glutaric acid has been researched along with Acquired Metabolic Diseases, Brain in 40 studies
glutaric acid: RN given refers to parent cpd
glutaric acid : An alpha,omega-dicarboxylic acid that is a linear five-carbon dicarboxylic acid.
| Excerpt | Relevance | Reference |
|---|---|---|
| "Glutaric acidemia type I (GA-I) is an inherited metabolic disease characterized by accumulation of glutaric acid (GA) and seizures." | 7.78 | m-Trifluoromethyl diphenyl diselenide attenuates glutaric acid-induced seizures and oxidative stress in rat pups: involvement of the γ-aminobutyric acidergic system. ( Brüning, CA; Fighera, MR; Gai, BM; Magni, DV; Nogueira, CW; Quines, CB; Rosa, SG, 2012) |
| " Antiquitin deficiency is the most common form of pyridoxine-dependent epilepsy." | 5.05 | Inherited Disorders of Lysine Metabolism: A Review. ( Bouchereau, J; Schiff, M, 2020) |
| "Glutaric acidemia type I (GA-I) is an inherited metabolic disease characterized by accumulation of glutaric acid (GA) and seizures." | 3.78 | m-Trifluoromethyl diphenyl diselenide attenuates glutaric acid-induced seizures and oxidative stress in rat pups: involvement of the γ-aminobutyric acidergic system. ( Brüning, CA; Fighera, MR; Gai, BM; Magni, DV; Nogueira, CW; Quines, CB; Rosa, SG, 2012) |
| "Glutaryl-CoA dehydrogenase deficiency or glutaric acidemia type I (GA I) is an inherited neurometabolic disorder biochemically characterized by tissue accumulation of predominantly glutaric (GA) and 3-hydroxyglutaric (3OHGA) acids and clinically by severe neurological symptoms and structural brain abnormalities, manifested as progressive cerebral atrophy and acute striatum degeneration following encephalopathic crises, whose pathophysiology is still in debate." | 3.76 | Induction of S100B secretion in C6 astroglial cells by the major metabolites accumulating in glutaric acidemia type I. ( de Souza, DF; Gonçalves, CA; Leipnitz, G; Quincozes-Santos, A; Rosa, RB; Seminotti, B; Wajner, M, 2010) |
| "A 9-month-old patient was admitted with encephalopathy and acute loss of acquired motor skills during the course of COVID-19 disease." | 1.62 | COVID-19 triggered encephalopathic crisis in a patient with glutaric aciduria type 1. ( Ahmadzada, S; Aktuglu-Zeybek, C; Kiykim, E; Yalcinkaya, C; Zubarioglu, T, 2021) |
| "Glutaric acidemia I (GA-I) is an inherited neurometabolic childhood disease characterized by bilateral striatal neurodegeneration upon brain accumulation of millimolar concentrations of glutaric acid (GA) and related metabolites." | 1.51 | Glutaric Acid Affects Pericyte Contractility and Migration: Possible Implications for GA-I Pathogenesis. ( Abudara, V; Attwell, D; Isasi, E; Korte, N; Olivera-Bravo, S, 2019) |
| "Glutaric aciduria type I is a rare, autosomal recessive, inherited defect of glutaryl-CoA dehydrogenase." | 1.48 | Impairment of astrocytic glutaminolysis in glutaric aciduria type I. ( Ediga, RD; Kölker, S; Komatsuzaki, S; Okun, JG; Sauer, SW, 2018) |
| "Glutaric aciduria type I is an inherited defect in L-lysine, L-hydroxylysine and L-tryptophan degradation caused by deficiency of glutaryl-CoA dehydrogenase (GCDH)." | 1.42 | Multifactorial modulation of susceptibility to l-lysine in an animal model of glutaric aciduria type I. ( Blank, AE; Burgard, P; Koeller, DM; Kölker, S; Komatsuzaki, S; Mittelbronn, M; Okun, JG; Opp, S; Sauer, SW, 2015) |
| " Oxidative stress and excitotoxicity have been involved in the toxic pattern exerted by these organic acids." | 1.42 | 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. ( Colín-González, AL; Leipnitz, G; Paz-Loyola, AL; Ribeiro, CA; Santamaría, A; Seminotti, B; Serratos, IN; Souza, DO; Wajner, M, 2015) |
| "Glutaric acid (GA) is a neurotoxic metabolite that accumulates in the CNS of patients with glutaric acidemia-I (GA-I), a neurometabolic disease caused by deficient activity of glutaryl-CoA dehydrogenase." | 1.40 | White matter injury induced by perinatal exposure to glutaric acid. ( Barbeito, L; Casanova, G; Fernández, A; Isasi, E; Jiménez, M; Olivera-Bravo, S; Rosillo, JC; Sarlabós, MN, 2014) |
| "Glutaric aciduria type 1 is an autosomal recessive disorder caused by deficiency of glutaryl-coenzyme A dehydrogenase, with accumulation of glutaric acid, 3-hydroxyglutaric acid and glutaconic acid." | 1.39 | Brain MRI findings as an important diagnostic clue in glutaric aciduria type 1. ( Alfaiate, C; Carvalho, S; Diogo, L; Faria, A; Garcia, P; Loureiro, S; Nunes, J; Pais, RP, 2013) |
| "Glutaric acidemia type I is an inherited metabolic disorder caused by a severe deficiency of the mitochondrial glutaryl-CoA dehydrogenase activity leading to accumulation of predominantly glutaric and 3-hydroxyglutaric acids in the brain tissue of the affected patients." | 1.34 | Evidence for a synergistic action of glutaric and 3-hydroxyglutaric acids disturbing rat brain energy metabolism. ( 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, 2007) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 1 (2.50) | 18.7374 |
| 1990's | 1 (2.50) | 18.2507 |
| 2000's | 8 (20.00) | 29.6817 |
| 2010's | 22 (55.00) | 24.3611 |
| 2020's | 8 (20.00) | 2.80 |
| Authors | Studies |
|---|---|
| Zubarioglu, T | 1 |
| Ahmadzada, S | 1 |
| Yalcinkaya, C | 1 |
| Kiykim, E | 1 |
| Aktuglu-Zeybek, C | 1 |
| Märtner, EMC | 1 |
| Thimm, E | 1 |
| Guder, P | 1 |
| Schiergens, KA | 1 |
| Rutsch, F | 1 |
| Roloff, S | 1 |
| Marquardt, I | 1 |
| Das, AM | 1 |
| Freisinger, P | 1 |
| Grünert, SC | 1 |
| Krämer, J | 1 |
| Baumgartner, MR | 1 |
| Beblo, S | 1 |
| Haase, C | 1 |
| Dieckmann, A | 1 |
| Lindner, M | 1 |
| Näke, A | 1 |
| Hoffmann, GF | 3 |
| Mühlhausen, C | 5 |
| Walter, M | 1 |
| Garbade, SF | 1 |
| Maier, EM | 2 |
| Kölker, S | 6 |
| Boy, N | 3 |
| Wongkittichote, P | 1 |
| Hong, X | 1 |
| Master, SR | 1 |
| Kaur, S | 1 |
| Cuddapah, SR | 1 |
| He, M | 1 |
| Zhou, J | 1 |
| Li, G | 1 |
| Deng, L | 1 |
| Zhao, P | 1 |
| Zeng, Y | 1 |
| Qiu, X | 1 |
| Luo, J | 1 |
| Xu, L | 1 |
| Gelener, P | 1 |
| Severino, M | 1 |
| Diker, S | 1 |
| Teralı, K | 1 |
| Tuncel, G | 1 |
| Tuzlalı, H | 1 |
| Manara, E | 1 |
| Paolacci, S | 1 |
| Bertelli, M | 1 |
| Ergoren, MC | 1 |
| Leandro, J | 1 |
| Dodatko, T | 1 |
| DeVita, RJ | 1 |
| Chen, H | 1 |
| Stauffer, B | 1 |
| Yu, C | 1 |
| Houten, SM | 1 |
| Bouchereau, J | 1 |
| Schiff, M | 1 |
| Kılavuz, S | 1 |
| Bulut, D | 1 |
| Kor, D | 1 |
| Şeker-Yılmaz, B | 1 |
| Özcan, N | 1 |
| Incecik, F | 1 |
| Onan, B | 1 |
| Ceylaner, G | 1 |
| Önenli-Mungan, N | 1 |
| Komatsuzaki, S | 2 |
| Ediga, RD | 1 |
| Okun, JG | 4 |
| Sauer, SW | 2 |
| Vargas, CR | 2 |
| Ribas, GS | 1 |
| da Silva, JM | 1 |
| Sitta, A | 1 |
| Deon, M | 1 |
| de Moura Coelho, D | 1 |
| Wajner, M | 7 |
| Hafeez, A | 1 |
| Fatima, S | 1 |
| Chaudhry, N | 1 |
| Khadim, MT | 1 |
| Isasi, E | 2 |
| Korte, N | 1 |
| Abudara, V | 1 |
| Attwell, D | 1 |
| Olivera-Bravo, S | 3 |
| Shaik, M | 1 |
| T P, KV | 1 |
| Kamate, M | 1 |
| A B, V | 1 |
| Thies, B | 1 |
| Meyer-Schwesinger, C | 1 |
| Lamp, J | 2 |
| Schweizer, M | 1 |
| Koeller, DM | 4 |
| Ullrich, K | 3 |
| Braulke, T | 3 |
| Nunes, J | 1 |
| Loureiro, S | 1 |
| Carvalho, S | 1 |
| Pais, RP | 1 |
| Alfaiate, C | 1 |
| Faria, A | 1 |
| Garcia, P | 1 |
| Diogo, L | 1 |
| Fernández, A | 1 |
| Rosillo, JC | 1 |
| Jiménez, M | 1 |
| Casanova, G | 1 |
| Sarlabós, MN | 1 |
| Barbeito, L | 2 |
| Busanello, EN | 1 |
| Fernandes, CG | 1 |
| Martell, RV | 1 |
| Lobato, VG | 1 |
| Goodman, S | 1 |
| Woontner, M | 1 |
| de Souza, DO | 1 |
| Opp, S | 1 |
| Blank, AE | 1 |
| Mittelbronn, M | 1 |
| Burgard, P | 2 |
| Harting, I | 2 |
| Heringer, J | 2 |
| Seitz, A | 1 |
| Bendszus, M | 1 |
| Pouwels, PJ | 1 |
| Colín-González, AL | 2 |
| Paz-Loyola, AL | 2 |
| Serratos, I | 1 |
| Seminotti, B | 3 |
| Ribeiro, CA | 2 |
| Leipnitz, G | 3 |
| Souza, DO | 2 |
| Santamaría, A | 2 |
| Serratos, IN | 1 |
| Zhang, Y | 1 |
| Li, H | 2 |
| Ma, R | 1 |
| Mei, L | 1 |
| Wei, X | 1 |
| Liang, D | 1 |
| Wu, L | 1 |
| Assmann, B | 1 |
| Dixon, M | 1 |
| Fleissner, S | 1 |
| Greenberg, CR | 1 |
| Karall, D | 1 |
| Krawinkel, MB | 1 |
| Opladen, T | 1 |
| Posset, R | 1 |
| Sahm, K | 1 |
| Zschocke, J | 1 |
| Lisyova, J | 1 |
| Petrovic, R | 1 |
| Jurickova, K | 1 |
| Brennerova, K | 1 |
| Urbanova, D | 1 |
| Behulova, D | 1 |
| Bzduch, V | 1 |
| Chandoga, J | 1 |
| Quincozes-Santos, A | 1 |
| Rosa, RB | 1 |
| de Souza, DF | 1 |
| Gonçalves, CA | 1 |
| Mushimoto, Y | 1 |
| Fukuda, S | 1 |
| Hasegawa, Y | 1 |
| Kobayashi, H | 1 |
| Purevsuren, J | 1 |
| Taketani, T | 1 |
| Yamaguchi, S | 2 |
| Keyser, B | 2 |
| Tian, F | 1 |
| Fu, X | 1 |
| Gao, J | 1 |
| Zhang, C | 1 |
| Ning, Q | 1 |
| Luo, X | 1 |
| Magni, DV | 1 |
| Brüning, CA | 1 |
| Gai, BM | 1 |
| Quines, CB | 1 |
| Rosa, SG | 1 |
| Fighera, MR | 1 |
| Nogueira, CW | 1 |
| Pawlak, V | 1 |
| Ahlemeyer, B | 1 |
| Hörster, F | 1 |
| Mayatepek, E | 1 |
| Krieglstein, J | 1 |
| Köhr, G | 1 |
| de Oliveira Marques, F | 1 |
| Hagen, ME | 1 |
| Pederzolli, CD | 1 |
| Sgaravatti, AM | 1 |
| Durigon, K | 1 |
| Testa, CG | 1 |
| Wannmacher, CM | 2 |
| de Souza Wyse, AT | 1 |
| Dutra-Filho, CS | 2 |
| Martínez Granero, MA | 1 |
| Garcia Pérez, A | 1 |
| Martínez-Pardo, M | 2 |
| Parra, E | 1 |
| Ferreira, GC | 1 |
| Tonin, A | 1 |
| Schuck, PF | 1 |
| Viegas, CM | 1 |
| Ceolato, PC | 1 |
| Latini, A | 1 |
| Perry, ML | 1 |
| Wyse, AT | 1 |
| Burckhardt, BC | 1 |
| Hagos, Y | 1 |
| Burckhardt, G | 1 |
| Lukacs, Z | 1 |
| Brismar, J | 1 |
| Ozand, PT | 1 |
| Prats Viñas, J | 1 |
| Corral, I | 1 |
| Martínez Castrillo, JC | 1 |
| Gimeno, A | 1 |
| Stutchfield, P | 1 |
| Edwards, MA | 1 |
| Gray, RG | 1 |
| Crawley, P | 1 |
| Green, A | 1 |
5 reviews available for glutaric acid and Acquired Metabolic Diseases, Brain
| Article | Year |
|---|---|
Inherited Disorders of Lysine Metabolism: A Review.
Topics: 2-Aminoadipic Acid; Aldehyde Dehydrogenase; Amino Acid Metabolism, Inborn Errors; Arginine; Brain; B | 2020 |
A role of astrocytes in mediating postnatal neurodegeneration in Glutaric acidemia-type 1.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Astrocytes; Brain Diseases, Metabolic; Glutarates; Gl | 2015 |
Proposed recommendations for diagnosing and managing individuals with glutaric aciduria type I: second revision.
Topics: Amino Acid Metabolism, Inborn Errors; Brain Diseases, Metabolic; Dietary Supplements; Glutarates; Gl | 2017 |
[Organic acid disorders: cerebral organic acidemia].
Topics: Brain Diseases, Metabolic; Canavan Disease; Glutarates; Glycerol; Humans; Hydroxybutyrates; Infant; | 2002 |
Membrane translocation of glutaric acid and its derivatives.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Biological Transport; Brain Diseases, Metabolic; Cell | 2008 |
35 other studies available for glutaric acid and Acquired Metabolic Diseases, Brain
| Article | Year |
|---|---|
COVID-19 triggered encephalopathic crisis in a patient with glutaric aciduria type 1.
Topics: Amino Acid Metabolism, Inborn Errors; Brain; Brain Diseases; Brain Diseases, Metabolic; Carnitine; C | 2021 |
The biochemical subtype is a predictor for cognitive function in glutaric aciduria type 1: a national prospective follow-up study.
Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Brain Diseases, Metabolic; Child; Child Developmen | 2021 |
2-Methylglutaconic acid as a biomarker in routine urine organic acids leading to the diagnosis of glutaric acidemia type I in a low excretor.
Topics: Amino Acid Metabolism, Inborn Errors; Biomarkers; Brain Diseases, Metabolic; Creatinine; Glutarates; | 2023 |
Biochemical and molecular features of chinese patients with glutaric acidemia type 1 from Fujian Province, southeastern China.
Topics: Amino Acid Metabolism, Inborn Errors; Brain Diseases, Metabolic; China; East Asian People; Female; G | 2023 |
Adult-onset glutaric aciduria type I: rare presentation of a treatable disorder.
Topics: Adult; Age of Onset; Amino Acid Metabolism, Inborn Errors; Brain; Brain Diseases, Metabolic; Female; | 2020 |
Deletion of 2-aminoadipic semialdehyde synthase limits metabolite accumulation in cell and mouse models for glutaric aciduria type 1.
Topics: 2-Aminoadipic Acid; Amino Acid Metabolism, Inborn Errors; Animals; Brain; Brain Diseases, Metabolic; | 2020 |
The outcome of 41 Late-Diagnosed Turkish GA-1 Patients: A Candidate for the Turkish NBS.
Topics: Amino Acid Metabolism, Inborn Errors; Brain Diseases, Metabolic; Glutarates; Glutaryl-CoA Dehydrogen | 2021 |
Impairment of astrocytic glutaminolysis in glutaric aciduria type I.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Astrocytes; Brain Diseases, Metabolic; Cell Death; Ce | 2018 |
Selective Screening of Fatty Acids Oxidation Defects and Organic Acidemias by Liquid Chromatography/tandem Mass Spectrometry Acylcarnitine Analysis in Brazilian Patients.
Topics: Acyl-CoA Dehydrogenase; Amino Acid Metabolism, Inborn Errors; Brain Diseases, Metabolic; Brazil; Car | 2018 |
Dyskinesia in a Child: A Concern for a Rare Neuro-Metabolic Disorder.
Topics: Amino Acid Metabolism, Inborn Errors; Brain; Brain Diseases, Metabolic; Child, Preschool; Dyskinesia | 2019 |
Glutaric Acid Affects Pericyte Contractility and Migration: Possible Implications for GA-I Pathogenesis.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Astrocytes; Brain Diseases, Metabolic; Capillaries; C | 2019 |
Is Expanded Newborn Screening Adequate to Detect Indian Biochemical Low Excretor Phenotype Patients of Glutaric Aciduria Type I?
Topics: Amino Acid Metabolism, Inborn Errors; Brain Diseases, Metabolic; Child, Preschool; DNA Mutational An | 2019 |
Acute renal proximal tubule alterations during induced metabolic crises in a mouse model of glutaric aciduria type 1.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Brain Diseases, Metabolic; Disease Models, Animal; Gl | 2013 |
Brain MRI findings as an important diagnostic clue in glutaric aciduria type 1.
Topics: Amino Acid Metabolism, Inborn Errors; Brain; Brain Diseases, Metabolic; Child, Preschool; Female; Gl | 2013 |
White matter injury induced by perinatal exposure to glutaric acid.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Animals, Newborn; Brain Diseases, Metabolic; Cell Dea | 2014 |
Disturbance of the glutamatergic system by glutaric acid in striatum and cerebral cortex of glutaryl-CoA dehydrogenase-deficient knockout mice: possible implications for the neuropathology of glutaric acidemia type I.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Brain Diseases, Metabolic; Cerebral Cortex; Corpus St | 2014 |
Multifactorial modulation of susceptibility to l-lysine in an animal model of glutaric aciduria type I.
Topics: Aconitate Hydratase; Amino Acid Metabolism, Inborn Errors; Animals; Brain; Brain Diseases, Metabolic | 2015 |
(1)H-MRS in glutaric aciduria type 1: impact of biochemical phenotype and age on the cerebral accumulation of neurotoxic metabolites.
Topics: Adolescent; Adult; Age Factors; Amino Acid Metabolism, Inborn Errors; Brain; Brain Diseases, Metabol | 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.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Brain; Brain Diseases, Metabolic; Disease Models, Ani | 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, | 2015 |
Clinical and molecular investigation in Chinese patients with glutaric aciduria type I.
Topics: Amino Acid Metabolism, Inborn Errors; Base Sequence; Brain Diseases, Metabolic; Child; Child, Presch | 2016 |
GAI - distinct genotype and phenotype characteristics in reported Slovak patients.
Topics: Amino Acid Metabolism, Inborn Errors; Base Sequence; Brain Diseases, Metabolic; Carnitine; Early Dia | 2016 |
Induction of S100B secretion in C6 astroglial cells by the major metabolites accumulating in glutaric acidemia type I.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Astrocytes; Atrophy; Brain Diseases, Metabolic; Cell | 2010 |
Clinical and molecular investigation of 19 Japanese cases of glutaric acidemia type 1.
Topics: Amino Acid Metabolism, Inborn Errors; Brain Diseases, Metabolic; Child, Preschool; Female; Gene Orde | 2011 |
Glutaric aciduria type 1 metabolites impair the succinate transport from astrocytic to neuronal cells.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Astrocytes; Biological Transport; Brain; Brain Diseas | 2011 |
Caspase-3 mediates apoptosis of striatal cells in GA I rat model.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Apoptosis; Brain Diseases, Metabolic; Caspase 3; Cell | 2012 |
m-Trifluoromethyl diphenyl diselenide attenuates glutaric acid-induced seizures and oxidative stress in rat pups: involvement of the γ-aminobutyric acidergic system.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Anticonvulsants; Brain Diseases, Metabolic; Disease M | 2012 |
NMDA receptor activation and respiratory chain complex V inhibition contribute to neurodegeneration in d-2-hydroxyglutaric aciduria.
Topics: Adenosine Triphosphatases; Animals; Brain Diseases, Metabolic; Calcium; Carrier Proteins; Cell Cultu | 2002 |
Glutaric acid induces oxidative stress in brain of young rats.
Topics: Aging; Animals; Animals, Newborn; Brain; Brain Diseases, Metabolic; Catalase; Dose-Response Relation | 2003 |
[Macrocephaly the first manifestation of glutaric aciduria type I: the importance of early diagnosis].
Topics: Brain; Brain Diseases, Metabolic; Carnitine; Diagnosis, Differential; Glutarates; Humans; Infant; Ma | 2005 |
Evidence for a synergistic action of glutaric and 3-hydroxyglutaric acids disturbing rat brain energy metabolism.
Topics: Animals; Antioxidants; Brain; Brain Chemistry; Brain Diseases, Metabolic; Creatine Kinase; Drug Syne | 2007 |
CT and MR of the brain in glutaric acidemia type I: a review of 59 published cases and a report of 5 new patients.
Topics: Atrophy; Brain; Brain Damage, Chronic; Brain Diseases, Metabolic; Child, Preschool; Diagnosis, Diffe | 1995 |
[Glutaric aciduria type I: an organic acidemia without acidosis with severe movement disorders].
Topics: Adult; Amino Acid Metabolism, Inborn Errors; Brain Diseases, Metabolic; Carnitine; Child, Preschool; | 2001 |
[Glutaric aciduria type I: diagnosis in adulthood and phenotypic variability].
Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Brain; Brain Diseases, Metabolic; Carnitine | 2001 |
Glutaric aciduria type I misdiagnosed as Leigh's encephalopathy and cerebral palsy.
Topics: Amino Acid Metabolism, Inborn Errors; Brain Diseases, Metabolic; Cerebral Palsy; Child; Child, Presc | 1985 |