| fumaric acidemia | Elevated levels of fumarate in blood plasma; due to a decrease in activity of fumarate hydratase. (05 Mar 2000) |
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| fumaric | <chemistry> Pertaining to, or derived from, fumitory (Fumaria officinalis). <chemistry> Fumaric acid, a widely occurring organic acid, exttracted from fumitory as a white crystallline substance, C2H2(CO2H)2, and produced artificially in many ways, as by the distillation of malic acid; boletic acid. It is found also in the lichen, Iceland moss, and hence was also called lichenic acid. Source: Websters Dictionary (01 Mar 1998) |
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| fumaric acid | Trans-Butanedioic acid;an unsaturated dicarboxylic acid occurring as an intermediate in the tricarboxylic acid cycle. Synonym: allomaleic acid. (05 Mar 2000) |
| fumaric aminase | <enzyme> An enzyme that catalyses the conversion of aspartic acid to ammonia and fumaric acid in plants and some microorganisms. Chemical name: L-Aspartate ammonia-lyase Registry number: EC 4.3.1.1 (12 Dec 1998) |
| fumaric hydrogenase | <enzyme> Registry number: EC 1.3.99.1 (12 Dec 1998) |
| acidemia | An increase in the H-ion concentration of the blood or a fall below normal in pH, notwithstanding alterations in bicarbonate concentration. Individual types of acidemia are listed by specific name, e.g., isovalericacidemia, aminoacidemia, etc. Origin: acid + G. Haima, blood (05 Mar 2000) |
| methylmalonic acidemia | ketotic hyperglycinaemia |
| propionic acidemia | ketotic hyperglycinaemia |
| hyperpipecolic acidemia | A metabolic disorder in which serum concentrations of pipecolic acid are greatly increased; characterised by hepatomegaly and progressive, generalised demyelination of the nervous system. Synonym: hyperpipecolic acidemia. (05 Mar 2000) |
| isovaleric acidemia | A disorder of leucine metabolism characterised by the excessive production of isovaleric acid upon protein ingestion or during infectious episodes; severe metabolic acidosis results from the large quantities of acid formed; autosomal recessive inheritance; due to a deficiency of isovaleryl-CoA dehydrogenase. Synonym: sweaty feet syndrome. (05 Mar 2000) |
| lactic acidemia | The presence of dextrorotatory lactic acid in the circulating blood. Synonym: lactacidemia. Origin: Lactic acid + G. Haima, blood (05 Mar 2000) |
| 2-ketoadipic acidemia | Elevated levels of 2-ketoadipic acid in the serum. (05 Mar 2000) |