methylmalonic acid and malonic acid

methylmalonic acid has been researched along with malonic acid in 22 studies

Research

Studies (22)

TimeframeStudies, this research(%)All Research%
pre-19902 (9.09)18.7374
1990's4 (18.18)18.2507
2000's7 (31.82)29.6817
2010's7 (31.82)24.3611
2020's2 (9.09)2.80

Authors

AuthorsStudies
Ajani, H; Fanfrlík, J; Hobza, P; Jansa, P; Jirásková-Vaníčková, J; Konvalinka, J; Lepšík, M; Nachtigallová, D; Otyepka, M; Rezáč, J; Vorlová, B1
Heine, A; Heitefuss, S; Seifert, HS1
Bankier, A; Brown, GK; Danks, DM; Scholem, RD1
Fenner, WR; Genders, A; Nyhan, WL; Oglesbee, M; Podell, M; Shelton, GD; Wagner, SO1
Gregg, AR; O'Brien, WE; Thorburn, DR; Warman, AW1
Barshop, BA; Faunt, KK; Gibson, KM; Johnson, GC; O'Brien, DP; Shelton, GD1
Farkas, LM; Feyh, P; Hinz, A; Hoffmann, GF; Hörster, F; Kölker, S; Mayatepek, E; Okun, JG; Sauer, S; Unsicker, K1
Ogier de Baulny, H; Saudubray, JM1
Hinz, A; Hoffmann, GF; Hörster, F; Kölker, S; Mayatepek, E; Okun, JG; Sauer, S; Schwab, M; Smeitink, JA; Wolf, NI1
BARNABEI, O1
Castilho, RF; Kowaltowski, AJ; Maciel, EN; Rodrigues, JM; Schwalm, FD; Souza, DO; Vercesi, AE; Wajner, M1
Castilho, RF; Maciel, EN; Mirandola, SR; Saad, LO1
Castilho, RF; Ferreira, GC; Melo, DR; Mirandola, SR; Schuck, PF; Wajner, M1
Cohen, A; Grimberg, H; Levin, G; Reshef, A; Shirvan, A; Yogev-Falach, M; Ziv, I1
Al-Thihli, K; Alfares, A; Anastasio, N; Braverman, N; Ha, KC; Majewski, J; Melançon, S; Mitchell, J; Nunez, LD; Rosenblatt, DS1
Cheng, Z; Colak, G; Dai, L; de Boer, VC; Huang, H; Liu, X; Locasale, JW; Lombard, DB; Park, J; Pougovkina, O; Tan, M; Te Brinke, H; Wan, X; Wanders, RJ; Yue, WW; Zhao, Y1
Gu, X; Han, L; Liu, H; Qiu, W; Tan, D; Ye, J; Zhang, H1
Hoque, MM; Kanazawa, H; Kawai, G; Kondo, J; Suzuki, K; Takénaka, A; Tsunoda, M; Yamamoto, T1
Abu-Elheiga, LA; Ambati, CS; Shetty, V; Yuan, F; Zhang, Y1
Auray-Blais, C; Braverman, NE; Brunel-Guitton, C; Buhas, D; Clarke, JTR; Laframboise, R; Lévesque, S; Levtova, A; Maranda, B; Mitchell, GA; Waters, PJ1
Ko, JM; Lee, SH; Park, KS; Song, J; Song, MK1
Abily-Donval, L; Bekri, S; Goldenberg, A; Marret, S; Salomons, GS; Snanoudj, S; Sudrié-Arnaud, B; Tebani, A; Torre, S1

Reviews

1 review(s) available for methylmalonic acid and malonic acid

ArticleYear
Branched-chain organic acidurias.
    Seminars in neonatology : SN, 2002, Volume: 7, Issue:1

    Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids, Branched-Chain; Diagnosis, Differential; Glycine; Hemiterpenes; Humans; Infant, Newborn; Malonates; Maple Syrup Urine Disease; Methylmalonic Acid; Pentanoic Acids; Prognosis; Propionates

2002

Other Studies

21 other study(ies) available for methylmalonic acid and malonic acid

ArticleYear
Malonate-based inhibitors of mammalian serine racemase: kinetic characterization and structure-based computational study.
    European journal of medicinal chemistry, 2015, Jan-07, Volume: 89

    Topics: Animals; Crystallography, X-Ray; Dose-Response Relationship, Drug; Enzyme Inhibitors; Kinetics; Malonates; Mice; Models, Molecular; Molecular Structure; Racemases and Epimerases; Structure-Activity Relationship; Thermodynamics

2015
Detection of non-volatile organic acids by head-space gas chromatography.
    Journal of chromatography, 1990, Nov-16, Volume: 532, Issue:2

    Topics: Bacteroides fragilis; Carboxylic Acids; Chromatography, Gas; Fumarates; Lactates; Lactic Acid; Malonates; Methylmalonic Acid; Oxalates; Oxalic Acid; Pyruvates; Pyruvic Acid; Succinates; Succinic Acid

1990
Malonyl coenzyme A decarboxylase deficiency.
    Journal of inherited metabolic disease, 1984, Volume: 7, Issue:1

    Topics: Acyl Coenzyme A; Carboxy-Lyases; Child, Preschool; Fatty Acid Synthases; Fibroblasts; Humans; Male; Malonates; Malonyl Coenzyme A; Metabolism, Inborn Errors; Methylmalonic Acid; Methylmalonyl-CoA Mutase; Mitochondria

1984
Methylmalonic and malonic aciduria in a dog with progressive encephalomyelopathy.
    Metabolic brain disease, 1996, Volume: 11, Issue:3

    Topics: Animals; Brain; Dog Diseases; Dogs; Electromyography; Encephalomyelitis; Female; Gas Chromatography-Mass Spectrometry; Hydrocephalus; Malonates; Metabolism, Inborn Errors; Methylmalonic Acid; Muscles; Nerve Fibers

1996
Combined malonic and methylmalonic aciduria with normal malonyl-coenzyme A decarboxylase activity: a case supporting multiple aetiologies.
    Journal of inherited metabolic disease, 1998, Volume: 21, Issue:4

    Topics: Amino Acid Metabolism, Inborn Errors; Carboxy-Lyases; Child; Diet; Fibroblasts; Humans; Male; Malonates; Methylmalonic Acid

1998
Malonic aciduria in Maltese dogs: normal methylmalonic acid concentrations and malonyl-CoA decarboxylase activity in fibroblasts.
    Journal of inherited metabolic disease, 1999, Volume: 22, Issue:8

    Topics: Animals; Carboxy-Lyases; Diet; Dog Diseases; Dogs; Epilepsy, Tonic-Clonic; Female; Fibroblasts; Malonates; Metabolism, Inborn Errors; Methylmalonic Acid; Pedigree

1999
Neurodegeneration in methylmalonic aciduria involves inhibition of complex II and the tricarboxylic acid cycle, and synergistically acting excitotoxicity.
    The Journal of biological chemistry, 2002, Apr-26, Volume: 277, Issue:17

    Topics: Animals; Cells, Cultured; Citrates; Citric Acid Cycle; Corpus Striatum; Electron Transport Complex II; Female; Malonates; Metabolism, Inborn Errors; Methylmalonic Acid; Multienzyme Complexes; N-Methylaspartate; Neurons; Oxidoreductases; Pregnancy; Rats; Rats, Wistar; Succinate Dehydrogenase

2002
Methylmalonic acid, a biochemical hallmark of methylmalonic acidurias but no inhibitor of mitochondrial respiratory chain.
    The Journal of biological chemistry, 2003, Nov-28, Volume: 278, Issue:48

    Topics: Acyl Coenzyme A; Adenosine Triphosphate; Aging; Amino Acid Metabolism, Inborn Errors; Animals; Cattle; Citrates; Citric Acid Cycle; Dose-Response Relationship, Drug; Electron Transport; Female; Hydrogen-Ion Concentration; Male; Malonates; Methylmalonic Acid; Mice; Mice, Inbred C57BL; Mitochondria; Mitochondrial Diseases; Muscles; Myocardium; Oxygen; Phosphorylation; Pyruvates; Spectrophotometry; Time Factors

2003
[Formation of methylmalonic acid and malonic acid during the catabolism of pyrimidine bases].
    Bollettino della Societa italiana di biologia sperimentale, 1958, Jul-31, Volume: 34, Issue:14

    Topics: Malonates; Methylmalonic Acid; Pyrimidines

1958
Mitochondrial permeability transition in neuronal damage promoted by Ca2+ and respiratory chain complex II inhibition.
    Journal of neurochemistry, 2004, Volume: 90, Issue:5

    Topics: Animals; Antimycin A; Bongkrekic Acid; Brain; Calcimycin; Calcium; Catalase; Cell Survival; Cyclosporins; Dose-Response Relationship, Drug; Drug Interactions; Electron Transport Complex II; Enzyme Inhibitors; Female; In Vitro Techniques; Ionophores; Malonates; Membrane Potentials; Methylmalonic Acid; Mitochondria; NADP; Neurons; Nitro Compounds; Oxygen Consumption; PC12 Cells; Permeability; Propionates; Rats; Rotenone; Tacrolimus; Tetrazolium Salts; Thiazoles; Uncoupling Agents

2004
Lactate dehydrogenase activity is inhibited by methylmalonate in vitro.
    Neurochemical research, 2006, Volume: 31, Issue:4

    Topics: Animals; Brain; Cattle; Female; Humans; L-Lactate Dehydrogenase; Lactates; Liver; Malonates; Methylmalonic Acid; Muscle, Skeletal; Myocardium; Nitro Compounds; Propionates; Pyruvic Acid; Rats; Rats, Wistar

2006
Methylmalonate inhibits succinate-supported oxygen consumption by interfering with mitochondrial succinate uptake.
    Journal of inherited metabolic disease, 2008, Volume: 31, Issue:1

    Topics: Animals; Biological Transport, Active; Dicarboxylic Acid Transporters; Down-Regulation; Female; Malonates; Methylmalonic Acid; Mitochondria; Organ Culture Techniques; Oxygen Consumption; Rats; Rats, Wistar; Succinate Dehydrogenase; Succinic Acid

2008
Monitoring of tumor response to chemotherapy in vivo by a novel small-molecule detector of apoptosis.
    Apoptosis : an international journal on programmed cell death, 2009, Volume: 14, Issue:3

    Topics: Animals; Antibiotics, Antineoplastic; Antimetabolites, Antineoplastic; Antineoplastic Agents, Alkylating; Apoptosis; Biomarkers; Carmustine; Cell Line, Tumor; Doxorubicin; Drug Monitoring; Fluorouracil; Humans; Jurkat Cells; Malonates; Methylmalonic Acid; Mice; Mice, Inbred BALB C; Neoplasms

2009
Combined malonic and methylmalonic aciduria: exome sequencing reveals mutations in the ACSF3 gene in patients with a non-classic phenotype.
    Journal of medical genetics, 2011, Volume: 48, Issue:9

    Topics: Alleles; Amino Acid Metabolism, Inborn Errors; Base Sequence; Carboxy-Lyases; Coenzyme A Ligases; Exome; Genetic Association Studies; Humans; Infant; Malonates; Malonyl Coenzyme A; Metabolism, Inborn Errors; Methylmalonic Acid; Molecular Sequence Data; Mutation; Pedigree; Phenotype; Sequence Analysis, DNA

2011
Proteomic and Biochemical Studies of Lysine Malonylation Suggest Its Malonic Aciduria-associated Regulatory Role in Mitochondrial Function and Fatty Acid Oxidation.
    Molecular & cellular proteomics : MCP, 2015, Volume: 14, Issue:11

    Topics: Animals; Carboxy-Lyases; Cell Line; Fatty Acids; Fibroblasts; Humans; Liver; Lysine; Male; Malonates; Malonyl Coenzyme A; Metabolism, Inborn Errors; Methylmalonic Acid; Mice; Mice, Knockout; Mitochondria; Models, Molecular; Oxidation-Reduction; Sirtuins

2015
A new case of malonyl-CoA decarboxylase deficiency with mild clinical features.
    American journal of medical genetics. Part A, 2016, Volume: 170A, Issue:5

    Topics: Acidosis; Adolescent; Base Sequence; Carboxy-Lyases; Cardiomyopathies; Child; Chromosomes; Exons; Female; Humans; Malonates; Malonyl Coenzyme A; Metabolism, Inborn Errors; Methylmalonic Acid; Microarray Analysis; Sequence Deletion

2016
Structural insights into the catalytic reaction trigger and inhibition of D-3-hydroxybutyrate dehydrogenase.
    Acta crystallographica. Section F, Structural biology communications, 2016, Volume: 72, Issue:Pt 7

    Topics: 3-Hydroxybutyric Acid; Alcaligenes faecalis; Amino Acid Motifs; Bacterial Proteins; Catalytic Domain; Crystallography, X-Ray; Enzyme Inhibitors; Gene Expression; Glutamine; Hydrogen Bonding; Hydrophobic and Hydrophilic Interactions; Hydroxybutyrate Dehydrogenase; Malonates; Methylmalonic Acid; Models, Molecular; NAD; Protein Binding; Protein Conformation, alpha-Helical; Protein Conformation, beta-Strand; Protein Interaction Domains and Motifs; Protein Multimerization; Protein Subunits; Recombinant Proteins; Substrate Specificity; Tyrosine

2016
Identification and Quantitation of Malonic Acid Biomarkers of In-Born Error Metabolism by Targeted Metabolomics.
    Journal of the American Society for Mass Spectrometry, 2017, Volume: 28, Issue:5

    Topics: Animals; Biomarkers; Carboxy-Lyases; Female; Humans; Limit of Detection; Male; Malonates; Malonyl Coenzyme A; Mass Spectrometry; Metabolism, Inborn Errors; Metabolomics; Methylmalonic Acid; Mice, Inbred C57BL; Phenylhydrazines

2017
Combined malonic and methylmalonic aciduria due to ACSF3 mutations: Benign clinical course in an unselected cohort.
    Journal of inherited metabolic disease, 2019, Volume: 42, Issue:1

    Topics: Adolescent; Adult; Alleles; Child; Child, Preschool; Coenzyme A Ligases; Cohort Studies; Creatinine; Cross-Sectional Studies; Female; Humans; Infant; Infant, Newborn; Male; Malonates; Metabolism, Inborn Errors; Methylmalonic Acid; Mutation; Neonatal Screening; Retrospective Studies; Young Adult

2019
A Korean child diagnosed with malonic aciduria harboring a novel start codon mutation following presentation with dilated cardiomyopathy.
    Molecular genetics & genomic medicine, 2020, Volume: 8, Issue:9

    Topics: Carboxy-Lyases; Cardiomyopathy, Dilated; Codon, Initiator; Humans; Infant; Male; Malonates; Malonyl Coenzyme A; Metabolism, Inborn Errors; Methylmalonic Acid; Mutation; Phenotype

2020
Heterogenous Clinical Landscape in a Consanguineous Malonic Aciduria Family.
    International journal of molecular sciences, 2021, Nov-23, Volume: 22, Issue:23

    Topics: Carboxy-Lyases; Carnitine; Child, Preschool; Consanguinity; Homozygote; Humans; Male; Malonates; Malonyl Coenzyme A; Metabolism, Inborn Errors; Methylmalonic Acid; Pedigree; Point Mutation

2021