Pattern of branched-chain α-keto acids in amniotic fluid☆
Pattern of branched-chain α-keto acids in amniotic fluid☆
复制标题
羊水中支链α-酮酸的模式☆
DOI：
10.1016/0009-8981(80)90044-3
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发表时间：
1980
影响因子：
5
通讯作者：
U. Langenbeck
中科院分区：
文献类型：
--
作者：
U. Wendel;U. Claussen;U. Langenbeck
支链谷酮酸已在羊水有机酸谱中被鉴定，但尚未被定量[11.枝链谷酮酸(α-酮基异戊酸、α-酮基异戊酸和谷酮基-甲基戊酸)及其氨基酸类似物(亮氨酸、缬氨酸和异亮氨酸)在枫糖浆尿液疾病患者体内积累是由于枝链谷酮酸脱氢酶遗传缺陷(S)[21.在患有枫糖尿疾病的胎儿怀孕期间，胎儿和羊水中支链氨基酸的正常内稳态得以维持[3]。本文报道了正常妊娠和1例枫糖尿症胎儿羊水中支链α-酮酸的变化。
Branched-chain cu-keto acids have been identified in the organic acid profile of amniotic fluid, but have not been quantified [11. The branched-chain c~-keto acids (a-ketoisocaproate, a-ketoisovalerate and cu-keto $-methylvalerate) and their amino acid analogues (leucine, valine and isoleucine) accumulate in patients with maple syrup urine disease due to an inherited deficiency of the branched-chain cu-keto acid dehydrogenase (s)[21. In a pregnancy with a fetus with maple syrup urine disease, the normal homeostasis of the branched-chain amino acids is maintained in the fetus and in amniotic fliod [3]. This report is concerned with the branched-chain a-keto acids in amniotic fluids of normal pregnancies and one pregnancy with a fetus with maple syrup urine disease.