| Record Information |
|---|
| Version |
3.5 |
| Creation Date |
2005-11-16 08:48:42 -0700 |
| Update Date |
2013-02-08 17:08:13 -0700 |
| HMDB ID |
HMDB00279 |
| Secondary Accession Numbers |
None |
| Metabolite Identification |
|---|
| Common Name |
Saccharopine |
| Description |
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 ). |
| Structure |
Download:
MOL |
SDF |
SMILES |
InChI
Display:
2D Structure |
3D Structure
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| Synonyms |
- (S)-N-(5-amino-5-carboxypentyl)-L-Glutamic acid
- epsilon-N-(L-Glutar-2-yl)-L-lysine
- L-N-(5-Amino-5-carboxypentyl)-Glutamic acid
- L-Saccharopin
- L-Saccharopine
- N(6)-(L-1,3-Dicarboxypropyl)-L-lysine
- N-(5-Amino-5-carboxypentyl)-glutamic acid
- N-(5-Amino-5-carboxypentyl)-L-glutamic acid
- N-[(5S)-5-Amino-5-carboxypentyl]-L-Glutamic acid
- N6-(L-1,3-Dicarboxypropyl)-L-lysine
- Saccharopin
|
| Chemical Formula |
C11H20N2O6 |
| Average Molecular Weight |
276.2863 |
| Monoisotopic Molecular Weight |
276.132136382 |
| IUPAC Name |
(2S)-2-{[(5S)-5-amino-5-carboxypentyl]amino}pentanedioic acid |
| Traditional IUPAC Name |
saccharopine |
| CAS Registry Number |
997-68-2 |
| SMILES |
N[C@@H](CCCCN[C@@H](CCC(O)=O)C(O)=O)C(O)=O |
| InChI Identifier |
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)/t7-,8-/m0/s1 |
| InChI Key |
ZDGJAHTZVHVLOT-YUMQZZPRSA-N |
| Chemical Taxonomy |
|---|
| Kingdom |
Organic Compounds |
| Super Class |
Amino Acids, Peptides, and Analogues |
| Class |
Amino Acids and Derivatives |
| Sub Class |
Alpha Amino Acids and Derivatives |
| Other Descriptors |
- Aliphatic Acyclic Compounds
- Amino Fatty Acids
- Organic Compounds
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| Substituents |
- Carboxylic Acid
- Polyamine
- Primary Aliphatic Amine (Alkylamine)
- Secondary Aliphatic Amine (Dialkylamine)
- Tricarboxylic Acid Derivative
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| Direct Parent |
Alpha Amino Acids and Derivatives |
| Ontology |
|---|
| Status |
Detected and Not Quantified |
| Origin |
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| Biofunction |
- Protein synthesis, amino acid biosynthesis
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| Application |
Not Available |
| Cellular locations |
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| Physical Properties |
|---|
| State |
Solid |
| Experimental Properties |
| Property |
Value |
Reference |
| Melting Point |
247 - 250 °C |
Not Available |
| Boiling Point |
Not Available |
Not Available |
| Water Solubility |
Not Available |
Not Available |
| LogP |
Not Available |
Not Available |
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| Predicted Properties |
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| Spectra |
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| Biological Properties |
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| Cellular Locations |
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| Biofluid Locations |
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| Tissue Location |
Not Available
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| Pathways |
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| Normal Concentrations |
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| Blood |
Expected and not Quantified |
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Not Applicable |
Not Available |
Not Available |
Normal |
Inferred from detection in urine
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| Urine |
Detected and not Quantified |
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Not Applicable |
Children (1-13 year old) |
Female |
Normal |
Not Available |
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| Abnormal Concentrations |
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Not Available |
| Associated Disorders and Diseases |
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| Disease References |
None |
| Associated OMIM IDs |
None |
| External Links |
|---|
| DrugBank ID |
DB04207 |
| Phenol Explorer Compound ID |
Not Available |
| Phenol Explorer Metabolite ID |
Not Available |
| FoodDB ID |
FDB000461 |
| KNApSAcK ID |
C00007227 |
| Chemspider ID |
141086 |
| KEGG Compound ID |
C00449 |
| BioCyc ID |
SACCHAROPINE |
| BiGG ID |
1484994 |
| Wikipedia Link |
Saccharopine |
| NuGOwiki Link |
HMDB00279 |
| Metagene Link |
HMDB00279 |
| METLIN ID |
383 |
| PubChem Compound |
160556 |
| PDB ID |
SHR |
| ChEBI ID |
16927 |
| References |
|---|
| Synthesis Reference |
Burkard, Ulrike; Walther, Ingrid; Effenberger, Franz. Amino acids. 6. Investigations on the synthesis of L-saccharopin. Liebigs Annalen der Chemie (1986), (6), 1030-43. |
| Material Safety Data Sheet (MSDS) |
Download (PDF)
|
| General References |
- Krieger I, Bachmann C, Gronemeyer WH, Cejka J: Propionic acidemia and hyperlysinemia in a case with ornithine transcarbamylase (OTC) deficiency. J Clin Endocrinol Metab. 1976 Oct;43(4):796-802.
Pubmed: 977722
- Cederbaum SD, Shaw KN, Dancis J, Hutzler J, Blaskovics JC: Hyperlysinemia with saccharopinuria due to combined lysine-ketoglutarate reductase and saccharopine dehydrogenase deficiencies presenting as cystinuria. J Pediatr. 1979 Aug;95(2):234-8.
Pubmed: 571908
- Casey RE, Zaleski WA, Philp M, Mendelson IS, MacKenzie SL: Biochemical and clinical studies of a new case of alpha-aminoadipic aciduria. J Inherit Metab Dis. 1978;1(4):129-35.
Pubmed: 117247
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