

2018