glutaric acid and 3-hydroxyglutaric acid

glutaric acid has been researched along with 3-hydroxyglutaric acid in 47 studies

Research

Studies (47)

Timeframe	Studies, this research(%)	All Research%
pre-1990	0 (0.00)	18.7374
1990's	4 (8.51)	18.2507
2000's	32 (68.09)	29.6817
2010's	11 (23.40)	24.3611
2020's	0 (0.00)	2.80

Authors

Authors	Studies
Christensen, E; Deufel, T; Falter, C; Fautek, JD; Fingerhut, R; Flott-Rahmel, B; Jakobs, C; Ludolph, A; Musshoff, U; Schluff, P; Ullrich, K	1
Ahlemeyer, B; Hoffmann, GF; Kölker, S; Krieglstein, J	4
Christensen, E; Das, A; Flott-Rahmel, B; Jakobs, C; Lücke, T; Ludolph, A; Musshoff, U; Neu, A; Röper, R; Schluff, P; Steinfeld, R; Ullrich, K	1
Baric, I; Buckel, W; Feyh, P; Hoffmann, GF; Liesert, M; Wagner, L	1
Corral, I; Gimeno, A; Martínez Castrillo, JC; Martínez-Pardo, M	1
Bjugstad, KB; Freed, CR; Goodman, S; Zawada, WM	1
Ahlemeyer, B; Hoffmann, GF; Hörster, F; Kohlmüller, D; Kölker, S; Krieglstein, J; Mayatepek, E; Okun, JG; Wajner, M; Wyse, AT	1
Das, AM; Lücke, T; Ullrich, K	1
Capone, A; Gago, LC; Wegner, RK; Williams, GA	1
Bain, MD; Baric, I; Chalmers, RA; Feyh, P; Hoffmann, GF; Jakobs, C; Jeffrey, I; Kölker, S; Okun, JG; Pourfarzam, M; Schor, DS; Wagner, L; Zschocke, J	1
Hoffmann, GF; Koeller, DM; Kölker, S; Okun, JG	1
Christensen, E; Kristensen, AS; Lund, AM; Lund, TM; Schousboe, A	1
Lai, CC; Lee, CC; Lin, WD; Tsai, FJ; Wang, CH	1
de Mello, CF; Goodman, SI; Koeller, DM; Kölker, S; Mühlhausen, C; Okun, JG; Sauer, S; Wajner, M; Woontner, M	1
Del Bigio, MR; Funk, CB; Prasad, AN	1
Surtees, R; Varadkar, S	1
Goodman, SI; Hoffmann, GF; Koeller, DM; Kölker, S; Okun, JG; Strauss, KA	1
Bührer, C; Gerstner, B; Gratopp, A; Marcinkowski, M; Obladen, M; Sifringer, M	1
Al-Amoudi, M; Al-Dirbashi, OY; Al-Kahtani, K; Al-Odaib, A; El-Badaoui, F; Jacob, M; Rashed, MS	1
Hata, I; Naito, E; Sakura, N; Shigematsu, Y; Tajima, G; Tanaka, Y; Yorifuji, T	1
Braulke, T; Chalajour, F; Ergün, S; Freudenberg, F; Mühlhausen, C; Ott, N; Shahhossini, M; Thiem, J; Tilki, D; Ullrich, K	1
Antonetti, DA; Cheng, KC; Connor, JR; Goodman, SI; Lazovic, J; Smith, MB; Wolpert, EB; Woontner, M; Zinnanti, WJ	2
Crnic, LR; Fricker, G; Goodman, SI; Harding, CO; Hoffmann, GF; Hörster, F; Koeller, DM; Kölker, S; Mahringer, A; Mühlhausen, C; Müller, I; Okun, JG; Sauer, SW	1
Basekim, CC; Keskin, AT; Kizilkaya, E; Mutlu, H; Ozturk, E; Sildiroglu, HO; Sonmez, G	1
Ceolato, PC; Dutra-Filho, CS; Ferreira, GC; Latini, A; Perry, ML; Schuck, PF; Tonin, A; Vargas, CR; Viegas, CM; Wajner, M; Wannmacher, CM; Wyse, AT	1
Dalcin, KB; Dutra-Filho, CS; Leipnitz, G; Porciúncula, LO; Rosa, RB; Schmidt, AL; Souza, DO; Wajner, M; Wannmacher, CM; Winter, JS	1
Sauer, SW	1
Dalcin, KB; Ferreira, GC; Gerhardt, D; Porciúncula, LO; Ribeiro, CA; Rosa, RB; Salbego, CG; Schmidt, AL; Schuck, PF; Souza, DO; Wajner, M; Wofchuk, S; Wyse, AT	1
da Costa Ferreira, G; Netto, CA; Pereira, LO; Pettenuzzo, LF; Ribeiro, CA; Schuck, PF; Tonin, A; Viegas, CM; Wajner, M	1
Braulke, T; Burckhardt, BC; Burckhardt, G; Hagos, Y; Keyser, B; Lukacs, Z; Mühlhausen, C; Ullrich, K	1
Barbeito, L; Casanova, G; Cassina, P; Fernandez, A; Latini, A; Olivera, S; Rosillo, JC; Wajner, M	1
Campistol, J; Capdevila, A; De La Osa, A; García-Cazorla, A; Leist, A; Navarro-Sastre, A; Pérez-Dueñas, B; Pineda, M; Ribes, A; Serrano, M	1
de Souza, DF; Gonçalves, CA; Leipnitz, G; Quincozes-Santos, A; Rosa, RB; Seminotti, B; Wajner, M	1
Brauburger, K; Burckhardt, BC; Burckhardt, G	1
Braulke, T; Keyser, B; Koeller, DM; Lamp, J; Mühlhausen, C; Ullrich, K	1
Alfaiate, C; Carvalho, S; Diogo, L; Faria, A; Garcia, P; Loureiro, S; Nunes, J; Pais, RP	1
Blank, AE; Burgard, P; Koeller, DM; Kölker, S; Komatsuzaki, S; Mittelbronn, M; Okun, JG; Opp, S; Sauer, SW	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
Li, H; Liang, D; Ma, R; Mei, L; Wei, X; Wu, L; Zhang, Y	1
Assmann, B; Boy, N; Burgard, P; Dixon, M; Fleissner, S; Greenberg, CR; Harting, I; Heringer, J; Hoffmann, GF; Karall, D; Koeller, DM; Kölker, S; Krawinkel, MB; Maier, EM; Mühlhausen, C; Okun, JG; Opladen, T; Posset, R; Sahm, K; Zschocke, J	1
Behulova, D; Brennerova, K; Bzduch, V; Chandoga, J; Jurickova, K; Lisyova, J; Petrovic, R; Urbanova, D	1
A B, V; Kamate, M; Shaik, M; T P, KV	1

Reviews

5 review(s) available for glutaric acid and 3-hydroxyglutaric acid

Article	Year
Pathomechanisms of neurodegeneration in glutaryl-CoA dehydrogenase deficiency. Annals of neurology, 2004, Volume: 55, Issue:1 Topics: Amino Acid Metabolism, Inborn Errors; Animals; Brain Diseases, Metabolic, Inborn; Child, Preschool; Glutarates; Glutaryl-CoA Dehydrogenase; Humans; Infant; Models, Neurological; Nerve Degeneration; Oxidoreductases Acting on CH-CH Group Donors	2004
Glutaric aciduria type I and kynurenine pathway metabolites: a modified hypothesis. Journal of inherited metabolic disease, 2004, Volume: 27, Issue:6 Topics: Amino Acid Metabolism, Inborn Errors; Animals; Glutarates; Humans; Kynurenine; Phenotype	2004
Challenges for basic research in glutaryl-CoA dehydrogenase deficiency. Journal of inherited metabolic disease, 2004, Volume: 27, Issue:6 Topics: Amino Acid Metabolism, Inborn Errors; Animals; Glutarates; Glutaryl-CoA Dehydrogenase; Humans; Neostriatum; Neurons; Oxidoreductases Acting on CH-CH Group Donors	2004
Membrane translocation of glutaric acid and its derivatives. Journal of inherited metabolic disease, 2008, Volume: 31, Issue:2 Topics: Amino Acid Metabolism, Inborn Errors; Animals; Biological Transport; Brain Diseases, Metabolic; Cell Membrane; Glutarates; Glutaryl-CoA Dehydrogenase; Humans; Kidney; Neurons	2008
Proposed recommendations for diagnosing and managing individuals with glutaric aciduria type I: second revision. Journal of inherited metabolic disease, 2017, Volume: 40, Issue:1 Topics: Amino Acid Metabolism, Inborn Errors; Brain Diseases, Metabolic; Dietary Supplements; Glutarates; Glutaryl-CoA Dehydrogenase; Humans; Lysine	2017

Trials

1 trial(s) available for glutaric acid and 3-hydroxyglutaric acid

Article	Year
Sensitivity and specificity of free and total glutaric acid and 3-hydroxyglutaric acid measurements by stable-isotope dilution assays for the diagnosis of glutaric aciduria type I. Journal of inherited metabolic disease, 1999, Volume: 22, Issue:8 Topics: Amino Acid Metabolism, Inborn Errors; Amniotic Fluid; Calibration; Child, Preschool; Creatinine; Female; Gas Chromatography-Mass Spectrometry; Glutarates; Glutaryl-CoA Dehydrogenase; Humans; Infant; Isotope Labeling; Male; Oxidoreductases; Oxidoreductases Acting on CH-CH Group Donors; Radioisotope Dilution Technique	1999

Article

Year

Sensitivity and specificity of free and total glutaric acid and 3-hydroxyglutaric acid measurements by stable-isotope dilution assays for the diagnosis of glutaric aciduria type I.

Journal of inherited metabolic disease, 1999, Volume: 22, Issue:8

Topics: Amino Acid Metabolism, Inborn Errors; Amniotic Fluid; Calibration; Child, Preschool; Creatinine; Female; Gas Chromatography-Mass Spectrometry; Glutarates; Glutaryl-CoA Dehydrogenase; Humans; Infant; Isotope Labeling; Male; Oxidoreductases; Oxidoreductases Acting on CH-CH Group Donors; Radioisotope Dilution Technique

1999

Other Studies

41 other study(ies) available for glutaric acid and 3-hydroxyglutaric acid

Article	Year
Nerve cell lesions caused by 3-hydroxyglutaric acid: a possible mechanism for neurodegeneration in glutaric acidaemia I. Journal of inherited metabolic disease, 1997, Volume: 20, Issue:3 Topics: Adenosine Triphosphate; Amino Acid Metabolism, Inborn Errors; Animals; Electrophysiology; Glutarates; Hippocampus; Nerve Degeneration; Nervous System Diseases; Neurons; Organ Culture Techniques; Rats	1997
3-Hydroxyglutaric and glutaric acids are neurotoxic through NMDA receptors in vitro. Journal of inherited metabolic disease, 1999, Volume: 22, Issue:3 Topics: Animals; Cell Survival; Cells, Cultured; Chick Embryo; Excitatory Amino Acid Antagonists; Glutarates; Neurons; Receptors, N-Methyl-D-Aspartate	1999
Glutaric aciduria type I: pathomechanisms of neurodegeneration. Journal of inherited metabolic disease, 1999, Volume: 22, Issue:4 Topics: Animals; Cerebral Cortex; Culture Techniques; Glutarates; Hippocampus; Humans; Rats; Rats, Wistar; Receptors, N-Methyl-D-Aspartate	1999
Maturation-dependent neurotoxicity of 3-hydroxyglutaric and glutaric acids in vitro: a new pathophysiologic approach to glutaryl-CoA dehydrogenase deficiency. Pediatric research, 2000, Volume: 47, Issue:4 Pt 1 Topics: Animals; Antibodies; Brain Diseases, Metabolic, Inborn; Cells, Cultured; Chick Embryo; Dizocilpine Maleate; Glutarates; Glutaryl-CoA Dehydrogenase; Neurons; Oxidoreductases; Oxidoreductases Acting on CH-CH Group Donors; Piperidines; Rats	2000
Cerebral organic acid disorders induce neuronal damage via excitotoxic organic acids in vitro. Amino acids, 2000, Volume: 18, Issue:1 Topics: Age Factors; Animals; Blotting, Western; Cell Survival; Cells, Cultured; Chick Embryo; Dose-Response Relationship, Drug; Electrophoresis, Polyacrylamide Gel; Excitatory Amino Acids; Glutarates; Glutaryl-CoA Dehydrogenase; L-Lactate Dehydrogenase; Neurons; Oxidoreductases; Oxidoreductases Acting on CH-CH Group Donors; Telencephalon; Time Factors	2000
Evaluation of trigger factors of acute encephalopathy in glutaric aciduria type I: fever and tumour necrosis factor-alpha. Journal of inherited metabolic disease, 2000, Volume: 23, Issue:4 Topics: Animals; Brain Diseases; Cells, Cultured; Chick Embryo; Dose-Response Relationship, Drug; Fever; Glutarates; Neurons; Tumor Necrosis Factor-alpha	2000
[Glutaric aciduria type I: diagnosis in adulthood and phenotypic variability]. Neurologia (Barcelona, Spain), 2001, Volume: 16, Issue:8 Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Brain; Brain Diseases, Metabolic; Carnitine; Fibroblasts; Glutarates; Glutaryl-CoA Dehydrogenase; Humans; Infant; Magnetic Resonance Imaging; Movement Disorders; Oxidoreductases; Oxidoreductases Acting on CH-CH Group Donors; Riboflavin; Tomography, X-Ray Computed	2001
IGF-1 and bFGF reduce glutaric acid and 3-hydroxyglutaric acid toxicity in striatal cultures. Journal of inherited metabolic disease, 2001, Volume: 24, Issue:6 Topics: Animals; Cell Death; Cells, Cultured; Cerebral Cortex; Excitatory Amino Acid Antagonists; Fibroblast Growth Factor 2; Glutarates; Growth Substances; Hydrogen-Ion Concentration; Immunohistochemistry; Insulin; Insulin-Like Growth Factor I; Male; Neostriatum; Nerve Degeneration; Quinolinic Acid; Rats; Rats, Sprague-Dawley; Tryptophan	2001
Chronic treatment with glutaric acid induces partial tolerance to excitotoxicity in neuronal cultures from chick embryo telencephalons. Journal of neuroscience research, 2002, May-15, Volume: 68, Issue:4 Topics: Animals; Blotting, Western; Calcium; Cell Culture Techniques; Chick Embryo; Down-Regulation; Drug Tolerance; Electrophoresis, Polyacrylamide Gel; Glutarates; Mitochondria; Reactive Oxygen Species; Receptors, N-Methyl-D-Aspartate; Sodium-Potassium-Exchanging ATPase; Telencephalon	2002
Glutaric aciduria I: creatine supplementation restores creatinephosphate levels in mixed cortex cells from rat incubated with 3-hydroxyglutarate. Molecular genetics and metabolism, 2003, Volume: 78, Issue:2 Topics: Adenine Nucleotides; Animals; Animals, Newborn; Cerebral Cortex; Creatine; Glutarates; Metabolism, Inborn Errors; Phosphocreatine; Rats	2003
Intraretinal hemorrhages and chronic subdural effusions: glutaric aciduria type 1 can be mistaken for shaken baby syndrome. Retina (Philadelphia, Pa.), 2003, Volume: 23, Issue:5 Topics: Amino Acid Metabolism, Inborn Errors; Diagnosis, Differential; Glutarates; Glutaryl-CoA Dehydrogenase; Hematoma, Subdural, Chronic; Humans; Infant; Magnetic Resonance Imaging; Male; Oxidoreductases Acting on CH-CH Group Donors; Retinal Hemorrhage; Shaken Baby Syndrome; Tomography, X-Ray Computed	2003
Glutaryl-CoA dehydrogenase deficiency: region-specific analysis of organic acids and acylcarnitines in post mortem brain predicts vulnerability of the putamen. Neuropediatrics, 2003, Volume: 34, Issue:5 Topics: Acidosis; Acute Disease; Adolescent; Anticonvulsants; Atrophy; Brain; Carnitine; DNA Mutational Analysis; Fatal Outcome; Gas Chromatography-Mass Spectrometry; Gene Expression; Glutarates; Glutaryl-CoA Dehydrogenase; Humans; Male; Muscle Hypotonia; N-Methylaspartate; Oxidoreductases Acting on CH-CH Group Donors; Point Mutation; Putamen; Spasm; Vigabatrin	2003
On the neurotoxicity of glutaric, 3-hydroxyglutaric, and trans-glutaconic acids in glutaric acidemia type 1. Journal of neuroscience research, 2004, Jul-01, Volume: 77, Issue:1 Topics: Animals; Brain Diseases, Metabolic, Inborn; Cell Death; Cells, Cultured; Cerebral Cortex; Corpus Striatum; Fetus; Glutarates; Glutaryl-CoA Dehydrogenase; Membrane Potentials; Mice; Nerve Degeneration; Neurons; Neurotoxins; Oocytes; Oxidoreductases Acting on CH-CH Group Donors; Rats; Receptors, N-Methyl-D-Aspartate; Xenopus	2004
Genetic and biochemical study in a patient with glutaric acidemia type I. Journal of the Formosan Medical Association = Taiwan yi zhi, 2004, Volume: 103, Issue:7 Topics: Amino Acid Metabolism, Inborn Errors; Carnitine; Child, Preschool; Glutarates; Glutaryl-CoA Dehydrogenase; Humans; Male; Mutation; Oxidoreductases Acting on CH-CH Group Donors	2004
Animal models for glutaryl-CoA dehydrogenase deficiency. Journal of inherited metabolic disease, 2004, Volume: 27, Issue:6 Topics: Amino Acid Metabolism, Inborn Errors; Animals; Chiroptera; Disease Models, Animal; Glutarates; Glutaryl-CoA Dehydrogenase; Injections; Mice; Mice, Knockout; Neostriatum; Oxidoreductases Acting on CH-CH Group Donors	2004
Preliminary attempts to establish a rat model of striatal injury in glutaric acidaemia type I. Journal of inherited metabolic disease, 2004, Volume: 27, Issue:6 Topics: Amino Acid Metabolism, Inborn Errors; Animals; Disease Models, Animal; Glutarates; Injections; Lipopolysaccharides; Male; Necrosis; Neostriatum; Neurons; Rats; Rats, Sprague-Dawley	2004
Glutaric acid and its metabolites cause apoptosis in immature oligodendrocytes: a novel mechanism of white matter degeneration in glutaryl-CoA dehydrogenase deficiency. Pediatric research, 2005, Volume: 57, Issue:6 Topics: Amino Acid Chloromethyl Ketones; Animals; Apoptosis; Base Sequence; Brain Diseases, Metabolic, Inborn; Caspase Inhibitors; Cell Differentiation; Cell Line; Glutarates; Glutaryl-CoA Dehydrogenase; Humans; Nerve Degeneration; Oligodendroglia; Oxidoreductases Acting on CH-CH Group Donors; Rats; Receptors, Glutamate; RNA, Messenger	2005
Quantification of glutaric and 3-hydroxyglutaric acids in urine of glutaric acidemia type I patients by HPLC with intramolecular excimer-forming fluorescence derivatization. Clinica chimica acta; international journal of clinical chemistry, 2005, Volume: 359, Issue:1-2 Topics: Case-Control Studies; Chromatography, High Pressure Liquid; Glutarates; Humans; Metabolism, Inborn Errors; Reference Standards; Reproducibility of Results; Sensitivity and Specificity; Spectrometry, Fluorescence	2005
Stable-isotope dilution gas chromatography-mass spectrometric measurement of 3-hydroxyglutaric acid, glutaric acid and related metabolites in body fluids of patients with glutaric aciduria type 1 found in newborn screening. Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 2005, Aug-25, Volume: 823, Issue:1 Topics: Amino Acid Metabolism, Inborn Errors; Deuterium; Gas Chromatography-Mass Spectrometry; Glutarates; Humans; Infant, Newborn; Neonatal Screening; Reproducibility of Results; Sensitivity and Specificity	2005
Endothelial effects of 3-hydroxyglutaric acid: implications for glutaric aciduria type I. Pediatric research, 2006, Volume: 59, Issue:2 Topics: Actins; Antigens, CD; Cadherins; Endothelium, Vascular; Glutarates; Humans; Metabolism, Inborn Errors; Vascular Endothelial Growth Factor A	2006
A diet-induced mouse model for glutaric aciduria type I. Brain : a journal of neurology, 2006, Volume: 129, Issue:Pt 4 Topics: Amino Acid Metabolism, Inborn Errors; Animals; Blood-Brain Barrier; Capillary Permeability; Corpus Striatum; Diet; Dietary Proteins; Disease Models, Animal; Female; Glutarates; Lysine; Magnetic Resonance Imaging; Male; Mice; Mice, Inbred C57BL; Neurons; Survival Analysis; Tissue Culture Techniques	2006
Intracerebral accumulation of glutaric and 3-hydroxyglutaric acids secondary to limited flux across the blood-brain barrier constitute a biochemical risk factor for neurodegeneration in glutaryl-CoA dehydrogenase deficiency. Journal of neurochemistry, 2006, Volume: 97, Issue:3 Topics: Amino Acids; Animals; Biological Transport; Blood-Brain Barrier; Blotting, Western; Brain; Carnitine; Cells, Cultured; Dicarboxylic Acids; Disease Models, Animal; Endothelial Cells; Glucose; Glutarates; Glutaryl-CoA Dehydrogenase; Heart; Liver; Mice; Mice, Inbred C57BL; Mice, Knockout; Models, Biological; Muscles; Neurodegenerative Diseases; Risk Factors; Statistics, Nonparametric; Swine; Time Factors; Tissue Distribution	2006
New insights for glutaric aciduria type I. Brain : a journal of neurology, 2006, Volume: 129, Issue:Pt 8 Topics: Amino Acid Metabolism, Inborn Errors; Animals; Brain Diseases, Metabolic, Inborn; Diet; Disease Models, Animal; Glutarates; Mice	2006
Magnetic resonance imaging findings of adult-onset glutaric aciduria type I. Acta radiologica (Stockholm, Sweden : 1987), 2007, Volume: 48, Issue:5 Topics: Adipates; Adult; Amino Acid Metabolism, Inborn Errors; Glutarates; Glutaryl-CoA Dehydrogenase; Humans; Magnetic Resonance Spectroscopy; Male	2007
Evidence for a synergistic action of glutaric and 3-hydroxyglutaric acids disturbing rat brain energy metabolism. International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience, 2007, Volume: 25, Issue:6 Topics: Animals; Antioxidants; Brain; Brain Chemistry; Brain Diseases, Metabolic; Creatine Kinase; Drug Synergism; Energy Metabolism; Glucose; Glutarates; Lactic Acid; Multiple Acyl Coenzyme A Dehydrogenase Deficiency; Organ Culture Techniques; Oxidative Stress; Pyruvate Dehydrogenase Complex; Quinolinic Acid; Rats; Rats, Wistar; Reactive Oxygen Species	2007
Age and brain structural related effects of glutaric and 3-hydroxyglutaric acids on glutamate binding to plasma membranes during rat brain development. Cellular and molecular neurobiology, 2007, Volume: 27, Issue:6 Topics: Age Factors; Animals; Brain; Cell Membrane; Dose-Response Relationship, Drug; Glutamic Acid; Glutarates; Rats; Rats, Wistar	2007
Biochemistry and bioenergetics of glutaryl-CoA dehydrogenase deficiency. Journal of inherited metabolic disease, 2007, Volume: 30, Issue:5 Topics: Acyl Coenzyme A; Animals; Blood-Brain Barrier; Brain; Brain Diseases, Metabolic, Inborn; Capillary Permeability; Disease Models, Animal; Energy Metabolism; Glutarates; Glutaryl-CoA Dehydrogenase; Humans; Ketoglutarate Dehydrogenase Complex; Mice; Mice, Knockout; Neurons	2007
Evidence that glutaric acid reduces glutamate uptake by cerebral cortex of infant rats. Life sciences, 2007, Dec-14, Volume: 81, Issue:25-26 Topics: Acetylcysteine; Animals; Animals, Newborn; Cerebral Cortex; Excitatory Amino Acid Transporter 1; Excitatory Amino Acid Transporter 2; Glutamates; Glutarates; Glutaryl-CoA Dehydrogenase; In Vitro Techniques; Kainic Acid; Neostriatum; Rats; Rats, Wistar; Synaptosomes	2007
Chronic early postnatal glutaric acid administration causes cognitive deficits in the water maze. Behavioural brain research, 2008, Mar-05, Volume: 187, Issue:2 Topics: Age Factors; Analysis of Variance; Animals; Animals, Newborn; Brain; Cognition Disorders; Drug Administration Schedule; Glutarates; Male; Maze Learning; Multiple Acyl Coenzyme A Dehydrogenase Deficiency; Neurotoxins; Rats; Rats, Wistar; Statistics, Nonparametric	2008
Astrocytic proliferation and mitochondrial dysfunction induced by accumulated glutaric acidemia I (GAI) metabolites: possible implications for GAI pathogenesis. Neurobiology of disease, 2008, Volume: 32, Issue:3 Topics: Amino Acid Metabolism, Inborn Errors; Animals; Animals, Newborn; Anthracenes; Antioxidants; Astrocytes; Brain; Brain Diseases, Metabolic, Inborn; Butadienes; Cell Count; Cell Proliferation; Cell Survival; Cells, Cultured; Glutarates; Immunohistochemistry; Membrane Potential, Mitochondrial; Mitochondria; Nitriles; Porphyrins; Rats; Rats, Sprague-Dawley	2008
Brain injury in glutaric aciduria type I: the value of functional techniques in magnetic resonance imaging. European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society, 2009, Volume: 13, Issue:6 Topics: Amino Acid Metabolism, Inborn Errors; Aspartic Acid; Brain; Brain Injuries; Brain Mapping; Creatine; Glutarates; Glutaryl-CoA Dehydrogenase; Humans; Infant; Magnetic Resonance Imaging; Magnetic Resonance Spectroscopy; Retrospective Studies	2009
Induction of S100B secretion in C6 astroglial cells by the major metabolites accumulating in glutaric acidemia type I. Metabolic brain disease, 2010, Volume: 25, Issue:2 Topics: Amino Acid Metabolism, Inborn Errors; Animals; Astrocytes; Atrophy; Brain Diseases, Metabolic; Cell Line, Tumor; Corpus Striatum; Gliosis; Glutarates; Glutaryl-CoA Dehydrogenase; Glutathione; Lipid Peroxidation; Nerve Growth Factors; Oxidative Stress; Rats; S100 Calcium Binding Protein beta Subunit; S100 Proteins; Thiobarbituric Acid Reactive Substances	2010
The sodium-dependent di- and tricarboxylate transporter, NaCT, is not responsible for the uptake of D-, L-2-hydroxyglutarate and 3-hydroxyglutarate into neurons. Journal of inherited metabolic disease, 2011, Volume: 34, Issue:2 Topics: Animals; Biological Transport; Electrophysiology; Glutarates; Humans; Kidney; Kinetics; Neurons; Oocytes; Patch-Clamp Techniques; RNA, Complementary; Symporters; Transcription, Genetic; Xenopus laevis	2011
Glutaric aciduria type 1 metabolites impair the succinate transport from astrocytic to neuronal cells. The Journal of biological chemistry, 2011, May-20, Volume: 286, Issue:20 Topics: Amino Acid Metabolism, Inborn Errors; Animals; Astrocytes; Biological Transport; Brain; Brain Diseases, Metabolic; Cell Death; Cell Line, Transformed; Citric Acid Cycle; Glutarates; Glutaryl-CoA Dehydrogenase; Mice; Mice, Knockout; Neurons; Organic Anion Transporters; Succinic Acid	2011
Brain MRI findings as an important diagnostic clue in glutaric aciduria type 1. The neuroradiology journal, 2013, Volume: 26, Issue:2 Topics: Amino Acid Metabolism, Inborn Errors; Brain; Brain Diseases, Metabolic; Child, Preschool; Female; Glutarates; Glutaryl-CoA Dehydrogenase; Humans; Infant; Magnetic Resonance Imaging; Retrospective Studies	2013
Multifactorial modulation of susceptibility to l-lysine in an animal model of glutaric aciduria type I. Biochimica et biophysica acta, 2015, Volume: 1852, Issue:5 Topics: Aconitate Hydratase; Amino Acid Metabolism, Inborn Errors; Animals; Brain; Brain Diseases, Metabolic; Diet; Disease Models, Animal; Dose-Response Relationship, Drug; Energy Metabolism; Female; Genetic Predisposition to Disease; Glutarates; Glutaryl-CoA Dehydrogenase; Glyceraldehyde-3-Phosphate Dehydrogenases; Ketoglutarate Dehydrogenase Complex; Lysine; Male; Mice, 129 Strain; Mice, Inbred C57BL; Mice, Knockout; Risk Factors; Sex Factors; Spectrophotometry	2015
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
Clinical and molecular investigation in Chinese patients with glutaric aciduria type I. Clinica chimica acta; international journal of clinical chemistry, 2016, Jan-30, Volume: 453 Topics: Amino Acid Metabolism, Inborn Errors; Base Sequence; Brain Diseases, Metabolic; Child; Child, Preschool; DNA Mutational Analysis; Female; Glutarates; Glutaryl-CoA Dehydrogenase; Heterozygote; Humans; Male; Models, Molecular; Mutation; Protein Conformation	2016
GAI - distinct genotype and phenotype characteristics in reported Slovak patients. Bratislavske lekarske listy, 2016, Volume: 117, Issue:11 Topics: Amino Acid Metabolism, Inborn Errors; Base Sequence; Brain Diseases, Metabolic; Carnitine; Early Diagnosis; Female; Gas Chromatography-Mass Spectrometry; Genotype; Glutarates; Glutaryl-CoA Dehydrogenase; Humans; Infant, Newborn; Male; Mutation, Missense; Phenotype; Sequence Analysis; Slovakia	2016
Is Expanded Newborn Screening Adequate to Detect Indian Biochemical Low Excretor Phenotype Patients of Glutaric Aciduria Type I? Indian journal of pediatrics, 2019, Volume: 86, Issue:11 Topics: Amino Acid Metabolism, Inborn Errors; Brain Diseases, Metabolic; Child, Preschool; DNA Mutational Analysis; Female; Glutarates; Glutaryl-CoA Dehydrogenase; Humans; India; Infant; Infant, Newborn; Male; Mutation; Neonatal Screening; Phenotype; Tandem Mass Spectrometry	2019