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succinic acid and Maple Syrup Urine Disease

succinic acid has been researched along with Maple Syrup Urine Disease in 2 studies

Succinic Acid: A water-soluble, colorless crystal with an acid taste that is used as a chemical intermediate, in medicine, the manufacture of lacquers, and to make perfume esters. It is also used in foods as a sequestrant, buffer, and a neutralizing agent. (Hawley's Condensed Chemical Dictionary, 12th ed, p1099; McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed, p1851)
succinic acid : An alpha,omega-dicarboxylic acid resulting from the formal oxidation of each of the terminal methyl groups of butane to the corresponding carboxy group. It is an intermediate metabolite in the citric acid cycle.

Maple Syrup Urine Disease: An autosomal recessive inherited disorder with multiple forms of phenotypic expression, caused by a defect in the oxidative decarboxylation of branched-chain amino acids (AMINO ACIDS, BRANCHED-CHAIN). These metabolites accumulate in body fluids and render a maple syrup odor. The disease is divided into classic, intermediate, intermittent, and thiamine responsive subtypes. The classic form presents in the first week of life with ketoacidosis, hypoglycemia, emesis, neonatal seizures, and hypertonia. The intermediate and intermittent forms present in childhood or later with acute episodes of ataxia and vomiting. (From Adams et al., Principles of Neurology, 6th ed, p936)

Research

Studies (2)

TimeframeStudies, this research(%)All Research%
pre-19901 (50.00)18.7374
1990's0 (0.00)18.2507
2000's1 (50.00)29.6817
2010's0 (0.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Vasques, Vde C1
Brinco, F1
Wajner, M1
Shigematsu, Y1
Kikuchi, K1
Momoi, T1
Sudo, M1
Kikawa, Y1
Nosaka, K1
Kuriyama, M1
Haruki, S1
Sanada, K1
Hamano, N1

Other Studies

2 other studies available for succinic acid and Maple Syrup Urine Disease

ArticleYear
Intrahippocampal administration of the branched-chain alpha-hydroxy acids accumulating in maple syrup urine disease compromises rat performance in aversive and non-aversive behavioral tasks.
    Journal of the neurological sciences, 2005, May-15, Volume: 232, Issue:1-2

    Topics: Animals; Antioxidants; Avoidance Learning; Behavior, Animal; Creatine; Dizocilpine Maleate; Excitato

2005
Organic acids and branched-chain amino acids in body fluids before and after multiple exchange transfusions in maple syrup urine disease.
    Journal of inherited metabolic disease, 1983, Volume: 6, Issue:4

    Topics: Amino Acids, Branched-Chain; Body Fluids; Carboxylic Acids; Exchange Transfusion, Whole Blood; Femal

1983