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MGP000718

UniProt Annotations

Entry Information
Gene Namecystathionine gamma-lyase
Protein EntryCGL_HUMAN
UniProt IDP32929
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=3; Name=1; IsoId=P32929-1; Sequence=Displayed; Name=2; IsoId=P32929-2; Sequence=VSP_006306; Name=3; IsoId=P32929-3; Sequence=VSP_047274;
Biophysicochemical PropertiesKinetic parameters: KM=0.5 mM for L-cystathionine {ECO:0000269|PubMed:10212249, ECO:0000269|PubMed:18476726}; KM=5.4 mM for homocysteine {ECO:0000269|PubMed:10212249, ECO:0000269|PubMed:18476726}; KM=3.5 mM for cysteine {ECO:0000269|PubMed:10212249, ECO:0000269|PubMed:18476726}; pH dependence: Optimum pH is 8.2. {ECO:0000269|PubMed:10212249, ECO:0000269|PubMed:18476726};
Catalytic ActivityL-cystathionine + H(2)O = L-cysteine + NH(3) + 2-oxobutanoate. {ECO:0000269|PubMed:10212249, ECO:0000269|PubMed:19019829}.
CofactorName=pyridoxal 5'-phosphate; Xref=ChEBI:CHEBI:597326; Evidence={ECO:0000269|PubMed:10212249, ECO:0000269|PubMed:18476726, ECO:0000269|PubMed:19019829};
DiseaseCystathioninuria (CSTNU) [MIM:219500]: Autosomal recessive phenotype characterized by abnormal accumulation of plasma cystathionine, leading to increased urinary excretion. {ECO:0000269|PubMed:12574942}. Note=The disease is caused by mutations affecting the gene represented in this entry.
Enzyme RegulationInhibited by propargylglycine, trifluoroalanine and aminoethoxyvinylglycine. {ECO:0000269|PubMed:10212249, ECO:0000269|PubMed:19019829}.
FunctionCatalyzes 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. {ECO:0000269|PubMed:19019829, ECO:0000269|PubMed:19261609, ECO:0000269|PubMed:22169477}.
PathwayAmino-acid biosynthesis; L-cysteine biosynthesis; L- cysteine from L-homocysteine and L-serine: step 2/2.
SimilarityBelongs to the trans-sulfuration enzymes family. {ECO:0000305}.
Subcellular LocationCytoplasm.
SubunitHomotetramer. Interacts with CALM in a calcium-dependent manner (By similarity). {ECO:0000250}.
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