| malonate saemialdehyde | OHC-CH2-COO-;the transaminated product of beta-alanine; elevated in hyper-beta-alaninaemia. (05 Mar 2000) |
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| malonate saemialdehyde decarboxylase | <enzyme> Forms acetaldehyde and carbon dioxide Registry number: EC 4.1.1.- (26 Jun 1999) |
| malonate | The ion from malonic acid, HOOC.CH2COOH. Malonate is a competitive inhibitor for succinate dehydrogenase in the citric acid cycle. Malonyl SCoA is an important precursor for fatty acid synthesis. (18 Nov 1997) |
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| isoflavone 7-O-glucoside-6''-malonate malonylesterase | <enzyme> From roots of chickpea (cicer arietenum) Registry number: EC 3.1.1.- Synonym: ifgmm esterase (26 Jun 1999) |
| aminoadipic d-saemialdehyde synthase | <enzyme> A bifunctional enzyme used in lysine degradation; it has a lysine:alpha-ketoglutarate reductase activity as well as a saccharopine dehydrogenase activity. A deficiency of this enzyme results in familial hyperlysinaemia. (05 Mar 2000) |
| aminoadipic saemialdehyde synthase | <enzyme> Bifunctional enzyme with both lysine-ketoglutarate reductase (EC 1.5.1.8) and saccharopine dehydrogenase (EC 1.5.1.9) activities Registry number: EC 1.5.1.- Synonym: aasa synthase (26 Jun 1999) |
| aspartate-saemialdehyde dehydrogenase | <enzyme> An enzyme that catalyses the conversion of l-aspartate 4-saemialdehyde, orthophosphate, and NADP+ to yield l-4-aspartyl phosphate and NADPH. Chemical name: L-Aspartate-4-saemialdehyde:NADP+ oxidoreductase (phosphorylating) Registry number: EC 1.2.1.11 (12 Dec 1998) |
| glutamate-1-saemialdehyde 2,1-aminomutase | <enzyme> Catalyses the conversion of glutamate 1-saemialdehyde to 5-aminolevulinate via 4,5-diaminovalerate in pyridoxane-dependent reactions; genbank x82072 (heml protein from pseudomonas aeruginosa) Registry number: EC 5.4.3.8 Synonym: glu-s-aminotransferase, glutamate-1-saemialdehyde aminotransferase, heml protein, heml gene product (26 Jun 1999) |
| glutamate gamma-saemialdehyde | -OOCCH(NH3)+CH2CH2C HO;an intermediate in l-proline and l-ornithine metabolism; becomes elevated in type II hyperprolinaemia. (05 Mar 2000) |
| glutamate-saemialdehyde dehydrogenase | <enzyme> Converts 5-glutamyl phosphate to glutamate 5-saemialdehyde in the presence of NADPH; see also glutamic-gamma-saemialdehyde dehydrogenase which catalyses the oxidation of 1-pyrroline-5-carboxylate to l-glutamate in the presence of nad Registry number: EC 1.2.1.41 Synonym: beta-glutamylphosphate reductase, glutamate 5-saemialdehyde dehydrogenase, gamma-glutamylphosphate reductase, proa gene product (26 Jun 1999) |
| methylmalonate saemialdehyde | OHC-CH(CH3)-COO-;an intermediate in l-valine catabolism; elevated in certain inborn disorders. (05 Mar 2000) |
| saemialdehyde | The monoaldehyde of a dicarboxylic acid, so called because half the COOH groups of the original acid are reduced to the aldehyde while the other half are unchanged; e.g., glutamic acid gamma-saemialdehyde, OHC-CH2CH2CH(NH3)+-COO-. Many saemialdehyde's are intermediates in the biosynthesis and metabolic degradation of amino acids (e.g., l-proline, l-lysine, l-glutamate). (05 Mar 2000) |
| succinate saemialdehyde | -OOC-CH2-CH2-CHO;an intermediate in the catabolism of gamma-aminobutyrate. Succinate saemialdehyde dehydrogenase, an enzyme that catalyses the reaction of succinate saemialdehyde and either NAD+ or NADP+ to form succinate and NADH (or NADPH); a deficiency of this enzyme is associated with 4-hydroxybutyric aciduria. (05 Mar 2000) |
| succinate saemialdehyde reductase | <enzyme> Nadp dependent Registry number: EC 1.1.1.- Synonym: sucinate saemialdehyde reductase (nadp) (26 Jun 1999) |
| N-succinylglutamate 5-saemialdehyde dehydrogenase | <enzyme> Catalyses conversion of n-succinylglutamate saemialdehyde to n-succinylglutamate; member of catabolic arginine succinyltransferase pathway; isolated from pseudomonas aeruginosa; genbank af011922 Registry number: EC 1.2.1.- Synonym: arud gene product, sgsd enzyme (26 Jun 1999) |
| ethylmalonate-saemialdehyde dehydrogenase | <enzyme> Forms butyryl-CoA Registry number: EC 1.2.1.- Synonym: ethylmalonic saemialdehyde dehydrogenase, emsa dehydrogenase (26 Jun 1999) |
| 2-hydroxy-4-carboxymucconate-6-saemialdehyde dehydrogenase | <enzyme> Catalyses alpha-hydroxy-gamma-carboxymuconic-epsilon-saemialdehyde to alpha-hydroxy-gamma-carboxymuconic acid Registry number: EC 1.2.1.45 Synonym: alpha-hydroxy-gamma-carboxymuconic-epsilon-saemialdehyde dehydrogenase, chms dehydrogenase (26 Jun 1999) |