Names & Taxonomy
- Uniprot ID:
- P32929
- Entry Name:
- CGL_HUMAN
- Status:
- reviewed
- Protein Names:
- Cystathionine gamma-lyase (EC 4.4.1.1) (Cysteine-protein sulfhydrase) (Gamma-cystathionase)
- Gene Names:
- CTH
- Gene Names Primary:
- CTH
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 405
- Sequence:
- MQEKDASSQGFLPHFQHFATQAIHVGQDPEQWTSRAVVPPISLSTTFKQGAPGQHSGFEYSRSGNPTRNCLEKAVAALDGAKYCLAFASGLAATVTITHLLKAGDQIICMDDVYGGTNRYFRQVASEFGLKISFVDCSKIKLLEAAITPETKLVWIETPTNPTQKVIDIEGCAHIVHKHGDIILVVDNTFMSPYFQRPLALGADISMYSATKYMNGHSDVVMGLVSVNCESLHNRLRFLQNSLGAVPSPIDCYLCNRGLKTLHVRMEKHFKNGMAVAQFLESNPWVEKVIYPGLPSHPQHELVKRQCTGCTGMVTFYIKGTLQHAEIFLKNLKLFTLAESLGGFESLAELPAIMTHASVLKNDRDVLGISDTLIRLSVGLEDEEDLLEDLDQALKAAHPPSGSHS
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Cytoplasm.
Function
- 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.
- Pathway:
- Amino-acid biosynthesis; L-cysteine biosynthesis; L-cysteine from L-homocysteine and L-serine: step 2/2.
- Catalytic Activity:
- L-cystathionine + H(2)O = L-cysteine + NH(3) + 2-oxobutanoate.
- Cofactor:
- COFACTOR: Name=pyridoxal 5'-phosphate; Xref=ChEBI:CHEBI:597326;
- Kinetics:
- BIOPHYSICOCHEMICAL PROPERTIES: Kinetic parameters: KM=0.5 mM for L-cystathionine
- Enzyme Regulation:
- ENZYME REGULATION: Inhibited by propargylglycine, trifluoroalanine and aminoethoxyvinylglycine.
- Cross Reference Drug Bank:
- DB00151
- Gene Ontology Go:
- cytoplasm
cytosol
extracellular exosome
nucleus
carbon-sulfur lyase activity
cystathionine gamma-lyase activity
homocysteine desulfhydrase activity
L-cysteine desulfhydrase activity
L-cystine L-cysteine-lyase (deaminating)
pyridoxal phosphate binding
cellular nitrogen compound metabolic process
cysteine biosynthetic process
cysteine biosynthetic process via cystathionine
cysteine metabolic process
endoplasmic reticulum unfolded protein response
hydrogen sulfide biosynthetic process
negative regulation of apoptotic signaling pathway
positive regulation of aortic smooth muscle cell differentiation
positive regulation of I-kappaB kinase/NF-kappaB signaling
positive regulation of NF-kappaB transcription factor activity
protein homotetramerization
protein sulfhydration
protein-pyridoxal-5-phosphate linkage via peptidyl-N6-pyridoxal phosphate-L-lysine
selenium compound metabolic process
small molecule metabolic process
sulfur amino acid catabolic process
sulfur amino acid metabolic process
transsulfuration - Gene Ontology Biological Process:
- cellular nitrogen compound metabolic process
cysteine biosynthetic process
cysteine biosynthetic process via cystathionine
cysteine metabolic process
endoplasmic reticulum unfolded protein response
hydrogen sulfide biosynthetic process
negative regulation of apoptotic signaling pathway
positive regulation of aortic smooth muscle cell differentiation
positive regulation of I-kappaB kinase/NF-kappaB signaling
positive regulation of NF-kappaB transcription factor activity
protein homotetramerization
protein-pyridoxal-5-phosphate linkage via peptidyl-N6-pyridoxal phosphate-L-lysine
protein sulfhydration
selenium compound metabolic process
small molecule metabolic process
sulfur amino acid catabolic process
sulfur amino acid metabolic process
transsulfuration - Gene Ontology Molecular Function:
- carbon-sulfur lyase activity
cystathionine gamma-lyase activity
homocysteine desulfhydrase activity
L-cysteine desulfhydrase activity
L-cystine L-cysteine-lyase (deaminating)
pyridoxal phosphate binding - Gene Ontology Cellular Component:
- cytoplasm
cytosol
extracellular exosome
nucleus - Keywords:
- 3D-structure
Alternative splicing
Amino-acid biosynthesis
Calmodulin-binding
Complete proteome
Cysteine biosynthesis
Cytoplasm
Disease mutation
Lyase
Polymorphism
Pyridoxal phosphate
Reference proteome - Interacts With:
- Q96NT3; Q6P9E2; Q96HA8