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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-02-11 01:03:23 UTC
HMDB IDHMDB00812
Secondary Accession NumbersNone
Metabolite Identification
Common NameN-Acetyl-L-aspartic acid
DescriptionN-Acetylaspartic acid is a derivative of aspartic acid. It is the second most concentrated molecule in the brain after the amino acid glutamate. It is synthesized in neurons from the amino acid aspartate and acetyl coenzyme A. The various functions served by N-acetylaspartic acid are still under investigation, but the primary proposed functions include:. 1) A neuronal osmolyte that is involved in fluid balance in the brain 2) A source of acetate for lipid and myelin synthesis in oligodendrocytes, the glial cells that myelinate neuronal axons 3) A precursor for the synthesis of the important neuronal dipeptide N-acetylaspartylglutamate 4)N-Acetylaspartic acid may also be involved in energy production from the amino acid glutamate in neuronal mitochondria.
Structure
Thumb
Synonyms
ValueSource
(S)-2-(acetylamino)Butanedioic acidChEBI
(S)-2-(acetylamino)Succinic acidChEBI
Acetyl-L-aspartic acidChEBI
Acetylaspartic acidChEBI
L-N-Acetylaspartic acidChEBI
N-Acetylaspartic acidChEBI
NAAChEBI
(S)-2-(acetylamino)ButanedioateGenerator
N-Acetyl-L-aspartateGenerator
(S)-2-(acetylamino)SuccinateGenerator
Acetyl-L-aspartateGenerator
AcetylaspartateGenerator
L-N-AcetylaspartateGenerator
N-AcetylaspartateGenerator
(2S)-2-AcetamidobutanedioateHMDB
(2S)-2-Acetamidobutanedioic acidHMDB
N-Acetyl-S-aspartateHMDB
N-Acetyl-S-aspartic acidHMDB
Chemical FormulaC6H9NO5
Average Molecular Weight175.1394
Monoisotopic Molecular Weight175.048072403
IUPAC Name(2S)-2-acetamidobutanedioic acid
Traditional Nameacetyl-L-aspartic acid
CAS Registry Number997-55-7
SMILES
CC(=O)N[C@@H](CC(O)=O)C(O)=O
InChI Identifier
InChI=1S/C6H9NO5/c1-3(8)7-4(6(11)12)2-5(9)10/h4H,2H2,1H3,(H,7,8)(H,9,10)(H,11,12)/t4-/m0/s1
InChI KeyInChIKey=OTCCIMWXFLJLIA-BYPYZUCNSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as n-acyl-aliphatic-alpha amino acids. These are alpha amino acids carrying a N-acylated aliphatic chain.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentN-acyl-aliphatic-alpha amino acids
Alternative Parents
Substituents
  • N-acyl-aliphatic-alpha amino acid
  • N-acyl-l-alpha-amino acid
  • Amino fatty acid
  • Fatty acyl
  • Fatty acid
  • Dicarboxylic acid or derivatives
  • Acetamide
  • Secondary carboxylic acid amide
  • Carboxamide group
  • Carboxylic acid
  • Carboxylic acid amide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Organonitrogen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
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 Point137 - 140 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility675 mg/mLNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility21.1 mg/mLALOGPS
logP-0.79ALOGPS
logP-1.4ChemAxon
logS-0.92ALOGPS
pKa (Strongest Acidic)3.41ChemAxon
pKa (Strongest Basic)-1.5ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count3ChemAxon
Polar Surface Area103.7 Å2ChemAxon
Rotatable Bond Count4ChemAxon
Refractivity35.98 m3·mol-1ChemAxon
Polarizability15.29 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies)splash10-0cdi-0910000000-80f173aa655a9e8604b5View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies)splash10-008a-0930000000-63ecd768773360e39272View in MoNA
GC-MSGC-MS Spectrum - GC-MS (3 TMS)splash10-001i-0950000000-beff64b09b14e79c535cView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-001i-2900000000-73b8786c25f0b59927c6View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-00dr-9000000000-f7f7d5d562cbcf3f69faView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-006x-9000000000-01988ea29217a99519e1View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-00di-0900000000-e8fc0d2735d5a8ce1818View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-000i-9800000000-10648516e9c8e71f36ecView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-000i-9000000000-5a1edaf86830da3de4a6View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-0a4r-9000000000-be6d8862f50bbbceda29View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negativesplash10-0a4l-9000000000-d8e588abce3e75dbb9e6View in MoNA
1D NMR13C NMR SpectrumNot Available
1D NMR1H NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
Biological Properties
Cellular Locations
  • Cytoplasm
  • Mitochondria
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Saliva
  • Urine
Tissue Location
  • Basal Ganglia
  • Brain
  • Fibroblasts
  • Hippocampus
  • Myelin
  • Neuron
  • Prostate
  • Temporal Lobe
  • Thalamus
Pathways
NameSMPDB LinkKEGG Link
Aspartate MetabolismSMP00067map00250
Canavan DiseaseSMP00175Not Available
HypoacetylaspartiaSMP00192Not Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
Cerebrospinal Fluid (CSF)Detected and Quantified0.81 +/- 0.38 uMAdult (>18 years old)BothNormal details
SalivaDetected and Quantified0.827 +/- 0.521 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified1.08 +/- 0.586 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified1.54 +/- 1.13 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified0.658 +/- 0.331 uMAdult (>18 years old)Male
Normal
    • Sugimoto et al. (...
details
UrineDetected and Quantified33.2 (4.9-65.5) umol/mmol creatinineNewborn (0-30 days old)BothNormal details
UrineDetected and Quantified18.0 (0.1-43.9) umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified10.3 (6.1-15.4) umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected and Quantified7.7 (3.1-18.7) umol/mmol creatinineAdolescent (13-18 years old)BothNormal details
UrineDetected and Quantified10.657 +/- 6.879 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected and Quantified11.3 umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected and Quantified1.65 +/- 1.50 umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected and Quantified0.83 +/- 0.83 umol/mmol creatinineAdolescent (13-18 years old)BothNormal details
UrineDetected and Quantified0.40 +/- 0.43 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified<44.29 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified4.0 (1.3-7.0) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified4.66 +/- 1.14 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified7.0 +/- 1.84 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified16.96 +/- 19.57 uMAdult (>18 years old)BothCanavan disease details
Cerebrospinal Fluid (CSF)Detected and Quantified0.80 +/- 0.62 uMAdult (>18 years old)BothSchizophrenia details
Cerebrospinal Fluid (CSF)Detected and Quantified380.0 (0.0-760.0) uMAdult (>18 years old)BothCanavan Disease details
UrineDetected and Quantified14.079 +/- 11.359 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected and Quantified1872.03 +/- 631.86 umol/mmol creatinineAdult (>18 years old)BothCanavan disease details
Associated Disorders and Diseases
Disease References
Canavan disease
  1. Rothstein JD, Tsai G, Kuncl RW, Clawson L, Cornblath DR, Drachman DB, Pestronk A, Stauch BL, Coyle JT: Abnormal excitatory amino acid metabolism in amyotrophic lateral sclerosis. Ann Neurol. 1990 Jul;28(1):18-25. [2375630 ]
  2. Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [16139832 ]
Schizophrenia
  1. Do KQ, Lauer CJ, Schreiber W, Zollinger M, Gutteck-Amsler U, Cuenod M, Holsboer F: gamma-Glutamylglutamine and taurine concentrations are decreased in the cerebrospinal fluid of drug-naive patients with schizophrenic disorders. J Neurochem. 1995 Dec;65(6):2652-62. [7595563 ]
Associated OMIM IDs
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022260
KNApSAcK IDNot Available
Chemspider ID58576
KEGG Compound IDC01042
BioCyc IDCPD-420
BiGG ID36685
Wikipedia LinkN-acetylaspartate
NuGOwiki LinkHMDB00812
Metagene LinkHMDB00812
METLIN ID5776
PubChem Compound65065
PDB IDNot Available
ChEBI ID21547
References
Synthesis ReferenceMontoro, Fernando; Calatayud, Jose; Vilar, Angel. N-Acetyl-L-aspartic acid. Span. (1983), 5 pp.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Kaul R, Gao GP, Balamurugan K, Matalon R: Cloning of the human aspartoacylase cDNA and a common missense mutation in Canavan disease. Nat Genet. 1993 Oct;5(2):118-23. [8252036 ]
  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. Guneral F, Bachmann C: Age-related reference values for urinary organic acids in a healthy Turkish pediatric population. Clin Chem. 1994 Jun;40(6):862-6. [8087979 ]
  4. Wevers RA, Engelke U, Wendel U, de Jong JG, Gabreels FJ, Heerschap A: Standardized method for high-resolution 1H-NMR of cerebrospinal fluid. Clin Chem. 1995 May;41(5):744-51. [7729054 ]
  5. Tedeschi G, Bonavita S, Banerjee TK, Virta A, Schiffmann R: Diffuse central neuronal involvement in Fabry disease: a proton MRS imaging study. Neurology. 1999 May 12;52(8):1663-7. [10331696 ]
  6. Tacke U, Olbrich H, Sass JO, Fekete A, Horvath J, Ziyeh S, Kleijer WJ, Rolland MO, Fisher S, Payne S, Vargiami E, Zafeiriou DI, Omran H: Possible genotype-phenotype correlations in children with mild clinical course of Canavan disease. Neuropediatrics. 2005 Aug;36(4):252-5. [16138249 ]
  7. Rocca MA, Mezzapesa DM, Falini A, Ghezzi A, Martinelli V, Scotti G, Comi G, Filippi M: Evidence for axonal pathology and adaptive cortical reorganization in patients at presentation with clinically isolated syndromes suggestive of multiple sclerosis. Neuroimage. 2003 Apr;18(4):847-55. [12725761 ]
  8. Izumiyama H, Abe T, Tanioka D, Fukuda A, Kunii N: Clinicopathological examination of glioma by proton magnetic resonance spectroscopy background. Brain Tumor Pathol. 2004;21(1):39-46. [15696968 ]
  9. Bal D, Gryff-Keller A, Gradowska W: Absolute configuration of N-acetylaspartate in urine from patients with Canavan disease. J Inherit Metab Dis. 2005;28(4):607-9. [15902566 ]
  10. Manji HK, Moore GJ, Chen G: Clinical and preclinical evidence for the neurotrophic effects of mood stabilizers: implications for the pathophysiology and treatment of manic-depressive illness. Biol Psychiatry. 2000 Oct 15;48(8):740-54. [11063971 ]
  11. Vermathen P, Laxer KD, Matson GB, Weiner MW: Hippocampal structures: anteroposterior N-acetylaspartate differences in patients with epilepsy and control subjects as shown with proton MR spectroscopic imaging. Radiology. 2000 Feb;214(2):403-10. [10671587 ]
  12. Clementi V, Tonon C, Lodi R, Malucelli E, Barbiroli B, Iotti S: Assessment of glutamate and glutamine contribution to in vivo N-acetylaspartate quantification in human brain by (1)H-magnetic resonance spectroscopy. Magn Reson Med. 2005 Dec;54(6):1333-9. [16265633 ]
  13. Rothstein JD, Tsai G, Kuncl RW, Clawson L, Cornblath DR, Drachman DB, Pestronk A, Stauch BL, Coyle JT: Abnormal excitatory amino acid metabolism in amyotrophic lateral sclerosis. Ann Neurol. 1990 Jul;28(1):18-25. [2375630 ]
  14. Surendran S, Matalon KM, Szucs S, Tyring SK, Matalon R: Metabolic changes in the knockout mouse for Canavan's disease: implications for patients with Canavan's disease. J Child Neurol. 2003 Sep;18(9):611-5. [14572139 ]
  15. Gordon N: Canavan disease: a review of recent developments. Eur J Paediatr Neurol. 2001;5(2):65-9. [11589315 ]
  16. Zhu XH, Chen W: Observed BOLD effects on cerebral metabolite resonances in human visual cortex during visual stimulation: a functional (1)H MRS study at 4 T. Magn Reson Med. 2001 Nov;46(5):841-7. [11675633 ]
  17. Lam WW, Wang ZJ, Zhao H, Berry GT, Kaplan P, Gibson J, Kaplan BS, Bilaniuk LT, Hunter JV, Haselgrove JC, Zimmermann RA: 1H MR spectroscopy of the basal ganglia in childhood: a semiquantitative analysis. Neuroradiology. 1998 May;40(5):315-23. [9638674 ]
  18. Martin RC, Sawrie S, Hugg J, Gilliam F, Faught E, Kuzniecky R: Cognitive correlates of 1H MRSI-detected hippocampal abnormalities in temporal lobe epilepsy. Neurology. 1999 Dec 10;53(9):2052-8. [10599780 ]
  19. Trope I, Lopez-Villegas D, Lenkinski RE: Magnetic resonance imaging and spectroscopy of regional brain structure in a 10-year-old boy with elevated blood lead levels. Pediatrics. 1998 Jun;101(6):E7. [9606249 ]
  20. Kvittingen EA, Guldal G, Borsting S, Skalpe IO, Stokke O, Jellum E: N-acetylaspartic aciduria in a child with a progressive cerebral atrophy. Clin Chim Acta. 1986 Aug 15;158(3):217-27. [3769199 ]
  21. Gideon P, Henriksen O, Sperling B, Christiansen P, Olsen TS, Jorgensen HS, Arlien-Soborg P: Early time course of N-acetylaspartate, creatine and phosphocreatine, and compounds containing choline in the brain after acute stroke. A proton magnetic resonance spectroscopy study. Stroke. 1992 Nov;23(11):1566-72. [1440704 ]

Enzymes

General function:
Involved in hydrolase activity, acting on ester bonds
Specific function:
Catalyzes the deacetylation of N-acetylaspartic acid (NAA) to produce acetate and L-aspartate. NAA occurs in high concentration in brain and its hydrolysis NAA plays a significant part in the maintenance of intact white matter. In other tissues it act as a scavenger of NAA from body fluids.
Gene Name:
ASPA
Uniprot ID:
P45381
Molecular weight:
35734.79
Reactions
N-Acetyl-L-aspartic acid + Water → Acetic acid + L-Aspartic aciddetails
General function:
Involved in hydrolase activity, acting on ester bonds
Specific function:
Plays an important role in deacetylating mercapturic acids in kidney proximal tubules (By similarity).
Gene Name:
ACY3
Uniprot ID:
Q96HD9
Molecular weight:
Not Available
Reactions
N-Acetyl-L-aspartic acid + Water → Acetic acid + L-Aspartic aciddetails
General function:
Involved in glycine N-acyltransferase activity
Specific function:
Mitochondrial acyltransferase which transfers an acyl group to the N-terminus of glycine and glutamine, although much less efficiently. Can conjugate numerous substrates to form a variety of N-acylglycines, with a preference for benzoyl-CoA over phenylacetyl-CoA as acyl donors. Thereby detoxify xenobiotics, such as benzoic acid or salicylic acid, and endogenous organic acids, such as isovaleric acid.
Gene Name:
GLYAT
Uniprot ID:
Q6IB77
Molecular weight:
18506.33
General function:
Involved in glycine N-acyltransferase activity
Specific function:
Acyltransferase which transfers an acyl group to the N-terminus of glutamine. Can use phenylacetyl-CoA as an acyl donor.
Gene Name:
GLYATL1
Uniprot ID:
Q969I3
Molecular weight:
35100.895
General function:
Involved in glycine N-acyltransferase activity
Specific function:
Mitochondrial acyltransferase which transfers the acyl group to the N-terminus of glycine. Conjugates numerous substrates, such as arachidonoyl-CoA and saturated medium and long-chain acyl-CoAs ranging from chain-length C8:0-CoA to C18:0-CoA, to form a variety of N-acylglycines. Shows a preference for monounsaturated fatty acid oleoyl-CoA (C18:1-CoA) as an acyl donor. Does not exhibit any activity toward C22:6-CoA and chenodeoxycholoyl-CoA, nor toward serine or alanine.
Gene Name:
GLYATL2
Uniprot ID:
Q8WU03
Molecular weight:
34277.055
General function:
Involved in catalytic activity
Specific function:
Catalyzes the synthesis of N-acetylaspartyl-glutamate (NAAG).
Gene Name:
RIMKLA
Uniprot ID:
Q8IXN7
Molecular weight:
42863.8
Reactions
Adenosine triphosphate + N-Acetyl-L-aspartic acid + L-Glutamic acid → ADP + Phosphoric acid + N-acetylaspartyl-glutamatedetails
General function:
Involved in catalytic activity
Specific function:
Catalyzes the synthesis of beta-citryl-glutamate and N-acetyl-aspartyl-glutamate. Beta-citryl-glutamate is synthesized more efficiently than N-acetyl-aspartyl-glutamate (By similarity).
Gene Name:
RIMKLB
Uniprot ID:
Q9ULI2
Molecular weight:
42463.575
Reactions
Adenosine triphosphate + N-Acetyl-L-aspartic acid + L-Glutamic acid → ADP + Phosphoric acid + N-acetylaspartyl-glutamatedetails
General function:
Not Available
Specific function:
Plays a role in the regulation of lipogenesis by producing N-acetylaspartate acid (NAA), a brain-specific metabolite. NAA occurs in high concentration in brain and its hydrolysis plays a significant part in the maintenance of intact white matter. Promotes dopamine uptake by regulating TNF-alpha expression. Attenuates methamphetamine-induced inhibition of dopamine uptake.
Gene Name:
NAT8L
Uniprot ID:
Q8N9F0
Molecular weight:
32836.875
Reactions
Acetyl-CoA + L-Aspartic acid → Coenzyme A + N-Acetyl-L-aspartic aciddetails