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

More general terms

• lyase
• phosphoprotein

Additional terms

• cystathionine
• cysteine
• methionine metabolism (trans-sulfuration pathway)

Database

• UniProt: http://www.uniprot.org/uniprot/P32929.html
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:1491
• OMIM: https://mirror.omim.org/entry/219500
• OMIM: https://mirror.omim.org/entry/607657