CYSTADANE ACTS AS A METHYL GROUP DONOR IN THE REMETHYLATION OF HOMOCYSTEINE TO METHIONINE IN PATIENTS WITH HOMOCYSTINURIA. AS A RESULT, TOXIC BLOOD LEVELS OF HOMOCYSTEINE ARE REDUCED IN THESE PATIENTS, USUALLY TO 20-30 PERCENT OR LESS OF PRE-TREATMENT LEVELS. BETAINE WAS OBSERVED TO LOWER PLASMA HOMOCYSTEINE LEVELS IN THE THREE TYPES OF HOMOCYSTINURIA, I.E., CYSTATHIONINE BETA-SYNTHASE (CBS) DEFICIENCY; 5,10-METHYLENETETRAHYDROFOLATE REDUCTASE (MTHFR) DEFICIENCY; AND COBALAMIN COFACTOR METABOLISM (CBL) DEFECT.
BETAINE OCCURS NATURALLY IN THE BODY. IT IS A METABOLITE OF CHOLINE AND IS PRESENT IN SMALL AMOUNTS IN FOODS SUCH AS BEETS, SPINACH, CEREALS, AND SEAFOOD