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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2020-11-09 23:12:38 UTC
HMDB IDHMDB0000192
Secondary Accession Numbers
  • HMDB00192
Metabolite Identification
Common NameL-Cystine
DescriptionCystine is an oxidized dimeric form of cysteine. It is formed by linking two cysteine residues via a disulfide bond (Cys-S-S-Cys) between the -SH groups. Cystine is found in high concentrations in digestive enzymes and in the cells of the immune system, skeletal and connective tissues, skin, and hair. Hair and skin are 10-14% cystine. Cystine is the preferred form of cysteine for the synthesis of glutathione in cells involved in the immune system (e.g. macrophages and astrocytes). Lymphocytes and neurons prefer cysteine for glutathione production. Optimizing glutathione levels in macrophages and astrocytes with cystine allows these cells to provide cysteine to lymphocytes and neurons directly upon demand (Wikipedia ).
Structure
Data?1582752115
Synonyms
ValueSource
(R,R)-3,3'-Dithiobis(2-aminopropanoic acid)ChEBI
(R-(R*,r*))-3,3'-dithiobis(2-aminopropanoic acid)ChEBI
3,3'-Dithiobis-L-alanineChEBI
beta,Beta'-diamino-beta,beta'-dicarboxydiethyl disulfideChEBI
beta,Beta'-dithiodialanineChEBI
Bis(beta-amino-beta-carboxyethyl) disulfideChEBI
e921ChEBI
L-alpha-Diamino-beta-dithiolactic acidChEBI
L-DicysteineChEBI
Oxidized L-cysteineChEBI
(R,R)-3,3'-Dithiobis(2-aminopropanoate)Generator
(R-(R*,r*))-3,3'-dithiobis(2-aminopropanoate)Generator
b,Beta'-diamino-b,beta'-dicarboxydiethyl disulfideGenerator
b,Beta'-diamino-b,beta'-dicarboxydiethyl disulphideGenerator
beta,Beta'-diamino-beta,beta'-dicarboxydiethyl disulphideGenerator
Β,beta'-diamino-β,beta'-dicarboxydiethyl disulfideGenerator
Β,beta'-diamino-β,beta'-dicarboxydiethyl disulphideGenerator
b,Beta'-dithiodialanineGenerator
Β,beta'-dithiodialanineGenerator
Bis(b-amino-b-carboxyethyl) disulfideGenerator
Bis(b-amino-b-carboxyethyl) disulphideGenerator
Bis(beta-amino-beta-carboxyethyl) disulphideGenerator
Bis(β-amino-β-carboxyethyl) disulfideGenerator
Bis(β-amino-β-carboxyethyl) disulphideGenerator
L-a-Diamino-b-dithiolactateGenerator
L-a-Diamino-b-dithiolactic acidGenerator
L-alpha-Diamino-beta-dithiolactateGenerator
L-Α-diamino-β-dithiolactateGenerator
L-Α-diamino-β-dithiolactic acidGenerator
(-)-CystineHMDB
(R-(R*,r*))-3,3'-dithiobisHMDB
2-Amino-3-(2-amino-2-carboxy-ethyl)disulfanyl-propanoateHMDB
2-Amino-3-(2-amino-2-carboxy-ethyl)disulfanyl-propanoic acidHMDB
2-Amino-3-[(2-amino-2-carboxyethyl)dithio]propanoateHMDB
2-Amino-3-[(2-amino-2-carboxyethyl)dithio]propanoic acidHMDB
3,3'-DithiobisHMDB
3,3'-Dithiobis[2-amino-[R-(r*,r*)]-propanoateHMDB
3,3'-Dithiobis[2-amino-[R-(r*,r*)]-propanoic acidHMDB
3,3'-DithiodialanineHMDB
b,B'-diamino-b,b'-dicarboxydiethyl disulfideHMDB
b,B'-dithiodialanineHMDB
beta,Beta'-dithiobisalanineHMDB
Bis(b-amino-beta-carboxyethyl) disulfideHMDB
Cysteine disulfideHMDB
CystinHMDB
CystineHMDB
Cystine acidHMDB
D(+)-3,3'-Dithiobis(2-aminopropanoateHMDB
D(+)-3,3'-Dithiobis(2-aminopropanoic acidHMDB
DicysteineHMDB
GelucystineHMDB
L-(-)-CystineHMDB
L-Cysteine disulfideHMDB
L-CystinHMDB
[R-(R*,r*)]-3,3'-dithiobisHMDB
L CystineHMDB
Copper cystinateHMDB
L-CystineKEGG
Chemical FormulaC6H12N2O4S2
Average Molecular Weight240.3
Monoisotopic Molecular Weight240.023848262
IUPAC Name(2R)-2-amino-3-{[(2R)-2-amino-2-carboxyethyl]disulfanyl}propanoic acid
Traditional NameL-cystine
CAS Registry Number56-89-3
SMILES
N[C@@H](CSSC[C@H](N)C(O)=O)C(O)=O
InChI Identifier
InChI=1S/C6H12N2O4S2/c7-3(5(9)10)1-13-14-2-4(8)6(11)12/h3-4H,1-2,7-8H2,(H,9,10)(H,11,12)/t3-,4-/m0/s1
InChI KeyLEVWYRKDKASIDU-IMJSIDKUSA-N
Chemical Taxonomy
Description belongs to the class of organic compounds known as l-cysteine-s-conjugates. L-cysteine-S-conjugates are compounds containing L-cysteine where the thio-group is conjugated.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentL-cysteine-S-conjugates
Alternative Parents
Substituents
  • L-cysteine-s-conjugate
  • Alpha-amino acid
  • L-alpha-amino acid
  • Dicarboxylic acid or derivatives
  • Organic disulfide
  • Dialkyldisulfide
  • Amino acid
  • Sulfenyl compound
  • Carboxylic acid
  • Organopnictogen compound
  • Organic nitrogen compound
  • Primary amine
  • Organosulfur compound
  • Organooxygen compound
  • Organonitrogen compound
  • Primary aliphatic amine
  • Organic oxygen compound
  • Carbonyl group
  • Hydrocarbon derivative
  • Amine
  • Organic oxide
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect

Health effect:

Disposition

Route of exposure:

Source:

Biological location:

Role

Industrial application:

Biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point260.5 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility0.19 mg/mLNot Available
LogP-5.08CHMELIK,J ET AL. (1991)
Predicted Properties
PropertyValueSource
Water Solubility16.8 g/LALOGPS
logP-3.2ALOGPS
logP-5.9ChemAxon
logS-1.2ALOGPS
pKa (Strongest Acidic)1.56ChemAxon
pKa (Strongest Basic)9.34ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count6ChemAxon
Hydrogen Donor Count4ChemAxon
Polar Surface Area126.64 ŲChemAxon
Rotatable Bond Count7ChemAxon
Refractivity54.87 m³·mol⁻¹ChemAxon
Polarizability22.77 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Spectra
Spectrum TypeDescriptionSplash KeyView
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (4 TMS)splash10-00kb-0950000000-a803bc05843192dd737dSpectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-00kb-0940000000-f82b905ef9e7c9e47552Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (4 TMS)splash10-00di-9850000000-49b9a52a8387d6d6f272Spectrum
GC-MSGC-MS Spectrum - GC-MS (4 TMS)splash10-014j-1970000000-9576699202733d4fd7edSpectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00kb-0950000000-a803bc05843192dd737dSpectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00di-9850000000-49b9a52a8387d6d6f272Spectrum
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-014j-1970000000-9576699202733d4fd7edSpectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00kb-0930000000-7cb336c3f272fd0fcd6bSpectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00kb-0920000000-1c414573da4bbe4a66ddSpectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-0076-9400000000-211575093fd775db5d54Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positivesplash10-0076-9112000000-ee49e3b674a4f8bb9ecdSpectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-0006-0890000000-51a32ee40240e45646bcSpectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-00di-9700000000-4054924ddfb90f120e67Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-00di-9100000000-085d45702e61de0f8e4dSpectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QFT , negativesplash10-00di-9710000000-9321541af2ad89bd0478Spectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 1V, negativesplash10-000i-0090000000-041bfa55cb183f42f454Spectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 1V, negativesplash10-000i-0190000000-3611e8b6715129842130Spectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 2V, negativesplash10-000i-0190000000-dbafdd64edad14af9281Spectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 2V, negativesplash10-000i-0290000000-6b80d283287249ba4b87Spectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 3V, negativesplash10-000i-0390000000-c091ce388dfcbd339e3fSpectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 3V, negativesplash10-0079-0690000000-c808ac294a0bc182c5ebSpectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 4V, negativesplash10-0079-0970000000-36297b29c4ef45c658e3Spectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 4V, negativesplash10-00dr-0940000000-a8c735a0f9082cfd7c7cSpectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 5V, negativesplash10-00di-1920000000-9d55705325536cca5eb2Spectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 5V, negativesplash10-00di-1910000000-cd18de2585913152e907Spectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 6V, negativesplash10-00di-1900000000-3118609f8682bee472c6Spectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 7V, negativesplash10-00di-2900000000-213ccc3c067486ff9444Spectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 8V, negativesplash10-00di-3900000000-df68db23afd937a9a75dSpectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 9V, negativesplash10-00di-4900000000-b46d26f57a194d2ff648Spectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 10V, negativesplash10-00di-6900000000-5535cccb24de733cada3Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-006w-2970000000-b80e0aef184b74ce7a36Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-00re-4910000000-a09277c5a6bfc432eb79Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-00xu-9600000000-0518bfcaab380952c123Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-000i-3590000000-aa667d3c092c5af3ae7fSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-00xr-5910000000-122bd251359ca80b184cSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-00dr-9300000000-bbb14e684ff7c06e7916Spectrum
MSMass Spectrum (Electron Ionization)splash10-000x-9000000000-ae0f516d71e6a49fe52fSpectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
1D NMR13C NMR SpectrumNot AvailableSpectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
1D NMR13C NMR SpectrumNot AvailableSpectrum
2D NMR[1H,1H] 2D NMR SpectrumNot AvailableSpectrum
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableSpectrum
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Urine
Tissue Locations
  • All Tissues
  • Placenta
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified17-98 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified26-77 uMNewborn (0-30 days old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified130.0 +/- 20.0 uMNewborn (0-30 days old)Not SpecifiedNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified26-61 uMInfant (1 - 3 months old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified91.0 +/- 20.0 uMChildren (1-13 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified8-68 uMChildren (3 months - 6 years old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified96.0 +/- 19.0 uMChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified15-65 uMChildren (6 - 18 years old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified118.0 +/- 17.0 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified109.0 +/- 24.0 uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified87.0 +/- 13.0 uMChildren (1-13 years old)Not SpecifiedNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified62.9 +/- 27.8 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified109.0 +/- 18.0 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified107.0 +/- 23.0 uMAdult (>18 years old)Not SpecifiedNormal
    • Geigy Scientific ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified0.1 +/- 0.1 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified0.29 +/- 0.27 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0-1.5 uMChildren (0 - 10 years old)Both
Normal
    • BC Children's Hos...
details
Cerebrospinal Fluid (CSF)Detected and Quantified0-1.5 uMAdolescent (>11 years old)Both
Normal
    • BC Children's Hos...
details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedBoth
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified<6.33 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified3.618 (1.316-5.921) umol/mmol creatinineAdult (>18 years old)BothNormal
      Not Available
details
UrineDetected and Quantified10.5 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified<11.76 umol/mmol creatinineAdolescent (13-18 years old)Not SpecifiedNormal details
UrineDetected and Quantified2.92-54.16 umol/mmol creatinineAdult (>18 years old)BothNormal
    • David F. Putnam C...
details
UrineDetected and Quantified12.10 - 43.64 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified6.22 - 26.12 umol/mmol creatinineInfant (1 - 6 months old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified5.09 - 15.60 umol/mmol creatinineInfant (6 months - <1 year old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified3.84 - 13.23 umol/mmol creatinineChildren (1 - 2 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified2.71 - 15.60 umol/mmol creatinineChildren (2 - 4 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified2.71 - 12.10 umol/mmol creatinineChildren (4 - 13 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified1.70 - 20.92 umol/mmol creatinineAdolescent (13 - 21 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified1.70 - 17.86 umol/mmol creatinineAdult (>21 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified1.13-11.086 umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal details
UrineDetected and Quantified10.05 +/- 28.34 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified12.5 (5.0-24.6) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified1.36-57.00 umol/mmol creatinineInfant (0-1 year old)Not SpecifiedNormal details
UrineDetected and Quantified0.15 (0.1-0.2) umol/mmol creatinineNewborn (0-30 days old)BothNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified3.6 +/- 2.0 umol/mmol creatinineChildren (1-13 years old)MaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified9.0 +/- 3.0 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified7.0 +/- 3.0 umol/mmol creatinineAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected and Quantified2.61 +/- 1.42 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified10.351 +/- 4.234 umol/mmol creatinineNot SpecifiedNot SpecifiedNormal details
UrineDetected and Quantified10.351 +/- 4.234 umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal details
UrineDetected and Quantified27.821 +/- 17.962 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected and Quantified5.314-22.612 umol/mmol creatinineInfant (0-1 year old)Not SpecifiedNormal details
UrineDetected and Quantified1.24-15.045 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified2-51 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified<14.71 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified89.32 +/- 56.53 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified128.89 +/- 56.53 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified152.63 +/- 76.88 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal cancer details
BloodDetected and Quantified28.6 (24.2-33.0) uMChildren (1-13 years old)BothJuvenile myoclonic epilepsy (JME) details
BloodDetected and Quantified30.1 (24.5-35.7) uMAdult (>18 years old)BothAcute seizures details
BloodDetected and Quantified37.2 (34.6-39.8) uMAdult (>18 years old)BothRefractory localization-related epilepsy (RLE) details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Schizophrenia
details
BloodDetected and Quantified209.0 +/- 46.0 uMElderly (>65 years old)BothAlzheimer's Disease details
Cerebrospinal Fluid (CSF)Detected and Quantified0.31 +/- 0.06 uMAdult (>18 years old)BothParkinson's Disease details
Cerebrospinal Fluid (CSF)Detected and Quantified7.8 +/- 2.5 umol/mmol creatinineAdult (>18 years old)BothAlzheimer's disease details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
UrineDetected and Quantified136.5-79.36 umol/mmol creatinineChildren (1-13 years old)Male
Cystinuria
details
UrineDetected and Quantified90.800-558.500 umol/mmol creatinineAdult (>18 years old)Both
Cystinuria
details
UrineDetected and Quantified140.0813-306.845 umol/mmol creatinineAdult (>18 years old)Not SpecifiedCystinuria Type I/III details
UrineDetected and Quantified100.0581-246.245 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedCystinuria Type I/III details
UrineDetected and Quantified111.929-196.724 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedCystinuria Type II details
UrineDetected and Quantified11.306-67.836 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedCystinuria Type III details
UrineDetected and Quantified65.0 (30.0-100.0) umol/mmol creatinineAdult (>18 years old)BothCystinuria
    • MetaGene: Metabol...
details
UrineDetected and Quantified575.0 (150.0 -1000.0) umol/mmol creatinineChildren (1-13 years old)BothCystinuria
    • MetaGene: Metabol...
details
UrineDetected and Quantified> 117 umol/mmol creatinineAdult (>18 years old)BothCystinuria details
UrineDetected and Quantified908-5585 umol/mmol creatinineAdult (>18 years old)Both
Hyperlysinuria
details
UrineDetected and Quantified4.423-24.136 umol/mmol creatinineChildren (1-13 years old)FemaleDibasic Amino Aciduria I details
UrineDetected and Quantified6.681-21.125 umol/mmol creatinineAdult (>18 years old)BothDibasic Amino Aciduria I details
UrineDetected and Quantified0 umol/mmol creatinineChildren (1-13 years old)Not Specified
Molybdenum cofactor deficiency
details
UrineDetected and Quantified7 umol/mmol creatinineNewborn (0-30 days old)Not Specified
Molybdenum cofactor deficiency
details
UrineDetected and Quantified135.900-156.400 umol/mmol creatinineInfant (0-1 year old)Not SpecifiedFumaric aciduria details
UrineDetected and Quantified17.486 +/- 14.104 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected and Quantified463.1 umol/mmol creatinineAdult (>18 years old)Both
Cystinuria
details
UrineDetected and Quantified5.975-24.136 umol/mmol creatinineChildren (1 - 13 years old)FemaleHyperdibasic aminoaciduria I details
UrineDetected and Quantified6.681-2.305 umol/mmol creatinineAdult (>18 years old)BothHyperdibasic aminoaciduria I details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Schizophrenia
details
UrineDetected and Quantified72.03 +/- 12.0 umol/mmol creatinineAdult (>18 years old)BothAlzheimer's disease details
Associated Disorders and Diseases
Disease References
Juvenile myoclonic epilepsy
  1. Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed:14992292 ]
Acute seizures
  1. Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed:14992292 ]
Refractory localization-related epilepsy
  1. Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed:14992292 ]
Alzheimer's disease
  1. Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed:17031479 ]
Colorectal cancer
  1. Ni Y, Xie G, Jia W: Metabonomics of human colorectal cancer: new approaches for early diagnosis and biomarker discovery. J Proteome Res. 2014 Sep 5;13(9):3857-70. doi: 10.1021/pr500443c. Epub 2014 Aug 14. [PubMed:25105552 ]
  2. 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 ]
Schizophrenia
  1. Yang J, Chen T, Sun L, Zhao Z, Qi X, Zhou K, Cao Y, Wang X, Qiu Y, Su M, Zhao A, Wang P, Yang P, Wu J, Feng G, He L, Jia W, Wan C: Potential metabolite markers of schizophrenia. Mol Psychiatry. 2013 Jan;18(1):67-78. doi: 10.1038/mp.2011.131. Epub 2011 Oct 25. [PubMed:22024767 ]
Parkinson's disease
  1. Engelborghs S, Marescau B, De Deyn PP: Amino acids and biogenic amines in cerebrospinal fluid of patients with Parkinson's disease. Neurochem Res. 2003 Aug;28(8):1145-50. [PubMed:12834252 ]
Cystinuria
  1. Sakhaee K: Pathogenesis and medical management of cystinuria. Semin Nephrol. 1996 Sep;16(5):435-47. [PubMed:8890399 ]
  2. Fjellstedt E, Harnevik L, Jeppsson JO, Tiselius HG, Soderkvist P, Denneberg T: Urinary excretion of total cystine and the dibasic amino acids arginine, lysine and ornithine in relation to genetic findings in patients with cystinuria treated with sulfhydryl compounds. Urol Res. 2003 Dec;31(6):417-25. Epub 2003 Oct 25. [PubMed:14586528 ]
  3. Rodriguez LM, Santos F, Malaga S, Martinez V: Effect of a low sodium diet on urinary elimination of cystine in cystinuric children. Nephron. 1995;71(4):416-8. [PubMed:8587621 ]
  4. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Eosinophilic esophagitis
  1. (). Mordechai, Hien, and David S. Wishart. .
Molybdenum cofactor deficiency
  1. van Gennip AH, Abeling NG, Stroomer AE, Overmars H, Bakker HD: The detection of molybdenum cofactor deficiency: clinical symptomatology and urinary metabolite profile. J Inherit Metab Dis. 1994;17(1):142-5. [PubMed:8051926 ]
Fumarase deficiency
  1. Allegri G, Fernandes MJ, Scalco FB, Correia P, Simoni RE, Llerena JC Jr, de Oliveira ML: Fumaric aciduria: an overview and the first Brazilian case report. J Inherit Metab Dis. 2010 Aug;33(4):411-9. doi: 10.1007/s10545-010-9134-2. Epub 2010 Jun 15. [PubMed:20549362 ]
Hyperdibasic aminoaciduria I
  1. Whelan DT, Scriver CR: Hyperdibasicaminoaciduria: an inherited disorder of amino acid transport. Pediatr Res. 1968 Nov;2(6):525-34. [PubMed:5727921 ]
Hyperlysinuria
  1. Fjellstedt E, Harnevik L, Jeppsson JO, Tiselius HG, Soderkvist P, Denneberg T: Urinary excretion of total cystine and the dibasic amino acids arginine, lysine and ornithine in relation to genetic findings in patients with cystinuria treated with sulfhydryl compounds. Urol Res. 2003 Dec;31(6):417-25. Epub 2003 Oct 25. [PubMed:14586528 ]
Associated OMIM IDs
DrugBank IDDB00138
Phenol Explorer Compound IDNot Available
FooDB IDFDB012672
KNApSAcK IDC00001352
Chemspider ID60997
KEGG Compound IDC00491
BioCyc IDNot Available
BiGG ID35134
Wikipedia LinkCystine
METLIN ID5207
PubChem Compound67678
PDB IDNot Available
ChEBI ID16283
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB IDMDB00000092
References
Synthesis ReferenceGrossi, Loris; Montevecchi, Pier Carlo. S-Nitrosocysteine and Cystine from Reaction of Cysteine with Nitrous Acid. A Kinetic Investigation. Journal of Organic Chemistry (2002), 67(24), 8625-8630.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Rumpold H, Mascher K, Untergasser G, Plas E, Hermann M, Berger P: Trans-differentiation of prostatic stromal cells leads to decreased glycoprotein hormone alpha production. J Clin Endocrinol Metab. 2002 Nov;87(11):5297-303. [PubMed:12414905 ]
  2. Alexander S, Ison C: Evaluation of commercial kits for the identification of Neisseria gonorrhoeae. J Med Microbiol. 2005 Sep;54(Pt 9):827-31. [PubMed:16091433 ]
  3. Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed:14992292 ]
  4. Terada T, Maeda H, Okamoto K, Nishinaka T, Mizoguchi T, Nishihara T: Modulation of glutathione S-transferase activity by a thiol/disulfide exchange reaction and involvement of thioltransferase. Arch Biochem Biophys. 1993 Jan;300(1):495-500. [PubMed:8424686 ]
  5. Sonies BC, Almajid P, Kleta R, Bernardini I, Gahl WA: Swallowing dysfunction in 101 patients with nephropathic cystinosis: benefit of long-term cysteamine therapy. Medicine (Baltimore). 2005 May;84(3):137-46. [PubMed:15879904 ]
  6. Mogil'naia GM, Shubich MG: [Comparison of the histochemical bases of epidermis differentiation in vertebrates]. Arkh Anat Gistol Embriol. 1976 Mar;70(3):46-52. [PubMed:1275714 ]
  7. Peters T, Thaete C, Wolf S, Popp A, Sedlmeier R, Grosse J, Nehls MC, Russ A, Schlueter V: A mouse model for cystinuria type I. Hum Mol Genet. 2003 Sep 1;12(17):2109-20. [PubMed:12923163 ]
  8. Park M, Helip-Wooley A, Thoene J: Lysosomal cystine storage augments apoptosis in cultured human fibroblasts and renal tubular epithelial cells. J Am Soc Nephrol. 2002 Dec;13(12):2878-87. [PubMed:12444206 ]
  9. Rossi S, Herrine SK, Navarro VJ: Cystinosis as a cause of noncirrhotic portal hypertension. Dig Dis Sci. 2005 Jul;50(7):1372-5. [PubMed:16047489 ]
  10. Sakhaee K: Pathogenesis and medical management of cystinuria. Semin Nephrol. 1996 Sep;16(5):435-47. [PubMed:8890399 ]
  11. Orakzai N, Hanbury DC, Farrington K: Screening for biochemical abnormalities in urolithiasis patients. J Ayub Med Coll Abbottabad. 2004 Apr-Jun;16(2):60-3. [PubMed:15455621 ]
  12. 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. [PubMed:2026685 ]
  13. Mitchell BF, Chibbar R: Synthesis and metabolism of oxytocin in late gestation in human decidua. Adv Exp Med Biol. 1995;395:365-80. [PubMed:8713992 ]
  14. Behr T, Becker W, Hannappel E, Goldenberg DM, Wolf F: Targeting of liver metastases of colorectal cancer with IgG, F(ab')2, and Fab' anti-carcinoembryonic antigen antibodies labeled with 99mTc: the role of metabolism and kinetics. Cancer Res. 1995 Dec 1;55(23 Suppl):5777s-5785s. [PubMed:7493346 ]
  15. Engelborghs S, Marescau B, De Deyn PP: Amino acids and biogenic amines in cerebrospinal fluid of patients with Parkinson's disease. Neurochem Res. 2003 Aug;28(8):1145-50. [PubMed:12834252 ]
  16. Coe FL, Evan A, Worcester E: Kidney stone disease. J Clin Invest. 2005 Oct;115(10):2598-608. [PubMed:16200192 ]
  17. Kubilus J, MacDonald MJ, Baden HP: Epidermal proteins of cultured human and bovine keratinocytes. Biochim Biophys Acta. 1979 Jun 19;578(2):484-92. [PubMed:486533 ]
  18. Tezuka T, Takahashi M: The cystine-rich envelope protein from human epidermal stratum corneum cells. J Invest Dermatol. 1987 Jan;88(1):47-51. [PubMed:3794387 ]
  19. Worcester EM, Coe FL, Evan AP, Parks JH: Reduced renal function and benefits of treatment in cystinuria vs other forms of nephrolithiasis. BJU Int. 2006 Jun;97(6):1285-90. [PubMed:16686727 ]
  20. Allen JW, Shanker G, Tan KH, Aschner M: The consequences of methylmercury exposure on interactive functions between astrocytes and neurons. Neurotoxicology. 2002 Dec;23(6):755-9. [PubMed:12520765 ]
  21. 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 ]

Enzymes

General function:
Involved in pyridoxal phosphate binding
Specific function:
Catalyzes the last step in the trans-sulfuration pathway from methionine to cysteine. Has broad substrate specificity. Converts cystathionine to cysteine, ammonia and 2-oxobutanoate. Converts two cysteine molecules to lanthionine and hydrogen sulfide. Can also accept homocysteine as substrate. Specificity depends on the levels of the endogenous substrates. Generates the endogenous signaling molecule hydrogen sulfide (H2S), and so contributes to the regulation of blood pressure. Acts as a cysteine-protein sulfhydrase by mediating sulfhydration of target proteins: sulfhydration consists of converting -SH groups into -SSH on specific cysteine residues of target proteins such as GAPDH, PTPN1 and NF-kappa-B subunit RELA, thereby regulating their function.
Gene Name:
CTH
Uniprot ID:
P32929
Molecular weight:
41259.91
Reactions
L-Cystine + Water → Pyruvic acid + Ammonia + Thiocysteinedetails
General function:
Involved in catalytic activity
Specific function:
Involved in the high-affinity, sodium-independent transport of cystine and neutral and dibasic amino acids (system B(0,+)-like activity). May function as an activator of SLC7A9 and be involved in the high-affinity reabsorption of cystine in the kidney tubule
Gene Name:
SLC3A1
Uniprot ID:
Q07837
Molecular weight:
78851.4
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
General function:
Involved in transport
Specific function:
Involved in the high-affinity, sodium-independent transport of cystine and neutral and dibasic amino acids (system b(0,+)-like activity). Thought to be responsible for the high- affinity reabsorption of cystine in the kidney tubule
Gene Name:
SLC7A9
Uniprot ID:
P82251
Molecular weight:
53480.8
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
General function:
Involved in transport
Specific function:
Sodium-independent, high-affinity exchange of anionic amino acids with high specificity for anionic form of cystine and glutamate.
Gene Name:
SLC7A11
Uniprot ID:
Q9UPY5
Molecular weight:
55422.44
References
  1. Tomi M, Hosoya K, Takanaga H, Ohtsuki S, Terasaki T: Induction of xCT gene expression and L-cystine transport activity by diethyl maleate at the inner blood-retinal barrier. Invest Ophthalmol Vis Sci. 2002 Mar;43(3):774-9. [PubMed:11867597 ]
  2. Hosoya K, Tomi M, Ohtsuki S, Takanaga H, Saeki S, Kanai Y, Endou H, Naito M, Tsuruo T, Terasaki T: Enhancement of L-cystine transport activity and its relation to xCT gene induction at the blood-brain barrier by diethyl maleate treatment. J Pharmacol Exp Ther. 2002 Jul;302(1):225-31. [PubMed:12065721 ]
  3. Ruiz E, Siow RC, Bartlett SR, Jenner AM, Sato H, Bannai S, Mann GE: Vitamin C inhibits diethylmaleate-induced L-cystine transport in human vascular smooth muscle cells. Free Radic Biol Med. 2003 Jan 1;34(1):103-10. [PubMed:12498985 ]
  4. Bridges CC, Zalups RK: Cystine and glutamate transport in renal epithelial cells transfected with human system x(-) (c). Kidney Int. 2005 Aug;68(2):653-64. [PubMed:16014042 ]
  5. Tomi M, Funaki T, Abukawa H, Katayama K, Kondo T, Ohtsuki S, Ueda M, Obinata M, Terasaki T, Hosoya K: Expression and regulation of L-cystine transporter, system xc-, in the newly developed rat retinal Muller cell line (TR-MUL). Glia. 2003 Sep;43(3):208-17. [PubMed:12898700 ]
General function:
Involved in sodium:dicarboxylate symporter activity
Specific function:
Transporter for alanine, serine, cysteine, and threonine. Exhibits sodium dependence
Gene Name:
SLC1A4
Uniprot ID:
P43007
Molecular weight:
55722.5
General function:
Involved in L-cystine transmembrane transporter activity
Specific function:
Thought to transport cystine out of lysosomes
Gene Name:
CTNS
Uniprot ID:
O60931
Molecular weight:
41737.6
References
  1. Kalatzis V, Cherqui S, Antignac C, Gasnier B: Cystinosin, the protein defective in cystinosis, is a H(+)-driven lysosomal cystine transporter. EMBO J. 2001 Nov 1;20(21):5940-9. [PubMed:11689434 ]

Transporters

General function:
Involved in transmembrane transport
Specific function:
Sodium-independent transporter that mediates the update of aromatic acid. Can function as a net efflux pathway for aromatic amino acids in the basosolateral epithelial cells
Gene Name:
SLC16A10
Uniprot ID:
Q8TF71
Molecular weight:
55492.1
References
  1. Kim DK, Kanai Y, Chairoungdua A, Matsuo H, Cha SH, Endou H: Expression cloning of a Na+-independent aromatic amino acid transporter with structural similarity to H+/monocarboxylate transporters. J Biol Chem. 2001 May 18;276(20):17221-8. Epub 2001 Feb 20. [PubMed:11278508 ]