3-methylglutaric acid has been researched along with Amino Acid Metabolism Disorders, Inborn in 12 studies
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 3 (25.00) | 18.7374 |
| 1990's | 2 (16.67) | 18.2507 |
| 2000's | 2 (16.67) | 29.6817 |
| 2010's | 5 (41.67) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Acquaviva-Bourdain, C; Briand, G; Dobbelaere, D; Fontaine, M; Jissendi-Tchofo, P; Mention, K; Roland, D; Ultré, V; Vamecq, J | 1 |
| Balbueno Guerreiro, GB; de Moura Coelho, D; Delgado, CA; Diaz Jacques, CE; Manfredini, V; Sitta, A; Vargas, CR; Wajner, M | 1 |
| Busanello, EN; da Rosa, MS; Fernandes, CG; Lagranha, VL; Leipnitz, G; Martell, RW; Pierozan, P; Seminotti, B; Wajner, M | 1 |
| Caruso, U; Cassanello, M; D'Onofrio, V; Enea, A; Garrone, G; Guala, G; Poma, F; Porta, F; Puccinelli, P; Santarelli, F; Spada, M | 1 |
| Colín-González, AL; Fernandes, CG; Quincozes-Santos, A; Rodrigues, MDN; Santamaria, A; Seminotti, B; Wajner, M | 1 |
| Engelke, UF; Kluijtmans, LA; Kremer, B; Loss, S; Loupatty, FJ; Morava, E; Moskau, D; van den Bergh, E; van der Graaf, M; Wanders, RJ; Wevers, RA | 1 |
| Beemer, FA; Bruinvis, L; Duran, M; Ketting, D; Tibosch, AS; Wadman, SK | 1 |
| Berry, HK; Denton, MD; Norman, EJ | 1 |
| Lerman-Sagie, T | 1 |
| Brandis, M; Ensenauer, R; Gibson, KM; Lehnert, W; Müller, CB; Schwab, KO | 1 |
| Bachmann, C; Barth, PG; Dianzani, I; Gibson, KM; Hoffman, GF; Schrynemackers-Pitance, P; Schutgens, RB; Sherwood, WG; Stumpf, DA; Weismann, U | 1 |
| Lehnert, W; Scharf, J; Wendel, U | 1 |
12 other study(ies) available for 3-methylglutaric acid and Amino Acid Metabolism Disorders, Inborn
| Article | Year |
|---|---|
Coupled brain and urine spectroscopy - in vivo metabolomic characterization of HMG-CoA lyase deficiency in 5 patients.
Topics: Acetyl-CoA C-Acetyltransferase; Amino Acid Metabolism, Inborn Errors; Brain; Brain Chemistry; Cerebellum; Child; Child, Preschool; Female; Humans; Hydrogen-Ion Concentration; Infant; Infant, Newborn; Magnetic Resonance Imaging; Male; Meglutol; Metabolomics; Proton Magnetic Resonance Spectroscopy; Urine; Valerates; White Matter | 2017 |
Prevention by L-carnitine of DNA damage induced by 3-hydroxy-3-methylglutaric and 3-methylglutaric acids and experimental evidence of lipid and DNA damage in patients with 3-hydroxy-3-methylglutaric aciduria.
Topics: 8-Hydroxy-2'-Deoxyguanosine; Acetyl-CoA C-Acetyltransferase; Adolescent; Amino Acid Metabolism, Inborn Errors; Carnitine; Child; Child, Preschool; Dinoprost; DNA Damage; Guanine; Guanosine; Humans; Infant; Lipid Peroxidation; Meglutol | 2019 |
In vivo experimental evidence that the major metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency induce oxidative stress in striatum of developing rats: a potential pathophysiological mechanism of striatal damage in this disorder.
Topics: Acetyl-CoA C-Acetyltransferase; Amino Acid Metabolism, Inborn Errors; Animals; Antioxidants; Basal Ganglia; Catalase; Dizocilpine Maleate; Glutathione; Glutathione Peroxidase; Male; Malondialdehyde; Meglutol; Oxidative Stress; Protein Carbonylation; Rats; Rats, Wistar; Receptors, N-Methyl-D-Aspartate; Superoxide Dismutase; Vitamin E | 2013 |
A neonatal case of 3-hydroxy-3-methylglutaric-coenzyme A lyase deficiency.
Topics: Acetyl-CoA C-Acetyltransferase; Acidosis; Acute Disease; Amino Acid Metabolism, Inborn Errors; Glutarates; Glycine; Humans; Hypoglycemia; Infant, Newborn; Male; Meglutol | 2013 |
Induction of a Proinflammatory Response in Cortical Astrocytes by the Major Metabolites Accumulating in HMG-CoA Lyase Deficiency: the Role of ERK Signaling Pathway in Cytokine Release.
Topics: Acetyl-CoA C-Acetyltransferase; Amino Acid Metabolism, Inborn Errors; Animals; Antioxidants; Astrocytes; Cell Shape; Cells, Cultured; Cerebral Cortex; Cytokines; Flavonoids; Gliosis; Heme Oxygenase (Decyclizing); Inflammation; Male; MAP Kinase Signaling System; Meglutol; Metabolome; Mitochondria; NF-kappa B; Protoporphyrins; Rats, Wistar; Reactive Oxygen Species | 2016 |
NMR spectroscopic studies on the late onset form of 3-methylglutaconic aciduria type I and other defects in leucine metabolism.
Topics: Amino Acid Metabolism, Inborn Errors; Brain; Female; Glutarates; Humans; Leucine; Magnetic Resonance Spectroscopy; Meglutol; Middle Aged; Valerates | 2006 |
Inherited 3-methylglutaconic aciduria in two brothers--another defect of leucine metabolism.
Topics: Amino Acid Metabolism, Inborn Errors; Child; Child, Preschool; Glutarates; Humans; Hydro-Lyases; Leucine; Male; Meglutol; Speech Disorders; Valerates | 1982 |
Gas-chromatographic/mass spectrometric detection of 3-hydroxy-3-methylglutaryl-CoA lyase deficiency in double first cousins.
Topics: Amino Acid Metabolism, Inborn Errors; Female; Gas Chromatography-Mass Spectrometry; Glutarates; Humans; Hydroxy Acids; Infant; Leucine; Male; Meglutol; Oxo-Acid-Lyases; Valerates | 1982 |
Behr syndrome.
Topics: Amino Acid Metabolism, Inborn Errors; Brain Diseases, Metabolic; Child; Glutarates; Humans; Meglutol; Optic Atrophy; Syndrome | 1995 |
3-Methylglutaconyl-CoA hydratase deficiency: a new patient with speech retardation as the leading sign.
Topics: Amino Acid Metabolism, Inborn Errors; Cells, Cultured; Diet, Protein-Restricted; Fibroblasts; Glutarates; Humans; Hydro-Lyases; Meglutol; Speech Disorders; Valerates | 2000 |
Phenotypic heterogeneity in the syndromes of 3-methylglutaconic aciduria.
Topics: Amino Acid Metabolism, Inborn Errors; Coenzyme A; Glutarates; Humans; Hydroxymethylglutaryl CoA Reductases; Meglutol; Phenotype | 1991 |
3-Methylglutaconic and 3-methylglutaric aciduria in a patient with suspected 3-methylglutaconyl-CoA hydratase deficiency.
Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Female; Fibroblasts; Glutarates; Humans; Hydro-Lyases; Infant; Intellectual Disability; Meglutol; Muscle Hypotonia; Skin | 1985 |