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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-05-30 20:55:54 UTC
HMDB IDHMDB0000099
Secondary Accession Numbers
  • HMDB0003614
  • HMDB00099
  • HMDB03614
Metabolite Identification
Common NameL-Cystathionine
DescriptionCystathionine is a dipeptide formed by serine and homocysteine. Cystathioninuria is a prominent manifestation of vitamin-B6 deficiency. The transsulfuration of methionine yields homocysteine, which combines with serine to form cystathionine, the proximate precursor of cysteine through the enzymatic activity of cystathionase. In conditions in which cystathionine gamma-synthase or cystathionase is deficient, for example, there is cystathioninuria. Although cystathionine has not been detected in normal human serum or plasma by most conventional methods, gas chromatographic/mass spectrometric methodology detected a mean concentration of cystathionine in normal human serum of 140 nM, with a range of 65 to 301 nM. Cystathionine concentrations in CSF have been 10, 1, and 0.5 uM, and "not detected". Only traces (i.e., <1 uM) of cystathionine are present in normal CSF.587. Gamma-cystathionase deficiency (also known as Cystathioninuria), which is an autosomal recessive disorder (NIH: 2428), provided the first instance in which, in a human, the major biochemical abnormality due to a defined enzyme defect was clearly shown to be alleviated by administration of large doses of pyridoxine. The response in gamma-cystathionase-deficient patients is not attributable to correction of a preexisting deficiency of this vitamin (OMMBID, Chap. 88).
Structure
Thumb
Synonyms
ValueSource
(R)-S-(2-Amino-2-carboxyethyl)-L-homocysteineChEBI
L-(+)-CystathionineChEBI
S-(beta-Amino-beta-carboxyethyl)homocysteineChEBI
S-(b-Amino-b-carboxyethyl)homocysteineGenerator
S-(Β-amino-β-carboxyethyl)homocysteineGenerator
CystathionineHMDB
S-[(2R)-2-Amino-2-carboxyethyl]-L-homocysteineHMDB
[R-(R*,s*)]-2-amino-4-[(2-amino-2-carboxyethyl)thio]-butanoateHMDB
[R-(R*,s*)]-2-amino-4-[(2-amino-2-carboxyethyl)thio]-butanoic acidHMDB
Chemical FormulaC7H14N2O4S
Average Molecular Weight222.262
Monoisotopic Molecular Weight222.067427636
IUPAC Name(2S)-2-amino-4-{[(2R)-2-amino-2-carboxyethyl]sulfanyl}butanoic acid
Traditional NameL-cystathionine
CAS Registry Number56-88-2
SMILES
N[C@@H](CCSC[C@H](N)C(O)=O)C(O)=O
InChI Identifier
InChI=1S/C7H14N2O4S/c8-4(6(10)11)1-2-14-3-5(9)7(12)13/h4-5H,1-3,8-9H2,(H,10,11)(H,12,13)/t4-,5-/m0/s1
InChI KeyILRYLPWNYFXEMH-WHFBIAKZSA-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
  • Thia fatty acid
  • Dicarboxylic acid or derivatives
  • Fatty acyl
  • Fatty acid
  • Amino acid
  • Carboxylic acid
  • Thioether
  • Dialkylthioether
  • Sulfenyl compound
  • Organic nitrogen compound
  • Hydrocarbon derivative
  • Organic oxide
  • Primary amine
  • Organosulfur compound
  • Organooxygen compound
  • Organonitrogen compound
  • Primary aliphatic amine
  • Organopnictogen compound
  • Organic oxygen compound
  • Carbonyl group
  • Amine
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effectNot Available
Disposition
Biological locationSource
Process
Role
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point312 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility22 mg/mLNot Available
LogPNot AvailableNot Available
Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct TypeData SourceCCS Value (Å2)Reference
[M-H]-Baker144.40530932474
[M-H]-MetCCS_train_neg139.51430932474
[M+H]+MetCCS_train_pos147.03930932474
[M-H]-Not Available142.1http://allccs.zhulab.cn/database/detail?ID=AllCCS00000006
[M+H]+Not Available147.3http://allccs.zhulab.cn/database/detail?ID=AllCCS00000006
Predicted Molecular Properties
Predicted Chromatographic Properties
Spectra
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Urine
Tissue Locations
  • Brain
  • Kidney
  • Liver
  • Prostate
Pathways
Normal Concentrations
Abnormal Concentrations
Associated Disorders and Diseases
Disease References
Folate deficiency
  1. Stabler SP, Lindenbaum J, Savage DG, Allen RH: Elevation of serum cystathionine levels in patients with cobalamin and folate deficiency. Blood. 1993 Jun 15;81(12):3404-13. [PubMed:8507876 ]
Vitamin B12 deficiency
  1. Stabler SP, Lindenbaum J, Savage DG, Allen RH: Elevation of serum cystathionine levels in patients with cobalamin and folate deficiency. Blood. 1993 Jun 15;81(12):3404-13. [PubMed:8507876 ]
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 ]
Autism
  1. James SJ, Cutler P, Melnyk S, Jernigan S, Janak L, Gaylor DW, Neubrander JA: Metabolic biomarkers of increased oxidative stress and impaired methylation capacity in children with autism. Am J Clin Nutr. 2004 Dec;80(6):1611-7. [PubMed:15585776 ]
Hypermethioninemia
  1. Baric I, Fumic K, Glenn B, Cuk M, Schulze A, Finkelstein JD, James SJ, Mejaski-Bosnjak V, Pazanin L, Pogribny IP, Rados M, Sarnavka V, Scukanec-Spoljar M, Allen RH, Stabler S, Uzelac L, Vugrek O, Wagner C, Zeisel S, Mudd SH: S-adenosylhomocysteine hydrolase deficiency in a human: a genetic disorder of methionine metabolism. Proc Natl Acad Sci U S A. 2004 Mar 23;101(12):4234-9. Epub 2004 Mar 15. [PubMed:15024124 ]
Crohn's disease
  1. Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
Ulcerative colitis
  1. Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
Cystathioninuria
  1. Sugahara K, Ohta J, Takemura M, Kodama H: Determination of cystathionine and perhydro-1,4-thiazepine-3,5-dicarboxylic acid in the urine of a patient with cystathioninuria using column liquid chromatography-mass spectrometry. J Chromatogr. 1992 Sep 2;579(2):318-23. [PubMed:1429979 ]
  2. Espinos C, Garcia-Cazorla A, Martinez-Rubio D, Martinez-Martinez E, Vilaseca MA, Perez-Duenas B, Kozich V, Palau F, Artuch R: Ancient origin of the CTH alelle carrying the c.200C>T (p.T67I) variant in patients with cystathioninuria. Clin Genet. 2010 Dec;78(6):554-9. doi: 10.1111/j.1399-0004.2010.01431.x. [PubMed:20584029 ]
  3. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Eosinophilic esophagitis
  1. Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Associated OMIM IDs
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB001976
KNApSAcK IDC00007498
Chemspider ID388392
KEGG Compound IDC02291
BioCyc IDL-CYSTATHIONINE
BiGG ID39523
Wikipedia LinkCystathionine
METLIN ID39
PubChem Compound439258
PDB IDNot Available
ChEBI ID17482
Food Biomarker OntologyNot Available
VMH IDCYST_L
MarkerDB IDMDB00000052
Good Scents IDNot Available
References
Synthesis ReferenceYamagata, Shuzo; Akamatsu, Tsuyoshi; Iwama, Tomonori. Immobilization of Saccharomyces cerevisiae cystathionine gamma-lyase and application of the product to cystathionine synthesis. Applied and Environmental Microbiology (2004), 70(6), 3766-3768.
Material Safety Data Sheet (MSDS)Not Available
General References

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-Cystathionine + Water → L-Cysteine + Ammonia + 2-Ketobutyric aciddetails
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
Serine + Homocysteine → L-Cystathionine + Waterdetails