Lawson-Yuen Amy, Levy Harvey L
Division of Genetics, Children's Hospital Boston, Department of Pediatrics, Harvard Medical School, Boston, MA, USA.
Mol Genet Metab. 2006 Jul;88(3):201-7. doi: 10.1016/j.ymgme.2006.02.004. Epub 2006 Mar 20.
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.
同型半胱氨酸水平升高与多种医学病症有关，包括典型的同型胱氨酸尿症、多种再甲基化障碍，以及最近发现的冠状动脉疾病。甜菜碱是一种甲基供体剂，通过甲硫氨酸的再甲基化作用，有助于降低同型半胱氨酸水平。单独使用甜菜碱疗法已被证明可预防同型胱氨酸尿症中的血管事件，并且在用作辅助疗法时，可能对其他高同型半胱氨酸血症性疾病具有临床益处。甜菜碱确实会提高甲硫氨酸水平，当血浆甲硫氨酸超过1000微摩尔/升时会发生脑水肿。因此，接受甜菜碱治疗的患者必须监测血浆甲硫氨酸和同型半胱氨酸水平。
