Lipman R D, Donohue L R, Hoppe P, Bronson R T
Jean Mayer USDA Human Nutrition Research Center on Aging at Tufts University, Boston, MA 02111, USA.
Neurosci Lett. 1996 Nov 22;219(2):111-4. doi: 10.1016/s0304-3940(96)13185-2.
The motor neuron degeneration (mnd) mouse has been documented to accumulate proteolipid and thus is a model of neuronal ceroid lipofuscinosis [Dunn, W.A., Raizada, M.K., Vogt, E.S. and Brown, E.A., Int. J. Dev. Neurosci., 12 (1994) 185-196; Faust, J.R., Rodman, J.S., Daniel, P.F., Dice, J.F. and Bronson, R.T., J. Biol. Chem., 269 (1994) 10150-10155]. While accumulation of proteolipid in the hippocampus of chimeric mice composed of mnd and +/+ cells was found to be proportional to the contribution of mnd in the brain, accumulation within individual cells was the same for cells from chimeric and age-matched mnd mice. Bone marrow transplantation was used to altering the milieu of circulating factors to determine whether this might modify the disease phenotype in mnd mice. Transplantation of bone marrow in neonatal or young mice did not reduce the age-associated accumulation of proteolipid within hippocampal neurons. The results of these experiments indicate that mnd results in a cell autonomous defect.
运动神经元变性（mnd）小鼠已被证明会积累蛋白脂质，因此是神经元蜡样脂褐质沉积症的模型[邓恩，W.A.，拉伊扎达，M.K.，沃格特，E.S.和布朗，E.A.，《国际发育神经科学杂志》，12（1994）185 - 196；福斯特，J.R.，罗德曼，J.S.，丹尼尔，P.F.，戴斯，J.F.和布朗森，R.T.，《生物化学杂志》，269（1994）10150 - 10155]。虽然在由mnd和+/+细胞组成的嵌合小鼠海马中蛋白脂质的积累被发现与mnd在大脑中的贡献成比例，但嵌合小鼠和年龄匹配的mnd小鼠细胞内的积累是相同的。骨髓移植被用于改变循环因子的环境，以确定这是否可能改变mnd小鼠的疾病表型。在新生或幼年小鼠中进行骨髓移植并没有减少海马神经元内与年龄相关的蛋白脂质积累。这些实验结果表明，mnd导致细胞自主缺陷。
