In-utero and post-delivery supplementation of motor neuron degeneration mutant mice with polyunsaturated fatty acids does not alter the clinical or pathological course.
In-utero and post-delivery supplementation of motor neuron degeneration mutant mice with polyunsaturated fatty acids does not alter the clinical or pathological course.
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运动神经元变性突变小鼠在子宫内和分娩后补充多不饱和脂肪酸不会改变临床或病理过程。
DOI：
10.1055/s-2007-973678
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发表时间：
1997
期刊：
影响因子：
1.4
通讯作者：
Birch,DG
中科院分区：
文献类型：
--
作者：
Bennett,MJ;Boriack,RL;Birch,DG
我们研究了多不饱和脂肪酸（PUFA）补充剂在子宫内和整个生命中的mnd突变小鼠，少年神经元蜡样脂褐质沉积症（CLN-3）的拟议模型的影响。与我们早期的CLN-3人体体外研究和CLN-3淋巴母细胞体外研究不同，我们在mnd小鼠的视网膜电图、电子显微镜或临床研究中没有看到有益的效果。脑的电子显微镜检查显示了一种与CLN-3中特征性蜡样模式不一致的模式。我们的数据表明，mnd小鼠对PUFA补充没有反应，可能不是CLN-3的适当动物模型。
We have studied the effects of polyunsaturated fatty acid (PUFA) supplementation in utero and throughout life in mnd mutant mice, a proposed model for juvenile neuronal ceroid lipofuscinosis (CLN-3). Unlike our earlier in-itro studies in humans with CLN-3, and in-vitro studies in CLN-3 lympho-blasts, we saw no beneficial effects in electroretinographic, electron microscopic or clinical studies in the mnd mice. Electron microscopy of brain revealed a pattern which was not consistent with the characteristic ceroid patterns in CLN-3. Our data suggest that the mnd mouse is not responsive to PUFA supplementation and may not be an appropriate animal model for CLN-3.