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Record Information

Version

5.0

Status

Detected and Quantified

Creation Date

2005-11-16 15:48:42 UTC

Update Date

2025-05-29 19:32:58 UTC

HMDB ID

HMDB0000510

Secondary Accession Numbers

HMDB00510

Metabolite Identification

Common Name

Aminoadipic acid

Description

Aminoadipic acid (CAS: 542-32-5), also known as 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. It antagonizes neuroexcitatory activity modulated by the glutamate receptor N-methyl-D-aspartate (NMDA). Aminoadipic acid has also been shown to inhibit the production of kynurenic acid, a broad spectrum excitatory amino acid receptor antagonist, in brain tissue slices (PMID: 8566117 ). 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 aciduria and 2-oxoadipic aciduria via impaired decarboxylation of 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 form 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 three branched-chain amino acids: leucine, valine, and isoleucine) 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 can also lead to elevated levels of aminoadipate which is thought 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 ). Therefore, depending on the circumstances aminoadipic acid can act as an acidogen, a diabetogen, an atherogen, and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A diabetogen is a compound that can lead to type 2 diabetes. An atherogen is a compound that leads to atherosclerosis and cardiovascular disease. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of aminoadipic acid are associated with at least two inborn errors of metabolism including 2-aminoadipic aciduria and 2-oxoadipic aciduria. Aminoadipic acid is an organic acid and abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart abnormalities, kidney abnormalities, liver damage, seizures, coma, and possibly death. These are also the characteristic symptoms of the untreated IEMs mentioned above. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and seizures. As a diabetogen, serum aminoadipic levels appear to regulate glucose homeostasis and have been highly predictive of individuals who later develop diabetes (PMID: 24091325 ). In particular, aminoadipic acid lowers fasting plasma glucose levels and enhances insulin secretion from human islets. As an atherogen, aminoadipic acid has been found to be produced at high levels via protein lysine oxidation in atherosclerotic plaques (PMID: 28069522 ).

Structure

MOL 3D MOL SDF 3D SDF PDB 3D PDB SMILES InChI

View in JSmol View NMR Assignments View Stereo Labels

Synonyms

Value	Source
(S)-2-Aminohexanedioic acid	ChEBI
L-2-Aminoadipate	ChEBI
L-2-Aminohexanedioate	ChEBI
L-alpha-Aminoadipate	ChEBI
L-alpha-Aminoadipic acid	ChEBI
L-2-Aminoadipic acid	Kegg
(S)-2-Aminohexanedioate	Generator
L-2-Aminohexanedioic acid	Generator
L-a-Aminoadipate	Generator
L-a-Aminoadipic acid	Generator
L-Α-aminoadipate	Generator
L-Α-aminoadipic acid	Generator
Aminoadipate	Generator
(2S)-2-Aminohexanedioic acid	HMDB
(2S)-2-Azaniumyl-6-hydroxy-6-oxohexanoate	HMDB
(S)-2-Aminoadipic acid	HMDB
2-Aminoadipate	HMDB
2-Aminoadipic acid	HMDB
2-Aminohexanedioic acid	HMDB
L-Aminoadipic acid	HMDB
alpha-Aminoadipic acid	HMDB
Α-aminoadipic acid	HMDB

Chemical Formula

C₆H₁₁NO₄

Average Molecular Weight

161.1558

Monoisotopic Molecular Weight

161.068807845

IUPAC Name

(2S)-2-aminohexanedioic acid

Traditional Name

aminoadipate

CAS Registry Number

1118-90-7

SMILES

N[C@@H](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)/t4-/m0/s1

InChI Key

OYIFNHCXNCRBQI-BYPYZUCNSA-N

Chemical Taxonomy

Description

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.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Carboxylic acids and derivatives

Sub Class

Amino acids, peptides, and analogues

Direct Parent

L-alpha-amino acids

Alternative Parents

Substituents

L-alpha-amino acid
Medium-chain fatty acid
Amino fatty acid
Dicarboxylic acid or derivatives
Fatty acid
Fatty acyl
Amino acid
Carboxylic acid
Organopnictogen compound
Organic nitrogen compound
Amine
Organic oxygen compound
Primary amine
Organooxygen compound
Organonitrogen compound
Primary aliphatic amine
Carbonyl group
Hydrocarbon derivative
Organic oxide
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

2-aminoadipic acid (CHEBI:37023 )

Ontology

Not Available

Physical Properties

State

Solid

Experimental Molecular Properties

Property	Value	Reference
Melting Point	196 - 198 °C	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	2.2 mg/mL	Not Available
LogP	Not Available	Not Available

Experimental Chromatographic Properties

Not Available

Predicted Molecular Properties

Property	Value	Source
Water Solubility	32.7 g/L	ALOGPS
logP	-3.4	ALOGPS
logP	-2.8	ChemAxon
logS	-0.69	ALOGPS
pKa (Strongest Acidic)	2.01	ChemAxon
pKa (Strongest Basic)	9.53	ChemAxon
Physiological Charge	-1	ChemAxon
Hydrogen Acceptor Count	5	ChemAxon
Hydrogen Donor Count	3	ChemAxon
Polar Surface Area	100.62 Å²	ChemAxon
Rotatable Bond Count	5	ChemAxon
Refractivity	35.89 m³·mol⁻¹	ChemAxon
Polarizability	15.53 Å³	ChemAxon
Number of Rings	0	ChemAxon
Bioavailability	Yes	ChemAxon
Rule of Five	Yes	ChemAxon
Ghose Filter	No	ChemAxon
Veber's Rule	No	ChemAxon
MDDR-like Rule	No	ChemAxon

Predicted Chromatographic Properties

Predicted Collision Cross Sections

Predictor	Adduct Type	CCS Value (Å²)	Reference
DeepCCS	[M+H]+	132.248	30932474
DeepCCS	[M-H]-	128.421	30932474
DeepCCS	[M-2H]-	166.079	30932474
DeepCCS	[M+Na]+	141.618	30932474
AllCCS	[M+H]+	136.4	32859911
AllCCS	[M+H-H2O]+	132.5	32859911
AllCCS	[M+NH4]+	140.0	32859911
AllCCS	[M+Na]+	141.1	32859911
AllCCS	[M-H]-	131.0	32859911
AllCCS	[M+Na-2H]-	132.8	32859911
AllCCS	[M+HCOO]-	134.8	32859911

Predicted Retention Times

Underivatized

Chromatographic Method	Retention Time	Reference
Measured using a Waters Acquity ultraperformance liquid chromatography (UPLC) ethylene-bridged hybrid (BEH) C18 column (100 mm × 2.1 mm; 1.7 μmparticle diameter). Predicted by Afia on May 17, 2022. Predicted by Afia on May 17, 2022.	0.97 minutes	32390414
Predicted by Siyang on May 30, 2022	9.3472 minutes	33406817
Predicted by Siyang using ReTip algorithm on June 8, 2022	8.04 minutes	32390414
AjsUoB = Accucore 150 Amide HILIC with 10mM Ammonium Formate, 0.1% Formic Acid	399.8 seconds	40023050
Fem_Long = Waters ACQUITY UPLC HSS T3 C18 with Water:MeOH and 0.1% Formic Acid	511.0 seconds	40023050
Fem_Lipids = Ascentis Express C18 with (60:40 water:ACN):(90:10 IPA:ACN) and 10mM NH4COOH + 0.1% Formic Acid	299.3 seconds	40023050
Life_Old = Waters ACQUITY UPLC BEH C18 with Water:(20:80 acetone:ACN) and 0.1% Formic Acid	48.6 seconds	40023050
Life_New = RP Waters ACQUITY UPLC HSS T3 C18 with Water:(30:70 MeOH:ACN) and 0.1% Formic Acid	176.4 seconds	40023050
RIKEN = Waters ACQUITY UPLC BEH C18 with Water:ACN and 0.1% Formic Acid	62.6 seconds	40023050
Eawag_XBridgeC18 = XBridge C18 3.5u 2.1x50 mm with Water:MeOH and 0.1% Formic Acid	271.4 seconds	40023050
BfG_NTS_RP1 =Agilent Zorbax Eclipse Plus C18 (2.1 mm x 150 mm, 3.5 um) with Water:ACN and 0.1% Formic Acid	225.5 seconds	40023050
HILIC_BDD_2 = Merck SeQuant ZIC-HILIC with ACN(0.1% formic acid):water(16 mM ammonium formate)	811.1 seconds	40023050
UniToyama_Atlantis = RP Waters Atlantis T3 (2.1 x 150 mm, 5 um) with ACN:Water and 0.1% Formic Acid	593.2 seconds	40023050
BDD_C18 = Hypersil Gold 1.9µm C18 with Water:ACN and 0.1% Formic Acid	37.8 seconds	40023050
UFZ_Phenomenex = Kinetex Core-Shell C18 2.6 um, 3.0 x 100 mm, Phenomenex with Water:MeOH and 0.1% Formic Acid	675.4 seconds	40023050
SNU_RIKEN_POS = Waters ACQUITY UPLC BEH C18 with Water:ACN and 0.1% Formic Acid	175.7 seconds	40023050
RPMMFDA = Waters ACQUITY UPLC BEH C18 with Water:ACN and 0.1% Formic Acid	226.4 seconds	40023050
MTBLS87 = Merck SeQuant ZIC-pHILIC column with ACN:Water and :ammonium carbonate	806.6 seconds	40023050
KI_GIAR_zic_HILIC_pH2_7 = Merck SeQuant ZIC-HILIC with ACN:Water and 0.1% FA	490.6 seconds	40023050
Meister zic-pHILIC pH9.3 = Merck SeQuant ZIC-pHILIC column with ACN:Water 5mM NH4Ac pH9.3 and 5mM ammonium acetate in water	478.3 seconds	40023050

Predicted Kovats Retention Indices

Underivatized

Metabolite	SMILES	Kovats RI Value	Column Type	Reference
Aminoadipic acid	N[C@@H](CCCC(O)=O)C(O)=O	2314.5	Standard polar	33892256
Aminoadipic acid	N[C@@H](CCCC(O)=O)C(O)=O	1346.5	Standard non polar	33892256
Aminoadipic acid	N[C@@H](CCCC(O)=O)C(O)=O	1771.2	Semi standard non polar	33892256

Derivatized

Derivative Name / Structure	SMILES	Kovats RI Value	Column Type	Reference
Aminoadipic acid,1TMS,isomer #1	C[Si](C)(C)OC(=O)CCC[C@H](N)C(=O)O	1613.1	Semi standard non polar	33892256
Aminoadipic acid,1TMS,isomer #2	C[Si](C)(C)OC(=O)[C@@H](N)CCCC(=O)O	1567.0	Semi standard non polar	33892256
Aminoadipic acid,1TMS,isomer #3	C[Si](C)(C)N[C@@H](CCCC(=O)O)C(=O)O	1630.7	Semi standard non polar	33892256
Aminoadipic acid,2TMS,isomer #1	C[Si](C)(C)OC(=O)CCC[C@H](N)C(=O)O[Si](C)(C)C	1619.8	Semi standard non polar	33892256
Aminoadipic acid,2TMS,isomer #2	C[Si](C)(C)N[C@@H](CCCC(=O)O[Si](C)(C)C)C(=O)O	1706.9	Semi standard non polar	33892256
Aminoadipic acid,2TMS,isomer #3	C[Si](C)(C)N[C@@H](CCCC(=O)O)C(=O)O[Si](C)(C)C	1676.2	Semi standard non polar	33892256
Aminoadipic acid,2TMS,isomer #4	C[Si](C)(C)N([C@@H](CCCC(=O)O)C(=O)O)[Si](C)(C)C	1829.2	Semi standard non polar	33892256
Aminoadipic acid,3TMS,isomer #1	C[Si](C)(C)N[C@@H](CCCC(=O)O[Si](C)(C)C)C(=O)O[Si](C)(C)C	1723.0	Semi standard non polar	33892256
Aminoadipic acid,3TMS,isomer #1	C[Si](C)(C)N[C@@H](CCCC(=O)O[Si](C)(C)C)C(=O)O[Si](C)(C)C	1763.3	Standard non polar	33892256
Aminoadipic acid,3TMS,isomer #1	C[Si](C)(C)N[C@@H](CCCC(=O)O[Si](C)(C)C)C(=O)O[Si](C)(C)C	1896.1	Standard polar	33892256
Aminoadipic acid,3TMS,isomer #2	C[Si](C)(C)OC(=O)CCC[C@@H](C(=O)O)N([Si](C)(C)C)[Si](C)(C)C	1874.8	Semi standard non polar	33892256
Aminoadipic acid,3TMS,isomer #2	C[Si](C)(C)OC(=O)CCC[C@@H](C(=O)O)N([Si](C)(C)C)[Si](C)(C)C	1807.8	Standard non polar	33892256
Aminoadipic acid,3TMS,isomer #2	C[Si](C)(C)OC(=O)CCC[C@@H](C(=O)O)N([Si](C)(C)C)[Si](C)(C)C	2057.4	Standard polar	33892256
Aminoadipic acid,3TMS,isomer #3	C[Si](C)(C)OC(=O)[C@H](CCCC(=O)O)N([Si](C)(C)C)[Si](C)(C)C	1861.9	Semi standard non polar	33892256
Aminoadipic acid,3TMS,isomer #3	C[Si](C)(C)OC(=O)[C@H](CCCC(=O)O)N([Si](C)(C)C)[Si](C)(C)C	1800.2	Standard non polar	33892256
Aminoadipic acid,3TMS,isomer #3	C[Si](C)(C)OC(=O)[C@H](CCCC(=O)O)N([Si](C)(C)C)[Si](C)(C)C	2049.0	Standard polar	33892256
Aminoadipic acid,4TMS,isomer #1	C[Si](C)(C)OC(=O)CCC[C@@H](C(=O)O[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C	1903.7	Semi standard non polar	33892256
Aminoadipic acid,4TMS,isomer #1	C[Si](C)(C)OC(=O)CCC[C@@H](C(=O)O[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C	1865.6	Standard non polar	33892256
Aminoadipic acid,4TMS,isomer #1	C[Si](C)(C)OC(=O)CCC[C@@H](C(=O)O[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C	1822.6	Standard polar	33892256
Aminoadipic acid,1TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)CCC[C@H](N)C(=O)O	1867.3	Semi standard non polar	33892256
Aminoadipic acid,1TBDMS,isomer #2	CC(C)(C)[Si](C)(C)OC(=O)[C@@H](N)CCCC(=O)O	1833.1	Semi standard non polar	33892256
Aminoadipic acid,1TBDMS,isomer #3	CC(C)(C)[Si](C)(C)N[C@@H](CCCC(=O)O)C(=O)O	1904.3	Semi standard non polar	33892256
Aminoadipic acid,2TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)CCC[C@H](N)C(=O)O[Si](C)(C)C(C)(C)C	2076.3	Semi standard non polar	33892256
Aminoadipic acid,2TBDMS,isomer #2	CC(C)(C)[Si](C)(C)N[C@@H](CCCC(=O)O[Si](C)(C)C(C)(C)C)C(=O)O	2204.0	Semi standard non polar	33892256
Aminoadipic acid,2TBDMS,isomer #3	CC(C)(C)[Si](C)(C)N[C@@H](CCCC(=O)O)C(=O)O[Si](C)(C)C(C)(C)C	2135.3	Semi standard non polar	33892256
Aminoadipic acid,2TBDMS,isomer #4	CC(C)(C)[Si](C)(C)N([C@@H](CCCC(=O)O)C(=O)O)[Si](C)(C)C(C)(C)C	2280.7	Semi standard non polar	33892256
Aminoadipic acid,3TBDMS,isomer #1	CC(C)(C)[Si](C)(C)N[C@@H](CCCC(=O)O[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C	2381.4	Semi standard non polar	33892256
Aminoadipic acid,3TBDMS,isomer #1	CC(C)(C)[Si](C)(C)N[C@@H](CCCC(=O)O[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C	2343.2	Standard non polar	33892256
Aminoadipic acid,3TBDMS,isomer #1	CC(C)(C)[Si](C)(C)N[C@@H](CCCC(=O)O[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C	2272.6	Standard polar	33892256
Aminoadipic acid,3TBDMS,isomer #2	CC(C)(C)[Si](C)(C)OC(=O)CCC[C@@H](C(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2549.6	Semi standard non polar	33892256
Aminoadipic acid,3TBDMS,isomer #2	CC(C)(C)[Si](C)(C)OC(=O)CCC[C@@H](C(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2399.4	Standard non polar	33892256
Aminoadipic acid,3TBDMS,isomer #2	CC(C)(C)[Si](C)(C)OC(=O)CCC[C@@H](C(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2342.7	Standard polar	33892256
Aminoadipic acid,3TBDMS,isomer #3	CC(C)(C)[Si](C)(C)OC(=O)[C@H](CCCC(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2526.9	Semi standard non polar	33892256
Aminoadipic acid,3TBDMS,isomer #3	CC(C)(C)[Si](C)(C)OC(=O)[C@H](CCCC(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2370.5	Standard non polar	33892256
Aminoadipic acid,3TBDMS,isomer #3	CC(C)(C)[Si](C)(C)OC(=O)[C@H](CCCC(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2341.2	Standard polar	33892256
Aminoadipic acid,4TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)CCC[C@@H](C(=O)O[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2774.1	Semi standard non polar	33892256
Aminoadipic acid,4TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)CCC[C@@H](C(=O)O[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2607.9	Standard non polar	33892256
Aminoadipic acid,4TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)CCC[C@@H](C(=O)O[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2313.2	Standard polar	33892256

Spectra

Biological Properties

Cellular Locations

Cytoplasm
Mitochondria

Biospecimen Locations

Blood
Feces
Saliva
Urine

Tissue Locations

Placenta
Prostate

Pathways

Name	SMPDB/PathBank	KEGG
Lysine degradation	Not Available
Glutaric Aciduria Type I		Not Available
Saccharopinuria/Hyperlysinemia II		Not Available
Hyperlysinemia I, Familial		Not Available
Hyperlysinemia II or Saccharopinuria		Not Available

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	0.65 ± 0.51 uM	Adult (>18 years old)	Both	Normal	Quantitative comp...	details
Blood	Detected and Quantified	2.0 (0.0-5.0) uM	Adult (>18 years old)	Both	Normal	8764822	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	32966057	details
Blood	Detected and Quantified	1.14(0.81-9.66) uM	Children (1-13 uears old)	Both	Normal	27294788	details
Blood	Detected and Quantified	0.30-3.40 uM	Adult (>18 years old)	Both	Normal	Molecular You	details
Blood	Detected and Quantified	0 uM	Children (1 - 13 years old)	Female	Normal	117247	details
Blood	Detected and Quantified	0.30-3.40 uM	Adult (>18 years old)	Both	Normal	Molecular You	details
Blood	Detected and Quantified	0.42 ± 0.37 uM	Adult (>18 years old)	Both	Normal	Quantitative comp...	details
Blood	Detected and Quantified	0.37 ± 0.31 uM	Adult (>18 years old)	Both	Normal	Quantitative comp...	details
Blood	Detected and Quantified	0.74 ± 0.75 uM	Adult (>18 years old)	Both	Normal	Quantitative comp...	details
Blood	Detected and Quantified	0.47 ± 0.33 uM	Adult (>18 years old)	Both	Normal	Quantitative comp...	details
Blood	Detected and Quantified	0.39 ± 0.28 uM	Adult (>18 years old)	Both	Normal	Quantitative comp...	details
Blood	Detected and Quantified	0.35 ± 0.29 uM	Adult (>18 years old)	Both	Normal	Quantitative comp...	details
Blood	Detected and Quantified	0.51 ± 0.5 uM	Adult (>18 years old)	Both	Normal	Quantitative comp...	details
Blood	Detected and Quantified	0.58 ± 0.47 uM	Adult (>18 years old)	Both	Normal	Quantitative comp...	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	27543620	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	29808030	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	25037050	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Normal	27609529	details
Saliva	Detected and Quantified	0.336 +/- 0.475 uM	Adult (>18 years old)	Male	Normal	Sugimoto et al. (...	details
Saliva	Detected and Quantified	0.804 +/- 1.44 uM	Adult (>18 years old)	Female	Normal	Sugimoto et al. (...	details
Saliva	Detected and Quantified	0.821 +/- 0.408 uM	Adult (>18 years old)	Female	Normal	Sugimoto et al. (...	details
Saliva	Detected and Quantified	0.952 +/- 1.49 uM	Adult (>18 years old)	Female	Normal	Sugimoto et al. (...	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	28157703	details
Urine	Detected and Quantified	<5.32 umol/mmol creatinine	Adult (>18 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	<8.60 umol/mmol creatinine	Adolescent (13-18 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	<9.50 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	1.13-15.27 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	1.70-36.65 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	1.13-31.1 umol/mmol creatinine	Infant (0-1 year old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	6.09 +/- 3.36 umol/mmol creatinine	Newborn (0-30 days old)	Male	Normal	32340350	details
Urine	Detected and Quantified	8.52 +/- 4.45 umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	32340350	details
Urine	Detected and Quantified	1.23-15.5 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	32340350	details
Urine	Detected and Quantified	24.87 +/- 15.83 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	19468821	details
Urine	Detected and Quantified	19.22 +/- 15.83 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	19468821	details
Urine	Detected and Quantified	15.83 +/- 12.44 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	19468821	details
Urine	Detected and Quantified	0.633 +/- 1.0288 umol/mmol creatinine	Not Specified	Not Specified	Normal	8487492	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	22024767	details
Urine	Detected and Quantified	1.9-4.3 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	27012787	details
Urine	Detected and Quantified	1.7-4.2 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	27012787	details
Urine	Detected and Quantified	40.736 +/- 34.234 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Analysis of 30 no...	details
Urine	Detected and Quantified	20.4023 +/- 11.4518 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Analysis of 30 no...	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	21389975	details
Urine	Detected and Quantified	7.3 (3.4-11.2) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	7.2 (2.5-16.4) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	4.6 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	19309105	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	48.0 uM	Children (1-13 years old)	Both	alpha-Aminoadipic aciduria	MetaGene: Metabol...	details
Blood	Detected and Quantified	55.0 (30.0-80.0) uM	Children (1-13 years old)	Both	2-Ketoadipic Acidemia	MetaGene: Metabol...	details
Blood	Detected and Quantified	18.7 uM	Children (1-13 years old)	Male	Alpha-aminoadipic and alpha-ketoadipic aciduria	8487492	details
Blood	Detected and Quantified	1.07(0.70-3.17) uM	Children (1-13 uears old)	Both	Environmental enteric dysfunction	27294788	details
Blood	Detected and Quantified	6.0 (7.0-8.0) uM	Adult (>18 years old)	Both	alpha-Aminoadipic aciduria	8764822	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Unclassified IBD	27609529	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	25037050	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	27015276	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Metastatic melanoma	28923537	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Ulcerative colitis	27609529	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Crohns disease	27609529	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Both	Ulcerative colitis	27609529	details
Urine	Detected and Quantified	148 umol/mmol creatinine	Adolescent (13-18 years old)	Male	alpha-Ketoadipic Aciduria	6774165	details
Urine	Detected and Quantified	240-1910 umol/mmol creatinine	Children (1-13 years old)	Not Specified	alpha-Aminoadipic aciduria	6796766	details
Urine	Detected and Quantified	1243 umol/mmol creatinine	Infant (0-1 year old)	Not Specified	alpha-ketoadipic aciduria	10503694	details
Urine	Detected and Quantified	176 umol/mmol creatinine	Newborn (0-30 days old)	Not Specified	alpha-ketoadipic aciduria	10503694	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Schizophrenia	22024767	details
Urine	Detected and Quantified	42.612 umol/mmol creatinine	Children (1-13 years old)	Male	Alpha-aminoadipic and alpha-ketoadipic aciduria	8487492	details
Urine	Detected and Quantified	45.554 +/- 68.592 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Analysis of 30 no...	details
Urine	Detected and Quantified	14.0068 +/- 9.2776 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Gastroesophageal reflux disease	Analysis of 30 no...	details
Urine	Detected and Quantified	14.3982 +/- 9.7148 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Analysis of 30 no...	details
Urine	Detected and Quantified	13000 umol/mmol creatinine	Children (1-13 years old)	Both	alpha-Aminoadipic aciduria	MetaGene: Metabol...	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Bladder cancer	28157703	details

Associated Disorders and Diseases

Disease 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:8764822 ] Gray RG, O'Neill EM, Pollitt RJ: Alpha-aminoadipic aciduria: chemical and enzymatic studies. J Inherit Metab Dis. 1980;2(4):89-92. [PubMed:6796766 ] G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
2-Ketoadipic acidemia
Chalmers RA, Lawson AM, Watts RW, Tavill AS, Kamerling JP, Hey E, Ogilvie D: D-2-hydroxyglutaric aciduria: case report and biochemical studies. J Inherit Metab Dis. 1980;3(1):11-5. [PubMed:6774165 ] Peng H, Shinka T, Inoue Y, Mitsubuchi H, Ishimatsu J, Yoshino M, Kuhara T: Asymptomatic alpha-ketoadipic aciduria detected during a pilot study of neonatal urine screening. Acta Paediatr. 1999 Aug;88(8):911-4. [PubMed:10503694 ] G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Ulcerative colitis
Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
Crohn's disease
Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
Metastatic melanoma
Frankel AE, Coughlin LA, Kim J, Froehlich TW, Xie Y, Frenkel EP, Koh AY: Metagenomic Shotgun Sequencing and Unbiased Metabolomic Profiling Identify Specific Human Gut Microbiota and Metabolites Associated with Immune Checkpoint Therapy Efficacy in Melanoma Patients. Neoplasia. 2017 Oct;19(10):848-855. doi: 10.1016/j.neo.2017.08.004. Epub 2017 Sep 15. [PubMed:28923537 ]
Colorectal cancer
Sinha R, Ahn J, Sampson JN, Shi J, Yu G, Xiong X, Hayes RB, Goedert JJ: Fecal Microbiota, Fecal Metabolome, and Colorectal Cancer Interrelations. PLoS One. 2016 Mar 25;11(3):e0152126. doi: 10.1371/journal.pone.0152126. eCollection 2016. [PubMed:27015276 ] Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ]
Eosinophilic esophagitis
Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Schizophrenia
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. [PubMed:22024767 ]
Alpha-aminoadipic and alpha-ketoadipic aciduria
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:8487492 ]

Associated OMIM IDs

245130 (2-Ketoadipic acidemia)
266600 (Crohn's disease)
114500 (Colorectal cancer)
610247 (Eosinophilic esophagitis)
181500 (Schizophrenia)

External Links

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

KNApSAcK ID

Chemspider ID

KEGG Compound ID

BioCyc ID

BiGG ID

Not Available

Wikipedia Link

Alpha-Aminoadipic_acid

METLIN ID

Not Available

PubChem Compound

92136

PDB ID

Not Available

ChEBI ID

37023

Food Biomarker Ontology

Not Available

VMH ID

L2AADP

MarkerDB ID

MDB00000178

Good Scents ID

Not Available

References

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.

Material Safety Data Sheet (MSDS)

Download (PDF)

General References

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:11083877 ]
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. doi: 10.1038/nature07762. [PubMed:19212411 ]
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:8759641 ]
Hori S: [Pathophysiology of intraocular neovascularization]. Nippon Ganka Gakkai Zasshi. 1990 Dec;94(12):1103-21. [PubMed:1707214 ]
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:9679552 ]
Mechanic GL: The qualitative and quantitative crosslink chemistry of collagen matrices. Adv Exp Med Biol. 1977;86B:699-708. [PubMed:20755 ]
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:8087205 ]
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:8487492 ]
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. [PubMed:8566117 ]
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 Dec 15;133(12):2914-24. doi: 10.1002/ijc.28303. Epub 2013 Jun 29. [PubMed:23737455 ]
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. [PubMed:23141293 ]
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. [PubMed:17313367 ]
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. [PubMed:15389298 ]
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. [PubMed:21961526 ]
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. [PubMed:21647514 ]
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. [PubMed:18448817 ]
Wang TJ, Ngo D, Psychogios N, Dejam A, Larson MG, Vasan RS, Ghorbani A, O'Sullivan J, Cheng S, Rhee EP, Sinha S, McCabe E, Fox CS, O'Donnell CJ, Ho JE, Florez JC, Magnusson M, Pierce KA, Souza AL, Yu Y, Carter C, Light PE, Melander O, Clish CB, Gerszten RE: 2-Aminoadipic acid is a biomarker for diabetes risk. J Clin Invest. 2013 Oct;123(10):4309-17. doi: 10.1172/JCI64801. Epub 2013 Sep 16. [PubMed:24091325 ]
Lin H, Levison BS, Buffa JA, Huang Y, Fu X, Wang Z, Gogonea V, DiDonato JA, Hazen SL: Myeloperoxidase-mediated protein lysine oxidation generates 2-aminoadipic acid and lysine nitrile in vivo. Free Radic Biol Med. 2017 Mar;104:20-31. doi: 10.1016/j.freeradbiomed.2017.01.006. Epub 2017 Jan 6. [PubMed:28069522 ]
Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043. [PubMed:32033212 ]

Enzymes

Enzyme Details

1. Alpha-aminoadipic semialdehyde dehydrogenase

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

Allysine + NAD(P)(+) + Water → Aminoadipic acid + NAD(P)H	details
Aminoadipic acid + Adenosine triphosphate → L-2-Aminoadipate adenylate + Pyrophosphate	details
Allysine + NAD + Water → Aminoadipic acid + NADH + Hydrogen Ion	details
Allysine + NADP + Water → Aminoadipic acid + NADPH + Hydrogen Ion	details

Enzyme Details

2. Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial

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 + Glutamic acid	details

Enzyme Details

3. Aminoadipate aminotransferase, isoform CRA_b

General function:: Transcription
Specific function:: Not Available
Gene Name:: AADAT
Uniprot ID:: Q4W5N8
Molecular weight:: 47351.2

Enzyme Details

4. Acyl-CoA synthetase family member 4

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

Enzyme Details

5. L-aminoadipate-semialdehyde dehydrogenase-phosphopantetheinyl transferase

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

Showing metabocard for Aminoadipic acid (HMDB0000510)

MOL for HMDB0000510 (Aminoadipic acid)

3D MOL for HMDB0000510 (Aminoadipic acid)

3D SDF for HMDB0000510 (Aminoadipic acid)

3D-SDF for HMDB0000510 (Aminoadipic acid)

PDB for HMDB0000510 (Aminoadipic acid)

3D PDB for HMDB0000510 (Aminoadipic acid)

SMILES for HMDB0000510 (Aminoadipic acid)

INCHI for HMDB0000510 (Aminoadipic acid)

Structure for HMDB0000510 (Aminoadipic acid)

3D Structure for HMDB0000510 (Aminoadipic acid)

Predicted Collision Cross Sections

Predicted Retention Times

Underivatized

Predicted Kovats Retention Indices

Underivatized

Derivatized

Enzymes

Reactions

Reactions