MiMeDB: Showing metabocard for Aminoadipic acid (MMDBc0000520)

Record Information

Version

1.0

Status

Detected and Quantified

Creation Date

2020-12-10 18:39:38 UTC

Update Date

2024-04-30 19:32:46 UTC

Metabolite ID

MMDBc0000520

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

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

Molecular Formula

C₆H₁₁NO₄

Average Mass

161.1558

Monoisotopic Mass

161.068807845

IUPAC Name

(2S)-2-aminohexanedioic acid

Traditional Name

aminoadipate

CAS Registry Number

Not Available

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 )

Functional Ontology

Not Available

Physical Properties

State

Expected Solid

Predicted 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

Spectra

Spectrum Type	Description	Splash Key	Deposition Date	View
GC-MS	GC-MS Spectrum - GC-MS (3 TMS)	splash10-02vi-1890000000-cb81ff688a4a1d2ee926	2014-06-16	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	splash10-00xu-9200000000-2a666aa0f9c15fd2295b	2016-09-22	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_3) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_3) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_4) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_3) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_2_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_2_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_2_3) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_2_4) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 10V, Positive	splash10-0002-9600000000-77ab58046847c3ea82f0	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 20V, Positive	splash10-0a4j-9000000000-fced5385ac147b0fd6a0	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 40V, Positive	splash10-0a4i-9000000000-3f7c5858ac3e97d89e6e	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 30V, Positive	splash10-0a4i-9000000000-af6e2d829df2d68bd262	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 40V, Positive	splash10-0a4i-9000000000-3664a98748e89e99cd2d	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 10V, Positive	splash10-0002-9700000000-e1040cea3fd3c274b468	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 20V, Positive	splash10-052b-9000000000-c9da617e7f36084d0276	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 10V, Positive	splash10-00kb-9800000000-2738b9d8b78b33fed9f7	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 35V, Positive	splash10-00ke-7900000000-461c76f7d777bbdee97e	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 40V, Positive	splash10-0a4i-9000000000-b096dea8b50a6e2c75c4	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 10V, Positive	splash10-00ke-7900000000-cfb9ae7bd0906a939368	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 20V, Positive	splash10-0a4j-9000000000-7c27445e72a5da0c3bb6	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 10V, Positive	splash10-014j-6900000000-f349746a9f0635231c65	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 0V, Positive	splash10-03di-0900000000-1bc82027155d45c9a43d	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 30V, Positive	splash10-0a4i-9000000000-2749c34e547a235a0f78	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 0V, Positive	splash10-03di-0900000000-97545962897518937c04	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 30V, Positive	splash10-0a4i-9000000000-0e2bf30c88b4dafc0c5f	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 10V, Positive	splash10-0002-9500000000-83d347e56da993118fba	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 10V, Negative	splash10-03xu-1900000000-db1ee9542c240bfe00b7	2021-09-20	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-00kg-2900000000-3e45ac769d3b229221a3	2015-09-15	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-01ba-9600000000-c51318834cc371625d46	2015-09-15	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-05fu-9000000000-edbc970f12ea186bcf06	2015-09-15	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Negative	splash10-03di-1900000000-0f7002659f604cc69395	2015-09-15	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Negative	splash10-01ox-4900000000-bf42ddc971a2a05516a2	2015-09-15	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Negative	splash10-0abc-9100000000-1979c27463bab7d33d58	2015-09-15	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)		2021-09-16	View Spectrum

Biological Properties

Cellular Locations

Cytoplasm
Mitochondria

Biospecimen Locations

Blood
Feces
Placenta
Prostate
Saliva
Urine

Tissue Locations

Placenta
Prostate

Associated OMIM IDs

114500 (Colorectal cancer)
610247 (Eosinophilic esophagitis)

Human Proteins and Enzymes

Protein ID	Protein Name	Protein Type	Uniprot ID	Species
MMDBp0191963	Alpha-aminoadipic semialdehyde dehydrogenase	Unknown	P49419	Homo sapiens
MMDBp0192045	Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial	Enzyme	Q8N5Z0	Homo sapiens
MMDBp0193784	Aminoadipate aminotransferase, isoform CRA_b	Enzyme	Q4W5N8	Homo sapiens
MMDBp0194177	Acyl-CoA synthetase family member 4	Enzyme	Q4L235	Homo sapiens
MMDBp0196643	L-aminoadipate-semialdehyde dehydrogenase-phosphopantetheinyl transferase	Enzyme	Q9NRN7	Homo sapiens

Human Pathways

Pathways

Name	SMPDB/PathBank

Metabolic Reactions

Reaction ID	Precursor	Product	Enzyme	Reaction Type	Data Source	Reference
MMDBr0000039	Oxoglutaric acid	Oxoglutaric acid	Solute carrier family 25 (mitochondrial oxoadipate carrier), member 21	L-2-Aminoadipate Shuttle (Cytosol/Mitochondria)	VMH	11083877
MMDBr0000142	Water	Hydrogen Ion	Not Available	L-Aminoadipate-Semialdehyde Dehydrogenase (NADH), Mitochondrial	VMH; KEGG	15865210
MMDBr0000143	Oxoglutaric acid	Oxoadipic acid	Not Available	2-Aminoadipate Transaminase, Irreversible	VMH	12126930
MMDBr0002563	Oxoglutaric acid	Oxoadipic acid	Aminoadipate aminotransferase	L-2-Aminoadipate:2-Oxoglutarate Aminotransferase Lysine Biosynthesis Lysine Degradation	VMH; KEGG	30371894
MMDBr0002614	Water	Hydrogen Ion	Not Available	L-2-Aminoadipate-6-Semialdehyde:NAD+ 6-Oxidoreductase Lysine Degradation	VMH	2160277
MMDBr0003825	Aminoadipic acid	Aminoadipic acid	Not Available	Postulated Transport Reaction	VMH	10049700 10574970 11389679 11557028 11564694 12824232 12930836 14574404 16288981 911815
MMDBr0005593	Aminoadipic acid	Aminoadipic acid	Not Available	Transport of L-2-Aminoadipate	VMH	30371894
MMDBr0011007	Water	Hydrogen Ion	Not Available	L-2-Aminoadipate-6-semialdehyde:NAD(P)+ 6-oxidoreductase	VMH	30371894
MMDBr0011008	Adenosine triphosphate	L-2-Aminoadipate adenylate	Not Available	L-2-Aminoadipate-6-semialdehyde:NAD(P)+ 6-oxidoreductase	VMH	30371894
MMDBr0013105	L-Allysine	Hydrogen Ion	L-2-aminoadipate reductase large subunit	Reduction	RHEA	34755880
MMDBr0013106	L-Allysine	Hydrogen Ion	L-2-aminoadipate reductase large subunit	Reduction	RHEA	34755880
MMDBr0013133	alpha-Ketoglutarate	Oxoadipic acid	Aspartate aminotransferase	Amine group addition	RHEA	34755880
MMDBr0014026	Water	CoA	Acyl-coenzyme A:6-aminopenicillanic-acid-acyltransferase 40 kDa form	Acylation	RHEA	34755880
MMDBr0014027	Water	CoA	Acyl-coenzyme A:6-aminopenicillanic-acid-acyltransferase 40 kDa form	Acylation	RHEA	34755880
MMDBr0016266	L-Allysine	Adenosine triphosphate	L-2-aminoadipate reductase large subunit	Reduction	RHEA	34755880
MMDBr0017909	Adenosine triphosphate	Adenosine monophosphate	N-(5-amino-5-carboxypentanoyl)-L-cysteinyl-D-valine synthase	Synthesis	RHEA	34755880
MMDBr0017910	Adenosine triphosphate	Adenosine monophosphate	N-(5-amino-5-carboxypentanoyl)-L-cysteinyl-D-valine synthase	Synthesis	RHEA	34755880
MMDBr0019593	Aminoadipic acid	Adenosine monophosphate	L-2-aminoadipate reductase	Reduction	PathBank	31602464
MMDBr0025029	Adenosine triphosphate	Hydrogen Ion	Alpha-aminoadipate--LysW ligase LysX	Ligation	RHEA	34755880

Health Effects and Bioactivity

Health Outcome/Bioactivity	Metabolite Response/Effect	Evidence Type	Host and Biospecimen	Data Source	Reference
2-ketoadipic acidemia		Association	Human Urine	HMDB	6774165
2-ketoadipic acidemia		Association	Human Urine	HMDB	10503694
Eosinophilic esophagitis	Slight Decrease	Association	Human Urine	HMDB	Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Eosinophilic esophagitis		Association	Human Urine	HMDB	Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
2-ketoadipic acidemia		Association	Human Blood	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)

Microbial Sources

Superkingdom/Kingdom	Phylum	Total species	Hosts and Body Sites	Microbial Links
Bacteria	Bacteroidetes	2	Human feces; Human stool; Human urine; Human saliva	Indirect association between microbiota composition and metabolites measured in host biospecimen
Bacteria	Firmicutes	2	Human ; Human feces	Indirect association between microbiota composition and metabolites measured in host biospecimen; Metabolic reconstruction
Archaea	Thaumarchaeota	1		Metabolic reconstruction
Bacteria	Synergistetes	1	Human feces	Metabolic reconstruction
Archaea	Euryarchaeota	1		Metabolic reconstruction
Bacteria	Proteobacteria	7	Human feces; Human	Metabolic reconstruction
Eukaryota/Fungi	Ascomycota	9	Human feces; Human saliva; Human supragingival plaque	Unknown
Bacteria	Deinococcus-thermus	1
Bacteria	Actinobacteria	2

Exposure Sources

Source Type	Source Sub-type	Species	Data Source	Reference

Host Biospecimen and Location

Host and Biospecimen	Status	Mean	Std	Min	Max	Units	Age	Sex	Health Condition	Data Source	Reference
Human Feces	Detected but not Quantified									HMDB
Human Urine	Detected and Quantified	148				umol/mmol creatinine	Adolescent (13-18 years old)	Male	alpha-Ketoadipic Aciduria	HMDB	6774165
Human Urine	Detected and Quantified	176				umol/mmol creatinine	Newborn (0-30 days old)	Not Specified	alpha-ketoadipic aciduria	HMDB	10503694
Human Urine	Detected and Quantified	1243				umol/mmol creatinine	Infant (0-1 year old)	Not Specified	alpha-ketoadipic aciduria	HMDB	10503694
Human Urine	Detected and Quantified			240	1910	umol/mmol creatinine	Children (1-13 years old)	Not Specified	alpha-Aminoadipic aciduria	HMDB	6796766
Human Blood	Detected and Quantified	6.0		7.0	8.0	uM	Adult (>18 years old)	Both	alpha-Aminoadipic aciduria	HMDB	8764822
Human Blood	Detected and Quantified	18.7				uM	Children (1-13 years old)	Male	Alpha-aminoadipic and alpha-ketoadipic aciduria	HMDB	8487492
Human Urine	Detected and Quantified	42.612				umol/mmol creatinine	Children (1-13 years old)	Male	Alpha-aminoadipic and alpha-ketoadipic aciduria	HMDB	8487492
Human Blood	Detected and Quantified	0				uM	Children (1 - 13 years old)	Female	Normal	HMDB	117247
Human Urine	Detected and Quantified	4.6				umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	19309105
Human Urine	Detected and Quantified	0.633	1.0288			umol/mmol creatinine	Not Specified	Not Specified	Normal	HMDB	8487492
Human Blood	Detected and Quantified	1.14		0.81	9.66	uM	Children (1-13 uears old)	Both	Normal	HMDB	27294788
Human Urine	Detected and Quantified			1.23	15.5	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	32340350
Human Urine	Detected and Quantified			1.7	4.2	umol/mmol creatinine	Adult (>18 years old)	Female	Normal	HMDB	27012787
Human Urine	Detected and Quantified			1.9	4.3	umol/mmol creatinine	Adult (>18 years old)	Male	Normal	HMDB	27012787
Human Urine	Detected and Quantified	15.83	12.44			umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	19468821
Human Urine	Detected and Quantified	19.22	15.83			umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	19468821
Human Blood	Detected and Quantified	2.0		0.0	5.0	uM	Adult (>18 years old)	Both	Normal	HMDB	8764822
Human Urine	Detected and Quantified	24.87	15.83			umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	19468821
Human Urine	Detected and Quantified	6.09	3.36			umol/mmol creatinine	Newborn (0-30 days old)	Male	Normal	HMDB	32340350
Human Urine	Detected and Quantified	7.2		2.5	16.4	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Urine	Detected and Quantified	7.3		3.4	11.2	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Urine	Detected and Quantified	8.52	4.45			umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	HMDB	32340350
Human Urine	Detected and Quantified	14.3982	9.7148			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified	45.554	68.592			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Blood	Detected and Quantified	55.0		30.0	80.0	uM	Children (1-13 years old)	Both	2-Ketoadipic Acidemia	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Blood	Detected and Quantified	48				uM	Children (1-13 years old)	Both	alpha-Aminoadipic aciduria	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Urine	Detected and Quantified	13000				umol/mmol creatinine	Children (1-13 years old)	Both	alpha-Aminoadipic aciduria	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Urine	Detected and Quantified	20.4023	11.4518			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified	40.736	34.234			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Saliva	Detected and Quantified	0.336	0.475			uM	Adult (>18 years old)	Male	Normal	HMDB	Sugimoto et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles.
Human Saliva	Detected and Quantified	0.804	1.44			uM	Adult (>18 years old)	Female	Normal	HMDB	Sugimoto et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles.
Human Saliva	Detected and Quantified	0.821	0.408			uM	Adult (>18 years old)	Female	Normal	HMDB	Sugimoto et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles.
Human Saliva	Detected and Quantified	0.952	1.49			uM	Adult (>18 years old)	Female	Normal	HMDB	Sugimoto et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles.
Human Blood	Detected and Quantified			0.30	3.40	uM	Adult (>18 years old)	Both	Normal	HMDB	Molecular You (https://molecularyou.com)
Human Blood	Detected and Quantified			0.30	3.40	uM	Adult (>18 years old)	Both	Normal	HMDB	Molecular You (https://molecularyou.com)
Human Urine	Detected and Quantified	<5.32				umol/mmol creatinine	Adult (>18 years old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)
Human Urine	Detected and Quantified	<8.60				umol/mmol creatinine	Adolescent (13-18 years old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)
Human Urine	Detected and Quantified	<9.50				umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)
Human Urine	Detected and Quantified			1.13	15.27	umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)
Human Urine	Detected and Quantified			1.13	31.1	umol/mmol creatinine	Infant (0-1 year old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)
Human Urine	Detected and Quantified			1.70	36.65	umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)
Human Placenta	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Prostate	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Urine	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	21389975
Human Urine	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	22024767
Human Feces	Detected but not Quantified						Children (6 - 18 years old)	Not Specified	Normal	HMDB	27609529
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	27543620
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	25037050
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	29808030
Human Urine	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	28157703
Human Blood	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	32966057

External Links

HMDB ID

HMDB0000510

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

References

Synthesis Reference

Not Available

General References

Mechanic GL: The qualitative and quantitative crosslink chemistry of collagen matrices. Adv Exp Med Biol. 1977;86B:699-708. doi: 10.1007/978-1-4757-9113-6_38. [PubMed:20755 ]
Hori S: [Pathophysiology of intraocular neovascularization]. Nippon Ganka Gakkai Zasshi. 1990 Dec;94(12):1103-21. [PubMed:1707214 ]
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. doi: 10.1159/000468669. [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. doi: 10.1007/BF00711325. [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. doi: 10.1016/0014-2999(95)00224-9. [PubMed:8566117 ]
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. doi: 10.1021/jm9602569. [PubMed:8759641 ]
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. doi: 10.1016/s0009-2797(97)00159-2. [PubMed:9679552 ]
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. doi: 10.1074/jbc.M009607200. Epub 2000 Nov 16. [PubMed:11083877 ]
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. doi: 10.1139/y04-067. [PubMed:15389298 ]
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. doi: 10.1042/BJ20061645. [PubMed:17313367 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]

Showing metabocard for Aminoadipic acid (MMDBc0000520)

MOL for #<Metabolite:0x00007fd0f806b708>

3D MOL for #<Metabolite:0x00007fd0f806b708>

3D SDF for #<Metabolite:0x00007fd0f806b708>

3D-SDF for #<Metabolite:0x00007fd0f806b708>

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Structure for #<Metabolite:0x00007fd0f806b708>

3D Structure for #<Metabolite:0x00007fd0f806b708>