HIDDEN SOURCES OF GALACTOSE IN THE ENVIRONMENT
HIDDEN SOURCES OF GALACTOSE IN THE ENVIRONMENT
复制标题
DOI：
10.1007/bf02143811
复制
发表时间：
1995-01-01
影响因子：
3.6
通讯作者：
GROSS, KC
中科院分区：
文献类型：
--
作者：
ACOSTA, PB;GROSS, KC
不含乳糖的半乳糖限制性饮食可挽救半乳糖-1-磷酸尿苷酰转移酶（GALT）缺乏症患者的生命，但不能预防长期并发症，如发育迟缓、言语异常、生长不良以及女性卵巢衰竭。乳糖存在于乳制品中，并作为药物中的补充剂，被认为是饮食中半乳糖的主要来源。最近的两篇出版物报告说，许多水果和蔬菜中含有少量的半乳糖。我们报告存在大量的游离半乳糖在一些豆类（干豆和豌豆）和存在的结合半乳糖在许多食品植物。半乳糖以各种糖苷键，如α-1，6、β-1，3和β-1，4，并作为脂质的组分，在动物和植物中普遍存在。α-1，6和β-1，3连接的半乳糖在食物中的生物利用度是未知的。然而，在植物和动物组织中发现的α-半乳糖苷酶可以以α-1，6键和从二半乳糖基二酰基甘油释放半乳糖。在动物和植物组织中，β-半乳糖苷酶可以释放β-1，4键和单半乳糖基二酰基甘油形式的半乳糖。用于制备或保存目的的微生物发酵食品可能含有游离半乳糖。谷物、水果、豆类、坚果、器官肉、种子和蔬菜中的游离和结合半乳糖在某些GALT缺乏症患者不良结局中的作用尚不清楚。可以肯定的是，没有GALT缺乏症患者曾经摄入过无半乳糖的饮食。
A galactose-restricted diet free of lactose is lifesaving in patients with galactose-1-phosphate uridyl transferase (GALT) deficiency, but does not prevent long term complications such as developmental delay, abnormal speech, poor growth and, in females, ovarian failure. Lactose, found in dairy products and as an extender in drugs, has been considered the primary source of galactose in the diet. Two recent publications reported that small amounts of galactose are present in many fruits and vegetables. We report the presence of considerable amounts of free galactose in some legumes (dried beans and peas) and the presence of bound galactose in many food plants. Galactose, in various glycosidic linkages, such as alpha-1,6, beta-1,3 and beta-1,4, and as a component of lipids, is ubiquitous in animals and plants. The bioavailability of alpha-1,6 and beta-1,3 linked galactose in foods is unknown. However, alpha-galactosidases found in plant and animal tissues may release galactose in alpha-1,6 linkage, and from digalactosyldiacylglycerol. Galactose in beta-1,4 linkage and as monogalactosyldiacylglycerol may be released by beta-galactosidases in animal and plant tissues. Foods fermented by microorganisms for preparation or preservation purposes may contain free galactose. The role of free and bound galactose in cereals, fruits, legumes, nuts, organ meats, seeds, and vegetables in the poor outcome seen in some patients with GALT deficiency is unknown. It is certain that no patients with GALT deficiency have ever ingested a galactose-free diet.