Record Information |
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Version | 2.0 |
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Creation Date | 2014-10-02 18:56:26 UTC |
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Update Date | 2018-03-21 17:46:18 UTC |
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Accession Number | T3D4960 |
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Identification |
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Common Name | Galactonic acid |
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Class | Small Molecule |
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Description | Galactonic acid is a sugar acid that is a metabolic breakdown product of galactose. Galactose dehydrogenase is responsible for converting galactose to galactonolactone, which then spontaneously or enzymatically converts to galactonic acid. Once formed, galactonic acid may enter the pentose phosphate pathway. Galactonic acid is increased in red blood cells of galactosemic patients, due to a galactose-1-phosphate uridyltransferase (GALT) deficiency (PMID: 14680973, OMMBID: The Online Metabolic and Molecular Bases of Inherited Disease, Ch.72). When present in sufficiently high levels, galactonic 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 galactonic acid are associated with at least two inborn errors of metabolism, including galactosemia and galactosemia type II. Galactonic 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, liver abnormalities (jaundice), kidney abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of untreated galactosemia. Many affected children with organic acidemias experience intellectual disability or delayed development. High levels of galactonic acid in infants are specifically associated with hepatomegaly (an enlarged liver), cirrhosis, renal failure, cataracts, vomiting, seizure, hypoglycemia, lethargy, brain damage, and ovarian failure. |
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Compound Type | - Animal Toxin
- Metabolite
- Natural Compound
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Chemical Structure | |
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Synonyms | Synonym | D-galactonate | D-galactonic acid | Galactonate |
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Chemical Formula | C6H12O7 |
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Average Molecular Mass | 196.155 g/mol |
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Monoisotopic Mass | 196.058 g/mol |
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CAS Registry Number | 13382-27-9 |
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IUPAC Name | (2S,3R,4R,5S)-2,3,4,5,6-pentahydroxyhexanoic acid |
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Traditional Name | L-galactonic acid |
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SMILES | OC[C@H](O)[C@@H](O)[C@@H](O)[C@H](O)C(O)=O |
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InChI Identifier | InChI=1S/C6H12O7/c7-1-2(8)3(9)4(10)5(11)6(12)13/h2-5,7-11H,1H2,(H,12,13)/t2-,3+,4+,5-/m0/s1 |
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InChI Key | InChIKey=RGHNJXZEOKUKBD-RSJOWCBRSA-N |
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Chemical Taxonomy |
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Description | belongs to the class of organic compounds known as medium-chain hydroxy acids and derivatives. These are hydroxy 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 | Hydroxy acids and derivatives |
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Sub Class | Medium-chain hydroxy acids and derivatives |
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Direct Parent | Medium-chain hydroxy acids and derivatives |
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Alternative Parents | |
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Substituents | - Medium-chain hydroxy acid
- Medium-chain fatty acid
- Beta-hydroxy acid
- Hydroxy fatty acid
- Alpha-hydroxy acid
- Monosaccharide
- Fatty acyl
- Fatty acid
- Secondary alcohol
- Polyol
- Monocarboxylic acid or derivatives
- Carboxylic acid
- Carboxylic acid derivative
- Organooxygen compound
- Hydrocarbon derivative
- Organic oxide
- Carbonyl group
- Primary alcohol
- Alcohol
- Organic oxygen compound
- Aliphatic acyclic compound
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Molecular Framework | Aliphatic acyclic compounds |
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External Descriptors | |
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Biological Properties |
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Status | Detected and Not Quantified |
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Origin | Endogenous |
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Cellular Locations | |
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Biofluid Locations | Not Available |
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Tissue Locations | |
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Pathways | Not Available |
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Applications | Not Available |
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Biological Roles | Not Available |
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Chemical Roles | Not Available |
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Physical Properties |
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State | Solid |
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Appearance | White powder. |
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Experimental Properties | Property | Value |
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Melting Point | 133 - 136 °C | Boiling Point | Not Available | Solubility | Not Available | LogP | Not Available |
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Predicted Properties | |
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Spectra |
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Spectra | Spectrum Type | Description | Splash Key | Deposition Date | View |
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Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 10V, Positive | splash10-004j-2900000000-42150530e3232b66059b | 2016-06-03 | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 20V, Positive | splash10-03di-9500000000-763d7b03787732284c19 | 2016-06-03 | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 40V, Positive | splash10-0bvi-9100000000-040569af699927ca6859 | 2016-06-03 | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 10V, Negative | splash10-05ic-9800000000-3571d4f2ff79bc0d7fa0 | 2016-08-03 | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 20V, Negative | splash10-052r-9400000000-3cb207fadbe1fcae5cb7 | 2016-08-03 | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 40V, Negative | splash10-0a4i-9100000000-8c900935a94065d0938b | 2016-08-03 | View Spectrum |
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Toxicity Profile |
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Route of Exposure | Not Available |
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Mechanism of Toxicity | Not Available |
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Metabolism | Not Available |
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Toxicity Values | Not Available |
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Lethal Dose | Not Available |
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Carcinogenicity (IARC Classification) | No indication of carcinogenicity to humans (not listed by IARC). |
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Uses/Sources | Not Available |
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Minimum Risk Level | Not Available |
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Health Effects | Chronically high levels of galactonate are associated with at least 2 inborn errors of metabolism including: Galactosemia and Galactosemia type II. |
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Symptoms | Not Available |
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Treatment | Not Available |
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Normal Concentrations |
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| Not Available |
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Abnormal Concentrations |
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| Not Available |
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External Links |
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DrugBank ID | Not Available |
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HMDB ID | HMDB00565 |
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PubChem Compound ID | 128869 |
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ChEMBL ID | Not Available |
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ChemSpider ID | 114198 |
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KEGG ID | C00880 |
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UniProt ID | Not Available |
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OMIM ID | |
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ChEBI ID | 16534 |
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BioCyc ID | D-GALACTONO-1,4-LACTONE |
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CTD ID | Not Available |
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Stitch ID | Not Available |
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PDB ID | Not Available |
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ACToR ID | Not Available |
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Wikipedia Link | Not Available |
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References |
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Synthesis Reference | Not Available |
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MSDS | Not Available |
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General References | - Shoemaker JD, Elliott WH: Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease. J Chromatogr. 1991 Jan 2;562(1-2):125-38. [2026685 ]
- Shinka T, Inoue Y, Peng H, Zhen-Wei X, Ose M, Kuhara T: Urine screening of five-day-old newborns: metabolic profiling of neonatal galactosuria. J Chromatogr B Biomed Sci Appl. 1999 Sep 24;732(2):469-77. [10517369 ]
- Yager CT, Chen J, Reynolds R, Segal S: Galactitol and galactonate in red blood cells of galactosemic patients. Mol Genet Metab. 2003 Nov;80(3):283-9. [14680973 ]
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Gene Regulation |
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Up-Regulated Genes | Not Available |
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Down-Regulated Genes | Not Available |
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