Kondo A, Sendoh S, Akazawa K, Sato Y, Nagara H
Department of Neuropathology, Hizen National Mental Hospital, Saga, Japan.
Brain Res Dev Brain Res. 1992 Jun 19;67(2):217-28. doi: 10.1016/0165-3806(92)90222-i.
Early myelination in zitter rat was investigated by light and electron microscopic observations, by immunostaining for myelin basic protein (MBP), proteolipid protein (PLP) and myelin-associated glycoprotein (MAG), and by morphometric analysis from the 1st to the 28th day of age. Although the commencement of myelination in zitter rats was not delayed in comparison with control rats, the density or the number of myelinated fibers in zitter rats was significantly below that in controls, both in the ventral column of the cervical spinal cord and in the optic nerve. In contrast, the density or the number of aberrant myelin sheaths was increased in zitter rats, and this difference became more marked with increasing age. The persistent presence of abnormal membranous structures associated with the oligodendroglial nuclear membrane and the increased number of aberrant myelin sheaths were characteristic to the zitter mutation, although these alterations were also observed transiently in control rats. Quantitative analysis supported the proposition that hypomyelination in zitter rats is primarily pathological and becomes more prominent with advancing age. However, the fundamental structure of the myelin lamellae appeared to be normal and the immunoreactivities for MBP, PLP and MAG were slightly delayed and weakend in comparison with age-matched controls. Thus, in zitter rat there is the functional abnormality of the oligodendrocytes to integrate the processes of membrane biosynthesis and to expel excessive production of membranous structures associated with the membranous organellae such as nuclear membrane, and it is postulated that this functional abnormality is characteristic to only the zitter mutation.
通过光镜和电镜观察、髓鞘碱性蛋白(MBP)、蛋白脂蛋白(PLP)和髓鞘相关糖蛋白(MAG)的免疫染色以及形态计量分析,对颤搐鼠从出生第1天到第28天的早期髓鞘形成进行了研究。尽管与对照鼠相比,颤搐鼠的髓鞘形成开始时间并未延迟,但在颈脊髓腹侧柱和视神经中,颤搐鼠有髓纤维的密度或数量均显著低于对照鼠。相反,颤搐鼠中异常髓鞘的密度或数量增加,且这种差异随年龄增长而更加明显。与少突胶质细胞核膜相关的异常膜结构持续存在以及异常髓鞘数量增加是颤搐突变的特征,尽管在对照鼠中也短暂观察到这些改变。定量分析支持了颤搐鼠髓鞘形成不足主要是病理性的且随年龄增长而更加突出这一观点。然而,髓鞘板层的基本结构似乎正常,与年龄匹配的对照相比,MBP、PLP和MAG的免疫反应性稍有延迟且减弱。因此,在颤搐鼠中,少突胶质细胞在整合膜生物合成过程以及排出与核膜等膜性细胞器相关的过量膜结构产生方面存在功能异常,据推测这种功能异常是颤搐突变所特有的。