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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-10-17 21:51:04 UTC
HMDB IDHMDB00510
Secondary Accession NumbersNone
Metabolite Identification
Common NameAminoadipic acid
DescriptionAminoadipic acid (2-aminoadipate) is a metabolite in the principal biochemical pathway of lysine. It is an intermediate in the metabolism (i.e. breakdown or degradation) of lysine and saccharopine.(Wikipedia). It antagonizes neuroexcitatory activity modulated by the glutamate receptor, N-methyl-D-aspartate; (NMDA). Aminoadipic has also been shown to inhibit the production of kynurenic acid in brain tissue slices (PMID: 8566117 ). Kynurenic acid is a broad spectrum excitatory amino acid receptor antagonist. Recent studies have shown that aminoadipic acid is elevated in prostate biopsy tissues from prostate cancer patients (PMID: 23737455 ). Mutations in DHTKD1 (dehydrogenase E1 and transketolase domain-containing protein 1) have been shown to cause human 2-aminoadipic and 2-oxoadipic aciduria via impaired turnover of decarboxylation 2-oxoadipate to glutaryl-CoA, which is the last step in the lysine degradation pathway (PMID: 23141293 ). Aging, diabetes, sepsis and renal failure are known to catalyze the oxidation of lysyl residues to 2-aminoadipic acid in human skin collagen and potentially other tissues (PMID: 18448817 ). Proteolytic breakdown of these tissues can lead to the release of free 2-aminoadipic acid. Studies in rats indicate that aminoadipic acid (along with the 3 branched chain amino acid – Leu, Val and Ile) levels are elevated in the pre-diabetic phase and so aminoadipic acid may serve as a predictive biomarker for the development of diabetes. (PMID: 15389298 ). Long-term hyperglycemia of endothelial cells leads to elevated levels of aminoadipate which is though to be a sign of lysine breakdown through oxidative stress and reactive oxygen species (ROS) (PMID: 21961526 ). 2-aminoadipate is a potential small-molecule marker of oxidative stress (PMID: 21647514 )
Structure
Thumb
Synonyms
ValueSource
(+/-)-2-aminoadipic acidChEBI
AadChEBI
alpha-Aminoadipic acidChEBI
Aminoadipic acidChEBI
DL-2-Aminoadipic acidChEBI
DL-2-Aminohexanedioic acidChEBI
DL-alpha-Aminoadipic acidChEBI
(+/-)-2-aminoadipateHMDB
2-AminoadipateHMDB
a-AminoadipateHMDB
a-Aminoadipic acidHMDB
alpha-amino-Adipic acidHMDB
alpha-AminoadipateHMDB
AminoadipateHMDB
DL-2-AminoadipateHMDB
DL-2-AminohexanedioateHMDB
DL-a-AminoadipateHMDB
DL-a-Aminoadipic acidHMDB
DL-alpha-AminoadipateHMDB
L-2-AminoadipateHMDB
L-2-Aminoadipic acidHMDB
L-2-AminohexanedioateHMDB
L-2-Aminohexanedioic acidHMDB
L-alpha-AminoadipateHMDB
L-alpha-Aminoadipic acidHMDB
Chemical FormulaC6H11NO4
Average Molecular Weight161.1558
Monoisotopic Molecular Weight161.068807845
IUPAC Name2-aminohexanedioic acid
Traditional Nameaminoadipic acid
CAS Registry Number542-32-5
SMILES
NC(CCCC(O)=O)C(O)=O
InChI Identifier
InChI=1S/C6H11NO4/c7-4(6(10)11)2-1-3-5(8)9/h4H,1-3,7H2,(H,8,9)(H,10,11)
InChI KeyInChIKey=OYIFNHCXNCRBQI-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as alpha amino acids. These are amino acids in which the amino group is attached to the carbon atom immediately adjacent to the carboxylate group (alpha carbon).
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentAlpha amino acids
Alternative Parents
Substituents
  • Alpha-amino acid
  • Medium-chain fatty acid
  • Amino fatty acid
  • Fatty acyl
  • Fatty acid
  • Dicarboxylic acid or derivatives
  • Carboxylic acid
  • Hydrocarbon derivative
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Primary aliphatic amine
  • Carbonyl group
  • Amine
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External DescriptorsNot Available
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
Biofunction
  • Protein synthesis, amino acid biosynthesis
ApplicationNot Available
Cellular locations
  • Cytoplasm
  • Mitochondria
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point196 - 198 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility2.2 mg/mLNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility32.7 mg/mLALOGPS
logP-3.4ALOGPS
logP-2.8ChemAxon
logS-0.69ALOGPS
pKa (Strongest Acidic)2.01ChemAxon
pKa (Strongest Basic)9.53ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count3ChemAxon
Polar Surface Area100.62 Å2ChemAxon
Rotatable Bond Count5ChemAxon
Refractivity35.89 m3·mol-1ChemAxon
Polarizability15.53 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-03xv-2900000000-58b4f0b3e06cbb7abb90View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-0a4j-9000000000-cc9c0c03f7c3890b531cView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-0a4i-9000000000-7b74167f01c922da9ef7View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-00kg-2900000000-3e45ac769d3b229221a3View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-01ba-9600000000-c51318834cc371625d46View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-05fu-9000000000-edbc970f12ea186bcf06View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-03di-1900000000-0f7002659f604cc69395View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-01ox-4900000000-bf42ddc971a2a05516a2View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0abc-9100000000-1979c27463bab7d33d58View in MoNA
1D NMR1H NMR SpectrumNot Available
1D NMR1H NMR SpectrumNot Available
1D NMR13C NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
Biological Properties
Cellular Locations
  • Cytoplasm
  • Mitochondria
Biofluid Locations
  • Blood
  • Saliva
  • Urine
Tissue Location
  • Prostate
Pathways
NameSMPDB LinkKEGG Link
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
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified2.0 (0.0-5.0) uMAdult (>18 years old)BothNormal details
SalivaDetected and Quantified0.804 +/- 1.44 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified0.821 +/- 0.408 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified0.952 +/- 1.49 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified0.336 +/- 0.475 uMAdult (>18 years old)Male
Normal
    • Sugimoto et al. (...
details
UrineDetected and Quantified20.4023 +/- 11.4518 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected and Quantified1.7-4.2 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified4.6 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified40.736 +/- 34.234 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothNormal details
UrineDetected and Quantified1.9-4.3 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified7.3 (3.4-11.2) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified7.2 (2.5-16.4) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified6.0 (7.0-8.0) uMAdult (>18 years old)Bothalpha-Aminoadipic aciduria details
BloodDetected and Quantified48.0 uMChildren (1-13 years old)Bothalpha-Aminoadipic aciduria details
BloodDetected and Quantified55.0 (30.0-80.0) uMChildren (1-13 years old)Both2-Ketoadipic Acidemia details
UrineDetected and Quantified14.3982 +/- 9.7148 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected and Quantified14.0068 +/- 9.2776 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Gastroesophageal reflux disease
    • Mordechai, Hien, ...
details
UrineDetected and Quantified45.554 +/- 68.592 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Schizophrenia
details
UrineDetected and Quantified13000 umol/mmol creatinineChildren (1-13 years old)Bothalpha-Aminoadipic aciduria details
UrineDetected and Quantified54.2 (9.8-98.6) umol/mmol creatinineChildren (1-13 years old)Both2-Ketoadipic Acidemia details
Associated Disorders and Diseases
Disease References
Alpha-aminoadipic aciduria
  1. Vallat C, Rivier F, Bellet H, Magnan de Bornier B, Mion H, Echenne B: Treatment with vigabatrin may mimic alpha-aminoadipic aciduria. Epilepsia. 1996 Aug;37(8):803-5. [8764822 ]
Schizophrenia
  1. Yang J, Chen T, Sun L, Zhao Z, Qi X, Zhou K, Cao Y, Wang X, Qiu Y, Su M, Zhao A, Wang P, Yang P, Wu J, Feng G, He L, Jia W, Wan C: Potential metabolite markers of schizophrenia. Mol Psychiatry. 2013 Jan;18(1):67-78. doi: 10.1038/mp.2011.131. Epub 2011 Oct 25. [22024767 ]
2-ketoadipic acidemia
  1. MetaGene [Link]
2-Aminoadipic aciduria
  1. MetaGene [Link]
Associated OMIM IDs
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB021812
KNApSAcK IDNot Available
Chemspider ID456
KEGG Compound IDC00956
BioCyc IDCPD-468
BiGG ID1485280
Wikipedia Linkalpha-Aminoadipate
NuGOwiki LinkHMDB00510
Metagene LinkHMDB00510
METLIN ID5496
PubChem Compound469
PDB IDNot Available
ChEBI ID37024
References
Synthesis ReferenceKhan, Naseem H.; Ali, Mohd.; Panda, H.; Rakhshinda. Synthesis of a-aminoadipic acid. Journal of the Indian Chemical Society (1982), 59(9), 1077-8.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Fiermonte G, Dolce V, Palmieri L, Ventura M, Runswick MJ, Palmieri F, Walker JE: Identification of the human mitochondrial oxodicarboxylate carrier. Bacterial expression, reconstitution, functional characterization, tissue distribution, and chromosomal location. J Biol Chem. 2001 Mar 16;276(11):8225-30. Epub 2000 Nov 16. [11083877 ]
  2. Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. [19212411 ]
  3. Brauner-Osborne H, Slok FA, Skjaerbaek N, Ebert B, Sekiyama N, Nakanishi S, Krogsgaard-Larsen P: A new highly selective metabotropic excitatory amino acid agonist: 2-amino-4-(3-hydroxy-5-methylisoxazol-4-yl)butyric acid. J Med Chem. 1996 Aug 2;39(16):3188-94. [8759641 ]
  4. Hori S: [Pathophysiology of intraocular neovascularization] Nippon Ganka Gakkai Zasshi. 1990 Dec;94(12):1103-21. [1707214 ]
  5. Ouwerkerk-Mahadevan S, Mulder GJ: Inhibition of glutathione conjugation in the rat in vivo by analogues of glutathione conjugates. Chem Biol Interact. 1998 Apr 24;111-112:163-76. [9679552 ]
  6. Mechanic GL: The qualitative and quantitative crosslink chemistry of collagen matrices. Adv Exp Med Biol. 1977;86B:699-708. [20755 ]
  7. Okuno E, Tsujimoto M, Nakamura M, Kido R: 2-Aminoadipate-2-oxoglutarate aminotransferase isoenzymes in human liver: a plausible physiological role in lysine and tryptophan metabolism. Enzyme Protein. 1993;47(3):136-48. [8087205 ]
  8. Takechi T, Okada T, Wakiguchi H, Morita H, Kurashige T, Sugahara K, Kodama H: Identification of N-acetyl-alpha-aminoadipic acid in the urine of a patient with alpha-aminoadipic and alpha-ketoadipic aciduria. J Inherit Metab Dis. 1993;16(1):119-26. [8487492 ]
  9. Wu HQ, Ungerstedt U, Schwarcz R: L-alpha-aminoadipic acid as a regulator of kynurenic acid production in the hippocampus: a microdialysis study in freely moving rats. Eur J Pharmacol. 1995 Jul 25;281(1):55-61. [8566117 ]
  10. Jung K, Reszka R, Kamlage B, Bethan B, Stephan C, Lein M, Kristiansen G: Tissue metabolite profiling identifies differentiating and prognostic biomarkers for prostate carcinoma. Int J Cancer. 2013 Jun 4. doi: 10.1002/ijc.28303. [23737455 ]
  11. Danhauser K, Sauer SW, Haack TB, Wieland T, Staufner C, Graf E, Zschocke J, Strom TM, Traub T, Okun JG, Meitinger T, Hoffmann GF, Prokisch H, Kolker S: DHTKD1 mutations cause 2-aminoadipic and 2-oxoadipic aciduria. Am J Hum Genet. 2012 Dec 7;91(6):1082-7. doi: 10.1016/j.ajhg.2012.10.006. Epub 2012 Nov 8. [23141293 ]
  12. Sell DR, Strauch CM, Shen W, Monnier VM: 2-aminoadipic acid is a marker of protein carbonyl oxidation in the aging human skin: effects of diabetes, renal failure and sepsis. Biochem J. 2007 Jun 1;404(2):269-77. [17313367 ]
  13. Wijekoon EP, Skinner C, Brosnan ME, Brosnan JT: Amino acid metabolism in the Zucker diabetic fatty rat: effects of insulin resistance and of type 2 diabetes. Can J Physiol Pharmacol. 2004 Jul;82(7):506-14. [15389298 ]
  14. Yuan W, Zhang J, Li S, Edwards JL: Amine metabolomics of hyperglycemic endothelial cells using capillary LC-MS with isobaric tagging. J Proteome Res. 2011 Nov 4;10(11):5242-50. doi: 10.1021/pr200815c. Epub 2011 Oct 17. [21961526 ]
  15. Zeitoun-Ghandour S, Leszczyszyn OI, Blindauer CA, Geier FM, Bundy JG, Sturzenbaum SR: C. elegans metallothioneins: response to and defence against ROS toxicity. Mol Biosyst. 2011 Aug;7(8):2397-406. doi: 10.1039/c1mb05114h. Epub 2011 Jun 7. [21647514 ]
  16. Sell DR, Strauch CM, Shen W, Monnier VM: Aging, diabetes, and renal failure catalyze the oxidation of lysyl residues to 2-aminoadipic acid in human skin collagen: evidence for metal-catalyzed oxidation mediated by alpha-dicarbonyls. Ann N Y Acad Sci. 2008 Apr;1126:205-9. doi: 10.1196/annals.1433.065. [18448817 ]

Enzymes

General function:
Involved in oxidoreductase activity
Specific function:
Multifunctional enzyme mediating important protective effects. Metabolizes betaine aldehyde to betaine, an important cellular osmolyte and methyl donor. Protects cells from oxidative stress by metabolizing a number of lipid peroxidation-derived aldehydes. Involved in lysine catabolism.
Gene Name:
ALDH7A1
Uniprot ID:
P49419
Molecular weight:
58486.74
Reactions
(S)-2-amino-6-oxohexanoate + NAD(P)(+) + Water → Aminoadipic acid + NAD(P)Hdetails
Aminoadipic acid + Adenosine triphosphate → L-2-Aminoadipate adenylate + Pyrophosphatedetails
(S)-2-amino-6-oxohexanoate + NAD + Water → Aminoadipic acid + NADH + Hydrogen Iondetails
(S)-2-amino-6-oxohexanoate + NADP + Water → Aminoadipic acid + NADPH + Hydrogen Iondetails
General function:
Involved in transferase activity, transferring nitrogenous groups
Specific function:
Transaminase with broad substrate specificity. Has transaminase activity towards aminoadipate, kynurenine, methionine and glutamate. Shows activity also towards tryptophan, aspartate and hydroxykynurenine. Accepts a variety of oxo-acids as amino-group acceptors, with a preference for 2-oxoglutarate, 2-oxocaproic acid, phenylpyruvate and alpha-oxo-gamma-methiol butyric acid. Can also use glyoxylate as amino-group acceptor (in vitro).
Gene Name:
AADAT
Uniprot ID:
Q8N5Z0
Molecular weight:
47351.17
Reactions
Aminoadipic acid + Oxoglutaric acid → Oxoadipic acid + L-Glutamic aciddetails
General function:
Transcription
Specific function:
Not Available
Gene Name:
AADAT
Uniprot ID:
Q4W5N8
Molecular weight:
47351.2
General function:
Involved in acyl carrier activity
Specific function:
Acyl-CoA synthases catalyze the initial reaction in fatty acid metabolism, by forming a thioester with CoA
Gene Name:
AASDH
Uniprot ID:
Q4L235
Molecular weight:
122596.1
General function:
Involved in magnesium ion binding
Specific function:
Catalyzes the post-translational modification of target proteins by phosphopantetheine. Can transfer the 4'-phosphopantetheine moiety from coenzyme A to a serine residue of a broad range of acceptors, such as the acyl carrier domain of FASN.
Gene Name:
AASDHPPT
Uniprot ID:
Q9NRN7
Molecular weight:
35775.64