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Record Information |
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Version | 4.0 |
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Status | Detected and Quantified |
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Creation Date | 2005-11-16 15:48:42 UTC |
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Update Date | 2020-11-09 23:12:54 UTC |
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HMDB ID | HMDB0000225 |
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Secondary Accession Numbers | |
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Metabolite Identification |
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Common Name | Oxoadipic acid |
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Description | 2-Oxoadipic acid is produced from lysine in the cytosol of cells via the saccharopine and the pipecolic acid pathways. Catabolites of hydroxylysine and tryptophan enter these pathways as 2-aminoadipic- -semialdehyde and 2-oxoadipate, respectively. In the matrix of mitochondria, 2-oxoadipate is decarboxylated to glutaryl-CoA by the 2-oxoadipate dehydrogenase complex and then converted to acetyl-CoA. 2-Oxoadipic aciduria is an in-born error of metabolism of lysine, tryptophan, and hydroxylysine, in which abnormal quantities of 2-aminoadipic acid are found in body fluids along with 2-oxoadipic acid. Patients with 2-Oxoadipic acidemias are mentally retarded with hypotonia or seizures. 2-Oxoadipic aciduria can occur in patients with Kearns-Sayre Syndrome, a progressive disorder with onset prior to 20 years of age in which multiple organ systems are affected, including progressive external ophthalmoplegia, retinopathy, and the age of onset, and these are associated classically with abnormalities in cardiac conduction, cerebellar signs, and elevated cerebrospinal fluid protein (PMID: 10655159 , 16183823 , 11083877 ). Oxoadipic acid is found to be associated with alpha-aminoadipic aciduria, which is an inborn error of metabolism. |
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Structure | |
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Synonyms | Value | Source |
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2-Oxoadipate | ChEBI | 2-Oxoadipic acid | Kegg | Oxoadipate | Generator | 2-Keto-adipate | HMDB | 2-Ketoadipate | HMDB | 2-Ketoadipic acid | HMDB | 2-oxo-Hexanedioate | HMDB | 2-oxo-Hexanedioic acid | HMDB | 2-Oxohexanedioate | HMDB | 2-Oxohexanedioic acid | HMDB | 2-Oxohexanedionic acid | HMDB | a-Ketoadipate | HMDB | a-Ketoadipic acid | HMDB | a-Oxoadipate | HMDB | a-Oxoadipic acid | HMDB | alpha-Ketoadipate | HMDB | alpha-Ketoadipic acid | HMDB | alpha-Oxoadipate | HMDB | alpha-Oxoadipic acid | HMDB | α-Ketoadipic acid | HMDB | α-Oxoadipic acid | HMDB |
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Chemical Formula | C6H8O5 |
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Average Molecular Weight | 160.1247 |
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Monoisotopic Molecular Weight | 160.037173366 |
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IUPAC Name | 2-oxohexanedioic acid |
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Traditional Name | oxoadipate |
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CAS Registry Number | 3184-35-8 |
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SMILES | OC(=O)CCCC(=O)C(O)=O |
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InChI Identifier | InChI=1S/C6H8O5/c7-4(6(10)11)2-1-3-5(8)9/h1-3H2,(H,8,9)(H,10,11) |
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InChI Key | FGSBNBBHOZHUBO-UHFFFAOYSA-N |
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Chemical Taxonomy |
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Description | belongs to the class of organic compounds known as medium-chain keto acids and derivatives. These are keto acids with a 6 to 12 carbon atoms long side chain. |
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Kingdom | Organic compounds |
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Super Class | Organic acids and derivatives |
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Class | Keto acids and derivatives |
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Sub Class | Medium-chain keto acids and derivatives |
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Direct Parent | Medium-chain keto acids and derivatives |
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Alternative Parents | |
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Substituents | - Medium-chain keto acid
- Dicarboxylic acid or derivatives
- Alpha-keto acid
- Alpha-hydroxy ketone
- Ketone
- Carboxylic acid
- Carboxylic acid derivative
- Organic oxygen compound
- Organic oxide
- Hydrocarbon derivative
- Organooxygen compound
- Carbonyl group
- Aliphatic acyclic compound
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Molecular Framework | Aliphatic acyclic compounds |
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External Descriptors | |
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Ontology |
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Physiological effect | Health effect: |
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Disposition | Route of exposure: Source: Biological location: |
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Process | Naturally occurring process: |
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Role | Indirect biological role: |
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Physical Properties |
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State | Solid |
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Experimental Properties | Property | Value | Reference |
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Melting Point | 127 °C | Not Available | Boiling Point | Not Available | Not Available | Water Solubility | Not Available | Not Available | LogP | Not Available | Not Available |
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Predicted Properties | |
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Spectra |
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| Spectrum Type | Description | Splash Key | View |
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GC-MS | GC-MS Spectrum - GC-EI-TOF (Non-derivatized) | splash10-002b-3910000000-66f39a8b247786a425e6 | Spectrum | GC-MS | GC-MS Spectrum - GC-EI-TOF (Non-derivatized) | splash10-002k-3910000000-977efef811bb47535b80 | Spectrum | GC-MS | GC-MS Spectrum - GC-EI-TOF (Non-derivatized) | splash10-0udr-6900000000-0eb27647b831277a2aef | Spectrum | GC-MS | GC-MS Spectrum - GC-EI-TOF (Non-derivatized) | splash10-0udr-6900000000-46cd4d782e3d19030993 | Spectrum | GC-MS | GC-MS Spectrum - GC-EI-TOF (Non-derivatized) | splash10-002b-3910000000-66f39a8b247786a425e6 | Spectrum | GC-MS | GC-MS Spectrum - GC-EI-TOF (Non-derivatized) | splash10-002k-3910000000-977efef811bb47535b80 | Spectrum | GC-MS | GC-MS Spectrum - GC-EI-TOF (Non-derivatized) | splash10-0udr-6900000000-0eb27647b831277a2aef | Spectrum | GC-MS | GC-MS Spectrum - GC-EI-TOF (Non-derivatized) | splash10-0udr-6900000000-46cd4d782e3d19030993 | Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive | splash10-00r6-9300000000-b3f156c173adabf3f1bb | Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positive | splash10-0079-9830000000-a3b1d5e6d378582a6fc2 | Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ , negative | splash10-0a4i-0900000000-aef94cc19e461712a097 | Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ , negative | splash10-0bt9-9500000000-9326906313591bb9da1c | Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ , negative | splash10-0a4i-9000000000-05db676896bb1e350497 | Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ , negative | splash10-0a4l-9000000000-1e566f572b14692461f2 | Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ , negative | splash10-0006-9000000000-527da1c1c271db4d8db4 | Spectrum | LC-MS/MS | LC-MS/MS Spectrum - , negative | splash10-0a4i-9600000000-38ade0e080ecb7a89588 | Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 10V, Positive | splash10-0006-2900000000-757eb57780db9df4f78d | Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 20V, Positive | splash10-016v-9700000000-675e5732632f0a0cabe1 | Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 40V, Positive | splash10-0006-9000000000-1da8154067fdb9a27459 | Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 10V, Negative | splash10-0a4i-1900000000-0e26a52af95eb287809d | Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 20V, Negative | splash10-066v-7900000000-d898e0593464c13b3f89 | Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 40V, Negative | splash10-052g-9100000000-34782bad1c8755ac897d | Spectrum | 1D NMR | 1H NMR Spectrum | Not Available | Spectrum | 1D NMR | 1H NMR Spectrum | Not Available | Spectrum | 1D NMR | 13C NMR Spectrum | Not Available | Spectrum | 2D NMR | [1H,13C] 2D NMR Spectrum | Not Available | Spectrum |
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Biological Properties |
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Cellular Locations | |
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Biospecimen Locations | |
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Tissue Locations | Not Available |
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Pathways | |
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Normal Concentrations |
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Blood | Expected but not Quantified | Not Quantified | Not Available | Not Available | Normal | | details | Blood | Detected but not Quantified | Not Quantified | Adult (>18 years old) | Both | Normal | | details | Urine | Detected but not Quantified | Not Quantified | Adult (>18 years old) | Both | Normal | | details | Urine | Detected and Quantified | 1.2 umol/mmol creatinine | Children (1-13 years old) | Both | Normal | | details | Urine | Detected and Quantified | 0.17 (0.02-0.57) umol/mmol creatinine | Adult (>18 years old) | Male | Normal | | details | Urine | Detected and Quantified | 0.21 (0.04-0.81) umol/mmol creatinine | Adult (>18 years old) | Female | Normal | | details | Urine | Detected and Quantified | 1.3 (1.0-1.5) umol/mmol creatinine | Children (1-13 years old) | Both | Normal | | details |
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Abnormal Concentrations |
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Urine | Detected and Quantified | 520-970 umol/mmol creatinine | Children (1-13 years old) | Female | 2-Aminoadipic aciduria | | details | Urine | Detected and Quantified | 62 umol/mmol creatinine | Adolescent (13-18 years old) | Male | alpha-Ketoadipic Aciduria | | details | Urine | Detected and Quantified | 20-120 umol/mmol creatinine | Children (1-13 years old) | Not Specified | alpha-Aminoadipic aciduria | | details | Urine | Detected and Quantified | 456 umol/mmol creatinine | Infant (0-1 year old) | Not Specified | alpha-ketoadipic aciduria | | details | Urine | Detected and Quantified | 135 umol/mmol creatinine | Newborn (0-30 days old) | Not Specified | alpha-ketoadipic aciduria | | details |
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Associated Disorders and Diseases |
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Disease References | 2-Ketoadipic acidemia |
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- 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.
| Alpha-aminoadipic aciduria |
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- 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 ]
| 2-Aminoadipic aciduria |
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- 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 ]
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Associated OMIM IDs | - 245130 (2-Ketoadipic acidemia)
- 204750 (2-Aminoadipic aciduria)
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External Links |
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DrugBank ID | Not Available |
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Phenol Explorer Compound ID | Not Available |
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FooDB ID | FDB003362 |
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KNApSAcK ID | C00000770 |
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Chemspider ID | 70 |
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KEGG Compound ID | C00322 |
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BioCyc ID | 2K-ADIPATE |
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BiGG ID | 1485244 |
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Wikipedia Link | Not Available |
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METLIN ID | 5234 |
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PubChem Compound | 71 |
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PDB ID | Not Available |
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ChEBI ID | 15753 |
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Food Biomarker Ontology | Not Available |
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VMH ID | 2OXOADP |
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MarkerDB ID | MDB00000107 |
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References |
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Synthesis Reference | Nelson, Randall B.; Gribble, Gordon W. Preparation of a-ketoadipic acid. Organic Preparations and Procedures International (1973), 5(2), 55-8. |
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Material Safety Data Sheet (MSDS) | Download (PDF) |
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General References | - 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. [PubMed:8087979 ]
- Lee SH, Kim SO, Chung BC: Gas chromatographic-mass spectrometric determination of urinary oxoacids using O-(2,3,4,5,6-pentafluorobenzyl)oxime-trimethylsilyl ester derivatization and cation-exchange chromatography. J Chromatogr B Biomed Sci Appl. 1998 Nov 20;719(1-2):1-7. [PubMed:9869358 ]
- Barshop BA, Nyhan WL, Naviaux RK, McGowan KA, Friedlander M, Haas RH: Kearns-Sayre syndrome presenting as 2-oxoadipic aciduria. Mol Genet Metab. 2000 Jan;69(1):64-8. [PubMed:10655159 ]
- Schulze-Bergkamen A, Okun JG, Spiekerkotter U, Lindner M, Haas D, Kohlmuller D, Mayatepek E, Schulze-Bergkamen H, Greenberg CR, Zschocke J, Hoffmann GF, Kolker S: Quantitative acylcarnitine profiling in peripheral blood mononuclear cells using in vitro loading with palmitic and 2-oxoadipic acids: biochemical confirmation of fatty acid oxidation and organic acid disorders. Pediatr Res. 2005 Nov;58(5):873-80. Epub 2005 Sep 23. [PubMed:16183823 ]
- 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 ]
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