Article
The effect of dietary modulation of sulfur amino acids on cystathionine β synthase-deficient mice.
Annals of the New York Academy of Sciences - 1 Jan 2016
Kruger Warren D, Gupta Sapna
Abstract excerpt
Cystathionine β synthase (CBS) is a key enzyme in the methionine and cysteine metabolic pathway, acting as a metabolic gatekeeper to regulate the flow of fixed sulfur from methionine to cysteine. Mutations in the CBS gene cause clinical CBS deficiency, a disease characterized by elevated plasma total homocysteine (tHcy) and methionine and decreased plasma cysteine. The treatment goal for CBS-deficient patients is...
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