propionic acid and methylmalonic acid

propionic acid has been researched along with methylmalonic acid in 69 studies

Research

Studies (69)

TimeframeStudies, this research(%)All Research%
pre-199014 (20.29)18.7374
1990's24 (34.78)18.2507
2000's26 (37.68)29.6817
2010's5 (7.25)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Hoffmann, JW; Lynch, RE; Shoemaker, JD; Sly, WS1
Kikawa, Y; Nakai, A; Saito, M; Shigematsu, Y; Sudo, M1
Bonnefont, JP; Charpentier, C; Cheron, G; Coude, MM; Kamoun, P1
Chadefaux, B; Kamoun, PP1
Chalmers, RA; Davies, SE; de Sousa, C; Iles, RA; Stacey, TE1
Ajami, A; Barshop, BA; Nyhan, WL; Prodanos, C; Smith, M; Sweetman, FR; Sweetman, L; Wolff, JA; Yoshida, I1
Dutra, JC; Dutra-Filho, CS; Wajner, M; Wannmacher, CF; Wannmacher, CM1
Bresson, JL; Chalmers, RA; Ford, GC; Halliday, D; Leonard, JV; Lyonnet, SL; Saudubray, JM; Thompson, GN; Walter, JH1
Chalmers, RA; Thompson, GN1
Batshaw, ML; Hyman, SL; Iwata, BA; Kissel, R; Page, TJ; Porter, CA1
Wendel, U1
Diekmann, E; Laryea, MD; Wendel, U1
Jeffrey, FM; Malloy, CR; Rajagopal, A; Roby, RE; Sherry, AD1
Bay, C; Kelts, DG; Ney, D; Nyhan, WL; Saudubray, JM1
Guibaud, P; Rousson, R1
Bartlett, K; Daish, P; Leonard, JV; Naughten, ER1
Charpentier, C; Coudé, FX; Depondt, E; Frézal, J; Mitchell, G; Munnich, A; Ogier, H; Rey, F; Rey, J; Saudubray, JM1
Bachmann, C; Bühlmann, R; Colombo, JP1
Benson, PF; Chalmers, RA; Fensom, AH; King, GS; Pettit, BR; Rodeck, CH; Tracey, BM; Watson, D1
Curless, RG; Dave, P; Steinman, L1
Di Donato, S; Garavaglia, B; Rimoldi, M; Uziel, G1
Donnell, GN; Giudici, TA; Ng, WG; Oizumi, J; Shaw, KN1
Dempsey, NJ; Ginder, GD; Little, JA; Tuchman, M1
Bachollet, B; Bodemer, C; De Prost, Y; Fraitag, S; Poggi, F; Saudubray, JM; Teillac-Hamel, D1
Allen, RH; Kolhouse, JF; Stabler, SP1
Hoppel, CL; Minkler, PE1
Kennedy, DG; Kennedy, S; Young, PB1
Murrell, M; Narasimhan, P; Sharp, FR; Sklar, R; Swanson, RA1
Blum, D; Clercx, A; Corazza, F; Fondu, P; Mardens, Y1
Coelho, J; de Mello, CF; Rubin, MA; Souza, DO; Wajner, M1
de Freitas, MS; de Mattos-Dutra, A; Lisboa, CS; Pessoa-Pureur, R; Wajner, M1
Chadefaux-Vekemans, B; Garcia, MJ; Jakobs, C; Kamoun, P; Merinero, B; Pérez-Cerdá, C; Tonetti, C; Ugarte, M; Zittoun, J1
Rocha, MP; Santos, KD; Schlottfeldt, JL; Wajner, M; Wannmacher, CM1
de Mattos-Dutra, A; Pessoa-Pureur, R; Sampaio de Freitas, M; Wajner, M1
Chalmers, RA; Down, S; Halket, JM; Mallard, WG; Przyborowska, A; Stein, SE1
Allen, DL; DeRose, EF; Gabel, SA; London, RE1
Calabrese, V; Rizza, V1
Bevilaqua da Rocha, B; de Mattos-Dutra, A; Kommers, T; Meirelles, R; Pessoa-Pureur, R; Wajner, M; Wofchuk, ST1
Dutra-Filho, CS; Fontella, FU; Gassen, E; Klein, AB; Pulrolnik, V; Wajner, M; Wannmacher, CM1
de Hoffmann, E; Libert, R; Nassogne, MC; Schanck, A; Thillaye, M; Van Hoof, F; Vincent, MF1
Blau, N; Bonafé, L; Burlina, AB; Chamoles, NA; Heizmann, CW; Kuster, T; Troxler, H1
Chace, DH; DiPerna, JC; Johnson, RW; Kalas, TA; Naylor, EW1
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, M1
Brusque, AM; Pettenuzzo, LE; Raasch, JR; Rocha, HP; Trindade, VM; Wajne, M; Wannmacher, CM1
de Almeida, LM; Funchal, C; Loureiro, Sde O; Pelaez, Pde L; Pessoa-Pureur, R; Pessutto, FD; Vivian, L; Wajner, M1
Walter, JH1
de Almeida, LM; Funchal, C; Loureiro, SO; Pelaez, Pde L; Pessoa-Pureur, R; Pessutto, FD; Vivian, L; Wajner, M1
Leonard, JV; Vijayaraghavan, S; Walter, JH1
Pettenuzzo, LF; Rosa, RB; Schuck, PF; Sitta, A; Wajner, M; Wannmacher, CM; Wyse, AT1
Hasegawa, Y; Hori, D; Kimura, M; Verma, IC; Yamaguchi, S; Yang, Y1
Benoist, JF; de Baulny, HO; Rabier, D; Rigal, O; Saudubray, JM; Touati, G1
de Lima Pelaez, P; Heimfarth, L; Loureiro, SO; Pessoa-Pureur, R; Souza, DO; Wajner, M1
Carrara, B; Gritti, P; Manzoni, D; Sonzogni, V; Spotti, A1
Gradowska, W; Krawczyk, H1
Endo, M; Hasegawa, Y; Kobayashi, H; Purevsuren, J; Yamaguchi, S1
Barshop, BA; Gangoiti, JA; Siuzdak, G; Wikoff, WR1
Bennett, MJ1
Van Gosen, L1
Abdoh, G; Burgard, P; Ho, S; Hoffmann, GF; Kölker, S; Lindner, M1
Botella, L; Eggeling, L; Lindley, ND1
Altiparmak, UE; Arnold, GL; Eggenberger, E; Feldon, SE; Hurley, PE; Mejico, LJ; Williams, ZR1
Fuller, GG; Hsu, TT; Nishimura, S; Sonner, JM; Weng, Y; Zhao, J1
Haarmann, A; Kölker, S; Morath, MA; Okun, JG; Opp, S; Sauer, SW1
Caggana, M; Cohen-Pfeffer, JL; Diaz, GA; Kirmse, BM; McGuire, PJ; Morrissey, MA; Salveson, BR; Sunny, S; Wasserstein, MP; Weisfeld-Adams, JD; Yu, C1
Biancini, GB; de Marco, MG; Manfredini, V; Ribas, GS; Vanzin, CS; Vargas, CR; Vieira, RB; Wajner, M; Wayhs, CY1
Colín-González, AL; Leipnitz, G; Paz-Loyola, AL; Ribeiro, CA; Santamaría, A; Seminotti, B; Serratos, I; Souza, DO; Wajner, M1
Colín-González, AL; Leipnitz, G; Paz-Loyola, AL; Ribeiro, CA; Santamaría, A; Seminotti, B; Serratos, IN; Souza, DO; Wajner, M1
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, S1

Reviews

2 review(s) available for propionic acid and methylmalonic acid

ArticleYear
Methylmalonic and propionic acidaemias: management and outcome.
    Journal of inherited metabolic disease, 2005, Volume: 28, Issue:3

    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.
    Journal of pediatric nursing, 2008, Volume: 23, Issue:3

    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

Other Studies

67 other study(ies) available for propionic acid and methylmalonic acid

ArticleYear
Misidentification of propionic acid as ethylene glycol in a patient with methylmalonic acidemia.
    The Journal of pediatrics, 1992, Volume: 120, Issue:3

    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.
    Pediatric research, 1991, Volume: 30, Issue:1

    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.
    Journal of inherited metabolic disease, 1991, Volume: 14, Issue:5

    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.
    Prenatal diagnosis, 1991, Volume: 11, Issue:9

    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.
    Clinica chimica acta; international journal of clinical chemistry, 1991, Dec-31, Volume: 204, Issue:1-3

    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.
    Pediatric research, 1991, Volume: 30, Issue:1

    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.
    Biochemical medicine and metabolic biology, 1991, Volume: 45, Issue:1

    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.
    Metabolism: clinical and experimental, 1990, Volume: 39, Issue:11

    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.
    Pediatric research, 1990, Volume: 27, Issue:4 Pt 1

    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.
    The Journal of pediatrics, 1987, Volume: 111, Issue:4

    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.
    Lancet (London, England), 1988, Jul-23, Volume: 2, Issue:8604

    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.
    Pediatric research, 1989, Volume: 25, Issue:2

    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.
    Journal of inherited metabolic disease, 1988, Volume: 11 Suppl 2

    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.
    The Biochemical journal, 1988, Sep-01, Volume: 254, Issue:2

    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.
    Pediatric research, 1985, Volume: 19, Issue:1

    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).
    Journal of inherited metabolic disease, 1984, Volume: 7 Suppl 1

    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.
    Journal of inherited metabolic disease, 1984, Volume: 7 Suppl 1

    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.
    Journal of inherited metabolic disease, 1984, Volume: 7 Suppl 1

    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.
    Journal of inherited metabolic disease, 1984, Volume: 7 Suppl 2

    Topics: Acids; Gas Chromatography-Mass Spectrometry; Humans; Metabolism, Inborn Errors; Methylmalonic Acid; Propionates

1984
Experience with prenatal diagnosis of propionic acidaemia and methylmalonic aciduria.
    Journal of inherited metabolic disease, 1984, Volume: 7 Suppl 2

    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.
    Archives of neurology, 1984, Volume: 41, Issue:12

    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.
    Clinica chimica acta; international journal of clinical chemistry, 1984, May-16, Volume: 139, Issue:1

    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.
    Biochemical medicine, 1981, Volume: 26, Issue:1

    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.
    Blood, 1995, Apr-01, Volume: 85, Issue:7

    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.
    The British journal of dermatology, 1994, Volume: 131, Issue:1

    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.
    Archives of biochemistry and biophysics, 1993, Volume: 303, Issue:2

    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.
    Analytical biochemistry, 1993, Aug-01, Volume: 212, Issue:2

    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.
    International journal for vitamin and nutrition research. Internationale Zeitschrift fur Vitamin- und Ernahrungsforschung. Journal international de vitaminologie et de nutrition, 1996, Volume: 66, Issue:1

    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.
    The Journal of neuroscience : the official journal of the Society for Neuroscience, 1996, Nov-15, Volume: 16, Issue:22

    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.
    Biology of the neonate, 1996, Volume: 70, Issue:5

    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.
    Biochemistry and molecular biology international, 1997, Volume: 42, Issue:6

    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.
    Neurochemistry international, 1998, Volume: 33, Issue:1

    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.
    Prenatal diagnosis, 1998, Volume: 18, Issue:9

    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.
    Clinical science (London, England : 1979), 1999, Volume: 96, Issue:1

    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.
    Neurochemistry international, 1998, Volume: 33, Issue:5

    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.
    Rapid communications in mass spectrometry : RCM, 1999, Volume: 13, Issue:4

    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.
    Journal of bacteriology, 1999, Volume: 181, Issue:11

    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.
    Alcohol (Fayetteville, N.Y.), 1999, Volume: 19, Issue:2

    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.
    Brain research, 2000, Feb-21, Volume: 856, Issue:1-2

    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.
    Neuroreport, 2000, Feb-28, Volume: 11, Issue:3

    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.
    Clinica chimica acta; international journal of clinical chemistry, 2000, Volume: 295, Issue:1-2

    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.
    Molecular genetics and metabolism, 2000, Volume: 69, Issue:4

    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.
    Clinical chemistry, 2001, Volume: 47, Issue:11

    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.
    Brain research, 2001, Nov-30, Volume: 920, Issue:1-2

    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.
    Metabolic brain disease, 2002, Volume: 17, Issue:2

    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.
    Neurochemical research, 2002, Volume: 27, Issue:12

    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?
    Journal of inherited metabolic disease, 2003, Volume: 26, Issue:2-3

    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.
    Metabolic brain disease, 2003, Volume: 18, Issue:3

    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.
    European journal of pediatrics, 2003, Volume: 162 Suppl 1

    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.
    Neurochemistry international, 2004, Volume: 45, Issue:5

    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.
    Brain & development, 2005, Volume: 27, Issue:1

    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.
    Biochimica et biophysica acta, 2005, Jun-10, Volume: 1740, Issue:3

    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.
    Pediatric transplantation, 2006, Volume: 10, Issue:5

    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.
    Journal of inherited metabolic disease, 2007, Volume: 30, Issue:2

    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.
    Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 2007, Volume: 855, Issue:1

    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.
    Clinical chemistry, 2007, Volume: 53, Issue:12

    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.
    Clinical chemistry, 2007, Volume: 53, Issue:12

    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.
    Journal of inherited metabolic disease, 2008, Volume: 31, Issue:3

    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.
    Journal of bacteriology, 2009, Volume: 191, Issue:8

    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.
    American journal of ophthalmology, 2009, Volume: 147, Issue:5

    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.
    Anesthesia and analgesia, 2009, Volume: 108, Issue:5

    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.
    Journal of inherited metabolic disease, 2009, Volume: 32, Issue:6

    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.
    Molecular genetics and metabolism, 2010, Volume: 99, Issue:2

    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.
    Mutation research, 2010, Sep-30, Volume: 702, Issue:1

    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.
    Neuroscience, 2015, Nov-12, Volume: 308

    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.
    Neuroscience, 2015, Dec-03, Volume: 310

    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.
    European journal of medical genetics, 2018, Volume: 61, Issue:8

    Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Brain Diseases; Humans; Male; Methionine; Methylmalonic Acid; Propionates; Protein Serine-Threonine Kinases; Syndrome

2018