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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 | 2009-11-02 23:11:25 UTC |
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Update Date | 2020-11-09 23:18:40 UTC |
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HMDB ID | HMDB0013127 |
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Secondary Accession Numbers | |
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Metabolite Identification |
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Common Name | 3-Hydroxybutyrylcarnitine |
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Description | 3-Hydroxybutyrylcarnitine, also known as hydroxybutyrylcarnitine, is classified as a member of the acyl carnitines. Acyl carnitines are organic compounds containing a fatty acid with the carboxylic acid attached to carnitine through an ester bond. 3-Hydroxybutyrylcarnitine is considered to be a slightly soluble (in water) and a weak acidic compound. 3-Hydroxybutyrylcarnitine is a fatty ester lipid molecule. 3-Hydroxybutyrylcarnitine can be found in blood. Within a cell, 3-hydroxybutyrylcarnitine is primarily located in the extracellular space and near the membrane. |
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Structure | |
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Synonyms | Value | Source |
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(3S)-3-{[(3R)-3-hydroxybutanoyl]oxy}-4-(trimethylazaniumyl)butanoic acid | Generator | (3S)-Hydroxybutyrylcarnitine | HMDB | (S)-3-Hydroxybutyrylcarnitine | HMDB | 3-Hydroxybutyrylcarnitine | HMDB | Hydroxybutyrylcarnitine | HMDB |
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Chemical Formula | C11H21NO5 |
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Average Molecular Weight | 247.291 |
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Monoisotopic Molecular Weight | 247.14197278 |
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IUPAC Name | (3S)-3-{[(3R)-3-hydroxybutanoyl]oxy}-4-(trimethylazaniumyl)butanoate |
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Traditional Name | (3S)-3-{[(3R)-3-hydroxybutanoyl]oxy}-4-(trimethylammonio)butanoate |
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CAS Registry Number | 1469900-92-2 |
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SMILES | C[C@@H](O)CC(=O)O[C@@H](CC([O-])=O)C[N+](C)(C)C |
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InChI Identifier | InChI=1S/C11H21NO5/c1-8(13)5-11(16)17-9(6-10(14)15)7-12(2,3)4/h8-9,13H,5-7H2,1-4H3/t8-,9+/m1/s1 |
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InChI Key | UEFRDQSMQXDWTO-BDAKNGLRSA-N |
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Chemical Taxonomy |
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Description | belongs to the class of organic compounds known as acyl carnitines. These are organic compounds containing a fatty acid with the carboxylic acid attached to carnitine through an ester bond. |
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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 acid esters |
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Direct Parent | Acyl carnitines |
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Alternative Parents | |
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Substituents | - Acyl-carnitine
- Beta-hydroxy acid
- Hydroxy acid
- Dicarboxylic acid or derivatives
- Tetraalkylammonium salt
- Quaternary ammonium salt
- Secondary alcohol
- Carboxylic acid salt
- Carboxylic acid ester
- Carboxylic acid
- Carboxylic acid derivative
- Organic nitrogen compound
- Organic oxygen compound
- Organopnictogen compound
- Organic oxide
- Hydrocarbon derivative
- Organic salt
- Organic zwitterion
- Organooxygen compound
- Organonitrogen compound
- Carbonyl group
- Amine
- Alcohol
- Aliphatic acyclic compound
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Molecular Framework | Aliphatic acyclic compounds |
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External Descriptors | Not Available |
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Ontology |
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Disposition | Route of exposure: Source: Biological location: |
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Process | Naturally occurring process: |
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Role | Industrial application: 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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| Not Available |
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Biological Properties |
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Cellular Locations | |
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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 | Detected but not Quantified | Not Quantified | Adult (>18 years old) | Both | Normal | | details | Blood | Detected and Quantified | 0.11 +/- 0.01 uM | Adult (>18 years old) | Both | Normal | | details | Blood | Detected and Quantified | 0.0960 (0.000-0.360) uM | Infant (0-1 year old) | Not Specified | Normal | | details | Blood | Detected and Quantified | <0.400 uM | Infant (0-1 year old) | Not Specified | Normal | | details | Blood | Detected and Quantified | 0.04 +/- 0.02 uM | Adult (>18 years old) | Both | Normal | | details | Feces | Detected but not Quantified | Not Quantified | Adult (>18 years old) | Both | Normal | | details | Feces | Detected and Quantified | 0.6 +/- 0.36 nmol/g wet feces | Adult (>18 years old) | Both | Normal | | details | Feces | Detected and Quantified | 0.32 +/- 0.2 nmol/g wet feces | Adult (>18 years old) | Both | Normal | | details | Urine | Detected but not Quantified | Not Quantified | Adult (>18 years old) | Both | Normal | | details |
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Abnormal Concentrations |
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Blood | Detected and Quantified | 1.780 (0.920-3.0400) uM | Infant (0-1 year old) | Female | 3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD) | | details | Blood | Detected and Quantified | 0.200 (0.0800-0.500) uM | Infant (0-1 year old) | Not Specified | Ketosis | | details | Blood | Detected and Quantified | 0.180 (0.0600-0.360) uM | Infant (0-1 year old) | Not Specified | Short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency | | details | Blood | Detected and Quantified | 0.940-1.720 uM | Infant (0-1 year old) | Male | 3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD) | | details | Blood | Detected and Quantified | 0.660-0.810 uM | Infant (0-1 year old) | Male | 3-Hydroxyisobutyryl-coa hydrolase deficiency | | details | Feces | Detected but not Quantified | Not Quantified | Adult (>18 years old) | Both | Colorectal cancer | | details |
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Associated Disorders and Diseases |
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Disease References | 3-Hydroxyacyl-CoA dehydrogenase deficiency |
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- Clayton PT, Eaton S, Aynsley-Green A, Edginton M, Hussain K, Krywawych S, Datta V, Malingre HE, Berger R, van den Berg IE: Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion. J Clin Invest. 2001 Aug;108(3):457-65. [PubMed:11489939 ]
- Popa FI, Perlini S, Teofoli F, Degani D, Funghini S, La Marca G, Rinaldo P, Vincenzi M, Antoniazzi F, Boner A, Camilot M: 3-hydroxyacyl-coenzyme a dehydrogenase deficiency: identification of a new mutation causing hyperinsulinemic hypoketotic hypoglycemia, altered organic acids and acylcarnitines concentrations. JIMD Rep. 2012;2:71-7. doi: 10.1007/8904_2011_50. Epub 2011 Sep 6. [PubMed:23430856 ]
| Ketosis |
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- Clayton PT, Eaton S, Aynsley-Green A, Edginton M, Hussain K, Krywawych S, Datta V, Malingre HE, Berger R, van den Berg IE: Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion. J Clin Invest. 2001 Aug;108(3):457-65. [PubMed:11489939 ]
| Short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency |
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- Clayton PT, Eaton S, Aynsley-Green A, Edginton M, Hussain K, Krywawych S, Datta V, Malingre HE, Berger R, van den Berg IE: Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion. J Clin Invest. 2001 Aug;108(3):457-65. [PubMed:11489939 ]
| 3-Hydroxyisobutyryl-coa hydrolase deficiency |
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- Reuter MS, Sass JO, Leis T, Kohler J, Mayr JA, Feichtinger RG, Rauh M, Schanze I, Bahr L, Trollmann R, Uebe S, Ekici AB, Reis A: HIBCH deficiency in a patient with phenotypic characteristics of mitochondrial disorders. Am J Med Genet A. 2014 Dec;164A(12):3162-9. doi: 10.1002/ajmg.a.36766. Epub 2014 Sep 23. [PubMed:25251209 ]
| Colorectal cancer |
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- 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 ]
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Associated OMIM IDs | - 231530 (3-Hydroxyacyl-CoA dehydrogenase deficiency)
- 250620 (3-Hydroxyisobutyryl-coa hydrolase deficiency)
- 114500 (Colorectal cancer)
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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 | Not Available |
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KNApSAcK ID | Not Available |
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Chemspider ID | Not Available |
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KEGG Compound ID | Not Available |
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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 | Not Available |
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PubChem Compound | Not Available |
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PDB ID | Not Available |
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ChEBI ID | Not Available |
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Food Biomarker Ontology | Not Available |
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VMH ID | Not Available |
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MarkerDB ID | MDB00029837 |
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References |
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Synthesis Reference | Not Available |
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Material Safety Data Sheet (MSDS) | Not Available |
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General References | - Simons K, Toomre D: Lipid rafts and signal transduction. Nat Rev Mol Cell Biol. 2000 Oct;1(1):31-9. [PubMed:11413487 ]
- Watson AD: Thematic review series: systems biology approaches to metabolic and cardiovascular disorders. Lipidomics: a global approach to lipid analysis in biological systems. J Lipid Res. 2006 Oct;47(10):2101-11. Epub 2006 Aug 10. [PubMed:16902246 ]
- Sethi JK, Vidal-Puig AJ: Thematic review series: adipocyte biology. Adipose tissue function and plasticity orchestrate nutritional adaptation. J Lipid Res. 2007 Jun;48(6):1253-62. Epub 2007 Mar 20. [PubMed:17374880 ]
- Lingwood D, Simons K: Lipid rafts as a membrane-organizing principle. Science. 2010 Jan 1;327(5961):46-50. doi: 10.1126/science.1174621. [PubMed:20044567 ]
- 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 ]
- Gunstone, Frank D., John L. Harwood, and Albert J. Dijkstra (2007). The lipid handbook with CD-ROM. CRC Press.
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