Induction of medium chain dicarboxylic aciduria by 2-tetradecyloxiranecarboxylate(TDOC) in rat.
نویسندگان
چکیده
منابع مشابه
The catabolism of medium- and long-chain dicarboxylic acids.
The w-oxidation of fatty acids results in the formation of mediumand long-chain dicarboxylates. By this accessory metabolic pathway monocarboxylic acids are first converted into w-hydroxymonocarboxylic acids, a reaction catalysed by the microsomal mixed-oxidase function system (Pettersen, 1972). These intermediates are subsequently converted into w-oxomonocarboxylic acids and dicarboxylic acids...
متن کاملDisease-related metabolites in culture medium of fibroblasts from patients with D-2-hydroxyglutaric aciduria, L-2-hydroxyglutaric aciduria, and combined D/L-2-hydroxyglutaric aciduria.
BACKGROUND D-2-Hydroxyglutaric aciduria (D-2-HGA), L-2-hydroxyglutaric aciduria (L-2-HGA), and the combined D/L-2-hydroxyglutaric aciduria (D/L-2-HGA) are poorly understood organic acidurias. To investigate the usefulness of cultured human skin fibroblasts for both diagnostic and research purposes, we measured disease-related metabolites in the cell culture medium. METHODS We measured D-2-hyd...
متن کاملIdentification of isomeric unsaturated medium-chain dicarboxylic acids in human urine.
Dicarboxylic aciduria caused by enhanced or inhibited fatty acid metabolism is usually described as increased urinary excretion of saturated medium-chain dicarboxylic acids, such as adipic, suberic, and sebacic acids. Besides these saturated acids, increased excretion of unsaturated dicarboxylic acids is also observed. However, the structural identities of these unsaturated dicarboxylic acids a...
متن کاملAbnormal urinary excretion of unsaturated dicarboxylic acids in patients with medium-chain acyl-CoA dehydrogenase deficiency.
Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is the most frequently described metabolic disorder of fatty acid oxidation in humans. Acute episodes are usually characterized biochemically by the appearance of nonketotic dicarboxylic aciduria. In addition, other abnormal metabolites, such as suberylglycine, n-hexanoylglycine, 3-phenylpropionylglycine, and octanoylcarnitine, are excreted ...
متن کاملPeroxisomal L-bifunctional enzyme (Ehhadh) is essential for the production of medium-chain dicarboxylic acids.
L-bifunctional enzyme (Ehhadh) is part of the classical peroxisomal fatty acid β-oxidation pathway. This pathway is highly inducible via peroxisome proliferator-activated receptor α (PPARα) activation. However, no specific substrates or functions for Ehhadh are known, and Ehhadh knockout (KO) mice display no appreciable changes in lipid metabolism. To investigate Ehhadh functions, we used a bio...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: The Kurume Medical Journal
سال: 1988
ISSN: 0023-5679,1881-2090
DOI: 10.2739/kurumemedj.35.107