Compounds > glutamic acid

glutamic acid and BCKD Deficiency

glutamic acid has been researched along with BCKD Deficiency in 13 studies

Research

Studies (13)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's3 (23.08)18.2507
2000's6 (46.15)29.6817
2010's2 (15.38)24.3611
2020's2 (15.38)2.80

Authors

AuthorsStudies
Bernardo, HT; Candiotto, G; da Silva Lemos, I; da Silva, GL; Duarte, MB; Petronilho, F; Rico, EP; Streck, EL; Torres, CA; Wessler, LB1
Ahrens-Nicklas, RC; Jakher, Y; Xu, J1
Bigler, DC; Bunce, SC; Mack, J; Moore, GJ; Morton, DH; Muelly, ER; Strauss, KA1
Downes, GB; Friedrich, T; Lambert, AM; Masino, MA1
Dall Bello Pessutto, F; de Almeida, LM; de Lima Pelaez, P; Funchal, C; Loureiro, SO; Pessoa Pureur, R; Tchernin Wofchuk, S; Vivian, L; Wajner, M1
Funchal, C; Pureur, RP; Rosa, AM; Wajner, M; Wofchuk, S1
Daikhin, Y; Horyn, O; Lazarow, A; Luhovyy, B; Nissim, I; Yudkoff, M1
Honig, J; Kalmijn, M; Korein, J; Lange, B; Sansaricq, C1
Danks, DM; Jones, LN; Negri, AP; Peet, DJ; Rivett, DE1
Baab, PJ; Collins, RM; Huang, Y; Tildon, JT; Zielke, CL; Zielke, HR1
Farage, M; Reis, M; Wolosker, H1
Dutra-Filho, CS; Santos, CE; Souza, DO; Tasca, CI; Tavares, RG; Wajner, M1
Chuang, DT; Chuang, JL; Mandel, H; Sansaricq, C; Wynn, RM1

Reviews

2 review(s) available for glutamic acid and BCKD Deficiency

ArticleYear
Brain Branched-Chain Amino Acids in Maple Syrup Urine Disease: Implications for Neurological Disorders.
    International journal of molecular sciences, 2020, Oct-11, Volume: 21, Issue:20

    Topics: Amino Acids, Branched-Chain; Aspartic Acid; Brain; Central Nervous System; Disease Susceptibility; Glutamic Acid; Humans; Lactic Acid; Maple Syrup Urine Disease; Metabolic Networks and Pathways; Nervous System Diseases

2020
Brain amino acid requirements and toxicity: the example of leucine.
    The Journal of nutrition, 2005, Volume: 135, Issue:6 Suppl

    Topics: Adult; Amino Acids, Branched-Chain; Animals; Brain; Glutamic Acid; Humans; Leucine; Maple Syrup Urine Disease; Nutritional Requirements

2005

Other Studies

11 other study(ies) available for glutamic acid and BCKD Deficiency

ArticleYear
Exposure to leucine alters glutamate levels and leads to memory and social impairment in zebrafish.
    Metabolic brain disease, 2022, Volume: 37, Issue:8

    Topics: Amino Acids, Branched-Chain; Animals; Glutamic Acid; Leucine; Maple Syrup Urine Disease; Zebrafish

2022
Biochemical correlates of neuropsychiatric illness in maple syrup urine disease.
    The Journal of clinical investigation, 2013, Volume: 123, Issue:4

    Topics: Adolescent; Adult; Affect; Anxiety; Aspartic Acid; Attention; Brain; Case-Control Studies; Child; Child, Preschool; Creatine; Depression; Female; Glutamic Acid; Humans; Impulsive Behavior; Liver Transplantation; Male; Maple Syrup Urine Disease; Prevalence; Psychomotor Agitation; Risk; Young Adult

2013
Mutation of zebrafish dihydrolipoamide branched-chain transacylase E2 results in motor dysfunction and models maple syrup urine disease.
    Disease models & mechanisms, 2012, Volume: 5, Issue:2

    Topics: Acyltransferases; Amino Acids, Branched-Chain; Animals; Base Sequence; Brain; Disease Models, Animal; Gene Expression Regulation, Developmental; Gene Expression Regulation, Enzymologic; Glutamic Acid; Humans; Larva; Maple Syrup Urine Disease; Mutation; Neuromuscular Diseases; RNA, Messenger; Swimming; Zebrafish; Zebrafish Proteins

2012
alpha-Ketoisocaproic acid regulates phosphorylation of intermediate filaments in postnatal rat cortical slices through ionotropic glutamatergic receptors.
    Brain research. Developmental brain research, 2002, Dec-15, Volume: 139, Issue:2

    Topics: Animals; Animals, Newborn; Cerebral Cortex; Excitatory Amino Acid Agonists; Excitatory Amino Acid Antagonists; Glial Fibrillary Acidic Protein; Glutamic Acid; Intermediate Filament Proteins; Intermediate Filaments; Keto Acids; Maple Syrup Urine Disease; Neurons; Organ Culture Techniques; Phosphorylation; Rats; Rats, Wistar; Receptors, AMPA; Receptors, Glutamate; Receptors, Kainic Acid; Receptors, Metabotropic Glutamate; Receptors, N-Methyl-D-Aspartate; Synaptic Transmission; Vimentin

2002
Reduction of glutamate uptake into cerebral cortex of developing rats by the branched-chain alpha-keto acids accumulating in maple syrup urine disease.
    Neurochemical research, 2004, Volume: 29, Issue:4

    Topics: Animals; Cerebral Cortex; Glutamic Acid; In Vitro Techniques; Keto Acids; Kinetics; Maple Syrup Urine Disease; Rats; Rats, Wistar

2004
Maple syrup urine disease: clinical, EEG, and plasma amino acid correlations with a theoretical mechanism of acute neurotoxicity.
    The International journal of neuroscience, 1994, Volume: 79, Issue:1-2

    Topics: Amino Acids; Dose-Response Relationship, Drug; Electroencephalography; gamma-Aminobutyric Acid; Glutamic Acid; Humans; Leucine; Maple Syrup Urine Disease

1994
Hairs from patients with maple syrup urine disease show a structural defect in the fiber cuticle.
    The Journal of investigative dermatology, 1996, Volume: 106, Issue:3

    Topics: Amino Acids; Child; Eicosanoic Acids; Fatty Acids; Female; Glutamic Acid; Hair; Humans; Maple Syrup Urine Disease; Microscopy, Electron

1996
Effect of alpha-ketoisocaproate and leucine on the in vivo oxidation of glutamate and glutamine in the rat brain.
    Neurochemical research, 1997, Volume: 22, Issue:9

    Topics: Animals; Brain; Caproates; Glutamic Acid; Glutamine; Keto Acids; Leucine; Male; Maple Syrup Urine Disease; Microdialysis; Oxidation-Reduction; Rats

1997
Chloride-dependent inhibition of vesicular glutamate uptake by alpha-keto acids accumulated in maple syrup urine disease.
    Biochimica et biophysica acta, 2000, Jul-03, Volume: 1475, Issue:2

    Topics: Amino Acid Transport System X-AG; Animals; ATP-Binding Cassette Transporters; Biological Transport; Brain; Cell Membrane; Chlorides; Electrochemistry; Glutamic Acid; Hemiterpenes; Hydrogen-Ion Concentration; In Vitro Techniques; Keto Acids; Maple Syrup Urine Disease; Membrane Potentials; Rats; Synaptic Vesicles

2000
Inhibition of glutamate uptake into synaptic vesicles of rat brain by the metabolites accumulating in maple syrup urine disease.
    Journal of the neurological sciences, 2000, Dec-01, Volume: 181, Issue:1-2

    Topics: Animals; Brain; Glutamic Acid; Hemiterpenes; Isoleucine; Keto Acids; Leucine; Male; Maple Syrup Urine Disease; Neurons; Rats; Rats, Wistar; Synaptic Vesicles; Synaptosomes; Valine

2000
Biochemical basis of type IB (E1beta ) mutations in maple syrup urine disease. A prevalent allele in patients from the Druze kindred in Israel.
    The Journal of biological chemistry, 2001, Sep-28, Volume: 276, Issue:39

    Topics: 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide); Alleles; Arginine; Blotting, Western; Cell Line; Child; Child, Preschool; Chromatography, Gel; Dimerization; Dose-Response Relationship, Drug; Escherichia coli; Family Health; Female; Glutamic Acid; Guanidine; Heterozygote; Homozygote; Humans; Israel; Ketone Oxidoreductases; Kinetics; Male; Maple Syrup Urine Disease; Models, Molecular; Multienzyme Complexes; Mutation; Plasmids; Protein Binding; Sequence Analysis, DNA; Serine; Temperature; Time Factors

2001