Bennett M J, Boriack R L, Birch D G
Department of Pathology and Pediatrics, University of Texas Southwestern Medical Center, Children's Medical Center of Dallas 75235, USA.
Neuropediatrics. 1997 Feb;28(1):82-4. doi: 10.1055/s-2007-973678.
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-vitro studies in humans with CLN-3, and in-vitro studies in CLN-3 lymphoblasts, 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.
我们研究了在子宫内及整个生命过程中补充多不饱和脂肪酸（PUFA）对mnd突变小鼠的影响，mnd突变小鼠是一种被认为可用于研究青少年神经元蜡样脂褐质沉积症（CLN - 3）的模型。与我们早期对患有CLN - 3的人类进行的体外研究以及对CLN - 3淋巴母细胞进行的体外研究不同，我们在mnd小鼠的视网膜电图、电子显微镜或临床研究中未观察到有益效果。对大脑进行电子显微镜检查发现的一种模式与CLN - 3中典型的蜡样模式不一致。我们的数据表明，mnd小鼠对补充PUFA无反应，可能不是CLN - 3的合适动物模型。
