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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2021-09-14 15:00:08 UTC
HMDB IDHMDB0000574
Secondary Accession Numbers
  • HMDB00574
Metabolite Identification
Common NameL-Cysteine
DescriptionCysteine (Cys), also known as L-cysteine is an alpha-amino acid. These are amino acids in which the amino group is attached to the carbon atom immediately adjacent to the carboxylate group (alpha carbon). Amino acids are organic compounds that contain amino (–NH2) and carboxyl (–COOH) functional groups, along with a side chain (R group) specific to each amino acid. L-alanine is one of 20 proteinogenic amino acids, i.e., the amino acids used in the biosynthesis of proteins. Cysteine is found in all organisms ranging from bacteria to plants to animals. It is classified as an aliphatic, non-polar, sulfur-containing amino acid. Cysteine is an important source of sulfur in human metabolism, and although it is classified as a non-essential amino acid, cysteine may be essential for infants, the elderly, and individuals with certain metabolic disease or who suffer from malabsorption syndromes. Cysteine can occasionally be considered as an essential or conditionally essential amino acid. Cysteine is unique amongst the twenty natural amino acids as it contains a thiol group. Thiol groups can undergo oxidation/reduction (redox) reactions; when cysteine is oxidized it can form cystine, which is two cysteine residues joined by a disulfide bond. This reaction is reversible since the reduction of this disulphide bond regenerates two cysteine molecules. The disulphide bonds of cystine are crucial to defining the structures of many proteins. Cysteine is often involved in electron-transfer reactions, and help the enzyme catalyze its reaction. Cysteine is also part of the antioxidant glutathione. N-Acetyl-L-cysteine (NAC) is a form of cysteine where an acetyl group is attached to cysteine's nitrogen atom and is sold as a dietary supplement. Cysteine is named after cystine, which comes from the Greek word kustis meaning bladder (cystine was first isolated from kidney stones). Oxidation of cysteine can produce a disulfide bond with another thiol and further oxidation can produce sulphfinic or sulfonic acids. The cysteine thiol group is also a nucleophile and can undergo addition and substitution reactions. Thiol groups become much more reactive when they are ionized, and cysteine residues in proteins have pKa values close to neutrality, so they are often in their reactive thiolate form in the cell. The thiol group also has a high affinity for heavy metals and proteins containing cysteine will bind metals such as mercury, lead, and cadmium tightly. Due to this ability to undergo redox reactions, cysteine has antioxidant properties. Cysteine is important in energy metabolism. As cystine, it is a structural component of many tissues and hormones. Cysteine has clinical uses ranging from treating baldness to psoriasis to preventing smoker's hack. In some cases, oral cysteine therapy has proved excellent for treatment of asthmatics, enabling them to stop theophylline and other medications. Cysteine also enhances the effect of topically applied silver, tin, and zinc salts in preventing dental cavities. In the future, cysteine may play a role in the treatment of cobalt toxicity, diabetes, psychosis, cancer, and seizures (http://www.dcnutrition.com/AminoAcids/). Cysteine has been identified as a uremic toxin according to the European Uremic Toxin Working Group (PMID: 22626821 ).
Structure
Data?1582752141
Synonyms
ValueSource
(2R)-2-Amino-3-mercaptopropanoic acidChEBI
(2R)-2-Amino-3-sulfanylpropanoic acidChEBI
(R)-2-Amino-3-mercaptopropanoic acidChEBI
CChEBI
CysChEBI
CYSTEINEChEBI
e 920ChEBI
e-920ChEBI
e920ChEBI
FREE cysteineChEBI
L-2-Amino-3-mercaptopropionic acidChEBI
L-CysteinChEBI
L-ZysteinChEBI
EcolanKegg
(2R)-2-Amino-3-mercaptopropanoateGenerator
(2R)-2-Amino-3-sulfanylpropanoateGenerator
(2R)-2-Amino-3-sulphanylpropanoateGenerator
(2R)-2-Amino-3-sulphanylpropanoic acidGenerator
(R)-2-Amino-3-mercaptopropanoateGenerator
L-2-Amino-3-mercaptopropionateGenerator
(+)-2-Amino-3-mercaptopropionic acidHMDB
(R)-(+)-CysteineHMDB
(R)-2-Amino-3-mercapto-propanoateHMDB
(R)-2-Amino-3-mercapto-propanoic acidHMDB
(R)-CysteineHMDB
2-Amino-3-mercaptopropanoateHMDB
2-Amino-3-mercaptopropanoic acidHMDB
2-Amino-3-mercaptopropionateHMDB
2-Amino-3-mercaptopropionic acidHMDB
3-Mercapto-L-alanineHMDB
AcetylcysteineHMDB
alpha-Amino-beta-thiolpropionic acidHMDB
b-MercaptoalanineHMDB
beta-MercaptoalanineHMDB
CarbocysteineHMDB
CisteinaHMDB
CisteinumHMDB
CysteinHMDB
CysteinumHMDB
Half-cystineHMDB
L CysteineHMDB
L-(+)-CysteineHMDB
L-2-Amino-3-mercaptopropanoateHMDB
L-2-Amino-3-mercaptopropanoic acidHMDB
PolycysteineHMDB
ThioserineHMDB
Cysteine hydrochlorideHMDB
Zinc cysteinateHMDB
Half cystineHMDB
Chemical FormulaC3H7NO2S
Average Molecular Weight121.158
Monoisotopic Molecular Weight121.019749163
IUPAC Name(2R)-2-amino-3-sulfanylpropanoic acid
Traditional NameL-cysteine
CAS Registry Number52-90-4
SMILES
N[C@@H](CS)C(O)=O
InChI Identifier
InChI=1S/C3H7NO2S/c4-2(1-7)3(5)6/h2,7H,1,4H2,(H,5,6)/t2-/m0/s1
InChI KeyXUJNEKJLAYXESH-REOHCLBHSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as cysteine and derivatives. Cysteine and derivatives are compounds containing cysteine or a derivative thereof resulting from reaction of cysteine at the amino group or the carboxy group, or from the replacement of any hydrogen of glycine by a heteroatom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentCysteine and derivatives
Alternative Parents
Substituents
  • Cysteine or derivatives
  • Alpha-amino acid
  • L-alpha-amino acid
  • Amino acid
  • Alkylthiol
  • Carboxylic acid
  • Monocarboxylic acid or derivatives
  • Organic oxygen compound
  • Primary amine
  • Organosulfur compound
  • Organooxygen compound
  • Organonitrogen compound
  • Organic nitrogen compound
  • Primary aliphatic amine
  • Carbonyl group
  • Amine
  • Hydrocarbon derivative
  • Organopnictogen compound
  • Organic oxide
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect

Adverse health effect

Disposition

Biological location

Source

Route of exposure

Process

Naturally occurring process

Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point220 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility277 mg/mL at 25 °CBEILSTEIN
LogP-2.49HANSCH,C ET AL. (1995)
Experimental Spectral Properties

Experimental Collision Cross Sections

PredictorAdduct TypeData SourceCCS Value (Å2)Reference
DeepCCS[M-H]-Baker144.47230932474
DeepCCS[M+H]+Astarita_pos112.730932474
DeepCCS[M+H]+Baker149.56630932474
AllCCS[M-H]-Not Available144.472http://allccs.zhulab.cn/database/detail?ID=AllCCS00001743
Predicted Molecular Properties
PropertyValueSource
Water Solubility23.1 g/LALOGPS
logP10(-2.6) g/LALOGPS
logP10(-2.8) g/LChemAxon
logS10(-0.72) g/LALOGPS
pKa (Strongest Acidic)2.35ChemAxon
pKa (Strongest Basic)9.05ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count3ChemAxon
Polar Surface Area63.32 ŲChemAxon
Rotatable Bond Count2ChemAxon
Refractivity28.22 m³·mol⁻¹ChemAxon
Polarizability11.41 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Spectral Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
DarkChem[M+H]+123.90831661259
DarkChem[M+H]+123.90831661259
DarkChem[M-H]-118.84131661259
DarkChem[M-H]-118.84131661259
AllCCS[M+H]+130.14632859911
AllCCS[M-H]-128.66632859911

Predicted Kovats Retention Indices

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
L-Cysteine,1TMS,#1C[Si](C)(C)OC(=O)[C@@H](N)CS1232.3Semi standard non polarhttps://arxiv.org/abs/1905.12712
L-Cysteine,1TMS,#2C[Si](C)(C)SC[C@H](N)C(=O)O1396.7Semi standard non polarhttps://arxiv.org/abs/1905.12712
L-Cysteine,1TMS,#3C[Si](C)(C)N[C@@H](CS)C(=O)O1356.8Semi standard non polarhttps://arxiv.org/abs/1905.12712
L-Cysteine,1TBDMS,#1CC(C)(C)[Si](C)(C)OC(=O)[C@@H](N)CS1477.3Semi standard non polarhttps://arxiv.org/abs/1905.12712
L-Cysteine,1TBDMS,#2CC(C)(C)[Si](C)(C)SC[C@H](N)C(=O)O1634.9Semi standard non polarhttps://arxiv.org/abs/1905.12712
L-Cysteine,1TBDMS,#3CC(C)(C)[Si](C)(C)N[C@@H](CS)C(=O)O1628.9Semi standard non polarhttps://arxiv.org/abs/1905.12712
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental GC-MSGC-MS Spectrum - L-Cysteine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (4 TMS)splash10-00kb-0950000000-df7e91c95b610ff21c792014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Cysteine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (4 TMS)splash10-00kb-0940000000-aefe34765fb447090a232014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Cysteine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (4 TMS)splash10-00kb-0970000000-10a155c40ea4990230522014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Cysteine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-00kb-0940000000-037a3a34651c3154b3b32014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Cysteine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (4 TMS)splash10-00di-9850000000-118c43e33861a6baa8d22014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Cysteine EI-B (Non-derivatized)splash10-014j-0690100000-0aeb88fd507505e6b7182017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Cysteine GC-EI-TOF (Non-derivatized)splash10-00kb-0950000000-df7e91c95b610ff21c792017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Cysteine GC-EI-TOF (Non-derivatized)splash10-00kb-0940000000-aefe34765fb447090a232017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Cysteine GC-EI-TOF (Non-derivatized)splash10-00kb-0970000000-10a155c40ea4990230522017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Cysteine GC-EI-TOF (Non-derivatized)splash10-00kb-0940000000-037a3a34651c3154b3b32017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Cysteine GC-EI-QQ (Non-derivatized)splash10-0uk9-5619100000-8cb2558373966174ced32017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Cysteine GC-EI-TOF (Non-derivatized)splash10-00di-9850000000-118c43e33861a6baa8d22017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Cysteine GC-EI-TOF (Non-derivatized)splash10-0gi0-0960000000-03b2097de6f9637d29cb2017-09-12HMDB team, MONA, MassBankView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - L-Cysteine GC-MS (Non-derivatized) - 70eV, Positivesplash10-004l-9100000000-4553906a941a5e87ec972016-09-22Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - L-Cysteine GC-MS (1 TMS) - 70eV, Positivesplash10-004i-9200000000-cfaf705cd0452d4284542017-10-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - L-Cysteine GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Cysteine Quattro_QQQ 10V, Positive-QTOF (Annotated)splash10-00b9-9600000000-374c5872d686628327692012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Cysteine Quattro_QQQ 25V, Positive-QTOF (Annotated)splash10-0a4i-9000000000-ddbd3df6b8dbb280ffba2012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Cysteine Quattro_QQQ 40V, Positive-QTOF (Annotated)splash10-0a4i-9000000000-320a2c77443b80ebf7332012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Cysteine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOFsplash10-0089-0900000000-9dcd3d757c5cd11eb18e2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Cysteine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOFsplash10-0udi-3900000000-212e081fe83ad70de0ad2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Cysteine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOFsplash10-000i-9000000000-2eb01f41c225f614db242012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Cysteine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOFsplash10-001i-0900000000-a19834eb7cb9f211fdf22012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Cysteine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOFsplash10-00di-0900000000-2458f2587761e779ed932012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Cysteine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOFsplash10-0a4i-9000000000-77e590f0ed26f69b31c02012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Cysteine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOFsplash10-0udi-3900000000-7b1857997392b006b95f2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Cysteine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOFsplash10-0a4i-3900000000-bc268c27ed5706a4bbd22012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Cysteine CE-ESI-TOF (CE-system connected to 6210 Time-of-Flight MS, Agilent) , Positive-QTOFsplash10-00di-0900000000-4573390bccc238e3c91b2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Cysteine LC-ESI-ITFT , positive-QTOFsplash10-0udi-3900000000-212e081fe83ad70de0ad2017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Cysteine LC-ESI-ITFT , positive-QTOFsplash10-0udi-3900000000-7b1857997392b006b95f2017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Cysteine QqQ 14V, negative-QTOFsplash10-001i-9000000000-5dc69d3b59bd15290aef2020-07-21HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Cysteine 10V, Positive-QTOFsplash10-0a6r-9100000000-ed3c9c47ed98679090ab2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Cysteine 0V, Positive-QTOFsplash10-00di-2900000000-8bb219d17ccdf7ac0a5d2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Cysteine 30V, Positive-QTOFsplash10-0a4i-9000000000-e623d5a27b5075c82b4d2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Cysteine 10V, Positive-QTOFsplash10-056r-9100000000-4e26cc13af7c878115f72021-09-20HMDB team, MONAView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - L-Cysteine 10V, Positive-QTOFsplash10-00b9-9600000000-4352a7b437c34c04d9f02016-09-12Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - L-Cysteine 20V, Positive-QTOFsplash10-004i-9200000000-7b4cd034c717561c61dd2016-09-12Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - L-Cysteine 40V, Positive-QTOFsplash10-052f-9000000000-8f351cbca6ffed356a9b2016-09-12Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - L-Cysteine 10V, Negative-QTOFsplash10-00di-6900000000-51ba0df80cc33423420b2016-09-12Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - L-Cysteine 20V, Negative-QTOFsplash10-0079-9400000000-4aa2b707c391d5838d292016-09-12Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - L-Cysteine 40V, Negative-QTOFsplash10-001i-9000000000-95c7672c7836c0fc51c92016-09-12Wishart LabView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Predicted 1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Experimental 1D NMR13C NMR Spectrum (1D, 400 MHz, H2O, experimental)2021-10-10Wishart LabView Spectrum
Experimental 2D NMR[1H, 1H]-TOCSY 2D NMR Spectrum (experimental)2012-12-04Wishart LabView Spectrum
Experimental 2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental)2012-12-05Wishart LabView Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Mitochondria
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Sweat
  • Urine
Tissue Locations
  • Adrenal Cortex
  • Epidermis
  • Fibroblasts
  • Intestine
  • Kidney
  • Liver
  • Neuron
  • Placenta
  • Platelet
  • Prostate
  • Skeletal Muscle
  • Spleen
  • Testis
  • Thyroid Gland
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified24-100 uMInfant (0-1 year old)BothNormal
    • Clinical and Labo...
details
BloodDetected and Quantified197.00 +/- 56.00 uMAdolescent (13-18 years old)BothNormal details
BloodDetected and Quantified131.00 +/- 40.00 uMInfant (0-1 year old)BothNormal details
BloodDetected and Quantified210.00 +/- 40.00 uMChildren (1-13 years old)BothNormal details
BloodDetected and Quantified0.36 +/- 0.02 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified212.00 +/- 23.00 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified197.0-283.0 uMAdult (>18 years old)FemaleNormal details
BloodDetected and Quantified52.0 (41.0-63.0) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified231.07-307.90 uMAdult (>18 years old)MaleNormal details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
BloodDetected and Quantified33.5 +/- 10.3 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.054 (0.017-0.108) uMAdult (>18 years old)Not SpecifiedNormal details
FecesDetected but not QuantifiedNot QuantifiedChildren (1-13 years old)BothNormal 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
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedNot Specified
Normal
details
SalivaDetected and Quantified1.30 +/- 0.26 uMAdult (>18 years old)BothNormal details
SweatDetected and Quantified< 10 uMAdult (60 years old)Male
Normal
details
SweatDetected and Quantified< 10 uMAdult (40 years old)Male
Normal
details
UrineDetected and Quantified34(26-50) umol/mmol creatinineAdolescent (13-18 years old)Not SpecifiedNormal details
UrineDetected and Quantified3.322 (1.447-5.197) umol/mmol creatinineAdult (>18 years old)BothNormal
      Not Available
details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected and Quantified22-27 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified30.32 +/- 24.58 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified81.0 (36.7-147.6) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified32.00 +/- 10.00 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified28.00 +/- 9.00 umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected and Quantified34.4 +/- 11.00 umol/mmol creatinineAdolescent (13-18 years old)BothNormal details
UrineDetected and Quantified33.4 +/- 15.5 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified19-27 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified65.8 (23.1-134.5) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified22.591 +/- 10.057 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected and Quantified14.9 umol/mmol creatinineAdult (>18 years old)BothNormal details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.355 (0.157-1.146) uMAdult (>18 years old)Not SpecifiedMultiple sclerosis details
BloodDetected and Quantified0.420 (0.154-0.874) uMAdult (>18 years old)Not SpecifiedStroke details
BloodDetected and Quantified0.415 (0.199-1.038) uMAdult (>18 years old)Not SpecifiedPeripheral neuropathy details
BloodDetected and Quantified0.491 (0.247-1.764) uMAdult (>18 years old)Not SpecifiedDementia (Alzheimer's and non-Alzheimer's) details
BloodDetected and Quantified5000000 uMChildren (1-13 years old)Female
Sulfite oxidase deficiency
details
BloodDetected and Quantified0.56 +/- 0.03 uMAdult (>18 years old)Bothuremia details
BloodDetected and Quantified<5 uMInfant (0-1 year old)Male
Sulfite oxidase deficiency
    • Clinical and Labo...
details
BloodDetected and Quantified13.0 (10.0-16.0) uMAdult (>18 years old)BothAIDS details
BloodDetected and Quantified12.81 +/- 2.17 uMElderly (>65 years old)BothAlzheimer's Disease details
Cerebrospinal Fluid (CSF)Detected and Quantified0.042 (0.022-0.10) uMAdult (>18 years old)Not SpecifiedMultiple sclerosis details
Cerebrospinal Fluid (CSF)Detected and Quantified0.048 (0.018-0.10) uMAdult (>18 years old)Not SpecifiedStroke details
Cerebrospinal Fluid (CSF)Detected and Quantified0.034 (0.018-0.075) uMAdult (>18 years old)Not SpecifiedPeripheral neuropathy details
Cerebrospinal Fluid (CSF)Detected and Quantified0.047 (0.025-0.125) uMAdult (>18 years old)Not SpecifiedDementia (Alzheimer's and non-Alzheimer's) details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not QuantifiedNot QuantifiedChildren (1-13 years old)BothAutism details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal Cancer details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal cancer details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
UrineDetected and Quantified81-106 umol/mmol creatinineAdolescent (13-18 years old)MaleCystinylglycinuria details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothBladder cancer details
UrineDetected and Quantified29.971 +/- 24.6 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
Associated Disorders and Diseases
Disease References
AIDS
  1. Naisbitt DJ, Vilar FJ, Stalford AC, Wilkins EG, Pirmohamed M, Park BK: Plasma cysteine deficiency and decreased reduction of nitrososulfamethoxazole with HIV infection. AIDS Res Hum Retroviruses. 2000 Dec 10;16(18):1929-38. [PubMed:11153075 ]
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 ]
Uremia
  1. Duranton F, Cohen G, De Smet R, Rodriguez M, Jankowski J, Vanholder R, Argiles A: Normal and pathologic concentrations of uremic toxins. J Am Soc Nephrol. 2012 Jul;23(7):1258-70. doi: 10.1681/ASN.2011121175. Epub 2012 May 24. [PubMed:22626821 ]
Sulfite oxidase deficiency, ISOLATED
  1. Rocha S, Ferreira AC, Dias AI, Vieira JP, Sequeira S: Sulfite oxidase deficiency--an unusual late and mild presentation. Brain Dev. 2014 Feb;36(2):176-9. doi: 10.1016/j.braindev.2013.01.013. Epub 2013 Feb 27. [PubMed:23452914 ]
  2. Choong T. et al. (2010). Clinical and Laboratory Barriers to the Timely Diagnosis of Sulphite Oxidase Deficiency. Proceedings of Singapore Healthcare, 19(2), 94-100.. Proceedings of Singapore Healthcare.
Multiple sclerosis
  1. Obeid R, Kasoha M, Knapp JP, Kostopoulos P, Becker G, Fassbender K, Herrmann W: Folate and methylation status in relation to phosphorylated tau protein(181P) and beta-amyloid(1-42) in cerebrospinal fluid. Clin Chem. 2007 Jun;53(6):1129-36. Epub 2007 Mar 23. [PubMed:17384003 ]
Stroke
  1. Obeid R, Kasoha M, Knapp JP, Kostopoulos P, Becker G, Fassbender K, Herrmann W: Folate and methylation status in relation to phosphorylated tau protein(181P) and beta-amyloid(1-42) in cerebrospinal fluid. Clin Chem. 2007 Jun;53(6):1129-36. Epub 2007 Mar 23. [PubMed:17384003 ]
Peripheral neuropathy
  1. Obeid R, Kasoha M, Knapp JP, Kostopoulos P, Becker G, Fassbender K, Herrmann W: Folate and methylation status in relation to phosphorylated tau protein(181P) and beta-amyloid(1-42) in cerebrospinal fluid. Clin Chem. 2007 Jun;53(6):1129-36. Epub 2007 Mar 23. [PubMed:17384003 ]
Dementia
  1. Obeid R, Kasoha M, Knapp JP, Kostopoulos P, Becker G, Fassbender K, Herrmann W: Folate and methylation status in relation to phosphorylated tau protein(181P) and beta-amyloid(1-42) in cerebrospinal fluid. Clin Chem. 2007 Jun;53(6):1129-36. Epub 2007 Mar 23. [PubMed:17384003 ]
Colorectal cancer
  1. Monleon D, Morales JM, Barrasa A, Lopez JA, Vazquez C, Celda B: Metabolite profiling of fecal water extracts from human colorectal cancer. NMR Biomed. 2009 Apr;22(3):342-8. doi: 10.1002/nbm.1345. [PubMed:19006102 ]
  2. 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 ]
  3. Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ]
  4. Sinha R, Ahn J, Sampson JN, Shi J, Yu G, Xiong X, Hayes RB, Goedert JJ: Fecal Microbiota, Fecal Metabolome, and Colorectal Cancer Interrelations. PLoS One. 2016 Mar 25;11(3):e0152126. doi: 10.1371/journal.pone.0152126. eCollection 2016. [PubMed:27015276 ]
  5. 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 ]
Autism
  1. De Angelis M, Piccolo M, Vannini L, Siragusa S, De Giacomo A, Serrazzanetti DI, Cristofori F, Guerzoni ME, Gobbetti M, Francavilla R: Fecal microbiota and metabolome of children with autism and pervasive developmental disorder not otherwise specified. PLoS One. 2013 Oct 9;8(10):e76993. doi: 10.1371/journal.pone.0076993. eCollection 2013. [PubMed:24130822 ]
Eosinophilic esophagitis
  1. (). Mordechai, Hien, and David S. Wishart. .
Cystinylglycinuria
  1. Bellet H, Rejou F, Vallat C, Mion H, Dimeglio A: Cystinylglycinuria: a new neurometabolic disorder? J Inherit Metab Dis. 1999 May;22(3):231-4. [PubMed:10384375 ]
Associated OMIM IDs
DrugBank IDDB00151
Phenol Explorer Compound IDNot Available
FooDB IDFDB012678
KNApSAcK IDC00001351
Chemspider ID5653
KEGG Compound IDC00097
BioCyc IDCYS
BiGG ID33843
Wikipedia LinkCysteine
METLIN ID5556
PubChem Compound5862
PDB IDNot Available
ChEBI ID17561
Food Biomarker OntologyNot Available
VMH IDCYS_L
MarkerDB IDMDB00000192
Good Scents IDNot Available
References
Synthesis ReferenceKumagai, Hidehiko; Tanaka, Hideyuki; Sejima, Shunsuke; Yamada, Hideaki. Elimination and replacement reactions of b-chloro-L-alanine by cysteine desulfhydrase from Aerobacter aerogenes. Agricultural and Biological Chemistry (1977), 41(10), 2071-5.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Yu FH, Westenbroek RE, Silos-Santiago I, McCormick KA, Lawson D, Ge P, Ferriera H, Lilly J, DiStefano PS, Catterall WA, Scheuer T, Curtis R: Sodium channel beta4, a new disulfide-linked auxiliary subunit with similarity to beta2. J Neurosci. 2003 Aug 20;23(20):7577-85. [PubMed:12930796 ]
  2. Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. doi: 10.1038/nature07762. [PubMed:19212411 ]
  3. Nicholson JK, O'Flynn MP, Sadler PJ, Macleod AF, Juul SM, Sonksen PH: Proton-nuclear-magnetic-resonance studies of serum, plasma and urine from fasting normal and diabetic subjects. Biochem J. 1984 Jan 15;217(2):365-75. [PubMed:6696735 ]
  4. Cynober LA: Plasma amino acid levels with a note on membrane transport: characteristics, regulation, and metabolic significance. Nutrition. 2002 Sep;18(9):761-6. [PubMed:12297216 ]
  5. Kersemans V, Cornelissen B, Kersemans K, Bauwens M, Achten E, Dierckx RA, Mertens J, Slegers G: In vivo characterization of 123/125I-2-iodo-L-phenylalanine in an R1M rhabdomyosarcoma athymic mouse model as a potential tumor tracer for SPECT. J Nucl Med. 2005 Mar;46(3):532-9. [PubMed:15750170 ]
  6. Sandmann J, Schwedhelm KS, Tsikas D: Specific transport of S-nitrosocysteine in human red blood cells: Implications for formation of S-nitrosothiols and transport of NO bioactivity within the vasculature. FEBS Lett. 2005 Aug 1;579(19):4119-24. [PubMed:16023102 ]
  7. Paivalainen S, Suokas M, Lahti O, Heape AM: Degraded myelin-associated glycoprotein (dMAG) formation from pure human brain myelin-associated glycoprotein (MAG) is not mediated by calpain or cathepsin L-like activities. J Neurochem. 2003 Feb;84(3):533-45. [PubMed:12558973 ]
  8. Iyer S, Leonidas DD, Swaminathan GJ, Maglione D, Battisti M, Tucci M, Persico MG, Acharya KR: The crystal structure of human placenta growth factor-1 (PlGF-1), an angiogenic protein, at 2.0 A resolution. J Biol Chem. 2001 Apr 13;276(15):12153-61. Epub 2000 Nov 7. [PubMed:11069911 ]
  9. Nishiya Y, Yoshida Y, Yoshimura M, Fukamachi H, Nakano Y: Homogeneous enzymatic assay for L-cysteine with betaC-S lyase. Biosci Biotechnol Biochem. 2005 Nov;69(11):2244-6. [PubMed:16306712 ]
  10. Santamaria I, Velasco G, Cazorla M, Fueyo A, Campo E, Lopez-Otin C: Cathepsin L2, a novel human cysteine proteinase produced by breast and colorectal carcinomas. Cancer Res. 1998 Apr 15;58(8):1624-30. [PubMed:9563472 ]
  11. Eriksson A, Tohonen V, Wedell A, Nordqvist K: Isolation of the human testatin gene and analysis in patients with abnormal gonadal development. Mol Hum Reprod. 2002 Jan;8(1):8-15. [PubMed:11756564 ]
  12. Kaminska J, Wisniewska A, Koscielak J: Chemical modifications of alpha1,6-fucosyltransferase define amino acid residues of catalytic importance. Biochimie. 2003 Mar-Apr;85(3-4):303-10. [PubMed:12770769 ]
  13. Li Y, Gamper N, Shapiro MS: Single-channel analysis of KCNQ K+ channels reveals the mechanism of augmentation by a cysteine-modifying reagent. J Neurosci. 2004 Jun 2;24(22):5079-90. [PubMed:15175377 ]
  14. Lindzen M, Gottschalk KE, Fuzesi M, Garty H, Karlish SJ: Structural interactions between FXYD proteins and Na+,K+-ATPase: alpha/beta/FXYD subunit stoichiometry and cross-linking. J Biol Chem. 2006 Mar 3;281(9):5947-55. Epub 2005 Dec 21. [PubMed:16373350 ]
  15. Norris FA, Wilson MP, Wallis TS, Galyov EE, Majerus PW: SopB, a protein required for virulence of Salmonella dublin, is an inositol phosphate phosphatase. Proc Natl Acad Sci U S A. 1998 Nov 24;95(24):14057-9. [PubMed:9826652 ]
  16. Foss CA, Mease RC, Fan H, Wang Y, Ravert HT, Dannals RF, Olszewski RT, Heston WD, Kozikowski AP, Pomper MG: Radiolabeled small-molecule ligands for prostate-specific membrane antigen: in vivo imaging in experimental models of prostate cancer. Clin Cancer Res. 2005 Jun 1;11(11):4022-8. [PubMed:15930336 ]
  17. Kozaki K, Miyaishi O, Asai N, Iida K, Sakata K, Hayashi M, Nishida T, Matsuyama M, Shimizu S, Kaneda T, et al.: Tissue distribution of ERp61 and association of its increased expression with IgG production in hybridoma cells. Exp Cell Res. 1994 Aug;213(2):348-58. [PubMed:8050492 ]
  18. Amberger VR, Hensel T, Ogata N, Schwab ME: Spreading and migration of human glioma and rat C6 cells on central nervous system myelin in vitro is correlated with tumor malignancy and involves a metalloproteolytic activity. Cancer Res. 1998 Jan 1;58(1):149-58. [PubMed:9426071 ]
  19. Zhang JT, Li QX, Wang D, Zhu ZL, Yang YH, Cui DS, Wang MW, Sun XF: Up-regulation of PINCH in the stroma of oral squamous cell carcinoma predicts nodal metastasis. Oncol Rep. 2005 Dec;14(6):1519-22. [PubMed:16273248 ]
  20. Taveau M, Bourg N, Sillon G, Roudaut C, Bartoli M, Richard I: Calpain 3 is activated through autolysis within the active site and lyses sarcomeric and sarcolemmal components. Mol Cell Biol. 2003 Dec;23(24):9127-35. [PubMed:14645524 ]
  21. Naisbitt DJ, Vilar FJ, Stalford AC, Wilkins EG, Pirmohamed M, Park BK: Plasma cysteine deficiency and decreased reduction of nitrososulfamethoxazole with HIV infection. AIDS Res Hum Retroviruses. 2000 Dec 10;16(18):1929-38. [PubMed:11153075 ]
  22. Baker DH, Czarnecki-Maulden GL: Pharmacologic role of cysteine in ameliorating or exacerbating mineral toxicities. J Nutr. 1987 Jun;117(6):1003-10. [PubMed:3298579 ]
  23. Bulaj G, Kortemme T, Goldenberg DP: Ionization-reactivity relationships for cysteine thiols in polypeptides. Biochemistry. 1998 Jun 23;37(25):8965-72. [PubMed:9636038 ]
  24. Duranton F, Cohen G, De Smet R, Rodriguez M, Jankowski J, Vanholder R, Argiles A: Normal and pathologic concentrations of uremic toxins. J Am Soc Nephrol. 2012 Jul;23(7):1258-70. doi: 10.1681/ASN.2011121175. Epub 2012 May 24. [PubMed:22626821 ]
  25. 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 ]

Only showing the first 10 proteins. There are 25 proteins in total.

Enzymes

General function:
Involved in transferase activity, transferring nitrogenous groups
Specific function:
Plays a key role in amino acid metabolism (By similarity).
Gene Name:
GOT1
Uniprot ID:
P17174
Molecular weight:
46247.14
Reactions
L-Cysteine + Oxoglutaric acid → 3-Mercaptopyruvic acid + DL-Glutamatedetails
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 transferase activity, transferring nitrogenous groups
Specific function:
Catalyzes the irreversible transamination of the L-tryptophan metabolite L-kynurenine to form kynurenic acid (KA). Plays a key role in amino acid metabolism. Important for metabolite exchange between mitochondria and cytosol. Facilitates cellular uptake of long-chain free fatty acids.
Gene Name:
GOT2
Uniprot ID:
P00505
Molecular weight:
47517.285
Reactions
L-Cysteine + Oxoglutaric acid → 3-Mercaptopyruvic acid + DL-Glutamatedetails
General function:
Involved in proteolysis
Specific function:
Broad specificity aminopeptidase. Plays a role in the final digestion of peptides generated from hydrolysis of proteins by gastric and pancreatic proteases. May play a critical role in the pathogenesis of cholesterol gallstone disease. May be involved in the metabolism of regulatory peptides of diverse cell types, responsible for the processing of peptide hormones, such as angiotensin III and IV, neuropeptides, and chemokines. Found to cleave antigen peptides bound to major histocompatibility complex class II molecules of presenting cells and to degrade neurotransmitters at synaptic junctions. Is also implicated as a regulator of IL-8 bioavailability in the endometrium, and therefore may contribute to the regulation of angiogenesis. Is used as a marker for acute myeloid leukemia and plays a role in tumor invasion. In case of human coronavirus 229E (HCoV-229E) infection, serves as receptor for HCoV-229E spike glycoprotein. Mediates as well human cytomegalovirus (HCMV) infection.
Gene Name:
ANPEP
Uniprot ID:
P15144
Molecular weight:
Not Available
Reactions
Cysteinylglycine + Water → L-Cysteine + Glycinedetails
General function:
Involved in glutamate-cysteine ligase activity
Specific function:
Not Available
Gene Name:
GCLC
Uniprot ID:
P48506
Molecular weight:
68629.42
Reactions
Adenosine triphosphate + L-Glutamic acid + L-Cysteine → ADP + Phosphate + gamma-Glutamylcysteinedetails
References
  1. Ashida H, Sawa Y, Shibata H: Cloning, biochemical and phylogenetic characterizations of gamma-glutamylcysteine synthetase from Anabaena sp. PCC 7120. Plant Cell Physiol. 2005 Apr;46(4):557-62. Epub 2005 Feb 2. [PubMed:15695431 ]
  2. Srivastava S, Chan C: Application of metabolic flux analysis to identify the mechanisms of free fatty acid toxicity to human hepatoma cell line. Biotechnol Bioeng. 2008 Feb 1;99(2):399-410. [PubMed:17615559 ]
General function:
Involved in oxidoreductase activity
Specific function:
Not Available
Gene Name:
GCLM
Uniprot ID:
P48507
Molecular weight:
30726.7
References
  1. Ashida H, Sawa Y, Shibata H: Cloning, biochemical and phylogenetic characterizations of gamma-glutamylcysteine synthetase from Anabaena sp. PCC 7120. Plant Cell Physiol. 2005 Apr;46(4):557-62. Epub 2005 Feb 2. [PubMed:15695431 ]
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-Cystathionine + Water → L-Cysteine + Ammonia + 2-Ketobutyric aciddetails
L-Cysteine + Water → Hydrogen sulfide + Pyruvic acid + Ammoniadetails
References
  1. Fiorucci S, Antonelli E, Mencarelli A, Orlandi S, Renga B, Rizzo G, Distrutti E, Shah V, Morelli A: The third gas: H2S regulates perfusion pressure in both the isolated and perfused normal rat liver and in cirrhosis. Hepatology. 2005 Sep;42(3):539-48. [PubMed:16108046 ]
  2. Zhang H, Zhi L, Moore PK, Bhatia M: Role of hydrogen sulfide in cecal ligation and puncture-induced sepsis in the mouse. Am J Physiol Lung Cell Mol Physiol. 2006 Jun;290(6):L1193-201. Epub 2006 Jan 20. [PubMed:16428267 ]
  3. Wallace JL, Dicay M, McKnight W, Martin GR: Hydrogen sulfide enhances ulcer healing in rats. FASEB J. 2007 Dec;21(14):4070-6. Epub 2007 Jul 18. [PubMed:17634391 ]
General function:
Involved in peptidase activity
Specific function:
Responsible for the biosynthesis of pyroglutamyl peptides. Has a bias against acidic and tryptophan residues adjacent to the N-terminal glutaminyl residue and a lack of importance of chain length after the second residue. Also catalyzes N-terminal pyroglutamate formation. In vitro, catalyzes pyroglutamate formation of N-terminally truncated form of APP amyloid-beta peptides [Glu-3]-beta-amyloid. May be involved in the N-terminal pyroglutamate formation of several amyloid-related plaque-forming peptides.
Gene Name:
QPCT
Uniprot ID:
Q16769
Molecular weight:
40876.14
General function:
Involved in cysteine biosynthetic process from serine
Specific function:
Only known pyridoxal phosphate-dependent enzyme that contains heme. Important regulator of hydrogen sulfide, especially in the brain, utilizing cysteine instead of serine to catalyze the formation of hydrogen sulfide. Hydrogen sulfide is a gastratransmitter with signaling and cytoprotective effects such as acting as a neuromodulator in the brain to protect neurons against hypoxic injury (By similarity).
Gene Name:
CBS
Uniprot ID:
P35520
Molecular weight:
60586.05
Reactions
L-Serine + Hydrogen sulfide → L-Cysteine + Waterdetails
References
  1. Zhang H, Zhi L, Moore PK, Bhatia M: Role of hydrogen sulfide in cecal ligation and puncture-induced sepsis in the mouse. Am J Physiol Lung Cell Mol Physiol. 2006 Jun;290(6):L1193-201. Epub 2006 Jan 20. [PubMed:16428267 ]
  2. Lowicka E, Beltowski J: Hydrogen sulfide (H2S) - the third gas of interest for pharmacologists. Pharmacol Rep. 2007 Jan-Feb;59(1):4-24. [PubMed:17377202 ]
  3. Tamizhselvi R, Moore PK, Bhatia M: Hydrogen sulfide acts as a mediator of inflammation in acute pancreatitis: in vitro studies using isolated mouse pancreatic acinar cells. J Cell Mol Med. 2007 Mar-Apr;11(2):315-26. [PubMed:17488480 ]
  4. Oh GS, Pae HO, Lee BS, Kim BN, Kim JM, Kim HR, Jeon SB, Jeon WK, Chae HJ, Chung HT: Hydrogen sulfide inhibits nitric oxide production and nuclear factor-kappaB via heme oxygenase-1 expression in RAW264.7 macrophages stimulated with lipopolysaccharide. Free Radic Biol Med. 2006 Jul 1;41(1):106-19. Epub 2006 Apr 25. [PubMed:16781459 ]
  5. Bhatia M, Wong FL, Fu D, Lau HY, Moochhala SM, Moore PK: Role of hydrogen sulfide in acute pancreatitis and associated lung injury. FASEB J. 2005 Apr;19(6):623-5. Epub 2005 Jan 25. [PubMed:15671155 ]
General function:
Involved in catalytic activity
Specific function:
Not Available
Gene Name:
GSS
Uniprot ID:
P48637
Molecular weight:
52384.325
References
  1. Tanaka T, Halicka HD, Huang X, Traganos F, Darzynkiewicz Z: Constitutive histone H2AX phosphorylation and ATM activation, the reporters of DNA damage by endogenous oxidants. Cell Cycle. 2006 Sep;5(17):1940-5. Epub 2006 Sep 1. [PubMed:16940754 ]
General function:
Involved in aminopeptidase activity
Specific function:
Presumably involved in the processing and regular turnover of intracellular proteins. Catalyzes the removal of unsubstituted N-terminal amino acids from various peptides.
Gene Name:
LAP3
Uniprot ID:
P28838
Molecular weight:
Not Available
Reactions
Cysteinylglycine + Water → L-Cysteine + Glycinedetails

Transporters

General function:
Involved in transport
Specific function:
Sodium-independent, high-affinity transport of small and large neutral amino acids such as alanine, serine, threonine, cysteine, phenylalanine, tyrosine, leucine, arginine and tryptophan, when associated with SLC3A2/4F2hc. Acts as an amino acid exchanger. Has higher affinity for L-phenylalanine than LAT1 but lower affinity for glutamine and serine. L-alanine is transported at physiological concentrations. Plays a role in basolateral (re)absorption of neutral amino acids. Involved in the uptake of methylmercury (MeHg) when administered as the L-cysteine or D,L-homocysteine complexes, and hence plays a role in metal ion homeostasis and toxicity. Involved in the cellular activity of small molecular weight nitrosothiols, via the stereoselective transport of L-nitrosocysteine (L-CNSO) across the transmembrane. Plays an essential role in the reabsorption of neutral amino acids from the epithelial cells to the bloodstream in the kidney
Gene Name:
SLC7A8
Uniprot ID:
Q9UHI5
Molecular weight:
58381.1
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 ]

Only showing the first 10 proteins. There are 25 proteins in total.