meglutol has been researched along with Metabolism, Inborn Errors in 18 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.
Metabolism, Inborn Errors: Errors in metabolic processes resulting from inborn genetic mutations that are inherited or acquired in utero.
| Excerpt | Relevance | Reference |
|---|---|---|
| "Costeff syndrome is a rare genetic neuro-ophthalmological syndrome consisting of early-onset bilateral optic atrophy along with a progressive complex motor disorder with elevated levels of urinary 3-methylglutaconic acid and 3-methylglutaric acid." | 3.81 | The neuropsychological profile of patients with 3-methylglutaconic aciduria type III, Costeff syndrome. ( Anikster, Y; Ben-Zeev, B; Blumkin, L; Hassin-Baer, S; Lerman-Sagie, T; Lev, D; Schweiger, A; Sofer, S; Yahalom, G, 2015) |
| "Creatine excretion was measured in two patients with methylmalonic aciduria and two patients with 3-hydroxy-3-methylglutaric aciduria." | 1.28 | Creatine metabolism during metabolic perturbations in patients with organic acidurias. ( Chalmers, RA; Davies, SE; Iles, RA; Stacey, TE, 1990) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 4 (22.22) | 18.7374 |
| 1990's | 11 (61.11) | 18.2507 |
| 2000's | 2 (11.11) | 29.6817 |
| 2010's | 1 (5.56) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Sofer, S | 1 |
| Schweiger, A | 1 |
| Blumkin, L | 1 |
| Yahalom, G | 1 |
| Anikster, Y | 1 |
| Lev, D | 1 |
| Ben-Zeev, B | 1 |
| Lerman-Sagie, T | 1 |
| Hassin-Baer, S | 1 |
| Menao, S | 1 |
| López-Viñas, E | 1 |
| Mir, C | 1 |
| Puisac, B | 1 |
| Gratacós, E | 1 |
| Arnedo, M | 1 |
| Carrasco, P | 1 |
| Moreno, S | 1 |
| Ramos, M | 1 |
| Gil, MC | 1 |
| Pié, A | 1 |
| Ribes, A | 2 |
| Pérez-Cerda, C | 1 |
| Ugarte, M | 1 |
| Clayton, PT | 1 |
| Korman, SH | 1 |
| Serra, D | 1 |
| Asins, G | 1 |
| Ramos, FJ | 1 |
| Gómez-Puertas, P | 1 |
| Hegardt, FG | 3 |
| Casals, N | 3 |
| Pié, J | 3 |
| Hagberg, B | 1 |
| Hjalmarson, O | 1 |
| Lindstedt, S | 1 |
| Ransnäs, L | 1 |
| Steen, G | 1 |
| Ozand, PT | 1 |
| Nyhan, WL | 1 |
| al Aqeel, A | 1 |
| Christodoulou, J | 1 |
| Ibel, H | 1 |
| Endres, W | 1 |
| Hadorn, HB | 1 |
| Deufel, T | 1 |
| Paetzke, I | 1 |
| Duran, M | 1 |
| Kennaway, NG | 1 |
| Gibson, KM | 2 |
| Ferreira, G | 1 |
| Freitas, S | 1 |
| Pereira, SA | 1 |
| Martins, I | 1 |
| Tavares, E | 1 |
| Vilarinho, L | 1 |
| Casale, CH | 1 |
| Zapater, N | 2 |
| Castro-Gago, M | 1 |
| Rodriguez-Segade, S | 1 |
| Wanders, RJ | 2 |
| Lloberas, J | 1 |
| Rolland, MO | 1 |
| Leroux, B | 1 |
| Vidailhet, M | 1 |
| Divry, P | 1 |
| Halket, JM | 1 |
| Przyborowska, A | 1 |
| Stein, SE | 1 |
| Mallard, WG | 1 |
| Down, S | 1 |
| Chalmers, RA | 3 |
| Bonafé, L | 1 |
| Troxler, H | 1 |
| Kuster, T | 1 |
| Heizmann, CW | 1 |
| Chamoles, NA | 1 |
| Burlina, AB | 1 |
| Blau, N | 1 |
| Shinka, T | 1 |
| Kuhara, T | 1 |
| Inoue, Y | 1 |
| Matsumoto, M | 1 |
| Matsumoto, I | 1 |
| Nakamura, H | 1 |
| Irimichi, H | 1 |
| Hasumi, K | 1 |
| Endo, A | 1 |
| Chitayat, D | 1 |
| Chemke, J | 1 |
| Mamer, OA | 1 |
| Kronick, JB | 1 |
| McGill, JJ | 1 |
| Rosenblatt, B | 1 |
| Sweetman, L | 1 |
| Scriver, CR | 1 |
| Davies, SE | 1 |
| Iles, RA | 1 |
| Stacey, TE | 1 |
| Buchanan, DN | 2 |
| Muenzer, J | 1 |
| Thoene, JG | 2 |
| Mills, GA | 1 |
| Hill, MA | 1 |
| Buchanan, R | 1 |
| Corina, DL | 1 |
| Walker, V | 1 |
| Schutgens, RB | 1 |
| Zoeters, PH | 1 |
| Mistry, J | 1 |
| Penketh, R | 1 |
| McFadyen, IR | 1 |
18 other studies available for meglutol and Metabolism, Inborn Errors
| Article | Year |
|---|---|
The neuropsychological profile of patients with 3-methylglutaconic aciduria type III, Costeff syndrome.
Topics: Adult; Chorea; Cognition Disorders; Executive Function; Female; Follow-Up Studies; Humans; Intellige | 2015 |
Ten novel HMGCL mutations in 24 patients of different origin with 3-hydroxy-3-methyl-glutaric aciduria.
Topics: Alleles; Amino Acid Sequence; Arabs; Catalytic Domain; DNA Mutational Analysis; Ecuador; Europe; Gen | 2009 |
3-Methylglutaconic aciduria in two infants.
Topics: Creatinine; Female; Fibroblasts; Glutarates; Hemiterpenes; Humans; Hydroxymethylglutaryl-CoA Synthas | 1983 |
Malonic aciduria.
Topics: Acidosis; Carboxy-Lyases; Fibroblasts; Glutaryl-CoA Dehydrogenase; Humans; Infant; Infant, Newborn; | 1994 |
Multiple respiratory chain abnormalities associated with hypertrophic cardiomyopathy and 3-methylglutaconic aciduria.
Topics: Acidosis, Lactic; Cardiomyopathy, Hypertrophic; Electron Transport Complex IV; Glutarates; Humans; H | 1993 |
3-Hydroxy-3-methylglutaric aciduria in a girl with trisomy 21.
Topics: Child, Preschool; Down Syndrome; Female; Humans; Lyases; Meglutol; Metabolism, Inborn Errors; Portug | 1996 |
A two-base deletion in exon 6 of the 3-hydroxy-3-methylglutaryl coenzyme A lyase (HL) gene producing the skipping of exons 5 and 6 determines 3-hydroxy-3-methylglutaric aciduria.
Topics: Adolescent; Amino Acid Sequence; Blotting, Northern; Blotting, Southern; Female; Fibroblasts; Frames | 1997 |
Two missense point mutations in different alleles in the 3-hydroxy-3-methylglutaryl coenzyme A lyase gene produce 3-hydroxy-3-methylglutaric aciduria in a French patient.
Topics: Alleles; Amino Acid Sequence; Child, Preschool; DNA Mutational Analysis; Female; Humans; Meglutol; M | 1998 |
Deconvolution gas chromatography/mass spectrometry of urinary organic acids--potential for pattern recognition and automated identification of metabolic disorders.
Topics: Acyl-CoA Dehydrogenase; Autoanalysis; Child, Preschool; Dicarboxylic Acids; Fatty Acid Desaturases; | 1999 |
Evaluation of urinary acylglycines by electrospray tandem mass spectrometry in mitochondrial energy metabolism defects and organic acidurias.
Topics: Acyl-CoA Dehydrogenase; Adolescent; Adult; Amidohydrolases; Biotinidase; Carbon-Nitrogen Ligases; Ch | 2000 |
GC/MS analysis of urine in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency.
Topics: Acidosis; Chromatography, Gas; Female; Humans; Hyperglycemia; Infant, Newborn; Leucine Zippers; Mass | 1992 |
3-Methylglutaconic aciduria: a marker for as yet unspecified disorders and the relevance of prenatal diagnosis in a 'new' type ('type 4').
Topics: Amniotic Fluid; Child; Female; Fibroblasts; Glutarates; Humans; Hydro-Lyases; Male; Meglutol; Metabo | 1992 |
Creatine metabolism during metabolic perturbations in patients with organic acidurias.
Topics: 3-Hydroxybutyric Acid; Carnitine; Child; Child, Preschool; Creatine; Creatinine; Female; Humans; Hyd | 1990 |
Positive-ion thermospray liquid chromatography-mass spectrometry: detection of organic acidurias.
Topics: Argininosuccinic Acid; Carboxylic Acids; Chromatography, Thin Layer; Female; Humans; Infant; Infant, | 1990 |
3-Hydroxy-3-methylglutaric aciduria: a possible pitfall in diagnosis.
Topics: False Negative Reactions; Fibroblasts; Gas Chromatography-Mass Spectrometry; Humans; Meglutol; Metab | 1991 |
Photodiode array detection for liquid chromatographic profiling of carboxylic acids in physiological fluids: 3-hydroxy-3-methylglutaric aciduria.
Topics: Chromatography, High Pressure Liquid; Gas Chromatography-Mass Spectrometry; Glutarates; Humans; Infa | 1986 |
3-Hydroxy-3-methylglutaryl-CoA lyase in human skin fibroblasts: study of its properties and deficient activity in 3-hydroxy-3-methylglutaric aciduria patients using a simple spectrophotometric method.
Topics: Fibroblasts; Glutarates; Humans; Hydrogen-Ion Concentration; Meglutol; Metabolism, Inborn Errors; Ox | 1988 |
First trimester prenatal diagnosis of 3-hydroxy-3-methylglutaric aciduria.
Topics: Chorionic Villi Sampling; Female; Glutarates; Humans; Meglutol; Metabolism, Inborn Errors; Oxo-Acid- | 1989 |