Cystathionine

Cystathionine

Names
IUPAC name S-((R)-2-amino-2-carboxyethyl)-L-homocysteine
Other names L-Cystathionine; S-[(2R)-2-Amino-2-carboxyethyl]-L-homocysteine
Identifiers
CAS Number	56-88-2
3D model (JSmol)	Interactive image
ChEBI	CHEBI:17755
ChEMBL	ChEMBL209241
ChemSpider	388392
ECHA InfoCard	100.000.269
KEGG	C00542
MeSH	Cystathionine
PubChem CID	439258
UNII	375YFJ481O
CompTox Dashboard (EPA)	DTXSID20971384
InChI 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 Key: ILRYLPWNYFXEMH-WHFBIAKZSA-N InChI=1/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) Key: ILRYLPWNYFXEMH-UHFFFAOYAH
SMILES C(CSC[C@@H](C(=O)O)N)[C@@H](C(=O)O)N
Properties
Chemical formula	C7H14N2O4S
Molar mass	222.26 g·mol−1
Except where otherwise noted, data are given for materials in their standard state (at 25 °C [77 °F], 100 kPa).

Cystathionine is an intermediate in the synthesis of cysteine.

Cystathionine is produced by the transsulfuration pathway which converts homocysteine into cystathionine. Cystathionine is then used by the enzymes cystathionine gamma-lyase (CTH), cysteine dioxygenase (CDO), and sulfinoalanine decarboxylase to produce hypotaurine and then taurine.^[1]

Alternately, the cysteine from the cystathionine gamma-lyase can be used by the enzymes glutamate–cysteine ligase (GCL) and glutathione synthetase (GSS) to produce glutathione.

An excess of cystathionine in the urine is called cystathioninuria.

Biosynthetically, cystathionine is generated from homocysteine and serine by cystathionine beta synthase (upper reaction in the diagram below). It is then cleaved into cysteine and α-ketobutyrate by cystathionine gamma-lyase (lower reaction).

Cysteine metabolism. Cystathionine beta synthase catalyzes the upper reaction and cystathionine gamma-lyase catalyzes the lower reaction.

References