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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 | 2006-05-22 14:17:39 UTC |
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Update Date | 2019-01-11 19:16:44 UTC |
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HMDB ID | HMDB0002169 |
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Secondary Accession Numbers | |
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Metabolite Identification |
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Common Name | S-(2-carboxypropyl)-Cysteamine |
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Description | S-(2-carboxypropyl)-Cysteamine enhances the procoagulant activity of factor VIII-East Hartford, a dysfunctional protein due to a light chain thrombin cleavage site mutation (PMID 1569180 ). S-(2-carboxypropyl)-Cysteamine is known to modify mutant proteins with an arg-to-cys substitution (PMID 1569181 ). |
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Structure | |
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Synonyms | Value | Source |
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3-((2-Aminoethyl)thio)-2-methyl-propanoate | HMDB | 3-((2-Aminoethyl)thio)-2-methyl-propanoic acid | HMDB | S-(2-Carboxypropyl)cysteamine | HMDB | S-2-CPCT | MeSH |
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Chemical Formula | C6H13NO2S |
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Average Molecular Weight | 163.238 |
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Monoisotopic Molecular Weight | 163.066699355 |
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IUPAC Name | 3-[(2-aminoethyl)sulfanyl]-2-methylpropanoic acid |
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Traditional Name | S-(2-carboxypropyl)cysteamine |
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CAS Registry Number | 80186-81-8 |
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SMILES | CC(CSCCN)C(O)=O |
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InChI Identifier | InChI=1S/C6H13NO2S/c1-5(6(8)9)4-10-3-2-7/h5H,2-4,7H2,1H3,(H,8,9) |
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InChI Key | UFRVABODKAYFCB-UHFFFAOYSA-N |
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Chemical Taxonomy |
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Description | This compound belongs to the class of organic compounds known as amino acids. These are organic compounds that contain at least one carboxyl group and one amino group. |
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Kingdom | Organic compounds |
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Super Class | Organic acids and derivatives |
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Class | Carboxylic acids and derivatives |
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Sub Class | Amino acids, peptides, and analogues |
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Direct Parent | Amino acids |
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Alternative Parents | |
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Substituents | - Amino acid
- Carboxylic acid
- Monocarboxylic acid or derivatives
- Thioether
- Sulfenyl compound
- Dialkylthioether
- Amine
- Hydrocarbon derivative
- Organic oxide
- Organopnictogen compound
- Primary amine
- Organosulfur compound
- Organooxygen compound
- Organonitrogen compound
- Organic oxygen compound
- Primary aliphatic amine
- Organic nitrogen compound
- Carbonyl group
- 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 | 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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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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Urine | Detected and Quantified | 14-30 umol/mmol creatinine | Infant (0-1 year old) | Both | Short-chain enoyl-CoA hydratase deficiency | | details | Urine | Detected and Quantified | 1.2-2.7 umol/mmol creatinine | Not Specified | Not Specified | Propionic acidemia | | details | Urine | Detected and Quantified | <10 umol/mmol creatinine | Not Specified | Not Specified | Normal | | details |
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Abnormal Concentrations |
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Urine | Detected and Quantified | 13-16 umol/mmol creatinine | Children (1-13 years old) | Female | 3-Hydroxyisobutyryl-coa hydrolase deficiency | | details |
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Associated Disorders and Diseases |
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Disease References | 3-Hydroxyisobutyryl-coa hydrolase deficiency |
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- Peters H, Ferdinandusse S, Ruiter JP, Wanders RJ, Boneh A, Pitt J: Metabolite studies in HIBCH and ECHS1 defects: Implications for screening. Mol Genet Metab. 2015 Aug;115(4):168-73. doi: 10.1016/j.ymgme.2015.06.008. Epub 2015 Jun 24. [PubMed:26163321 ]
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Associated OMIM IDs | - 250620 (3-Hydroxyisobutyryl-coa hydrolase deficiency)
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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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FoodDB ID | FDB022881 |
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KNApSAcK ID | Not Available |
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Chemspider ID | 117681 |
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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 | 6522 |
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PubChem Compound | 133402 |
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PDB ID | Not Available |
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ChEBI ID | Not Available |
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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 | - Brown GK, Hunt SM, Scholem R, Fowler K, Grimes A, Mercer JF, Truscott RM, Cotton RG, Rogers JG, Danks DM: beta-hydroxyisobutyryl coenzyme A deacylase deficiency: a defect in valine metabolism associated with physical malformations. Pediatrics. 1982 Oct;70(4):532-8. [PubMed:7122152 ]
- Truscott RJ, Malegan D, McCairns E, Halpern B, Hammond J, Cotton RG, Mercer JF, Hunt S, Rogers JG, Danks DM: Two new sulphur-containing amino acids in man. Biomed Mass Spectrom. 1981 Mar;8(3):99-104. [PubMed:7236859 ]
- Aly AM, Arai M, Hoyer LW: Cysteamine enhances the procoagulant activity of Factor VIII-East Hartford, a dysfunctional protein due to a light chain thrombin cleavage site mutation (arginine-1689 to cysteine). J Clin Invest. 1992 May;89(5):1375-81. [PubMed:1569180 ]
- Aly AM, Hoyer LW: Factor VIII-East Hartford (arginine 1689 to cysteine) has procoagulant activity when separated from von Willebrand factor. J Clin Invest. 1992 May;89(5):1382-7. [PubMed:1569181 ]
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