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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 | 2020-10-09 20:59:05 UTC |
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HMDB ID | HMDB0000321 |
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Secondary Accession Numbers | |
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Metabolite Identification |
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Common Name | 2-Hydroxyadipic acid |
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Description | 2-Hydroxyadipic acid is a hydroxy-dicarboxylic acid formed by the reduction of 2-ketoadipic acid. A deficiency of 2-ketoadipic dehydrogenase causes 2-ketoadipic acidemia or 2-oxoadipic acidemia (OMIM: 245130 ), a genetic disorder characterized by accumulation and excretion of 2-hydroxyadipic acid (with 2-ketoadipic and 2-aminoadipic) (OMMBID - The Online Metabolic and Molecular Bases of Inherited Disease, CH.95). When present in sufficiently high levels, 2-hydroxyadipic acid can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of 2-hydroxyadipic acid are associated with at least three inborn errors of metabolism including 2-oxoadipic acidemia, 2-aminoadipic aciduria, and 2-oxoadipic aciduria. 2-Hydroxyadipic acid is an organic acid. 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. |
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Structure | |
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Synonyms | Value | Source |
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2-Hydroxyadipate | ChEBI | 2-Hydroxyhexanedioic acid | ChEBI | 2-Hydroxyhexanedioate | Generator | 2,3,4-Trideoxyhexarate | HMDB | 2,3,4-Trideoxyhexaric acid | HMDB | 2-Hydroxy-adipate | HMDB | 2-Hydroxy-adipic acid | HMDB | 2-Hydroxy-hexanedioate | HMDB | 2-Hydroxy-hexanedioic acid | HMDB | a-Hydroxyadipate | HMDB | a-Hydroxyadipic acid | HMDB | alpha-Hydroxyadipate | HMDB | alpha-Hydroxyadipic acid | HMDB | DL-2-Hydroxyadipate | HMDB | DL-2-Hydroxyadipic acid | HMDB |
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Chemical Formula | C6H10O5 |
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Average Molecular Weight | 162.1406 |
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Monoisotopic Molecular Weight | 162.05282343 |
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IUPAC Name | 2-hydroxyhexanedioic acid |
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Traditional Name | α-hydroxyadipic acid |
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CAS Registry Number | 18294-85-4 |
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SMILES | OC(CCCC(O)=O)C(O)=O |
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InChI Identifier | InChI=1S/C6H10O5/c7-4(6(10)11)2-1-3-5(8)9/h4,7H,1-3H2,(H,8,9)(H,10,11) |
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InChI Key | OTTXIFWBPRRYOG-UHFFFAOYSA-N |
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Chemical Taxonomy |
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Description | belongs to the class of organic compounds known as medium-chain fatty acids. These are fatty acids with an aliphatic tail that contains between 4 and 12 carbon atoms. |
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Kingdom | Organic compounds |
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Super Class | Lipids and lipid-like molecules |
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Class | Fatty Acyls |
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Sub Class | Fatty acids and conjugates |
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Direct Parent | Medium-chain fatty acids |
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Alternative Parents | |
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Substituents | - Medium-chain fatty acid
- Hydroxy fatty acid
- Monosaccharide
- Hydroxy acid
- Dicarboxylic acid or derivatives
- Alpha-hydroxy acid
- Secondary alcohol
- Carboxylic acid
- Carboxylic acid derivative
- Hydrocarbon derivative
- Organic oxygen compound
- Organooxygen compound
- Alcohol
- Carbonyl group
- Organic oxide
- 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: 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 | Not Available | 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-MS (3 TMS) | splash10-00c0-2920000000-14e371ab410997076c08 | Spectrum | GC-MS | GC-MS Spectrum - GC-MS (Non-derivatized) | splash10-00c0-2920000000-14e371ab410997076c08 | Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive | splash10-00xu-9200000000-a5e63603281ceddefa05 | Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - GC-MS (3 TMS) - 70eV, Positive | splash10-01w0-9273000000-da104d83dee59e6410a4 | Spectrum | LC-MS/MS | LC-MS/MS Spectrum - Quattro_QQQ 10V, Negative (Annotated) | splash10-03di-2900000000-00b8bf461ba454c702f9 | Spectrum | LC-MS/MS | LC-MS/MS Spectrum - Quattro_QQQ 25V, Negative (Annotated) | splash10-0ab9-9000000000-cbfcbe2f1e74c7fa8544 | Spectrum | LC-MS/MS | LC-MS/MS Spectrum - Quattro_QQQ 40V, Negative (Annotated) | splash10-052f-9000000000-1a3f1223d56568a37ca5 | Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 10V, Positive | splash10-0002-1900000000-c97aa5c57fc34910b084 | Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 20V, Positive | splash10-00kb-9800000000-8b422e7bd31b7715f936 | Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 40V, Positive | splash10-0596-9000000000-145898d967788e6a4370 | Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 10V, Negative | splash10-03dl-0900000000-b8d680487ea735b08660 | Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 20V, Negative | splash10-014m-5900000000-2748f3664e3329b9721b | Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 40V, Negative | splash10-0aba-9000000000-7bd0454dd4e607bf327d | Spectrum | 1D NMR | 1H 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 | - Cytoplasm (predicted from logP)
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Biospecimen Locations | |
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Tissue Locations | |
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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 | Feces | Detected but not Quantified | Not Quantified | Adult (>18 years old) | Both | Normal | | details | Feces | Detected but not Quantified | Not Quantified | Adult (>18 years old) | Both | Normal | | details | Feces | Detected but not Quantified | Not Quantified | Adult (>18 years old) | Both | Normal | | details | Urine | Detected and Quantified | 7.7 (0.1-19.6) umol/mmol creatinine | Newborn (0-30 days old) | Both | Normal | | details | Urine | Detected and Quantified | 7.3 (0.1-15.4) umol/mmol creatinine | Infant (0-1 year old) | Both | Normal | | details | Urine | Detected and Quantified | 2.2 (0.1-6.8) umol/mmol creatinine | Children (1-13 years old) | Both | Normal | | details | Urine | Detected and Quantified | 1.6 (0.1-5.1) umol/mmol creatinine | Children (1-13 years old) | Both | Normal | | details | Urine | Detected and Quantified | 2.4 (0.1-5.8) umol/mmol creatinine | Adolescent (13-18 years old) | Both | Normal | | details |
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Abnormal Concentrations |
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Feces | Detected but not Quantified | Not Quantified | Adult (>18 years old) | Both | Colorectal Cancer | | details | Feces | Detected but not Quantified | Not Quantified | Adult (>18 years old) | Both | Colorectal cancer | | details | Urine | Detected but not Quantified | Not Quantified | Adult (>18 years old) | Both | Bladder cancer | | details | Urine | Detected and Quantified | 1.0 (0.0-2.0) umol/mmol creatinine | Adult (>18 years old) | Both | 2-ketoadipic acidemia | | details | Urine | Detected and Quantified | 135.0 (50.0-220.0) umol/mmol creatinine | Children (1-13 years old) | Both | 2-Ketoadipic Acidemia | | details | Urine | Detected and Quantified | 80-170 umol/mmol creatinine | Children (1-13 years old) | Not Specified | alpha-Aminoadipic aciduria | | details | Urine | Detected and Quantified | 20 umol/mmol creatinine | Infant (0-1 year old) | Not Specified | alpha-ketoadipic aciduria | | details | Urine | Detected and Quantified | 12.9 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 | Colorectal cancer |
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- Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ]
- 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 ]
| 2-Ketoadipic acidemia |
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- 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 ]
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Associated OMIM IDs | |
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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 | FDB021947 |
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KNApSAcK ID | Not Available |
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Chemspider ID | 167943 |
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KEGG Compound ID | C02360 |
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BioCyc ID | Not Available |
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BiGG ID | Not Available |
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Wikipedia Link | Not Available |
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METLIN ID | 5310 |
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PubChem Compound | 193530 |
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PDB ID | Not Available |
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ChEBI ID | 17023 |
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Food Biomarker Ontology | Not Available |
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VMH ID | Not Available |
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MarkerDB ID | MDB00000145 |
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References |
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Synthesis Reference | Griot, Rudolf. a-Hydroxyadipic acid. Helvetica Chimica Acta (1958), 41 2236-41. |
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Material Safety Data Sheet (MSDS) | Not Available |
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General References | - Sewell AC, Heil M, Podebrad F, Mosandl A: Chiral compounds in metabolism: a look in the molecular mirror. Eur J Pediatr. 1998 Mar;157(3):185-91. [PubMed:9537483 ]
- 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 ]
- Inoue Y, Shinka T, Ohse M, Kuhara T: Differential chemical diagnosis of primary hyperoxaluria type II. Highly sensitive analysis of optical isomers of glyceric acid by GC/MS as diastereoisomeric derivatives. J Chromatogr B Analyt Technol Biomed Life Sci. 2005 Aug 25;823(1):2-6. Epub 2005 Apr 25. [PubMed:16055048 ]
- Svendsen JS, Whist JE, Sydnes LK: Absolute configuration of 3-hydroxyadipic acid in human urine. J Chromatogr. 1985 Jan 11;337(1):9-19. [PubMed:3980660 ]
- 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 ]
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