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Human Metabolome Database Version 2.5

 

Showing metabocard for Aminoadipic acid (HMDB00510)

Legend: metabolite field enzyme field

Version 2.5
Creation Date 2005-11-16 15:48:42
Update Date 2010-04-28 13:25:36
Accession Number HMDB00510
Secondary Accession Numbers Not Available
Common Name Aminoadipic acid
Description A metabolite in the principal biochemical pathway of lysine. It antagonizes neuroexcitatory activity modulated by the glutamate receptor, N-methyl-D-aspartate; (NMDA). Alpha-aminoadipate is an intermediate in the metabolism of lysine and saccharopine.(Wikipedia)
Synonyms
  1. (+/-)-2-Aminoadipate
  2. (+/-)-2-Aminoadipic acid
  3. 2-Aminoadipate
  4. 2-Aminoadipic acid
  5. Aminoadipate
  6. DL-2-Aminoadipate
  7. DL-2-Aminoadipic acid
  8. DL-2-Aminohexanedioate
  9. DL-2-Aminohexanedioic acid
  10. DL-a-Aminoadipate
  11. DL-a-Aminoadipic acid
  12. a-Aminoadipate
  13. a-Aminoadipic acid
  14. alpha-Amino-adipic acid
  15. DL-alpha-Aminoadipate
  16. DL-alpha-Aminoadipic acid
  17. alpha-Aminoadipate
  18. alpha-Aminoadipic acid
  19. L-2-aminoadipate
  20. L-alpha-Aminoadipate
  21. L-alpha-Aminoadipic acid
  22. L-2-Aminoadipic acid
  23. L-2-Aminohexanedioate
  24. L-2-Aminohexanedioic acid
Chemical IUPAC Name 2-aminohexanedioic acid
Chemical Formula C6H11NO4
Chemical Structure Structure
Chemical Taxonomy
Kingdom
  • Organic
Super Class
  • Amino acids and Amino Acid conjugates
Class
  • Amino Acids
Sub Class
  • NA
Family
  • Mammalian Metabolite
Species
  • primary amine
  • primary aliphatic amine (alkylamine)
  • carboxylic acid
  • alpha-aminoacid
Biofunction
  • Protein synthesis, amino acid biosynthesis
Application
—
Source
  • Endogenous
Average Molecular Weight 161.156
Monoisotopic Molecular Weight 161.068802
Isomeric SMILES NC(CCCC(O)=O)C(O)=O
Canonical SMILES NC(CCCC(O)=O)C(O)=O
KEGG Compound ID C00956 Link Image
BioCyc ID CPD-468 Link Image
BiGG ID 1485280 Link Image
Wikipedia Link alpha-Aminoadipate Link Image
NuGOwiki Link HMDB00510 Link Image
Metagene Link HMDB00510 Link Image
METLIN ID 5496 Link Image
PubChem Compound 92136 Link Image
PubChem Substance 841339 Link Image
ChEBI ID 17082 Link Image
CAS Registry Number 542-32-5
InChI Identifier InChI=1/C6H11NO4/c7-4(6(10)11)2-1-3-5(8)9/h4H,1-3,7H2,(H,8,9)(H,10,11)
Synthesis Reference Khan, Naseem H.; Ali, Mohd.; Panda, H.; Rakhshinda. Synthesis of a-aminoadipic acid. Journal of the Indian Chemical Society (1982), 59(9), 1077-8.
Melting Point (Experimental) 196-198 oC
Experimental Water Solubility 2.2 mg/mL [YALKOWSKY,SH & DANNENFELSER,RM (1992)] Source: PhysProp
Predicted Water Solubility 32.7 mg/mL [Predicted by ALOGPS] Calculated using ALOGPS
Physiological Charge -1
State Solid
Experimental LogP/Hydrophobicity Not Available Source: PhysProp
Predicted LogP/Hydrophobicity -3.43 [Predicted by ALOGPS]; -3 [Predicted by PubChem via XLOGP]; -3.34 [MEYLAN,WM & HOWARD,PH (1995)] Calculated using ALOGPS
Material Safety Data Sheet (MSDS)
MOL File Show
SDF File Show
PDB File Show
2D Structure
3D Structure
Experimental PDB ID Not Available
Experimental 1H NMR Spectrum Download Spectrum
Download FID (Varian)
Show Experimental Conditions Link Image
Experimental 13C NMR Spectrum Not Available
Experimental 13C HSQC Spectrum Download Spectrum
Download FID (Bruker)
Show Experimental Conditions Link Image
Predicted 1H NMR Spectrum Show Image
Show Peaklist
Predicted 13C NMR Spectrum Show Image
Show Peaklist
Mass Spectrum
Low Energy
Download File
Show Experimental Conditions Link Image
Medium Energy
Download File
Show Experimental Conditions Link Image
High Energy
Download File
Show Experimental Conditions Link Image
Simplified TOCSY Spectrum Not Available
BMRB Spectrum Not Available
Cellular Location
  • Cytoplasm
  • mitochondria
Biofluid Location
  • Blood
  • Urine
Tissue Location Not Available
Concentrations (Normal)
Biofluid Blood
Value 2.0 (0.0-5.0) uM
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • 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. [PubMed Link Image]
Biofluid Urine
Value 4.6 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • Guo K, Li L: Differential (12)C-/(13)C-Isotope Dansylation Labeling and Fast Liquid Chromatography/Mass Spectrometry for Absolute and Relative Quantification of the Metabolome. Anal Chem. 2009 Mar 23. [PubMed Link Image]
Biofluid Urine
Value 54.2 (9.8-98.6) umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Patient information 2-Ketoadipic Acidemia
Comments Not Available
References
Concentrations (Abnormal)
Biofluid Blood
Value 6.0 (7.0-8.0) uM
Age Adult:>18 yrs old
Sex Both
Condition Alpha-aminoadipic aciduria
Comments Not Available
References
  • 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. [PubMed Link Image]
Biofluid Blood
Value 48.0 uM
Age Adult:>18 yrs old
Sex Both
Comments Not Available
References
Biofluid Blood
Value 55.0 (30.0-80.0) uM
Age Adult:>18 yrs old
Sex Both
Comments Not Available
References
Biofluid Urine
Value 13.00 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Comments Not Available
References
Associated Disorders
Condition References
Alpha-aminoadipic aciduria
  • 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. [PubMed Link Image]
OMIM ID Not Available
Pathways
Name SMPDB Link KEGG Link
Lysine Degradation SMP00037 Link Image map00310 Link Image
General References
  1. 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. [PubMed Link Image]
  2. Hori S: [Pathophysiology of intraocular neovascularization] Nippon Ganka Gakkai Zasshi. 1990 Dec;94(12):1103-21. [PubMed Link Image]
  3. 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. [PubMed Link Image]
  4. Mechanic GL: The qualitative and quantitative crosslink chemistry of collagen matrices. Adv Exp Med Biol. 1977;86B:699-708. [PubMed Link Image]
  5. 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. [PubMed Link Image]
  6. 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. [PubMed Link Image]
  7. 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. [PubMed Link Image]
  8. Wikipedia Link Image
Metabolic Enzymes
  1. Hypothetical protein AADAT
  2. 2-aminoadipic 6-semialdehyde dehydrogenase
  3. cDNA, FLJ92898, Homo sapiens aminoadipate-semialdehydedehydrogenase-phosphopantetheinyl transferase (AASDHPPT), mRNA (Aminoadipate-semialdehyde dehydrogenase-phosphopantetheinyl transferase, isoform CRA_b)
Enzyme 1 [top]
Enzyme 1 ID 8585
Enzyme 1 Name Hypothetical protein AADAT
Enzyme 1 Synonyms Not Available
Enzyme 1 Gene Name AADAT
Enzyme 1 Protein Sequence >Hypothetical protein AADAT 
MNYARFITAASAARNPSPIRTMTDILSRGPKSMISLAGGLPNPNMFPFKTAVITVENGKT
IQFGEEMMKRALQYSPSAGIPELLSWLKQLQIKLHNPPTIHYPPSQGQMDLCVTSGSQQG
LCKVFEMIINPGDNVLLDEPAYSGTLQSLHPLGCNIINVASDESGIVPDSLRDILSRWKP
EDAKNPQKNTPKFLYTVPNGNNPTGNSLTSERKKEIYELARKYDFLIIEDDPYYFLQFNK
FRVPTFLSMDVDGRVIRADSFSKIISSGLRIGFLTGPKPLIERVILHIQVSTLHPSTFNQ
LMISQLLHEWGEEGFMAHVDRVIDFYSNQKDAILAAADKWLTGLAEWHVPAAGMFLWIKV
KGINDVKELIEEKAVKMGVLMLPGNAFYVDSSAPSPYLRASFSSASPEQMDVAFQVLAQL
IKESL
Enzyme 1 Number of Residues 425
Enzyme 1 Molecular Weight 47352
Enzyme 1 Theoretical pI 6.96
Enzyme 1 GO Classification
Function
  • catalytic activity
  • transferase activity
  • transferase activity, transferring nitrogenous groups
Process
  • biosynthesis
  • metabolism
  • physiological process
Component
—
Enzyme 1 General Function Transcription
Enzyme 1 Specific Function Not Available
Enzyme 1 Pathways Not Available
Enzyme 1 Reactions Not Available
Enzyme 1 Pfam Domain Function
Enzyme 1 Signals Not Available
Enzyme 1 Transmembrane Regions Not Available
Enzyme 1 Essentiality Not Available
Enzyme 1 GenBank ID Protein 63995204 Link Image
Enzyme 1 UniProtKB/Swiss-Prot ID Q4W5N8 Link Image
Enzyme 1 UniProtKB/Swiss-Prot Entry Name Q4W5N8_HUMAN Link Image
Enzyme 1 PDB ID Not Available
Enzyme 1 Cellular Location Not Available
Enzyme 1 Gene Sequence >1278 bp 
ATGAATTACGCACGGTTCATCACGGCAGCGAGCGCAGCCAGAAACCCTTCTCCCATCCGG
ACCATGACTGACATATTGAGCAGAGGACCAAAATCGATGATCTCCTTGGCTGGTGGCTTA
CCAAATCCAAACATGTTTCCTTTTAAGACTGCCGTAATCACTGTAGAAAATGGAAAGACC
ATCCAATTTGGAGAAGAGATGATGAAGAGAGCACTTCAGTATTCTCCGAGTGCTGGAATT
CCAGAGCTTTTGTCCTGGCTAAAACAGTTACAAATAAAATTGCATAATCCTCCTACCATC
CATTACCCACCCAGTCAAGGACAAATGGATCTATGTGTCACATCTGGCAGCCAACAAGGT
CTTTGTAAGGTGTTTGAAATGATCATTAATCCTGGAGATAATGTCCTCCTAGATGAACCT
GCTTATTCAGGAACTCTTCAAAGTCTGCACCCACTGGGCTGCAACATTATTAATGTTGCC
AGTGATGAAAGTGGGATTGTTCCAGATTCCCTAAGAGACATACTTTCCAGATGGAAACCA
GAAGATGCAAAGAATCCCCAGAAAAACACCCCCAAATTTCTTTATACTGTTCCAAATGGC
AACAACCCTACTGGAAACTCATTAACCAGTGAACGCAAAAAGGAAATCTATGAGCTTGCA
AGAAAATATGATTTCCTCATAATAGAAGATGATCCTTACTATTTTCTCCAGTTTAACAAG
TTCAGGGTACCAACATTTCTTTCCATGGATGTTGATGGACGTGTCATCAGAGCTGACTCT
TTTTCAAAAATCATTTCCTCTGGGTTGAGAATAGGATTTTTAACTGGTCCAAAACCCTTA
ATAGAGAGAGTTATTTTACACATACAAGTTTCAACATTGCACCCCAGCACTTTTAACCAG
CTCATGATATCACAGCTTCTACACGAATGGGGAGAAGAAGGTTTCATGGCTCATGTAGAC
AGGGTTATTGATTTCTATAGTAACCAGAAGGATGCAATACTGGCAGCTGCAGACAAGTGG
TTAACTGGTTTGGCAGAATGGCATGTTCCTGCTGCTGGAATGTTTTTATGGATTAAAGTT
AAAGGCATTAATGATGTAAAAGAACTGATTGAAGAAAAGGCCGTTAAGATGGGGGTATTA
ATGCTCCCTGGAAATGCTTTCTACGTCGATAGCTCAGCTCCTAGCCCTTACTTGAGAGCA
TCCTTCTCTTCAGCTTCTCCAGAACAGATGGATGTGGCCTTCCAGGTATTAGCACAACTT
ATAAAAGAATCTTTATGA
Enzyme 1 GenBank Gene ID AC084866 Link Image
Enzyme 1 GeneCard ID AADAT Link Image
Enzyme 1 GenAtlas ID AADAT Link Image
Enzyme 1 HGNC ID HGNC:17929 Link Image
Enzyme 1 Chromosome Location 4
Enzyme 1 Locus 4q33
Enzyme 1 SNPs SNPJam Report Link Image
Enzyme 1 General References Not Available
Enzyme 1 Metabolite References Not Available
Enzyme 2 [top]
Enzyme 2 ID 13066
Enzyme 2 Name 2-aminoadipic 6-semialdehyde dehydrogenase
Enzyme 2 Synonyms Not Available
Enzyme 2 Gene Name Not Available
Enzyme 2 Protein Sequence >2-aminoadipic 6-semialdehyde dehydrogenase 
MTLQELVHKAASCYMDRVAVCFDECNNQLPVYYTYKTVVNAASELSNFLLLHCDFQGIRE
IGLYCQPGIDLPSWILGILQVPAAYVPIEPDSPPSLSTHFMKKCNLKYILVEKKQINKFK
SFHETLLNYDTFTVEHNDLVLFRLHWKNTEVNLMLNDGKEKYEKEKIKSISSEHVNEEKA
EEHMDLRLKHCLAYVLHTSGTTGIPKIVRVPHKCIVPNIQHFRVLFDITQEDVLFLASPL
TFDPSVVEIFLALSSGASLLIVPTSVKLLPSKLASVLFSHHRVTVLQATPTLLRRFGSQL
IKSTVLSATTSLRVLALGGEAFPSLTVLRSWRGEGNKTQIFNVYGITEVSSWATIYRIPE
KTLNSTLKCELPVQLGFPLLGTVVEVRDTNGFTIQEGSGQVFLGGRNRVCFLDDEVTVPL
GTMRATGGFVTVKDGEIFFLGRKDSQIKRHGKRLNIELVQQVAEELQQVESCAVTWYNQE
KLILFMVSKDASVKEYIFKELQKYLPSHAVPDELVLIDSLPFTSHGKIDVSELNKIYLNY
INLKSENKLSGKEDLWEKLQYLWKSTLNLPEDLLRVPDESLFLNSGGDSLKSIRLLSEIE
KLVGTSVPGLLEIILSSSILEIYNHILQTVVPDEDVTFRKSCATKRKLSDINQEEASGTS
LHQKAIMTFTCHNEINAFVVLSRGSQILSLNSTRFLTKLGHCSSACPSDSVSQTNIQNLK
GLNSPVLIGKSKDPSCVAKVSEEGKPAIGTQKMELHVRWRSDTGKCVDASPLVVIPTFDK
SSTTVYIGSHSHRMKAVDFYSGKVKWEQILGDRIESSACVSKCGNFIVVGCYNGLVYVLK
SNSGEKYWMFTTEDAVKSSATMDPTTGLIYIGSHDQHAYALDIYRKKCVWKSKCGGTVFS
SPCLNLIPHHLYFATLGGLLLAVNPATGNVIWKHSCGKPLFSSPQCCSQYICIGCVDGNL
LCFTHFGEQVWQFSTSGPIFSSPCTSPSEQKIFFGSHDCFIYCCNMKGHLQWKFETTSRV
YATPFAFHNDNGSNEMLLAAASTDGKVWILESQSGQLQSVYELPGEVFSSPVVLESMLII
GCRDNYVYCLDLLGGNQK
Enzyme 2 Number of Residues 1098
Enzyme 2 Molecular Weight 122492
Enzyme 2 Theoretical pI 7.26
Enzyme 2 GO Classification
Function
  • binding
  • catalytic activity
  • cofactor binding
Process
  • cellular metabolism
  • electron transport
  • generation of precursor metabolites and energy
  • metabolism
  • physiological process
Component
—
Enzyme 2 General Function Secondary metabolites biosynthesis, transport and catabolism
Enzyme 2 Specific Function Not Available
Enzyme 2 Pathways Not Available
Enzyme 2 Reactions Not Available
Enzyme 2 Pfam Domain Function
Enzyme 2 Signals
  • None
Enzyme 2 Transmembrane Regions
  • None
Enzyme 2 Essentiality Not Available
Enzyme 2 GenBank ID Protein 34867980 Link Image
Enzyme 2 UniProtKB/Swiss-Prot ID Q4L235 Link Image
Enzyme 2 UniProtKB/Swiss-Prot Entry Name Q4L235_HUMAN Link Image
Enzyme 2 PDB ID Not Available
Enzyme 2 Cellular Location Not Available
Enzyme 2 Gene Sequence Not Available
Enzyme 2 GenBank Gene ID AY314787 Link Image
Enzyme 2 GeneCard ID Q4L235 Link Image
Enzyme 2 GenAtlas ID AASDH Link Image
Enzyme 2 HGNC ID HGNC:23993 Link Image
Enzyme 2 Chromosome Location Not Available
Enzyme 2 Locus Not Available
Enzyme 2 SNPs Not Available
Enzyme 2 General References Not Available
Enzyme 2 Metabolite References Not Available
Enzyme 3 [top]
Enzyme 3 ID 16433
Enzyme 3 Name cDNA, FLJ92898, Homo sapiens aminoadipate-semialdehydedehydrogenase-phosphopantetheinyl transferase (AASDHPPT), mRNA (Aminoadipate-semialdehyde dehydrogenase-phosphopantetheinyl transferase, isoform CRA_b)
Enzyme 3 Synonyms Not Available
Enzyme 3 Gene Name AASDHPPT
Enzyme 3 Protein Sequence >cDNA, FLJ92898, Homo sapiens aminoadipate-semialdehydedehydrogenase-phosphopantetheinyl transferase (AASDHPPT), mRNA (Aminoadipate-semialdehyde dehydrogenase-phosphopantetheinyl transferase, isoform CRA_b) 
MVFPAKRFCLVPSMEGVRWAFSCGTWLPSRAEWLLAVRSIQPEEKERIGQFVFARDAKAA
MAGRLMIRKLVAEKLNIPWNHIRLQRTAKGKPVLAKDSSNPYPNFNFNISHQGDYAVLAA
EPELQVGIDIMKTSFPGRGSIPEFFHIMKRKFTNKEWETIRSFKDEWTQLDMFYRNWALK
ESFIKAIGVGLGFELQRLEFDLSPLNLDIGQVYKETRLFLDGEEEKEWAFEESKIDEHHF
VAVALRKPDGSRHQDVPSQDDSKPTQRQFTILNFNDLMSSAVPMTPEDPSFWDCFCFTEE
IPIRNGTKS
Enzyme 3 Number of Residues 309
Enzyme 3 Molecular Weight 35776
Enzyme 3 Theoretical pI 6.79
Enzyme 3 GO Classification
Function
  • binding
  • catalytic activity
  • ion binding
  • magnesium ion binding
  • metal ion binding
  • phosphopantetheinyltransferase activity
  • phosphotransferase activity, for other substituted phosphate groups
  • transferase activity
  • transferase activity, transferring phosphorus-containing groups
Process
  • carboxylic acid metabolism
  • cellular metabolism
  • fatty acid biosynthesis
  • fatty acid metabolism
  • metabolism
  • organic acid metabolism
  • physiological process
Component
—
Enzyme 3 General Function Coenzyme transport and metabolism
Enzyme 3 Specific Function Not Available
Enzyme 3 Pathways Not Available
Enzyme 3 Reactions Not Available
Enzyme 3 Pfam Domain Function
Enzyme 3 Signals
  • None
Enzyme 3 Transmembrane Regions
  • None
Enzyme 3 Essentiality Not Available
Enzyme 3 GenBank ID Protein Not Available
Enzyme 3 UniProtKB/Swiss-Prot ID B2R6D1 Link Image
Enzyme 3 UniProtKB/Swiss-Prot Entry Name B2R6D1_HUMAN Link Image
Enzyme 3 PDB ID Not Available
Enzyme 3 Cellular Location Not Available
Enzyme 3 Gene Sequence Not Available
Enzyme 3 GenBank Gene ID AK312529 Link Image
Enzyme 3 GeneCard ID B2R6D1 Link Image
Enzyme 3 GenAtlas ID Not Available
Enzyme 3 HGNC ID Not Available
Enzyme 3 Chromosome Location 11
Enzyme 3 Locus 11q22
Enzyme 3 SNPs SNPJam Report Link Image
Enzyme 3 General References Not Available
Enzyme 3 Metabolite References Not Available