L-Saccharopine
public_id
FDB000461
IUPAC 名称
(2S)-2-{[(5S)-5-amino-5-carboxypentyl]amino}pentanedioic acid
描述
Amino acid from Saccharomyces cerevisiae and Neurospora crassaand is also found in mushrooms and seeds Saccharopine is an intermediate in the degradation of lysine, formed by condensation of lysine and alpha-ketoglutarate. The saccharopine pathway is the main route for lysine degradation in mammal and its first two reactions are catalyzed by enzymatic activities known as lysine-oxoglutarate reductase (LOR) and saccharopine dehydrogenase (SDH), which reside on a single bifunctional polypeptide (EC EC 1.5.1.8, LOR/SDH). The reactions involved by saccharopine dehydrogenases have a very strict substrate specificity for L-lysine, 2-oxoglutarate and NADPH. LOR/SDH has been detected in a number of mammalian tissues, mainly in the liver and kidney, contributing not only to the general nitrogen balance in the organism but also to the controlled conversion of lysine into ketone bodies. A tetrameric form has also been observed in human liver and placenta. LOR activity has also been detected in brain mitochondria during embryonic development, and this opens the question of whether the degradation of lysine has any functional significance during brain development and puts a new focus on the nutritional requirements for lysine in gestation and infancy. Finally, LOR and/or SDH deficiencies seem to be involved in a human autosomic genetic disorder known as familial hyperlysinemia, which is characterized by serious defects in the functioning of the nervous system, and characterized by deficiency in lysine-ketoglutarate reductase, saccharopine dehydrogenase, and saccharopine oxidoreductase activities. Saccharopinuria (high amounts of saccharopine in the urine) and saccharopinemia (an excess of saccharopine in the blood) are conditions present in some inherited disorders of lysine degradation. (PMID: 463877, 10567240, 10772957, 4809305); Saccharopine is an intermediate in the metabolism of amino acid lysine. It is a precursor of lysine in the alpha-aminoadipate pathway which occurs in a few lower fungi, the higher fungi, and euglenids. In mammals and higher plants saccharopine is an intermediate in the degradation of lysine, formed by condensation of lysine and alpha-ketoglutarate.
CAS号
997-68-2
异构
NC(CCCCNC(CCC(O)=O)C(O)=O)C(O)=O
InChI标识符
InChI=1S/C11H20N2O6/c12-7(10(16)17)3-1-2-6-13-8(11(18)19)4-5-9(14)15/h7-8,13H,1-6,12H2,(H,14,15)(H,16,17)(H,18,19)
ZDGJAHTZVHVLOT-UHFFFAOYSA-N
平均分子量
276.132136382
id
461
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