Record Information
Version1.0
StatusDetected and Quantified
Creation Date2020-12-10 18:39:04 UTC
Update Date2024-04-30 19:32:37 UTC
Metabolite IDMMDBc0000495
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
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
Molecular FormulaC7H14N2O4S
Average Mass222.262
Monoisotopic Mass222.067427636
IUPAC Name(2S)-2-amino-4-{[(2R)-2-amino-2-carboxyethyl]sulfanyl}butanoic acid
Traditional NameL-cystathionine
CAS Registry NumberNot Available
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