meglutol has been researched along with Amino Acid Metabolism Disorders, Inborn in 24 studies
Meglutol: An antilipemic agent which lowers cholesterol, triglycerides, serum beta-lipoproteins and phospholipids. It acts by interfering with the enzymatic steps involved in the conversion of acetate to hydroxymethylglutaryl coenzyme A as well as inhibiting the activity of HYDROXYMETHYLGLUTARYL COA REDUCTASES which is the rate limiting enzyme in the biosynthesis of cholesterol.
3-hydroxy-3-methylglutaric acid : A dicarboxylic acid that is glutaric acid in which one of the two hydrogens at position 3 is substituted by a hydroxy group, while the other is substituted by a methyl group. It has been found to accumulate in urine of patients suffering from HMG-CoA lyase (3-hydroxy-3-methylglutaryl-CoA lyase, EC 4.1.3.4) deficiency. It occurs as a plant metabolite in Crotalaria dura.
| Excerpt | Relevance | Reference |
|---|---|---|
| "A girl suffering from marked muscular hypotonia, severe statomotor and mental retardation, bilateral optic atrophy with chorioretinal degeneration, convulsions and a moderate compensated metabolic acidosis is described." | 1.27 | 3-Methylglutaconic and 3-methylglutaric aciduria in a patient with suspected 3-methylglutaconyl-CoA hydratase deficiency. ( Lehnert, W; Scharf, J; Wendel, U, 1985) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 10 (41.67) | 18.7374 |
| 1990's | 4 (16.67) | 18.2507 |
| 2000's | 3 (12.50) | 29.6817 |
| 2010's | 7 (29.17) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Roland, D | 1 |
| Jissendi-Tchofo, P | 1 |
| Briand, G | 1 |
| Vamecq, J | 1 |
| Fontaine, M | 1 |
| Ultré, V | 1 |
| Acquaviva-Bourdain, C | 1 |
| Mention, K | 1 |
| Dobbelaere, D | 1 |
| Delgado, CA | 1 |
| Balbueno Guerreiro, GB | 1 |
| Diaz Jacques, CE | 1 |
| de Moura Coelho, D | 2 |
| Sitta, A | 2 |
| Manfredini, V | 1 |
| Wajner, M | 4 |
| Vargas, CR | 2 |
| Fernandes, CG | 2 |
| da Rosa, MS | 1 |
| Seminotti, B | 2 |
| Pierozan, P | 1 |
| Martell, RW | 1 |
| Lagranha, VL | 1 |
| Busanello, EN | 1 |
| Leipnitz, G | 1 |
| Santarelli, F | 1 |
| Cassanello, M | 1 |
| Enea, A | 1 |
| Poma, F | 1 |
| D'Onofrio, V | 1 |
| Guala, G | 1 |
| Garrone, G | 1 |
| Puccinelli, P | 1 |
| Caruso, U | 1 |
| Porta, F | 1 |
| Spada, M | 1 |
| Dos Santos Mello, M | 1 |
| Ribas, GS | 1 |
| Wayhs, CA | 1 |
| Hammerschmidt, T | 1 |
| Guerreiro, GB | 1 |
| Favenzani, JL | 1 |
| Rodrigues, MDN | 1 |
| Colín-González, AL | 1 |
| Santamaria, A | 1 |
| Quincozes-Santos, A | 1 |
| Pierron, S | 1 |
| Giudicelli, H | 1 |
| Moreigne, M | 1 |
| Khalfi, A | 1 |
| Touati, G | 1 |
| Caruba, C | 1 |
| Rolland, MO | 1 |
| Acquaviva, C | 1 |
| Engelke, UF | 1 |
| Kremer, B | 1 |
| Kluijtmans, LA | 1 |
| van der Graaf, M | 1 |
| Morava, E | 1 |
| Loupatty, FJ | 1 |
| Wanders, RJ | 1 |
| Moskau, D | 1 |
| Loss, S | 1 |
| van den Bergh, E | 1 |
| Wevers, RA | 1 |
| Mir, C | 1 |
| Lopez-Viñas, E | 1 |
| Aledo, R | 1 |
| Puisac, B | 1 |
| Rizzo, C | 1 |
| Dionisi-Vici, C | 1 |
| Deodato, F | 1 |
| Pié, J | 1 |
| Gomez-Puertas, P | 1 |
| Hegardt, FG | 1 |
| Casals, N | 1 |
| Jakobs, C | 1 |
| Bojasch, M | 1 |
| Duran, M | 4 |
| Ketting, D | 3 |
| Wadman, SK | 4 |
| Leupold, D | 1 |
| Ketel, A | 2 |
| Ket, JL | 1 |
| Schutgens, RB | 3 |
| Beemer, FA | 1 |
| Tibosch, AS | 1 |
| Bruinvis, L | 1 |
| Chalmers, RA | 4 |
| Stacey, TE | 2 |
| Tracey, BM | 2 |
| de Sousa, C | 1 |
| Roe, CR | 1 |
| Millington, DS | 1 |
| Hoppel, CL | 1 |
| Norman, EJ | 1 |
| Denton, MD | 1 |
| Berry, HK | 1 |
| Lerman-Sagie, T | 1 |
| Yoshida, I | 1 |
| Ensenauer, R | 1 |
| Müller, CB | 1 |
| Schwab, KO | 1 |
| Gibson, KM | 3 |
| Brandis, M | 1 |
| Lehnert, W | 2 |
| Heymans, H | 1 |
| Bertssen, MW | 1 |
| Thompson, GN | 1 |
| Halliday, D | 1 |
| Sherwood, WG | 1 |
| Hoffman, GF | 1 |
| Stumpf, DA | 1 |
| Dianzani, I | 1 |
| Barth, PG | 1 |
| Weismann, U | 1 |
| Bachmann, C | 1 |
| Schrynemackers-Pitance, P | 1 |
| Iles, RA | 1 |
| Jago, JR | 1 |
| Williams, SR | 1 |
| Dasouki, M | 1 |
| Buchanan, D | 1 |
| Mercer, N | 1 |
| Thoene, J | 1 |
| Mistry, J | 1 |
| McFadyen, IR | 1 |
| Scharf, J | 1 |
| Wendel, U | 1 |
1 review available for meglutol and Amino Acid Metabolism Disorders, Inborn
| Article | Year |
|---|---|
[3-Hydroxy-3-methylglutaryl-CoA-lyase deficiency].
Topics: Acyl Coenzyme A; Amino Acid Metabolism, Inborn Errors; Diagnosis, Differential; Humans; Leucine; Meg | 1998 |
23 other studies available for meglutol 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; Cerebe | 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, Inbo | 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 G | 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; Gluta | 2013 |
Increased oxidative stress in patients with 3-hydroxy-3-methylglutaric aciduria.
Topics: Acetyl-CoA C-Acetyltransferase; Adolescent; Amino Acid Metabolism, Inborn Errors; Case-Control Studi | 2015 |
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; Astrocy | 2016 |
[Late onset 3-HMG-CoA lyase deficiency: a rare but treatable disorder].
Topics: Alleles; Amino Acid Metabolism, Inborn Errors; Carnitine; Child, Preschool; Chromosome Aberrations; | 2010 |
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 | 2006 |
A single-residue mutation, G203E, causes 3-hydroxy-3-methylglutaric aciduria by occluding the substrate channel in the 3D structural model of HMG-CoA lyase.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acid Sequence; Animals; Child; DNA Mutational Analysis; | 2006 |
3-methyl-3-butenoic acid: an artefact in the urinary metabolic pattern of patients with 3-hydroxy-3-methylglutaryl-CoA lyase deficiency.
Topics: Acyl Coenzyme A; Amino Acid Metabolism, Inborn Errors; Butyrates; Crotonates; Humans; Meglutol; Oxo- | 1980 |
Clinical and biochemical observations on a child with a deficiency of 3-hydroxy-3-methylglutaryl coenzyme A lyase.
Topics: Acyl Coenzyme A; Amino Acid Metabolism, Inborn Errors; Carboxylic Acids; Glucose; Humans; Infant; Me | 1980 |
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; Leu | 1982 |
L-Carnitine insufficiency in disorders of organic acid metabolism: response to L-carnitine by patients with methylmalonic aciduria and 3-hydroxy-3-methylglutaric aciduria.
Topics: Amino Acid Metabolism, Inborn Errors; Carnitine; Child; Glutarates; Humans; Malonates; Meglutol; Met | 1984 |
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; Huma | 1982 |
Behr syndrome.
Topics: Amino Acid Metabolism, Inborn Errors; Brain Diseases, Metabolic; Child; Glutarates; Humans; Meglutol | 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; Glutar | 2000 |
3-hydroxy-3-methylglutaryl coenzyme A lyase deficiency: postnatal management following prenatal diagnosis by analysis of maternal urine.
Topics: Acyl Coenzyme A; Amino Acid Metabolism, Inborn Errors; Female; Humans; Hypoglycemia; Infant, Newborn | 1979 |
The contribution of protein catabolism to metabolic decompensation in 3-hydroxy-3-methylglutaric aciduria.
Topics: Amino Acid Metabolism, Inborn Errors; Child; Diseases in Twins; Female; Glutarates; Humans; Leucine; | 1990 |
Phenotypic heterogeneity in the syndromes of 3-methylglutaconic aciduria.
Topics: Amino Acid Metabolism, Inborn Errors; Coenzyme A; Glutarates; Humans; Hydroxymethylglutaryl CoA Redu | 1991 |
3-Hydroxy-3-methylglutaryl-CoA lyase deficiency studied using 2-dimensional proton nuclear magnetic resonance spectroscopy.
Topics: Amino Acid Metabolism, Inborn Errors; Female; Humans; Hydroxy Acids; Hydroxymethylglutaryl-CoA Synth | 1986 |
3-Hydroxy-3-methylglutaric aciduria: response to carnitine therapy and fat and leucine restriction.
Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Carnitine; Dietary Fats; Dietary Proteins; Female; G | 1987 |
Prenatal diagnosis of 3-hydroxy-3-methylglutaric aciduria by GC-MS and enzymology on cultured amniocytes and chorionic villi.
Topics: Amino Acid Metabolism, Inborn Errors; Amnion; Cells, Cultured; Chorionic Villi; Consanguinity; Femal | 1989 |
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-Lyase | 1985 |