甘氨酸脑病治疗策略。
A strategy for glycine encephalopathy therapy.
作者信息
Mendelson I S
出版信息
J Ment Defic Res. 1982 Jun;26 (Pt 2):107-10. doi: 10.1111/j.1365-2788.1982.tb00134.x.
An inherited defect in the glycine cleavage enzyme results in the condition of neonatal glycine encephalopathy which has not responded to the current innovative methods of therapy. A re-examination of the enzyme structure and metabolic pathways, leads us to recommend future clinical evaluation of (1) vitamin-responsiveness, e.g., pyridoxine, folate and lipoic acid, (2) methionine, (3) N5, N10-methylene tetrahydrofolate and (4) alpha-methylserine therapy during the critical period of neonatal brain growth and development.
甘氨酸裂解酶的遗传性缺陷会导致新生儿甘氨酸脑病，目前的创新治疗方法对此病并无疗效。对该酶结构和代谢途径的重新审视，使我们建议在新生儿脑生长发育的关键时期，对以下方面进行未来的临床评估：（1）维生素反应性，如吡哆醇、叶酸和硫辛酸；（2）蛋氨酸；（3）N5，N10-亚甲基四氢叶酸；（4）α-甲基丝氨酸疗法。
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