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Record Information
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-02-11 01:02:29 UTC
Secondary Accession NumbersNone
Metabolite Identification
Common NameSaccharopine
DescriptionSaccharopine 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, 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 ).
  1. (S)-N-(5-amino-5-carboxypentyl)-L-Glutamic acid
  2. epsilon-N-(L-Glutar-2-yl)-L-lysine
  3. L-N-(5-Amino-5-carboxypentyl)-Glutamic acid
  4. L-Saccharopin
  5. L-Saccharopine
  6. N(6)-(L-1,3-Dicarboxypropyl)-L-lysine
  7. N-(5-Amino-5-carboxypentyl)-glutamic acid
  8. N-(5-Amino-5-carboxypentyl)-L-glutamic acid
  9. N-[(5S)-5-Amino-5-carboxypentyl]-L-Glutamic acid
  10. N6-(L-1,3-Dicarboxypropyl)-L-lysine
  11. Saccharopin
Chemical FormulaC11H20N2O6
Average Molecular Weight276.2863
Monoisotopic Molecular Weight276.132136382
IUPAC Name(2S)-2-{[(5S)-5-amino-5-carboxypentyl]amino}pentanedioic acid
Traditional Namesaccharopine
CAS Registry Number997-68-2
InChI Identifier
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as l-alpha-amino acids. These are alpha amino acids which have the L-configuration of the alpha-carbon atom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentL-alpha-amino acids
Alternative Parents
  • L-alpha-amino acid
  • Tricarboxylic acid or derivatives
  • Amino fatty acid
  • Fatty acyl
  • Secondary amine
  • Secondary aliphatic amine
  • Carboxylic acid
  • Hydrocarbon derivative
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Primary aliphatic amine
  • Carbonyl group
  • Amine
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
StatusDetected but not Quantified
  • Endogenous
  • Protein synthesis, amino acid biosynthesis
ApplicationNot Available
Cellular locations
  • Mitochondria
Physical Properties
Experimental Properties
Melting Point247 - 250 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
Water Solubility5.25 mg/mLALOGPS
pKa (Strongest Acidic)1.44ChemAxon
pKa (Strongest Basic)10.89ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count8ChemAxon
Hydrogen Donor Count5ChemAxon
Polar Surface Area149.95 Å2ChemAxon
Rotatable Bond Count11ChemAxon
Refractivity63.95 m3·mol-1ChemAxon
Polarizability28.14 Å3ChemAxon
Number of Rings0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies)splash10-3zbd100000-1964addb7be546c2a45aView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies)splash10-1z4d100000-49fae417f545c42253ddView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Positivesplash10-zaj0000000-d841eea4ebc5da1106f7View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negativesplash10-0bz0000000-c0b375ed0505ad2eab6cView in MoNA
1D NMR1H NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
Biological Properties
Cellular Locations
  • Mitochondria
Biofluid Locations
  • Blood
  • Urine
Tissue LocationNot Available
2-aminoadipic 2-oxoadipic aciduriaSMP00719Not Available
Glutaric Aciduria Type ISMP00186Not Available
Hyperlysinemia I, FamilialSMP00527Not Available
Hyperlysinemia II or SaccharopinuriaSMP00528Not Available
Lysine DegradationSMP00037map00310
Pyridoxine dependency with seizuresSMP00571Not Available
Saccharopinuria/Hyperlysinemia IISMP00239Not Available
Normal Concentrations
BloodExpected but not QuantifiedNot ApplicableNot AvailableNot Available
  • Not Applicable
UrineDetected but not QuantifiedNot ApplicableChildren (1-13 years old)FemaleNormal details
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDDB04207
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB000461
KNApSAcK IDC00007227
Chemspider ID141086
KEGG Compound IDC00449
BiGG ID1484994
Wikipedia LinkSaccharopine
NuGOwiki LinkHMDB00279
Metagene LinkHMDB00279
PubChem Compound160556
ChEBI ID16927
Synthesis ReferenceBurkard, 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
  1. IJlst L, de Kromme I, Oostheim W, Wanders RJ: Molecular cloning and expression of human L-pipecolate oxidase. Biochem Biophys Res Commun. 2000 Apr 21;270(3):1101-5. [10772957 ]
  2. 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. [977722 ]
  3. 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. [571908 ]
  4. 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. [117247 ]
  5. Fellows FC, Carson NA: Enzyme studies in a patient with saccharopinuria: a defect of lysine metabolism. Pediatr Res. 1974 Jan;8(1):42-9. [4809305 ]
  6. Dancis J, Hutzler J, Cox RP: Familial hyperlysinemia: enzyme studies, diagnostic methods, comments on terminology. Am J Hum Genet. 1979 May;31(3):290-9. [463877 ]
  7. Papes F, Kemper EL, Cord-Neto G, Langone F, Arruda P: Lysine degradation through the saccharopine pathway in mammals: involvement of both bifunctional and monofunctional lysine-degrading enzymes in mouse. Biochem J. 1999 Dec 1;344 Pt 2:555-63. [10567240 ]


General function:
Involved in oxidoreductase activity
Specific function:
Bifunctional enzyme that catalyzes the first two steps in lysine degradation. The N-terminal and the C-terminal contain lysine-ketoglutarate reductase and saccharopine dehydrogenase activity, respectively.
Gene Name:
Uniprot ID:
Molecular weight:
Saccharopine + NADP + Water → L-Lysine + Oxoglutaric acid + NADPH + Hydrogen Iondetails
Saccharopine + NAD + Water → L-Glutamic acid + (S)-2-amino-6-oxohexanoate + NADH + Hydrogen Iondetails
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [17139284 ]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [17016423 ]
  3. Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. [10592235 ]
General function:
Involved in catalytic activity
Specific function:
N(6)-(L-1,3-dicarboxypropyl)-L-lysine + NAD(+) + H(2)O = L-glutamate + 2-aminoadipate 6-semialdehyde + NADH
Gene Name:
Uniprot ID:
Molecular weight:
General function:
Involved in oxidoreductase activity
Specific function:
Not Available
Gene Name:
Uniprot ID:
Molecular weight: