Page last updated: 2024-10-17

chlorine and Maple Syrup Urine Disease

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

chloride : A halide anion formed when chlorine picks up an electron to form an an anion.

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 Excerpts

ExcerptRelevanceReference
"Maple syrup urine disease is a metabolic disorder caused by mutations of the branched chain keto acid dehydrogenase complex, leading to accumulation of alpha-keto acids and their amino acid precursors in the brain."1.31Chloride-dependent inhibition of vesicular glutamate uptake by alpha-keto acids accumulated in maple syrup urine disease. ( Farage, M; Reis, M; Wolosker, H, 2000)

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
MENKES, JH1
Reis, M1
Farage, M1
Wolosker, H1

Other Studies

2 other studies available for chlorine and Maple Syrup Urine Disease

ArticleYear
MAPLE SYRUP DISEASE AND OTHER RARE DISORDERS OF AMINO ACID METABOLISM.
    Clinical proceedings - Children's Hospital of the District of Columbia, 1964, Volume: 20

    Topics: Acer; Amino Acid Metabolism, Inborn Errors; Amino Acids; Child; Chlorides; Chromatography; Diagnosis

1964
Chloride-dependent inhibition of vesicular glutamate uptake by alpha-keto acids accumulated in maple syrup urine disease.
    Biochimica et biophysica acta, 2000, Jul-03, Volume: 1475, Issue:2

    Topics: Amino Acid Transport System X-AG; Animals; ATP-Binding Cassette Transporters; Biological Transport;

2000