Fig. 1 | Genetics in Medicine

Fig. 1

From: Cystathionine β-synthase deficiency: Effects of betaine supplementation after methionine restriction in B6-nonresponsive homocystinuria

Fig. 1

Metabolism of homocysteine. Transsulfuration proceeds by first condensing homocysteine with serine, producing cystathionine. Hydrolysis of cystathionine by cystathionase yields cysteine and α-ketobutyrate. Remethylation of homocysteine by betaine-homocysteine methyl transferase-catalyzed transfer of an N-methyl group from betaine to homocysteine, yielding methionine and N, N-dimethylglycine.

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