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meglutol and Metabolic Acidosis

meglutol has been researched along with Metabolic Acidosis in 10 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.

Research Excerpts

ExcerptRelevanceReference
"Here, we report the case of a 17-year-old girl who presented in both ten months and five years of age a clinical picture characterized by lethargy leading to apnea and coma, hepatomegaly, hypoglycemia, metabolic acidosis, hyperammoniemia, elevated serum transaminases and absence of ketonuria."3.70[3-hydroxy-3-methylglutaric aciduria and recurrent Reye-like syndrome]. ( Castro-Gago, M; Eirís, J; Fernández-Prieto, R; Ribes, A; Rodríguez-García, J; Rodríguez-Segade, S, 1998)
"A 4-month-old infant with hypotonia and macrocephaly was diagnosed as having 3-hydroxy-3-methylglutaric aciduria, using gas chromatography and mass spectrometry and confirmatory enzyme studies."1.27Dizygotic twins with 3-hydroxy-3-methylglutaric aciduria; unusual presentation, family studies and dietary management. ( Chalmers, RA; de Sousa, C; Mistry, J; Stacey, TE; Timbrell, P; Tracey, BM; Whitelaw, A, 1985)
"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."1.273-Methylglutaconic and 3-methylglutaric aciduria in a patient with suspected 3-methylglutaconyl-CoA hydratase deficiency. ( Lehnert, W; Scharf, J; Wendel, U, 1985)

Research

Studies (10)

TimeframeStudies, this research(%)All Research%
pre-19905 (50.00)18.7374
1990's4 (40.00)18.2507
2000's0 (0.00)29.6817
2010's1 (10.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Santarelli, F1
Cassanello, M1
Enea, A1
Poma, F1
D'Onofrio, V1
Guala, G1
Garrone, G1
Puccinelli, P1
Caruso, U1
Porta, F1
Spada, M1
Mulder, I1
Van den Bergh, SG1
Ozand, PT1
Nyhan, WL1
al Aqeel, A1
Christodoulou, J1
Fiumara, A1
Barone, R1
Nigro, F1
Ribes, A2
Pavone, L1
Eirís, J1
Fernández-Prieto, R1
Rodríguez-García, J1
Rodríguez-Segade, S1
Castro-Gago, M1
Shinka, T1
Kuhara, T1
Inoue, Y1
Matsumoto, M1
Matsumoto, I1
Nakamura, H1
Irimichi, H1
Hasumi, K1
Endo, A1
Stacey, TE1
de Sousa, C1
Tracey, BM1
Whitelaw, A1
Mistry, J1
Timbrell, P1
Chalmers, RA1
Dasouki, M1
Buchanan, D1
Mercer, N1
Gibson, KM1
Thoene, J1
Haan, EA1
Scholem, RD1
Pitt, JJ1
Wraith, JE1
Brown, GK1
Lehnert, W1
Scharf, J1
Wendel, U1

Other Studies

10 other studies available for meglutol and Metabolic Acidosis

ArticleYear
A neonatal case of 3-hydroxy-3-methylglutaric-coenzyme A lyase deficiency.
    Italian journal of pediatrics, 2013, May-24, Volume: 39

    Topics: Acetyl-CoA C-Acetyltransferase; Acidosis; Acute Disease; Amino Acid Metabolism, Inborn Errors; Gluta

2013
Changes in rat-liver mitochondria during ketosis.
    The International journal of biochemistry, 1981, Volume: 13, Issue:4

    Topics: Acidosis; Acyl Coenzyme A; Adenosine Triphosphate; Animals; Calcium; Female; Freezing; Glutamate Deh

1981
Malonic aciduria.
    Brain & development, 1994, Volume: 16 Suppl

    Topics: Acidosis; Carboxy-Lyases; Fibroblasts; Glutaryl-CoA Dehydrogenase; Humans; Infant; Infant, Newborn;

1994
Pancreatitis and organic acidemias.
    The Journal of pediatrics, 1995, Volume: 126, Issue:5 Pt 1

    Topics: Acidosis; Acute Disease; Glutarates; Humans; Infant; Male; Meglutol; Pancreatitis

1995
[3-hydroxy-3-methylglutaric aciduria and recurrent Reye-like syndrome].
    Revista de neurologia, 1998, Volume: 26, Issue:154

    Topics: Acidosis; Adolescent; Apnea; Carnitine; Coma; Diagnosis, Differential; Fatty Liver; Female; Fibrobla

1998
GC/MS analysis of urine in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency.
    Acta paediatrica Japonica : Overseas edition, 1992, Volume: 34, Issue:2

    Topics: Acidosis; Chromatography, Gas; Female; Humans; Hyperglycemia; Infant, Newborn; Leucine Zippers; Mass

1992
Dizygotic twins with 3-hydroxy-3-methylglutaric aciduria; unusual presentation, family studies and dietary management.
    European journal of pediatrics, 1985, Volume: 144, Issue:2

    Topics: Acidosis; Brain; Chromatography, Gas; Diet; Diseases in Twins; Female; Glutarates; Humans; Infant; M

1985
3-Hydroxy-3-methylglutaric aciduria: response to carnitine therapy and fat and leucine restriction.
    Journal of inherited metabolic disease, 1987, Volume: 10, Issue:2

    Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Carnitine; Dietary Fats; Dietary Proteins; Female; G

1987
Episodes of severe metabolic acidosis in a patient with 3-methylglutaconic aciduria.
    European journal of pediatrics, 1987, Volume: 146, Issue:5

    Topics: Abnormalities, Multiple; Acidosis; Acidosis, Lactic; Child, Preschool; Citric Acid Cycle; Follow-Up

1987
3-Methylglutaconic and 3-methylglutaric aciduria in a patient with suspected 3-methylglutaconyl-CoA hydratase deficiency.
    European journal of pediatrics, 1985, Volume: 143, Issue:4

    Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Female; Fibroblasts; Glutarates; Humans; Hydro-Lyase

1985