The use of betaine in the treatment of elevated homocysteine.
Elevation of homocysteine is implicated in multiple medical conditions, including classical homocystinuria, a variety of remethylation disorders, and most recently in coronary artery disease. Betaine is a methyl donor agent that is beneficial in lowering homocysteine through the remethylation of methionine. Betaine therapy alone has been shown to prevent vascular events in homocystinuria and may have clinical benefits in other hyperhomocysteinemic disorders when used as adjunctive therapy. Betaine does raise the methionine level and cerebral edema has occurred when plasma methionine exceeds 1000 micromol/L. Thus the plasma methionine as well as homocysteine must be monitored in patients receiving betaine.
Division of Genetics, Children's Hospital Boston, Department of Pediatrics, Harvard Medical School, Boston, MA, USA.
Amy
A
Lawson-Yuen
Harvey L
HL
Levy
D000592:Amino Acid Metabolism, Inborn Errors / Q000188:drug therapy*; D001622:Betaine / Q000627:therapeutic use*; D006710:Homocysteine / Q000097:blood*; D006712:Homocystinuria / Q000188:drug therapy; D006801:Humans; D020138:Hyperhomocysteinemia / Q000188:drug therapy; D008715:Methionine / Q000378:metabolism; D008745:Methylation
D006710:Homocysteine; D001622:Betaine; D008715:Methionine