Seven different CBS mutants can produce thioethers from cysteine and homocysteine.
Metabolism of thioethers is deranged in disorders of sulfur amino acid metabolism.
Cystathionine and lanthionine are decreased in plasma of CBS deficient patients.
In homocystinurias homolanthionine produced by CTH correlates with total homocysteine.
All these data suggest altered hydrogen sulfide metabolism in homocystinurias.