Mendell J R, Sahenk Z, Saida K, Weiss H S, Savage R, Couri D
Brain Res. 1977 Sep 9;133(1):107-18. doi: 10.1016/0006-8993(77)90052-x.
Methyl n-butyl ketone (MBK) is known to produce a giant axonal neuropathy in man and experimental animals characterized pathologically by a gradual increase in the number of neurofilaments which become associated with focal areas of axonal swelling and thinning of the myelin sheath. Fast axoplasmic transport was studied in rats exposed to MBK. In 10 severely paralyzed rats exposed to MBK there was a significant impediment of fast axoplasmic transport following dorsal root ganglion injections (x +/- S.D. = 283.2 +/- 20.34 mm/day) compared to normal controls (417.6 +/- 23.78 mm/day). In rats undergoing injections into the ventral horn of the spinal cord there was a gradual impairment of the mean down flow rate for transport of [3H]leucine which correlated with the severity of the MBK induced neuropathy. Quantitative morphological determinations showed that the total number of neurotubules per unit cross-sectional myelin area and the number of neurotubules associated with mitochondria in swollen axons was unchanged from normal. The total number of mitochondria in randomly sampled axons varied significantly from controls but the absolute number of mitochondria associated with neurotubules was unchanged from normal. The results of these studies suggest that the impediment of fast axoplasmic transport may be related to the increased neurofilaments producing focal areas of axonal blockage.
已知甲基正丁基酮（MBK）会在人类和实验动物中引发巨大轴索性神经病，其病理特征为神经丝数量逐渐增加，这些神经丝与轴突肿胀和髓鞘变薄的局部区域相关。对暴露于MBK的大鼠的快速轴浆运输进行了研究。在10只暴露于MBK的严重瘫痪大鼠中，与正常对照组（417.6±23.78毫米/天）相比，在背根神经节注射后，快速轴浆运输存在显著障碍（x±标准差=283.2±20.34毫米/天）。在向脊髓腹角注射的大鼠中，[3H]亮氨酸运输的平均下行流速逐渐受损，这与MBK诱导的神经病的严重程度相关。定量形态学测定表明，每单位横截面积髓鞘区域的神经微管总数以及肿胀轴突中与线粒体相关的神经微管数量与正常情况相比没有变化。随机抽样轴突中的线粒体总数与对照组相比有显著差异，但与神经微管相关的线粒体绝对数量与正常情况相比没有变化。这些研究结果表明，快速轴浆运输的障碍可能与产生轴突阻塞局部区域的神经丝增加有关。
