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cystathionine gamma-lyase (gamma-cystathionase, CTH)
Function:
- amino-acid biosynthesis; L-cysteine biosynthesis
- L-cysteine from L-homocysteine & L-serine: step 2/2
- enzyme of trans-sulfuration pathway in the conversion of methionine into cysteine final step
- phosphorylated upon DNA damage, probably by ATM or ATR
L-cystathionine + H2O L-cysteine + NH3 + alpha-ketobutyrate
Cofactor: pyridoxal phosphate
Structure:
- homotetramer (putative)
- belongs to the trans-sulfuration enzymes family
Compartment: cytoplasm
Alternative splicing: named isoforms=2
Pathology:
- defects in CTH are the cause of cystathioninuria
Interactions
molecular events
Related
cystathionine
cysteine
methionine metabolism (trans-sulfuration pathway)
General
lyase
phosphoprotein
Properties
SIZE: entity length = 405 aa
MW = 45 kD
COMPARTMENT: cytoplasm
MOTIF: Ser phosphorylation site {S8}
cofactor-binding site [212-212]
FOR-BINDING-OF: pyridoxal phosphate
Ser phosphorylation site {S282}
Ser phosphorylation site {S377}
Database Correlations
OMIM correlations
MORBIDMAP 607657
UniProt P32929
Pfam PF01053
Kegg hsa:1491
ENZYME 4.4.1.1
References
- OMIM :accession 219500
- Stryer Biochemistry WH Freeman & Co, New York,
1988, pg 583
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=CTH
- UniProt :accession P32929