propionic acid has been researched along with methylmalonic acid in 69 studies
Timeframe | Studies, this research(%) | All Research% |
---|---|---|
pre-1990 | 14 (20.29) | 18.7374 |
1990's | 24 (34.78) | 18.2507 |
2000's | 26 (37.68) | 29.6817 |
2010's | 5 (7.25) | 24.3611 |
2020's | 0 (0.00) | 2.80 |
Authors | Studies |
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Hoffmann, JW; Lynch, RE; Shoemaker, JD; Sly, WS | 1 |
Kikawa, Y; Nakai, A; Saito, M; Shigematsu, Y; Sudo, M | 1 |
Bonnefont, JP; Charpentier, C; Cheron, G; Coude, MM; Kamoun, P | 1 |
Chadefaux, B; Kamoun, PP | 1 |
Chalmers, RA; Davies, SE; de Sousa, C; Iles, RA; Stacey, TE | 1 |
Ajami, A; Barshop, BA; Nyhan, WL; Prodanos, C; Smith, M; Sweetman, FR; Sweetman, L; Wolff, JA; Yoshida, I | 1 |
Dutra, JC; Dutra-Filho, CS; Wajner, M; Wannmacher, CF; Wannmacher, CM | 1 |
Bresson, JL; Chalmers, RA; Ford, GC; Halliday, D; Leonard, JV; Lyonnet, SL; Saudubray, JM; Thompson, GN; Walter, JH | 1 |
Chalmers, RA; Thompson, GN | 1 |
Batshaw, ML; Hyman, SL; Iwata, BA; Kissel, R; Page, TJ; Porter, CA | 1 |
Wendel, U | 1 |
Diekmann, E; Laryea, MD; Wendel, U | 1 |
Jeffrey, FM; Malloy, CR; Rajagopal, A; Roby, RE; Sherry, AD | 1 |
Bay, C; Kelts, DG; Ney, D; Nyhan, WL; Saudubray, JM | 1 |
Guibaud, P; Rousson, R | 1 |
Bartlett, K; Daish, P; Leonard, JV; Naughten, ER | 1 |
Charpentier, C; Coudé, FX; Depondt, E; Frézal, J; Mitchell, G; Munnich, A; Ogier, H; Rey, F; Rey, J; Saudubray, JM | 1 |
Bachmann, C; Bühlmann, R; Colombo, JP | 1 |
Benson, PF; Chalmers, RA; Fensom, AH; King, GS; Pettit, BR; Rodeck, CH; Tracey, BM; Watson, D | 1 |
Curless, RG; Dave, P; Steinman, L | 1 |
Di Donato, S; Garavaglia, B; Rimoldi, M; Uziel, G | 1 |
Donnell, GN; Giudici, TA; Ng, WG; Oizumi, J; Shaw, KN | 1 |
Dempsey, NJ; Ginder, GD; Little, JA; Tuchman, M | 1 |
Bachollet, B; Bodemer, C; De Prost, Y; Fraitag, S; Poggi, F; Saudubray, JM; Teillac-Hamel, D | 1 |
Allen, RH; Kolhouse, JF; Stabler, SP | 1 |
Hoppel, CL; Minkler, PE | 1 |
Kennedy, DG; Kennedy, S; Young, PB | 1 |
Murrell, M; Narasimhan, P; Sharp, FR; Sklar, R; Swanson, RA | 1 |
Blum, D; Clercx, A; Corazza, F; Fondu, P; Mardens, Y | 1 |
Coelho, J; de Mello, CF; Rubin, MA; Souza, DO; Wajner, M | 1 |
de Freitas, MS; de Mattos-Dutra, A; Lisboa, CS; Pessoa-Pureur, R; Wajner, M | 1 |
Chadefaux-Vekemans, B; Garcia, MJ; Jakobs, C; Kamoun, P; Merinero, B; Pérez-Cerdá, C; Tonetti, C; Ugarte, M; Zittoun, J | 1 |
Rocha, MP; Santos, KD; Schlottfeldt, JL; Wajner, M; Wannmacher, CM | 1 |
de Mattos-Dutra, A; Pessoa-Pureur, R; Sampaio de Freitas, M; Wajner, M | 1 |
Chalmers, RA; Down, S; Halket, JM; Mallard, WG; Przyborowska, A; Stein, SE | 1 |
Allen, DL; DeRose, EF; Gabel, SA; London, RE | 1 |
Calabrese, V; Rizza, V | 1 |
Bevilaqua da Rocha, B; de Mattos-Dutra, A; Kommers, T; Meirelles, R; Pessoa-Pureur, R; Wajner, M; Wofchuk, ST | 1 |
Dutra-Filho, CS; Fontella, FU; Gassen, E; Klein, AB; Pulrolnik, V; Wajner, M; Wannmacher, CM | 1 |
de Hoffmann, E; Libert, R; Nassogne, MC; Schanck, A; Thillaye, M; Van Hoof, F; Vincent, MF | 1 |
Blau, N; Bonafé, L; Burlina, AB; Chamoles, NA; Heizmann, CW; Kuster, T; Troxler, H | 1 |
Chace, DH; DiPerna, JC; Johnson, RW; Kalas, TA; Naylor, EW | 1 |
Brusque, AM; de Souza Wyse, AT; Dutra-Filho, CS; Duval Wannmacher, CM; Emanuelli, T; Gomes de Souza, DO; Rotta, LN; Schwarzbold, CV; Tavares, RG; Wajner, M | 1 |
Brusque, AM; Pettenuzzo, LE; Raasch, JR; Rocha, HP; Trindade, VM; Wajne, M; Wannmacher, CM | 1 |
de Almeida, LM; Funchal, C; Loureiro, Sde O; Pelaez, Pde L; Pessoa-Pureur, R; Pessutto, FD; Vivian, L; Wajner, M | 1 |
Walter, JH | 1 |
de Almeida, LM; Funchal, C; Loureiro, SO; Pelaez, Pde L; Pessoa-Pureur, R; Pessutto, FD; Vivian, L; Wajner, M | 1 |
Leonard, JV; Vijayaraghavan, S; Walter, JH | 1 |
Pettenuzzo, LF; Rosa, RB; Schuck, PF; Sitta, A; Wajner, M; Wannmacher, CM; Wyse, AT | 1 |
Hasegawa, Y; Hori, D; Kimura, M; Verma, IC; Yamaguchi, S; Yang, Y | 1 |
Benoist, JF; de Baulny, HO; Rabier, D; Rigal, O; Saudubray, JM; Touati, G | 1 |
de Lima Pelaez, P; Heimfarth, L; Loureiro, SO; Pessoa-Pureur, R; Souza, DO; Wajner, M | 1 |
Carrara, B; Gritti, P; Manzoni, D; Sonzogni, V; Spotti, A | 1 |
Gradowska, W; Krawczyk, H | 1 |
Endo, M; Hasegawa, Y; Kobayashi, H; Purevsuren, J; Yamaguchi, S | 1 |
Barshop, BA; Gangoiti, JA; Siuzdak, G; Wikoff, WR | 1 |
Bennett, MJ | 1 |
Van Gosen, L | 1 |
Abdoh, G; Burgard, P; Ho, S; Hoffmann, GF; Kölker, S; Lindner, M | 1 |
Botella, L; Eggeling, L; Lindley, ND | 1 |
Altiparmak, UE; Arnold, GL; Eggenberger, E; Feldon, SE; Hurley, PE; Mejico, LJ; Williams, ZR | 1 |
Fuller, GG; Hsu, TT; Nishimura, S; Sonner, JM; Weng, Y; Zhao, J | 1 |
Haarmann, A; Kölker, S; Morath, MA; Okun, JG; Opp, S; Sauer, SW | 1 |
Caggana, M; Cohen-Pfeffer, JL; Diaz, GA; Kirmse, BM; McGuire, PJ; Morrissey, MA; Salveson, BR; Sunny, S; Wasserstein, MP; Weisfeld-Adams, JD; Yu, C | 1 |
Biancini, GB; de Marco, MG; Manfredini, V; Ribas, GS; Vanzin, CS; Vargas, CR; Vieira, RB; Wajner, M; Wayhs, CY | 1 |
Colín-González, AL; Leipnitz, G; Paz-Loyola, AL; Ribeiro, CA; Santamaría, A; Seminotti, B; Serratos, I; Souza, DO; Wajner, M | 1 |
Colín-González, AL; Leipnitz, G; Paz-Loyola, AL; Ribeiro, CA; Santamaría, A; Seminotti, B; Serratos, IN; Souza, DO; Wajner, M | 1 |
Akamine, S; Fukai, R; Hara, T; Ishizaki, Y; Kimura, M; Koga, H; Matsumoto, N; Miyake, N; Ohga, S; Ohkubo, K; Saitsu, H; Sakai, Y; Sakamoto, O; Sakata, A; Sanefuji, M; Torisu, H; Yamaguchi, S | 1 |
2 review(s) available for propionic acid and methylmalonic acid
Article | Year |
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Methylmalonic and propionic acidaemias: management and outcome.
Topics: Child, Preschool; Humans; Infant; Infant, Newborn; Long-Term Care; Metabolism, Inborn Errors; Methylmalonic Acid; Nutritional Support; Propionates; Toxins, Biological; Treatment Outcome | 2005 |
Organic acidemias: a methylmalonic and propionic focus.
Topics: Acidosis; Aftercare; Amino Acid Metabolism, Inborn Errors; Diet, Protein-Restricted; Early Diagnosis; Fluid Therapy; Genes, Recessive; Genetic Testing; Hemofiltration; Humans; Incidence; Infant, Newborn; Intensive Care, Neonatal; Liver Transplantation; Methylmalonic Acid; Neonatal Nursing; Neonatal Screening; Nurse's Role; Nursing Assessment; Prenatal Diagnosis; Propionates; Survival Rate | 2008 |
67 other study(ies) available for propionic acid and methylmalonic acid
Article | Year |
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Misidentification of propionic acid as ethylene glycol in a patient with methylmalonic acidemia.
Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Chromatography, Gas; Diagnostic Errors; Ethylene Glycol; Ethylene Glycols; Gas Chromatography-Mass Spectrometry; Humans; Infant; Male; Methylmalonic Acid; Poisoning; Propionates | 1992 |
Pathophysiologic study on methylmalonic aciduria: decrease in liver high-energy phosphate after propionate loading in rats.
Topics: Adenosine Triphosphate; Animals; Citric Acid Cycle; Energy Metabolism; Liver; Male; Methylmalonic Acid; Methylmalonyl-CoA Mutase; Propionates; Rats; Rats, Inbred Strains; Vitamin B 12 Deficiency | 1991 |
Organic acids in aqueous humour and plasma: post mortem study in infants and diagnosis of enzymopathies.
Topics: Adipates; Aqueous Humor; Carboxylic Acids; Fatty Acids; Gas Chromatography-Mass Spectrometry; Humans; Infant; Infant, Newborn; Metabolism, Inborn Errors; Methylmalonic Acid; Propionates; Retrospective Studies | 1991 |
Eleventh week amniocentesis for prenatal diagnosis of some metabolic diseases.
Topics: Amniocentesis; Amniotic Fluid; Argininosuccinic Acid; Chorionic Villi Sampling; Citrulline; Female; Humans; Metabolism, Inborn Errors; Methylmalonic Acid; Pregnancy; Pregnancy Trimester, First; Prenatal Diagnosis; Propionates | 1991 |
Carnitine therapy and metabolism in the disorders of propionyl-CoA metabolism studied using 1H-NMR spectroscopy.
Topics: Acyl Coenzyme A; Amino Acid Metabolism, Inborn Errors; Carnitine; Child, Preschool; Female; Gas Chromatography-Mass Spectrometry; Humans; Infant; Infant, Newborn; Kinetics; Magnetic Resonance Spectroscopy; Male; Methylmalonic Acid; Propionates | 1991 |
Metabolism of 1-13C-propionate in vivo in patients with disorders of propionate metabolism.
Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Biotin; Carbon Dioxide; Carboxy-Lyases; Carnitine; Child; Child, Preschool; Female; Half-Life; Humans; Infant; Male; Methylmalonic Acid; Methylmalonyl-CoA Decarboxylase; Propionates; Transcobalamins; Vitamin B 12 | 1991 |
Effects of methylmalonate and propionate on uptake of glucose and ketone bodies in vitro by brain of developing rats.
Topics: 3-Hydroxybutyric Acid; Acetoacetates; Animals; Biological Transport, Active; Brain; Female; Glucose; Hydroxybutyrates; In Vitro Techniques; Ketone Bodies; Male; Methylmalonic Acid; Propionates; Rats; Rats, Inbred Strains | 1991 |
Sources of propionate in inborn errors of propionate metabolism.
Topics: Amino Acids; Child; Child, Preschool; Humans; Metabolism, Inborn Errors; Methylmalonic Acid; Metronidazole; Osmolar Concentration; Propionates | 1990 |
Increased urinary metabolite excretion during fasting in disorders of propionate metabolism.
Topics: Adult; Amino Acid Metabolism, Inborn Errors; Child; Child, Preschool; Citrates; Fasting; Fatty Acids; Glycine; Humans; Infant; Lactates; Lactic Acid; Methylmalonic Acid; Propionates | 1990 |
Behavior management of feeding disturbances in urea cycle and organic acid disorders.
Topics: Adult; Amino Acid Metabolism, Inborn Errors; Anorexia; Argininosuccinic Acid; Behavior Therapy; Child; Child Behavior Disorders; Child, Preschool; Energy Intake; Feeding and Eating Disorders; Feeding Behavior; Female; Homovanillic Acid; Humans; Hydroxyindoleacetic Acid; Infant; Male; Methylmalonic Acid; Ornithine Carbamoyltransferase Deficiency Disease; Propionates | 1987 |
Gut bacterial metabolism.
Topics: Child, Preschool; Fermentation; Hirschsprung Disease; Humans; Infant, Newborn; Intestines; Male; Malonates; Methylmalonic Acid; Phenylacetates; Propionates | 1988 |
Abnormality of odd-numbered long-chain fatty acids in erythrocyte membrane lipids from patients with disorders of propionate metabolism.
Topics: Adolescent; Adult; Child; Child, Preschool; Erythrocyte Membrane; Fatty Acids; Humans; Infant; Infant, Newborn; Malonates; Membrane Lipids; Metabolism, Inborn Errors; Methylmalonic Acid; Phosphatidylcholines; Phosphatidylethanolamines; Propionates | 1989 |
Odd-numbered long-chain fatty acid contents in erythrocyte membrane phospholipids in patients with an impaired propionate utilization.
Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Erythrocyte Membrane; Fatty Acids; Humans; Infant; Infant, Newborn; Malonates; Methylmalonic Acid; Phosphatidylcholines; Phosphatidylethanolamines; Phospholipids; Propionates | 1988 |
Propionate metabolism in the rat heart by 13C n.m.r. spectroscopy.
Topics: Animals; Carbon Isotopes; Carnitine; In Vitro Techniques; Magnetic Resonance Spectroscopy; Methylmalonic Acid; Myocardium; Perfusion; Propionates; Pyruvates; Pyruvic Acid; Rats | 1988 |
Studies on requirements for amino acids in infants with disorders of amino acid metabolism. I. Effect of alanine.
Topics: Alanine; Amino Acid Metabolism, Inborn Errors; Amino Acids; Dietary Proteins; Energy Intake; Female; Humans; Infant; Methylmalonic Acid; Nutritional Requirements; Propionates | 1985 |
Long term outcome of organic acidurias: survey of 105 French cases (1967-1983).
Topics: Acids; Adolescent; Child; Child, Preschool; France; Hemiterpenes; Humans; Infant; Infant, Newborn; Maple Syrup Urine Disease; Metabolism, Inborn Errors; Methylmalonic Acid; Pentanoic Acids; Prognosis; Propionates; Time Factors | 1984 |
The management and long term outcome of organic acidaemias.
Topics: Acids; Amidohydrolases; Biotinidase; Carbon-Carbon Ligases; Humans; Infant; Infant, Newborn; Ligases; Maple Syrup Urine Disease; Metabolism, Inborn Errors; Methylmalonic Acid; Prognosis; Propionates | 1984 |
Hudson memorial lecture. Neonatal management of organic acidurias. Clinical update.
Topics: Acids; Amino Acid Metabolism, Inborn Errors; Diuresis; Exchange Transfusion, Whole Blood; Hemiterpenes; Humans; Infant, Newborn; Maple Syrup Urine Disease; Methylmalonic Acid; Pentanoic Acids; Peritoneal Dialysis; Propionates | 1984 |
Organic acids in urine: sample preparation for GC/MS.
Topics: Acids; Gas Chromatography-Mass Spectrometry; Humans; Metabolism, Inborn Errors; Methylmalonic Acid; Propionates | 1984 |
Experience with prenatal diagnosis of propionic acidaemia and methylmalonic aciduria.
Topics: Amniotic Fluid; Carboxy-Lyases; Female; Humans; Malonates; Methylmalonic Acid; Methylmalonyl-CoA Decarboxylase; Pregnancy; Prenatal Diagnosis; Propionates | 1984 |
Cerebellar hemorrhage complicating methylmalonic and propionic acidemia.
Topics: Acidosis; Bicarbonates; Cerebellar Diseases; Female; Hemorrhage; Humans; Infant, Newborn; Male; Malonates; Metabolism, Inborn Errors; Methylmalonic Acid; Propionates | 1984 |
Propionylcarnitine excretion in propionic and methylmalonic acidurias: a cause of carnitine deficiency.
Topics: Carnitine; Child, Preschool; Humans; Infant; Infant, Newborn; Malonates; Metabolism, Inborn Errors; Methylmalonic Acid; Muscles; Propionates | 1984 |
Propionate metabolism by cultured skin fibroblasts from normal individuals and patients with methylmalonicaciduria and propionicacidemia.
Topics: Amino Acid Metabolism, Inborn Errors; Cells, Cultured; Fatty Acids, Nonesterified; Fibroblasts; Humans; Lactates; Lactic Acid; Lipid Metabolism; Malonates; Methylmalonic Acid; Propionates; Succinates; Succinic Acid; Tricarboxylic Acids | 1981 |
Metabolic persistence of fetal hemoglobin.
Topics: Acyl-CoA Dehydrogenase; Adult; Amino Acid Metabolism, Inborn Errors; Animals; Base Sequence; Carboxy-Lyases; Child; Child, Preschool; DNA Fingerprinting; DNA Mutational Analysis; Fatty Acid Desaturases; Fatty Acids; Female; Fetal Hemoglobin; Gene Expression Regulation; Globins; Hemoglobinopathies; Humans; Infant; Infant, Newborn; Leukemia, Erythroblastic, Acute; Male; Methylmalonic Acid; Methylmalonyl-CoA Decarboxylase; Methylmalonyl-CoA Mutase; Mice; Molecular Sequence Data; Polymorphism, Single-Stranded Conformational; Promoter Regions, Genetic; Propionates; Tumor Cells, Cultured | 1995 |
Cutaneous manifestations of methylmalonic and propionic acidaemia: a description based on 38 cases.
Topics: Amino Acid Metabolism, Inborn Errors; Child, Preschool; Dermatitis; Edema; Erythema; Humans; Infant; Infant, Newborn; Male; Methylmalonic Acid; Propionates; Skin; Skin Diseases | 1994 |
Identification and perturbation of mutant human fibroblasts based on measurements of methylmalonic acid and total homocysteine in the culture media.
Topics: 5-Methyltetrahydrofolate-Homocysteine S-Methyltransferase; Cell Line; Culture Media; Fibroblasts; Homocysteine; Humans; Methylmalonic Acid; Methylmalonyl-CoA Mutase; Mutation; Propionates; Vitamin B 12 | 1993 |
Quantification of free carnitine, individual short- and medium-chain acylcarnitines, and total carnitine in plasma by high-performance liquid chromatography.
Topics: Acetylcarnitine; Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Acylation; Carnitine; Chromatography, High Pressure Liquid; Humans; Methylmalonic Acid; Molecular Weight; Propionates; Reference Standards; Reproducibility of Results | 1993 |
Effects of low concentrations of dietary cobalt on rumen succinate concentration in sheep.
Topics: Acyl Coenzyme A; Animals; Cobalt; Diet; Liver; Methylmalonic Acid; Methylmalonyl-CoA Mutase; Propionates; Rumen; Sheep; Succinates; Succinic Acid; Vitamin B 12 | 1996 |
Methylmalonyl-CoA mutase induction by cerebral ischemia and neurotoxicity of the mitochondrial toxin methylmalonic acid.
Topics: Animals; Basal Ganglia; Base Sequence; Blotting, Northern; Blotting, Western; Brain Chemistry; Brain Ischemia; Cell Death; DNA, Complementary; Gene Expression Regulation, Developmental; Gene Expression Regulation, Enzymologic; Gerbillinae; Isoleucine; Male; Methylmalonic Acid; Methylmalonyl-CoA Mutase; Microinjections; Mitochondria; Molecular Sequence Data; Neurons; Neurotoxins; Oxidative Stress; Propionates; RNA, Messenger; Sequence Homology, Amino Acid | 1996 |
Erythroblastopenia associated with methylmalonic aciduria. Case report and in vitro studies.
Topics: Amino Acid Metabolism, Inborn Errors; Anemia; Blood; Cells, Cultured; Erythroblasts; Erythroid Precursor Cells; Erythropoiesis; Female; Humans; Infant, Newborn; Methylmalonic Acid; Propionates | 1996 |
Effects of methylmalonate and propionate on [3H]glutamate binding, adenylate cyclase activity and lipid synthesis in rat cerebral cortex.
Topics: Acetates; Adenylyl Cyclases; Animals; Cerebral Cortex; Glutamic Acid; Guanylyl Imidodiphosphate; Lipids; Male; Methylmalonic Acid; Propionates; Rats; Rats, Wistar; Sodium; Tritium | 1997 |
Effects of acute and chronic administration of methylmalonic and propionic acids on the in vitro incorporation of 32P into cytoskeletal proteins from cerebral cortex of young rats.
Topics: Animals; Autoradiography; Cerebral Cortex; Cytoskeletal Proteins; Methylmalonic Acid; Organ Size; Phosphorus Radioisotopes; Propionates; Rats; Rats, Wistar | 1998 |
Reliability of biochemical parameters used in prenatal diagnosis of combined methylmalonic aciduria and homocystinuria.
Topics: Amino Acid Metabolism, Inborn Errors; Amniocentesis; Amniotic Fluid; Cells, Cultured; Chorion; Chorionic Villi Sampling; Cobamides; Female; Gestational Age; Homocysteine; Homocystinuria; Humans; Male; Methylmalonic Acid; Pregnancy; Propionates; Tetrahydrofolates; Vitamin B 12 | 1998 |
Inhibition of mitogen-activated proliferation of human peripheral lymphocytes in vitro by propionic acid.
Topics: Adult; Cells, Cultured; Concanavalin A; Humans; Immunosuppressive Agents; Lymphocyte Activation; Lymphocytes; Methylmalonic Acid; Mitogens; Phytohemagglutinins; Pokeweed Mitogens; Propionates | 1999 |
Propionic and methylmalonic acids inhibit the in vitro phosphorylation of a 85 kDa cytoskeletal protein from cerebral cortex of rats.
Topics: Animals; Calcium-Calmodulin-Dependent Protein Kinase Type 2; Calcium-Calmodulin-Dependent Protein Kinases; Cerebral Cortex; Cyclic AMP-Dependent Protein Kinases; Cytoskeletal Proteins; Methylmalonic Acid; Phosphorylation; Propionates; Rats; Rats, Wistar | 1998 |
Deconvolution gas chromatography/mass spectrometry of urinary organic acids--potential for pattern recognition and automated identification of metabolic disorders.
Topics: Acyl-CoA Dehydrogenase; Autoanalysis; Child, Preschool; Dicarboxylic Acids; Fatty Acid Desaturases; Gas Chromatography-Mass Spectrometry; Glutarates; Hemiterpenes; Humans; Infant; Meglutol; Metabolism, Inborn Errors; Methylmalonic Acid; Pattern Recognition, Automated; Pentanoic Acids; Propionates | 1999 |
Carbon-13 nuclear magnetic resonance study of metabolism of propionate by Escherichia coli.
Topics: Acyl Coenzyme A; Alanine; Carbon Isotopes; Cell Wall; Citrates; Citric Acid; Citric Acid Cycle; Escherichia coli; Formates; Gluconeogenesis; Glutamic Acid; Glycine; Magnetic Resonance Spectroscopy; Methylmalonic Acid; Oxaloacetic Acid; Propionates; Protons; Putrescine; Pyruvic Acid; Succinic Acid; Trehalose | 1999 |
Formation of propionate after short-term ethanol treatment and its interaction with the carnitine pool in rat.
Topics: Acids, Acyclic; Adrenergic beta-Antagonists; Animals; Carnitine; Central Nervous System Depressants; Enzyme Inhibitors; Ethanol; Male; Methylmalonic Acid; Propionates; Propranolol; Pyrazoles; Rats | 1999 |
Methylmalonic and propionic acids increase the in vitro incorporation of 32P into cytoskeletal proteins from cerebral cortex of young rats through NMDA glutamate receptors.
Topics: 2-Amino-5-phosphonovalerate; 6-Cyano-7-nitroquinoxaline-2,3-dione; Animals; Cerebral Cortex; Cytoskeletal Proteins; Glutamic Acid; In Vitro Techniques; Methylmalonic Acid; N-Methylaspartate; Phosphates; Phosphorus Radioisotopes; Phosphorylation; Propionates; Rats; Rats, Wistar; Receptors, N-Methyl-D-Aspartate | 2000 |
Propionic and L-methylmalonic acids induce oxidative stress in brain of young rats.
Topics: Animals; Antioxidants; Cerebral Cortex; Luminescent Measurements; Methylmalonic Acid; Oxidative Stress; Propionates; Rats; Rats, Wistar; Thiobarbituric Acid Reactive Substances | 2000 |
Identification of undescribed medium-chain acylcarnitines present in urine of patients with propionic and methylmalonic acidemias.
Topics: Adult; Carboxy-Lyases; Carnitine; Case-Control Studies; Gas Chromatography-Mass Spectrometry; Humans; Infant; Lipid Metabolism, Inborn Errors; Male; Methylmalonic Acid; Methylmalonyl-CoA Decarboxylase; Propionates; Spectrometry, Mass, Fast Atom Bombardment | 2000 |
Evaluation of urinary acylglycines by electrospray tandem mass spectrometry in mitochondrial energy metabolism defects and organic acidurias.
Topics: Acyl-CoA Dehydrogenase; Adolescent; Adult; Amidohydrolases; Biotinidase; Carbon-Nitrogen Ligases; Child; Child, Preschool; Fatty Acid Desaturases; Glycine; Hemiterpenes; Humans; Infant; Infant, Newborn; Mass Spectrometry; Meglutol; Metabolism, Inborn Errors; Methylmalonic Acid; Mitochondria; Pentanoic Acids; Propionates | 2000 |
Rapid diagnosis of methylmalonic and propionic acidemias: quantitative tandem mass spectrometric analysis of propionylcarnitine in filter-paper blood specimens obtained from newborns.
Topics: Amino Acid Metabolism, Inborn Errors; Blood Specimen Collection; Carnitine; False Positive Reactions; Humans; Infant, Newborn; Methylmalonic Acid; Paper; Propionates; Spectrometry, Mass, Electrospray Ionization | 2001 |
Effects of methylmalonic and propionic acids on glutamate uptake by synaptosomes and synaptic vesicles and on glutamate release by synaptosomes from cerebral cortex of rats.
Topics: Animals; Cerebral Cortex; Glutamic Acid; L-Lactate Dehydrogenase; Male; Methylmalonic Acid; Nerve Tissue Proteins; Potassium; Propionates; Rats; Rats, Wistar; Synaptic Vesicles; Synaptosomes | 2001 |
Ganglioside alterations in the central nervous system of rats chronically injected with methylmalonic and propionic acids.
Topics: Animals; Animals, Newborn; Body Weight; Central Nervous System; Cerebellum; Cerebral Cortex; Drug Administration Schedule; Female; G(M1) Ganglioside; G(M2) Ganglioside; Gangliosides; Metabolism, Inborn Errors; Methylmalonic Acid; Organ Size; Propionates; Rats; Rats, Wistar | 2002 |
Effect of propionic and methylmalonic acids on the high molecular weight neurofilament subunit (NF-H) in rat cerebral cortex.
Topics: Animals; Blotting, Western; Cerebral Cortex; Cytoskeleton; In Vitro Techniques; Methylmalonic Acid; Molecular Weight; Neurofilament Proteins; Phosphorylation; Propionates; Rats; Rats, Wistar | 2002 |
L-carnitine in inborn errors of metabolism: what is the evidence?
Topics: Acyl-CoA Dehydrogenase; Carnitine; Humans; Metabolism, Inborn Errors; Methylmalonic Acid; Propionates | 2003 |
Effect of propionic and methylmalonic acids on the in vitro phosphorylation of intermediate filaments from cerebral cortex of rats during development.
Topics: Animals; Cerebral Cortex; Cytoskeletal Proteins; Electrophoresis, Polyacrylamide Gel; In Vitro Techniques; Intermediate Filaments; Methylmalonic Acid; Phosphoric Monoester Hydrolases; Phosphorylation; Propionates; Protein Serine-Threonine Kinases; Rats; Rats, Wistar | 2003 |
The impact of screening for propionic and methylmalonic acidaemia.
Topics: Amino Acid Metabolism, Inborn Errors; Dietary Proteins; Humans; Infant, Newborn; Mass Spectrometry; Methylmalonic Acid; Prognosis; Propionates; Time Factors | 2003 |
Inhibition of mitochondrial creatine kinase activity from rat cerebral cortex by methylmalonic acid.
Topics: Animals; Antioxidants; Cerebral Cortex; Creatine Kinase; Crotonates; Cytosol; Energy Metabolism; Enzyme Inhibitors; Hemiterpenes; In Vitro Techniques; Indicators and Reagents; Male; Methylmalonic Acid; Mitochondria; Nerve Tissue Proteins; Nitric Oxide Synthase; Nitric Oxide Synthase Type I; Propionates; Rats | 2004 |
Clinical onset and prognosis of Asian children with organic acidemias, as detected by analysis of urinary organic acids using GC/MS, instead of mass screening.
Topics: Adolescent; Age of Onset; Asia; Carboxylic Acids; Child; Child, Preschool; Disease Progression; Drug Resistance; Family Health; Female; Gas Chromatography-Mass Spectrometry; Humans; Infant; Infant, Newborn; Male; Mass Screening; Metabolism, Inborn Errors; Methylmalonic Acid; Multiple Carboxylase Deficiency; Ornithine Carbamoyltransferase; Ornithine Carbamoyltransferase Deficiency Disease; Prognosis; Propionates; Treatment Outcome; Vitamin B 12 | 2005 |
Propionic and methylmalonic acids increase cAMP levels in slices of cerebral cortex of young rats via adrenergic and glutamatergic mechanisms.
Topics: Adrenergic beta-Agonists; Adrenergic beta-Antagonists; Analysis of Variance; Animals; Cerebral Cortex; Cyclic AMP; Epinephrine; Glutamic Acid; Glycine; Methylmalonic Acid; Propionates; Propranolol; Rats; Rats, Wistar | 2005 |
Anaesthesia for liver transplantation in two infants with an organic acidaemia.
Topics: Anesthesia; Female; Humans; Infant; Liver Transplantation; Metabolism, Inborn Errors; Methylmalonic Acid; Propionates; Quality of Life | 2006 |
1H NMR spectra of methylcitric acid in urine.
Topics: Citrates; Humans; Hydrogen-Ion Concentration; Magnetic Resonance Spectroscopy; Methylmalonic Acid; Nuclear Magnetic Resonance, Biomolecular; Propionates; Protons; Stereoisomerism | 2007 |
ESI-MS/MS study of acylcarnitine profiles in urine from patients with organic acidemias and fatty acid oxidation disorders.
Topics: Carnitine; Fatty Acids; Humans; Infant; Lipid Metabolism, Inborn Errors; Metabolism, Inborn Errors; Methylmalonic Acid; Oxidation-Reduction; Propionates; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry | 2007 |
Metabolomics identifies perturbations in human disorders of propionate metabolism.
Topics: Acetylcarnitine; Adult; Amino Acid Metabolism, Inborn Errors; Betaine; Biomarkers; Carnitine; Child; Chromatography, High Pressure Liquid; Humans; Methylmalonic Acid; Plasma; Propionates; Spectrometry, Mass, Electrospray Ionization | 2007 |
Untargeted metabolomic analysis hits the target.
Topics: Amino Acid Metabolism, Inborn Errors; Chromatography, High Pressure Liquid; Humans; Methylmalonic Acid; Propionates; Spectrometry, Mass, Electrospray Ionization | 2007 |
Newborn screening for methylmalonic acidurias--optimization by statistical parameter combination.
Topics: Amino Acid Metabolism, Inborn Errors; Humans; Infant, Newborn; Methylmalonic Acid; Neonatal Screening; Propionates; Sensitivity and Specificity; Tandem Mass Spectrometry | 2008 |
Formation and metabolism of methylmalonyl coenzyme A in Corynebacterium glutamicum.
Topics: Acyl Coenzyme A; Bacterial Proteins; Corynebacterium glutamicum; Escherichia coli; Gene Deletion; Methylmalonic Acid; Methylmalonyl-CoA Decarboxylase; Methylmalonyl-CoA Mutase; Propionates; Succinic Acid | 2009 |
Late onset optic neuropathy in methylmalonic and propionic acidemia.
Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Dietary Supplements; Female; Humans; Male; Methylmalonic Acid; Optic Nerve Diseases; Propionates; Retrospective Studies; Scotoma; Visual Acuity; Visual Fields; Young Adult | 2009 |
Isovaleric, methylmalonic, and propionic acid decrease anesthetic EC50 in tadpoles, modulate glycine receptor function, and interact with the lipid 1,2-dipalmitoyl-Sn-glycero-3-phosphocholine.
Topics: 1,2-Dipalmitoylphosphatidylcholine; Anesthetics, Inhalation; Animals; Cell Membrane; Chlorides; Dose-Response Relationship, Drug; Female; Hemiterpenes; Isoflurane; Larva; Membrane Potentials; Methylmalonic Acid; Mutation; Pentanoic Acids; Pressure; Propionates; Receptors, GABA-A; Receptors, Glycine; Surface Properties; Xenopus laevis | 2009 |
Long-term exposure of human proximal tubule cells to hydroxycobalamin[c-lactam] as a possible model to study renal disease in methylmalonic acidurias.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Cells, Cultured; Electron Transport; Glutathione; Humans; Kidney Diseases; Kidney Tubules, Proximal; Methylmalonic Acid; Propionates; Time Factors; Vitamin B 12 | 2009 |
Newborn screening and early biochemical follow-up in combined methylmalonic aciduria and homocystinuria, cblC type, and utility of methionine as a secondary screening analyte.
Topics: Algorithms; Carnitine; Demography; False Positive Reactions; Female; Follow-Up Studies; Genetic Association Studies; Homocystinuria; Humans; Infant, Newborn; Male; Metabolism, Inborn Errors; Methionine; Methylmalonic Acid; Neonatal Screening; New York; Propionates; Referral and Consultation; Vitamin B 12 | 2010 |
Prevention by L-carnitine of DNA damage induced by propionic and L-methylmalonic acids in human peripheral leukocytes in vitro.
Topics: Carnitine; Comet Assay; DNA Damage; Humans; Leukocytes; Methylmalonic Acid; Mutagens; Propionates | 2010 |
Toxic synergism between quinolinic acid and organic acids accumulating in glutaric acidemia type I and in disorders of propionate metabolism in rat brain synaptosomes: Relevance for metabolic acidemias.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Brain; Brain Diseases, Metabolic; Disease Models, Animal; Glutarates; Glutaryl-CoA Dehydrogenase; Lipid Peroxidation; Male; Methylmalonic Acid; Neuroprotective Agents; Oxidative Stress; Propionates; Quinolinic Acid; Rats, Wistar; Reactive Oxygen Species; Synaptosomes | 2015 |
The effect of WIN 55,212-2 suggests a cannabinoid-sensitive component in the early toxicity induced by organic acids accumulating in glutaric acidemia type I and in related disorders of propionate metabolism in rat brain synaptosomes.
Topics: Acids, Acyclic; Amino Acid Metabolism, Inborn Errors; Animals; Benzoxazines; Brain; Brain Diseases, Metabolic; Cannabinoid Receptor Agonists; Glutarates; Glutaryl-CoA Dehydrogenase; Lipid Peroxidation; Male; Methylmalonic Acid; Mitochondria; Morpholines; Naphthalenes; Oxidative Stress; Propionates; Rats; Rats, Wistar; Reactive Oxygen Species; Synaptosomes | 2015 |
A male case with CDKL5-associated encephalopathy manifesting transient methylmalonic acidemia.
Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Brain Diseases; Humans; Male; Methionine; Methylmalonic Acid; Propionates; Protein Serine-Threonine Kinases; Syndrome | 2018 |