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首页> 外文期刊>Pharmacology and Toxicology: An International Journal >The in vitro effects of galactose and its derivatives on rat brain Mg2+-ATPase activity.
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The in vitro effects of galactose and its derivatives on rat brain Mg2+-ATPase activity.

机译:半乳糖及其衍生物对大鼠脑Mg2 + -ATPase活性的体外影响。

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摘要

Galactosaemia is an inborn error of metabolism characterized by irreversible damage to neural tissue. To evaluate whether galactose metabolic disorders, (e.g. classical galactosaemia, galactokinase deficiency galactosaemia), is implicated for alterations of brain Mg2+-ATPase activity, various concentrations (1-16 mM) of galactose, galactose-1-phosphate, galactitol, glucose-1-phosphate or glucose were preincubated with whole brain homogenates of suckling rats at 37 degrees for 1 hr. Mg2+-ATPase activities were determined according to Bowler & Tirri's (1974). Galactose-1-phosphate or glucose-1-phosphate excessively activated the brain Mg2+-ATPase in a concentration-dependent way. Additionally, galactitol, galactose or glucose stimulated the enzyme up to 35-45% (P < 0.001) at concentrations >4 mM. A mixture of galactose-1-phosphate (2 mM), glactitol (2 mM) and galactose (4 mM), concentrations commonly found in blood and brain of untreated patients with classical galactosaemia, resulted in a 500% enzyme activation (P < 0.001) as compared to control. Moreover, a mixture of galactitol (2 mM) and galactose (1 mM), concentrations measured in patients with galactokinase deficiency, caused an enzyme stimulation (35%, P < 0.001). These findings suggest: a) The great Mg2+-ATPase activation by galactose-1-phosphate or glucose-1-phosphate may be due to the epimer of galactose and the presence of phosphorus. b) The brain Mg2+-ATPase stimulation by galactose and its derivatives could be toxic by modulating the Mg2+ concentration, the ATP availability, the activity of other ATP- and Mg2+-dependent enzymes as well as the rates of protein synthesis and cell growth.
机译:半乳糖血症是一种先天性的代谢错误,其特征是对神经组织造成不可逆的损害。为了评估半乳糖代谢异常(例如经典半乳糖血症,半乳糖激酶缺乏症半乳糖血症)是否与脑Mg2 + -ATPase活性的改变,半乳糖,半乳糖1-磷酸半乳糖,半乳糖醇,葡萄糖-1的各种浓度(1-16 mM)有关将磷酸酯或葡萄糖与乳鼠的全脑匀浆在37度下预孵育1小时。 Mg2 + -ATPase活性根据Bowler&Tirri's(1974)的方法测定。 1-半乳糖或1-葡萄糖葡萄糖以浓度依赖性方式过度激活大脑Mg2 + -ATPase。另外,半乳糖醇,半乳糖或葡萄糖在浓度> 4 mM时最多可刺激酶至35-45%(P <0.001)。半乳糖-1-磷酸半乳糖(2 mM),半乳糖醇(2 mM)和半乳糖(4 mM)的混合物在未经治疗的经典半乳糖血症患者的血液和脑中常见浓度,导致酶活化500%(P <0.001 )与对照相比。此外,在半乳糖激酶缺乏症患者中测得的半乳糖醇(2 mM)和半乳糖(1 mM)的混合物引起了酶刺激(35%,P <0.001)。这些发现表明:a)1-半乳糖或1-葡萄糖葡萄糖对Mg2 + -ATPase的极大活化可能是由于半乳糖的差向异构体和磷的存在。 b)半乳糖及其衍生物对脑中Mg2 + -ATPase的刺激作用可能是通过调节Mg2 +的浓度,ATP的利用率,其他ATP和Mg2 +依赖性酶的活性以及蛋白质合成和细胞生长的速率而引起的。

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