You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.
Record Information
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2020-02-26 21:22:27 UTC
Secondary Accession Numbers
  • HMDB00656
Metabolite Identification
Common NameCysteineglutathione disulfide
DescriptionCysteineglutathione disulfide is a molecule that is formed upon oxidative stress of glutathione, that will form mixed disulfides with protein thiol groups, causing reversible S-glutathionylation. S-glutathionylation is an important post-translational modification responsible for transducing oxidant signals. S-glutathionylation of thiols confers protection against their irreversible oxidation, like for instance the formation of sulphonic acid moieties. If the targeted cysteine is a functionally critical amino acid, S-glutathionylation will however also modify protein function. (PMID 16515838 ). S-sulfonation and S-thiolation of transthyretin Phe33Cys has been detected in a patient with familial transthyretin amyloidosis. (PMID 12876326 ). In Cystinotic human skin fibroblasts in tissue culture there is an accumulation of cystine. Stored cystine in cystinotic tissues may derive in part from glutathione-cysteine mixed disulfide via transpeptidation. (PMID 6130452 ). Cystinosis is an autosomal recessive disorder caused by an impaired transport of cystine out of lysosomes. (PMID 15042893 ).
Cysteineglutathione disulphideGenerator
Cysteine-glutathione disulfideHMDB
Cysteine-glutathione mixed disulfideHMDB
(2S)-2-Amino-4-{[(1R)-2-[(2-amino-2-carboxyethyl)disulphanyl]-1-[(carboxymethyl)-C-hydroxycarbonimidoyl]ethyl]-C-hydroxycarbonimidoyl}butanoic acidHMDB
Chemical FormulaC13H22N4O8S2
Average Molecular Weight426.466
Monoisotopic Molecular Weight426.08790508
IUPAC Name(2S)-2-amino-4-{[(1R)-2-[(2-amino-2-carboxyethyl)disulfanyl]-1-[(carboxymethyl)carbamoyl]ethyl]carbamoyl}butanoic acid
Traditional Name(2S)-2-amino-4-{[(1R)-2-[(2-amino-2-carboxyethyl)disulfanyl]-1-(carboxymethylcarbamoyl)ethyl]carbamoyl}butanoic acid
CAS Registry Number13081-14-6
InChI Identifier
Chemical Taxonomy
Description belongs to the class of organic compounds known as oligopeptides. These are organic compounds containing a sequence of between three and ten alpha-amino acids joined by peptide bonds.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentOligopeptides
Alternative Parents
  • Alpha-oligopeptide
  • Gamma-glutamyl alpha peptide
  • Glutamine or derivatives
  • N-acyl-alpha-amino acid
  • N-acyl-alpha amino acid or derivatives
  • Alpha-amino acid amide
  • Cysteine or derivatives
  • Alpha-amino acid
  • N-substituted-alpha-amino acid
  • Alpha-amino acid or derivatives
  • L-alpha-amino acid
  • Tricarboxylic acid or derivatives
  • N-acyl-amine
  • Fatty amide
  • Fatty acyl
  • Carboxamide group
  • Amino acid
  • Dialkyldisulfide
  • Organic disulfide
  • Secondary carboxylic acid amide
  • Amino acid or derivatives
  • Carboxylic acid
  • Sulfenyl compound
  • Organic nitrogen compound
  • Primary aliphatic amine
  • Organonitrogen compound
  • Organooxygen compound
  • Organosulfur compound
  • Primary amine
  • Carbonyl group
  • Hydrocarbon derivative
  • Amine
  • Organic oxide
  • Organopnictogen compound
  • Organic oxygen compound
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External DescriptorsNot Available


Biological location:

Physical Properties
Experimental Properties
Melting Point234 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
Water Solubility1.48 g/LALOGPS
pKa (Strongest Acidic)1.39ChemAxon
pKa (Strongest Basic)9.5ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count10ChemAxon
Hydrogen Donor Count7ChemAxon
Polar Surface Area222.14 ŲChemAxon
Rotatable Bond Count14ChemAxon
Refractivity95.76 m³·mol⁻¹ChemAxon
Polarizability40.45 ųChemAxon
Number of Rings0ChemAxon
Rule of FiveNoChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Spectrum TypeDescriptionSplash KeyView
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-0uec-3329000000-54650021a7baf21933b2Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (3 TMS) - 70eV, Positivesplash10-0fmi-8910444000-17e528b5f6f1ff73ae33Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-0002-0390100000-5fa8a02559e5d3e0f3d8Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-01t9-1910000000-7ca978670daa10642074Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-00e9-1972100000-95e2b60e54e43eab9368Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-06ur-1129400000-b0d6b6a600c3dbf18ab7Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-056r-8479000000-6e999052fbd8a78b9941Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-05di-9753000000-210c7ac127ef822cecd7Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0kdr-0547900000-7b6571351f964333b549Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-00di-3934100000-55bcddfdc78aa646ed93Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-006w-9521000000-8db026e657cf9f90c5a0Spectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableSpectrum
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biospecimen Locations
  • Saliva
Tissue Locations
  • Placenta
Normal Concentrations
SalivaDetected and Quantified0.227 +/- 0.209 uMAdult (>18 years old)Female
    • Sugimoto et al. (...
SalivaDetected and Quantified0.234 +/- 0.267 uMAdult (>18 years old)Not Specified
    • Sugimoto et al. (...
SalivaDetected and Quantified0.362 +/- 1.18 uMAdult (>18 years old)Not Specified
    • Sugimoto et al. (...
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB022166
KNApSAcK IDNot Available
Chemspider IDNot Available
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
PubChem Compound53477713
PDB IDNot Available
ChEBI ID143243
Food Biomarker OntologyNot Available
VMH IDNot Available
Synthesis ReferenceEriksson, Bengt; Eriksson, Stellan A. Synthesis and characterization of the L-cysteine-glutathione mixed disulfide. Acta Chemica Scandinavica (1947-1973) (1967), 21(5), 1304-12.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. 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 ]
  2. Reynaert NL, Ckless K, Guala AS, Wouters EF, van der Vliet A, Janssen-Heininger YM: In situ detection of S-glutathionylated proteins following glutaredoxin-1 catalyzed cysteine derivatization. Biochim Biophys Acta. 2006 Mar;1760(3):380-7. Epub 2006 Feb 20. [PubMed:16515838 ]
  3. Lim A, Prokaeva T, McComb ME, Connors LH, Skinner M, Costello CE: Identification of S-sulfonation and S-thiolation of a novel transthyretin Phe33Cys variant from a patient diagnosed with familial transthyretin amyloidosis. Protein Sci. 2003 Aug;12(8):1775-85. [PubMed:12876326 ]
  4. Butler JD, Spielberg SP: Accumulation of cystine from glutathione-cysteine mixed disulfide in cystinotic fibroblasts; blockade by an inhibitor of gamma-glutamyl transpeptidase. Life Sci. 1982 Dec 6;31(23):2563-70. [PubMed:6130452 ]
  5. Levtchenko EN, Wilmer M, de Graaf-Hess AC, van den Heuvel LP, Blom H, Monnens LA: [From gene to disease: cystinosis]. Ned Tijdschr Geneeskd. 2004 Mar 6;148(10):476-8. [PubMed:15042893 ]
  6. 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 ]